Pediatric Tricuspid Atresia
Tricuspid atresia may be defined as congenital absence or agenesis of the tricuspid valve.  It is the third most common cyanotic congenital heart defect; the other 2 frequently observed cyanotic congenital cardiac anomalies are transposition of the great arteries and tetralogy of Fallot. Tricuspid atresia is the most common cause of cyanosis with left ventricular hypertrophy. 
Although some authors state that Holmes (1824) or Kuhne (1906) first described tricuspid atresia,  Rashkind’s methodical and thorough historical review indicates that Kreysig (1817) reported the first case in 1817.  An 1812 report by the editors of the London Medical Review (1812) appears to fit the description of tricuspid atresia, but they did not use this specific term. 
Little more than 3 decades ago, the terminology for this defect (eg, tricuspid atresia, univentricular heart, univentricular atrioventricular connection) was intensely debated. [5, 6, 7, 8, 9, 10, 11] This debate was summarized in a 1990 issue of The American Journal of Cardiology,  in which Rao offered strong evidence and argued on the basis of data that Bharati and Lev, [10, 11] Wenink and Ottenkamp,  Gessner,  and Rao gathered support for tricuspid atresia as the correct and logical term to describe this well-characterized pathologic and clinical condition.
The atrioventricular valves develop shortly after the atrioventricular canal divides. The tricuspid valve leaflets have several origins. The septal leaflet of the tricuspid valve mostly develops from the inferior endocardial cushion with a small contribution from the superior cushion. The anterior and posterior tricuspid valve leaflets develop by undermining of a skirt of ventricular muscle tissue. The process of undermining extends until the atrioventricular valve junction is reached. Resorption of the muscle tissue produces normal-appearing valve leaflets and chordae tendineae. [15, 16, 17] Fusion of developing valve leaflet components results in stenosis (partial fusion) or atresia (complete fusion) of the valve. [17, 18]
Whether a muscular type of tricuspid atresia develops or whether well-formed but fused tricuspid-valve leaflets develop depends on the stage of development when the embryologic aberration takes place. [17, 18] The classic muscular form of tricuspid atresia develops if the embryologic insult occurs early in gestation, and fused valve leaflets occur if the embryologic abnormality occurs slightly later than this in gestation. If the valve fusion is incomplete, stenosis of the tricuspid valve develops.
The pathologic, clinical, and electrocardiographic features of tricuspid stenosis and atresia are similar.  Therefore, the fact that isolated congenital tricuspid stenosis belongs to the group of tricuspid atresia defects and that their embryologic developments are similar is no surprise. Thus, the tricuspid valve stenosis, tricuspid atresia with well-formed but fused valve leaflets, and the muscular type of tricuspid atresia represent a spectrum of morphologic abnormalities. [12, 18]
The pathologic anatomy of tricuspid atresia is best understood by reviewing variations in valvar morphology.
The most common type of tricuspid atresia is muscular (see the image below). [9, 20] It is characterized by a dimple or a localized fibrous thickening in the floor of the right atrium at the expected site of the tricuspid valve. The muscular variety accounts for 89% of cases. 
In the membranous type (6.6%), the atrioventricular portion of the membranous septum forms the floor of the right atrium at the expected location of the tricuspid valve. This particular type appears to be associated with absent pulmonary valve leaflets.
Minute valvar cusps are fused together in the valvar type (1%).
In the Ebstein type (2.6%), fusion of the tricuspid valve leaflets occurs; attachment is displaced downward, and plastering of the leaflets to the right ventricular wall occurs.  This variant is rare but well documented.
The atrioventricular canal type is extremely rare (0.2%). In this type, a leaflet of the common atrioventricular valve seals off the only entrance into the right ventricle. 
In the final type, unguarded with a muscular shelf (0.6%), the atrioventricular junction is unguarded, but the inlet component of the morphologic right ventricle is separated from its outlet by a muscular shelf. 
The right atrium is enlarged and hypertrophied. An interatrial communication is necessary for survival. This communication most commonly is a stretched patent foramen ovale. Sometimes, an ostium secundum or an ostium primum atrial septal defect (ASD) is present. In rare cases, the patent foramen ovale is obstructive and may form an aneurysm of the fossa ovalis, which is sometimes large enough to produce mitral inflow obstruction. The left atrium may be enlarged, especially when the pulmonary blood flow is increased. The mitral valve is morphologically normal; it is rarely incompetent and has a large orifice. The left ventricle is enlarged and hypertrophied but often morphologically normal.
The ventricular septal defect (VSD) is usually small; however, it can be large, or several VSDs may be present.  The ventricular septum is rarely intact. When present, the VSD may be conoventricular or perimembranous in type (inferior to the septal band), it may be of conal septal malalignment type (between the limbs of the septal band), or it may be of the muscular or atrioventricular canal type. [24, 25] Muscular VSDs are the most common defects and are usually restrictive; they produce subpulmonary stenosis in patients with normally related great arteries and simulate subaortic obstruction in patients with transposition of the great arteries. [26, 27]
The right ventricle is small and hypoplastic, and its size largely depends on the anatomic type.  In patients with a large VSD or transposition of the great arteries, the size of the right ventricle may be larger, but, even in these patients, the right ventricle is smaller than normal. In patients with pulmonary atresia and normally related great arteries, the right ventricle is small and may escape detection. However, it is a true right ventricle in most patients; it is composed of a sharply demarcated infundibulum with septal and parietal bands and a sinus with trabeculae, which may communicate with the left ventricle by means of a VSD. By definition, the inflow region is absent, although papillary muscles may occasionally be present.
The great artery relationship is variable and forms the basis of a major classification and will be described in the next section. Obstruction to the pulmonary outflow tract is present in most cases of tricuspid atresia and is used in the scheme of classification. The aorta is either normal or slightly larger than normal. In 30% of patients, various associated cardiac defects are present; aortic coarctation and persistent left superior vena cava are particularly notable.
Associated cardiac defects in tricuspid atresia outlined below. 
Defects that form the basis for classification are as follows:
D-Transposition of the great arteries
L-Transposition of the great arteries
Double outlet left ventricle
Other malpositions of the great arteries
Truncus arteriosus 
Defects that may need attention before or during palliative or total surgical correction are as follows:
Absent pulmonary valve
Aneurysm of the atrial septum
Anomalous origin of the coronary arteries from the pulmonary artery
Anomalous origin of the left subclavian artery
Anomalous origin of the right subclavian artery
Coarctation of the aorta
Cor triatriatum dexter
Coronary sinus atrial septal defect
Double aortic arch
Double-outlet left atrium
Hypoplastic ascending aorta and/or aortic atresia
Ostium primum ASD
Parchment right ventricle
Patent ductus arteriosus
Persistent left superior vena cava
Right aortic arch
Total anomalous pulmonary venous connection
Tubular hypoplasia of the aortic arch
Valvar aortic stenosis
Other associated defects are as follows:
Juxtaposition of the atrial appendages
Anomalous entry of coronary sinus into the left atrium
Tricuspid atresia is classified according to the morphology of the valve, [20, 31] the radiographic appearance of pulmonary vascular markings, [32, 33] and the associated cardiac defects. [3, 34, 35, 36, 37]
Van Praagh and associates (1971) initially proposed a classification based on the morphology of the atretic tricuspid valve.  He and others later modified and expanded the classification, as described in Tricuspid Atresia. [1, 6] All other morphologic types are described above in the Anatomy section. For pathologic, echocardiographic, and angiographic examples, particularly the rare anatomic types, the interested reader is referred to Tricuspid Atresia  and the Atlas of Heart Disease: Congenital Heart Disease. 
