Guzzo D, Castellano C, Muñoz-Castellanos L, de Rubens J, Calderón J, Attié F
Departamento de Cardiología Pediátrica, Instituto Nacional de Cardiología Ignacio Chávez, México, D.F.
Arch Inst Cardiol Mex. 1989 Mar-Apr;59(2):133-8.
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.
肺动脉闭锁且室间隔完整的患者，无论接受或不接受传统外科治疗，预后都很差。对于右心室轻度发育不全且存在微小窦状交通、三尖瓣无严重功能障碍的病例，手术治疗能取得最佳效果。我们呈现了5例肺动脉闭锁且室间隔完整并伴有三尖瓣功能障碍的病例。基于影像学、心电图和血流动力学检查结果，这组患者无法与其他无三尖瓣发育异常的患者区分开来。超声心动图和心血管造影检查对于鉴别诊断是必不可少的。对于这些畸形的外科治疗，在可能的情况下，应采用体肺动脉吻合术联合肺动脉瓣切开术，并重建右心室流出道。然而，在某些情况下则需要进行三尖瓣置换术。
