A 25-year-old woman, gravida 1 para 0, comes to the office to establish prenatal care. The patient reports no nausea or vomiting, abdominal pain, or vaginal bleeding. However, over the past year, she has experienced progressive shortness of breath "during usual daily activities" and has noticed that her fingers turn blue with exertion. The patient has an unrepaired congenital ventricular septal defect and pulmonary hypertension, and has heart failure that is managed with diuretics. She had an episode of unprotected intercourse 2 months ago but is not currently sexually active. The patient does not use tobacco, alcohol, or illicit drugs. Blood pressure is 110/80 mm Hg, pulse is 104/min, and respirations are 24/min. Pulse oximetry shows 89% on room air. BMI is 23 kg/m². A transvaginal ultrasound reveals a 6-week intrauterine gestation with a normal fetal heart rate. Recent echocardiogram showed an ejection fraction of 25%. The patient asks how her medical condition and pregnancy should be managed. Which of the following is the most appropriate recommendation?

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Explanation: 11ec2b69_c027_8b3d_8f63_f5d5ae8b2a98_MD0005_00 Eisenmenger syndrome is a complication of an unrepaired ventricular septal defect that leads to pulmonary hypertension, right-to-left shunting, cyanosis, and eventual right- and left-sided heart failure. Decreased systemic vascular resistance (which occurs as a normal physiologic adaptation to pregnancy) can exacerbate the right-to-left shunting and worsen the cyanosis and heart failure. Therefore, pregnancy can cause cardiovascular decompensation in patients with Eisenmenger syndrome and is associated with high maternal mortality rates (up to 50%) (Choice E). Maternal mortality can occur during gestation, labor, or delivery; however, mortality is highest during the immediate postpartum when rapid hemodynamic shifts (eg, increased systemic vascular resistance) occur, for which the heart is unable to compensate. Additional obstetric complications include an increased risk of preterm delivery, fetal growth restriction, and perinatal mortality. Due to high maternal mortality rates and poor fetal prognosis, Eisenmenger syndrome is a medical contraindication to pregnancy, and termination of pregnancy should be recommended. Eisenmenger syndrome Patients with Eisenmenger syndrome are encouraged to use reliable contraception; first-line options include hysteroscopic sterilization or a subdermal progestin implant. Estrogen-containing contraceptives are contraindicated due to an increased risk of thromboembolism. subdermal progestin implant (Choices A and B) Surgical management for patients with Eisenmenger syndrome includes heart-lung or lung transplantation plus cardiac defect repair. However, this surgery is avoided during pregnancy due to a high rate of maternal mortality. The acute blood loss that occurs during surgery coupled with the physiologic anemia and decreased systemic vascular resistance associated with pregnancy can cause increased cardiac demand that the cardiovascular system of patients with Eisenmenger syndrome cannot accommodate. (Choice D) A cesarean delivery is not recommended in patients with functionally significant cyanotic heart disease as it is associated with a higher mortality rate than vaginal delivery. A cesarean delivery is associated with increased rates of volume depletion (eg, blood loss), hemodynamic instability, thromboembolism, and postoperative complications (eg, incisional hematoma). Educational objective: Eisenmenger syndrome is considered a medical contraindication to pregnancy due to high maternal mortality rates and poor fetal prognosis. Termination of pregnancy should be recommended to these patients. References: Pregnancy and pulmonary hypertension. (http://www.ncbi.nlm.nih.gov/pubmed/24685319) Eisenmenger syndrome in pregnancy. (http://www.ncbi.nlm.nih.gov/pubmed/27849306)

A 25-Year-Old Woman, Gravida 1 Para 0, Comes to the Office