Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Intervention with Balloon Valvuloplasty followed by Patent Ductus Arteriosus Stent in a Patient with Pulmonary Atresia with Intact Ventricular Septum

풍선판막성형술과 동맥관 스텐트를 이용하여 치료한 심실중격결손을 동반하지 않은 폐동맥 폐쇄 1례

  • Lim, Han Hyuk (Department of Pediatrics College of Medicine, Chungnam National University) ;
  • Kim, Young Deuk (Department of Pediatrics College of Medicine, Chungnam National University) ;
  • Lee, Jae Hwan (Department of Pediatrics College of Medicine, Chungnam National University) ;
  • Chang, Mea Young (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Kil, Hong Ryang (Department of Pediatrics College of Medicine, Chungnam National University)
  • 임한혁 (충남대학교 의과대학 소아과학교실) ;
  • 김영득 (충남대학교 의과대학 소아과학교실) ;
  • 장미영 (충남대학교 의과대학 소아과학교실) ;
  • 이재환 (충남대학교 의과대학 내과학교실) ;
  • 길홍량 (충남대학교 의과대학 소아과학교실)
  • Received : 2005.06.27
  • Accepted : 2005.08.09
  • Published : 2005.11.15
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Abstract

Pulmonary atresia with intact ventricular septum (PAIVS) is rare, less than 1% of congenital heart disease. It needs a therapeutic approach according to its individual morphologic feature. Surgical treatment of valvotomy and modified Blalock-Taussig shunt or non-surgical interventional catheter balloon valvuloplasty can be used for mild to moderate hypoplasia of right ventricle. Fontan operation can be considered for less optimum morphological substrate of two ventricular repair. A 3-day-old male neonate was admitted with cyanosis and cardiac murmur. On echocardiogram, he had membranous pulmonary atresia with intact ventricular septum, normal sized tripartite right ventricle, large atrial septal defect with right-to-left shunt, small sized patent ductus arteriosus, and moderate tricuspid regurgitation. He was treated with intravenous continuous infusion of prostaglandin $E_1$ ($PGE_1$) at once. On the third day of hospitalization, Balloon valvuloplasty was performed. After insertion of patent ductus arteriosus stent on the tenth day, $PGE_1$ infusion was discontinued. On the fifteenth day, he was discharged. Now, he is 9 months old and has nearly normal cardiac structure and function with 97% of percutaneous oxygen saturation.

심실중격결손을 동반하지 않은 폐동맥 폐쇄(pulmonary atresia with intact ventricular septum)는 전체 선천적 심장병의 1%를 차지하는 드문 질환으로 형태학적 특성에 따라 다양한 치료적 접근이 필요하다. 이에 저자들은 정상 크기발달의 우심실을 가진 심실중격결손을 동반하지 않은 폐동맥 폐쇄가 있는 신생아에서 풍선판막성형술 후 지속적인 저산소증을 보여 동맥관 스텐트를 이용하여 거의 정상적인 심장구조와 기능으로 호전된 1례를 경험하였기에 보고하는 바이다.

Keywords

References

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