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",isbn:"978-1-80356-777-8",printIsbn:"978-1-80356-776-1",pdfIsbn:"978-1-80356-778-5",doi:null,price:0,priceEur:0,priceUsd:0,slug:null,numberOfPages:0,isOpenForSubmission:!1,isSalesforceBook:!1,isNomenclature:!1,hash:"84908e027f884ec3fcbaea42eb69b698",bookSignature:"Dr. Hayri Baytan Ozmen",publishedDate:null,coverURL:"https://cdn.intechopen.com/books/images_new/11524.jpg",keywords:"Computational Intelligence, Fuzzy Clustering, Fuzzy Sets Theory, Genetic Algorithm, Neural Network, Artificial Intelligence, Decision Making, Control Theory, Computer-Aided Diagnosis, Fuzzy Optimization, Pattern Recognition, Feature Extraction",numberOfDownloads:null,numberOfWosCitations:0,numberOfCrossrefCitations:null,numberOfDimensionsCitations:null,numberOfTotalCitations:null,isAvailableForWebshopOrdering:!0,dateEndFirstStepPublish:"April 1st 2022",dateEndSecondStepPublish:"April 29th 2022",dateEndThirdStepPublish:"June 28th 2022",dateEndFourthStepPublish:"September 16th 2022",dateEndFifthStepPublish:"November 15th 2022",dateConfirmationOfParticipation:null,remainingDaysToSecondStep:"2 months",secondStepPassed:!0,areRegistrationsClosed:!0,currentStepOfPublishingProcess:4,editedByType:null,kuFlag:!1,biosketch:"Researcher with more than sixty-five research papers published in international journals and has been involved in more than ten national and international research projects. 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Ear prostheses (which in common parlance are called “artificial ear”) are an example of progress in this area. They are commonly used in patients with microtia, which is a congenital malformation characterized by the underdevelopment of an ear or two and which is usually broken down into four categories:
\nGrade I is a slightly small ear with identifiable structures and a small but present external ear canal.
Grade II is a partially formed ear, usually with a closed off or stenotic external ear canal producing a conductive hearing loss.
Grade III is the absence of the external ear with a small “peanut” skin and cartilage structure and the absence of the external ear canal and ear drum.
Grade IV is absence of the total ear (anotia).
In addition to the psychologic effect of a grossly deformed ear, the child or adult with microtia has moderate to severe conductive hearing loss. Although those patients have a normal inner ear, it is frequently observed an absence of the external ear (or pinna), an absent external canal and ear drum as well as a smaller middle ear cavity, fused middle ear bones (ossicles), and an open (patent) eustachian tube. Therefore, these patients have a maximum conductive hearing loss. Because it is critical for these patients to have normal hearing in order to have normal speech and bilateral hearing in order to detect directionality, ear prostheses have become the most recommendable alternative, allowing the patient to recover both functionality and appearance of a natural ear [1].
\nThis chapter will present the results including the stages of design, fabrication, and characterization of a 3D-printed ear prosthesis using PVDF, a polymeric smart material which is used as either a sensor or a transducer due to its high piezoelectric, pyroelectric, photopyroelectric, and ferroelectric properties [2–6].
\nPVDF is the most significant and studied polymer by Lovinger, “Ferroelectric Polymers” in
The prosthesis was subjected to pressure, temperature, light, and acoustic stimuli. Thus, the response of these stimuli is presented. This way it is expected to restore sensory function, giving the user the ability to perceive heat, cold, touch, and pressure ae did before as well as acoustic waves and light.
\nAn ear prosthesis was created according to anthropometric parameters. It was designed using a 3D computer-aided design (CAD) software. After that, the prosthesis was manufactured with a 3D printer from bits from bytes, model 3D touch with a Z-axis resolution of 0.125 mm (0.005″/125 microns), nozzle has a diameter of 0.3 mm, using the fused deposition modeling (FDM) process and printed in PVDF (C2H2F2) which is a smart material that exhibits piezoelectric and pyroelectric properties. PVDF used was pure and bought in rods with 3 mm of diameter, from Goodfellow Limited Cambridge (FV307910), and this PVDF is subjected to strict quality control to assure repeatability in its ferroelectric properties, that is, piezoelectric and pyroelectric properties of the printed ear are not affected.
\nTypes of 3D printers:
\nStereolithography (SLA)
Fused deposition modeling (FDM)
Selective laser sintering (SLS)
Selective laser melting (SLM)
Electronic beam melting (EBM)
Laminobject manufacturing (LOM)
PVDF is polymorphic and has a crystalline phase (ß-phase). The ß-phase is the most relevant phase for practical ferroelectric, piezoelectric, and pyroelectric applications. Several methods are commonly used in order to achieve this phase, such as stretching the material, applying a voltage of at least 0.5 MV/cm, or using plasma or corona poling [7].
\nThe ear prosthesis made of PVDF was poled by corona poling, where a high voltage (15 kV) was applied between a needle and a plate, situated 1 cm apart. PVDF prosthesis was placed over the plate, so the molecules of this polymer are aligned [7, 10].
\nIn this stage, the ear prosthesis was characterized by means of applying different stimuli such as pressure, heat, and cold (imitating human skin receptors) and also acoustic waves and light, that is, temperature, sound, pressure, and light (TSPL) stimuli, in order to register different responses of the PVDF prosthesis as a multisensory unit. Figure 1 shows the general diagram of the experiments that are mentioned above.
\nDiagram of different applied stimuli TSPL to the PVDF prosthesis.
In order to measure ferroelectric hysteresis loop, the Sawyer–Tower circuit was implemented for this study (Figure 2). By measuring voltage (
Measure of ferroelectric hysteresis loop using the Sawyer–Tower circuit.
In this experiment, a sound source was used as a sound emitter (a) excited by an audio generator at 60 Hz. As the receiver, it was used a face of the PVDF prosthesis which was metalized (mirror finish) (b) by in situ thermal evaporation of aluminum in a vacuum chamber at 10−4 mmHg. A sound source was situated 5 mm from the speaker; at the same time, it was reflected a red laser beam of helium–neon at 633 nm, JDSU model 1145AP (c) which is detected by a light detector, light-to-frequency converter TSL230 from Texas Instruments (d); thus obtaining two electric signals, one from PVDF and another from the light detector in the oscilloscope, Tektronix model MSO3014, (e) displayed. Figure 3 shows a schematic diagram of the experiment mentioned above.
\n(a) Sound source, (b) section of PVDF (mirror finish), (c) laser HeNe 633 nm, (d) light detector, and (e) oscilloscope.
The interest of recording photopyroelectric current (Y-axis) is to show that the PVDF used in the manufacture of hearing aid responds to light, PVDF was stimulated with a modulated laser light (electronically chopped by the internal oscillator) with a maximum power of 150 mW through an optical fiber with a wave length λ of 650 nm (laser BWTEK model BWF-650-15E/55369). Two frequency sweeps were carried out, one from 0 to 10 Hz and the other from 0 to 100 Hz [frequency (X-axis)] were used. This experimental arrangement is based on Balderas-Lopez and Mandelis “New Technique for Precise Measurements of the thermal Effusivity of Transparent Liquids,” 2003 [11] and Mandelis and Wang “A Novel PVDF Thin-Film Photopyroelectric Thermal-Wave Interferometry,” 2000 [12]. Our group have compared and discussed the use of PVDF with the ferroelectric ceramic PLZT as a pyroelectric sensor, “Comparative performance of PLZT and PVDF Sensors Used Phyroelectric to the Thermal Characterization of Liquid Samples,” 2013 [13].
\nIn order to evaluate photopyroelectric response of the printed PVDF, the experimental arrangement of Figure 4 was done. The printed PDVF prosthesis (c) was excited with a laser system, BWTEK model BWF-650-15E/55369 λ = 650 nm (e). The laser beam was modulated under two ranges: from 0 to 100 Hz and from 0 to 10 Hz. The intensity of the laser is a function of the emitter current. To register the photopyroelectric response of the printed PVDF prosthesis, a current preamplifier, Standford Research Systems model SR570 and an oscilloscope, Tektronix model MSO3014, were used.
\nExperimental arrangement of the photopyroelectric response of printed PVDF. (a) Oscilloscope, (b) current preamplifier, (c) printed PVDF prosthesis, (d) fiber optic, (e) laser system, and (f) function generator.
The prosthesis was tested as a pressure sensor with different pressure loads between 0 and 16.35 kPa using a certified weight set from OHAUS. The prosthesis was set in horizontal position, and weights between 25 g and 3 kg were placed over the printed ear as shown in Figure 5.
\nExample of pressure characterization.
From Newton’s second law (Eq. 1), we have a relation between force (F) and mass (m).
where
\nF = force (N)
m = mass (kg)
a = 9.81 m/s2
Equation (2) was used in order to determine the pressure generated over the ear prosthesis.
where
\nF = force (N)
A = area of the surface of the ear on contact with the weights (m2).
Regarding to temperature characterization, it has been performed laying the ear prosthesis in an ice bath from 5 to 25°C and in a chamber furnace from 25 to 90°C (in 5°C intervals). The characterization of temperature was done up to 150°C; however, responses between 90° and 150°C remained unchanged, and a fact that was preliminary confirmed by Davis in “Piezoelectric and Pyroelectric Polymers,” 1993 [14] who recommends that the maximum temperature of operation of the PVDF must be 80°C (melting point of around 177°C) [14]. Moreover, for the ear prosthesis of PVDF, the temperature response is in the range of normal temperature of human be [36.19°C (97°F) to 37.2°C (99°F)].
