Skip to main content Accessibility help

Diagnosis and outcome of dilated cardiomyopathy in the fetus

  • Steven A. Webber (a1), George G. S. Sandor (a1), Duncan Farquharson (a1), Glenn P. Taylor (a1) and Sue Jamieson (a1)...


To identify the echocardiographic features and natural history of dilated cardiomyopathy in fetal life, we reviewed records of all fetal echocardiograms over the 10 year period 1980–1989 to identify cases of dilated cardiomyopathy without left ventricular outflow obstruction and conducted a retrospective review of the fetal echocardiograms and clinical course of those fetuses with dilated cardiomyopathy. In addition, all fetal losses, perinatal deaths and live-born infants presenting with dilated cardiomyopathy in the first seven days of life were identified from the British Columbia Pediatric Cardiovascular Pathology Registry and from inpatient records. The pathological findings and outcome for this group were also reviewed. Marked left ventricular dilatation and dysfunction without left ventricular outflow obstruction was diagnosed in five of 1,158 fetuses undergoing feta lechocardiography. These five fetuses were correctly distinguished from two others with critical aortic stenosis and severe left ventricular dysfunction. Two of the five fetuses had additional right ventricular dysfunction and dilatation and developed hydrops, while the remaining three with isolated severe left ventricular dysfunction did not and showed normal somatic growth. All fetuses were live-born with spontaneous labour occurring in four of five cases at a mean gestational age of 36.5 weeks. Four of the five infants died, with three deaths occurring in the first week of life. During the same 10 year period, two of 2450 autopsied stillbirths (both hydropic) were diagnosed with dilated cardiomyopathy. An additional 15 neonates (including the five identified by fetal echocardiography) were diagnosed in the first week of life with dilated cardiomyopathy, congenital myocarditis or endocardial fibroelastosis. Four were hydropic (all with associated right ventricular dysfunction). Only three of the 15 survive. Fetal echocardiography can accurately distinguish dilated cardiomyopathy from left ventricular outflow obstruction. In the absence of right-sided disease or premature closure of the oval fossa, severe left ventricular dysfunction is well tolerated in utero. The prognosis for fetal dilated cardiomyopathy is very poor.


Corresponding author

Dr. Steven A. Webber, Wessex Cardiothoracic Centre, Southampton General Hospital, Tremona Road, Southampton S09 4XY, United Kingdom.


Hide All
Allan, LD, Crawford, DC, Anderson, RH, Tynan, M.Spectrum of congenital heart disease detected echocardiographically in prenatal life. Br Heart J 1985; 54: 523526.
Schmidt, KG, Birk, E, Silverman, NH, Scagnelli, SA.Echocardiographic evaluation of dilated cardiomyopathy in the human fetus. Am J Cardiol 1989; 63: 599605.
DeVore, GR, Siassi, B, Plait, LD.Fetal echocardiography IV. M-mode assessment of ventricular size and contractility during the second and third trimesters of pregnancy in the normal fetus. Am J Obstet Gynecol 1984; 150: 981988.
DeVore, GR, Siassi, B, Platt, LD.Use of femur length as a means of assessing M-mode ventricular dimensions during second and third trimesters of pregnancy in normal fetus. J Clin Ultrasound 1985; 13: 619625.
Bovicelli, L, Picchio, FM, Pilu, G, Baccarani, G, Orsini, LF, Rizzo, N, Alampi, G, Benenati, PM, Hobbins, JC.Prenatal diagnosis of endocardial fibroelastosis. Prenat Diagn 1984; 4: 6772.
Reiter, AA.Huhta, JC, Carpenter, RJ, Klima, T.Prenatal diagnosis of endocardial fibroelastosis in a monozygotic twin. J Cardiovasc Ultrasonogr 1985; 4: 225228.
Ben-Ami, M, Shalev, E, Romano, S, Zuckerman, H.Midtrimester diagnosis of endocardial flbroelastosis and atrial septal defect: A case report. Am J Obstet Gynecol 1986; 155: 662663.
Huhta, JC.Uses and abuses of fetal echocardiography: A pediatric cardiologist's view. J Am Coil Cardiol 1986; 8:451458.
Veille, JC, Sivakoff, M.Fetal echocardiographic signs of congenital endocardial fibroelastosis. Obstet and Gynecol 1988; 72: 219222.
Carceller, AM, Maroto, E, Fouron, JC.Dilated and contracted forms of primary endocardial fibroelastosis: a single fetal disease with two stages of development. Br Heart J 1990; 63: 311313.
Robertson, MA, Byrne, PJ, Penkoske, PA.Perinatal management of critical aortic valve stenosis diagnosed by fetal echocardiography. Br Heart J 1989; 61: 365367.
Huhta, JC, Carpenter, RJ Jr, Moise, KJ Jr, Deter, RL, Ott, DA, McNamara, DG.The prenatal diagnosis and postnatal management of critical aortic stenosis. Circulation 1987; 75: 573576.
Karl, TR, Sano, S, Brawn, WJ, Mee, RBB.Critical aortic stenosis in the first month of life: Surgical results in 26 infants. Ann Thorac Surg 1990; 50: 105109.
Burch, M, Redington, AN, Carvalho, JS, Rusconi, P, Shinebourne, EA, Rigby, ML, Paneth, M, Lincoln, C.Open valvotomy for critical aortic stenosis in infancy. Br Heart J 1990; 63: 3740.
Sharland, GK, Chita, SK, Fagg, NLK, Anderson, RH, Tynan, M, Cook, AC, Allan, LD.Left ventricular dysfunction in the fetus: relation to aortic valve anomalies and endocardial fibroelastosis. Br Heart J 1991; 64: 419424.
McFadden, DE, Taylor, GP.Cardiac abnormalities and nonimmune hydrops fetalis: A coincidental, not causal, relationship. Pediatr Pathol 1989; 9: 1117.



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed