Adult Congenital Heart Disease. Pregnancy and Heart Disease

Adolescent/Adult Congenital Heart Disease Pregnancy and Heart Disease Schooler Family Foundation Patient Advocacy/Support Group Research/Database Te...
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Adolescent/Adult Congenital Heart Disease Pregnancy and Heart Disease

Schooler Family Foundation Patient Advocacy/Support Group Research/Database Teaching/Fellowship Program

Congenital Heart Disease and Pregnancy Curt Daniels, MD Stephen Cook, MD Lindsey Cook, MD Steve Crumb, APN Patty Woods, APN Jenne Hickey, RN Julie Mueller, RN Lindsay Arnott David Colombo, MD Funmi Ajayi, MD

Objectives Review the spectrum of and identify the complexities in CHD Discuss the rising interest in pregnancy among ACHD patients Describe cardiovascular changes associated with pregnancy Discuss the importance of PLANNING pregnancy in women with CHD

Ratio of Pediatric to Adult Patients with CHD Pediatric patients Adult patients

700,000

1965

1985

1,000,000

2005

Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707.

Adult vs Pediatric CHD Populations in USA 800,000 – 700,000 – 600,000 – 500,000 – Number 400,000 – 300,000 – 200,000 – 100,000 – 0–

787,000

Adults Children

623,000

368,000 320,000

302,500

165,000 117,000

Simple

Moderate

138,000

Complex

Total

Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707.

Patients Reaching Adulthood with CHD 1,300,000

1,300,000 – – – 1,000,000 –

Adult CHD Patients



1,000,000

– 700,000 –

750,000

– 500,000 – 500,000

– 300,000 – –

325,000

100,000 – 0–

1970

1980

1990

2000

2010

Spectrum of CHD Noncomplex/Acyanotic

Complex/Cyanotic

ASD VSD AVSD

Combination of defects TOF D-TGA Single ventricle DORV DILV

L-TGA Outflow obstruction Subvalvar Valvar Supravalvar Coarctation of the aorta

Valvular atresia TAPVR Ebstein anomaly

Eisenmenger syndrome

Complexity Arrhythmias

Heart Failure • Right Heart Failure

Adults with Congenital Heart Disease Vascular Lesions

Valvular Disease

• Left Heart Failure •Systolic •Diastolic • Pulmonary Hypertension

Residual Shunts

You are Unique! Every woman with CHD here tonight has unique cardiac structure, function and prior care To make generalizations or predictions about pregnancy in women with CHD is unwise Therefore, the risks, course, surveillance and therapy during pregnancy are unique for each woman

Successful Therapy Women and Men Surviving to Adulthood Committed Relationship Desire Children

Genetic Fitness Capable of having biological children? Is it safe for me to become pregnant?

BEFORE becoming pregnant… Discuss desire to become pregnancy with Primary or Maternal Health Care Providers Thyroid, Diabetes, Blood Pressure, Toxic Substances Discuss the use of vitamins, folic acid, and medications including over the counter medications If you take COUMADIN, this requires additional discussion If you smoke STOP! Avoid passive smoke

How does my unique form of heart disease match the changes that occur with pregnancy?

Maternal Cardiovascular Demands Blood Volume

INCREASES

Cardiac Volume

INCREASES

Heart Rate

INCREASES

Cardiac Output

INCREASES

Blood pressure

MILD Changes

Risk Stratification Remember… You are Unique!

Low

Moderate

High

Atrial septal defect

Repaired heart disease

Pulmonary hypertension

Mild/moderate valvular regurgitation

Mechanical valve replacement

Shunt Lesions with Eisenmenger syndrome

Mild/moderate pulmonary stenosis

Uncomplicated repaired coarctation of the aorta Hypertrophic cardiomyopathy

Severe heart muscle disease (Cardiomyopathy)

