Amaral F T, Moreira-Neto F F, Sgarbieri R N, Carvalho S R, Haddad J L
Hospital do Coração de Ribeirào Preto/Fundação Waldemar B. Pessoa, SP.
Arq Bras Cardiol. 1996 May;66(5):277-9.
Two cases of congenital trabecular hypoplasia of the right ventricle are reported. In the first, the neonatal diagnosis was missed and the child did well until the 13th month of life when a modified Blalock-Taussig shunt was done because of increasing cyanosis. Outcome was good until the 4th year of life when symptomatic atrioventricular block was detected in an emergency situation. A bidirectional Glenn anastomosis and pacemaker implantation were successfully carried out after clinical establization and the child is doing well up to now. The second case presents the disease with its worst features: severe cyanosis and acidosis in the first day of life. A modified Blalock-Taussig shunt was performed and death occurred soon after the operation.
报告了两例右心室先天性小梁发育不全的病例。第一例中，新生儿期漏诊，患儿情况良好，直至13月龄时因青紫加重行改良布莱洛克-陶西格分流术。直至4岁时在急诊情况下发现有症状性房室传导阻滞前，预后良好。经临床评估后成功进行了双向格林吻合术和起搏器植入，患儿至今情况良好。第二例呈现了该病最严重的特征：出生第一天即出现严重青紫和酸中毒。行改良布莱洛克-陶西格分流术，术后不久死亡。
