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.

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.

Matching procedure to morphology improves outcomes in neonates with tricuspid atresia.

OBJECTIVE: This study was undertaken to characterize morphologic substrate of tricuspid atresia with ventriculoarterial concordance and discriminate management strategies that lead to successful definitive repair. METHODS: From 1999 to 2004, a total of 150 babies with type I tricuspid atresia were enrolled from first diagnosis at 26 institutions. Antegrade pulmonary blood flow was absent in 19%, restricted in 54%, and unrestricted in 28%. Competing-risk methodology determined the time-related prevalence and risk factors for death versus cavopulmonary anastomosis and subsequent death versus Fontan completion. RESULTS: Overall 5-year survival was 86%. Initial palliation included systemic-pulmonary arterial shunt in 64%, pulmonary artery banding in 11%, and cavopulmonary anastomosis in 24%. Median age at cavopulmonary anastomosis was 6 months, with 83% undergoing bidirectional Glenn shunt and 17% undergoing hemi-Fontan procedure. By the age of 2 years, 89% had cavopulmonary anastomosis, 6% were dead, and 4% remained alive without cavopulmonary anastomosis. Risk factors for death without cavopulmonary anastomosis included presence of mitral regurgitation (P = .03) and palliation with systemic-pulmonary arterial shunts not originating from the innominate artery (P = .04). Factors associated with decreased transition rate to cavopulmonary connection included patient variables (younger admission age to a participating institution, noncardiac anomalies) and procedural variables (larger systemic-pulmonary arterial shunt diameter, previous palliation). Of patients undergoing cavopulmonary anastomosis, 75% had undergone a Fontan operation within 3 years. CONCLUSION: Smaller shunt size and decreased pulmonary blood flow decrease mortality after initial palliation and increase the rate of successful transition to cavopulmonary anastomosis. Outcomes can be improved by placing smaller shunts from the innominate artery, especially in patients with any mitral regurgitation.