Congenital pulmonary atresia with tricuspid insufficiency: Morphologic study

In an anatomic study of 21 cases of pulmonary atresia with tricuspid insufficiency (pulmonary atresia with intact ventricular septum, type II), the morphologic features of the tricuspid valve and the right ventricle were found to differ greatly from those seen in pulmonary atresia with tricuspid stenosis (pulmonary atresia with intact ventricular septum, type I). Morphologically, pulmonary atresia with tricuspid insufficiency (type II) has a greater resemblance to Ebstein's disease with pulmonary atresia than to type I pulmonary atresia. The anomaly may be more amenable to surgery than pulmonary atresia with tricuspid stenosis because the right ventricle in the former may be converted into a functional chamber by a valvotomy combined with a shunting procedure and atrial septostomy.     

Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.     

Morphological variations in pulmonary atresia with intact ventricular septum.

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle. Successful pulmonary valvotomy with subsequent adequate right ventricular function would have been precluded by a tiny right ventricular cavity or infundibular atresia in 14 specimens and by severe tricuspid stenosis or regurgitation in an additional 4. Severe right or left ventricular endocardial fibroelastosis may have adversely affected ventricular function in several others. Ten specimens displayed convex bulging of the left ventricular septal surface. If these these anatomical findings are representative for the condition as a whole, they provide a good explanation for its disastrous prognosis.     

Morphological variations in pulmonary atresia with intact ventricular septum.

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle. Successful pulmonary valvotomy with subsequent adequate right ventricular function would have been precluded by a tiny right ventricular cavity or infundibular atresia in 14 specimens and by severe tricuspid stenosis or regurgitation in an additional 4. Severe right or left ventricular endocardial fibroelastosis may have adversely affected ventricular function in several others. Ten specimens displayed convex bulging of the left ventricular septal surface. If these these anatomical findings are representative for the condition as a whole, they provide a good explanation for its disastrous prognosis.     

Transient neonatal tricuspid regurgitation: a Doppler echocardiographic study of three cases.

Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Doppler echocardiography showed clinically significant tricuspid regurgitation, a right to left shunt through the foramen ovale, reduced flow through the pulmonary valve, and in two patients ductal flow into the pulmonary artery. In one patient tricuspid regurgitation was so great that it impeded the opening of the pulmonary valve and produced functional "atresia" of the pulmonary valve. The presence of regurgitant blood flow through the pulmonary valve showed that the "atresia" was functional rather than organic. Doppler echocardiographic study is useful in distinguishing functional neonatal tricuspid regurgitation from structural abnormality of the tricuspid valve.     

Imperforate tricuspid valve with single cardiac outlet from right ventricle.

A cyanosed neonate was diagnosed as having concordant atrioventricular connection and single cardiac outlet and aorta from right ventricle with atresia of the pulmonary valve. She underwent a systemic to pulmonary artery shunt and is now thriving. Additionally, the tricuspid valve was imperforate and a large ventricular septal defect caused an unusual pattern of intracardiac blood flow. The tricuspid valve ring and right ventricular cavity appeared to be of normal size. The good outcome in this patient suggests that these features may be advantageous for corrective operation.     

Transient neonatal tricuspid regurgitation: a Doppler echocardiographic study of three cases

Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Doppler echocardiography showed clinically significant tricuspid regurgitation, a right to left shunt through the foramen ovale, reduced flow through the pulmonary valve, and in two patients ductal flow into the pulmonary artery. In one patient tricuspid regurgitation was so great that it impeded the opening of the pulmonary valve and produced functional "atresia" of the pulmonary valve. The presence of regurgitant blood flow through the pulmonary valve showed that the "atresia" was functional rather than organic. Doppler echocardiographic study is useful in distinguishing functional neonatal tricuspid regurgitation from structural abnormality of the tricuspid valve.     

Surgical treatment of pulmonary atresia with intact ventricular septum.

