Menstrual problems are associated with elevated central venous pressure and predict adverse clinical events in women with congenital heart disease

Objectives: Despite the high prevalence of menstrual problems (MPs), their associations with hemodynamics and their prognostic value in women with congenital heart disease (WCHD) remain unknown. Methods: We retrospectively evaluated characteristics related to menstruation (age at menarche, menstrual cycle, and menstrual blood loss) in 143 consecutive WCHD, and investigated the associations of these characteristics with hemodynamics and prognosis. Results: A total of 132 WCHD were analyzed after excluding 11 patients in menopause. Diagnoses of abnormal onset of menarche (delayed menarche or primary amenorrhea) (AOM) and menstrual cycle disorders (MCDs) were made in 11 (8.3%) and 64 (48.5%) patients, respectively. Cyanotic CHD, non-left systemic ventricle, multiple surgeries before menarche, and high central venous pressure (CVP) during puberty (≥13 ​mmHg) were associated with AOM (p ​< ​0.05). Multiple surgeries and current high CVP (≥6 ​mmHg) (odds ratio [OR]: 2.8, 95% confidence interval [CI], 1.2–6.7, p ​< ​0.05) were associated with MCDs. Abnormal menstrual blood loss occurred in 38 patients (28.8%) without clinical predictors. During a mean follow-up period of 69 months, 47 patients experienced clinical events. High CVP (p ​< ​0.01), and also AOM (OR 5.1, 95%CI 1.2–20.5, p ​= ​0.012) and MCDs (OR 2.9, 95%CI 1.3–6.7, p ​= ​0.007), were associated with clinical events. The prognostic value of MCDs tended to be independent of CVP (p ​= ​0.055). Conclusions: We reconfirmed the high prevalence of MPs in WCHD. In addition to known risk factors, elevations of pubertal and current CVP were associated with high risks of AOM and MCDs, and MPs may be unique prognostic factors in WCHD