Hypertrophic cardiomyopathy, ten years' experience.

Summary  A RETROSPECTIVE study was performed of patients who were diagnosed as having hypertrophic cardiomyopathy by left ventricular angiography in order to assess the incidence, the clinical and haemodynamic features and prognosis of this condition. The records of the cardiac catheter laboratory were reviewed for the ten year period from May 1975 to June 1985 to obtain haemodynamic and angiographic findings at diagnosis. Clinical features were obtained from the hospital records. To assess the prognosis, the clinical status of all patients, where possible, was reviewed in June 1985. During the ten year period 64 patients were diagnosed as having hypertrophic cardiomyopathy which was approximately 1% of all cardiac catheterizations. At diagnosis 22% of patients were asymptomatic and the commonest symptoms were chest pain or dyspnoea. Only 12% of patients had a family history of hypertrophic cardiomyopathy or sudden death. A systolic murmur was the most common abnormality on physical examination. Although evidence of left ventricular hypertrophy demonstrated by ECG was very common, only 13% of patients have evidence of an intraventricular pressure gradient. Of the 52 patients (81% of total) available for follow-up in June 1985, seven (13%) had died since diagnosis, all suddenly. The patients who died were significantly younger than the survivors and evidence of left ventricular hypertrophy was also more common in those who died. Our study confirms that although hypertrophie cardiomyopathy is uncommon, it carries a definite mortality. Thus definitive diagnosis of this condition is important especially as there is now evidence to suggest that the prognosis may be improved by treatment with amiodarone.