Impact of Pregnancy on the Natural History of Women with Hypertrophic Cardiomyopathy.

Whether pregnancy is a modifier of the long-term course and outcome of women with hypertrophic cardiomyopathy (HCM) is unknown. We assessed the association of pregnancy with long-term outcomes in HCM women. Retrospective evaluation of women with HCM from 1970 to 2021. Only women with pregnancy-related information (pregnancy present or absent) and a follow-up period lasting ≥1 year were included. The peripartum period was defined as -1 to 6 months after delivery. The primary endpoint was a composite for major adverse cardiovascular events (MACE: cardiovascular death, sudden cardiac death, appropriate defibrillator shock and heart failure [HF] progression). Overall, 379 (58%) women were included. There were 432 pregnancies in 242 (63%) patients. In 29 (7.6%) cases, pregnancies (n=39) occurred after HCM diagnosis. Among these, three carrying likely pathogenic sarcomeric variants suffered MACEs in the peripartum period. At 10±9 years follow-up, age at diagnosis (hazard Ratio [HR]: 1.034, 95% confidence interval [C.I.]: 1.018-1.050, p<0.001) and NYHA Class (II vs I: HR 1.944, 95% C.I. 0.896-4.218; III vs I: HR 5.291, 95% C.I. 2.392-11.705, p<0.001) were associated with MACE. Conversely, pregnancy was associated with reduced risk (HR 0.605; 95% C.I. 0.380-0.963, p=0.034). Among women with pregnancy, multiple occurrences did not modify risk. Pregnancy is not a modifier of long-term outcome in women with HCM, and mostly occurs before a cardiac diagnosis. Most patients tolerate pregnancy well and do not show a survival disadvantage compared to women without. Pregnancy should not be discouraged, except in the presence of severe HF symptoms or high-risk features. Hypertrophic Cardiomyopathy (HCM) is the most common genetic disorder of the myocardium and is characterized by important gender-related differences: women are typically 5 years older than men at diagnosis, over half are diagnosed >50 years of age and consistently show greater propensity than men for heart failure-related complications and adverse outcome. Whether pregnancy is a modifier of the long-term course and outcome of women with HCM is unknown. In this study, pregnancy was not a modifier of long-term outcome in women with HCM. In particular: At 10±7 years, most patients tolerated pregnancy well and did not show a survival disadvantage compared to women without pregnancies. Only baseline heart failure symptoms and age were associated with adverse outcome. Pregnancy should not be discouraged, except in the presence of severe HF symptoms or high-risk features. Nevertheless, cardio-obstetric counseling and close supervision is key in all instances, particularly in the peripartum period.

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