Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-24T14:00:01.679Z Has data issue: false hasContentIssue false

Pulmonary atresia with intact atrial and ventricular septums, unroofed coronary sinus, and absent aortocoronary arterial connections

Published online by Cambridge University Press:  08 August 2008

Lance K. Erickson*
Affiliation:
Department of Pathology, Primary Children’s Medical Center, Salt Lake City, Utah, United States of America
Michael D. Puchalski
Affiliation:
Department of Cardiology, Primary Children’s Medical Center, Salt Lake City, Utah, United States of America
Theodore J. Pysher
Affiliation:
Department of Pathology, Primary Children’s Medical Center, Salt Lake City, Utah, United States of America
*
Correspondence to: Lance K Erickson, Department of Pathology, Primary Children’s Medical Center, 100 N Mario Capecchi Dr, Salt Lake City, UT 84113, USA. Tel: (801) 662 2150; Fax: (801) 662 2165; E-mail: [email protected]

Abstract

A stillborn fetus with pulmonary valvar atresia and intact atrial and ventricular septums also had absence of coronary arterial connections from the aorta and an unroofed coronary sinus. A left superior caval vein drained to the dilated coronary sinus. The left coronary artery was anomalously connected to the proximal branch of the right pulmonary artery, and a fistula from the right ventricle supplied the right coronary artery.

Type
Brief Report
Copyright
Copyright © Cambridge University Press 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Opitz JM, ZuRhein GM, Vitale L, et al. The Zellweger syndrome (cerebrohepatorenal syndrome). Birth Defects: Original Article Series, Vol. V, No. 2; February, 1969.Google Scholar
2. Daubeney, PEF, Delany, DJ, Anderson, RH, et al. Pulmonary atresia with intact ventricular septum: Range of morphology in a population-based study. J Am Cardiol 2002; 39: 16701679.Google Scholar
3. Calder, AL, Peebles, CR, Occleshaw, CJ. The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results. Cardiol Young 2007; 17: 387396.Google Scholar
4. Gerlis, LM, Ho, SY, Milo, S. Three anomalies of the coronary arteries co-existing in a case of pulmonary atresia with intact ventricular septum. Int J Cardiol 1990; 29: 9395.Google Scholar
5. Gittenberger-de Groot, AC, Sauer, U, Bindl, L, Babic, R, Essed, CE, Buhlmeyer, K. Competition of coronary arteries and ventriculocoronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988; 18: 243258.Google Scholar
6. Lenox, CC, Briner, J. Absent proximal coronary arteries associated with pulmonic atresia. Am J Cardiol 1972; 30: 666669.Google Scholar
7. Wald, RM, Juraszek, AL, Pigula, FA, Geva, T. Echocardiographic diagnosis and management of bilateral coronary ostial atresia in a patient with pulmonary atresia and intact ventricular septum. J Am Soc Echocardiogr 2006; 19: 939.e1–e3.Google Scholar