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Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death

Published online by Cambridge University Press:  19 August 2008

J. Thierrien
Affiliation:
Jane Somerville Grown Up Congenital Heart Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
L. M. Gerlis*
Affiliation:
Department of Cardiac Morphology, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
Philip Kilner
Affiliation:
Magnetic Resonance Imaging Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
Jane Somerville
Affiliation:
Jane Somerville Grown Up Congenital Heart Unit, National Heart and Lung Institute, Imperial College School of Medicine, Royal Brompton HospitalLondon, UK
*
Dr L. M. Gerlis, The National Heart and Lung Institute, Imperial College School of Medicine, Dovehouse Street, London SW3. Tel: 0171 352 8121; Fax: 0171 351 8230.

Abstract

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

Type
Clinico-Pathological Correlation
Copyright
Copyright © Cambridge University Press 1999

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References

1.Somerville, J. Clinical indentification of the pulmonary blood supply in pulmonary atresia. Anderson, RH, Neches, VM, Park, SC, Zuberbuhler, JR, eds: Perspectives in Pediatric Cardiology: Mount Kisco, NY, Futura Publishing Co. 1988. Vol. 1: pp 155168.Google Scholar
2.Solowiejczyk, DE, Cooper, MM, Barst, RJ, Quaegebeur, JM, Gersony, WM. Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature. Pediatr Cardiol 1995; 16: 9095.CrossRefGoogle ScholarPubMed
3.Caprioli, G, Aiazzi, L, Castelli, C, Baldrighi, G, Parenzan, L, Balrighi, V. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circ 1980; 61: 95102.Google Scholar
4.Dabizzi, R-P, Vigneswaran, WT, Pollock, JCS. Pulmonary atresia with ventricular septal defect and coronary artery fistula: a late presentation. Br Heart J 1988; 59: 387388.Google Scholar
5.Krongrad, E, Ritter, DG, Hawe, A, Kincaid, OW, McGoon, DC. Pulmonary atresia or severe stenosis and coronary artery-to-pulmonary artery fistula. Circulation 1972; 66: 10051012.CrossRefGoogle Scholar
6.Bartter, T, Irwin, RS, Nash, G. Aneurysms of the pulmonary arteries. Chest 1988; 94: 10651075.CrossRefGoogle ScholarPubMed
7.Bull, K, Somerville, J, Spiegelhalter, D. Presentation and attrition in complex pulmonary atresia. JACC 1995; 25: 491499.CrossRefGoogle ScholarPubMed
8.Nwaneri, NJ and Fortune, RL. Aneurysm of the pulmonary artery. Rare long term complication of central aorto-pulmonary shunts for congenital heart disease. J Cardiovasc Surg 1986; 27: 9499.Google ScholarPubMed
9.Marelli, AJ, Perloff, JK, Child, JS, Laks, H. Pulmonary atresia with ventricular septal defect in adults. Circulation 1994; 89: 243251.CrossRefGoogle ScholarPubMed
10.Sudhir, K, Gupta, K, Abraham, AK, Cherian, MP, Reddy, NK, Cherian, MK. Pulmonary atresia with ventricular septal defect in adult patients. Clin Cardiol 1987; 10: 350354.CrossRefGoogle ScholarPubMed
11.Bertranou, EG, Blackstone, EH, Hazelrig, JB, Turner, ME, Kirklin, JW. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol 1978; 42: 458466.CrossRefGoogle ScholarPubMed