Astley and associates (1953) proposed a classification based on pulmonary vascular markings on a chest radiograph: Group A are cases with decreased pulmonary vascular markings, and group B are those with increased pulmonary vascular markings.  Dick et al (1975) added a third group, group C, to describe cases with a transition from increased to decreased pulmonary vascular markings.  This type of classification has some clinical value, although a more precise definition than these can often be made by using noninvasive 2-dimensional (2D) and Doppler echocardiography.
In 1906, Kuhne first proposed a classification based on great-artery relationships,  which Edwards and Burchell expanded in 1949.  Keith, Rowe, and Vlad popularized this classification in 1967.  Other investigators have offered various other classifications. These are reviewed in detail in the American Heart Journal  and Tricuspid Atresia.  Although these classifications are generally good, their exclusion of some variations in great-artery relationships and the lack of consistency in subgroups are problematic. Therefore, the following comprehensive-yet-unified classification was proposed  :
The principle grouping continues to be based on the following interrelationships of the great arteries:
Type I – Normally related great arteries
Type II – D-Transposition of the great arteries
Type III – Great artery positional abnormalities other than D-transposition of the great arteries: (1) Subtype 1 involves L-transposition of the great arteries, (2) subtype 2 involves double outlet right ventricle, (3) subtype 3 involves double outlet left ventricle, (4) subtype 4 involves D-malposition of the great arteries (anatomically corrected malposition), and (5) subtype 5 involves L-malposition of the great arteries (anatomically corrected malposition)
Type IV – Persistent truncus arteriosus
All types and subtypes are subdivided into the following subgroups:
Subgroup a – Pulmonary atresia
Subgroup b – Pulmonary stenosis or hypoplasia
Subgroup c – No pulmonary stenosis (normal pulmonary arteries)
After the above categorization, the status of the ventricular septum (intact or VSD) and the presence of other associated malformations are described.
This unified classification includes all the previously described abnormalities in the positions of the great arteries and can be further expanded if new variations are revealed. This classification maintains uniformity of the subgroups and preserves the basic principles of classification that Kuhne, Edwards and Burchell, and Keith, Rowe, and Vlad devised.
Despite the clinically significant alterations in fetal circulation in tricuspid atresia, such changes are not detrimental to normal fetal development.
In a fetus with a normally developed heart, a substantial portion of the highly saturated blood in the inferior vena cava, which carries umbilical venous return from the placenta, is diverted into the left atrium through the patent foramen ovale. From there, it traverses into the left ventricle and aorta. Thus, the brain and heart receive blood with a high partial pressure of oxygen (PO2). [38, 39] In the normal fetus, the desaturated blood in the superior vena cava passes through the tricuspid valve, right ventricle, and pulmonary artery. Because of high pulmonary vascular resistance (PVR), the desaturated blood is then diverted through the ductus arteriosus into the descending aorta and umbilical arteries. The blood then returns to the placenta for oxygenation. [38, 39]
In tricuspid atresia, blood from both venae cavae is forced across the patent foramen ovale into the left heart. As a consequence, the PO2 differential present in a normally developed fetus is not present in the fetus with tricuspid atresia. The lowered PO2 to the brain and heart and elevated PO2 to the lungs do not seem to produce clinically discernible postnatal abnormalities. [18, 38, 39]
In patients with tricuspid atresia and associated pulmonary atresia (types Ia and IIa), the pulmonary blood flow is supplied entirely through the ductus arteriosus. Therefore, the ductus only carries 8-10% of combined ventricular output compared with 66% of combined ventricular output in a normally developed fetus. Also, acute angulation of the ductus arteriosus occurs at its origin because of reversed direction of ductal flow. These 2 factors may make the ductus arteriosus less responsive to postnatal stimuli than it usually is.
In a fetus with tricuspid atresia type I anatomy and a small or absent VSD (types Ia and Ib), almost all the left ventricular output is ejected into the aorta and transported down to the placenta. As a consequence, the isthmus of the aorta carries a larger-than-normal proportion of cardiac output; this is thought to be the reason for rarity of aortic coarctation in this subset of patients with tricuspid atresia.
In contrast, in patients with tricuspid atresia type II (transposition of the great arteries), an increased portion of the blood goes through the ductus arteriosus into the descending aorta. Therefore, the flow across the aortic isthmus is minimal, which accounts for the relatively high incidence of aortic coarctation in this subset of patients. [18, 38, 39]
Because of the atretic tricuspid valve, all systemic venous blood must be shunted across the interatrial septal communication into the left atrium. This obligatory shunting causes admixture of all systemic venous and pulmonary venous returns. This blood then passes onto the left ventricle across the mitral valve. [18, 38, 39] This flow pattern occurs in all types but type III subtypes 1 and 5. In these exceptions, the atretic morphologic tricuspid valve is left sided because of ventricular inversion; therefore, the pathophysiology is that of mitral atresia with consequent left-to-right shunting of pulmonary venous return. [18, 39]
In patients with normally related great arteries (type I) and a VSD, shunting across the VSD permits perfusion of the lungs. In the absence of VSD, pulmonary blood flow is derived through patent ductus arteriosus or aortopulmonary collateral vessels. [18, 38, 39] Some means of lung perfusion is crucial for patient survival. The systemic blood flow is derived directly from the left ventricle.
In patients with D-transposition of the great arteries (type II), the lungs receive the blood flow from the left ventricle. The aorta receives blood from the left ventricle via the VSD and right ventricle. [18, 38, 39] In other types of tricuspid atresia, the routes of aortic and pulmonary artery flow depend on the size of the VSD and associated cardiac defects.
Systemic arterial desaturation is present in all patients with tricuspid atresia because of obligatory admixture of the systemic, coronary, and pulmonary venous returns in the left atrium. The degree of arterial desaturation depends on the amount of pulmonary blood-flow. [18, 39] The arterial oxygen saturation has a curvilinear relationship (see the image below), with a pulmonary-to-systemic blood flow ratio (Qp:Qs) that reflects the pulmonary blood flow. A Qp:Qs ratio of 1.5-2.5 seems to result in adequate oxygen saturation. Higher pulmonary flow does not significantly increase oxygen saturation but instead produces left ventricular volume overloading.
