9/2/2015

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Clyde Meckel MD, FACC, FSCAI

Bryan Heart

9-5-15

Pregnancy and Heart Disease

Objectives •To recognize the physiological changes

that occur during pregnancy

•To identify risk factors for adverse

maternal and fetal outcomes

•To recognize that cardiovascular risks

extend into the postpartum period

•To manage cardiac conditions during

pregnancy

A 25 year old asymptomatic woman is referred for

evaluation of a murmur noted during the early second

trimester of pregnancy. She has no history of cardiac

disease. Physical examination confirms a pulse of 95

beats per minute, blood pressure of 110/85 mmHg, a II/VI

systolic ejection murmur at the left sternal border and an

apical S3. What is the most likely cause of this murmur?

(A) M

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ate ao

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is

(B) T

ricu

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itat

ion

(C) S

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urm

ur

(E) Bicusp

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0% 0% 0%0%0%

A. (A) Moderate aortic stenosis

B. (B) Tricuspid regurgitation

C. (C) Severe pulmonary stenosis

D. (D) Physiologic murmur

E. (E) Bicuspid aortic valve with

severe aortic stenosis

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Cardiovascular Changes in

Pregnancy

CV Exam During Pregnancy

NORMAL

•Brisk carotid upstrokes

•Mildly elevated JVP

•Systolic ejection murmur at LSB

•Mammary souffle

•S3 gallop

•Mild pedal edema

•Varicose veins

NOT NORMAL

•Lung rales

•Diastolic murmur

•Holosystolic murmur

•Fixed split S2 •S4

Changes in Existing Murmurs•Stenotic lesions will get louder due to increased

preload and cardiac output

•Regurgitant lesions will get softer due to

decreased systemic vascular resistance

•A murmur from a ventricular septal defect also

gets softer due to decreased SVR

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Hemodynamic Changes Labor &

Delivery Labor:

↑ Cardiac output

↑ Heart rate

↑ Blood pressure

↑ Venous return

↑ Circulating blood volume

with uterine contraction

Post-partum:

Autotransfusion from placenta

↑ preload and CO

Cardiac Output (L/min) Hunter S. Br Heart J 1992

Pregnancy is a Hypercoaguable

State •Many hematological changes occur

↑ Clotting factors (VII, VIII, X, vWF)

↑ Platelet adhesion

↑ PAI-1, PAI-2 (produced by placenta)

↓ Fibrinolysis (due to ↑ fibrinogen)

↓ protein S activity

Brenner B. Thrombosis Research 2004

James A. Hematology 2009

Prepregnancy Risk Assessment

Cardiovascular disease does not preclude pregnancy,

but it poses increased risk to mother and fetus

• 37% of women denied having been told they were at

increased risk of complications

• Only 50% had received contraceptive counseling

Kovacs A. J Am Coll Cardiol 2008

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Why Risk Stratification is

Important

•Increased morbidity and mortality associated with

pregnancy

•Up to 20% maternal cardiac complications in women

with congenital heart disease

•Rates of hypertensive syndromes, such as

preeclampsia, are increasing

•Deaths attributable to maternal cardiac conditions have

increased in the past decade

Berg C. J. Obstet Gynecol 2009

Maternal Cardiovascular Risk

The 8th Report of Confidential Inquiries into Maternal Deaths in the UK

Greutmann M. European Heart Journal 2012

An 22 yr old woman is referred to clinic to discuss the

option of future conception. She has a history of “heart

disease” which was surgically repaired in infancy. In

performing your evaluation, which of the following factors

poses the highest risk of maternal cardiac complications

associated with pregnancy?

