Take My Breath Away, A Rare Case of Pulmonary Hypertension: A Case Report and Literature Review

Abstract

Introduction/Background

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare condition in which there is elevated blood pressure in the pulmonary arteries caused by chronic blood clots (thromboembolic), which obstruct the free flow of blood through the lungs. When presented, chronic thromboembolic pulmonary hypertension presents considerable management difficulties.

Objectives

This report aims to illustrate the management of chronic thromboembolic pulmonary hypertension.

Case presentation

33 y.o. male with history of PE/DVT (2019), HTN, and Burkitt Lymphoma (in remission for over 10 years), presented to the ED with complaints of shortness of breath. Symptoms occurred for one week with associated dry cough. Patient's dyspnea was constant, occurred at rest, and worsened with exertion. On exam, patient had a BMI of 48.4. Acanthosis nigricans noted along the neck. Patient was diaphoretic, and required 2 L of nasal cannula, which was a new oxygen requirement. CTPE showed chronic thromboembolic pulmonary hypertension.

Echo showed an EF 60-65% with normal LV function. RV was moderate to severely dilated. The interatrial septum bowed toward left atrium consistent with elevated right atrial pressure. V/Q scan showed multiple bilateral segmental and subsegmental wedge-shaped perfusion defects, Large segmental perfusion defect in left upper lobe, right middle lobe and right lower lobe. Findings characteristic of pulmonary embolism, CTEPH. Cardiology was consulted with recommendations for a right heart cath. Cardiothoracic surgery was consulted which recommendation stat transfer to a pulmonary hypertension center and initiation of a guanylate cyclase stimulator outpatient. Due to cost, the patient was not able to be initiated on the guanylate cyclase stimulator. The patient was emergently transferred to a hospital that specializes in pulmonary hypertension and underwent open heart surgery via pulmonary endarterectomy.

Methods

How Is CTEPH Diagnosed? Workup includes V/Q Scan (imaging test of choice), Transthoracic echocardiogram (TTE), CT pulmonary angiography, Right heart catheterization, Pulmonary function tests, Cardiopulmonary exercise test

Literature review and Results

The true incidence of CTEPH is unknown because it commonly goes undiagnosed. Current reported cases after PE range from 0.5-0.9%. The landmark study by Pengo et al followed 223 patients prospectively after an acute PE for 10 years. There was a reported incidence of CTEPTH in 3.1% of patients. Still, the true incidence remains underdiagnosed and even fewer cases are being diagnosed in the United States. Management includes three options: Pulmonary Thromboendarterectomy, Balloon Pulmonary Angioplasty and medical therapy. Medical therapy includes lifelong anticoagulation to prevent new clots and remains an area of active research.

Conclusion

CTEPH is a rare complication of chronic PE and has high rates of morbidity and mortality. If suspected, CTEPH requires prompt evaluation and management to provide the best outcome. Still, there are many unanswered questions in the management of CTEPH. Not limited to the natural history of the disease, as well as the treatment risk to benefit ratio. CTEPH remains a unique area of investigation and continues to require ongoing research for the progression of treatment as options continue to evolve.