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Morphologic and hemodynamic results of staged repair of pulmonary atresia with ventricular septal defect in the presence of hypoplastic pulmonary arteries and systemic-to-pulmonary collateral arteries

Published online by Cambridge University Press:  19 August 2008

James L. Wilkinson*
Affiliation:
From the Departments of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne
Yin Ming Ng
Affiliation:
From the Departments of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne
Krishnan S. Iyer
Affiliation:
From the Departments of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne
Roger B. B. Mee
Affiliation:
From the Departments of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne
*
Correspondence to Dr. James L. Wilkinson, Department of Cardiology, Royal Children's Hospital, Parkville, Victoria 3052, Australia. Tel. (03) 345 5716; Fax. (03) 342 5708.

Abstract

Follow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 1993

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References

1.Miller, WW, Nadas, AS, Bernhard, WF, Gross, RE. Congenital pulmonary atresia with ventricular septal defect: Review of the clinical course of fifty patients with assessment of the results of palliative surgery. Am J Cardiol 1968; 21: 673680.CrossRefGoogle ScholarPubMed
2.Kirklin, JW, Bargeron, LM Jr, Pacifico, AD. The enlargement of small pulmonary arteries by preliminary palliative operations. Circulation 1977; 56: 612617.CrossRefGoogle ScholarPubMed
3.McGoon, MD, Fulton, RE, Davis, GD, Ritter, DG, Neil, CA, White, RI Jr. Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect. Circulation 1977; 56: 473479.CrossRefGoogle ScholarPubMed
4.Rabinovitch, M, Herrera–deLeon, V, Castañeda, AR, Reid, L. Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia. Circulation 1981; 64: 12341248.CrossRefGoogle ScholarPubMed
5.Iyer, KS, Mee, RBB. Staged repair of pulmonary atresia and ventricular Septal defect with hypoplastic pulmonary arteries and major systemic to pulmonary artery collaterals. Ann Thorac Surg 1991; 51: 6572.CrossRefGoogle ScholarPubMed
6.Haworth, SG, MacArtney, FJ. Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J 1980; 44: 1424.CrossRefGoogle ScholarPubMed
7.Snider, AR, Enderlein, MA, Teitel, DF, Juster, RP. Two–dimensional echocardiographic determination of aortic and pulmonary artery sizes from infancy to adulthood in normal subjects. Am J Cardiol 1984; 53: 218224.CrossRefGoogle ScholarPubMed