Cardiac disease in pregnancy and repaired 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-In-Chief: Anjan K. Chakrabarti, M.D. [2]; Assistant Editor-In Chief: Ara Tachjian, MD [3]


Despite successful repair of congenital heart disease, many patients will still have a residual defect. As a result, careful planning should be undertaken prior to pregnancy.[1] [2][3]

Repaired Congenital Heart Disease

Tetralogy of Fallot

Individuals with TOF usually present very early in life with cyanosis making it necessary to undergo surgical repair. The repair is is almost always done during infancy, particularly in developed countries. During the surgery, the pulmonary valve and right ventricular outflow tract are both surgically dilated. This usually leads to pulmonary valve regurgitation as well as arrhythmias originating from the right side of the heart. Therefore, subsequent right sided heart failure is sometimes observed. 20 years following repair of Tetralogy of Fallot, 10-15% of patients develop late complications, the most important of which is right sided heart failure. [4] [5] [6]

Some TOF patients will have a palliative surgically-created systemic-pulmonary shunt such as Blalock-Taussig or Waterston, but without corrective surgery.[4] In such situations, pregnancy might worsen an already existing right-to-left shunt (through a VSD) by reducing the systemic vascular resistance, leading to worsening cyanosis. In previous studies, maternal NYHA functional class > 2 and cyanosis are identified as independent predictors of both maternal and fetal complications. [7]Therefore, worsening cyanosis is not favored in such patients and pregnancy should be avoided.

Elements to be evaluated for through history, physical exam, EKG and echocardiogram prior to pregnancy (in women with surgical correction): 1. Residual pulmonary regurgitation and severity 2. Residual right ventricular outflow tract obstruction 3. Right sided heart failure 4. Tricuspid regurgitation 5. Atrial or ventricular arrhythmias 6. VSD

Generally, it is accepted that most women with corrected TOF can have non-restricted lifestyle and tolerate pregnancy. However, they still need to be evaluated by a cardiologist prior to and during pregnancy. Women with severe pulmonary regurgitation as a complication of the corrective surgery might benefit from pulmonary valve replacement prior to pregnancy to reduce possibility of complications. [4]


  1. Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F (1994). "Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus". Circulation. 89 (6): 2673–6. PMID 8205680.
  2. Zuber M, Gautschi N, Oechslin E, Widmer V, Kiowski W, Jenni R (1999). "Outcome of pregnancy in women with congenital shunt lesions". Heart. 81 (3): 271–5. PMC 1728980. PMID 10026351.
  3. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
  4. 4.0 4.1 4.2 Meijer JM, Pieper PG, Drenthen W, Voors AA, Roos-Hesselink JW, van Dijk AP; et al. (2005). "Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot". Heart. 91 (6): 801–5. doi:10.1136/hrt.2004.034108. PMC 1768963. PMID 15894783.
  5. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM; et al. (1993). "Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot". N Engl J Med. 329 (9): 593–9. doi:10.1056/NEJM199308263290901. PMID 7688102.
  6. Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B (1997). "Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair". J Am Coll Cardiol. 30 (5): 1374–83. PMID 9350942.
  7. Siu SC, Sermer M, Harrison DA, Grigoriadis E, Liu G, Sorensen S; et al. (1997). "Risk and predictors for pregnancy-related complications in women with heart disease". Circulation. 96 (9): 2789–94. PMID 9386139.

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