A. Restrictive ventricular septal

defect

B. Left ventricular ejection

fraction of 30%

C. Moderate aortic regurgitation

w/ normal systolic function

D. Palpitations with occasional

ventricular couplets

E. Bicuspid aortic valve with

aortic stenosis and a peak

F. LVOT gradient of 25 mmHg Restrict

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Maternal Cardiac Risk Factors

• Prior cardiac event

• NYHA Class >II or cyanosis

• Left heart obstruction

• Left ventricular dysfunction

Mnemonic15 (Aortic valve area <1.5 cm2) 20 (Mitral valve area <2.0 cm2) 30 (LVOT peak grad >30 mm Hg) 40 (LVEF <40%)

Siu S. Circulation 2001

Late Cardiovascular Events

NYHA class or cyanosis

Cardiac event during pregnancy

Subaortic ventricular dysfunction

Subpulmonary ventricular dysfunction

Left heart obstruction

Balint O. Heart 2010

High Risk Patients

WHO III• Mechanical valve

• Systemic right ventricle

• Fontan circulation

• Other complex congenital heart disease

• Aortic dilation 40-45 mm in Marfan syndrome

• Aortic dilation 45-50 mm in aortic disease

associated with bicuspid aortic valve

• Pulmonary arterial hypertension of any

cause

• Severe systemic ventricular dysfunction

(NYHA III-IV)

• Previous peripartum cardiomyopathy

with any residual impairment of left

ventricular function

• Severe mitral stenosis, severe

symptomatic aortic stenosis

• Marfan syndrome with aorta dilated

>45 mm

• Aortic dilation >50 mm in aortic disease

associated with bicuspid aortic valve

• Native severe coarctation

WHO IV

(pregnancy contraindicated)

Significantly increased risk of maternal

mortality or severe morbidity

Regitz-Zagrosek V. European Heart J 2011

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A 35 yr old woman with a history of peripartum

cardiomyopathy returns 18 months following the birth of

her child. She desires a second pregnancy. She is

asymptomatic, on no medications and her

echocardiogram reveals a left ventricular ejection fraction

of 30%. What is your recommendation?

A. (A) Proceed with pregnancyB. (B) Start an ace-inhibitor and

proceed with pregnancyC. (C) Obtain a cardiac MRI and if her

EF is improved, proceed with pregnancy

D. (D) Proceed with pregnancy with echos every trimester

E. (E) Counsel against pregnancy

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Peripartum Cardiomyopathy

• Onset of heart failure in the last month of pregnancy through

5 months postpartum

• No other etiology identified

• No prior history of heart disease

• Demonstrable impairment in left ventricular systolic function:

-Echocardiogram: LVEF <45%, SF <30%

Demakis J. Circulation 1971

Pearson G. JAMA 2000

Major cause of pregnancy related deaths in

the United States

Pathogenesis of PPCM

Silwa K. Lancet 2006

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PPCM: Incidence

• Not well established

• Reports range from 1 in 1,485 to 1 in 15,000

• Although certain geographic locations have reported a

higher incidence 1 in 1,000 (Africa)

• It is estimated that there are between 1,000 and 1,300

cases in the US annually

Pearson G. JAMA 2000

PPCM: Presentation

•Typical symptoms and signs of CHF

-Shortness of breath

-Fatigue

-Chest pain

-Palpitations

-Weight gain, peripheral edema

•Findings are often masked by pregnancy

•Often first diagnosed in the post-partum period

PPCM: Prognosis

Elkayam U. J Am Coll Cardiol 2011

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Risk Factors and Management

Homans D. NEJM 1985

Elkayam U. Circulation 2005

Elkayam U. J Am Coll Cardiol 2011

Risk factors:

–Multiple gestation

–Advanced maternal age

–Pregnancy induced HTN

–Tocolytic therapy

Management:

–CHF management

–Consider anticoagulation due

to ↑ risk of thrombosis

–Deliver baby as soon as fetal

lungs are mature

Onset of PPCM:

PPCM: Recovery of LV Function

Fett J. Mayo Clin Proc 2005 Elkayam U. J Am Coll Cardiol 2011

•It is difficult to predict who will have recovery of function

•Subsequent pregnancies are associated with significant

morbidities

A 22 year old woman who is 25 weeks pregnant presents

with dizziness. On evaluation, her pulse is 150 and blood

pressure is 80/50 and an electrocardiogram confirms atrial

flutter. What is your initial management?

