Title: Pregnancy with Suspected Pulmonary Arterial Hypertension: A Referral for Confirmation Prior to Termination of Pregnancy
Authors: Cody Schwartz DO, Neeraj Jain MD, Murtuza Ali MD, Viviana Falco MD
Background: Certain cardiovascular conditions pose significant maternal-fetal risk during pregnancy and, therefore, pregnancy may be contraindicated. The condition with greatest risk is seen in pulmonary arterial hypertension (PAH). Pregnancy with PAH usually requires termination of the pregnancy but only after the diagnosis of PAH has been confirmed by right heart catheterization. Case: A 37-year-old pregnant female was referred to Cardiology by Maternal-Fetal Medicine (MFM) for right heart catheterization (RHC) to confirm the diagnosis of suspected pulmonary arterial hypertension (PAH) discovered on transthoracic echocardiogram (TTE). The patient had a past medical history notable for a murmur as a child which she was told not to worry about. Otherwise, she had no medical history of cardiopulmonary disease. During an outpatient primary care clinic visit, the patient complained of intermittent chest pain and palpitations thus a TTE was ordered for further evaluation. Results of the TTE showed right-sided chamber enlargement with a right ventricular (RV) systolic pressure of 74 mmHg and a normal left ventricular systolic function and ejection fraction. The patient was then referred to Cardiology for RHC for confirmation of PAH, however, pre-procedure testing, including a urinary pregnancy test, confirmed that the patient was pregnant. At this time, the RHC was canceled and the patient was referred to MFM for further management of a high-risk pregnancy. MFM was considering terminating the pregnancy but wanted confirmation of PAH prior to making any final decisions. The patient was once again referred to Cardiology and scheduled for RHC with minimal (or no) fluoroscopy in order to avoid undue risks to the fetus. Hemodynamic results of the RHC showed a right atrial pressure of 3 mmHg, a RVbase pressure of 53/3 mmHg, a RV outflow tract pressure of 27/3 mmHg, a pulmonary artery pressure of 27/7 mmHg, and a pulmonary capillary wedge pressure of 7 mmHg. The results of the RHC came as a surprise as it did not show PAH but did show a significant RV intracavitary gradient. Subsequently, further evaluation of the RV intracavitary gradient was performed with a repeat TTE. The results of the TTE showed a prominent RV moderator band producing a double chambered RV, no evidence of a ventricular septal defect (VSD), and hemodynamics congruent with the hemodynamics demonstrated on RHC. From a cardiac standpoint, given the results of the RHC and the repeat TTE, it was deemed appropriate for the patient to proceed with the pregnancy but under close clinical monitoring. Discussion: In women with certain preexisting cardiovascular conditions, pregnancy may be contraindicated, and they should be advised to avoid pregnancy. These conditions include significant pulmonary arterial hypertension of any cause, severe mitral stenosis, severe symptomatic aortic stenosis, bicuspid aortic valve with an aorta diameter >50 mm, Marfan syndrome with a dilated aorta >45 mm, severe systemic ventricular systolic dysfunction (left ventricular ejection fraction <30 percent, New York Heart Association III to IV), and native severe coarctation. 1 Cardiac disease in the pregnant patient can present challenges in cardiovascular and maternal-fetal management. 2 Even in patients with no history of cardiopulmonary disease, pregnancy imposes some dramatic physiologic changes upon the
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