Pulmonary blood flow
The clinical features of tricuspid atresia largely depend on the quantity of pulmonary blood flow. [2, 18, 40, 41, 42] A neonate with markedly decreased pulmonary flow is likely to present early in the neonatal period with signs of severe cyanosis, hypoxemia, and acidosis. On the contrary, if the pulmonary blood flow is increased, the neonate may not appear cyanotic but may present with signs of heart failure later in infancy. Patients with pulmonary oligemia generally have type I (normally related great arteries); those with pulmonary plethora usually have type II (transposition of the great arteries) and, rarely, type Ic.
The magnitude of pulmonary blood flow without previous surgery largely depends on the degree of pulmonary outflow tract obstruction and patency of the ductus arteriosus. In patients with a type I defect, the obstruction is valvar, subvalvar, or, most frequently, at the VSD level. In patients with a type II defect, the obstruction is either valvar or subvalvar. In patients with a type I defect, if the VSD is large and nonrestrictive without pulmonary stenosis, the pulmonary flow is inversely proportional to the pulmonary-to-systemic vascular resistance ratio. If the ductus is patent or if a surgical systemic-to-pulmonary artery shunt was performed, the pulmonary blood flow is proportional to the size of the natural or surgical aortopulmonary connection.
Left ventricular volume overloading
The left ventricle ejects the entire systemic, coronary, and pulmonary outputs. Therefore, left ventricular volume overloading is present in all patients with tricuspid atresia. [18, 40] The degree of volume overloading increases further if mild or absent pulmonary outflow obstruction is noted or if systemic-to-pulmonary artery shunt was performed. Because normal left ventricular function is critical to a successful Fontan operation, maintenance of normal left ventricular function is essential. Left ventricular function tends to decrease with increasing age, increasing Qp:Qs, and arterial desaturation. [43, 44, 45]
Obstruction of the interatrial communication
Patency of the interatrial communication, usually a patent foramen ovale, is essential for survival. Because the entire systemic venous blood must egress through the interatrial communication, development of interatrial obstruction is not unexpected, but it is rarely clinically significant, especially in the neonate. Right-to-left shunting occurs in late atrial diastole, with augmentation of flow during atrial systole.
Obstruction of the patent foramen ovale is presumed to be present if the mean pressure difference between the atria is more than 5 mm Hg and a tall a wave is present in the right atrial pressure trace.  Clinical evaluation may reveal prominent a waves in the jugular venous pulse, presystolic hepatic pulsations, and hepatomegaly. One study suggested that atrial septal aneurysm and an atrial septal defect diameter smaller than 5 mm are associated with an increased risk for developing an atrial septal obstruction. 
Several changes in hemodynamics occur as infants with tricuspid atresia grow. These involve the ductus arteriosus, ASD, and VSD.
Closure of the ductus arteriosus in a neonate with severe pulmonary outflow tract obstruction or atresia results in severe hypoxemia, and the administration of prostaglandin E1 (PGE1) or surgical creation of systemic-to-pulmonary artery shunt is required.
Regarding ASD, restrictive interatrial communication may develop, causing systemic venous congestion. Transcatheter or surgical atrial septostomy may be needed.
Patency of the VSD is essential to maintain intracardiac shunting necessary for patient survival; these VSDs have been named physiologically advantageous VSDs. [27, 47] Functional  and partial or complete anatomic [23, 26, 47, 49] closures have been documented. Intermittent functional closure of the VSD results in cyanotic spells in tricuspid atresia.  The etiology of such closures has not been identified but is likely similar to that postulated for tetralogy of Fallot.
Closure of a VSD in type I may result in progressive cyanosis, increasing polycythemia, and diminution or disappearance of the murmur of VSD. Both partial and complete closures are reported and require surgical intervention earlier than otherwise anticipated.
Closure of a VSD in type II (transposition) produces subaortic (ie, systemic) outflow obstruction. Partial closures have been reported; however, to the author’s knowledge, complete closures have not been documented. Partial closures result in increased left ventricular mass, complicating Fontan operations.
From the author’s studies [26, 50] and those of Sauer and Hall,  the estimated prevalence of spontaneous VSD closures is 38-48%.  This prevalence is similar to that of isolated VSDs. [52, 53] VSD closures are documented in patients aged 1 year to 20 years, with a median of age 1.3 years. These statistics are also similar to those observed in isolated defects.
The most common mechanism of closure is progressive muscular encroachment of margins of the defect with subsequent fibrosis and covering by endocardial proliferation, although other mechanisms of closure seen in isolated VSDs have been observed in tricuspid atresia patients. How such closures are initiated is unknown.
The etiology of tricuspid atresia is unknown.
A multifactorial inheritance hypothesis is offered to explain all congenital heart defects, including tricuspid atresia. This hypothesis states that disease results if a predisposed fetus is exposed to a given environmental trigger (to which the fetus is sensitive) during a critical period of cardiac morphogenesis. This genetic and environmental interaction is most likely the pathogenic mechanism for congenital heart defects in general and for tricuspid atresia in particular.
Various risk factors are statistically associated with certain heart defects. However, no specific factors are clearly identified for tricuspid atresia.
Although the true incidence of tricuspid atresia is not well defined, the prevalence of tricuspid atresia among congenital heart defects was estimated to be 2.9% in autopsy series and 1.4% in clinical series after extensive review.  Given the prevalence of congenital heart defects in 0.8% of live births, tricuspid atresia may be estimated to occur in approximately 1 per 10,000 live births. 
Extensive review of the literature indicated no differences in prevalence in tricuspid atresia between the United States and countries on other continents (see the image below), although geographic differences in prevalence for aortic stenosis and coarctation have been documented.
Although data in the 1950s and early 1960s indicated that the prevalence of congenital heart disease was higher in whites than in blacks, a thorough and appropriate statistical analysis by Mitchell et al suggests that the prevalences of congenital heart disease are similar in whites and blacks (8.3 vs 8.1 per 1000).  According to Schriere, the incidences of tricuspid atresia among congenital heart defects in South Africa are 1.2% in whites and 1.4% in African blacks , indicating no racial predilection. 
Furthermore, extensive review and tabulation of the prevalences of tricuspid atresia in populations on several continents revealed no difference in prevalences despite different racial compositions on these continents (see the image below). Therefore, no specific racial predilection is noted for tricuspid atresia. 
Some researchers have found a slight male preponderance for tricuspid atresia. An extensive review of 1857 cases revealed that 53% of cases occurred in male individuals and 47% occurred in female individuals. However, these findings were not statistically significant (P >.1), indicating no evidence for sex predilection. 