A. (A) Intravenous adenosine

B. (B) Intravenous beta-blocker

C. (C) Oral calcium channel

blocker

D. (D) Intravenous amiodarone

E. (E) DC cardioversion

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Arrhythmias

• Incidence of both atrial and

ventricular arrhythmias increase

during pregnancy

• Physiological changes in pregnancy

alter the absorption, excretion and

plasma concentration of

antiarrhythmic drugs

• All antiarrhythmic drugs are

myocardial depressants

-Use the lowest effective doses

• DC cardioversion is safe

Hypertension in Pregnancy

• Definition: SBP ≥140 mmHg or DBP ≥90 mmHg

• ~5% of women have pre-existing HTN

• ~10% develop HTN after 20 weeks gestation

• Common therapies: labetalol, methyldopa, nifedipine

-Hospitalization: SBP ≥170mmHg or DBP≥ 110mmHg

Women with hypertension during pregnancy are at increased risk for long-term vascular events

Classification of HTN• Pre-existing HTN (≈5%)

-Prior to 20 weeks gestation

• Pregnancy induced HTN (≈10%)

-Increase in systolic (≥30 mmHg) and diastolic (≥15 mmHg)

-After 20 weeks gestation & resolution by 6 weeks

postpartum

• HELLP syndrome

-Hemolytic anemia, elevated liver enzymes, low platelets

-10-20% of women with preeclampsia

-After 20 weeks gestation & resolution by 6 weeks

postpartum

• Eclampsia

-Tonic clonic seizures Seely E. N Engl J Med 2011

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Preeclampsia

• A systemic vascular disorder

• New onset of hypertension and

proteinuria during the 2nd half of

pregnancy

• Occurs in 3-5% of pregnancies

• Sustained systolic or diastolic

blood pressure ≥140 or ≥90

mmHg, respectively, with

concurrent proteinuria ≥ 0.3 grams

in 24 hrs

Cardiac Output (L/min)

Powe C. Circulation 2011

A 21 year old woman with Marfan syndrome and an aortic

root of 4.8 cm presents in the 2nd trimester of pregnancy.

You counsel her that:

A. (A) She should start a beta-blocker, be followed closely for strict BP control and monthly echocardiograms

B. (B) She is not at an increased risk for aortic complications following pregnancy

C. (C) Future pregnancies should be avoided due to the risk of aortic dissection

D. (D) An ace-inhibitor should be started immediately, as she is beyond the 1st trimester

E. (E) The chance that her child will have Marfan syndrome is <50%

(A) S

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Connective Tissue Disorders

Pregnancy increases the risk of long-term aortic complications in women

with Marfan syndrome

Class Ia

•Counsel about the risk of aortic dissection and the heritable nature of the

condition

•Strict blood pressure control

•Monthly/bimonthly echo measurements of the ascending aorta

Class IIa

•It is reasonable to replace the aortic root and ascending aorta if the

diameter >4.0 cm in Marfan syndrome

•Fetal delivery via cesarean section is reasonable for patients with

significant aortic enlargement, dissection, or severe aortic valve

regurgitation

Hiratzka L. J Am Coll Cardiol 2010

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Valvular Heart Disease

• Stenotic lesions are not well tolerated

• Mitral stenosis: ↑ heart rate and ↓ diastolic filling results

in ↑ LA pressure and pulmonary edema

• Management: maintain sinus rhythm, beta-blockers,

diuretics, invasive strategies only if severe compromise

• Prosthetic heart valves: specific challenges

No pregnancy in a woman with a mechanical valve is safe

•Warfarin is recommended for all pregnant patients with a mechanical valve

in the 2nd and 3rd trimesters

•Warfarin should be discontinued with initiation of UFH before a planned

vaginal delivery

•Low dose aspirin (75-100 mg/day) is recommended in the 2nd and 3rd

trimesters for pregnant women with both mechanical valve or bioprosthesis

•Continuation of warfarin during the 1st trimester of pregnancy is reasonable if

the dose is <5mg/day

Nishimura R. Circulation 2014

Class IA

Class IIA

Endocarditis in Pregnancy

• Rare, yet life-threatening

• High maternal mortality rate, between 11 and 33%

• Death due to emboli, heart failure

• Highest maternal mortality for aortic valves

• Rheumatic heart disease cases declining, IVDA cases

increasing

• High fetal mortality, between 15 and 33%

• Most common species: Streptococcus

Campuzano K Arch Gynecol Obstet 2003

Kebed Y Mayo Clinic Proc 2014

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Question 6A 43 yr old woman who is 30 weeks pregnant presents

with chest pressure and shortness of breath. She is a

smoker, and is on medical therapy for HTN and

diabetes. Her electrocardiogram is shown here:

A 43 yr old woman who is 30 weeks pregnant presents

with chest pressure and shortness of breath. She is a

smoker, and is on medical therapy for HTN and diabetes

What is your initial management?

