Korean Circ J.  2018 Apr;48(4):251-276. 10.4070/kcj.2018.0070.

Adult Congenital Heart Disease with Pregnancy

Affiliations
  • 1Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan. koniwa@luke.ac.jp

Abstract

The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Most frequent complications during pregnancy and delivery are heart failure, arrhythmias, bleeding or thrombosis, and rarely maternal death. Complications of fetus are prematurity, low birth weight, abortion, and stillbirth. Risk stratification of pregnancy and delivery relates to functional status of the patient and is lesion specific. Medication during pregnancy and post-delivery (breast feeding) is a big concern. Especially prescribing medication with teratogenicity should be avoidable. Adequate care during pregnancy, delivery, and the postpartum period requires a multidisciplinary team approach with cardiologists, obstetricians, anesthesiologists, neonatologists, nurses and other related disciplines. Caring for a baby is an important issue due to temporarily pregnancy-induced cardiac dysfunction, and therefore familial support is mandatory especially during peripartum and after delivery. Timely pre-pregnancy counseling should be offered to all women with CHD to prevent avoidable pregnancy-related risks. Successful pregnancy is feasible for most women with CHD at relatively low risk when appropriate counseling and optimal care are provided.

Keyword

Pregnancy; Delivery; Congenital heart disease

MeSH Terms

Adult*
Arrhythmias, Cardiac
Arteries
Constriction, Pathologic
Counseling
Female
Fetus
Fontan Procedure
Heart Defects, Congenital*
Heart Failure
Heart Ventricles
Hemorrhage
Humans
Hypertension, Pulmonary
Infant, Low Birth Weight
Infant, Newborn
Maternal Death
Peripartum Period
Postpartum Period
Pregnancy*
Stillbirth
Thrombosis
Ventricular Function

Figure

  • Figure 1 Incidence of structural heart disease in pregnancy in Japan. In 138 departments of obstetrics in Japan, 80,455 pregnancies were underwent during 2002 to 2003, 769 of them (0.96%) were from women with cardiovascular disease. Four hundred and seventy (0.58%) were structural heart disease and CHD is the most frequent with 245 of 470 (52.1%).AoRo D = aortic root disease; CHD = congenital heart disease; CM = cardiomyopathy; KD = Kawasaki disease; PH = pulmonary hypertension; VHD = valvular heart disease.

  • Figure 2 Risk of pregnancy in women with CHD.CHD = congenital heart disease; IUGR = intrauterine growth restriction.

  • Figure 3 Hematologic changes during pregnancy.CHD = congenital heart disease.


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