Delayed Termination of Pregnancy in the Setting of Pulmonary Hypertension: A Case Report and Review of the Literature

Background: Nonspecific interstitial pneumonia (NSIP) is a diffuse parenchymal lung disease typically present in young female patients.Pulmonary arterial hypertension (PAH) is a comorbidity of NSIP that can cause many complications during pregnancy due to the physiologic changes of increased estrogen and progesterone, increased metabolic rate, and high-volume states.In pregnant patients with PAH, early termination of pregnancy is recommended to avoid increased maternal risks and mortality.Case Report: Our case involves a 31-year-old female patient with a history of NSIP on home oxygen therapy, diabetes, obesity, and hypertension who was previously evaluated for lung transplantation.She presented to our hospital as a transfer of care at 21.1 weeks with twin IUP from an outside hospital following a 5-week admission.She was initially admitted at 16.1 weeks due to worsening dyspnea on exertion associated with dry cough and orthopnea.She was counseled by pulmonology, cardiology, obstetrics, and maternal-fetal medicine regarding the option for pregnancy termination to improve maternal outcomes, but she declined.The transfer was placed to our institution for the availability of an in-house obstetrics service in the setting of increasing oxygen requirement at greater than 20 weeks.After arrival at our institution, her clinical status began to deteriorate significantly, with her oxygen requirement steadily growing until she was on a high-flow nasal cannula with a flow rate of 50 liters and FiO2 of 0.9 with persistent tachycardia in the 130s.She was transferred to the ICU (Intensive Care Unit), where termination of pregnancy was strongly recommended as the only measure to save her life.The patient at first continued to decline termination with the goal of delivery after viability.She, however, continued to deteriorate with worsening dyspnea and orthopnea, and she elected for termination of pregnancy by dilation and evacuation (D&E) as a lifesaving measure at 22.0 weeks.This was performed without complication under epidural anesthesia.Her dyspnea and overall clinical condition rapidly improved following her D&E, and she was discharged from the hospital 6 days after her procedure on her baseline home oxygen therapy via nasal cannula.Unfortunately, she was readmitted to an outside hospital 1 month later, where she died secondary to healthcare-associated pneumonia, severe PAH secondary to her pulmonary fibrosis, and acute on chronic respiratory failure.Discussion: In patients with high-risk illnesses such as PAH, preconception counseling should elucidate the high risks associated with pregnancy.Pregnant patients who present with PAH should be counseled on termination of pregnancy as a first-line treatment to decrease the risk of maternal death due to the high stakes of significant cardiac and pulmonary morbidity and mortality associated with PAH and pregnancy.Patients who decide to proceed with their pregnancy should be closely monitored for hypoxia, thrombolytic events, and intravascular volume shifts due to the physiologic changes of pregnancy.Due to the overturning of Roe v. Wade in the United States and variable state legislation regarding pregnancy termination, cases such as this illustrate the necessity for honest discussion with patients about the standard of care for pregnancies with critical comorbidities where termination of pregnancy is a lifesaving treatment.Clinicians should be able to have open, truthful, and frank discussions with patients regarding the termination of pregnancy without the fear of legal repercussions.