A. (A) Serial troponins and observation

B. (B) Coronary angiography

C. (C) Thrombolysis D. (D) Cardiac MRI with

viability E. (E) Start aspirin and

deliver the baby (A

) Ser

ial t

roponin

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d obs.

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(B) C

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(C) T

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(E) S

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0% 0% 0%0%0%

Acute Myocardial Infarction

• Acute MI in pregnancy in rare

• Must rule out coronary artery dissection

• Case fatality rate is between 5 and 37%

• Risk factors include:

- Hypertension - Diabetes

- Age > 30 yrs - Smoking

- Thrombophilia - Tranfusions

- Postpartum infection

• Occurrence up to 6 weeks postpartum

James A. Circulation 2006

Ladner H. Obstet Gynecol 2005

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Cardiopulmonary bypass during

Pregnancy

• Results in utero-placental hypoperfusion

• Maternal outcomes are similar to cardiac surgery in non-

pregnant women

• Poor fetal outcomes (up to 33% mortality): fetal mortality

improved if CPB is delayed

• Perfusion strategy to ensure adequate placental

homeostasis includes high-flow, high-pressure,

normothermia and brief CPB time

Kapoor MC. Ann of Cardiac Anesthesia 2014

John AS. et al. Ann Thorac Surg 2011

Resuscitation Guidelines:

Cardiac Arrest

• Place the patient in the full left-lateral position to relieve

possible compression of the inferior vena cava

• Give 100% oxygen

• Establish IV access above the diaphragm

• Assess for hypotension

• Consider & treat reversible causes

• Emergency cesarean section may be considered at 4

minutes after onset of maternal cardiac arrest if there is no

return of spontaneous circulation

(Class IIb, LOE C)

AHA Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care

Vanden Hoek T. Circulation 2010

It’s Not Just the Mother…

• Adverse Obstetric Outcomes -Higher rates of premature rupture of membranes, post-

partum hemorrhage

• Adverse Neonatal Outcomes -Higher rates of preterm birth, small for gestational age

neonates, respiratory distress, intraventricular hemorrhage

and death

-Congenital heart disease in the offspring

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Summary• Physiologic changes are dramatic during pregnancy and

delivery

• Established maternal cardiac risk factors include:

-Prior event, poor functional capacity, cyanosis

-Elevated pulmonary vascular resistance, connective tissue

disorders

Mnemonic

15 (Aortic valve area < 1.5 cm2)

20 (Mitral valve area < 2.0 cm2)

30 (LVOT peak gradient > 30 mm Hg)

40 (Systemic ventricular EF < 40%)

• These risks extend into the postpartum period

• Women with cardiac events in pregnancy have a higher risk

of cardiac complications later in life

ReferencesHunter S, Robson, SC. Adaptation of the maternal heart in pregnancy. Br Heart J. 1992;68:540-543.

Brenner B. Haemostatic changes in pregnancy. Thrombosis Research 2004; 114: 409-414.

James AH. Pregnancy-associated thrombosis. Hematology AM Soc Hematol Educ Program. 2009; 277-285.

Kovacs A. et al. Pregnancy and contraception in congenital heart disease: what women are not told. J Am

Coll Cardiol 2008; 52(7):577-578.

Berg CJ et al. Overview of maternal morbidity during hospitalization for labor and delivery in the United

States: 1993-1997 and 2001-2005. Obstet Gynecol 2009; 113(5): 1075-1081.

Greutmann M. et al. The ROPAC registry: a multicentre collaboration on pregnancy outcomes in women with

heart disease. European Heart Journal 2012: doi:10.1093.

Siu S et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation

2001; 104(5): 515-521.

References

Balint OH et al. Cardiac outcomes after pregnancy in women with congenital heart disease. Heart

2010;96(15):1223-1226.

