Cardiovascular Disease in Pregnancy Part II: Classifications and Valvular Disease

There are multiple systems for the classification of cardiac disease in pregnancy:

  • Modified WHO Pregnancy Risk Classification - this scale is based on the presumptive risk of cardiac disease towards pregnancy outcomes.

    • Class I: no detectable increase in maternal mortality, and no or only mild increase in morbidity.

      • Ex: uncomplicated/small/mild pulmonary stenosis, PDA, mitral valve prolapse; successfully repaired simple lesions (PDA or septal defects); isolated atrial or ventricular ectopic beats

    • Class II: small increased risk in maternal mortality, moderate increase in morbidity.

      • Ex: unoperated ASD or VSD; repaired tetralogy of Fallot; most arrhythmias.

    • Class III: significantly increased risk of maternal mortality or severe morbidity. Recommended intensive specialist cardiac and obstetric monitoring throughout pregnancy, delivery, and postpartum.

      • Ex: mechanical valve, systemic right ventricle, Fontan circulation, unrepaired cyanotic heart disease, aortic root dilation 40-45mm in Marfan patient or 45-50mm in bicuspid valvular disease.

      • Some patients may fall into an in between II-III category (i.e.,m mild LV impairment, HCM, Marfan syndrome without root dilation).

    • Class IV: extremely high risk of maternal mortality or severe morbidity; pregnancy considered contraindicated with recommendation for termination if pregnancy occurs. If continues, manage with specialist involvement as per class III.

      • Ex: pulmonary arterial hypertension, severe systemic ventricular dysfunction (LVEF < 30%), previous peripartum cardiomyopathy with any residual LV dysfunction, severe mitral or aortic stenosis; aortic root dilation in Marfan syndrome > 45mm or >50mm in bicuspid disease; native severe aortic coarctation.

  • NYHA Functional Classification - likely a familiar scale, based off of cardiac disease symptoms and functional status.

    1. Class I - cardiac disease, but no symptoms and no limitations in ordinary physical activity 

    2. Class II - mild symptoms and slight limitations during ordinary activity

    3. Class III - significant limitation in activity due to symptoms. Comfortable at rest 

    4. Class IV - severe limitations. Symptoms even while at rest.  

  • CARPREG II or ZAHARA - point-based scales for risk prediction for a significant cardiac event in pregnancy:

Valvular Disease

  • Native Valvular Disease

    • Stenosis - the valve is narrowed or stiffened and does not allow for passage of blood.

      • Tricuspid stenosis - makes it hard for blood to pass from the right atrium into the right ventricle.

        • With increased cardiac output as well as increased systemic volume, stenosis of the tricuspid valve can lead to systemic overload (ie. swelling, JVD, etc); severe stenosis can make it so that less blood gets into the pulmonary system.

      • Pulmonic stenosis - blood is impaired in passage from RV to lungs.

        • In isolation, this is generally well tolerated since pregnancy provides additional volume, thus providing more blood to pump to the lungs in totality.

        • However, can be associated with other complex heart disease, in which outcomes are not as good.

      • Mitral stenosis - blood flow challenged from LA to LV.

        • Most common cause worldwide: rheumatic heart disease.

        • Increased CO and HR leads to decreased filling (diastolic) time. This promotes increased left atrial pressure and dilation, leading to atrial fibrillation, pulmonary edema.

        • Maternal mortality with severe MS is 3%.

        • Treatment in pregnancy is with beta blockers to decrease HR and increase filling time. Anticoagulation needed if atrial fibrillation develops.

      • Aortic stenosis - blood flow impaired from LV to aorta.

        • Most common cause: bicuspid aorta (congenital).

        • Pregnancy well tolerated except for patients with severe disease; need good flow systemically to accommodate increased cardiac output and stroke volume demand of pregnancy.

        • Severe AS can result in heart failure, arrhythmias, and pulmonary edema.

    • Regurgitation - insufficiency across a valve, allowing for backflow of blood.

      • Tricuspid regurgitation: overall well-tolerated, but higher risk of right-sided failure and atrial arrhythmia.

      • Pulmonic regurgitation: moderate or severe cases may lead to RV failure and arrhythmia.

      • Mitral regurgitation: usually well tolerated as long as there is absence of LV systolic dysfunction or pulmonary hypertension.

        • If severe, ideally valve is repaired or replaced prior to pregnancy.

      • Aortic regurgitation: also well-tolerated overall, unless signifiant baseline symptoms.

        • Risk of heart failure due to volume overload; can also occur alongside LV systolic dysfunction, severe LV dilation, or pulmonary hypertension.

  • Mechanical or Prosthetic Valves

    • There is an increased risk of thrombosis/VTE in pregnancy, and these patients will need to be on anticoagulation.

    • Warfarin is the preferred anticoagulant despite risk of embryopathy, as the risk of thrombus is higher on heparin or heparin-like agents.

Pearls on managing valvular disease in various stages of pregnancy:

  • Prepregnancy 

    • Depending on severity, may need have preconception counseling with cardiologist and MFM.

    • Patients with severe mitral and aortic stenosis may need balloon valvuloplasty beforehand.

  • During pregnancy 

    • Really, this is based on their cardiovascular risk score (ie. WHO classification) 

    • Most of these patients will need an echocardiogram during early pregnancy, and likely follow up at an interval depending on hemodynamic tolerance.

    • Evaluate and treat any symptoms of heart failure!

    • Labor and delivery: vaginal delivery is usually the preferred method of delivery unless: 

      • Mitral stenosis with NYHA class III/IV or have pulmonary hypertension 

      • Severe, symptomatic AS 

    • Especially with more severe symptoms, consider early epidural (especially in mitral stenosis) and assisted second stage.

    • Telemetry if risk for arrythmia exists.

  • After delivery 

    • For many of these lesions, need to reevaluate an echocardiogram.

    • Many of these patients, due to fluid shifts, are at higher risk for arrhythmias and heart failure after delivery.

    • Strict I/Os in the postpartum period!

    • Consider telemetry if risk for arrhythmia.