\nConcerning low temperatures (under 0°C), our research group tested the response of the 3D printed ear (poled and unpoled) at temperatures from −160 to 5°C, giving as a result a minimum temperature of −43.15°C for the unpoled prosthesis and −47 for the poled prosthesis. In this context, manufacturers of PVDF reported the minimum operating temperature at −35°C. It has been also reported in the literature and technical notes that PVDF has a glass transition temperature (Tg) of about −35°C where PVDF is typically 50–60% crystalline and the lowest operating temperature is −50°C (Hylar® Kynar®).
\nMoreover, PVDF can be used at temperatures from −80° to 300°F (−62° to 149°C). Polyvinylidene fluoride (PVDF) is a fluorocarbon classified as “self-extinguishing, group 1″ Underwriters Laboratories, Inc. It is not affected by prolonged exposure to sunlight or other sources of ultraviolet radiation. It retains its properties under high vacuum and gamma radiation, and it is also resistant to most acids and alkalis (Porex Corporation). Additionally, the company Bove-ag indicates a temperature range of −30 to 150°C. Goodfellow reports thermal properties of PVDF, minimum operating temperature: −40°C, and maximum operating temperature: 135–150°C.
\nThe basic circuit used for detecting pressure and temperature of the PVDF prosthesis is shown in Figure 6. The circuit is a relaxation oscillator (Astable Circuit Operation LM555, Texas instrument). Changes in PVDF prosthesis capacitance are observed according to the variations in temperature and pressure.
\nAstable multivibrator circuit LM555 to register changes of temperature and pressure of the PVDF prosthesis.
The circuit shown in Figure 6 generates a clock signal, where the frequency of oscillation depends on 2 resistors, RA, RB, and one capacitor, C, as shown in Eq. (3). Instead of C, the PVDF prosthesis was set and the frequency response was observed.
We have also used the circuit of Figure 6 as a moisture sensor [8]. Moreover, pressure and temperature measurements were done to 3D-printed PVDF samples (FV307910), noting that if thickness and/or dimensions change, the response also changes but the trend still remains. This is clearly explained by the Eq. (4):
where C is the capacitance, εr is the relative permittivity of the dielectric, ε0 is the vacuum permittivity (ε0 = 8.854 × 10−12 F/m), A is the area of the PVDF or capacitor plates, and d is the PVDF thickness or the distance between the electrodes or plates (see Equation (5) of Ref. [16]).
\nEquation (4) describes the relationship between permittivity, capacity, and physical dimensions of the ferroelectric PVDF when it is subjected to pressure (see standard weights Figure 5) or it is deformed see Figure 3 article our group “Polyvinylidene Flouride in an Applied Polymer Intraocular Pressure Sensor,” 2005 [6].
\nThermal behavior of PVDF dielectric response changes can be widely seen in the following articles: Casar et al. “Electrical and thermal properties of vinylidene fluoride–trifluoroethylene-based polymer System with coexisting ferroelectric and relaxor states,” 2013 [15]; Jafer et al. “The Use of PE/PVDF Pressure and Temperature Sensors in Smart Wireless Sensor Network for Environmental Monitoring System Developed,” 2008 [16]. And Jia et al. “Simulation and Experiment of PVDF temperature sensor,” 2013 [17].
\nFinally, using Eqs. (3) and (4), the frequency of the circuit LM555 where PVDF was set instead of C is; we have, 1.44/(RA + 2RB)
Demonstrating that any alteration of PVDF in its intrinsic permittivity and/or physical dimensions changes
Response of the prosthesis of PVDF as a pressure sensor from 0 to 16350 Pa.
Thermal responses of prostheses made of PVDF from 2 to 90°C.
Figure 9a shows the design of a 3D human ear model according to anthropometric parameters [18–20] and created with a computer-aided design (CAD) software. It was exported as a stereolithography file in order to print it using a 3D printer.
\n(a) Human ear created with a 3D CAD program. (b) Ear prosthesis printed of PVDF.
The ear was printed in polyvinylidene fluoride as it exhibits ferroelectric properties. Figure 9b shows the 3D-printed ear prosthesis. The dimensions of the printed ear are 60.1 mm wide by 34.74 mm long and has an average thickness of 6.88 mm. Electrodes were painted over the printed ear with silver paint from SPI Supplies. The area of these electrodes is 6.26 mm2, and the distance between them is 41 mm (Figure 10). Dimensions were measured with a Starrett Vernier with 0.02 mm precision.
\nDimensions of ear prosthesis printed of PVDF.
Sawyer–Tower circuit (Figure 2) was used to measure ferroelectric properties such as hysteresis. Figure 11 shows P–E hysteresis loop of the printed PVDF, and X axis shows the electric field (E) in kV/cm and Y axis polarization (P) in μC/cm2.
\nPrinted PVDF hysteresis loop.
The acoustic response using a metalized printed PVDF sample is presented in Figure 12. The electrical signal from the light detector was recorded after PVDF reflected the laser beam. Variations of the laser intensity correspond to fluctuations of amplitude and frequency in the electrical signal.
\nReference signal for printed PVDF (Ch1) and the signal obtained from a commercial light detector (Ch2).
Results of photopyroelectric response of printed PVDF prosthesis at 10 and 100 Hz are shown in Figure 13a, b, respectively.
\n(a) Printed PVDF sample photopyroelectric response (100 Hz), (b) printed PVDF sample photopyroelectric response (10 Hz).
The results in Figure 13a, b showed an asymptotic behavior, similar to the ones described in the theory in the case of frequency modulation of laser stimulation. References amply illustrate the use of PVDF in the photopyroelectric technique [11–13].
\nThe prosthesis was also tested as a pressure sensor, applying pressure loads between 0 and 16.35 kPa.
\nWith regard to the characterization of PVDF, ß-phase was reached by means of corona poling. Figures 7 and 8 show the difference between unpoled and poled PVDF (Tests 1, 2, and 3); the average of these three characterizations (mean) and the line adjustment (fit line).
\nFigure 7 shows the response of the ear prosthesis of PVDF as a pressure sensor using Eq. (3).
\nEquation (6) fits a line through the points at 0–16,350 Pa for the corona-poled PVDF prosthesis with correlation coefficient of 0.9670.
The temperature characterization of the ear prosthesis has been performed from 5 to 90°C in 5°C intervals, and Figure 8 illustrates the response of the PVDF ear prosthesis as a temperature sensor using Eq. (5). After 90°C, no changes were observed as Davis reported [14].
\nEquation (7) fits a line through the points at 5 and 90°C for corona-poled PVDF prosthesis with correlation coefficient of 0.9940.
The fabrication of an ear prosthesis at present has led to restore the functionality of the middle or inner ear with an electronic hearing aid or implant, respectively [21–26], for patient suffering a dysfunction in the mentioned parts of the ear. By the other side, the ear prosthesis developed in this work focuses on aesthetics and the recovery of acoustic functionality involving only the reconstruction of some of the outer and middle ear structures [27–29]. Both kind of prosthesis (electronic and mechanical) had been designed by separate but an active prosthesis, which covers both aesthetic functionality and the electronic hearing aid (in cases where external, middle, and even the inner ear are damaged either by accident or by a congenital malformation) has not been developed so deeply as the first two mentioned above [30]. In this context, our 3D prosthesis is oriented to provide an aesthetic prosthesis with the ability to sense sound waves without the requirement of another kind of sensor like a microphone or membrane that needs to be placed on the patient’s as cochlear implants does. PVDF has demonstrated its ability to work as a microphone [3, 31] due to its piezoelectric properties which can be exploited in the proposed 3D-printed prosthesis.
\nRegarding the light response of PVDF, results (Figure 13a, b) showed an asymptotic behavior, coinciding with the one reported in photopyroelectric techniques [11–13].
\nIn the case of the prosthesis as a temperature sensor, it was observed that it works linearly between 5 and 90°C; after this temperature, there are no more variations. The latter consideration has no impact on the use of the prosthesis usually, as the ambient temperature does not exceed these ranges and the direct application of a higher temperature may cause deformation thereof. It has also been reported that the optimum working temperature is 80°C [14].
\nThe experimental results show an almost linear and inversely proportional behavior between the stimuli of pressure (Figure 7) and temperature (Figure 8) with the frequency response. The repeatability of the results allows to evaluate the PVDF as a reliable material because each stimulus applied (pressure and temperature) was tested in triplicate, thus obtaining results with slight variations but the same trend. The prosthesis tested as a pressure sensor showed effectiveness in the range of 0–16.35 kPa, values that also fall within the range of applied pressure that could hold an ear without malformation [32, 33].
\nBiocompatibility is a subject that is also covered by a prosthesis because of their permanent contact with the skin or any other organ. That is why a PVDF prosthesis is ideal to prevent exposure to hazardous substances. This polymer has been used in many other kinds of biocompatible applications, and it has been widely studied as a safety material for biomedical applications [34, 35]. By the other hand, using a 3D printer, a functional ear prosthesis could be fabricated in a few hours with all the advantages mentioned above.
