Pregnancy and cardiac maternal outcomes in women with inherited cardiomyopathy: interest of the new CARPREG II risk score

Abstract

Abstract Introduction Inherited cardiomyopathies are rare but at high risk of cardiac maternal morbidity and mortality during pregnancy and post-partum. However data for risk stratification remains scarce. The new CARPREG II index score emerged to better predict prognosis in pregnancies with heart disease. Still, role of this score in inherited cardiomyopathies is unclear. Objective To describe characteristics and cardiac maternal outcomes in patients with inherited cardiomyopathy during pregnancy, and to evaluate the interest of the various index scores in this population. Methods In this retrospective monocentric study, 90 pregnancies of 74 patients were included (mean age 32 ± 5 years), with 28 dilated cardiomyopathies (DCM), 46 hypertrophic cardiomyopathies, 11 arrhythmogenic right ventricular cardiomyopathies and 5 left ventricular non compaction, excluding peri partum cardiomyopathies. Results A cardiac maternal complication was observed for 18 (20%) pregnancies, mainly driven by arrythmia and heart failure, and with 3 cardiovascular deaths. 43 (48 %) presented fetal or neo natal complications, with 18 premature delivery and 3 fetal or neonatal death. Median CARPREG II score was 2 [0;3], higher in the DCM subgroup. CARPREG II was significantly associated with the onset of cardiac maternal complication (p< 0.05 for all) and seemed to show a better performance for risk stratification than previous scores (Area under curve of ROC: 0.782 for CARPREG II, AUC 0.755 and 0.697 for CARPREG and mWHO respectively). Prior hospitalization for heart failure, left ventricular dysfunction, multiple line treatments, cardiac symptoms prior to pregnancy and late pregnancy assessment were associated with cardiac complications (p < 0.05 for all) Conclusion Pregnancy in women with inherited cardiomyopathy remains at high risk of maternal cardiovascular major events. Late pregnancy assessment and initial severity of cardiomyopathy were found associated with cardiac prognosis. The new CARPREG II score appeared as the most efficient for cardiac risk stratification in this population.