How should a woman of reproductive age with rheumatic heart disease and a history of three or more consecutive miscarriages be evaluated and managed before and during pregnancy?

Management of Recurrent Pregnancy Loss in Women with Rheumatic Heart Disease

Women with rheumatic heart disease and recurrent pregnancy loss (≥3 consecutive miscarriages) require urgent evaluation for antiphospholipid syndrome and other autoimmune conditions before attempting another pregnancy, followed by cardiac risk stratification and optimization to achieve both disease quiescence and hemodynamic stability prior to conception. 1, 2, 3

Immediate Evaluation for Recurrent Pregnancy Loss

Autoimmune and Thrombophilia Workup

• Obtain a comprehensive antiphospholipid antibody panel immediately: anticardiolipin antibodies (IgG and IgM), anti-β2-glycoprotein I antibodies, and lupus anticoagulant testing, as recurrent pregnancy loss is a defining criterion for obstetric antiphospholipid syndrome 1, 3
• Screen for systemic lupus erythematosus and other connective tissue diseases: anti-dsDNA, anti-Sm, anti-Ro/SSA, and anti-La/SSB antibodies, particularly given the association between rheumatic fever and autoimmune conditions 3
• Repeat positive antiphospholipid antibodies at 12 weeks to confirm persistence, as transient positivity does not meet diagnostic criteria for antiphospholipid syndrome 1

Additional Baseline Assessment

• Complete blood count, comprehensive metabolic panel, liver function tests, and 24-hour urine protein or protein/creatinine ratio to establish baseline organ function and differentiate potential lupus nephritis from other renal pathology 3
• Thyroid function testing and assessment for other endocrine causes of recurrent pregnancy loss using general medical knowledge

Cardiac Risk Stratification Before Conception

Echocardiographic Assessment

• Perform detailed echocardiography to assess mitral valve area, transvalvular gradient, pulmonary artery pressure, and left ventricular function, as these parameters determine pregnancy risk stratification 2, 4
• Pregnancy may be contraindicated if severe pulmonary hypertension (≥80 mmHg systolic), severe symptomatic mitral stenosis (valve area <1.0 cm²), or NYHA class III-IV symptoms are present, given maternal mortality rates of 17-33% in severe pulmonary hypertension 1, 2

Functional Status Documentation

• Document current NYHA functional class, as prepregnancy NYHA class >II is the strongest independent predictor of maternal cardiac events during pregnancy 2, 5
• Assess for history of heart failure, arrhythmias, or prior cardiac interventions, which identify higher-risk patients requiring tertiary center management 5, 6

Preconception Optimization Strategy

Cardiac Optimization

• Initiate beta-blocker therapy (metoprolol or atenolol) if mitral stenosis is present to control heart rate and optimize diastolic filling time across the stenotic valve 2, 4
• Consider percutaneous balloon mitral valvuloplasty before conception for moderate-severe mitral stenosis (valve area ≤1.5 cm²) to reduce pregnancy complications, as this is strongly recommended by the European Society of Cardiology 2
• Achieve stable NYHA class I-II status for at least 6 months before attempting conception, as entering pregnancy with quiescent cardiac disease dramatically improves maternal and fetal outcomes 1, 2

Anticoagulation Planning for Antiphospholipid Syndrome

• If antiphospholipid syndrome is confirmed (positive antibodies plus ≥3 consecutive losses <10 weeks or fetal loss ≥10 weeks), plan for prophylactic low molecular weight heparin plus low-dose aspirin throughout pregnancy, as this is the standard treatment for obstetric antiphospholipid syndrome 1
• If mechanical heart valve is present, anticoagulation management becomes extremely complex: vitamin K antagonist therapy is associated with unsuccessful pregnancy outcomes but may be necessary for valve thrombosis prevention 7

Medication Reconciliation

• Discontinue ACE inhibitors and angiotensin receptor blockers immediately if prescribed, as these are absolutely contraindicated throughout pregnancy due to fetal renal toxicity and teratogenicity 2
• If systemic lupus erythematosus or other autoimmune disease is diagnosed, strongly recommend initiating or continuing hydroxychloroquine, as it is safe throughout pregnancy and reduces maternal and fetal complications including preeclampsia 1, 3, 8

Multidisciplinary Team Assembly

Essential Specialist Involvement

• Establish care with a cardio-obstetrics team at a tertiary center including cardiologists, maternal-fetal medicine specialists, obstetric anesthesiologists, and neonatologists before conception 2, 4
• Obtain rheumatology consultation within 24-48 hours if autoimmune disease is suspected or confirmed, as the American College of Rheumatology emphasizes that early co-management is essential for distinguishing pregnancy complications from active rheumatic disease 1, 3
• Coordinate with hematology if antiphospholipid syndrome is confirmed for anticoagulation management throughout pregnancy

Pregnancy Management Protocol (Once Conception Occurs)

High-Risk Period Monitoring

• Intensify surveillance between 24-32 weeks gestation when cardiac output peaks (30-50% increase), placing maximum hemodynamic stress on stenotic valves 2
• The fixed mitral valve orifice cannot accommodate increased stroke volume during pregnancy, causing sharp rises in transvalvular gradient and left atrial pressure that precipitate pulmonary edema 2

Medical Management During Pregnancy

• Continue beta-blockers throughout pregnancy as first-line therapy for mitral stenosis 2, 4
• Use diuretics cautiously for pulmonary congestion, avoiding excessive volume depletion that compromises uteroplacental perfusion 2
• Continue therapeutic anticoagulation with low molecular weight heparin if antiphospholipid syndrome is present, switching from warfarin in the first trimester and near delivery 1, 7

Delivery Planning

• Vaginal delivery is preferred for most women with compensated rheumatic heart disease, including those with mitral stenosis 2, 4
• Epidural analgesia is strongly preferred as it provides pain control, reduces catecholamine surge, and stabilizes cardiac output 2
• Administer single intravenous dose of furosemide (20-40 mg) immediately after delivery to prevent fluid overload during the high-risk postpartum period 2

Critical Pitfalls to Avoid

• Do not delay antiphospholipid antibody testing: three consecutive pregnancy losses mandate immediate evaluation, as untreated antiphospholipid syndrome carries extremely high recurrence risk 1, 3
• Do not assume rheumatic heart disease alone explains the pregnancy losses: the combination of cardiac disease and recurrent miscarriage strongly suggests coexisting antiphospholipid syndrome or other autoimmune pathology requiring specific treatment 3
• Do not allow pregnancy to proceed without cardiac optimization: women with symptomatic mitral stenosis will not predictably tolerate the hemodynamic burden of pregnancy 2
• Do not miss the 24-48 hour postpartum window: this represents a second critical high-risk period with significant hemodynamic shifts requiring close monitoring 2, 4