Risk associated with pregnancy in hypertrophic cardiomyopathy

We sought to assess mortality and morbidity in pregnant women with hypertrophic cardiomyopathy (HCM). The risk associated with pregnancy in women with HCM is an important and increasingly frequent clinical issue for which systematic data are not available and a large measure of uncertainty persists. Maternal mortality in 91 consecutively evaluated families with HCM was compared with that reported in the general population. The study cohort included 100 women with HCM with one or more live births, for a total of 199 live births. Morbidity related to HCM during pregnancy was investigated in 40 women evaluated within five years of their pregnancy. Two pregnancy-related deaths occurred, both in patients at a particularly high risk. The maternal mortality rate was 10 per 1,000 live births (95% confidence interval [CI] 1.1 to 36.2/1,000) and was in excess of the expected mortality in the general Italian population (relative risk 17.1, 95% CI 2.0 to 61.8). In the 40 patients evaluated within close proximity of their pregnancy, 1 (4%) of the 28 who were previously asymptomatic and 5 (42%) of the 12 with symptoms progressed to functional class III or IV during pregnancy (p < 0.01). One patient had atrial fibrillation and one had syncope, both of whom had already experienced similar and recurrent events before their pregnancy. Maternal mortality is increased in patients with HCM compared with the general population. However, absolute maternal mortality is low and appears to be principally confined to women at a particularly high risk. In the presence of a favorable clinical profile, the progression of symptoms, atrial fibrillation, and syncope are also uncommon during pregnancy.