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Medical City Arlington
Eisenmenger complex, congenital heart defects, spontaneous abortion
Cardiology | Cardiovascular Diseases | Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Internal Medicine
Background: Eisenmenger's syndrome (ES) is a congenital cardiac abnormality in which a significant chronic left-to-right shunt results in pulmonary arterial hypertension and a reversal of the shunting direction. A woman with ES should ideally avoid conception given the increased risk of unexpected fetal demise and maternal mortality. Case: We present a case of a 35-year-old female patient G3, P0, at 9 weeks gestation with reported PMHx of erythrocytosis and spontaneous abortions who presented to the hospital with complaints of vaginal bleeding and worsening SOB. She was noted to have a Hct of 70 and a Hb of 23.8, with SaO2 of 86% on room air upon initial evaluation. A transthoracic echocardiogram revealed a medium to large perimembranous ventricular septal defect (VSD) measuring 6 mm with predominantly right to left shunting with a QP/QS ratio of 0.66. The patient's right ventricle was dilated with systolic and diastolic septal flattening suggestive of pulmonary hypertension. CMR confirmed enlargement of the right and left ventricular areas at both end-systole and end-diastole. Further workup revealed persistent hypoxia caused by ES secondary to VSD. JAK2 studies were negative, ruling out a myeloproliferative neoplasm. Measured beta-hCG was not in range with 9-weeks of pregnancy. Additionally, the pregnancy USS revealed a non-viable fetus, concluding that a spontaneous abortion was inevitable. Transfer to another facility for higher level of care and evaluation by adult congenital heart disease cardiologist was not available due to financial reasons. She was ultimately discharged home on oxygen with regular outpatient follow ups. Conclusion: The prognosis of ES pregnancy has not greatly improved despite contemporary advancements in obstetric and cardiovascular medicine. Pregnancy termination is typically advised because spontaneous pregnancy loss/perinatal morbidity are about 30% and maternal mortality for women with ES is reported to be 30-50%. Prolonged bed rest, continuous oxygen therapy, and anticoagulation under hemodynamic observation can positively influence maternal and fetal outcomes.
HCA Healthcare Graduate Medical Education
Patel, Riddhiben; Hamadeh, Anas; Montoya, Laura; and Thambidorai, Senthil, "The Domino Effect: Spontaneous Abortions as a Sequela of Eisenmenger Syndrome" (2023). North Texas Research Forum 2023. 23.