Reproductive health in adults with congenital heart disease: a review on fertility, sexual health, assisted reproductive technology and contraception

Figures & data

Figure 1. Causes of infertility attributed by sex.

Figure 2. Factors associated with infertility in both men and women.

Figure 3. The Assisted Reproductive Technology (ART) cycle and associated complications. OHSS – ovarian hyperstimulation syndrome.

Table 1. Modified World Health Organization (mWHO) classification [15].

Risk class	Diagnosis	Maternal cardiac event rate	Care during pregnancy and location of delivery
mWHO I	Small or mild Pulmonary stenosis PFO Mitral valve prolapse Succesfully repaired simple lesions (ASD, VSD, PFO, anomalous pulmonary venous drainage)Isolated atrial or ventricular ectopic beats	2.5–5%	Local hospital
mWHO II	Unoperated ASD or VSD Repaired TOF Most arrhythmias (supraventricular arrhythmias) Turner syndrome without aortic dilatation	5.7–10.5%	Local hospital
mWHO II-III	Mild left ventricular impairment (EF > 45%) Hypertrophic cardiomyopathy Native or tissue valve disease not considered mWHO I or IV Marfan or other HTAD syndrome without aortic dilatation BAV pathology with aorta <45 mm Repaired coarctation AVSD	10–19%	Referral hospital
mWHO III	Moderate left ventricular impairment (EF 30–45%) Previous peripartum CMP without any residual left ventricular impairment Mechanical valve Systemic right ventricle with good or mildly decreased ventricular function Fontan circulation without complications Unrepaired cyanotic heart disease Other complex heart disease Moderate mitral stenosis Severe asymptomatic aortic stenosis Moderate aortic dilatation (40-45 mm in Marfan syndrome or other HTAD; 45-50 mm in BAV, Turner syndrome ASI 20-25 mm/m^2, TOF <50 mm) Ventricular tachycardia	19–27%	Expert center
mWHO IV	Pulmonary arterial hypertension Severe systemic ventricular dysfunction (EF < 30% or NYHA class III-IV) Previous peripartum CMP with any residual left ventricular impairment Severe mitral stenosis Severe symptomatic aortic stenosis Systemic right ventricle with moderate or severely decreased ventricular function Severe aortic dilatation (>45 mm in Marfan syndrome or other HTAD, >50 mm in BAV, Turner syndrome ASI >25 mm/m^2, TOF >50 mm) Vascular Ehlers-Danlos Severe (re)coarctation Fontan circulation with any complication	40–100%	Expert center

ASD indicates atrial septal defect; AVSD – atrioventricular septal defect; BAV – bicuspid aortic valve; CMP – cardiomyopathy; EF – ejection fraction; PFO – patent foramen ovale; HTAD – hereditary thoracic aortic disease; NYHA – New York Heart Association; TOF – tetralogy of Fallot; VSD – ventricular septal defect.

Figure 4. Changes in estrogen level and cardiac output before and during ART, and during pregnancy. Modified from Manau et al [53].

Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018;39(34):3165–3241. doi: 10.1093/eurheartj/ehy340

 PubMed Web of Science ®Google Scholar

Manau D, Balasch J, Arroyo V, et al. Circulatory dysfunction in asymptomatic in vitro fertilization patients. Relationship with hyperestrogenemia and activity of endogenous vasodilators. J Clin Endocrinol Metab. 1998;83(5):1489–1493. doi: 10.1210/jc.83.5.1489

 PubMed Web of Science ®Google Scholar