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

Abstract

Aims: Inherited cardiomyopathies are relatively rare but carry a high risk of cardiac maternal morbidity and mortality during pregnancy and postpartum. However, data for risk stratification are scarce. The new CARPREG II score improves prediction of prognosis in pregnancies associated with heart disease, though its role in inherited cardiomyopathies is unclear. We aim to describe characteristics and cardiac maternal outcomes in patients with inherited cardiomyopathy during pregnancy, and to evaluate the interest of the CARPREG II risk score in this population.

Methods and results: In this retrospective single-centre study, 90 consecutive pregnancies in 74 patients were included (mean age 32 ± 5 years), including 28 cases of dilated cardiomyopathy (DCM), 46 of hypertrophic cardiomyopathy, 11 of arrhythmogenic right ventricular cardiomyopathy and 5 of left ventricular noncompaction, excluding peripartum cardiomyopathy. The discriminatory power of several risk scores was assessed by the area under the receiver-operating characteristic curve (AUC). Median CARPREG II score was 2 [0;3] and was higher in the DCM subgroup. A severe cardiac maternal complication was observed in 18 (20%) pregnancies, mainly driven by arrhythmia and heart failure (each event in 10 pregnancies), with 3 cardiovascular deaths. Forty-three pregnancies (48%) presented foetal/neonatal complications (18 premature delivery, 3 foetal/neonatal death). CARPREG II was significantly associated with cardiac maternal complications (P < 0.05 for all) and showed a higher AUC (0.782) than CARPREG (0.755), mWHO (0.697) and ZAHARA (0.604).

Conclusions: Pregnancy in women with inherited cardiomyopathy carries a high risk of maternal cardiovascular complications. CARPREG II is the most efficient predictor of cardiovascular complications in this population.

Keywords: Cardiomyopathy; Pregnancy; Prognosis; Risk score.

Figure 1

Flowchart of the cohort. ARVC, arrhythmogenic right ventricular cardiomyopathy; DCM, dilated cardiomyopathy; HCM, hypertrophic cardiomyopathy; LVNC, left ventricular noncompaction.

Figure 2

Incidence of cardiac maternal outcomes stratified according to CARPREG II risk score. Blue bars represent number of pregnancies according to CARPREG II risk score (categorized into four groups: in 0–1, 2, 3, and ≥4). Red bars represent number of cardiac maternal complications.

Figure 3

Performance of CARPREG II and other risk scores in prediction of cardiac maternal outcomes. Left figure: ROC curve analysis of CARPREG II risk score for cardiac maternal outcomes. Right figure: ROC curves of CARPREG II and previous scores CARPREG, ZAHARA, and mWHO, for cardiac maternal outcomes. *Cardiac maternal outcome is defined as the onset of any of the following cardiac maternal outcomes during pregnancy or within 6 months postpartum: maternal cardiac death, cardiac arrest, arrhythmia requiring treatment, heart failure, stroke or transient ischaemic attack, cardiac thromboembolism, myocardial infarction, and vascular dissection.