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Ann Thorac Surg 2000;70:287-289
© 2000 The Society of Thoracic Surgeons

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Case report

Strategy for pulmonary atresia and intact ventricular septum

^a Departments of Thoracic and Cardiovascular Surgery and of Pediatrics, Mie University School of Medicine Mie, Japan

Address reprint requests to Dr Shimpo, Department of Thoracic and Cardiovascular Surgery, Mie University School of Medicine, 2–174 Edobashi, Tsu, Mie, Japan 5148507
e-mail: shimpo{at}clin.medic.mie-u.ac.jp

	Abstract

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Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.

	Introduction

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Pulmonary atresia with an intact ventricular septum (PA IVS) is still one of the most difficult congenital cardiac defects to treat. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate continues to be more than 20% in most series [1]. We describe a case of PA IVS in which biventricular surgical repair was done using an atrial septal defect (ASD) patch with a one-way valve [2] and a right ventricular (RV) overhaul [3] in early infancy after an initial balloon valvotomy [4].

This male infant was born after a full-term pregnancy and weighed 3,672 g at birth. Physical examination revealed a heart murmur and moderate cyanosis. An echocardiograph showed PA IVS with a tripartite but severely hypoplastic RV, ASD, and a patent ductus arteriosus. The diameter of the RV outflow was 2 mm, and that of the tricuspid annulus was 8 mm by echocardiography (z value = -2.5).

The patient was started on prostaglandin E1 (PGE1) to maintain patency of the ductus. At 9 days of age, cardiac catheterization and balloon valvotomy were performed. Cardiac catheterization showed a suprasystemic RV pressure of 135/10 mm Hg and a left ventricular pressure of 60/8 mm Hg. A 0.018-inch guide wire was introduced through the 5 F end-hole catheter to the RV outflow and punctured the atretic pulmonary valve. The balloon angiography catheter was advanced over the guide wire and inflated. After balloon valvotomy, the systolic RV pressure fell from 135 to 46 mm Hg. The right ventricular end diastolic volume (RVEDV) [5] was 2.7mL (30% of the predicted normal value) (Fig 1A). The patient tolerated the procedure well.

View larger version (62K): [in this window] [in a new window]   Fig 1. Right ventriculography. (A) Before balloon valvotomy, 9 days of age. (B) Post-balloon valvotomy, 1 month of age. (C) After biventricular repair, 6 months of age.

After recovering from enteritis, the patient underwent definitive repair at the age of 68 days; he weighed 3,198 g at the time. After cardiopulmonary bypass was established, a vertical incision was made in the proximal pulmonary trunk. A hole approximately 2 mm in diameter was present in the original atretic valve tissue. An infundibular resection was performed through a transverse ventriculotomy. In addition to the infundibular resection, we resected as much of the hypertrophied muscle of the inflow portion as possible to obtain a satisfactory RV cavity (RV overhaul) [5]. The pulmonary trunk and RV outflow were augmented with autologous pericardium. The ASD was closed with a one-way valve patch, which consisted of two layers: one of bovine pericardium and the other of autologous pericardium. A central circular opening, which was 3 mm in diameter, was created in the bovine pericardium and covered by a smaller layer of autopericardium.

The postoperative course was uneventful. Early in the postoperative period, echocardiography showed a right-to-left shunt (Fig 2), and an arterial oxygen saturation of 90% was noted. One week after the operation, echocardiography showed no interatrial shunt and the arterial oxygen saturation was 98%. Postoperative angiography (Fig 1C) demonstrated increased RV volume and tricuspid regurgitation. The right atrial pressure was 5 mm Hg and the RV pressure was 35/4 mm Hg. No pressure gradient was present between the RV and pulmonary artery. The patient was discharged from the hospital and, after 2 years, is still doing well.

View larger version (21K): [in this window] [in a new window]   Fig 2. Echocardiography shows a right-to-left shunt through a one-way valve patch.

	Comment

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Another alternative is "one and half repair" [7] in case of marginal RV size. After biventricular repair, the systemic venous pressures may be excessive and the cardiac output may be low. Right-to-left interatrial shunting reduces the RV preload and augments left ventricular preload and cardiac output.

The major disadvantage of ASD left open, however, is that the ASD will require closure by a subsequent invasive procedure [8], including percutaneous closure of ASD. A second operation may be avoided by using an atrial septal patch with a one-way valve. Our experience suggests that balloon valvotomy, RV overhaul, and flap-valve ASD creation might be a useful strategy for PA IVS.

	References

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1. Hanley F.L., Sade R.M., et al. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993;105:406-427.[Abstract]
2. Ad N., Birk E., Barak J., Diamant S., Snir E., Vidne B.A. A one-way valved atrial septal patch. J Thorac Cardiovasc Surg 1996;111:841-848.[Abstract/Free Full Text]
3. Pawade A., Capuani A., Penny D.J., Karl T.R., Mee R.B.B. Pulmonary atresia with intact ventricular septum. J Card Surg 1993;8:371-383.[Medline]
4. Fedderly R.T., Lloyd T.R., Mendelsohn A.M., Beekman R.H. Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum. J Am Coll Cardiol 1995;25:460-465.[Abstract]
5. Graham T.P., Jr, Jamakani J.M., Atwood G.F., Canet R.V., Jr Right ventricular volume determination in children. Circulation 1973;47:144-153.[Abstract/Free Full Text]
6. Alboliras E.T., Julsrud P.R., Danielson G.K., et al. Definitive operation for pulmonary atresia with intact ventricular septum. Results in twenty patients. J Thorac Cardiovasc Surg 1987;93:454-464.[Abstract]
7. Billingsley A.M., Laks H., Boyce S.W., George B., Santuli T., Williams R.G. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1989;97:746-754.[Abstract]
8. Steinberger J., Berry J.M., Bass J.L., et al. Results of a right ventricular outflow patch for pulmonary atresia with intact ventricular septum. Circulation 1992;86(Suppl II):167-175.[Abstract/Free Full Text]

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This Article Abstract 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): Isao Yada Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Shimpo, H. Articles by Yada, I. Search for Related Content PubMed PubMed Citation Articles by Shimpo, H. Articles by Yada, I.