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Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions

Korean Journal of Pediatrics 2010;53(2):195-202.
Published online February 15, 2010.
Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions
Cha Gon Lee1, Su In Jeong1, June Huh1, I-Seok Kang1, Heung Jae Lee1, Ji-Hyuk Yang2, Tae Gook Jun2
1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
2Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
심한 폐동맥 고혈압을 동반한 좌우 단락 질환 환자의 수술 후 경과
이차곤1, 정수인1, 허준1, 강이석1, 이흥재1, 양지혁2, 전태국2
1성균관대학교 의과대학 삼성서울병원 소아청소년과
2성균관대학교 의과대학 삼성서울병원 흉부외과
Correspondence: 
I-Seok Kang, Tel: +82.2-3410-3539, Fax: +82.2-3410-0043, Email: kis4298@gmail.com
Abstract
Purpose
: Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity.
Methods
: We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (≥8 Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively.
Results
: Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ≥1.5). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class.
Conclusion
: Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.
Key Words: Pulmonary hypertension, Balloon occlusion, Congenital heart disease, Surgery


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