\nIn this work, a prosthesis made of PVDF was manufactured satisfactorily with a 3D printer. It was also tested as pressure and temperature sensors. The characterization could be achieved satisfactorily. As shown in Figures 7 and 8, the typical response of the PVDF pressure and temperature sensors was found to be very reliable. It was seen that PVDF displayed a high sensitivity to pressure changes in the range 0–16.35 kPa.
\nSmart PVDF prostheses provide a promising tool for measuring pressure and temperature variations due to its ferroelectric properties (piezoelectricity and pyroelectricity) [5, 36]. These kind of smart prostheses have great potentialities in the biomedical engineering field because of their ability to generate an electrical potential in response to applied mechanical stress or variations of temperature, as well as flexibility [37].
\nBesides this, the prosthesis has displayed to be not only a reliable temperature and pressure sensor but also an acoustic one [31, 38]. This is a quite important characteristic due to the fact that the outer ear collects sound waves and channels them into the ear canal where the sound is amplified.
\nThe light response was also satisfactory of the PVDF coinciding with the photopyroelectric techniques reported [11–13].
\nFinally, it is possible to manufacture sensors for TSPL based on PVDF with their respective feedback, that is, TSPL responses proportionate electrical stimulation of skin sensory nerves.
\nAuthors express their gratitude to the Mexican National Council for Science and Technology (CONACYT) for financing this book chapter through the Project 151894.
\nFollowing the initial description of the physiologically corrective operation for tricuspid atresia by Fontan and Baudet [1] and Kreutzer and his associates [2] almost simultaneously, such surgery was widely adapted by most pediatric cardiologists and pediatric cardiac surgeons. This concept of bypassing the right ventricle (RV) was further extended to manage other cardiac defects with a functionally single ventricle.
The original surgery as described by Fontan and Baudet [1] consisted of (1) end-to-end anastomosis of superior vena cava (SVC) with the right pulmonary artery (PA) (classical Glenn procedure [3]), (2) connection of the separated right PA to the right atrium (RA) either directly or through an aortic homograft, (3) closure of the defect in the atrial septum, (4) insertion of a pulmonary valve homograft into the orifice of the inferior vena cava (IVC), and (5) ligation of the main PA, to entirely bypass the RV. On the basis of the procedures performed, one must infer that Fontan’s concept was to use the right atrium as a pumping chamber; therefore, he inserted a prosthetic valve into the IVC and right atrial-pulmonary artery junction.
On the contrary, Kreutzer et al. [2] anastomosed the right atrial appendage to the PA directly or by a pulmonary homograft and closed the ASD. Neither a Glenn procedure was performed nor a prosthetic valve was inserted in the IVC. Kreutzer’s view appears to be that the RA does not function as a pump and that the left ventricle functions as a suction pump in the system.
The surgical procedure as generally performed appears to shadow Kreutzer’s principle, and consequently, I have used the term “Fontan-Kreutzer operation” to describe this procedure [4, 5, 6, 7, 8]. However, because of priority of publication and more common usage in the literature, I will use the term “Fontan operation” in this chapter.
In this review, I will discuss the evolution of the Fontan concepts, the indications for Fontan operation, the Fontan procedure as used currently, and the results of old and current types of Fontan.
A number of modifications of the aforementioned surgery were made by these [1, 2] and other groups of investigators [9, 10] in the field. In this section, these concepts/procedures will be reviewed.
During the first 20 years after Fontan’s [1] and Kruetzer’s [2] description of the procedure, a number of modifications of the surgery were undertaken by several surgeons, as extensively reviewed and referenced elsewhere [9, 10]. In general, there was a consensus that there is no need for a classic Glenn anastomosis and that a prosthetic valve is not necessary in the IVC. Detailed review of these papers revealed that four major types of Fontan operations were being performed for physiologic correction of tricuspid atresia. These include (1) RA-PA anastomosis, direct or through a non-valved conduit; (2) RA-PA anastomosis through a valved conduit; (3) RA-RV anastomosis, direct or non-valved anastomosis; and (4) RA-RV anastomosis through a valved conduit.
In order to understand the advantages of one operation over the other, 17 papers published as of December 1990 that have documented adequate information to evaluate mortality and reoperation rates for each of the four types of Fontan surgery were reviewed. Pooled data from these 17 articles and statistical comparisons were presented in Tables I–IV for the interested reader [9]. This analysis revealed that atriopulmonary (RA-PA) connection appears to be better than atrioventricular (RA-RV) anastomosis and direct connection is better than valved or non-valved conduit anastomosis. Nevertheless, atrioventricular valved (homograft) conduit anastomosis appears to have advantages of (1) restoring a four-valved, four-chambered, biventricular heart and (2) lower right atrial pressure than with atriopulmonary connection. Based on these data [9, 10], the following conclusions were drawn: (1) direct atriopulmonary connection for children with tricuspid atresia with normally related great arteries and a small (<30% of normal) right ventricle without trabecular right ventricular component and for patients who had tricuspid atresia with transposition of the great arteries and (2) atrioventricular valved (homograft) conduit anastomosis for patients with tricuspid atresia and normally related great arteries but with a right ventricular size greater than 30% of normal and a trabecular right ventricular component [9, 10].
Bidirectional cavopulmonary anastomosis is a modified version of classic Glenn procedure in which the upper end of the divided SVC is anastomosed end to side to the right PA without disconnecting the latter from the main PA. Thus, the SVC blood is diverted into both the right and left PAs, justifying the term, “bidirectional.”
Experimental bidirectional cavopulmonary anastomosis was first studied by Haller et al. [11] in animal models, and its first clinical use was described by Azzolina et al. [12] in 1972. Other investigators [13, 14, 15, 16, 17] later applied this technique to palliate complex heart defects with decreased pulmonary blood flow. Hemodynamic advantages of the bidirectional Glenn procedure are improvement of effective pulmonary blood flow, decrease in total pulmonary blood flow, and reduction of left ventricular volume overloading. In addition, preservation of continuity of the pulmonary artery is an added advantage and may help facilitate a low-risk Fontan procedure. When both right and left SVCs are present, bilateral bidirectional Glenn shunts should be performed, especially if the bridging innominate vein is absent or small. Based on our own experience and that published in the literature [13, 14, 15, 16, 17], the author recommended serious consideration in employing bidirectional cavopulmonary anastomosis as an interim palliative procedure for patients who are at an increased risk for the Fontan procedure [9, 10].
Puga et al. [18] positioned a patch inside the right atrium to divert the IVC blood into the PAs; they had good results in the 12 patients that they used this technique. This was later called lateral tunnel and was widely used until extra-cardiac conduits came into vogue.
To better understand the valve-less atriopulmonary anastomosis type of Fontan, de Leval et al. [19] performed hydrodynamic studies and found that (1) the right atrium is not an efficient pump in non-valved atriopulmonary connections, (2) pulsations in a non-valved circulation truly generate turbulence with consequent decrease in net flow, and (3) energy losses occur in the non-pulsatile chambers, corners, and obstructions. In an attempt to address these deficiencies, they developed a procedure which they named “total cavopulmonary connection.” In this procedure, they connected the divided SVC, end to side, to the undivided right pulmonary artery (bidirectional Glenn), and the IVC blood is diverted through a composite intra-atrial tunnel (with the use of posterior wall of the right atrium as posterior wall of the tunnel) into the cardiac end of the divided superior vena cava, which in turn was connected to the PA. They felt that technical simplicity, maintenance of low right atrial and coronary sinus pressure, and reduction of risk of atrial thrombus formation are advantages of this type of operation. They performed this procedure on 20 patients and observed two early deaths and one late death. Postoperative hemodynamic studies were performed in 10 of the survivors which indicated good results. They recommended this type of operation for patients with a non-hypertrophied right atrium. While the total cavopulmonary connection was initially devised for patients with complex atrial anatomy and/or systemic venous anomalies, it has since been used extensively for all types of cardiac anatomy with one functioning ventricle and irrespective of venous anomalies.
Subsequent experimental studies by Sharma and his associates [20] indicated that complete or minimal offset between the orifices of the SVC and IVC into the right pulmonary artery decreases energy losses.
Marcelletti et al. [21, 22] used an interposition extra-cardiac conduit from the IVC to the PA in place of lateral tunnel used in total cavopulmonary connection in 1990. Subsequently, most surgeons adopted this modification of total cavopulmonary connection, and currently extra-cardiac conduits are used in most Fontan operations.
Since the vast majority of patients requiring Fontan operation present as neonates or in the early infancy, palliative procedures are performed at the time of presentation, and subsequently (at 12–18 months of age) the Fontan operation is undertaken. A considerable mortality (~16%) was seen with primary Fontan surgery, largely related to the impact of rapid changes in ventricular geometry and development of ventricular diastolic dysfunction. The concept of further staging the procedure by performing bidirectional Glenn procedure around 6 months of age followed by final Fontan between 12 and 18 months of age was introduced in early 1990s [23, 24]. Performing the Fontan procedure in stages appears to decrease overall mortality, most likely related to improving the ventricular function by correction of the afterload mismatch that is associated with one-stage Fontan procedure. At the current time, most centers prefer staged Fontan with bidirectional Glenn initially, followed later by extra-cardiac conduit diversion of the inferior vena caval blood into the PA.
