A Case of Restrictive Cardiomyopathy and Findings of Family Screening [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2002; 30(5): 302-309

A Case of Restrictive Cardiomyopathy and Findings of Family Screening

Dilek URAL1, Ahmet VURAL1, Ayşen AĞAÇDİKEN2, Ertan URAL1, Teoman KILIÇ1, Göksel KAHRAMAN1, Baki KOMSUOĞLU1
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Restrictive cardiomyopathy is a rare disease of the heart muscle. We present here the clinical findings and results of family screening of a case with restrictive cardiomyopathy. All affected cases had palpitation and atypical chest pain. Echocardiographic examination revealed mild to moderate and localized left ventricular hypertrophy, marked left atrial enlargement and restrictive filling pattern in two, mid-segmental hypertrophy and relaxation abnormality in one patient. Ambulatory ECG recordings showed frequent supraventricular arrhythmias and ST segment depression in all three cases. Two of them died with sudden death, nine months after diagnosis. The third case received antiarrhythmic, anticoagulant and heart failure therapy. In electrophysiological study an atrioventricular nodal reentrant tachycardia, a ventricular tachycardia and ventricular fibrillation were detected. The patient underwent a successful slow-pathway radiofrequency catheter ablation and an ICD was implanted. The cardiomyopathy presenting with different phenotypic findings in the same family, high risk for sudden death and poor prognosis and its characteristics consistent with restrictive and hypertrophic cardiomyopathy were discussed.


How to cite this article
Dilek URAL, Ahmet VURAL, Ayşen AĞAÇDİKEN, Ertan URAL, Teoman KILIÇ, Göksel KAHRAMAN, Baki KOMSUOĞLU. A Case of Restrictive Cardiomyopathy and Findings of Family Screening. Turk Kardiyol Dern Ars. 2002; 30(5): 302-309
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