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Pulmonary Vascular Disease |

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Diagnosed During Pregnancy

Sara Hegab, MD; Niral Patel, MD; Michael Mendez, MD; Rana Awdish, MD; Hector Cajigas, MD
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Henry Ford Health System, Detroit, MI


Chest. 2015;148(4_MeetingAbstracts):977A. doi:10.1378/chest.2269341
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Abstract

SESSION TITLE: Pulmonary Vascular Disease Cases II

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Monday, October 26, 2015 at 03:15 PM - 04:15 PM

INTRODUCTION: Evidence regarding the best management approach of a pregnant patient with inoperable CTEPH is scarce.

CASE PRESENTATION: A 25-year-old female presented at 24 weeks gestation with hypoxemia and dyspnea with a history of peripartum acute pulmonary embolism (PE) 2 years prior and morbid obesity (BMI of 83.5). Due to intermittent adherence to outpatient anticoagulation and high risk for recurrent PE, unfractionated heparin was started. Transthoracic echocardiogram (TTE) showed enlarged right ventricle (RV) with evidence of RV pressure and volume overload. Estimated pulmonary artery systolic pressure (PASP) was 92mmHg. Ventilation perfusion scan showed multiple mismatched perfusion defects corresponding to prior PE. Acute PE was ruled out with contrast-enhanced computed tomography. Right heart catheterization revealed (in mmHg), mean right atrial pressure 4, RV 82/7, mean pulmonary artery pressure 45, mean pulmonary capillary wedge pressure 10, Fick cardiac output (CO) and cardiac index of 7.5 L/min and 2.58 L/min/m2 respectively and PVR of 4.67 WU. Pulmonary angiogram showed multiple filling defects bilaterally confirming diagnosis of CTEPH. Patient was not a candidate for pulmonary artery endarterectomy due to pregnancy and morbid obesity. Sildenafil and inhaled iloprost were initiated for inoperable CTEPH. TTE 1 month later showed PASP to 63mmHg with stable cardiac function. At the time of this submission, the patient had reached 30 weeks of gestation without complication to mother or fetus with plans for cesarean (CS) under regional anesthesia planned at 32 weeks of gestation.

DISCUSSION: Pregnancy in the setting of pulmonary hypertension continues to confer a high maternal mortality. Limited evidence suggests that early initiation of vasodilator therapy and a shift towards CS delivery under regional anesthesia have improved mortality. There is little evidence on the use of vasodilators in CTEPH and pregnancy. We postulate that in patients with inoperable CTEPH and RV dysfunction, the use of vasodilators may reduce vascular resistance in non-affected areas, preserving CO during the hemodynamic stress of pregnancy.

CONCLUSIONS: In this pregnant patient with inoperable CTEPH, the use of sildenafil and inhaled prostacyclin therapy was well tolerated and prevented further HD compromise.

Reference #1: Elisabeth Bédard,Konstantinos Dimopoulos,Michael A.Gatzoulis,Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?EuropeanHeartJournalJan2009,DOI:10.1093/eurheartj/ehn597

DISCLOSURE: The following authors have nothing to disclose: Sara Hegab, Niral Patel, Michael Mendez, Rana Awdish, Hector Cajigas

No Product/Research Disclosure Information


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