In 1978, Choussat et al. proposed several criteria for performing Fontan operation [25]. Many cardiologists and surgeons have modified these criteria. Patients not meeting these criteria were deemed to be at a higher risk for a poor prognosis following a Fontan operation than patients who are within the limits of the set criteria. For the high-risk group, several investigators have proposed the concept of leaving a small atrial septal defect (ASD) open to facilitate decompression of the right atrium [26, 27, 28]. Laks et al. advocated closure of the atrial defect by constricting the preplaced suture in the postoperative period [28], while Bridges et al. [27] used a transcatheter closure method at a later date.
Higher cardiac output and significant decreases in the postoperative pleural effusions and systemic venous congestion were noted after a fenestrated Fontan procedure. In addition, the duration of hospitalization appears to have decreased. Nonetheless, these beneficial effects are at the expense of mild arterial hypoxemia and potential for paradoxical embolism.
While the fenestrated Fontan procedure was initially designed for patients at high risk for Fontan surgery, it has since been used in patients with modest or even low risk. Although rare, reports of cerebrovascular or other systemic arterial embolic events occurring after a fenestrated Fontan operation tend to contraindicate the use of fenestrations in patients with low or usual risk. In following years, fenestrated Fontan have been routinely used at most institutions. Some data indicate that routine fenestration is not necessary [29].
Patients who have undergone a fenestrated Fontan operation or patients who have a residual atrial defect, despite correction, may have clinically significant right-to-left shunt causing varying degrees of hypoxemia. These residual defects should be closed not only to address arterial desaturation but also for prevention of paradoxical embolism [30, 31]. Although two types of fenestration closure, namely, constriction of the preplaced suture in the postoperative period [26, 28] and device closure later [27] were described, device closure is opted at most institutions. Closure of such defects can be performed by using transcatheter techniques [32, 33, 34, 35]. The procedure is usually performed 6–12 months following fenestrated Fontan procedure. Although a number of devices have been used in the past [32, 33, 34, 35], at the present time, Amplatzer septal occluders are the most commonly used devices to accomplish such closures.
The indications for opting for a Fontan operation are patients who have one functioning ventricle. At first, patients with tricuspid atresia were selected for this procedure [1, 2]. Shortly thereafter, patients with double-inlet left (single) ventricle were added to the indications for Fontan [36]. Following description of surgical palliation of hypoplastic left heart syndrome (HLHS) by Norwood et al. [37, 38] in the early 1980s, HLHS became the major lesion requiring Fontan operation. Subsequently, mitral atresia (with normal aortic root), unbalanced atrioventricular septal defects (AVSDs), pulmonary atresia with intact ventricular septum with markedly hypoplastic right ventricle, and other complex heart defects with one functioning ventricle were selected for Fontan surgery.
Attempts to insert prosthetic ventricular septum for single ventricle patients met with problems, leading to abandoning such procedures. Thereafter, Fontan became a preferred treatment method. With reasonably good results of Fontan, the pendulum swung so that any patient who could not undergo complete repair became a candidate for Fontan.
A middle of the road method, the so-called one-and-one-half ventricle repair was developed for patients with pulmonary atresia with intact ventricular septum with modest-sized right ventricle. In this procedure, a bidirectional Glenn procedure to divert the SVC flow into the PA is performed and allows the small right ventricle to pump the IVC blood into the pulmonary circuit, and the patent foramen ovale is closed. It is generally considered to be a better option than Fontan, although, to my knowledge, there are no comparative studies to assess this issue.
Because of relatively high mortality rates (17.0–31.7%) [39, 40] and low actuarial survival rates (66.5% at 5 years and 64.4% at 15 years) [41] for unbalanced AVSD patients following Fontan, a number of institutions attempted single stage or staged biventricular repair or conversion from single ventricle (Fontan) to biventricular repair [39, 42, 43, 44, 45, 46, 47]. Detailed analysis by Nathan et al. [39] suggested that the biventricular repair and conversion from single ventricle (Fontan) to biventricular repair groups had reasonably similar mortality rates and a similar need for cardiac transplantation, but these parameters were lower than those seen in the Fontan palliation cohort.
Cardiac transplantation is a surgical alternative in the management of HLHS [48] and other single ventricle lesions. While heart transplantation was used at several institutions initially for HLHS, because of non-availability of donor hearts, most institutions have reverted to the Norwood/Fontan route. In addition, following successful cardiac transplantation, multiple medications for the prevention of graft rejection, frequent outpatient visits and periodic endomyocardial biopsy, to recognize rejection very early, are necessary in the management of these children. At the present time, cardiac transplantation is used for patients failing Fontan operation at a limited number of institutions.
As reviewed above, since the original description in the early 1970s, the Fontan procedure has undergone numerous modifications, and, at the present time it is best described as staged total cavopulmonary connection (TCPC) with an extra-cardiac conduit and fenestration. It is performed in three stages.
The majority, if not all, of patients who require Fontan operation (see Section 3. Indications for Fontan Operation) present during the neonatal and early infancy period, and the Fontan cannot be performed at that time because of naturally high PA pressure and high pulmonary vascular resistance (PVR). Therefore, Fontan, by necessity, becomes a multistage procedure. These babies should receive palliative intervention to allow them to reach the age and size to undergo successful Fontan surgery. The type of palliation is largely dependent upon the hemodynamic disturbance produced by multiple defects associated with a given congenital heart defect (CHD).
In neonates with decreased pulmonary blood flow, the ductus arteriosus should be kept open by administration of prostaglandin E1 (PGE1) intravenously at a dose of 0.05–0.1 mcg/kg/min. Once the O2 saturation improves, the dosage of PGE1 is gradually reduced to 0.02–0.025 mcg/kg/min to minimize the side effects of the prostaglandins. Following stabilization and diagnostic studies, as necessary to confirm the diagnosis, a more permanent way of providing pulmonary blood flow should be instituted. A number of methods to augment pulmonary blood flow have been used over the years [49, 50]. These include subclavian artery to ipsilateral PA anastomosis (classic Blalock-Taussig shunt), descending aorta to the left PA anastomosis (Potts shunt), ascending aorta to the right PA anastomosis (Waterston-Cooley shunt), SVC to right PA anastomosis, end-to-end (classic Glenn shunt), enlargement of the ventricular septal defect (VSD), formalin infiltration of the wall of the ductus arteriosus, central aortopulmonary fenestration or expanded polytetrafluoroethylene (Gore-Tex; W. L. Gore and Associates, Inc., Newark, Delaware) shunt, Gore-Tex interposition graft between the subclavian artery and the ipsilateral PA (modified Blalock-Taussig shunt), balloon pulmonary valvuloplasty, and stent implantation into the ductus arteriosus. Currently modified Blalock-Taussig (BT) shunt [51] by insertion of a Gore-Tex graft between the subclavian artery to the ipsilateral PA (Figure 1a) is performed by most surgeons to address pulmonary oligemia. More recently connecting the RV outflow tract with the PA via non-valve Gore-Tex graft is being used at several institutions to palliate pulmonary oligemia. Placement of a stent in the ductus arteriosus [52, 53, 54] and balloon pulmonary valvuloplasty (if the predominant obstruction is at the pulmonary valve level) [55, 56, 57] are other available options to augment the pulmonary blood flow.
Stage I Fontan. Selected frames form cineangiograms in two different babies; the first with pulmonary oligemia who received Blalock-Taussig (BT) shunt (a) and the second with pulmonary plethora who had pulmonary artery banding (PB) (b). C, catheter; LPA, left pulmonary artery; RPA, right pulmonary artery (Reproduced from [
In babies with increased pulmonary blood flow, optimal anti-congestive measures should be started immediately. Once the congestive heart failure (CHF) is adequately addressed, PA banding (Figure 1b) is performed [58] irrespective of control of CHF.
Infants with near normal pulmonary blood flow with O2 saturations in the low 80s do not need intervention and are clinically followed until Stage II.
Neonates with hypoplastic left heart syndrome usually have Norwood palliation (Figure 2) [37, 59] in the neonatal period; in this operation, the following procedures are performed: (1) the main pulmonary artery and the aorta are anastomosed together; additional prosthetic material is used as needed; (2) the pulmonary circulation receives blood supply by connecting the aorta to the PA via a modified BT shunt [51] (Figure 2b); (3) atrial septum is excised to allow unhindered blood flow from the left to the atrium; and (4) ductal tissue is removed, and coarctation of the aorta, if present is repaired. Some surgeons use alternative Sano shunt [60], connecting the RV outflow tract to the PA (Figure 2c) instead of BT shunt.
Stage I Fontan for hypoplastic left heart syndrome. Selected frames from cineangiograms demonstrating Norwood operation in which the neoaorta (NAo) and hypoplastic aorta (HAo) perfuse the coronary arteries (CAs) as shown in (a), Blalock-Taussig (BT) shunt as illustrated in (b) and Sano shunt as depicted in (c). (b) and (c) are from two different babies. LPA, left pulmonary artery; RPA, right pulmonary artery (Reproduced from [
In patients with inter-atrial obstruction, it should be relieved either by transcatheter methodology or by surgery as deemed appropriate for a given clinical scenario. If there is associated coarctation of the aorta, it should also be relieved. Some patients with double-inlet left ventricle may have significant obstruction at the level of bulboventricular foramen [61]. Similarly some babies with tricuspid atresia with transposition of the great arteries may have obstruction at the VSD level, causing obstruction to systemic blood flow [61, 62]. Such babies require Damus-Kaye-Stansel (connection of the aorta to the PA) [63] along with a BT shunt. Inter-atrial obstruction may be present frequently in babies with mitral atresia and single ventricle [64]. In such babies, predictable fall in PVR occurs following balloon or surgical relief of inter-atrial obstruction [64]; consequently, PA banding should be undertaken without hesitation at the time of relieving the atrial septal obstruction, so as to reduce the probability for CHF, lower the PVR and PA pressure, prevent pulmonary vascular obstructive disease (PVOD), and pave the way for Fontan approach [64].
