SUMMARY Cardiac abnormalities were identified in patients with familial palmoplantar keratosis. All of them were descended from families on the Greek island of Naxos. Four families were studied and nine cases of palmoplantar keratosis were identified; seven of them showed symptoms and signs of heart disease. Cardiomegaly on chest x ray and electrocardiographic abnormalities were common findings. Three cases had episodes of ventricular tachycardia and a fourth patient died suddenly. All patients with cardiac signs and symptoms showed echocardiographic enlargement of the right ventricle and a right ventricular band; in three the left ventricle was also affected.Several forms of hereditary palmoplantar keratosis are recognised in Mediterranean countries. In Greece the most common form is Meleda disease. 1 2Because recurrent ventricular tachycardia developed in two young patients with palmoplantar keratosis from the Greek island of Naxos we sought evidence of cardiac abnormalities in families on this island that included members with this dermatosis.
Patients and methodsWe identified four families from the Greek island of Naxos that included known cases of palmoplantar keratosis (fig 1)
SUMMARYEighty-seven patients with proven mitral leaflet prolapse were studied emphasizing cardiodynamics and left ventricular asynergy. Significant associated features were female preponderance (83%), skeletal anomalies (pectus excavatum, straight back, scoliosis, narrow antero-posterior diameter of the chest), and anomalous coronary arteries (cork-screw patterns, short left main coronary artery, anomalous origin of the coronary arteries). Prolapse of the tricuspid leaflets was found in 15 (54%) who had right ventriculography.
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