Pregnant Women With Uncorrected Congenital Heart Disease: Heart Failure and Mortality

JACC Heart Fail. 2020 Feb;8(2):100-110. doi: 10.1016/j.jchf.2019.09.001. Epub 2019 Sep 11.

Abstract

Objectives: The purpose of this work was to study maternal and fetal outcomes of women with uncorrected congenital heart disease (CHD).

Background: Globally, CHD is an important cause of maternal morbidity and mortality in women reaching reproductive stage. Data are lacking from larger cohorts of women with uncorrected CHD.

Methods: The 10-year data from the European Society of Cardiology EORP ROPAC (EURObservational Research Programme Registry of Pregnancy and Cardiac disease) registry of women with uncorrected CHD were analyzed.

Results: Of 5,739 pregnancies in 53 countries, 3,295 women had CHD, 1,059 of which were uncorrected cases. Of these, 41.4% were from emerging countries. There were marked differences between the cardiac defects in uncorrected cases versus those in corrected CHD cases with primary shunt lesions (44.7% vs. 32.4%, respectively), valvular abnormalities (33.5% vs. 12.6%, respectively), and Tetralogy of Fallot and pulmonary atresia (0.8% vs. 20.3%, respectively; p < 0.001). In patients with uncorrected CHD, 6.8% were in modified World Health Organization risk class IV, approximately 10% had pulmonary hypertension (PH), and 3% were cyanotic prior to pregnancy. Maternal mortality and heart failure (HF) in the women with uncorrected CHD were 0.7% and 8.7%, respectively. Eisenmenger syndrome was associated with a very high risk of cardiac events (65.5%), maternal mortality (10.3%), and HF (48.3%). Coming from an emerging country was associated with higher pre-pregnancy signs of HF, PH, and cyanosis (p < 0.001) and worse maternal and fetal outcomes, with a 3-fold higher rate of hospital admissions for cardiac events and intrauterine growth retardation (p < 0.001).

Conclusions: Marked differences between cardiac conditions in pregnant women with uncorrected CHD and those in corrected CHD were found, with a markedly worse outcome, particularly in women with Eisenmenger syndrome and from emerging countries.

Keywords: Eisenmenger; emerging country; peripartum; pregnancy; pulmonary hypertension.

Publication types

  • Multicenter Study
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Female
  • Follow-Up Studies
  • Global Health
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / epidemiology
  • Heart Failure / epidemiology*
  • Heart Failure / etiology
  • Humans
  • Incidence
  • Maternal Mortality / trends
  • Pregnancy
  • Pregnancy Complications, Cardiovascular*
  • Prospective Studies
  • Registries*
  • Time Factors