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Medico-Surgical Cooperative Treatment of Pulmonary Atresia with Intact Ventricular Septum

Journal of the Korean Pediatric Society 2003;46(3):250-258.
Published online March 15, 2003.
Medico-Surgical Cooperative Treatment of Pulmonary Atresia with Intact Ventricular Septum
Kyeong Sik Kim1, Byeong Chul Kweon1, Jong Kyun Lee1, Jae Young Choi1, Jun Hee Sul1, Sung Kyu Lee1, Young Whan Park2, Bum Koo Cho2
1Division of Pediatric Cardiology, Yonsei Cardiovascular Center, Cardiovascular Research Institute, Yonsei University College of Medicine, Seoul, Korea
2Division of Pediatric Cardiovascular Surgery, Yonsei Cardiovascular Center, Cardiovascular Research Institute, Yonsei University College of Medicine, Seoul, Korea
심실중격 결손이 없는 폐동맥 폐쇄의 내과-외과적 협동치료
김경식1, 권병철1, 이종균1, 최재영1, 설준희1, 이순규1, 박영환2, 조범구2
1연세대학교 의과대학 심장혈관병원 심혈관연구소 소아심장과
2연세대학교 의과대학 심장혈관병원 심혈관연구소 심장혈관외과
Correspondence: 
Jae Young Choi, Email: cjy0122@yumc.yonsei.ac.kr
Abstract
Purpose
: The actual clinical examples of co-appliance of catheter intervention with surgical procedures in the treatment of pulmonary atresia with an intact ventricular septum(PA/IVS) which we have experienced in our institution are here shown, and the anatomical and hemodynamical profiles between each method is compared.
Methods
: Medical records of 33 patients with PA/IVS who underwent various treatment from January, 1995 to December, 2000 were reviewed for a retrograde study.
Results
: In three out of 10 patients who underwent percutaneous balloon pulmonary valvotomy (PPV), residual pulmonary stenosis were observed in their out patient department(OPD) follow-ups, eventually necessitatig balloon pulmonary valvuloplasty(BPV). One out of three patients exhibited deterioration of tricuspid regurgitation after BPV, requiring surgical tricuspid annuloplasty(TAP). Two out of the seven patients who received primarily surgical right ventricle outlet tract(RVOT) repair without any systemic-pulmonary shunt or intervention needed additional intervention employing cardiac catheterization after operation. Two patients received interventional catheterization before surgical RVOT repair. In five out of 11 cases of Fontan type operation, coil embolization of collateral circulation was done before total cavo-pulmonary connection(TCPC), and in three cases, interventional catheterization was needed after TCPC.
Conclusion
: Both medical and surgical treatment modalities are widely used in management of PA/IVS patients, and recent results prove that medico-surgical cooperative treatment is essential.
Key Words: Percutaneous pulmonary balloon valvotomy(PPV), Surgical RVOT repair, Total cavo- pulmonary connection(TCPC), Right ventricle dependent coronary circulation(RVDCC), Balloon pulmonary valvuloplasty(BPV)


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