Irrespective of the type of palliative surgery in the neonatal period, bidirectional Glenn procedure [12, 13, 14, 17, 23], namely, anastomosis of the SVC to the right PA, end-to-side (Figure 3) is performed around the age of 6 months. The previously performed BT or Sano shunt is ligated at the same time. Although performing the procedure at 6 months is generally adopted, it can be performed as early as 3 months provided normalcy of PA pressure and anatomy can be documented.
Stage II of Fontan. Selected frames from cineangiograms in two different children illustrating bidirectional Glenn operation in which the superior vena cava is anastomosed to the right pulmonary artery (RPA). Unobstructed flow from the SVC to the right (RPA) and left (LPA) pulmonary arteries is clearly seen. (Reproduced from [
In patients with persistent left SVC, bilateral bidirectional Glenn (Figure 4) is undertaken especially in patients with a small or absent left innominate vein. A bidirectional Glenn procedure may also be performed for patients with infrahepatic interruption of the IVC with azygos or hemiazygos continuation, and such a procedure is called a Kawashima procedure by some authorities.
Stage II Fontan. Selected frames from cineangiograms in a different child than shown in
Prior to the bidirectional Glenn procedure, normal PA pressures and adequate size of the branch PAs should be ensured by cardiac catheterization and cineangiography. Echo-Doppler or other imaging studies (magnetic resonance imaging [MRI] or computed tomography [CT]) is advocated at some institutions.
If PA stenosis is present, it may be addressed with balloon angioplasty or stent implantation, as deemed appropriate, or it may be addressed during the bidirectional Glenn procedure. Atrioventricular valve regurgitation, aortic coarctation, subaortic obstruction, and other abnormalities should also be repaired/addressed at the time of this operation.
During the final Stage III, the IVC flow is diverted into the PA along with creation of a fenestration. We arbitrarily divided [30] these procedures into Stage IIIA (diversion of IVC into the PA) and Stage IIIB (closure of the fenestration).
In the final Stage III, the total cavopulmonary connection is achieved by diverting the IVC flow into the PA either by a lateral tunnel [18, 65] or by an extra-cardiac, non-valved conduit (Figures 5 and 6) [21, 22]; the procedure is usually performed between the ages of 1 and 2 years, usually 1 year following the bidirectional Glenn procedure. Most surgeons seem to prefer extra-cardiac conduit to accomplish this final stage of Fontan. The majority of surgeons construct a fenestration, 4–6 mm in size, between the conduit and the atria (Figures 5 and 6) [27]. While the creation of fenestration during the Fontan operation was initially proposed for high-risk patients [27, 28], most surgeons now seem to prefer fenestration, since fenestration during the Fontan improves mortality rate and reduces morbidity during the immediate postoperative period [30].
Selected cine frames in posteroanterior (a) and lateral (b) views, demonstrating Stage IIIA Fontan procedure diverting the inferior vena caval flow into the pulmonary arteries via a non-valve conduit (Cond). Flow across the fenestration (fen) is shown by arrows in (a) and (b). HV, hepatic veins; LPA, left pulmonary artery; PG, pigtail catheter in the descending aorta; RPA, right pulmonary artery.
Selected cine frames in posteroanterior (a) and lateral (b) views in a different patient to the one shown in
Cardiac catheterization and selective cineangiography are usually performed shortly prior to Fontan conversion in order to assess the PA anatomy and pressures, trans-pulmonary gradient, PVR, and ventricular end-diastolic pressure and to assure that they are normal prior to proceeding with Fontan completion. At some institutions, MRI is used for this assessment instead of catheterization and angiography; however, the author’s preference is catheterization. During this catheterization, any significant collateral vessels that are present are also transcatheter-occluded by most cardiologists.
In the final stage, Stage IIIB, the fenestration is closed (Figures 7b, 8b, and 9B and C) by transcatheter methodology [27, 30, 31, 32, 33, 34, 35], usually 6–12 months after Fontan Stage, IIIA. In the past, most devices used to occlude ASDs [32, 33, 34, 35] were employed for this purpose, but at the present time, Amplatzer septal occluders are the most commonly used devices to accomplish such closures. If there are any other residual shunts, they should also be occluded (Figure 10) by device closure.
Stage IIIB. (a) Selected frames from cineangiograms in anteroposterior projection illustrating Stage IIIA of the Fontan operation in which the inferior vena caval (IVC) flow is diverted into the pulmonary arteries by a non-valve conduit (Cond). The fenestration (fen) is shown by the arrow in (a). (b) Closure of the fenestration with an Amplatzer septal occluder device (D) is shown with an arrow in (b). HV, hepatic veins; LPA, left pulmonary artery; RPA, right pulmonary artery (Reproduced from [
Stage IIIB. (a) Selected frames from cineangiograms in lateral view of the same patient illustrated in
(A) Selected cine frame from a Fontan conduit cineangiogram in anteroposterior view, demonstrating tubular fenestration (Tu fen) with opacification of the left atrium (LA). (B) The Tu fen is closed with an Amplatzer vascular plug (AVP). (C) A follow-up conduit cineangiogram after AVP implantation, showing complete occlusion of the Tu fen. TEE, transesophageal probe.
(A) A selected cineangiographic frame showing the Fontan conduit in lateral view, demonstrating a residual shunt (RS) at the superior aspect of the conduit (Cond). (B) The RS was occluded with an Amplatzer septal occluder device (AD); the residual shunt is no longer seen. TEE, transesophageal echo probe.
In children who have one functioning ventricle requiring Fontan correction, the systemic and pulmonary circulations work in-parallel in place of the usual in-series circulation. A fragile equilibrium between the two circulations must be preserved so that adequate systemic and pulmonary perfusions are maintained. There is substantial interstage mortality ranging from 5 to 15% [66, 67, 68] which may be due to restrictive atrial communication, obstruction of the aortic arch, blockage of the shunt, distortion of the PAs, atrioventricular valve insufficiency, or a combination thereof [66]. Intercurrent illnesses such as dehydration, respiratory tract illness, or fever disturb this balance and make the patients to become critically ill and have been blamed for interstage mortality [66, 68]. The surgically created BT and Sano shunts may also get thrombosed producing severe hypoxemia [69]. Indeed, these abnormalities produce significant interstage mortality [67]; these appear to occur more frequently between Stages I and II than between Stages II and III. Consequently, extreme vigilance in managing these patients should be maintained by the caregiver [68, 70]; even trivial illnesses must be aggressively monitored and addressed as appropriate.
Immediate and follow-up results of both older and current types of Fontan will be reviewed in this section.
The results of original Fontan [1, 2] and its earlier modifications, namely, RA-to-PA or RA-to-RV anastomosis either directly or via valved or non-valved conduits, revealed high initial mortality rates. The initial mortality rates ranged from 10 to 26% [9, 10, 71, 72]. Furthermore, the postoperative stay in the intensive care setting was prolonged.
The initial mortality following staged, total cavopulmonary connection has decreased remarkably [73, 74, 75, 76, 77, 78]. Patients who had total cavopulmonary connection without fenestration had initial mortality rates ranging from 8 to 10.5% [73, 74, 75], while subjects who had total cavopulmonary connection with fenestration had slightly lower (4.5–7.5%) initial mortality rates [76, 77, 78].
In one large single institutional study examining the results of 500 consecutive Fontan surgery patients [77], early failure was associated with high (≥19 mm Hg) mean PA pressure, young age at surgery, heterotaxy syndrome, a right-sided tricuspid valve as systemic atrioventricular valve, distorted pulmonary arteries, an atriopulmonary connection, no Fontan fenestration, and longer cardiopulmonary bypass time.
These investigators also observed that a significant improvement in morbidity and mortality from early (first quartile—early failures: 27.1%) to the more recent time (last quartile—early failures: 7.5%) occurred [77]. This progress appears to be related to increasing surgical and intensive care experience as well as to more recently introduced Fontan modifications.
Long-term follow-up results were also poor with older types of Fontan [9, 10]. The late mortality rates varied from 1 to 11%, and when early and late mortality rates were combined, they varied between 11 and 25%. The need for reoperations was present in 1–11% of patients. Factors adversely influencing late mortality and reoperation rates are earlier calendar year of operation, age of patient at the time of surgery, type of prior palliative procedures, hypoplasia, distortion or obstruction of PAs, subaortic obstruction, significant mitral valve insufficiency, elevated PA pressure or resistance, decreased left ventricular function, increased left ventricular muscle mass, asplenia syndrome, and others [9, 10].
