Cardiac disease in pregnancy and congenital heart disease

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Cardiac disease in pregnancy Microchapters




Epidemiology and Demographics

Risk Factors


History and Symptoms

Physical Examination


Exercise Testing

Radiation Exposure

Chest X Ray





Pulmonary artery catheterization
Cardiac catheterization
Cardiac Ablation


Cardiovascular Drugs in Pregnancy

Labor and delivery

Resuscitation in Late Pregnancy

Contraindications to pregnancy

Special Scenarios:

I. Pre-existing Cardiac Disease:
Congenital Heart Disease
Repaired Congenital Heart Disease
Pulmonary Hypertension
Rheumatic Heart Disease
Connective Tissue Disorders
II. Valvular Heart Disease:
Mitral Stenosis
Mitral Regurgitation
Aortic Insufficiency
Aortic Stenosis
Mechanical Prosthetic Valves
Tissue Prosthetic Valves
III. Cardiomyopathy:
Dilated Cardiomyopathy
Hypertrophic Cardiomyopathy
Peripartum Cardiomyopathy
IV. Cardiac diseases that may develop During Pregnancy:
Acute Myocardial Infarction

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Anjan K. Chakrabarti, M.D. [2]; Cafer Zorkun, M.D., Ph.D. [3]


The most common maternal cardiac disease in Western societies is now congenital heart disease. This includes a wide spectrum of abnormalities, which are delineated in this section. This is largely due in part to the fact that children born with congenital heart defects in Western societies are living to the ages at which they can bear children. This section will not detail those patients that have repaired congenital defects, which are highlighted in the Repaired Congenital Heart Disease section.[1]

Congenital Heart Disease


Can classify lesions into 3 classes:

I. Volume Overload (L → R shunt)

  • Maternal and fetal mortality ≥50%
  • Consider termination if detected early
  • Careful medical management
-Supplemental O2 during pregnancy
-Hospitalization at 20 weeks gestation
-Prompt treatment of CHF
-Avoid shifts in preload/afterload

II. Pressure Overload

  • Degree of obstruction determines outcome
  • Gradient >80 mm Hg mandates correction
  • Accounts for 9% of all congenital disease in adults
  • Class I or II patients usually do well
  • Overall 3.5% mortality in unoperated patients (aortic dissection/rupture, CVA, CHF, endocarditis)
  • HTN needs careful management
  • Avoid Valsalva
  • Encourage left lateral decubitus position
  • Maximum risk period during delivery when blood loss can result in increased gradient + systemic hypotension

III. Cyanotic Heart Disease (R → L shunt)

  • Poor prognosticators:[2]
  • O2 sat<85%
  • Livebirth 12% vs. 92% is sat >90%
  • Systemic right ventricular pressures

Atrial Septal Defects

Pregnancy causes an increase in cardiac output and stroke volume. This can cause an increased left-to-right shunting of blood. Despite the increased workload on heart, females with isolated asymptomatic atrial septal defects tolerate the pregnancy well. Pregnant females with an atrial septal defect may have increased frequencies of some complications for instance arrhythmias, thromboembolism, and bleeding. Despite this, there are no studies suggesting that there are different indications for ASD closure in pregnant females with atrial septal defect when compared to a non-pregnant female with atrial septal defect. These patients are at risk from a paradoxical embolism, and early ambulation is warranted to minimize venous clot formation. The ACC/AHA guidelines, however, do dictate clear deviations in course of treatment in certain special circumstances. According to ACC/AHA guidelines pregnancy could be harmful in females with atrial septal defect and severe pulmonary hypertension (Eisenmenger syndrome)

For further information on ASD, click here

Ventricular Septal Defects

Congential ventricular septal defects VSDs are collectively the most common congenital heart defect. The incidence of VSD in adulthood has decreased over past decades due to successful surgical closure of large defects.[3] Pregnancy in patients with VSD and severe pulmonary arterial hypertension (PAH) (Eisenmenger syndrome) is not recommended owing to excessive maternal and fetal mortality and should be strongly discouraged. See ACC/AHA guidelines for details.

For further information on VSD, click here

Patent Ductus Arteriosus

Generally small defects do not cause significant hemodynamic issues during pregnancy. The exception, as in other cases, is with those with pulmonary hypertension. PDAs with a very large shunt may also lead to ventricular failure in the setting of the hemodynamic changes of pregnancy.

For further information on PDA, click here

Aortic Coarctation

Aortic coarctation occurs in approximately 6-10% of all congenital heart disease cases. It often presents in pregnancy due to systemic hypertension. This can lead to small-for-dates babies and fetal loss. Most women have a successful pregnancy with coarctation but may require imaging of the aorta during prenatal care. Treatment options include medical, surgical, and catheter-based therapies.

For further information on aortic coarctation, click here

Pulmonary Stenosis

Generally congenital pulmonary stenosis is well tolerated if it is mild or repaired by surgery or valvuloplasty. It should be noted that although a woman with severe pulmonic stenosis may be asymptomatic prior to pregnancy, the hemodynamics of pregnancy can lead to right heart failure or atrial arrhythmias. Balloon valvuloplasty is feasible even during pregnancy in the setting of symptoms.[4]

For further information on PS, click here

Congenital Aortic Stenosis

Generally aortic stenosis in women of childbearing age will be secondary to a bicuspid aortic valve. Whether or not pregnancy is contraindicated depends on the severity of aortic stenosis. An echocardiogram should be performed to determine severity; mild aortic stenosis is usually well tolerated in pregnancy, however moderate and severe aortic stenosis may not be well-tolerated. Moderate stenosis may be tolerated in a compliant patient who is monitored closely. In addition, aortic root dilation > 4.5 cm is a contraindication to pregnancy.

Balloon valvuloplasty has been safely performed in a small subset of pregnancy patients with some success, as described by Myerson et al.[5]

For further information on AS, click here

Ebstein Anomaly

Ebstein's anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart (congenital apical displacement of the tricuspid valve that typically causes significant tricuspid regurgitation).

The presence of atrial communication and maternal cyanosis greatly increases the risk of pregnancy. In addition, increased right ventricular size, a high degree of tricuspid regurgitation, and atrial arrhythmias all contribute to increased risk during pregnancy. Therefore, safety in pregnancy is quite variable and patients should be followed very closely.

For further information on Ebstein Anomaly, click here


  1. Whittemore R, Hobbins JC, Engle MA (1982). "Pregnancy and its outcome in women with and without surgical treatment of congenital heart disease". Am J Cardiol. 50 (3): 641–51. PMID 7113941.
  2. Presbitero P. et al. Circulation 1994;89:2673-6.
  3. Allwork SP, Anderson RH. Developmental anatomy of the membranous part of the ventricular septum in the human heart. Br Heart J 1979; 41:275-280
  4. Siu SC, Colman JM (2001). "Heart disease and pregnancy". Heart. 85 (6): 710–5. PMC 1729784. PMID 11359761.
  5. Myerson SG, Mitchell AR, Ormerod OJ, Banning AP (2005). "What is the role of balloon dilatation for severe aortic stenosis during pregnancy?". J Heart Valve Dis. 14 (2): 147–50. PMID 15792172.

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