Dick et al suggested that a male preponderance exists only in patients with tricuspid atresia with associated transposition.  To test this hypothesis, the authors (1992) evaluated data of patients in whom sex and great-artery relationships were known. In patients without transposition of the great arteries, the prevalences were 54% in male patients and 46% in female patients (P >.1). In patients with transposition of the great arteries, the prevalence was higher in male patients than in female patients (66% vs 34%, P< .05). Therefore, a male preponderance for tricuspid atresia was observed in patients with transposition of the great arteries (type II).
Patients with tricuspid atresia present early in life. One half of patients present on the first day of life, two thirds present by the end of the first week, and 80% present by the first month of life. [2, 18, 41] No more than 15% of patients present with symptoms for the first time after 2 months of life.
The magnitude of pulmonary blood flow determines the timing and mode of presentation. Neonates with pulmonary oligemia present early in life with cyanosis, whereas those with pulmonary plethora present slightly later with signs of congestive heart failure, cyanosis, or both, depending on the magnitude of pulmonary flow.
Poor prognosis of untreated tricuspid atresia patients is well known; only 10-20% of infants may live through the first year of life.
The image below shows actuarial survival rates from 3 medical centers that Dick and Rosenthal compiled.  Considerable early mortality occurs and may be related to hypoxemia, cardiac failure, surgical intervention, or their combination. Surgical palliation to normalize pulmonary blood flow by means of systemic-to-pulmonary artery shunts in neonates with pulmonary oligemia and banding of the pulmonary artery in infants with markedly increased pulmonary flow improves survival rates.
The availability of PGE1 to keep the ductus open and advances in neonatal care (eg, early identification, safe transport to a tertiary care institution, noninvasive diagnosis by means of echocardiography), anesthesia, and surgical techniques should further decrease the initial mortality rate. 
After the early high mortality rate, survival curves become stable and reach a plateau, as shown in the figure below. In patients aged approximately 15 years, a second fall in survival begins and continues through the remaining observation period. Physiologically corrective Fontan procedures may reverse this late mortality. Whether the benefits of Fontan procedure (ie, improving hypoxemia and eliminating left ventricular volume overloading) improve survival rates is not clear. Preliminary data suggest that they do, even after the immediate and late mortality of the surgery itself are accounted for. This potential for improved prognosis means that each patient with tricuspid atresia should undergo aggressive medical and surgical treatment. The natural history after a Fontan operation is shown below.
Adult patients who had classic Fontan operation have high initial mortality (28%) and high morbidity rates.  The latter is related to reoperation (58%) to revise Fontan connections, arrhythmia (56%) and thromboembolic events (25%). Patients with a total cavopulmonary connection appear to have improved survival and decreased morbidity rates, although follow-up of these patients has been relatively short.
The natural history of the component defects (ie, patent ductus arteriosus, ASD and/or patent foramen ovale, and VSD) is described above.
Development of bacterial endocarditis, brain abscess, and stroke may be considered as complications of the disease itself. Arrhythmias, obstructed venous pathway, and protein-losing enteropathy are some of the complications observed after Fontan surgery.
Tricuspid atresia is a complex cardiac defect requiring multiple and sometimes frequent medical, transcatheter, and surgical interventions. A detailed explanation of the cardiac defects (including pictorial drawings and heart models) and treatment required should be given to the parents at the time of diagnosis and repeated as needed.
Rao PS. Tricuspid atresia: anatomy, imaging, and natural history. Braunwald E, Freedom R, eds. Atlas of Heart Disease: Congenital Heart Disease. Philadelphia, PA: Current Medicine; 1997. Vol 12: 14.1.
Rao PS. Tricuspid atresia. Curr Treat Options Cardiovasc Med. 2000 Dec. 2(6):507-20. [Medline].
Kuhne M. Uber zwei falle kongenitaler atreside des ostium venosum dextrum. Jahrb Kinderh. 1906. 63:235.
Rashkind WJ. Tricuspid atresia: a historical review. Pediatr Cardiol. 1982. 2(1):85-8. [Medline].
Rao PS. Terminology: is tricuspid atresia the correct term to use?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 3-15.
Rao PS. Classification of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 59-79.
Rao PS. Classification of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 41-7.
Rao PS. Terminology: tricuspid atresia or univentricular heart?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 3-6.
Anderson RH, Wilkinson JL, Gerlis LM, et al. Atresia of the right atrioventricular orifice. Br Heart J. 1977 Apr. 39(4):414-28. [Medline].
Bharati S, Lev M. The concept of tricuspid atresia complex as distinct from that of the single ventricle complex. Pediatr Cardiol. 1979. 1:57.
Bharati S, McAllister HA Jr, Tatooles CJ, et al. Anatomic variations in underdeveloped right ventricle related to tricuspid atresia and stenosis. J Thorac Cardiovasc Surg. 1976 Sep. 72(3):383-400. [Medline].
Rao PS. Is the term “tricuspid atresia” appropriate?. Am J Cardiol. 1990 Nov 15. 66(17):1251-4. [Medline].
Wenink AC, Ottenkamp J. Tricuspid atresia. Microscopic findings in relation to “absence” of the atrioventricular connexion. Int J Cardiol. 1987 Jul. 16(1):57-73. [Medline].
Gessner IH. Embryology of atrioventricular valve formation and embryogenesis of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 25-40.
Ando M, Santomi G, Takao A. Atresia of tricuspid and mitral orifice: anatomic spectrum and morphogenetic hypothesis. Van Praagh R, Takao A, eds. Etiology and Morphogenesis of Congenital Heart Disease. NY: Futura: Mount Kisco; 1980: 421-87.
Van Mierop LH, Gessner IH. Pathogenetic mechanisms in congenital cardiovascular malformations. Prog Cardiovasc Dis. 1972 Jul-Aug. 15(1):67-85. [Medline].
Wilson AD, Rao PS. Embryology. Kambam J, ed. Cardiac Anesthesia for Infants and Children. St Louis, Mo: Mosby; 1994. 3-9.
Rao PS. Tricuspid atresia. Long WA, ed. Fetal and Neonatal Cardiology. Philadelphia, Pa: WB Saunders; 1990. 525-40.
Keefe JF, Wolk MJ, Levine HJ. Isolated tricuspid valvular stenosis. Am J Cardiol. 1970 Feb. 25(2):252-7. [Medline].
Van Praagh R, Ando M, Dungan WT. Anatomic types of tricuspid atresia: clinical and developmental implications [abstract]. Circulation. 1971. 44:115.
Rao PS, Jue KL, Isabel-Jones J, Ruttenberg HD. Ebstein’s malformation of the tricuspid valve with atresia. Differentiation from isolated tricuspid atresia. Am J Cardiol. 1973 Dec. 32(7):1004-9. [Medline].
