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.

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.

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.

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.

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.

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.

Diagnóstico prenatal de atresia tricuspidea / Prenatal diagnosis of tricuspid atresia

La atresia tricuspidea es una cardiopatía rara caracterizada por oclusión de la válvula tricúspide y asociada a hipoplasia de ventrículo derecho y estenosis pulmonar. Clásicamente se presenta con un defecto en septun ventricular. El diagnóstico puede establecerse con ecocardiografía fetal y usualmente se sospecha ante una imagen de cuatro cámaras anormal.

Successful staged Fontan completion for a tricuspid atresia patient with left ventricular non-compaction.

We report a case of Fontan completion for a tricuspid atresia (TA) patient with left ventricular non-compaction (LVNC). The patient was diagnosed with TA (Ia) with LVNC by fetal echocardiography. Because the unfavourable prognosis of LVNC was anticipated, Imidapril as well as Carvedilol were administered to improve cardiac function, from the early stages of infancy. Staged Fontan completion with fenestration was successfully achieved with improvement of LV function.

Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.

OBJECTIVES: To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia. METHODS: Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes. RESULTS: Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA. CONCLUSIONS: Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.

Terapia de ressincronização cardíaca em portador de coração univentricular para correção de dissincroniomiopatia causada por mudança do sítio de estimulação / Cardiac resynchronization therapy in severe systolic dysfunction in a univentricular heart patient to correct dyssynchrony cardiomyopathy caused by change of stimulation site

Paciente com 6 anos de idade, do sexo masculino, portador de coração univentricular decorrente deatresia tricúspide com hipoplasia de ventrículo direito tipo IIC e submetido a implante de marcapasso por bloqueio atrioventricular total no pós-operatório de cirurgia de Fontan, em 2012. Houve necessidade de troca do sistema de estimulação em decorrência de infecção de loja logo após a troca do marcapasso, em 2014. Seis meses após amudança do sítio de estimulação, começou a apresentar quadro de insuficiência cardíaca refratária e disfunção sistólica grave, e o transplante cardíaco foi indicado. O paciente foi submetido a terapia de ressincronização cardíaca orientada por eletrocardiografia triaxial como ponte para transplante e apresentou melhora clínico-estrutural após10 semanas, recebendo alta hospitalar...

We report the case of a 6-year-old male patient with univentricular heart due to tricuspid atresia with right ventricular hypoplasia (IIc), who had a pacemaker implanted for complete heart block after a Fontan surgery in 2012. The stimulation system had to be exchanged due to a pocket infection soon after the pacemaker was exchanged in 2014. Six months after the stimulation site was exchanged he presented refractory heart failure and severe systolic dysfunction and a heart transplantation was indicated. He was submitted to cardiac resynchronization therapy guided by triaxial electrocardiography as a bridge for transplantation. The patient presented clinical and structural improvement after 10 weeks and was discharged...

Absent pulmonary valve, tricuspid atresia, and congenital heart block.

We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed. Cardiac catheterization revealed high cavopulmonary pressures and ventricular dysfunction precluding Fontan completion. Heart transplantation was performed at 3.75 years of age. The patient is alive and well 26 months posttransplantation.

[Two uncomplicated pregnancies with a univentricular heart]. / To vellykkede graviditeter med et univentrikulært hjerte.

Fontan type operations provide long-term palliation for patients with functionally univentricular hearts. The haemodynamics of these patients were previously considered inhibitory for contemplating pregnancy. We describe the first Danish woman, who gave birth to two healthy children after relatively uncomplicated pregnancies.

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.

Cardiac emergencies.

The diagnosis and management of pediatric cardiac emergencies can be challenging and complicated. Early presentations are usually the result of ductal-dependent lesions and appear with cyanosis and shock. Later presentations are the result of volume overload or pump failure and present with signs of congestive heart failure. Acquired diseases also present as congestive heart failure or arrhythmias.