Pregnancy in women with valvular heart disease

Patients with valvular disease who desire pregnancy or are already pregnant require specialised care. Ideally, women undergo preconceptual counselling that addresses any procedures needed to decrease the risks of pregnancy, including valve replacement, if the patient has symptoms at baseline. Management during pregnancy includes replacing any contraindicated medications with safer alternatives, optimising loading conditions, careful monitoring and aggressive treatment of any exacerbating factors. Rarely, percutaneous or surgical intervention is required during pregnancy. Labour and delivery often require invasive haemodynamic monitoring and a multi-disciplinary team for optimal maternal and fetal outcomes.     

Pregnancy and congenital heart disease

Congenital heart disease as a complicating factor in pregnancy has assumed increasing clinical importance because improved techniques of surgical repair have resulted in a larger proportion of affected women living to the reproductive age. The most serious forms are those associated with pulmonary hypertension (such as the Eisenmenger syndrome), which carry a prohibitively high risk of maternal death. Complex forms of cyanotic heart disease, of which the commonest is the tetralogy of Fallot, are only slightly less dangerous. It has recently been recognized that children born to women with congenital heart disease are at increased risk of having cardiac defects; fetal echocardiography is therefore an important diagnostic test. Optimal care of the pregnant woman with congenital heart disease is best provided by a team consisting of internist-cardiologist, obstetrician-perinatologist, obstetric anesthesiologist, and ultrasonographer-echocardiographer.     

Pregnancy and congenital heart disease

Congenital cardiopathies currently represent the majority of cardiac anomalies observed during pregnancy. The latter leads to significant haemodynamic modifications which can sometimes be poorly tolerated in the case of severe cardiopathy. However, as a rule pregnancy is generally well supported if the functional state of the patient beforehand is satisfactory (class I or II of the NYHA). The type of cardiopathy also plays a role. Left-right shunts tolerate pregnancy well in most cases. On the other hand severe aortic stenoses expose serious complications. Above all it is the cyanogenic cardiopathies which are poorly tolerated by the mother and the fetus, and more so the lower the oxygen saturation. Eisenmenger's syndrome causes a major risk to life for the young mother and is a formal contra-indication to pregnancy, as is pulmonary arterial hypertension. Patients with a Fontan anastomosis or an intra-atrial repair of transposition of the great vessels can support pregnancy very well if the systemic ventricle has good function. In Marfan's syndrome the aortic diameter is the essential prognostic element. In most congenital cardiopathies pregnancy nevertheless has a favourable outcome for the mother and infant. Vaginal delivery should be the rule. A close cardio-obstetric collaboration is the principal condition to allow the young cardiac female to well endure a pregnancy.     

Pregnancy in women with congenital heart disease: the importance of evaluation and counselling

Issues surrounding the growing population of pregnant women with congenital heart disease are becoming increasingly important as more women with surgically corrected heart conditions reach childbearing age.     

Contraception in women with congenital heart disease

The present study reports on contraceptive use, methods used, and counseling received on contraceptive issues for women with congenital heart disease and provides a brief review of current knowledge of the risks in relation to the different cardiac situations encountered with these specific patients. A total of 536 consecutive adult women with congenital heart disease (median age 29 years) were recruited from 2 tertiary care centers. They underwent a clinical assessment and completed a questionnaire regarding their contraceptive use. Oral contraceptives, condoms, and intrauterine devices were the most commonly used methods. Pregnancy occurred in almost every tenth woman despite the use of contraception. We identified a substantial number of women (20%) who were presently using contraceptive methods that were contraindicated for their specific cardiac condition. Additionally, a high proportion of patients (28%), in the group with high pregnancy-associated risks, were not using contraception despite having a sexual relationship. In our study, 43% of the women had not been counseled about contraception, and 48% had not been informed of the pregnancy-related risks by their treating physician. In conclusion, timely and competent counseling about contraception is important for women with congenital heart disease. Collaboration between cardiologists and gynecologists should be strengthened. Failure to give adequate family planning advice to this patient group could have hazardous consequences, causing an unnecessary risk to mother and child.     

