Tricuspid atresia: Where are we now?

Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Double Orifice Mitral Valve in Tricuspid Atresia: A Rare Association.

We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.

Aortic root replacement in a patient with unoperated tricuspid atresia.

A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Aortic root replacement was performed and then his heart failure was controlled.

Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas.

The association of tricuspid atresia with aortopulmonary window is exceptionally rare. We report a patient with tricuspid atresia, normally related great arteries, non-restrictive ventricular septal defect, and no pulmonary stenosis (type IC) with an aortopulmonary window who underwent successful initial surgical palliation. The unique anatomical feature of this case, other than the presence of the aortopulmonary window, was the absence of pulmonary stenosis at the level of either the ventricular septal defect or the pulmonary valve. All other reported cases have described some degree of restriction of anterograde pulmonary flow due to pulmonary stenosis or atresia.

Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum.

Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock-Taussig shunt at 11 days of age.

Living with Tricuspid Atresia: Case Report with Review of Literature.

Tricuspid atresia (TA) is a rare congenital heart defect in which the right atrioventricular connection, the tricuspid valve, is absent. As a result, there is no direct communication between the right atrium and right ventricle. Surgical treatment, including the Fontan procedure, is indicated yet palliative, leaving patients with various lifelong complications. A comprehensive literature review revealed a paucity of evidence-based education on the identification, evaluation, management, treatment, and life span implications of TA. We present a case of TA from birth through adulthood, while simultaneously assessing the literature, to report the most current evidence relative to living with TA after surgical palliation. In addition, the embryology, methods of prenatal and postnatal diagnosis, potential complications, management, anticipatory guidance, and educational needs of both parents and patient are discussed.

Percutaneous transluminal pulmonary valvuloplasty in a child with tricuspid atresia, ventricular septal defect, and severe pulmonary valve stenosis: usefulness of the femoral artery approach.

Percutaneous transluminal pulmonary valvuloplasty may be indicated in not only isolated pulmonary valve stenosis, but also complex congenital heart diseases. Because palliative surgery for increasing pulmonary blood flow entails a risk of scar formation and immediate postoperative complications, catheter intervention is preferred, if possible. However, an acute-angled, twisted, or tortuous access route or a small valve orifice occasionally makes it difficult for the catheter to reach or cross the target. We succeeded in performing this intervention for such a complex stenosis effectively and safely in a patient with tricuspid atresia, ventricular septal defect (VSD), and severe pulmonary valve stenosis, thereby evading surgery. In previous reports, the catheter for this cardiac anomaly was accessed via the femoral vein. In the present case, the catheter was advanced through the femoral artery via the aorta, left ventricle, VSD, and right ventricle to the pulmonary valve, using a micro-catheter in a telescopic manner, in combination with a coronary balloon dilatation catheter. This maneuver, which has not been reported previously, made it much easier to perform the procedure as compared to the femoral vein approach, despite the acute turn and the pinhole orifice. Moreover, reported complications of the femoral vein approach, including bradycardia, hypotension, and valve regurgitation, were not observed in this case. We conclude that the femoral artery approach can be a safe and effective alternative in patients for whom a more conventional procedure has been unsuccessful.

Percutaneous closure of tricuspid paravalvular leak.

While transcatheter closure of mitral and aortic paravalvular leaks has been well-described, there are no prior reports of such procedures for prosthetic tricuspid valves. We describe our experience with percutaneous closure of a tricuspid paravalvular leak in a 34-year-old patient with a history of tricuspid atresia. He had previously undergone repair with placement of a right atrium-to-right ventricle conduit and most recently, placement of a bio-prosthetic tricuspid valve. He subsequently developed significant tricuspid paravalvular leak with symptoms of severe right heart failure. His clinical status was improved following successful transcatheter closure of the defect.

Modified bjork procedure for tricuspid atresia with pulmonary hypertension.

Tricuspid atresia (TA) is a rare and life-threatening cyanotic congenital heart anomaly, which is traditionally treated by the Fontan procedure. However, this procedure may be limited by the development of pulmonary hypertension and lead to long-term complications. The Bjork procedure offers an alternative for biventricular repair and right ventricular growth, especially in patients with pulmonary hypertension. We report a case of TA with pulmonary hypertension successfully treated with the modified Bjork procedure using an aortic homograft valved conduit.

Subclavian pseudoaneurysm repair after Blalock-Taussig shunt placement.

A 7-day-old male with tricuspid atresia, pulmonary atresia, and D-transposition of the great vessels underwent surgical placement of a modified Blalock-Taussig (mBT) shunt and was found to have a pseudoaneurysm on the follow-up echocardiogram. Surgical exploration identified a pseudoaneurysm arising from the right subclavian artery and not from the innominate artery at the site of the mBT shunt. It was concluded that this was caused by an inadvertent needlestick during central line placement. The fistula tract between the right subclavian artery and the false aneurysm was oversewn, and the postoperative course was unremarkable.

