Heart disease in pregnancy Kelly Hansul, Do metro health university of Michigan health

I have no relevant financial relationships to disclose I will not discuss any off-label use and/or investigational use in my presentation

Why can this cause heart palpitations?

Pregnancy and heart disease: on the rise! Congenital heart disease Now > 1% of pregnancies and accounts for 20% of non-obstetric maternal deaths Kuklina, BJOG, 2011

hypothetical 3 AM 19 yo immigrant – first pregnancy No prenatal care History Rheumatic heart disease Vaginal bleeding/abruption SOB Would you know what to do?

When you have cardiac disease…. Do you think heart failure? Do you think too much fluid? Do you think treatment is fluid restriction?

Pregnancy is different Rule # 1 Pregnancy is different

Normal heart physiology

Basic physiologic changes in pregnancy Increased blood volume Cardiac output fluctuations Blood volume Labor Postpartum volume shifts Decreased SVR Decreased colloid osmotic pressure

Pregnant heart considerations All is well if the heart is normal Not good if there is heart disease present Right heart disease tends to do well Left heart disease usually does not do well

Maternal mortality associated with pregnancy Group 1= mortality <1% Group 2 = mortality 5-15% ASD VSD PDA Mitral stenosis NYHA I and II Corrected Tetralogy of Fallot Bioprosthetic valve Mitral Stenosis NYHA III and IV Mitral stenosis with a fib Aortic Stenosis Coarctation of aorta without valvular involvement Uncorrected Tetralogy of Fallot Previous MI Marfan syndrome with normal aorta Mechanical heart valve

Maternal mortality associated with pregnancy Group 3 = mortality 25-50% Pulmonary HTN Coarctation of the aorta with valvular involvement Marfan syndrome with aortic involvement Peripartum cardiomyopathy with persistent left ventricular dysfunction Severe systemic ventricular dysfunction Severe left – sided obstructive lesions Modified from Foley, et all Critical Care Obstetrics, 2010

Normal changes that mimic heart disease Dyspnea Many pregnant women complain prior to 20 weeks and 75% by the third trimester To differentiate between normal physiologic change and disease process “Normal” dyspnea occurs early and does not significantly worsen Heart disease dyspnea usually worsens second half of pregnancy Physiologic dyspnea does not stop women from performing ADL’s and does not occur at rest Other symptoms that mimic heart disease Decreased exercise tolerance, fatigue, occasional orthopnea, syncope, chest discomfort

Diagnosis and evaluation of heart disease Indications for further cardiac testing History of known cardiac disease Symptoms in excess of those expected in normal pregnancy Pathologic murmur Evidence of heart failure on physical exam Arterial oxygen desaturation in the absence of known pulmonary disease Most next appropriate step Transthoracic echocardiography

Labor, delivery and postpartum period Heart rate, blood pressure and cardiac output all increase with contractions Immediately after delivery, the uterus auto transfuses During the first 2 weeks postpartum, extravascular fluid is mobilized and diuresis ensues Unsuspected cardiac disease may be diagnosed when a woman returns to the ED several days PP with dyspnea and oxygen desaturation

Atrial Septal Defect Most common repaired and unrepaired congenital lesion in pregnant women In general, it is asymptomatic even with large left to right shunts Three significant complications seen with ASD Arrhythmias Heart failure Paradoxical embolism

ASD management in labor Avoidance of fluid overload Oxygen administration Labor in the lateral recumbent position Pain relief with epidural Goal is to reduce cardiac work

Ventricular septal defect May occur as an isolated lesion or in conjunction with other congenital anomalies Size and location most important predictors during pregnancy Small are tolerated well Larger defects or those associated with other anomalies are more complicated Can lead to heart failure, congestive failure, arrhythmias or pulmonary HTN Echo or cardiac cath is essential in any adult patient in whom persistent VSD is suspected

Watch out for pulmonary edema in mitral stenosis patients, especially Rule # 2 Watch out for pulmonary edema in mitral stenosis patients, especially postpartum

Cardiac case studies: mitral stenosis 19 yo, G-1, P-0 at 31 weeks; history class II mitral stenosis; presents with chest pain and SOB VS: 104/40, 122, 28, 98.4 SaO2 94% Breath sounds – wet ABG’s 7.41, 42, 60, 19.3 ECG – sinus tach

Classification of mitral stenosis Recommendations for classification of mitral stenosis severity   Mild Moderate Severe Specific findings Valve area (cm2) >1.5 1.0-1.5 <1.0 Supportive findings Mean gradient (mmHg)a <5 5-10 >10 Pulmonary artery pressure (mmHg) <30 30-50 >50 aAt heart rates between 60 and 80 bpm and in sinus rhythm.