Regitz-Zagrosek V. et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy:

the Task Force on the Management of Cardiovascular Disease during Pregnancy of the European Society of

Cardiology. European Heart Journal 2011; 32(24): 3147-3197.

Demakis J, Rahmimtoola SH. Peripartum Cardiomyopathy. Circulation 1971; 44(5): 964-968.

Pearson, GD et al. Peripartum cardiomyopathy: National Heart Lung and Blood Institute and Office of Rare

Diseases (National Institutes of Health) workshop recommendations and review. JAMA 2000; 283(9): 1183-

1188.

Silwa K. et al. Peripartum cardiomyopathy. Lancet 2006; 368(9536): 687-693.

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ReferencesElkayam U. Clinical characteristics of peripartum cardiomyopathy in the United States: diagnosis, prognosis,

and management. J Am Coll Cardiol 2011; 58(7): 659-670

Elkayam U. et al. Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between

early and late presentation. Circulation 2005; 111(16): 2050-2055.

Homans D. et al. Peripartum cardiomyopathy. N Engl J Med 1985; 312(22): 1432-1437.

Fett J. et al. Five-year prospective study of the incidence and prognosis of peripartum cardiomyopathy at a

single institution. Mayo Clin Proc 2005; 80(12):1602-1606

Seely EW, Ecker J. Clinical practice: Chronic hypertension in pregnancy. N Engl J Med 2011; 365(17): 439-

446.

Powe CE et al. Preeclampsia, a disease of the maternal endothelium: the role of antiangiogenic factors and

implications for later cardiovascular disease. Circulation 2011. 123(24):2856-2869.

Hiratzka L. et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis

and management of patients with thoracic aortic disease. A Report of the American College of Cardiology

Foundation/American Heart Association Task Force on Practice Guidelines, American Association for

Thoracic Surgery, American College of Radiology,American Stroke Association, Society of Cardiovascular

Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional

Radiology, Society of Thoracic Surgeons,and Society for Vascular Medicine. J Am Coll Cardiol

2010;55(14):e27-e129.

References

Nishimura R. et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A

Report of the American College of Cardiology/American Heart Association Task

Force on Practice Guidelines. Circulation. 2014 Jun 10;129(23):e521-643.

Campuzano K. Bacterial endocarditis complicating pregnancy: case report and systematic review of the

literature. Arch Gynecol Obstet. 2003 Oct;268(4):251-5.

Kebed Y. Pregnancy and Postpartum Infective Endocarditis: A Systemic Review. Mayo Clinic Proc. 2014:

89(8): 1143-1152.

James A. et al. Acute myocardial infarction in pregnancy: A United States population-based study. Circulation

2006; 113: 1564-1571.

Ladner HE. et al. Acute myocardial infarction in pregnancy and the puerperium: a population-based study.

Obstet Gynecol 2005;105(3):480-4.

Kapoor MC. et al. Cardiopulmonary Bypass in Pregnancy. Ann of Cardiac Anesthesia. 2014; 17:33-39.

John A. et al. Cardiopulmonary Bypass During Pregnancy. The Annals of Thoracic Surgery 2011; 91 (4):

1191-1196.

References

Vanden Hoek et al. Part 12: Cardiac Arrest in Special Situations: 2010 American Heart Association Guidelines

for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation 2010; 122(suppl

3):S829-S861.

*Donnelly et al. The immediate and long-term impact of pregnancy on aortic growth rate and mortality in

women with Marfan syndrome. J Am Coll Cardiol 2012;60:224-229.

*Hilfinker-Kleiner D et al. A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum

cardiomyopathy. Cell 2007;128(3): 589-600.

*Stergiopoulos K et al. Pregnancy in patients with pre-existing cardiomyopathies. J Am Coll Cardiol 2011;

58(4):337-350.

*Khairy P et al. Pregnancy outcomes in women with congenital heart disease. Circulation 2006; 113 (4):517-

524.

*Roos-Heselink JW et al. Outcome of pregnancy in patients with structural or ischaemicheart disease : Result

of a registry of the European Society of Cardiology. Eur Heart J. epub 2012.

*Denotes articles not included in presentation, but contains helpful information.