Atrioventricular septal defect with coexisting tricuspid atresia.

We describe two infants having an atrioventricular septal defect in the setting of a double inlet atrioventricular connection, but with patency of the left-sided valvar orifice and an imperforate right-sided valvar component, and a further case with atrioventricular septal defect and an imperforate Ebstein's malformation, all producing the haemodynamic effect of tricuspid atresia. We make comparisons with the arrangement in trisomy 16 mice, in whom deficient atrioventricular septation is seen at times with the common atrioventricular junction exclusively connected to the left ventricle, a situation similar to that seen in two of our infants. We also review previous reports emphasising the important theoretical implication of the findings despite their rarity.

Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation.

RATIONALE: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks. PATIENT CONCERNS: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone. DIAGNOSES: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis. INTERVENTIONS: Cardiac catheterization, electrophysiological examination, and ablation. OUTCOMES: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation. LESSONS: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways.

Tricuspid atresia with normal axis on ECG palliated with a central shunt; a case report.

BACKGROUND: Tricuspid Atresia is the 3rd commonest cyanotic congenital Heart disease. It is characterized by lack of communication between the right atrium and right ventricle. The treatment often requires a palliative systemic to pulmonary shunt before definite surgery. The use of a central shunt via a median sternotomy is suggested here as an alternative to other traditional shunts via a thoracotomy. METHOD: The management of a 3-month-old boy who presented with dyspnoea, fever, cough and cyanosis is presented here as obtained from Clinical records. RESULT: Following resuscitation, a central shunt (Ascending Aorta to main Pulmonary Artery) was constructed and the patient did well despite a turbulent post-operative period. CONCLUSION: The management of tricuspid atresia likes other cyanotic heart disease is daunting but palliative treatment is possible in our environment and definitive treatment where possible affords a fairly satisfactory prognosis.

[Tricuspid atresia associated with common trunk. A case report]. / Atresia tricuspídea asociada a tronco común. Reporte de un caso.

The tricuspid atresia associated with persistent truncus arteriosus is a rare anomaly. A case is presented of one male patient of three months age in which was detected a cardiac murmur. The diagnosis was made by echocardiography and confirmed by cardiac catheterization. The first step of the palliation was done with disconnection of the trunk of the pulmonary artery combined with a systemic- to- pulmonary shunt of 4 mm. The patient died 24 hours later for persistent metabolic acidosis.

A persistent giant Eustachian valve: functional tricuspid atresia in a newborn.

Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Echocardiographic examination showed a giant Eustachian valve moving towards the tricuspid valve during late systole and leading to complete obstruction of the inflow. Blood flow was redirected through the persistent foramen ovale, producing a right-to-left shunt.

Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience.

BACKGROUND: The outcome of prenatally diagnosed tricuspid atresia (TA) is undefined. We sought to characterize clinical and echocardiographic features of fetal TA and to determine factors associated with mortality. METHODS AND RESULTS: All fetuses with TA (n = 88) seen at 3 tertiary care institutions from 1990 to 2005 were reviewed. There were 58 liveborn infants (median gestational age 38 weeks, range 24-40 weeks), 4 in utero demises, 25 terminations of pregnancy, and 1 mother lost to follow-up. Obstruction was present at the pulmonary valve in 27 (45%), aortic valve in 6 (10%), and aortic arch in 15 (25%). Three neonates received compassionate care, 1 died with multiple extracardiac anomalies, 2 were lost to follow-up, and 52 liveborns were actively managed with Blalock-Taussig shunt (23), Norwood palliation (14), pulmonary artery band (10), bidirectional cavopulmonary connection (3), atrial septostomy (1), and right outflow stent (1). Of those actively managed, there were 7 (14%) of 52 who died. Kaplan-Meier estimates of survival were 91% at 1 month, 87% at 6 months, and 83% at 1 year with no subsequent deaths for 13 years. By multivariate analysis, 2 independent factors were associated with an increase in time-related mortality in the actively managed group: presence of chromosomal anomaly or syndrome (P = .005) and use of extracorporeal membrane oxygenation (P = .002). CONCLUSIONS: This is the largest study describing TA in fetus. Compared with published observations of TA diagnosed postnatally, antenatal diagnosis of TA appears to have similar short-term survival in pregnancies surviving to birth.

Successful surgical correction of total anomalous systemic venous drainage: a report of 2 cases.

Total anomalous systemic venous drainage is a rare malformation and only limited cases have been reported previously. Here we report two cases of total anomalous systemic venous drainage with successful surgical correction.