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Symptoms to Diagnosis

Pregnancy in a woman with congenitally corrected transposition of the great arteries

Sarah A. Goldstein, MD, Lisa Sorenson, CNP, Jeffrey B. Chapa, MD and Richard A. Krasuski, MD
Cleveland Clinic Journal of Medicine April 2021, 88 (4) 228-236; DOI: https://doi.org/10.3949/ccjm.88a.20136
Sarah A. Goldstein
Adult Congenital Heart Disease Fellow, Division of Cardiology, Duke University Medical Center, Durham, NC
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Lisa Sorenson
Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH
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Jeffrey B. Chapa
Section of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Cleveland Clinic, Cleveland, OH; Clinical Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH
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Richard A. Krasuski
Professor of Medicine and Pediatrics, Director of the Adult Congenital Heart Disease Center, Duke University Medical Center, Durham, NC
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    Figure 1

    (Left) Congenitally corrected transposition of the great arteries (CC-TGA). The right ventricle pumps oxygenated blood in the systemic circulation, while the left ventricle pumps deoxygenated blood in the pulmonary circulation. No intervention may be needed. (Right) Dextro- or D-TGA. Pulmonary and systemic blood flows are separate, leading to rapid clinical deterioration in the first day of life unless a communication between the circulations exists or is created.

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    Figure 2

    Electrocardiogram demonstrating complete heart block with a junctional escape rhythm in a patient with CC-TGA. The typical findings in patients with CC-TGA found here include Q waves in the inferior leads (II, III, and aVF) and right limb lead (aVR), and the absence of Q waves in V5 and V6.

    From Yoon et al, reference 9.

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    TABLE 1

    World Health Organization classification of maternal cardiovascular risk

    Class I
    Uncomplicated, small or mild pulmonary stenosis, patent ductus arteriosus, or mitral valve prolapse; successfully repaired simple lesions (atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous return)
    Cardiovascular risk: No increased risk of maternal mortality, possible mild increase in morbidity
    Class II
    Unrepaired atrial or ventricular septal defect; repaired tetralogy of Fallot; supraventricular tachycardia
    Cardiovascular risk: Small increased risk of maternal mortality, moderate increase in morbidity
    Class II–III
    Mild left ventricular dysfunction; native or tissue valvular heart disease not considered World Health Organization class I or IV; Marfan syndrome without aortic dilation; aorta less than 45 mm in association with bicuspid aortic valve; repaired coarctation of the aorta; hypertrophic cardiomyopathy
    Cardiovascular risk: As in class II or III, depending on individual
    Class III
    Mechanical valve; systemic right ventricle with good or mildly reduced function; Fontan circulation without complication; unrepaired cyanotic heart disease; other complex congenital heart disease; aortic dilation 40 to 45 mm in Marfan syndrome; aortic dilation 45 to 50 mm in bicuspid aortic valve disease; previous peripartum cardiomyopathy with complete recovery of ventricular function
    Cardiovascular risk: Significantly increased risk of maternal mortality or severe morbidity. Expert counseling required. If pregnancy continues, intensive specialist cardiac and obstetric monitoring needed throughout pregnancy, childbirth, and for 6 weeks afterwards
    Class IV
    Pulmonary arterial hypertension from any cause; severe systemic ventricular dysfunction (ejection fraction < 30%, New York Heart Association functional class III-IV); systemic right ventricle with moderate or severely reduced function; Fontan circulation with any complication; severe mitral stenosis; severe symptomatic aortic stenosis; Marfan syndrome with aortic dilation more than 45 mm; aortic dilation more than 50 mm in bicuspid aortic valve disease; native severe coarctation of the aorta; previous peripartum cardiomyopathy without complete recovery of ventricular function
    Cardiovascular risk: Pregnancy is contraindicated because of extremely high risk of maternal mortality or severe morbidity. If pregnancy occurs, discuss termination. If pregnancy continues, care as for class III
    • Information from reference 13.

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Cleveland Clinic Journal of Medicine: 88 (4)
Cleveland Clinic Journal of Medicine
Vol. 88, Issue 4
1 Apr 2021
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Pregnancy in a woman with congenitally corrected transposition of the great arteries
Sarah A. Goldstein, Lisa Sorenson, Jeffrey B. Chapa, Richard A. Krasuski
Cleveland Clinic Journal of Medicine Apr 2021, 88 (4) 228-236; DOI: 10.3949/ccjm.88a.20136

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Pregnancy in a woman with congenitally corrected transposition of the great arteries
Sarah A. Goldstein, Lisa Sorenson, Jeffrey B. Chapa, Richard A. Krasuski
Cleveland Clinic Journal of Medicine Apr 2021, 88 (4) 228-236; DOI: 10.3949/ccjm.88a.20136
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  • Article
    • FIRST STEP IN EVALUATION
    • CASE CONTINUED: A POSITIVE PREGNANCY TEST
    • HEMODYNAMIC CHANGES OF PREGNANCY
    • EFFECTS OF TRANSPOSITION OF THE GREAT ARTERIES
    • CASE CONTINUED: THE PATIENT CONSULTS A SPECIALIST
    • CARDIOVASCULAR RISK IN PREGNANCY
    • COMPLICATIONS IN PREGNANCY IN WOMEN WITH CC-TGA
    • CASE CONTINUED: ANTICOAGULATION IN PREGNANCY
    • CASE CONTINUED: POSSIBLE DECOMPENSATION
    • CASE CONTINUED: A HEALTHY BABY
    • CLINICAL PEARLS
    • DISCLOSURES
    • Acknowledgment
    • REFERENCES
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