Following the introduction of staged cavopulmonary anastomosis (both lateral tunnel and extra-cardiac conduit diversion of IVC blood to the PA), the long-term outcomes have improved. In one study in which results of follow-up for 10.2 ± 0.6 years of 196 patients were examined, the estimated Kaplan-Meier survival was 93 and 91% at 5 and 10 years, respectively [79]. An equally impressive finding was freedom from supraventricular arrhythmias in 96 and 91% of patients at 5 and 10 years following surgery. In a different study, the actuarial survival 15 years following surgery was 85% [80]. But, late re-interventions were necessary in 12.7% of patients. When lateral tunnel and extra-cardiac conduit types of Fontan were compared, the outcomes were found to be similar for both groups [81, 82].
Using fenestration during Fontan appears to improve early mortality and morbidity, particularly demonstrated in high-risk patents [83]. A more recent analysis in a smaller group of patients did not demonstrate significant advantage of fenestrated Fontan over the non-fenestrated [84]. However, the general consensus is that using fenestration during Fontan decreases mortality and morbidity during the postoperative period [30, 76, 77, 78].
Periodic follow-up following Fontan is generally recommended. These patients are evaluated at 1, 6, and 12 months after Stage IIIB (device closure of fenestration) and yearly thereafter. During the follow-up, platelet-inhibiting doses of aspirin 2–5 mg/kg/day in children or clopidogrel 75 mg/day in adults to prevent thrombus formation and angiotensin-converting enzyme inhibitors for afterload reduction are generally prescribed. Electrocardiograms and echocardiograms are generally performed during evaluation of these patients with additional imaging studies, as indicated. Any abnormalities, as and when detected, are addressed.
During follow-up, a number of complications were reported, and these include arrhythmias, obstructed Fontan pathways, cyanosis, paradoxical emboli, thrombi, development of collateral vessels, and protein loosing enteropathy [30, 31, 85]. These complications appear to be more frequent with older types of Fontan than with the currently used staged, total cavopulmonary connection with extra-cardiac conduit and fenestration. When such complications develop, they should be promptly investigated and treated. In the ensuing paragraphs, a brief review of some of these complications will be presented.
Arrhythmias were more frequently seen in patients with old Fontan (atriopulmonary connection) than with staged TCPC. The observed arrhythmias were typically atrial arrhythmias, namely, atrial flutter/fibrillation and supraventricular tachycardia. Initially, anti-arrhythmic medications are used to control the rhythm disturbance. This should be followed by hemodynamic and angiographic assessment to identify obstructive lesions in the Fontan pathways. The obstructive lesions should be treated with balloon angioplasty, stent, or surgery, as applicable. Continued rhythm abnormality calls for radiofrequency ablation. Although the success rate of radiofrequency ablation is high in 80% range [86], rates of recurrence range from 30 to 40%. In subjects who have resistant arrhythmias, reducing the atrial mass, switch to TCPC with concomitant Maize procedure is advisable [87]. A few patients develop atrioventricular block or sick sinus syndrome which may require pacemaker implantation. Fortunately, ventricular arrhythmias are less frequent.
Obstructions in Fontan circulation may occur. Obstructive lesions in the SVC or IVC may arise but are less frequently seen. However, branch pulmonary artery stenoses may be seen more often. Obstructions within the lateral tunnel or extra-cardiac conduit are also uncommon, but may occur due to thrombus formation and will be addressed in the section on “Thrombus formation.” In the presence of signs and symptoms indicative of obstruction in the Fontan pathway, prompt investigation to confirm such obstruction should be made. While echo studies are useful in young children, poor echo windows in adolescents and adults may require MRI and CT, and/or angiographic studies to confirm or exclude such obstructive lesions. If the obstructive lesions are detected, they should be promptly relieved by balloon angioplasty or stent implantation (Figure 11) [88]. Surgery may be needed in rare occasions.
Selected frames from cineangiograms of the pulmonary artery in posteroanterior view illustrating normal right pulmonary artery (RPA) and narrowed (arrow) left pulmonary artery (LPA) prior to (a) and after (b) stent (arrow) placement in an adolescent who had Fontan surgery several years earlier (Reproduced from [
Sometimes connections between lateral tunnel and extra-cardiac conduit on the one hand and the atrium on the other persist. These residual defects and intentionally created Fontan fenestrations result in right-to-left shunt because the pressure in the Fontan conduit is higher than that of the atrial pressures. These residual defects will result in arterial desaturation and may become the site of paradoxical embolism with consequential transient ischemic attacks (TIAs), cerebrovascular accidents (CVAs), and systemic emboli. These residual defects as well as Fontan fenestrations should be occluded by transcatheter techniques to return O2 saturations to normal and decrease the likelihood for paradoxical embolism [30, 32, 33, 83, 88, 89]. Amplatzer septal occluder (St. Jude Medical, Inc., St Paul, MN) is currently most common device used to accomplish this (Figures 7,8, and 10). Tubular fenestrations may be closed with Amplatzer vascular plug devices (St. Jude Medical, Inc.) (Figure 9). Test occlusion of the residual defect or fenestration is suggested to ensure that adequate cardiac output is maintained following defect occlusion [89, 90], especially if the procedure is performed shortly after fenestrated Fontan. Late follow-up results of fenestration closure are good [33].
There is a tendency for thrombus formation in the Fontan pathway; the reported prevalence was 15–30% [91, 92]. Regrettably the usual transthoracic echo-Doppler evaluation may not discover these thrombi. However, transesophageal echocardiography, MRI, or CT studies may be necessary to detect these thrombi. In an attempt to prevent thrombus formation in the Fontan circuit, thromboprophylaxis is commonly recommended; both warfarin and aspirin have been utilized in the past for this purpose. A multicenter, randomized trial was conducted to compare the efficacy of these two drugs; results showed less than optimal results with both drugs and no significant difference between the two regimens [93]. In the author’s experience, most children are prescribed with aspirin for thromboprophylaxis which may be switched to clopidogrel (Plavix) as the children approach adulthood.
Despite seemingly adequate thromboprophylaxis, some patients develop thrombosis of the Fontan conduits (Figure 12A). We initially employ thrombus dissolving drug therapy (tPA, heparin, etc.). If the thrombi do not resolve, we have employed stenting of the conduit to compress the thrombi against the conduit wall [94]. An example from our experience is shown in Figure 12.
(A) Selected frame from a cineangiogram of a Fontan conduit in lateral view, illustrating a thrombus (arrow in (A)). (B) and (C) position of a stent (St) before (B) and after (C) its complete expansion. (D) Cineangiographic frame demonstrating the widely patent stent after stent deployment. Also, note the residual shunt (RS) at the superior aspect of the conduit (seen in (A) and (D)). The RS was occluded with an Amplatzer septal occluder device (AD) shortly after the cine shown in (D). (F) A follow-up cineangiogram 1 year later shows the continued patency of the conduit with no RS. TEE, transesophageal echo probe (Reproduced from [
Systemic venous to pulmonary venous and systemic arterial to pulmonary arterial collateral vessels may develop in some patients after the Fontan procedure [88, 95]. These may develop both shortly after the procedure and during late follow-up. Systemic venous to pulmonary venous collateral vessels produce arterial hypoxemia. In addition, they may also become potential sites for paradoxical embolism. Systemic arterial to pulmonary arterial (or venous) collateral vessels produce left ventricular volume overload. These abnormal vessels should be transcatheter-occluded with coils, vascular plugs, and ductal occluding devices depending upon the size and accessibility. Examples from the author’s experience of occluding these vessels are shown in Figures 13–16 [88, 95, 96].
(a) Selected frame from a left innominate vein (L inn) cineangiogram in posteroanterior view demonstrating an anomalous vein (AV) opacifying the atrial mass (not marked). (b) Following occlusion with Gianturco coil (arrow), the AV is completely occluded and the systemic arterial saturation improved (Reproduced from [
(A) Selected frame from a cineangiogram in lateral view with the catheter positioned at the superior vena cava/azygos junction illustrating a fistula which results in opacification of the left atrium (LA). (B) The fistula was occluded with an Amplatzer vascular plug (arrow—AVP) with some residual flow. (C) Follow-up SVC injection shows complete occlusion by the AVP (Reproduced from [
(A) Selected cine frame from an internal mammary artery (IMA) cineangiogram in the lateral view, demonstrating multiple small collateral vessels arising from the pericardiophrenic (PCP) branch, which resulted in a significant levophase (not shown). (B) Following occlusion with a Gianturco coil (C), there is complete occlusion of this vessel (Reproduced from [
(A) Selected cine frame from a right subclavian artery (RSA) cineangiogram showing branches (white arrows) of the thyrocervical (TC) trunk which supplied a number of small vessels, giving a good degree of levophase. (B) Complete occlusion occurred following the implantation of a Gianturco coil (C) (Reproduced from [
Protein losing enteropathy (PLE) is a grave long-term complication of Fontan with a prevalence of 11.1% in older types of Fontan [85, 97]. However, the incidence appears to have come down to 1.2% with staged TCPC [85, 98]. The reason for development of PLE is not understood. Intestinal protein loss secondary to lymphatic distension which in turn may be due to elevated pressure in systemic veins is considered to be a pathogenic mechanism. But, PLE has been seen even in patients with “normal” Fontan circuit pressures. Therefore, the true cause of PLE remains a mystery. The symptoms and signs of PLE are diarrhea, edema, ascites, and/or pleural effusions. Laboratory abnormalities include reduced serum albumin and elevated fecal alpha-1 antitrypsin levels. The PLE diagnosis may be confirmed with technetium 99m-labeled human serum albumin scintigraphy [99].
