法洛氏四合羣症合併左側肺動脈缺失或發育不全

Tetralogy of Fallot with Absence or Hypoplasia of Left Pulmonary Artery

蘇文鉁(Wen-Jen Su) ； 廖佩芹(Pui-Kan Liao) ； 張昭雄(Chau-Hsiung Chang)

《Acta Paediatrica Sinica》 27卷4期 (1986/08) Pp. 328-332

摘要

本文報告自民國66年至73年間，在本院小兒科經心臟導管檢查診斷為法洛氏四合羣症之病人中，合併左側肺動脈缺乏或發育不全者各有2例及6例，年齡分佈由2歲至10歲。6例接受開心矯正手衍：其中4例在右心室出口除了用補片修浦外並在其內側放一 monocusp, 2例病人則只作漏斗部切除而沒有用補片。另2例病人僅接受分流手術。無死亡病例，手術後之運動能耐均有明顯增加，不須藥物幫忙。追蹤檢查期限為8~82個月，平均29個月。1例病人在手術後有較長期的右側肋膜積水，2例分別有2至3星期之房室傳導完全阻塞後再回復到實性心律。

並列摘要

From 1977 through 1984, among the patients diagnosed as tetralogy of Fallot in the Pediatric Department of this hospital, there were two cases associated with absence, six associated with hypoplasia, of the left pulmonary artery. The age at operation ranged from 2 to 10 years. Six patients had received reconstruction of the right ventricular outflow tract (RVOT), in addition to infundibular resection; four cases had patch reconstruction with monocusp, and one had pulmonary valvulotomy only. The remaining two patients had undergone shunt procedure and are waiting for total correction. Postoperatively, one patient had prolonged right pleural effusion and another two had the complication of complete A-V block for two to three weeks before returning to sinus rhythm. There was no surgical death. The post-operative follow-up interval was 8-82 months (mean=29). Three cases had cardiac recatheterization which revealed that the two cases with RVOT reconstruction with monocusp had good hemodynamic results; the one who had pulmonary valvulotomy without outflow patch had severe pulmonary regurgitation causing diastolic, volume overloading and systolic hypertension.

並列關鍵字

tetralogy of Fallot ； absent left pulmonary artery ； hypoplastic left pulmonary artery