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Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

Published online by Cambridge University Press:  19 August 2008

Kiyoshi Suzuki ,
Toshio Kikuchi  and
Shigekazu Mimori
Kiyoshi Suzuki*
Affiliation:
From the Departments of Pediatric Cardiology and Cardiac Surgery, Sakakibara Heart Institute, Tokyo.
Toshio Kikuchi
Affiliation:
From the Departments of Pediatric Cardiology and Cardiac Surgery, Sakakibara Heart Institute, Tokyo.
Shigekazu Mimori
Affiliation:
From the Departments of Pediatric Cardiology and Cardiac Surgery, Sakakibara Heart Institute, Tokyo.
*
Dr. Kiyoshi Suzuki, Department of Pediatric Cardiology, Sakakibara Heart Institute, 2-5-4 Yoyogi, Shibuya-ku, Tokyo 151, Japan. Tel. 3-3375-3111; Fax. 3-3375-9218.
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Summary

We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

Keywords

Atrioventricular septal defectdouble outlet right atriumlung biopsypulmonary venous obstruction
Type
Brief Reports
Information
Cardiology in the Young , Volume 4 , Issue 4 , October 1994 , pp. 402 - 404
Copyright
Copyright © Cambridge University Press 1994

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References

1. Van Mierop, LHS. Pathology and pathogenesis of endocardial cushion defects: Surgical implications. In: Davila, JC (ed). A Second Henry Ford Hospital International Symposium on Cardiac Surgery. Appleton-Century-Crofts, New York, 1977, pp 201–207.Google Scholar
2.Yamaki, S, Tezuka, F. Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries. Circulation 1976; 54: 805–809.Google Scholar
3.Yamaki, S, Mohri, H, Haneda, K, Endo, M, Akimoto, H. Indications for surgery based on lung biopsy in cases of ventricular septal defect and/or patent ductus arteriosus with severe pulmonary hypertension. Chest 1989; 96: 31–39.CrossRefGoogle ScholarPubMed
4.Utley, JR, Noonan, JA, Walters, LR, Frist, RA. Anomalous position of atrial septum with anomalous pulmonary and systemic venous drainage: correction including ligation of persistent left superior vena cava. J Thorac Cardiovasc Surg 1974; 67: 730–732.CrossRefGoogle ScholarPubMed
5.Corwin, RD. Singh, AK, Karlson, KE. Double outlet right atrium: a rare endocardial cushion defect. Am Heart J 1983; 106: 1156–1157.CrossRefGoogle ScholarPubMed
6.Pérez-Martínez, VM, García-Fernández, F, Oliver-Ruiz, J, Nuñez-González, L. Double outlet right atrium with two atrioventricular valves and left atrial outlet atresia. J Am Coll Cardiol 1984; 3: 375–380.CrossRefGoogle ScholarPubMed
7.Alivizatos, P, Anderson, RH, Macartney, FJ, Zuberbuhler, JR, Stark, J. Atrioventricular septal defect with balanced ventricles and malaligned atrial septum: double-outlet right atrium. J Thorac Cardiovasc Surg 1985; 89: 295–297.Google Scholar
8.Ahmadi, A, Mocellin, R, Spillner, G, Gildein, HP. Atrioven tricular septal defect with double-outlet right atrium. Pediatr Cardiol 1989; 10: 170–173.Google Scholar
9.Stare, TJ, Bierman, FZ, Bowman, FO Jr, Steeg, CN, Wang, NK, Krongrad, E. Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium. J Am Coll Cardiol 1987; 9: 830–833.CrossRefGoogle Scholar
10.Cazzaniga, M, Gómez, MC, de Melo, WD. The role of colorflow mapping in confirming the association of divided left atrium with an atrioventricular septal defect. Cardiol Young 1993; 3: 57–59.CrossRefGoogle Scholar