Hostname: page-component-586b7cd67f-vdxz6 Total loading time: 0 Render date: 2024-11-24T13:51:43.507Z Has data issue: false hasContentIssue false
  • English
  • Français

Fistulous communications with the coronary arteries in the setting of hypoplastic ventricles

Published online by Cambridge University Press:  01 December 2010

Robert H. Anderson  and
Diane Spicer
Robert H. Anderson*
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
Diane Spicer
Affiliation:
Division of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America All Children’s Hospital, Congenital Institute of Florida, St. Petersburg, Florida, United States of America
*
Correspondence to: Professor R. H. Anderson, BSc, MD, FRCPath, 60 Earlsfield Road, London SW18 3DN, United Kingdom. Tel: 00 44 20 8870 4368; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Neonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.

Keywords

Coronary arterial anomaliespulmonary atresia with intact ventricular septumhypoplastic left heart syndrome
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 86 - 91
Copyright
Copyright © Cambridge University Press 2010

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.Norwood, WI, Lang, P, Hansen, DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983; 308: 23–26.Google Scholar
2.Freedom, RM, Jaeggi, E, Perrin, D, Yoo, SJ, Anderson, RH. The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum. Cardiol Young 2006; 16: 18–29.Google Scholar
3.Anderson, RH, Anderson, C, Zuberbuhler, JR. Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum. Cardiol Young 1991; 1: 105–114.CrossRefGoogle Scholar
4.Freedom, RM, Harrington, DP. Contribution of intramyocardial sinusoids in pulmonary atresia and intact ventricular septum to a right-sided circular shunt. Br Heart J 1974; 36: 1061–1065.Google Scholar
5.Freedom, RM, Anderson, RH, Perrin, D. The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum. Cardiol Young 2005; 15: 447–468.Google Scholar
6.O’Connor, WN, Cash, JB, Cottrell, CM, Johnson, GL, Noonan, JA. Ventriculocoronary connections in hypoplastic left hearts. An autopsy microscopic study. Circulation 1982; 66: 1078–1086.Google Scholar
7.Sauer, U, Gittenberger-de Groot, AC, Geishauser, M, et al. Coronary arteries in the hypoplastic left heart syndrome. Histopathologic and histometrical studies and implications for surgery. Circulation 1989; 80: I168–I176.Google Scholar
8.Roberson, DA, Cui, W, Cuneo, BF, Van Bergen, AH, Javois, AJ, Bharati, S. Extensive left ventricular to coronary artery connections in hypoplastic left heart syndrome. Echocardiography 2008; 25: 529–533.CrossRefGoogle ScholarPubMed
9.Oberhoffer, R, Cook, AC, Lang, D, et al. Correlation between echocardiographic and morphological investigations of lesions of the tricuspid valve diagnosed during fetal life. Br Heart J 1992; 68: 580–585.CrossRefGoogle ScholarPubMed
10.De Leval, M, Bull, C, Stark, J, Anderson, RH, Taylor, JFN, Macartney, FJ. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification. Circulation 1982; 66: 272–280.CrossRefGoogle ScholarPubMed
11.Sandor, GGS, Cook, AC, Sharland, GK, Ho, SY, Potts, JE, Anderson, RH. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life. Cardiol Young 2002; 12: 436–444.CrossRefGoogle ScholarPubMed
12.Dusek, J, Ostadal, B, Duskova, M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 1975; 99: 312–317.Google Scholar
13.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: 243–258.CrossRefGoogle ScholarPubMed
14.Calder, AL, Co, EE, Sage, MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987; 59: 437–442.CrossRefGoogle ScholarPubMed
15.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: 387–396.CrossRefGoogle ScholarPubMed
16.Dyamenahalli, U, McCrindle, BW, McDonald, CM, et al. Pulmonary atresia with intact ventricular septum: management and outcomes of 210 consecutive patients. Cardiol Young 2004; 14: 299–308.CrossRefGoogle ScholarPubMed
17.Lenox, CC, Briner, J. Absent proximal coronary arteries associated with pulmonic atresia. Am J Cardiol 1972; 30: 666–669.CrossRefGoogle ScholarPubMed
18.Hubbard, JF, Girod, DA, Caldwell, RA, Hurwitz, RA, Mahoney, LA, Waller, BF. Right ventricular infarction with cardiac rupture in an infant with pulmonary atresia with intact ventricular septum. JACC 1983; 2: 363–368.CrossRefGoogle Scholar