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

New insight into left ventricular function in tricuspid atresia after total cavopulmonary connection: a three-dimensional echocardiographic study

BACKGROUND: In patients with tricuspid atresia palliated by construction of a total cavopulmonary connection, both pulmonary and systemic circulations depend on the performance of the dominant left ventricle. When estimating the volume of such ventricles using cross-sectional echocardiography, it is necessary to make assumptions concerning the geometry of the ventricular shape. This is avoided by three-dimensional echocardiography, which provides direct volumetric data. Our purpose was to apply this new method to quantify left ventricular volumes and function in patients with tricuspid atresia after construction of a total cavopulmonary connection. METHODS: We studied ten patients (median age: 8 years) with tricuspid atresia who had undergone a total cavopulmonary connection, comparing them with 10 normal children matched for age, sex and size. The three-dimensional echocardiography was performed with electrocardiographic and respiratory gating. A new transthoracic integrated probe designed for small children was used with a rotational scanning increment of 3 degrees. The 60 slices obtained from the ventricular cavity were stored and formatted in a commercial system (TomTec). End-diastolic and end-systolic volumes, stroke volume and ejection fraction were calculated after manual tracing of the endocardial surfaces. The volumes were indexed to the body surface area. RESULTS: As seen in the reconstructions, the dominant left ventricle in tricuspid atresia had a spherical shape, whereas the normal left ventricle is oblong. The left ventricular volumes and function in tricuspid atresia were 54+/-4 ml/m2 (end-diastolic volume), 28+/-3 ml/m2 (end-systolic volume), 26+/-7 ml/m2 (stroke volume) and 48+/-6% (ejection fraction). These volumes were not different from those obtained in the controls (p = NS). The left ventricular stroke volume and ejection fraction in 10 patients with tricuspid atresia were lower than those calculated for the controls (p < 0.05). CONCLUSIONS: Three-dimensional echocardiography provides a quantitative insight into the pathophysiologic function of the dominant left ventricle in tricuspid atresia after construction of a total cavopulmonary connection.     

Imperforate tricuspid valve with single cardiac outlet from right ventricle

A cyanosed neonate was diagnosed as having concordant atrioventricular connection and single cardiac outlet and aorta from right ventricle with atresia of the pulmonary valve. She underwent a systemic to pulmonary artery shunt and is now thriving. Additionally, the tricuspid valve was imperforate and a large ventricular septal defect caused an unusual pattern of intracardiac blood flow. The tricuspid valve ring and right ventricular cavity appeared to be of normal size. The good outcome in this patient suggests that these features may be advantageous for corrective operation.     

Successful transcatheter coil embolization of coronary artery to left ventricular fistula associated with absent pulmonary valve with tricuspid atresia in early infancy.

Transcatheter coil embolization for coronary artery to left ventricular fistula was successfully performed in a neonate. At 30 weeks' gestation, fetal echocardiography showed a hypoplastic right ventricle with intact ventricular septum, absent pulmonary valve, tricuspid atresia, and marked distension of the right coronary artery. After birth, the neonate had congestive heart failure and the electrocardiogram showed myocardial ischemic changes in the left ventricular area. Aortography showed a dilated right coronary artery arising from the ascending aorta and draining into the left ventricle. Transcatheter coil embolization was carried out on the 9th day after birth. Since the procedure, no myocardial ischemic changes have been detected. Transcatheter coil embolization is a useful therapy for coronary artery fistula associated with myocardial ischemia.     

Right ventricular growth in a case of pulmonic stenosis with intact ventricular septum and hypoplastic right ventricle.

Adequate growth of the hypoplastic right ventricle in a patient with severe pulmonary stenosis with an intact ventricular septum was documented after pulmonary valvotomy in infancy. It is postulated that the growth of the ventricular chamber is largely the result of pulmonary regurgitation resulting from successful pulmonary valvotomy. Based on this and the observations of others on the growth of the hypoplastic right ventricle in pulmonary atresia (with intact septum) cases, an organized approach to eventual total surgical correction is recommended.     

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.     

Catheter‐based Therapy for Small Preterm Infants with Pulmonary Atresia/Intact Ventricular Septum

Catheter‐based therapy is the preferred treatment for term infants with pulmonary atresia and intact ventricular septum without right ventricular‐dependent coronaries, membranous atresia with patent infundibulum, and acceptable‐sized tricuspid valve. However, in smaller preterm infants, it is more difficult to determine the adequacy of the tricuspid valve and right ventricle for two‐ventricle repair and there are increased procedural risks. An excellent result in a small preterm infant with pulmonary atresia and intact ventricular septum is described using the following algorithm: determination of the tricuspid/mitral annular ratio, right‐sided catheter‐based intervention, and a combination of transthoracic echocardiography and angiography.     