Scalia D, Russo P, Anderson RH, et al. The surgical anatomy of hearts with no direct communication between the right atrium and the ventricular mass–so-called tricuspid atresia. J Thorac Cardiovasc Surg. 1984 May. 87(5):743-55. [Medline].
Rao PS. Natural history of ventricular septal defects in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 32(7): 261-93.
Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg. 1980 Apr. 29(4):306-11. [Medline].
Weinberg PM. Pathologic anatomy of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Futura: Mount Kisco, NY; 1992. 49-67.
Rao PS. Physiologically advantageous ventricular septal defects. Pediatr Cardiol. 1983 Jan-Mar. 4(1):59-61. [Medline].
Ottenkamp J, Wenink AC, Quaegebeur JM, et al. Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications. J Thorac Cardiovasc Surg. 1985 Apr. 89(4):597-603. [Medline].
Rao PS, Covitz W, Chopra PS. Principles of palliative management of patients with tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 4(1): 297-320.
Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF. Tricuspid atresia: association with persistent truncus arteriosus. Am Heart J. 1991 Sep. 122(3 Pt 1):829-35. [Medline].
Van Praagh R. Discussion after paper by Vlad P: Pulmonary atresia with intact ventricular septum. Barrett-Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy: Diagnosis and Surgical Treatment. 2nd ed. London: Churchill Livingstone; 1992. 1973:236.
Dick M, Fyler DC, Nadas AS. Tricuspid atresia: clinical course in 101 patients. Am J Cardiol. 1975 Sep. 36(3):327-37. [Medline].
Edwards JE, Burchell HB. Congenital tricuspid atresia: a classification. Med Clin North Am. 1949. 33:1117.
Keith J, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. Tricuspid Atresia. 2nd ed. New York: Macmillian; 1967. 434: 664.
Vlad P. Tricuspid atresia. Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Childhood. 3rd ed. New York: Macmillian; 1977. 518-41.
Rao PS. A unified classification for tricuspid atresia. Am Heart J. 1980 Jun. 99(6):799-804. [Medline].
Rudolph AM. Tricuspid atresia with hypoplastic right ventricle. Congenital Disease of the Heart. Chicago, Ill: Year Book Medical; 1974. 429-61.
Rao PS. Perinatal circulatory physiology. Indian J Pediatr. 1991 Jul-Aug. 58(4):441-51. [Medline].
Rao PS. Tricuspid atresia. Moller JH, Hoffman JIE, eds. Pediatric Cardiovascular Medicine. 2nd ed. Oxford, UK: Wiley-Blackwell; 2012. 487-508.
Dick M, Rosenthal A. The clinical profile of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1982. 83-111.
Rosenthal A, Dick M, II. Tricuspid atresia. Adams FH, Emmanouilides GC, eds. Moss’ Heart Disease in Infants, Children, and Adolescents. 3rd ed. Baltimore, Md: Lippincott Williams & Wilkins; 1983. 271.
La Corte MA, Dick M, Scheer G, La Farge CG, Fyler DC. Left ventricular function in tricuspid atresia. Angiographic analysis in 28 patients. Circulation. 1975 Dec. 52(6):996-1000. [Medline].
Graham TP Jr, Erath HG Jr, Boucek RJ Jr, Boerth RC. Left ventricular function in cyanotic congenital heart disease. Am J Cardiol. 1980 Jun. 45(6):1231-6. [Medline].
Rao PS, Alpert BS, Covitz W. Left ventricular function in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 52(6): 247-59.
Tzifa A, Gauvreau K, Geggel RL. Factors associated with development of atrial septal restriction in patients with tricuspid atresia involving the right-sided atrioventricular valve. Am Heart J. 2007 Dec. 154(6):1235-41. [Medline].
Rao PS, Sissman NJ. Spontaneous closure of physiologically advantageous ventricular septal defects. Circulation. 1971 Jan. 43(1):83-90. [Medline].
Rao PS, Linde LM, Liebman J, Perrin E. Functional closure of physiologically advantageous ventricular septal defects. Observations in three cases with tricuspid atresia. Am J Dis Child. 1974 Jan. 127(1):36-40. [Medline].
Gallaher ME, Fyler DC. Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels. Circulation. 1967 Feb. 35(2):381-8. [Medline].
Rao PS. Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: surgical implications. Ann Thorac Surg. 1983 Feb. 35(2):121-31. [Medline].
Sauer U, Hall D. Spontaneous closure or critical decrease in size of the ventricular septal defect in tricuspid atresia with normally connected great arteries: surgical implications. 1980. 5:369.
Bloomfield DK. The natural history of ventricular septal defect in patients surviving infancy. Circulation. 1964 Jun. 29:914-55. [Medline].
Hoffman JI, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965 Nov. 16(5):634-53. [Medline].
Rao PS. Demographic features of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 23-37.
Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circulation. 1971 Mar. 43(3):323-32. [Medline].
Schrire V. Experience with congenital heart disease at Groote Schuur Hospital, Cape Town. An analysis of 1,439 patients over an eleven-year period. S Afr Med J. 1963 Nov 23. 37:1175-80. [Medline].
Rao PS. Management of neonate with suspected serious heart disease. King Faisal Spec Hosp Med J. 1984. (4):209.
Marcelletti CF, Hanley FL, Mavroudis C, et al. Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience. J Thorac Cardiovasc Surg. 2000 Feb. 119(2):340-6. [Medline].
Marcano BA, Riemenschneider TA, Ruttenberg HD, Goldberg SJ, Gyepes M. Tricuspid atresia with increased pulmonary blood flow. An analysis of 13 cases. Circulation. 1969 Sep. 40(3):399-410. [Medline].
Rao PS. Pathophysiologic consequences of cyanotic congenital heart disease. Indian J Pediatr. Sep-Oct 1983. 50(406):479-87.
Covitz W, Rao PS. Non-invasive evaluation of patients with tricuspid atresia (roentgenography, echocardiography and nuclear angiography). Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992: 165-82.
Rao PS. Tricuspid atresia. Vijayalakshmi IB, Rao PS, Chugh R, eds. A Comprehensive Approach to Congenital Heart Diseases. New Delhi, India: Jaypee Brothers Medical Publishers; 2013. 397-413.
Rao PS. Tricuspid atresia. Rao PS, Vidyasagar D, eds. Perinatal Cardiology: A Multidisciplinary Approach: A Multidisciplinary Approach. Minneapolis, Mn: Cardiotext Publishing; 2015. Chapter 30.
Gamboa R, Gersony WM, Nadas AS. The electrocardiogram in tricuspid atresia and pulmonary atresia with intact ventricular septum. Circulation. 1966 Jul. 34(1):24-37. [Medline].
Rao PS, Kulungara RJ, Boineau JP. Electrovectorcardiographic features of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 141-64.
Rao PS. Cardiac catheterization in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 153-78.
Rao PS. Cardiac catheterization in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, New York: Futura; 1992: 193-221.