Mild atrial and ventricular arrhythmias

Uncorrected cyanotic congenital heart disease Aortic Aneurysm

Risk of CHD in the Baby General population

0.8%

Parent with CHD

3-6%

Mom with Cyanotic CHD

up to 16%

British Cardiac Society Working Party, Heart, 2002

Risk Factors Associated with Congenital Heart Disease Alcohol or drug abuse during pregnancy Exposure to environmental agents (pesticides, lead) Maternal viral infection, such as German measles Maternal fever early in pregnancy or around conception Maternal diabetes (not gestational diabetes) Maternal obesity Poor nutrition Chromosomal or genetic abnormalities (Down syndrome) in the child Certain medications taken during pregnancy (ACE inhibitors, Coumadin)

Importance of Planning Long Term Complications Example: Tetralogy of Fallot

“Complete Repair”

Relieve narrowed passage to lungs

Close the hole (VSD)

Pulmonary Valvular Regurgitation

Right Ventricular Enlargement Over Time

Right Ventricular Enlargement

RV

LV

Narrowing of Pulmonary Arteries

Interventional Cardiac Catheterization

Pulmonary Valve

Pulmonary Rehab/Valve Replacement RV Volume (mL)

RV Function

Before PVR

235

8%

4 Months After PVR

153

35%

“I’m Pregnant!!!” Confirm pregnancy (urine, blood, U/S) Discuss medications immediately (stop or change?) High Risk Maternal Fetal Medicine ACHD Evaluation first trimester Fetal Ultrasound ~week 20-23 (Check Baby’s heart)

Symptoms Normal Fatigue Chest Pain Mild breathlessness Rapid breathing Palpitations Fainting without activity Mild swelling in feet

Abnormal Symptoms at rest Chest pain with activity Breathlessness with activity Unable to lie without pillows Awaken short of breath Racing heart, palpitations Fainting with activity

Call Immediately if you have: Heart Failure symptoms - Shortness of breath, fatigue, swelling Cardiac Arrhythmias -Skipping, racing, lightheadedness, fainting, chest pain, shortness of breath Loss of speech, vision, ability to feel or move hands and feet, facial features uneven

Cardiac Management Diagnostic testing; echocardiogram, ECG, rhythm monitoring, cardiac MRI Medications Bedrest/Hospitalization Delivery Plan

Pregnancy among women with CHD at NCH/OSU ACHD Program 1999-2007 212 pregnancies Average age 27 92 documented vaginal deliveries 55 documented Cesarean sections Confirmed CHD in 14 offspring No maternal fatalities 7 spontaneous abortions 2 Therapeutic abortions

Pregnancies

Pregnancies in ACHD Patients since program inception 70 60 50 40 30 20 10 0 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 Year

ACHD Diagnostic Categories in pregnant Women

Single Ventricle 6 55

LVOTO

44

Connective Tissue TOF PS 8 26 14

18

20

TGA CM Other

N = no. of patients

Congenital Diagnoses of Pregnant Women in ACHD Program

Tri Atr BAV PS VSD SVT AVSD EDS PPCM MR HTN

CoA ALCAPA MS RCM ASD HCM Holt-Oran WPW PPS

marfan AS d-TGA PAH LVOTO l-TGA MVP LQTS Cong AVB

ebstein TOF DCM DORV PDA AI MV mass TR AMI

Which statement is incorrect? a- Alcohol, medications and viruses can cause congenital heart disease b- Children born to a parent with CHD have the same CHD risk as children with a parent that does not have CHD c- Women with congenital heart disease who plan pregnancy are more likely to have successful outcome (maternal and fetal) d- Heart rate, blood volume, cardiac volume and cardiac output increase during pregnancy

ANSWER All statements are correct except b. Children born to a parent with CHD have a 3-5 fold increased risk of also having CHD. This is one reason for recommending fetal ultrasound in the fetus.

What Did You Learn? The risks to Moms with CHD during pregnancy are related to increased cardiovascular demands. What Maternal organ provides nutrition and oxygen to the growing baby?

ANSWER

The Placenta

Placental Facts Some ancient cultures felt that eating the placenta was equivalent to breast feeding. Some cultures grilled and salted the organ for consumption Consumption of the placenta was recommended to decrease post-partum bleeding and depression

What Did You Learn? Planning for pregnancy allows for Moms to be in the best possible condition prior to pregnancy. Name are 2 things women with congenital heart disease should do PRIOR to becoming pregnant?