Cardiomyopathy presenting prenatally with functional tricuspid and pulmonary atresia

Cardiomyopathy is a rare diagnosis in the fetus that usually presents as a dilated, poorly functioning ventricle. We present the case of a fetus that developed functional tricuspid and pulmonary atresia due to progressive right ventricular cardiomyopathy. The baby was supported with prostaglandin and inotropic infusions after delivery, eventually weaning off without need for surgical intervention. This case illustrates the prenatal findings that evolved and the successful postnatal management.     

Angiocardiographic evaluation of right ventricular size and morphology in tricuspid atresia

The potential for right ventricular growth and physiological repair in tricuspid atresia may influence the type of Fontan procedure. To evaluate preoperative right ventricular assessment, we compared the right ventricular size and morphology determined by selective right ventricular catheterization with axial left ventricular angiography. In seven consecutive patients with tricuspid atresia and ventriculo-arterial concordance, the right ventricular volume was 12.8 +/- 9.4 cc, with a predicted normal volume (based on body surface area) of 31 +/- 16 cc, 43% (range 24-78%) of normal. Right ventricular injection outlined a right ventricular area twice that visualized from an axial left ventricular injection (33.2 vs. 16.5 cm). All patients had a well developed but small trabecular portion of the right ventricle, often unopacified with left ventricular injection. Subinfundibular narrowing adjacent to the ventricular septal defect was invariably present, creating, in effect, a two-chambered right ventricle. Selective right ventriculography demonstrates the unique morphology of the right ventricle in patients with tricuspid atresia not visualized by axial left ventriculography.     

Noninvasive estimation of pulmonary artery diastolic pressure in patients with tricuspid regurgitation by Doppler echocardiography.

OBJECTIVES: The purpose of this study was to determine whether Doppler echocardiographic assessment of right ventricular pressure at the time of pulmonary valve opening could predict pulmonary artery diastolic pressure. BACKGROUND: Doppler echocardiography has been used to estimate right ventricular systolic pressure noninvasively. Because right ventricular and pulmonary artery diastolic pressure are equal at the time of pulmonary valve opening, Doppler echocardiographic estimation of right ventricular pressure at this point might provide an estimate of pulmonary artery diastolic pressure. METHODS: We studied 31 patients who underwent right heart catheterization and had tricuspid regurgitation. Pulmonary flow velocity was recorded by pulsed wave Doppler echocardiography, and tricuspid regurgitant velocity was recorded by continuous wave Doppler echocardiography. The time of pulmonary valve opening was determined as the onset of systolic flow in the pulmonary artery. Tricuspid velocity at the time of pulmonary valve opening was measured by superimposing the interval between the onset of the QRS complex on the ECG and the onset of pulmonary flow on the tricuspid regurgitant envelope. The tricuspid gradient at this instant was calculated from the measured tricuspid velocity using the Bernoulli equation. This gradient was compared to the pulmonary artery diastolic pressure obtained by right heart catheterization. MEASUREMENTS AND RESULTS: The pressure gradient between the right atrium and right ventricle obtained at the time of pulmonary valve opening ranged from 9 to 31 mm Hg (mean, 19+/-5) and correlated closely with invasively measured pulmonary artery diastolic pressure (range, 9 to 36 mm Hg; mean, 21+/-7 mm Hg; r = 0.92; SEE, 1.9 mm Hg). CONCLUSION: Doppler echocardiographic measurement of right ventricular pressure at the time of pulmonary valve opening is a reliable noninvasive method for estimating pulmonary diastolic pressure.     

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Absence of the tricuspid valve. A case report]

An anatomical embryological and histological study of a malformed heart is presented in which the right concordant atrioventricular connexion is unguarded by the tricuspid valve, due to absence of the three leaflets, chordae tendinae and papillary muscles; only the fibrous ring is present in the atrioventricular junction. This very rare malformation is associated with atresia of the valve connected with the right ventricle generally being the pulmonary valve; the ventricular septum is intact. This congenital lesion always present fibrosis in the free wall of the right ventricle. We believe a primary pathogenetic step occurs in the wall of the right ventricle which prevents the morphogenesis of the tricuspid valve from this ventricle. This malformation must be distinguish from Ebstein's disease and Uhl's disease, although they are pathogenetically related.