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Dilated cardiomyopathy presenting during fetal life

Published online by Cambridge University Press:  13 July 2005

Sivasubramonian Sivasankaran ,
Gurleen K. Sharland  and
John M. Simpson
Sivasubramonian Sivasankaran
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, Fetal Cardiology Unit, London, United Kingdom
Gurleen K. Sharland
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, Fetal Cardiology Unit, London, United Kingdom
John M. Simpson
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, Fetal Cardiology Unit, London, United Kingdom
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Abstract

Objectives: To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy. Design: A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology. Patients: Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation. Main outcomes: A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses. Conclusions: Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.

Keywords

Fetusfetal heartcardiomyopathyechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 4 , August 2005 , pp. 409 - 416
Copyright
© 2005 Cambridge University Press

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References

Strauss A, Lock JE. Pediatric cardiomyopathy – a long way to go. N Engl J Med 2003; 348: 1703–1705.Google Scholar
Pedra SR, Smallhorn JF, Ryan G, et al. Fetal cardiomyopathies: pathogenic mechanisms, hemodynamic findings, and clinical outcome. Circulation 2002; 106: 585–591.Google Scholar
Webber SA, Sandor GGS, Farquharson D, Taylor GP, Jamieson S. Diagnosis and outcome of dilated cardiomyopathy in the fetus. Cardiol Young 1993; 3: 27–33.Google Scholar
Schmidt KG, Birk E, Silverman NH, Scagnelli SA. Echocardiographic evaluation of dilated cardiomyopathy in the human fetus. Am J Cardiol 1989; 63: 599–605.Google Scholar
Tan J, Silverman NH, Hoffman JI, Villegas M, Schmidt KG. Cardiac dimensions determined by cross-sectional echocardiography in the normal human fetus from 18 weeks to term. Am J Cardiol 1992; 70: 1459–1467.Google Scholar
Simpson JM, Cook A. Repeatability of echocardiographic measurements in the human fetus. Ultrasound Obstet Gynecol 2002; 20: 332–339.Google Scholar
Simpson JM. Echocardiographic evaluation of cardiac function in the human fetus. MD Thesis, University of London, 2000.Google Scholar
Paladini D, Chita SK, Allan LD. Prenatal measurement of cardiothoracic ratio in evaluation of heart disease. Arch Dis Child 1990; 65: 20–23.Google Scholar
Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639–1646.Google Scholar
Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348: 1647–1655.Google Scholar
Johnson P, Maxwell DJ, Tynan MJ, Allan LD. Intracardiac pressures in the human fetus. Heart 2000; 84: 59–63.Google Scholar
Ross RS. Regulation of gene expression: how can molecules make the right ventricle distinct from the left? Nucl Cardiol 1998; 5: 609–621.Google Scholar
Thomas T, Yamagishi H, Overbeek PA, Olson EN, Srivastava D. The bHLH factors, dHAND and eHAND, specify pulmonary and systemic cardiac ventricles independent of left-right sidedness. Dev Biol 1998; 196: 228–236.Google Scholar
Feldman AM, McNamara D. Myocarditis. N Engl J Med 2000; 343: 1388–1398.Google Scholar
Schwartz RS, Curfman GD. Can the heart repair itself? N Engl J Med 2002; 346: 2–4.Google Scholar
Bowles NE, Kearney DL, Ni J, Perez-Atayde AR. The detection of viral genomes by polymerase chain reaction in the myocardium of pediatric patients with advanced HIV disease. J Am Coll Cardiol 1999; 34: 857–865.Google Scholar
Hornberger LK, Lipshultz SE, Easley KA, et al. Cardiac structure and function in fetuses of mothers infected with HIV: the prospective PCHIV multicenter study. Am Heart J 2000; 140: 575–584.Google Scholar
Levy R, Weissman A, Blomberg G, Hagay ZJ. Infection by parvovirus B 19 during pregnancy: a review. Obstet Gynecol Surv 1997; 52: 254–259.Google Scholar
Kawai C. From myocarditis to cardiomyopathy: mechanisms of inflammation and cell death: learning from the past for the future. Circulation 1999; 99: 1091–1100.Google Scholar
Schwartz ML, Cox GF, Lin AE, et al. Clinical approach to genetic cardiomyopathy in children. Circulation 1996; 94: 2021–2038.Google Scholar
Huhta JC, Moise KJ, Fisher DJ, Sharif DS, Wasserstrum N, Martin C. Detection and quantitation of constriction of the fetal ductus arteriosus by Doppler echocardiography. Circulation 1987; 75: 406–412.Google Scholar
Zeltser I, Parness IA, Ko H, Holzman IR, Kamenir SA. Midaortic syndrome in the fetus and premature newborn: a new etiology of nonimmune hydrops fetalis and reversible fetal cardiomyopathy. Pediatrics 2003; 111: 1437–1442.Google Scholar
Karatza AA, Holder SE, Gardiner HM. Isolated non-compaction of the ventricular myocardium: prenatal diagnosis and natural history. Ultrasound Obstet Gynecol 2003; 21: 75–80.Google Scholar
Guntheroth W, Komarniski C, Atkinson W, Figner CL. Criterion for fetal primary spongiform cardiomyopathy: restrictive pathophysiology. Obstet Gynecol 2002; 99: 882–885.Google Scholar
Brook WH, Connell S, Cannata J, Maloney JE, Walker AM. Ultrastructure of the myocardium during development from early fetal life to adult life in sheep. J Anat 1983; 137: 729–741.Google Scholar