Because of high mortality rate seen with PLE, speedy diagnosis and implementing aggressive management strategies are important [85]. At first, supportive therapy such as medium-chain triglycerides diet, infusion of intravenous albumin, and replacement of immunoglobulins should be undertaken. Obstructive lesions in the Fontan pathway should be scrutinized, and aortopulmonary connections should be screened for. If identified, they should be treated with appropriate transcatheter measures. Surgical therapy is indicated if they cannot be adequately addressed with transcatheter intervention. A variety of other treatment regimens, including prednisone, elementary diet, calcium replacement, regular high-molecular-weight heparin, low-molecular-weight heparin, somatostatin, high-dose spironolactone, sildenafil, and resection of localized intestinal lymphangiectasia, have been utilized in the past with varying degrees of success [85].
Following a short trial of any of the above treatment modes, largely on the basis of the cardiologist’s preference, a more definitive treatment methods such as lessening the conduit pressure by creating a fenestration between the conduit and the atrium [99, 100, 101], converting atriopulmonary type of Fontan to TCPC [87, 102, 103], instituting sequential atrioventricular pacing [104, 105], and performing cardiac transplantation [106, 107, 108] should all be considered. Again, it is essential to emphasize that timely treatment should be instituted as soon as PLE is identified [85]. Fortunately, the need for use of these methods has progressively diminished since the wide use of staged TCPC.
Since the initial description of the Fontan operation in the early 1970s by Fontan, Kruetzer, and their associates, several modifications have been introduced. These include avoiding classic Glenn anastomosis; not using a prosthetic valve in the IVC; RA-PA anastomosis, direct or through a non-valved conduit; RA-PA anastomosis through a valved conduit; RA-RV anastomosis, direct or non-valved anastomosis; RA-RV anastomosis through a valved conduit; bidirectional Glenn procedure (cavopulmonary anastomosis); lateral tunnel; total cavopulmonary connection; extra-cardiac conduit, staged Fontan; fenestrated Fontan; and closure of Fontan fenestration. Currently staged, total cavopulmonary connection with extra-cardiac conduit and fenestration has become the most commonly used multistage surgery in accomplishing the Fontan.
The indications for Fontan are patients who have one functioning ventricle, and these include tricuspid atresia, double-inlet left ventricle, HLHS, mitral atresia with normal aortic root, unbalanced AVSDs, pulmonary atresia with intact ventricular septum with markedly hypoplastic right ventricle, and other complex heart defects with one functioning ventricle. Recently there has been a trend for biventricular repair, particularly for patients with unbalanced AVSDs.
Stage I consists of performing palliative procedures on the basis of pathophysiology of the defect complex at presentation, usually in the neonatal period. Stage II involves performing a bidirectional Glenn procedure (diversion of the superior vena caval blood flow into both lungs) usually at about the age of 6 months. During stage IIIA diversion of the IVC blood flow into the lungs, usually by an extra-cardiac conduit plus a fenestration, usually at about the age of 2 years. Stage IIIB consists of transcatheter closure of the fenestration 6–12 months after Stage IIIA.
Both the immediate and follow-up results have remarkably improved, both in terms of mortality and morbidity, following the introduction of staged total cavopulmonary connection with extra-cardiac conduit and fenestration with subsequent catheter closure of Fontan fenestration. Complications do occur during follow-up, and they should be addressed as and when they are detected.
The author declares no conflict of interest.
The Internet has irrevocably changed the dynamics of scholarly communication and publishing. Consequently, we find it necessary to indicate, unambiguously, our definition of what we consider to be a published scientific work.
",metaTitle:"Prior Publication Policy",metaDescription:"Prior Publication Policy",metaKeywords:null,canonicalURL:"/page/prior-publication-policy",contentRaw:'[{"type":"htmlEditorComponent","content":"A significant number of working papers, early drafts, and similar work in progress are openly shared online between members of the scientific community. It has become common to announce one’s own research on a personal website or a blog to gather comments and suggestions from other researchers. Such works and online postings are, indeed, published in the sense that they are made publicly available. However, this does not mean that if submitted for publication by IntechOpen they are not original works. We differentiate between reviewed and non-reviewed works when determining whether a work is original and has been published in a scholarly sense or not.
\\n\\nThe significance of Peer Review cannot be overstated when it comes to defining, in our terms, what constitutes a published scientific work. Peer Review is widely considered to be the cornerstone of modern publishing processes and the key value-adding contribution to a scholarly manuscript that a publisher can make.
\\n\\nOther than the issue of originality, research misconduct is another major issue that all publishers have to address. IntechOpen’s Retraction & Correction Policy and various publication ethics guidelines identify both redundant publication and (self)plagiarism to fall within the definition of research misconduct, thus constituting grounds for rejection or the issue of a Retraction if the work has already been published.
\\n\\nIn order to facilitate the tracking of a manuscript’s publishing history and its development from its earliest draft to the manuscript submitted, we encourage Authors to disclose any instances of a manuscript’s prior publication, whether it be through a conference presentation, a newspaper article, a working paper publicly available in a repository or a blog post.
\\n\\nA note to the Academic Editor containing detailed information about a submitted manuscript’s previous public availability is the preferred means of reporting prior publication. This helps us determine if there are any earlier versions of a manuscript that should be disclosed to our readers or if any of those earlier versions should be cited and listed in a manuscript’s references.
\\n\\nSome basic information about the editorial treatment of different varieties of prior publication is laid out below:
\\n\\n1. CONFERENCE PAPERS & PRESENTATIONS
\\n\\nGiven that conference papers and presentations generally pass through some sort of peer or editorial review, we consider them to be published in the accepted scholarly sense, particularly if they are published as a part of conference proceedings.
\\n\\nAll submitted manuscripts originating from a previously published conference paper must contain at least 50% of new original content to be accepted for review and considered for publication.
\\n\\nAuthors are required to report any links their manuscript might have with their earlier conference papers and presentations in a note to the Academic Editor, as well as in the manuscript itself. Additionally, Authors should obtain any necessary permissions from the publisher of their conference paper if copyright transfer occurred during the publishing process. Failure to do so may prevent Us from publishing an otherwise worthy work.
\\n\\n2. NEWSPAPER & MAGAZINE ARTICLES
\\n\\nNewspaper and magazine articles usually do not pass through any extensive peer or editorial review and we do not consider them to be published in the scholarly sense. Articles appearing in newspapers and magazines rarely possess the depth and structure characteristic of scholarly articles.
\\n\\nSubmitted manuscripts stemming from a previous newspaper or magazine article will be accepted for review and considered for publication. However, Authors are strongly advised to report any such publication in an accompanying note to the External Editor.
\\n\\nAs with the conference papers and presentations, Authors should obtain any necessary permissions from the newspaper or magazine that published the work, and indicate that they have done so in a note to the External Editor.
\\n\\n3. GREY LITERATURE
\\n\\nWhite papers, working papers, technical reports and all other forms of papers which fall within the scope of the ‘Luxembourg definition’ of grey literature do not pass through any extensive peer or editorial review and we do not consider them to be published in the scholarly sense.
\\n\\nAlthough such papers are regularly made publicly available via personal websites and institutional repositories, their general purpose is to gather comments and feedback from Authors’ colleagues in order to further improve a manuscript intended for future publication.
\\n\\nWhen submitting their work, Authors are required to disclose the existence of any publicly available earlier drafts in a note to the Academic Editor. In cases where earlier drafts of the submitted version of the manuscript are publicly available, any overlap between the versions will generally not be considered an instance of self-plagiarism.
\\n\\n4. SOCIAL MEDIA, BLOG & MESSAGE BOARD POSTINGS
\\n\\nWe feel that social media, blogs and message boards are generally used with the same intention as grey literature, to formulate ideas for a manuscript and gather early feedback from like-minded researchers in order to improve a particular piece of work before submitting it for publication. Therefore, we do not consider such internet postings to be publication in the scholarly sense.
\\n\\nNevertheless, Authors are encouraged to disclose the existence of any internet postings in which they outline and describe their research or posted passages of their manuscripts in a note to the Academic Editor. Please note that we will not strictly enforce this request in the same way that we would instructions we consider to be part of our conditions of acceptance for publication. We understand that it may be difficult to keep track of all one’s internet postings in which the researcher´s current work might be mentioned.
\\n\\nIn cases where there is any overlap between the Author´s submitted manuscript and related internet postings, we will generally not consider it to be an instance of self-plagiarism. This also holds true for any co-Author as well.
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\\n\\nPolicy last updated: 2017-03-20
\\n"}]'},components:[{type:"htmlEditorComponent",content:'A significant number of working papers, early drafts, and similar work in progress are openly shared online between members of the scientific community. It has become common to announce one’s own research on a personal website or a blog to gather comments and suggestions from other researchers. Such works and online postings are, indeed, published in the sense that they are made publicly available. However, this does not mean that if submitted for publication by IntechOpen they are not original works. We differentiate between reviewed and non-reviewed works when determining whether a work is original and has been published in a scholarly sense or not.
\n\nThe significance of Peer Review cannot be overstated when it comes to defining, in our terms, what constitutes a published scientific work. Peer Review is widely considered to be the cornerstone of modern publishing processes and the key value-adding contribution to a scholarly manuscript that a publisher can make.
