HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Naoki Yoshimura Masahiro Yamaguchi Hidetaka Ohashi Yoshihiro Oshima Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yoshimura, N. Articles by Tei, T. Search for Related Content PubMed PubMed Citation Articles by Yoshimura, N. Articles by Tei, T. Related Collections Congenital - cyanotic

J Thorac Cardiovasc Surg 2003;126:1417-1426
© 2003 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Pulmonary atresia with intact ventricular septum: Strategy based on right ventricular morphology

^a Department of Cardiothoracic, Kobe Children's Hospital, Kobe, Japan Surgery
^b Department of Cardiology, Kobe Children's Hospital, Kobe, Japan

Received for publication November 25, 2002; revisions received February 10, 2003; revisions received April 27, 2003; accepted for publication June 10, 2003.

^* Address for reprints: Naoki Yoshimura, MD, Department of Cardiothoracic Surgery, Kobe Children's Hospital, 1-1-1, Takakura-dai, Suma-ku, Kobe, 654-0081 Japan
y-naoki{at}za2.so-net.ne.jp

OBJECTIVES: Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle–tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure.

METHODS: Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10.

RESULTS: There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years.

CONCLUSIONS: We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.

This article has been cited by other articles:

				S.-C. Huang, K. Ishino, S. Kasahara, K. Yoshizumi, Y. Kotani, and S. Sano The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J. Thorac. Cardiovasc. Surg., November 1, 2009; 138(5): 1160 - 1166. [Abstract] [Full Text] [PDF]

				M Marasini, P F Gorrieri, G Tuo, L Zannini, P Guido, M Pellegrini, S Bondanza, M G Calevo, and G Pongiglione Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum Heart, September 15, 2009; 95(18): 1520 - 1524. [Abstract] [Full Text] [PDF]

				T. Krasemann Radiofrequency perforation: the optimum treatment for pulmonary atresia? Heart, September 15, 2009; 95(18): 1473 - 1474. [Full Text] [PDF]

				R. L. Hannan, J. A. Zabinsky, R. M. Stanfill, R. A. Ventura, A. F. Rossi, D. G. Nykanen, E. M. Zahn, and R. P. Burke Midterm Results for Collaborative Treatment of Pulmonary Atresia With Intact Ventricular Septum Ann. Thorac. Surg., April 1, 2009; 87(4): 1227 - 1233. [Abstract] [Full Text] [PDF]

				E. A Shinebourne, M. L Rigby, and J. S Carvalho Pulmonary atresia with intact ventricular septum: from fetus to adult Heart, October 1, 2008; 94(10): 1350 - 1357. [Full Text] [PDF]

				J. W. Salvin, D. B. McElhinney, S. D. Colan, K. Gauvreau, P. J. del Nido, K. J. Jenkins, J. E. Lock, and W. Tworetzky Fetal Tricuspid Valve Size and Growth as Predictors of Outcome in Pulmonary Atresia With Intact Ventricular Septum Pediatrics, August 1, 2006; 118(2): e415 - e420. [Abstract] [Full Text] [PDF]

				P. E.F. Daubeney, D. Wang, D.J. Delany, B.R. Keeton, R.H. Anderson, Z. Slavik, M. Flather, S.A. Webber, and for the UK and Ireland Collaborative Study of Pulm Pulmonary atresia with intact ventricular septum: Predictors of early and medium-term outcome in a population-based study J. Thorac. Cardiovasc. Surg., October 1, 2005; 130(4): 1071 - 1071. [Abstract] [Full Text] [PDF]

				Y. Tanoue, H. Kado, T. Maeda, Y. Shiokawa, N. Fusazaki, and S. Ishikawa Left ventricular performance of pulmonary atresia with intact ventricular septum after right heart bypass surgery J. Thorac. Cardiovasc. Surg., November 1, 2004; 128(5): 710 - 717. [Abstract] [Full Text] [PDF]