左心发育不良综合征的临床及超声心动图特点

The Clinical and Echocardiographic Features of Hypoplastic Left Heart Syndrome

目的 :分析和探讨左心发育不良综合征 (HLHS)的临床及超声心动图特点及其对临床早期干预的意义。方法 :对1989年 4月～ 2 0 0 0年 8月间我院收治 10例HLHS病例进行回顾性分析。年龄为生后 2h～ 36d ,男性 4例 ,女性 6例。临床表现青紫 5例 ,呼吸窘迫 5例 ,心力衰竭 8例 ,代谢性酸中毒 8例。超声心动图检查示Ⅰ型 3例 ,Ⅱ型 1例 ,Ⅲ型 1例 ,Ⅳ型 5例。平均左室舒张末期内径 10 .1± 2 .1mm ,平均主动脉根部内径 6 .1± 1.7mm。结果 :死亡 6例 ,放弃治疗及病情好转出院各 2例。结论 :HLHS的临床及心超特点明显 ,超声心动图有很高诊断价值 。

Objective: To explore the clinical and echocardiaographic features of hypoplastic left heart syndrome (HLHS), and its effects on the early surgical intervention. Methods: Totally 10 cases of HLHS (admitted during 1989.4～2000.8) were studied retrospectively. (Age from 2 hours after birth～36 days, 4 males and 6 females). Cyanosis was found in 5 cases while respiratory distress in 5, congestive heart failure in 8, metabolic acidosis in 8. Echocardiogram study had been applied in all cases with M-mode, 2-D and Doppler. In the ten cases morphologic study demonstrated HLHS type Ⅰ (3), typeⅡ (1), type Ⅲ (1) and type Ⅳ (5). The mean left ventricular diastolic diameter was 10.1±2.1mm and the mean aortic root diameter was 6.1±1.7mm. Results: 6 cases died shortly after the diagnosis. 4 cases ( with type Ⅰand type Ⅳ ) were stabilized and discharged late on. Conclusion: The clinical and echocardiographic manifestations are characteristic. Early recognition and diagnosis is vital to the outcome of further sigical intervention.