Giamberti A, Marino B, di Carlo D, Iorio F S, Formigari R, de Zorzi A, Marcelletti C
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Hospital, Rome, Italy.
Ann Thorac Surg. 1996 Jul;62(1):151-4. doi: 10.1016/0003-4975(96)00262-7.
An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure.
From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter.
There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy.
Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.
部分房室通道患者中的一个重要亚组因难治性充血性心力衰竭需要在出生后第一年接受手术。
1982年6月至1995年4月，128例部分房室通道患者中，35例（27%）在1岁以内接受了手术治疗。22例患者存在相关心脏异常。仅7例（20%）患有唐氏综合征。5例左心室发育不全患者接受了主动脉缩窄切除术（3例）或诺伍德手术（2例）。其他30例患者中，24例行解剖修复，6例行主动脉缩窄切除术。对接受解剖修复患者的手术结果与超声心动图测量的二尖瓣直径进行回顾性关联分析。
解剖修复后有7例死亡（29%），主动脉缩窄切除术后2例（22%），诺伍德手术后2例（100%）。二尖瓣直径小于2.5×10⁻²m/m²的婴儿在修复时死亡。平均随访73.5个月，有5例二次二尖瓣成形术和3例主动脉缩窄切除术后修复。
在部分房室通道患者中，有一个重要亚组在出生后第一年出现心力衰竭临床体征。左侧梗阻性病变和复杂的二尖瓣异常似乎在这些患者的临床进展和预后中起重要作用。超声心动图测量的二尖瓣直径可能有助于确定正确的手术指征。
