Clinical Course and Prognosis of Hypertrophic Cardiomyopathy in an Outpatient Population

Abstract

Hypertrophic cardiomyopathy has been investigated mainly at referral institutions. Thus, the clinical history of the disease that emerges from published studies could be influenced by a bias in patient selection. In the present study, we compared the clinical features of an outpatient population of 25 patients who had hypertrophic cardiomyopathy with those reported in 78 studies published during the past five years. In the 25 study patients, age, sex, and the extent of left ventricular hypertrophy, as well as the prevalence of diastolic filling abnormalities, subaortic obstruction, and ventricular arrhythmias, were similar to those in patients described in the literature. Cardiac symptoms, however, were much less severe in the study patients. Eighteen patients (72 percent) were asymptomatic, six (24 percent) had mild symptoms, and only one (4 percent) had moderate-to-severe symptoms. Of 24 patients followed for a mean period of 4.4 years (range, 2.9 to 5.7), none died or had clinical deterioration. Of 3404 patients described in the 78 studies we reviewed, 2483 (73 percent) came from only two referral institutions. Of the 1721 patients in whom severity of symptoms was reported, 757 (44 percent) had moderate-to-severe symptoms. However, 727 (96 percent) of these patients were studied at one of the same two referral institutions. We conclude that the natural history of hypertrophic cardiomyopathy may be more benign than can be inferred from published reports.

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

“…The survival rates in these 2 studies are much better than previously reported in children with HCM (3% to 8%) thought to be idiopathic. 22,23,25 Our experience parallels that in adults, in which the annual incidence of sudden death in HCM patients referred to tertiary care centers was reported to be 3% to 5%, but subsequent population studies in adults indicated a much lower annual mortality of 0.1% to 1%, [27][28][29][30][31] with asymptomatic adults at even lower risk. 32 Referral bias appears largely responsible for these differences.…”

Canadian Cardiovascular Society Consensus Conference guidelines on heart failure – 2008 update: Best practices for the transition of care of heart failure patients, and the recognition, investigation and treatment of cardiomyopathies

“…The onset of HCM can occur in both early and late adulthood (95), and there is a well-defined risk of sudden death, often at a young age. The majority of patients with HCM are asymptomatic (96). Symptoms may include chest pain, dyspnea, palpitations or syncope.…”

A redução do gradiente na via de saída do ventrículo esquerdo pelo marcapasso DDD em pacientes com miocardiopatia hipertrófica obstrutiva

“…(15) São freqüentes a angina (75% dos pacientes), o cansaço, a pré-síncope e a síncope. Estão também presentes: a palpitação, a dispnéia paroxística noturna, a insuficiência cardíaca congestiva e as tonturas.…”