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Cardiac function is affected in up to 90% of patients with Friedreich's ataxia, the most common spinocerebellar degenerative disease. Friedreich's ataxia typically causes motor abnormalities of the extremities, mainly impairing walking and the coordination of the legs and arms. The myocardium is affected at a later stage of the disease. The extent and timing of myocardial involvement determines the clinical course. Some patients have no cardiac symptoms and cardiac involvement can be established only by electrocardiographic or echocardiographic examination. In addition some pathological studies have found evidence of coronary abnormalities, mainly in the small vessels. There are no reports that such lesions cause angina. In a 16 year old patient chest pain on exercise had been the presenting symptom of Friedreich's ataxia at the age of 9. Considerable alterations in ventricular repolarisation on the electrocardiogram suggested a congenital coronary abnormality or hypertrophic myocardiopathy. The results of a Doppler echocardiography, Holter monitoring, and a haemodynamic study with coronary arteriography were all normal. An exercise test when the boy was 13 indicated significant changes in ventricular repolarisation. Myocardial scintigraphy (99mTc-MIBI) at that time, however, was normal. He improved slightly when he was treated with verapamil. When he was 15 neurological symptoms developed and Friedreich's ataxia was diagnosed. Typical angina during exercise seems to have been the first symptom of Friedreich's ataxia. [ABSTRACT FROM PUBLISHER]

Due to the continual progress being made in the surgical treatment of congenital heart diseases, very few symptomatic cardiac malformations can not now benefit from surgery, or more recently, from interventional catheterization. The result of such progress has led to an extraordinary change in survival patterns of congenital heart disease. With few exceptions, heart surgery is not curative. Residua and sequelae are the rule, and impose important medical, and sometimes surgical problems in the follow-up. Adequate control of this growing population is not easy within most of our adult cardiological departments. Adult patients with congenital heart disease represent a new area of cardiovascular interest.

A child with a membranous obstruction of the inferior vena cava (MOIVC) is presented. The diagnosis was confirmed by angiography. The clinic significance, the evolution, the surgical treatment and the possible complications are described.

A case of anomalous origin of the left coronary artery from the pulmonary artery (ALCA) is presented. The noninvasive diagnosis was made by 2D-color flow Doppler. Application of this technique may alleviate the necessity for angiography in patients with ALCA.

The arterial blood pressure and hypertension data obtained from an epidemiological study of endemic goiter in the rural population of Catalonia was analyzed. The prevalence of hypertension is of 25.1 +/- 2.16%, existing a predominance of women over men. The distribution of the arterial pressure values according to sex are presented, as well as the relationship between arterial blood pressure, weight and obesity.