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