先天性二尖瓣瓣上狭窄的诊断和外科治疗

DIAGNOSIS AND SURGICAL TREATMENT OF CONGENITAL SUPRAMITRAL VALVE STENOSIS

先天性二尖瓣瓣上狭窄是一种罕见的心脏畸形，常伴有其它心内病变，临床表现为充血性心力衰竭、肺静脉高压。超声心动图检查对诊断有重要意义，一经诊断，均应在体外循环心内直视下纠治。本文报道３例，均伴继发孔房间隔缺损，手术效果满意。

Supramitral valve stenosis is a rare congenital heart malformation and may be isolated or complicated with other intracardiac and extracardiac defects. From 1990 to 1991, 3 cases, aged 2 1/2-6 yrs, of this disease, all with secundum atrial septal defect, were operated in our hospital. The clinical manifestations were co-ngestive heart failure and pulmonary venous hypertension.Chest X-ray and electrocardiogram revealed medium to severe enlargement of the heart. There were left atrial, right atrial, and ventricular hypertrophy with strain. After definitive diagnosis by colour Doppler echocardiography, operations were done under cardiopulmonary bypass. Supramitral valvular fibrous membrane was resected and ASD was repaired. The operative and postoperative courses were uneventful. All patients were well when followed up twelve to eighteen months postoperatively. The cause, diagnosis, differential diagnosis and treatment of this disease were discussed.