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Pregnancy After Repaired Congenital Pulmonary Atresia
A 37-year-old G1P0 woman with repaired congenital pulmonary atresia with intact ventricular septum, atrial septal defect, and a hypoplastic right ventricle (RV) presented at 10 weeks of intrauterine pregnancy. She had excellent functional status (New York Heart Association [NYHA] class I heart failure [HF] symptoms). She could walk and lift weights at the gym without limitation. She denied any shortness of breath (SOB), dyspnea on exertion, lower extremity swelling, lightheadedness, syncope, or palpitations. Her physical examination findings were remarkable for regular rate and rhythm with a grade 2/6 holodiastolic murmur, clear lung fields, and no lower extremity edema.
Her medical history was remarkable for cyanotic presentation at birth, which led to the diagnosis of congenital pulmonary atresia with intact ventricular septum. She underwent a classic Blalock-Thomas-Taussig (BTT) shunt at 4 months of age. She underwent percutaneous valvotomy at 14 months of age followed by ligation of the BTT shunt at 18 months of age.
An electrocardiogram had findings of a right bundle branch block and right-axis deviation. Laboratory study results were remarkable for B-type natriuretic peptide (BNP) level 82 pg/mL. A transthoracic echocardiogram (TTE) had findings of preserved left ventricular ejection fraction (LVEF) 55-60%, moderately enlarged RV, mildly depressed RV function (tricuspid annular plane systolic excursion [TAPSE] 1.5 cm), moderate tricuspid regurgitation (TR), right ventricular systolic pressure (RVSP) 28 mm Hg, severe pulmonary regurgitation (PR), and no pulmonary stenosis (PS).
As this was an unplanned pregnancy, no preconception counseling was provided. She established care in the cardio-obstetrics clinic under the care of maternal-fetal medicine and cardiology providers. The cardio-obstetrics team decided to provide serial monitoring with echocardiograms and BNP assay every trimester. No significant changes were observed.
Given her excellent functional capacity, absence of symptoms, preserved LVEF, and only mildly reduced RV systolic function, induction of labor was planned at 39 weeks for vaginal delivery under epidural anesthesia. The team agreed to proceed with delivery in the labor and delivery unit without telemetry monitoring given the absence of signs or symptoms of arrhythmia throughout pregnancy. During delivery, no palpitations, SOB, or hemodynamic changes were observed. She received a short course of prophylactic diuretics postpartum to prevent volume-shift–related stress on the mildly dysfunctional RV. She was discharged home on postpartum day 2 with a scheduled follow-up appointment with cardiology at 4 weeks postpartum.
A postpartum TTE has findings of LVEF 55-60%, left ventricular (LV) internal dimension–diastole 4.3 cm, moderately dilated RV with internal diastolic diameter 4.2 cm, normal RV function (TAPSE 2.1 cm), severe PR, no PS (peak velocity 1.6 m/sec), moderate to severe TR, and RVSP 33 mm Hg. At 8 weeks postpartum, cardiac magnetic resonance imaging (cMRI) has findings of normal LV size (left ventricular end-diastolic volume [LVEDV] 124 mL, indexed LVEDV 73.81 mL/m2), normal LV function (LVEF 55%), moderately dilated right ventricular end-diastolic volume (RVEDV; RVEDV 254 mL, indexed RVEDV 151.19 mL/m2), moderately dilated right ventricular end-systolic volume (RVESV; RVESV 164 mL, indexed RVESV 97.62 mL/m2), moderately decreased RV function (RV ejection fraction 35%), severe PR with regurgitant fraction 66%, no PS, and severe pulmonary artery enlargement 38 mm.
Which one of the following is the best next step in managing her PR?
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