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Cardiomyopathy associated with congenital heart disease.

1971; BMJ; Volume: 33; Issue: 5 Linguagem: Inglês

10.1136/hrt.33.5.782

1468-201X

Abraham Shem-Tov, V Deutsch, Joseph H. Yahini, Henry N. Neufeld,

Cardiac Structural Anomalies and Repair

Thirteen patients with congenital heart disease associated with cardiomyopathy, all proven by right andlor left heart study or necropsy are described.The presence of cardiomyopathy was established before the age of 12 in 10 cases, 4 of them before 2 years.In 2 instances, familial occurrence of cardiomyopathy was observed.In 3 cases, cardiomyopathy was an integral part of the parachute mitral valve complex.The association of cardiomyopathy with congenital heart disease, itsfamilial occurrence, its discovery in childhood and infancy, and itsfrequent appearance in the parachute mitral valve complex all favour a congenital origin of some forms of cardio- myopathy, and not a coincidental occurrence of two conditions.It is felt that this combination is not as rare as is thought.The presence of cardiomyopathy associated with congenital heart disease should be suspected whenever the latter cannot explain one or more of thefollowing: the presence of heartfailure, evidence of left ventricular hypertrophy in the electrocardiogram andlor chest x-ray, or the suggestive carotid pulse pressure curve and/or apex cardiogram.The aetiology of most forms of cardiomyo- pathy remains unknown.There is evidence that in some instances the condition may be familial (Brent et al., i960), and in others acquired (Brachfeld and Gorlin, I959; Brock, I957).Furthermore, the presence of addi- tional congenital heart disease in some patients does point towards a congenital aetiology of the condition (Somerville and McDonald, I968).In reviewing the published material, we have found less than 40 documented cases of cardiomyopathy associated with congenital heart disease.The present article deals with I3 additional patients displaying this com- bination and supplies further support for the possibility that in some instances cardiomyopathy may be congenital in origin.Further- more, in the light of the above-mentioned experience, the clinical clues suggesting the presence of associated cardiomyopathy in patients with congenital heart disease are commented upon. Patients and methodsThis study comprises I3 patients: I2 with obstruc- tive and I with nonobstructive cardiomyopathy.The sex, age, and associated congenital heart disease are shown in Table I.The diagnosis of