Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Tricuspid Atresia Associated with Truncus Arteriosus versus Aortopulmonary Window: Combining Fetal and Postnatal Echocardiography to Make the Diagnosis

The coincident occurrence of tricuspid atresia and aortopulmonary window (APW) is exceedingly rare, with one previous case reported in the literature. We present a patient with tricuspid atresia, pulmonary atresia, and APW. Postnatal echocardiograms demonstrated no visible pulmonary valve, and additional defects including a bicuspid aortic valve, right aortic arch and anomalous coronary arteries raised suspicion for tricuspid atresia with persistent truncus arteriosus. However, fetal echocardiography and direct visualization of the anatomy confirmed the alternate diagnosis. The patient underwent successful palliation consisting of APW repair, atrial septectomy and a 3.5 mm modified Blalock–Taussig shunt, followed by a bidirectional cavopulmonary connection.     

Simulated tricuspid valve echoes in tricuspid atresia

A case of tricuspid atresia with unusual echocardiographic findings is presented. The echocardiogram was successful in defining great artery interrelationships, ventricular looping, and cavity sizes. An echo pattern resembling the motion of a small tricuspid valve was observed; this was proven to be spurious at catheterization and necropsy. Caution should be exercised in diagnosing the presence of a tricuspid valve on the basis of atypical echoes from the tricuspid valve area.     

Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients

The more common associated cardiac anomalies in any cohort of patients with discordant atrioventricular and ventriculo-arterial connexions (congenitally corrected transposition) include ventricular septal defect, left ventricular (subpulmonary) outlet obstruction, and displacement and/or dysplasia of the morphologically tricuspid valve. There is scant mention in the literature of severe obstruction to systemic blood flow at aortic valve level or beyond in these hearts. The present study reviews those pertinent morphological-clinical and follow-up data on 14 patients with congenitally corrected transposition and aortic valve atresia (one patient); aortic arch interruption or aortic arch atresia (two patients) and coarctation of the aorta (11 patients). All but one patient (the patient with aortic valve atresia) had a perimembranous ventricular septal defect. Mild outflow tract obstruction of the morphologically left ventricle was identified in one patient. Nine patients, however, demonstrated a significant structural and functional disturbance (Ebstein-like with dysplasia) of the systemic morphologically tricuspid valve. No patient in this series was identified with isolated aortic coarctation.     

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.     

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.     

Functional aortic valve atresia in transposition of the great arteries

The criterion for the diagnosis of functional atresia of a patient semilunar valve is met when the pressure in a ventricle remains lower than that in the related great artery throughout systole so that no forward flow can occur. Functional pulmonary valve atresia has been well recognized in infants with normally related great arteries and massive tricuspid valve incompetence. The cardiac physiology and anatomy of an infant with transposed great arteries and functional aortic valve atresia is reported for the first time. The peak systolic pressure in the right ventricle was 30 mm Hg and in the aorta 64 mm Hg. The causes for right ventricular incompetence were abnormalities of the tricuspid valve and hypoplasia of the ventricular free wall. Three other cases with similar ventricular anatomy and physiology but with anatomic atresia of the aortic valve are reviewed. The possibility that under these physiologic circumstances during fetal life functional atresia develops first, and that anatomic fusion of idle semilunar cusps develops as a secondary phenomenon, is discussed.     

Congenital tricuspid stenosis. Case treated by heterograft replacement of the tricuspid valve

This is the first reported case of successful porcine valve replacement in a patient with congenital tricuspid stenosis. The importance of careful diagnostic studies is stressed, particularly with regard to differentiation from tricuspid atresia and identification of patients with tricuspid stenosis who are anatomically suited for corrective surgery.     

Differentiation of functional and structural pulmonary atresia: Role of aortography

Among certain critically ill neonates with severe tricuspid regurgitation, including those with Ebstein's anomaly of the tricuspid valve, tricuspid valve dysplasia, transient tricuspid regurgitation of the newborn, Uhl's anomaly and hypoplasia of the right ventricle, selective right ventriculography may not distinguish between patients with associated functional and those with structural pulmonary atresia. In patients with a normal pulmonary valve and infundibulum, functional obstruction (nonvisualization of the pulmonary artery on right ventricular angiography when the pulmonary valve is anatomically patent and nonobstructive) results from a combination of massive tricuspid insufficiency and increased perinatal pulmonary vascular resistance. This differentiation between functional and structural pulmonary atresia is essential because of the therapeutic implications.In 33 neonates studied at cardiac catheterization from January 1974 through October 1977 standard hemodynamic measurements and right ventriculograms could not distinguish between functional and structural pulmonary atresia. Retrograde aortography, in the presence of a patent ductus arteriosus, provided excellent visualization of the pulmonary root and valve in these cases and, without exception, pulmonary regurgitation across the valve was observed in the patients with functional pulmonary atresia. Usually, the amount of regurgitated contrast material was dense and both the right ventricle and the right atrium were opacified. In addition, among the patients with functional pulmonary atresia, the pulmonary root and valve appeared wider and more patulous than in the patients with structural pulmonary atresia and normal or mildly increased right ventricular pressure was appreciated.     

"Carcinoid-like" tricuspid valvulopathy associated with nephrogenic systemic fibrosis

We present a case of a patient with end-stage renal disease and nephrogenic fibrosing dermopathy (NFD) whose echocardiogram demonstrated rare tricuspid valve abnormality with malcoaptation due to tethering and restricted motion of the leaflets causing severe tricuspid regurgitation. The cardiac abnormalities developed 3 years after her initial diagnosis of NFD was made by skin biopsy. The echocardiographic appearance of the tricuspid valve resembled that seen in patients with carcinoid heart disease; however, an evaluation for carcinoid tumor in our patient was negative. Myocardial biopsy performed at the time of right heart catheterization demonstrated trace gadolinium within the lysosomes of one cardiac fibroblast. This report is the first to describe the association between nephrogenic systemic fibrosis and severe valvular heart disease requiring valve replacement.     

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.     

Echocardiographic diagnosis of pulmonary atresia with intact interventricular septum and associated Ebstein anomaly

Pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. We describe a case of pulmonary atresia and intact ventricular septum associated with Ebstein's malformation of the tricuspid valve first diagnosed with echocardiography and confirmed by angiocardiography and anatomic studies.     

Tricuspid atresia with I-transposition

Five cases of tricuspid atresia with the rare association of l-transposition are reported. In one case, classical ventricular inversion was present (bulboventricular inversion) and the tricuspid atresia involved the left A-V valve.In the remaining four cases, only the outflow portions of the ventricles were inverted (isolated bulbar inversion). The atretic tricuspid valve was right-sided.In addition to documenting rare anatomic states, this report identifies unusual electrocardiographic findings in tricuspid atresia. Of two of four cases with atresia of the right A-V valve, the electrocardiogram showed right axis deviation (+110°) in one and a normal axis (+75°) in another.     

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.     

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.     

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.     

Absent tricuspid valve with aortic atresia in mixed levocardia (atria situs solitus, L-loop). A hitherto undescribed entity.

Clinical, angiographic and pathologic features are described in a 36-hour-old male infant with mixed (discordant) levocardia, aortic valve atresia, absent tricuspid (left atrioventricular) valve and resultant massive cardiomegaly. This is the first reported case of this type of complex in a heart with discordant chambers. A possible embryologic explanation is offered for the concomitant presence of semilunar valve atresia and absence of the tricuspid valve.     

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.     

Aneurysm of the atrial septum in tricuspid atresia: Diagnosis during life and therapy

Massive aneurysmal dilatation of the foramen ovale was diagnosed angiocardiographically in a patient with tricuspid atresia. The angiographic findings are distinct for this condition, and the pathogenesis appears to be related to a restrictive atrial communication in the patient with obligatory right to left shunting at atrial level. The anatomic potential for atrial restriction in the patient with tricuspid or pulmonary atresia, intact ventricular septum and diminutive right ventricle necessitates balloon atrial septostomy at the initial diagnostic cardiac catheterization. In the patient with aneurysmal dilatation of the foramen ovale, satisfactory decompression may be achieved by Blalock-Hanlon atrial septectomy, open atrial septectomy or, possibly, balloon septostomy.     

Transcatheter tricuspid valve replacement With the Edwards SAPIEN valve

We describe a case of percutaneous tricuspid valve implantation in a 20‐year‐old man with previous tricuspid valve replacement in the setting of pulmonary atresia with intact ventricular septum. He developed symptomatic endocarditis‐induced tricuspid regurgitation of the tricuspid bioprosthesis. Tricuspid valvar competence was restored with implantation of a 26‐mm Edwards SAPIEN valve. © 2011 Wiley‐Liss, Inc.