\n\nOther than the issue of originality, research misconduct is another major issue that all publishers have to address. IntechOpen’s Retraction & Correction Policy and various publication ethics guidelines identify both redundant publication and (self)plagiarism to fall within the definition of research misconduct, thus constituting grounds for rejection or the issue of a Retraction if the work has already been published.
\n\nIn order to facilitate the tracking of a manuscript’s publishing history and its development from its earliest draft to the manuscript submitted, we encourage Authors to disclose any instances of a manuscript’s prior publication, whether it be through a conference presentation, a newspaper article, a working paper publicly available in a repository or a blog post.
\n\nA note to the Academic Editor containing detailed information about a submitted manuscript’s previous public availability is the preferred means of reporting prior publication. This helps us determine if there are any earlier versions of a manuscript that should be disclosed to our readers or if any of those earlier versions should be cited and listed in a manuscript’s references.
\n\nSome basic information about the editorial treatment of different varieties of prior publication is laid out below:
\n\n1. CONFERENCE PAPERS & PRESENTATIONS
\n\nGiven that conference papers and presentations generally pass through some sort of peer or editorial review, we consider them to be published in the accepted scholarly sense, particularly if they are published as a part of conference proceedings.
\n\nAll submitted manuscripts originating from a previously published conference paper must contain at least 50% of new original content to be accepted for review and considered for publication.
\n\nAuthors are required to report any links their manuscript might have with their earlier conference papers and presentations in a note to the Academic Editor, as well as in the manuscript itself. Additionally, Authors should obtain any necessary permissions from the publisher of their conference paper if copyright transfer occurred during the publishing process. Failure to do so may prevent Us from publishing an otherwise worthy work.
\n\n2. NEWSPAPER & MAGAZINE ARTICLES
\n\nNewspaper and magazine articles usually do not pass through any extensive peer or editorial review and we do not consider them to be published in the scholarly sense. Articles appearing in newspapers and magazines rarely possess the depth and structure characteristic of scholarly articles.
\n\nSubmitted manuscripts stemming from a previous newspaper or magazine article will be accepted for review and considered for publication. However, Authors are strongly advised to report any such publication in an accompanying note to the External Editor.
\n\nAs with the conference papers and presentations, Authors should obtain any necessary permissions from the newspaper or magazine that published the work, and indicate that they have done so in a note to the External Editor.
\n\n3. GREY LITERATURE
\n\nWhite papers, working papers, technical reports and all other forms of papers which fall within the scope of the ‘Luxembourg definition’ of grey literature do not pass through any extensive peer or editorial review and we do not consider them to be published in the scholarly sense.
\n\nAlthough such papers are regularly made publicly available via personal websites and institutional repositories, their general purpose is to gather comments and feedback from Authors’ colleagues in order to further improve a manuscript intended for future publication.
\n\nWhen submitting their work, Authors are required to disclose the existence of any publicly available earlier drafts in a note to the Academic Editor. In cases where earlier drafts of the submitted version of the manuscript are publicly available, any overlap between the versions will generally not be considered an instance of self-plagiarism.
\n\n4. SOCIAL MEDIA, BLOG & MESSAGE BOARD POSTINGS
\n\nWe feel that social media, blogs and message boards are generally used with the same intention as grey literature, to formulate ideas for a manuscript and gather early feedback from like-minded researchers in order to improve a particular piece of work before submitting it for publication. Therefore, we do not consider such internet postings to be publication in the scholarly sense.
\n\nNevertheless, Authors are encouraged to disclose the existence of any internet postings in which they outline and describe their research or posted passages of their manuscripts in a note to the Academic Editor. Please note that we will not strictly enforce this request in the same way that we would instructions we consider to be part of our conditions of acceptance for publication. We understand that it may be difficult to keep track of all one’s internet postings in which the researcher´s current work might be mentioned.
\n\nIn cases where there is any overlap between the Author´s submitted manuscript and related internet postings, we will generally not consider it to be an instance of self-plagiarism. This also holds true for any co-Author as well.
\n\nFor more information on this policy please contact permissions@intechopen.com.
\n\nPolicy last updated: 2017-03-20
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From 1985 to 1986, he was a Research Fellow in the Research Institute for Electronic Equipment, ZZU AD, Plovdiv, Bulgaria. In 1986, he joined the Department of Control Systems, Technical University of Sofia at the Plovdiv campus, where he is presently a Full Professor. He has held long-term visiting Professor/Scholar positions at various institutions in South Korea, Turkey, Mexico, Greece, Belgium, UK, and Germany. And he has coauthored one book and authored or coauthored more than 80 research papers in conference proceedings and journals. 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After finishing his P. hD degree in 1992, he served in the Industry as a Scientific Officer and continued his academic career as a visiting scholar for a number of educational institutions. In 1996 he joined National University of Science & Technology Pakistan (NUST) as an Associate Professor; NUST is one of the top few universities in Pakistan. In 1999 he joined an International Company Lineo Inc, Canada as Manager Compiler Group, where he headed the group for developing Compiler Tool Chain and Porting of Operating Systems for the BLACKfin processor. The processor development was a joint venture by Intel and Analog Devices. In 2002 Lineo Inc., was taken over by another company, so he joined Aalborg University Denmark as an Assistant Professor.\nProfessor Akbar has truly a multi-disciplined career and he continued his legacy and making progress in many areas of his interests both in teaching and research. 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Subsequently, the alteration in the shape or composition of the fine particles such as clay (water-wet solids), as a result of the stress on it, in the flow path of the second phase can lead to the permeability decline of reservoir. Consequently, the solvents such as surfactants (as demulsifiers) to lower the surface tension as a phenomenon associated with intermolecular forces (known as capillary action) during flowback are consumed to avoid the emulsions and sludge mostly in the near-wellbore zone or undertreatment and under-injection radius of the reservoir. However, in addition to surging or swabbing the wells to lower the surface tension, using solvents as the wettability changing agent along with base fluid is a common method in the water block elimination from the wellbore, especially in the low permeability porous media or the reservoirs latter its average pressure declined below bubble point. For more profitability, after using solvents in various reservoir characterizations, the trend of their behavior variations in the different lithologies is required to decide on the removed damage percentage. The investigations on this subject involve many experimental studies and have not been presented any mathematical formulas for the damage of water block in the water, oil, and gas reservoirs. These formulas determine selection criteria for the applied materials and increase variable performance. An integrated set of procedures and guidelines for one or more phases in a porous media is necessary to carry out the step-by-step approach at wellhead. Erroneous decisions and difficult situations can also be addressed in the injection wells or saltwater disposal wells, in which water block is a formation damage type. Misconceptions and difficult situations resulting from these injuries can increase water saturation in borehole and affect the fluid transmissibility power in reaching far and near distances of the wellbore, which results in injection rate loss at the wellhead. Accordingly, for the equations of water block here, a set of variables, of a particular domain, for defining relationships between rock- and fluid-based parameters are required. For these equations, at first, the structural classifications of fracture and grain in the layers (d1, d2, and d3) are defined. Afterward, the equations of overburden pressure (Pob) for a definite sectional area surrounding the wellbore for any lithology (in the three categories relative to porosity) are obtained by these structural classifications and other characteristics of rock and fluid. Naturally, prior to equations of overburden pressure in a definite layer or a definite sectional area around the wellbore, the overburden pressure of a point in a layer in the first four equations is expressed. In the second, the estimated overburden pressure equations are applied in driving the equations of removed water block (Bk). The equations of removed water block, themselves, are divided into two groups of equations, i.e., equations of oil wells and equations of saltwater disposal wells, and each group of equations is again classified based on the wettability of reservoir rock (oil-wet or water-wet) in the two ranges of porosity. In the third, after describing these equations (i.e., equations of Bk), the other new variable included in the equations of removed water block, that is, the acid expanding ability (Ik) for a definite oil layer around the wellbore, is presented, which is extracted from (1) the full characteristics of reservoir (including experimental and empirical equations of overburden pressure), (2) the history of producing well, (3) core flooding displacement experiments at laboratory, and (4) the acidic and alkaline solvent properties. Finally, the rate of forming water block (q) is calculated using the value calculated for the removed water block, and, additionally, the trend of using solvents is determined for different rocks using these sets of equations. The acceptance criteria are the nature of rock and fluid in the reservoir circumstances. Equations as a quick and cost-efficient method are also introduced, providing computational methods to determine how much and how the blocked fluid in the reservoir layers is removed from the definite strata around the wellbore after injection operation of acids and solvents, with various degrees of acidity, to the types of lithology during acidizing operations. Moreover, these equations can calculate the removed water block (Bk) after injecting solvents to the different acidic properties in the acidizing, for two categories of porosity which cover all lithologies. The equations also ascertain in the current reservoir conditions how much solvent for a type of lithology is to be mixed with other base fluids.",book:{id:"8229",slug:"oil-and-gas-wells",title:"Oil and Gas Wells",fullTitle:"Oil and Gas Wells"},signatures:"Mohammad Karimi, Mohammad Reza Adelzadeh, Mojtaba Mosleh Tehrani, Maryam Mohammadipour, Ruhangiz Mohammadian and Abbas Helalizade",authors:[{id:"298820",title:"Mr.",name:"M.",middleName:null,surname:"Karimi",slug:"m.-karimi",fullName:"M. 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