Rao PS. The femoral route for cardiac catheterization of infants and children. Chest. 1973 Feb. 63(2):239-41. [Medline].
Rao PS. Left to right atrial shunting in tricuspid atresia. Br Heart J. 1983 Apr. 49(4):345-9. [Medline].
Rao PS, Sissman NJ. The relationship of pulmonary venous wedge to pulmonary arterial pressures. Circulation. 1971 Oct. 44(4):565-74. [Medline].
Mair DD, Hagler DJ, Puga FJ, Schaff HV, Danielson GK. Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation. 1990 Nov. 82(5 Suppl):IV164-9. [Medline].
Schwartz DC, Rao PS. Angiography in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992. 1(3): 223-46.
Rao PS. Value of pulmonary vein wedge angiography in visualization of obstructed ipsilateral pulmonary artery. Cardiovasc Radiol. 1978 Jul 25. 1(3):151-2. [Medline].
Rao PS. Principles of management of the neonate with congenital heart disease neonatology Today. 2007. 2(8):1-10.
Freed MD, Heymann MA, Lewis AB, Roehl SL, Kensey RC. Prostaglandin E1 infants with ductus arteriosus-dependent congenital heart disease. Circulation. 1981 Nov. 64(5):899-905. [Medline].
Rao PS. Transcatheter blade atrial septostomy. Cathet Cardiovasc Diagn. 1984. 10(4):335-42. [Medline].
Rao PS. Role of interventional cardiology in neonates: Part I. Non-surgical atrial septostomy. Congenital Cardiol Today. 2007 Dec. 5(12):1-12.
Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002 Sep 24. 106(12 Suppl 1):I82-9. [Medline].
Yates MC, Rao PS. Pediatric cardiac emergencies. Emerg Med. 2013. 3(164):doi:10.4172/2165-7548.1000164. [Full Text].
Tsounias E, Rao PS. Stent therapy for clotted Blalock-Taussig shunts. Congenital Cardiol Today. 2010. 8(7):1-9.
Lichtman SW, Caravano M, Schneyman M, Howell B, King ML. Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: a case study. J Cardiopulm Rehabil Prev. 2008 Jan-Feb. 28(1):48-51. [Medline].
Strong WB, Morera JA, Rao PS. Sexuality, contraception and pregancy in patients with cyanotic congenital heart disease with special reference to tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992. 16(1): 415-27.
Rashkind W, Waldhausen J, Miller W, Friedman S. Palliative treatment in tricuspid atresia. Combined balloon atrioseptostomy and surgical alteration of pulmonary blood flow. J Thorac Cardiovasc Surg. 1969 Jun. 57(6):812-8. [Medline].
McCredie RM, Swinburn MJ, Lee CL, Warner G. Balloon dilatation pulmonary valvuloplasty in pulmonary stenosis. Aust N Z J Med. 1986 Feb. 16(1):20-3. [Medline].
Rao PS, Levy JM, Chopra PS. Balloon angioplasty of stenosed Blalock-Taussig anastomosis: role of balloon-on-a-wire in dilating occluded shunts. Am Heart J. 1990 Nov. 120(5):1173-8. [Medline].
Rao PS. Current status of balloon angioplasty for neonatal and infant aortic coarctation. Progress Pediat Cardiol. 2001. 14:35-44.
Rao PS, Jureidini SB, Balfour IC, Singh GK, Chen S. Severe aortic coarctation in infants less than 3 months: Successful palliation by balloon angioplasty. J Invasive Cardiol. 2003. 15:203-208.
Rao PS, Balfour IC, Singh GK, Jureidini SB, Chen S. Bridge stents in the management of obstructive vascular lesions in children. Am J Cardiol. 2001. 88:699-702.
Rao PS. Stents in the management of congenital heart disease in pediatric and adult patients. Indian Heart J. 2001 Nov-Dec. 53(6):714-30. [Medline].
Siblini G, Rao PS. Coil Embolization in the Management of Cardiac Problems in Children. J Invasive Cardiol. 1996. 8:332-40.
Rao PS. Transcatheter embolization of unwanted blood vessels in children. Rao PS, Kern MJ, eds. Catheter Based Devices for Treatment of Noncoronary Cardiovascular Disease in Adults and Children. Philadelphia, PA: Lippincott, Williams & Wilkins; 2003. 251(16): 2123-38.
Rao PS, Chandar JS, Sideris EB. Role of inverted buttoned device in transcatheter occlusion of atrial septal defects or patent foramen ovale with right-to-left shunting associated with previously operated complex congenital cardiac anomalies. Am J Cardiol. 1997 Oct 1. 80(7):914-21. [Medline].
Goff DA, Blume ED, Gauvreau K, Mayer JE, Lock JE, Jenkins KJ. Clinical outcome of fenestrated Fontan patients after closure: the first 10 years. Circulation. 2000 Oct 24. 102(17):2094-9. [Medline].
Boudjemline Y, Bonnet D, Sidi D, Agnoletti G. [Closure of extrocardiac Fontan fenestration by using the Amplatzer duct occluder]. Arch Mal Coeur Vaiss. 2005 May. 98(5):449-54. [Medline].
Rothman A, Evans WN, Mayman GA. Percutaneous fenestration closure with problematic residual native atrial septum. Catheter Cardiovasc Interv. 2005 Oct. 66(2):286-90. [Medline].
Sugiyama H, Yoo SJ, Williams W, Benson LN. Characterization and treatment of systemic venous to pulmonary venous collaterals seen after the Fontan operation. Cardiol Young. 2003 Oct. 13(5):424-30. [Medline].
Tsounias E, Rao PS. Versatility of Amplatzer Vascular Plug in occlusion of different types of vascular channels. Catheter Cardiovasc Interv. 2008. 71:63.
Alsoufi B, Schlosser B, Mori M, et al. Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 2015 Oct. 100 (4):1403-9; discussion 1409-10. [Medline].
Blalock A, Taussig HB. Landmark article May 19, 1945: The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. By Alfred Blalock and Helen B. Taussig. JAMA. 1984 Apr 27. 251(16):2123-38. [Medline].
de Leval MR, McKay R, Jones M, Stark J, Macartney FJ. Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts. J Thorac Cardiovasc Surg. 1981 Jan. 81(1):112-9. [Medline].
Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: a report of 5 patients. Ann Thorac Surg. 1980 Apr. 29(4):317-21. [Medline].
Gibbs JL, Rothman MT, Rees MR, Parsons JM, Blackburn ME, Ruiz CE. Stenting of the arterial duct: a new approach to palliation for pulmonary atresia. Br Heart J. 1992 Mar. 67(3):240-5. [Medline]. [Full Text].
Alwi M, Choo KK, Latiff HA, Kandavello G, Samion H, Mulyadi MD. Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation. J Am Coll Cardiol. 2004 Jul 21. 44(2):438-45. [Medline].