Pregnancy and its outcome in women with and without surgical treatment of congenital heart disease

In the state of Connecticut, 233 women with congenital heart defects were prospectively followed up through 482 pregnancies that resulted in 372 infants who were examined frequently during their first 3 years of life. Approximately half of the women had undergone cardiac surgery and they were compared with the women without operation. There was no maternal mortality, and no patient had infective endocarditis, brain abscess or a cerebrovascular accident. The proportion of pregnancies resulting in live births did not differ significantly in mothers with and without cardiac surgery; the average live birth rate was 77 percent in all. However, the number and size of live-born infants was much greater in mothers who had become acyanotic as a result of reparative surgery than in the still cyanotic women, whether or not they had had palliative surgery. In cyanotic women, placental size was abnormally large in relation to birth weight, which was abnormally low.When the mothers were classified according to cardiac function, there was a significant difference between the number of infants born alive to mothers in good to excellent status and the number born to mothers in fair to poor condition. The latter had a significant increase in interrupted pregnancies as well as in cardiovascular complications during pregnancy. The total group had a 16.1 percent incidence rate of infants with congenital heart disease. This rate was corrected to 14.2 percent by removal of seven mothers, two with Noonan's syndrome, one with hypertrophic cardiomyopathy and four with a family history of congenital heart defects.     

Outcome of pregnancy in women with congenital heart disease.

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm. Fetal outcome is complicated by a high rate of spontaneous abortions (20-25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation. Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.     

Pregnancy in women with congenital factor VII deficiency

The aim was to review the pregnancy and obstetric outcome in women with factor VII (FVII) deficiency. The study group contained women with FVII deficiency, registered with Haemophilia centre and Haemostasis Unit at the Royal Free Hospital, London. The women were interviewed and case notes were reviewed. The main outcome measures were changes in FVII levels in pregnancy, maternal and perinatal outcome. The FVII levels ranged from 7-36 IU dL(-1) in the 13 women included in the study. There were a total of 14 pregnancies in seven women. Ten pregnancies in four women were prior to the diagnosis of FVII deficiency. Following the diagnosis of FVII deficiency, there were four pregnancies in three women. There was an increase in the FVII level during pregnancy in these women from a mean baseline level of 33 IU dL(-1) to a mean of 73 IU dL(-1). These women received recombinant FVIIa replacement during labour and delivery. There were two early pregnancy losses, both associated with excessive haemorrhage. There was only one postpartum haemorrhage in the study. There is a significant increase in FVII levels in pregnancy in women with heterozygous FVII deficiency. The risk of bleeding in early pregnancy might be higher than that at term, due to inadequate rise in the FVII level in early pregnancy.     

Accuracy of risk prediction scores in pregnant women with congenital heart disease

Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
Design: Single‐center retrospective study.
Setting: Tertiary care academic hospital.
Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myo‐ cardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum.
Results: Of 178 women, the most common CHD lesions were congenital aortic ste‐ nosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and te‐ tralogy of Fallot (12.9%). Thirty‐five women (19.7%) sustained 39 cardiac events. Observed vs expected event rates were 9.9% vs 5% (P = .02) for CARPREG I score 0 and 26.1% vs 7.5% (P < .001) for ZAHARA scores 0.51‐1.5. ZAHARA outperformed CARPREG I at predicting adverse cardiovascular outcomes (AUC 0.80 vs 0.72, P = .03) but was not significantly better than modified WHO. Clinical predictors of adverse cardiac event were symptoms (P = .002), systemic ventricular dysfunction (P < .001), and subpulmonary ventricular dysfunction (P = .03) with an AUC 0.83 comparable to ZAHARA (P = .66).
Conclusions: CARPREG I and ZAHARA scores underestimate cardiac risk for lower risk pregnancies in these women. Of the three risk schemes, CARPREG I performed least well in predictive capacity. Clinical factors specific to the population studied are comparable to stratification schemes.