E-Table. Specific risks of individual heart conditions in the event of pregnancy and advice on their management.

Relative risk	Lesion	Exclude before pregnancy	Potential hazards	Recommended treatment during pregnancy and peripartum
Low	Ventricular septal defects	Pulmonary arterial hypertension	Arrhythmias Endocarditis (unoperated or residual defect)	Antibiotic prophylaxis for unoperated or residual defect
	Atrial septal defects (unoperated)	Pulmonary arterial hypertension Ventricular dysfunction	Arrhythmias Thromboembolic events	Thromboprophylaxis if bed rest is required Consider low-dose aspirin during pregnancy
	Coarctation (repaired)	Recoarctation Aneurysm formation at side Associated lesion such as bicuspid aortic valve (with or without aortic regurgitation), ascending aortopathy Systemic hypertension Ventricular dysfunction	Pre-eclampsia (coarctation is the only congenital heart lesion known as an Aortic dissection Congestive heart failure Endarteritis	Beta-blockers if necessary to control systemic blood pressure of repair(MRI) independent predictor of pre-eclampsia) Consider elective caesarean section before term in case of aortic aneurysm formation or uncontrollable systemic hypertension Antibiotic prophylaxis
	Tetralogy of Fallot	Severe right ventricular outflow tract obstruction Severe pulmonary regurgiation Right ventricular dysfunction DiGeorge syndrome	Arrhythmias Right ventricular failure Endocarditis	Consider preterm delivery in the rare case of right ventricular failure Antibiotic prophylaxis
Moderate	Mitral stenosis	Severe stenosis Pulmonary venous hypertension	Atrial fibrillation Thromboembolic events Pulmonary oedema	Beta-blockers Low dose aspirin Consider bed rest during third tri-mester with appropriate thrombo-prophylaxis Antibiotic prophylaxis
	Aortic stenosis	Severe stenosis (peak pressure gradient on Doppler echocardiog-raphy > 80 mmHg, ST segment, depression, symptoms) Left ventricular dysfunction	Arrhythmias Angina Endocarditis Left ventricular failure	Bed rest during third trimester with hromboprophylaxis Consider balloon aortic valvotomy (for severe symptomatic valvar stenosis) or preterm caesarean section if cardiac decompensation ensues (bypass surgery entails a 20% risk of fetal death) Antibiotic prophylaxis
	Systemic right ventricle D-TGA after atrial switch procedure ccTGA	Ventricular dysfunction Severe systemic atrioventricular valve regurgitation Bradyarrhythmias and tachyarrhythmias) Herat Failure (NYHA>II) Obstruction of venous pathways after atrial switch as venous blood pregnancy)	Right ventricular dysfunction (potentially persisting after pregnancy) Heart failure Arrhythmias Thromboembolic events Endocarditis	Regular monitoring of heart rhythm Restore sinus rhythm in case of atrial flutter (cardioversion is usually effective and safe) Stop ACE inhibitors, consider beta-blockers Low-dose aspirin (75mg) flow significantly increases during Antibiotic prophylaxis
	Cyanotic lesions without pulmonary hypertension	Ventricular dysfunction	Hemorrhage (bleeding diathesis) Thromboembolitic events Increased cyanosis Heart failure Endocarditis	Consider bed rest and oxygen supple- mentation in order to maintain oxygen- saturation and promote oxygen-tissue delivery Thromboprophylaxis with low molecular weight heparin Antibiotic prophylaxis
	Fontan-type circulation	Ventricular dysfunction Arrhythmias Heart failure (NYHA>II)	Cardiac insufficiency Arrhythmia Thromboembolism	Consider anticoagulation with low molecular weight heparin and aspirin throughout pregnancy Endocarditis Maintain sufficient filling pressures and avoid dehydration during delivery Antibiotic prophylaxis
High	Marfan syndrome	Aortic root dilatation >4 cm	Type A dissection of aorta	Beta-blockers in all patients Elective caesarean section when aortic root is >45 mm (~ 35 gestation week)
	Eisenmenger syndrome; pulmonary hypertension	Ventricular dysfunction Arrhythmias	30–50% risk of death related to pregnancy Arrhythmia Heart failure Endocarditis for Eisenmenger syndrome	Therapeutic termination should be offered If pregnancy continues, close cardio-vascular monitoring, early bed rest pulmonary vasodilator therapy with supplemental oxygen should be con sidered Close monitoring necessary for 10 days postpartum

VSD, ventricular septum defect; ASD, atrial septum defect; D-TGA, dextro-transposition of the great arteries; ccTGA, congenitally corrected transposition of the great arteries; LMH, low molecular weight heparin; ASA, acetylsalicylic acid; RVOT, right ventricular outflow tract.

From: Uebing A, Steer PJ, Yentis SM, Gatzoulis MA: Pregnancy and congenital heart disease. BMJ 2006; 332:401-6. With kind permission of the BMJ Publishing Group Ltd.