Rao PS. Subaortic obstruction after pulmonary artery banding in patients with tricuspid atresia and double-inlet left ventricle and ventriculoarterial discordance. J Am Coll Cardiol. 1991 Nov 15. 18(6):1585-6. [Medline].
Bonnet D, Sidi D, Vouhe PR. Absorbable pulmonary artery banding in tricuspid atresia. Ann Thorac Surg. 2001 Jan. 71(1):360-1; discussion 361-2. [Medline].
Rao PS. Absorbable pulmonary artery band in tricuspid atresia (Editorial). Ann Thorac Surg. 2001. 71:361-362.
Park SC, Neches WH, Zuberbuhler JR, et al. Clinical use of blade atrial septostomy. Circulation. 1978 Oct. 58(4):600-6. [Medline].
Seliem M, Muster AJ, Paul MH, Benson DW Jr. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coll Cardiol. 1989 Sep. 14(3):750-5. [Medline].
Smolinsky A, Castaneda AR, Van Praagh R. Infundibular septal resection: surgical anatomy of the superior approach. J Thorac Cardiovasc Surg. 1988 Mar. 95(3):486-94. [Medline].
Kreutzer G, Bono H, Galindez E. Una operacion para la correccion de la atresia tricuspidea. Ninth Argent Congress of Cardiology; Buenos Aires, Argentina. October 31-November 6, 1971.
Glenn WW. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application. N Engl J Med. 1958 Jul 17. 259(3):117-20. [Medline].
Chopra PS, Rao PS. Corrective surgery for tricuspid atresia: which modification of Fontan-Kreutzer procedure should be used? A review. Am Heart J. 1992 Mar. 123(3):758-67. [Medline].
Rao PS, Chopra PS. Modifications of Fontan-Kreutzer procedure for tricuspid atresia: can a choice be made?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, New York: Futura; 1992. 34(2): 361-75.
Haller JA Jr, Adkins JC, Worthington M, Rauenhorst J. Experimental studies on permanent bypass of the right heart. Surgery. 1966 Jun. 59(6):1128-32. [Medline].
Choussat A, Fontan F, Besse P, et al. Selection criteria for Fontan procedure. Anderson RH, Shinebourne EA, eds. Pediatric Cardiology. White Plains, NY: Churchill Livingstone; 1978. 559.
Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1989 May. 97(5):746-54. [Medline].
Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990 Nov. 82(5):1681-9. [Medline].
Laks H, Pearl JM, Haas GS, et al. Partial Fontan: advantages of an adjustable interatrial communication. Ann Thorac Surg. 1991 Nov. 52(5):1084-94; discussion 1094-5. [Medline].
Thompson LD, Petrossian E, McElhinney DB, et al. Is it necessary to routinely fenestrate an extracardiac Fontan?. J Am Coll Cardiol. 1999 Aug. 34(2):539-44. [Medline].
de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988 Nov. 96(5):682-95. [Medline].
Sharma S, Goudy S, Walker P, et al. In vitro flow experiments for determination of optimal geometry of total cavopulmonary connection for surgical repair of children with functional single ventricle. J Am Coll Cardiol. 1996 Apr. 27(5):1264-9. [Medline].
Kumar SP, Rubinstein CS, Simsic JM, Taylor AB, Saul JP, Bradley SM. Lateral tunnel versus extracardiac conduit Fontan procedure: a concurrent comparison. Ann Thorac Surg. 2003 Nov. 76(5):1389-96; discussion 1396-7. [Medline].
Ro PS, Rychik J, Cohen MS, Mahle WT, Rome JJ. Diagnostic assessment before Fontan operation in patients with bidirectional cavopulmonary anastomosis: are noninvasive methods sufficient?. J Am Coll Cardiol. 2004 Jul 7. 44(1):184-7. [Medline].
Adachi I, Yagihara T, Kagisaki K, et al. Fontan operation with a viable and growing conduit using pedicled autologous pericardial roll: serial changes in conduit geometry. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1517-22. [Medline].
Yalcinbas YK, Erek E, Salihoglu E, Sarioglu A, Sarioglu T. Early results of extracardiac fontan procedure with autologous pericardial tube conduit. Thorac Cardiovasc Surg. 2005 Feb. 53(1):37-40. [Medline].
Lemler MS, Ramaciotti C, Stromberg D, Scott WA, Leonard SR. The extracardiac lateral tunnel Fontan, constructed with bovine pericardium: comparison with the extracardiac conduit Fontan. Am Heart J. 2006 Apr. 151(4):928-33. [Medline].
Sreeram N, Alvarado O, Peart I. Tricuspid atresia with common arterial trunk: surgical palliation in a neonate. Int J Cardiol. 1991 Aug. 32(2):251-3. [Medline].
Gonzalez-Lopez MT, Crucean A, Seale A, McGuirk S. Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart. Interact Cardiovasc Thorac Surg. 2015 Aug. 21(2):252-3. [Medline].
Milovanovic V, Stefanovic I, Ilic S. Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas. Cardiol Young. 2016 Sep 29. 1-4. [Medline].
Futuki A, Fujiwara K, Yoshizawa K, Sakazaki H. Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful Fontan completion. World J Pediatr Congenit Heart Surg. 2016 Aug 22 [Epub ahead of print]. [Medline].
Yokoyama S, Kaneda K, Nagasaka S, et al. Tricuspid atresia IIc with a vascular ring: novel approach for Fontan completion. Ann Thorac Surg. 2016 Mar. 101(3):1188-90. [Medline].
McMahon CJ, Nolke L. Successful palliation of a child with left ventricular noncompaction cardiomyopathy and tricuspid atresia to Fontan procedure. Ann Thorac Surg. 2014 Aug. 98(2):719-21. [Medline].
Shimada M, Sakamoto T, Umezu K, Harada Y. Successful staged Fontan completion for a tricuspid atresia patient with left ventricular non-compaction. Interact Cardiovasc Thorac Surg. 2016 Mar. 22(3):387-9. [Medline].
Konertz W, Schneider M, Herwig V, Kampmann C, Waldenberger F, Hausdorf G. Modified hemi-Fontan operation and subsequent nonsurgical Fontan completion. J Thorac Cardiovasc Surg. 1995 Sep. 110(3):865-7. [Medline].
Hausdorf G, Schneider M, Konertz W. Surgical preconditioning and completion of total cavopulmonary connection by interventional cardiac catheterisation: a new concept. Heart. 1996 Apr. 75(4):403-9. [Medline]. [Full Text].
Sidiropoulos A, Ritter J, Schneider M, Konertz W. Fontan modification for subsequent non-surgical Fontan completion. Eur J Cardiothorac Surg. 1998 May. 13(5):509-12; discussion 512-3. [Medline].