ANSWER Discuss pregnancy with Primary or Maternal Health Care provider Discuss medications (prescribed and OTC), and vitamin supplements Coumadin requires additional discussion Don’t smoke; avoid passive smoke and exposure to toxins

What Did You Learn? Many women experience symptoms during pregnancy. What are some symptoms that suggest the heart cannot keep up with increased demands of pregnancy?

ANSWER

In general, symptoms of normal pregnancy are mild, occur randomly and do not persist, whereas symptoms suggestive of cardiac deterioration are progressive and are more likely to occur with activity.

Summary „

„ „ „ „ „ „

Every women is unique; CHD diagnosis, surgery, medications, heart function, valve disease Pre-pregnancy planning Assess risk of pregnancy, delivery and postpartum Close follow up once pregnant High risk OB-Gyn Avoid high risk behaviors Success is based on teamwork; patient, family, cardiology, OB, anesthesia.

ACHD- High Risk Maternal-Fetal 1999 to 2006 101 consecutive pregnancies Maternal age 15.3-41.9 (average 26) years Maternal complications Obstetric complications Fetal/Neonatal complications Maternal interventions Medical, Catheter-based, Surgical

Population Diagnostic Category

Pregnancies =101

Mean Age

Complex CHD

*25

23.9

Noncomplex CHD

*51

25.2

Cardiomyopathy

11

21.8

Connective Tissue

*5

23.0

Rheumatic

*3

39.5

*Nine women followed during two or more pregnancies 65% underwent surgical palliation/repair prior to pregnancy

Cardiovascular Complications Aortic Neurologic Cyanosis Instability

Total

2

Complex Noncomplex

1

Connective Tissue

1

2

3

1

3

1

Cardiovascular Complications Arrhythmia Hypertension

Heart Failure

Total

4

4

13

Complex

2

1

2

Noncomplex

2

3

5

Cardiomyopathy

4

Rheumatic

2

Obstetric Complications Maternal Placental Death Abruption Total Complex Noncomplex Cardiomyopathy Connective Tissue Rheumatic

0

4 1 3

Cesarean Section *22 4 14 1 2 1

Obstetric reasons (16); Fetal distress (3); Cardiovascular (3)

Fetal/Neonatal Complications SAB TAB

Total Complex Noncomplex Cardiomyo

4 1 3

1

SB

2 2

Neonatal CHD in Neonatal Death Offspring ICU Care 0 3 1 *1 2

1

*A child with truncus arteriosus was born to a mother with PDA ligation and medically controlled hypertension

Heart Failure Therapy Diagnosis Cardiomyopathy Hypertrophic Hypertrophic Dilated Rheumatic Mitral Stenosis Mitral Stenosis

NYHA III III III IV III

Treatment Verapamil Metoprolol Hydralazine Balloon valvuloplasty Balloon valvuloplasty

Catheter-Based Therapy (n-6) Category Diagnosis

Intervention Results

Noncomplex ASD/TIAs

ASO Device

Rheumatic

Elimination of shunt

Aortic Coarctation

Balloon/Covered Elimination of Stent gradient

Pulmonary Stenosis

Balloon Valvuloplasty

Reduction in gradient

Aortic Stenosis

Balloon Valvuloplasty

Reduction in gradient

Mitral Stenosis (2)

Balloon Valvuloplasty

Reduction in gradient

Surgical Intervention Category

Diagnosis

Noncomplex

Aortic Descending Aortic Coarctation Aneurysm Resection

No complications

Aortic Stenosis

Aortic Valve Replacement

No complications

Marfan syndrome

Abdominal Aortic Resection

No maternal or fetal complications

Connective Tissue

Surgery

Outcome

Lindsey Cook, FNP, Jenne Hickey, RN Stephen Cook, MD, Curt Daniels, MD Karen Carter, Our Boss

Pregnancy and Heart Disease 20 – 15 – Patients10 –(n) 5– 0–

19

101 pregnancies in 95 women No maternal or neonatal deaths 11 10

10 8 7 6 5

5 4

4

4 3

3 2

Aortic CMP ASD Valve

COA

TOF

PS

TGA Marfan VSD

MS Ebstein DORV TA

PHT Other

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