Congenital heart disease · 2010
Discontinuous pulmonary arteries do not preclude good Fontan outcomes.
СтатьяНазвание и аннотация: Перевод в очереди
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Randall Alyssa, Carberry Kathleen, Fraser Charles D
Аннотация
OBJECTIVE: Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation.
DESIGN: The study is a retrospective case series.
PATIENTS: From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%.
OUTCOME MEASURES: The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up.
RESULTS: The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1-2.1). Median follow-up after Fontan = 4.4 years (range: 1.2-9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428-0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I.
CONCLUSIONS: This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.
DESIGN: The study is a retrospective case series.
PATIENTS: From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%.
OUTCOME MEASURES: The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up.
RESULTS: The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1-2.1). Median follow-up after Fontan = 4.4 years (range: 1.2-9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428-0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I.
CONCLUSIONS: This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.