Galantowicz M, Cheatham JP. Fontan completion without surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004. 7:48-55. [Medline].
van den Bosch AE, Roos-Hesselink JW, Van Domburg R, Bogers AJ, Simoons ML, Meijboom FJ. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol. 2004 May 1. 93(9):1141-5. [Medline].
Freedom RM, et al. The Fontan procedure for patients with tricuspid atresia: long-term follow-up. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 128(1): 377.
Stamm C, Friehs I, Mayer JE Jr, et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001 Jan. 121(1):28-41. [Medline].
Lubiszewska B, Rozanski J, Demkow M, et al. Long-term results of Fontan procedure in 43 patients. Kardiol Pol. 2003 Mar. 58(3):207-16. [Medline].
Alphonso N, Baghai M, Sundar P, Tulloh R, Austin C, Anderson D. Intermediate-term outcome following the Fontan operation: a survival, functional and risk-factor analysis. Eur J Cardiothorac Surg. 2005 Oct. 28(4):529-35. [Medline].
Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S, Spray TL. Intermediate outcomes after the Fontan procedure in the current era. J Thorac Cardiovasc Surg. 2006 Jan. 131(1):172-80. [Medline].
McCrindle BW, Williams RV, Mitchell PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation. 2006 Feb 28. 113(8):1123-9. [Medline].
Sheikh AM, Tang AT, Roman K, et al. The failing Fontan circulation: successful conversion of atriopulmonary connections. J Thorac Cardiovasc Surg. 2004 Jul. 128(1):60-6. [Medline].
Hill DJ, Feldt RH, Porter C. Protein losing enteropathy after Fontan operation: a preliminary report. Circulation. 1989. 80(Suppl II):490.
Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998 May. 115(5):1063-73. [Medline].
Rao PS. Protein-losing enteropathy following the Fontan operation. J Invasive Cardiol. 2007 Oct. 19(10):447-8. [Medline].
Feldt RH, Driscoll DJ, Offord KP, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996 Sep. 112(3):672-80. [Medline].
Giannico S, Hammad F, Amodeo A, et al. Clinical outcome of 193 extracardiac Fontan patients: the first 15 years. J Am Coll Cardiol. 2006 May 16. 47(10):2065-73. [Medline].
Chiu NT, Lee BF, Hwang SJ, Chang JM, Liu GC, Yu HS. Protein-losing enteropathy: diagnosis with (99m)Tc-labeled human serum albumin scintigraphy. Radiology. 2001 Apr. 219(1):86-90. [Medline].
Masetti P, Marianeschi SM, Cipriani A, Iorio FS, Marcelletti CF. Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt. Ann Thorac Surg. 1999 Jan. 67(1):235-6. [Medline].
Zellers TM, Brown K. Protein-losing enteropathy after the modified Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol. 1996 Mar-Apr. 17(2):115-7. [Medline].
Therrien J, Webb GD, Gatzoulis MA. Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?. Heart. 1999 Aug. 82(2):241-3. [Medline]. [Full Text].
Guariso G, Cerutti A, Moreolo GS, Milanesi O. Protein-losing enteropathy after Fontan operation: treatment with elementary diet in one case. Pediatr Cardiol. 2000 May-Jun. 21 (3):292. [Medline].
Kim SJ, Park IS, Song JY, Lee JY, Shim WS. Reversal of protein-losing enteropathy with calcium replacement in a patient after Fontan operation. Ann Thorac Surg. 2004 Apr. 77(4):1456-7. [Medline].
Donnelly JP, Rosenthal A, Castle VP, Holmes RD. Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr. 1997 Mar. 130(3):474-8. [Medline].
Kelly AM, Feldt RH, Driscoll DJ, Danielson GK. Use of heparin in the treatment of protein-losing enteropathy after Fontan operation for complex congenital heart disease. Mayo Clin Proc. 1998 Aug. 73(8):777-9. [Medline].
Facchini M, Guldenschuh I, Turina J, Jenni R, Schalcher C, Attenhofer Jost CH. Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia. J Cardiovasc Surg (Torino). 2000 Aug. 41(4):567-70. [Medline].
Ringel RE, Peddy SB. Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol. 2003 Apr 15. 91(8):1031-2, A9. [Medline].
Uzun O, Wong JK, Bhole V, Stumper O. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg. 2006 Dec. 82(6):e39-40. [Medline].
Connor FL, Angelides S, Gibson M, et al. Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy. Pediatrics. 2003 Sep. 112(3 Pt 1):e242-7. [Medline].
Mertens L, Dumoulin M, Gewillig M. Effect of percutaneous fenestration of the atrial septum on protein-losing enteropathy after the Fontan operation. Br Heart J. 1994 Dec. 72(6):591-2. [Medline]. [Full Text].
Lemes V, Murphy AM, Osterman FA, Laschinger JC, Kan JS. Fenestration of extracardiac Fontan and reversal of protein-losing enteropathy: case report. Pediatr Cardiol. 1998 Jul-Aug. 19(4):355-7. [Medline].
Fraisse A, Bonnet JL. Protein-losing enteropathy: radiofrequency fenestration of the atrial septum after failure of transseptal needle puncture. Pediatr Cardiol. 2004 Jan-Feb. 25(1):84-6. [Medline].
Kreutzer J, Keane JF, Lock JE, et al. Conversion of modified Fontan procedure to lateral tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 1997. 1169-117.
Brancaccio G, Carotti A, D’Argenio P, Michielon G, Parisi F. Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation. J Heart Lung Transplant. 2003 Apr. 22(4):484-6. [Medline].
Estner HL, Kolb C, Schmitt C, et al. Long-term transvenous AV-sequential pacing in a failing atriopulmonary Fontan patient. Int J Cardiol. 2008 Jul 4. 127(2):e93-5. [Medline].
Lopez JA. Transvenous right atrial and left ventricular pacing after the Fontan operation: long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization. Texas Heart Institut. 2007. 34(1):96-101.
Gamba A, Merlo M, Fiocchi R, et al. Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg. 2004 Feb. 127(2):555-62. [Medline].
Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004 Nov 16. 44(10):2065-72. [Medline].
Petko M, Myung RJ, Wernovsky G, et al. Surgical reinterventions following the Fontan procedure. Eur J Cardiothorac Surg. 2003 Aug. 24(2):255-9. [Medline].
Syamasundar Rao Patnana, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children’s Memorial Hermann Hospital
Syamasundar Rao Patnana, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.
Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children’s Hospital
Howard S Weber, MD, FSCAI is a member of the following medical societies: Society for Cardiovascular Angiography and Interventions
Disclosure: Received income in an amount equal to or greater than $250 from: Abbott Medical .
Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children’s Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions
Disclosure: Nothing to disclose.
Pediatric Tricuspid Atresia
Research & References of Pediatric Tricuspid Atresia|A&C Accounting And Tax Services