MS therapy

X-ray fluoroscopy during balloon mitral valvuloplasty

Intrapartum management Avoid tachycardia Decreases L-ventricular filling time B-blockers for pulse >90-100 Avoid hypervolemia, but also hypovolemia ! Keep PCWP approximately 14 Accommodate postpartum volume load May need diuretics Individualize PCWP and CO

Despite heart disease, diuresis is usually the incorrect strategy Rule #3 Despite heart disease, diuresis is usually the incorrect strategy

Cardiac case Studies Aortic stenosis: 24 yo G-2, P-1 at 36 weeks; admitted with severe chest pain, numbness radiating down left arm; and contractions Vitals signs: 160/104, 126, 38, 97.8 SaO2 99% Breath sounds clear ABG’s 7.46, 28, 140, 18.7 ECG Inverted T-waves, slightly prolonged S-T segment

Aortic stenosis Most common etiology is congenital bicuspid valve Ideally, correction of the valvular abnormality occurs prior to pregnancy Surgical valve repair Surgical valve replacement Or percutaneous balloon valvotomy If diagnosed during pregnancy, maximal medical therapy is preferred

Aortic stenosis Fixed CO in severe disease Critical stenosis (valve area , 1-1.5cm2) LVH CHF Fixed CO in severe disease May not be adequate to perfuse coronary and cerebral systems Angina, MI, syncope, death

Intrapartum management Spinal and epidural anesthesia are discouraged during labor due to vasodilatory effects Balloon valvuloplasty can be considered in severe cases that require intervention prior to delivery Avoid hypotension Decreased venous return will decrease CO Avoid hemorrhage Hypovolemia more dangerous than pulmonary edema Keep on the “wetter” side

Rule # 4 Certain patients should be advised that pregnancy is prohibitively risky

Overview 22 yo Gravida 1 Hx: congenital heart disease/single ventricle Repaired / follow up Residual problem: pulmonary hypertension

Pulmonary hypertension and pregnancy PHTN=PAP > 30mmhg Traditionally, 50% mortality Contraception and early pregnancy risk counseling remain paramount

Avoid hypotension Systemic hypotension Decreased R ventricle filling pressure Decreases pulmonary perfusion in face of PHTN Profound hypoxemia Sudden death

Marfan syndrome Autosomal dominant, variable expression Mutation of the fibrillin gene on 15q21 Generalized connective tissue weakness Can lead to aneurysms, rupture, dissection 60% also have mitral or aortic regurgitation RISK IN PREGNANCY SURROUNDS AORTIC ROOT DISSECTION OR RUPTURE

AORTIC ROOT RISK IN PREGNANCY Aortic root diameter < 4cm > 4 cm Risk of dissection or rupture 1% during pregnancy 10% during pregnancy Risk of maternal mortality <5% Up to 50%

Prophylactic B-blockade Reduces hemodynamic stress on ascending aorta Slows rate of dilation Reduces pregnancy aortic complication rate 5 fold Pacini, Int Card, 2009

Delivery Route < 4 cm root = vaginal + assisted second stage > 4 cm root = cesarean

Peripartum cardiomyopathy Cardiomyopathy in the last month of pregnancy or first 5 months postpartum with exclusion of other causes Peak incidence is within 1 month postpartum Greater risk with older gravidas, multiparas, African Americans, twins and PIH Manifests as biventricular failure and dilatation 50% will develop chronic dilated cardiomyopathy

Outcome Even today, high risk for adverse outcome despite normal EF Peripartum cardiomyopathy: prognostic factors for long term maternal outcome EF > 40% = 29% had worsening cardiac symptoms EF < 25% = 57% end stage cardiac disease Habli, AJOG 2008

Acute treatment Reduce preload Reduce afterload if hypertension Lasix 20-40mg daily Use carefully in the undelivered patient Reduce afterload if hypertension Hydralazine 25-100mg up to QID Postpartum = ACE inhibitor Improve contractility Digoxin 0.25-0.5mg daily Reduce myocardial demand and remodeling Beta blocker such as metoprolol (Lopressor) 12.5 – 100mg daily

In conclusion: a summary With respect to CHD, patients with any of the following are considered at high risk: Aortic regurgitation or mitral regurgitation with NYHA class III to IV symptoms Marfan syndrome, especially with aortic regurgitation Severe aortic stenosis with valve area< 1.5cm or gradient > 30mmgHg Severe Mitral stenosis < 2cm Any condition that results in LV dysfunction (LVEF <40%) or severe pulmonary HTN Mechanical valves that require chronic antigoagulation Poor functional class (NYA class III or IV) or cyanosis

Summary continued In comparison, patients with the following conditions are considered at low risk Asymptomatic aortic stenosis with an LVEF >50% and a mean gradient less than 25mmHg Aortic regurgitation or mitral regurgitation with no or mild symptoms (NYHA class 1 to II) Mild to moderate pulmonary valve stenosis