Acute on Chronic Thromboembolic Pulmonary Hypertension: Case Series and Review of Management

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of precapillary pulmonary hypertension classified as group 4 by the World Symposium on Pulmonary Hypertension (WSPH) and should be excluded during an episode of acute pulmonary embolism (PE). Patients presenting to emergency departments with sudden onset of signs and symptoms of acute PE may already have a pre-existing CTEPH condition decompensated by the new PE episode. Identifying an underlying and undiagnosed CTEPH during acute PE, while challenging, is an important consideration as it will alter the patients’ acute and long-term management. Differential diagnosis and evaluation require an interdisciplinary expert team. Analysis of the clinical condition, the CT angiogram, and the hemodynamic situation are important considerations; patients with CTEPH usually have significantly higher sPAP at the time of index PE, which is unusual and unattainable in the context of acute PE and a naïve right ventricle. The imaging may reveal signs of chronic disease such as right ventricle hypertrophy bronchial collaterals and atypical morphology of the thrombus. There is no standard for the management of acute on chronic CTEPH. Herein, we provide a diagnostic and management algorithm informed by several case descriptions and a review of the literature.

A Case of Chronic Pulmonary Embolism Resulting in Pulmonary Hypertension and Decompensated Right Heart Failure

“…Pharmacological management +/-balloon angioplasty should be offered for a patient who is not a candidate for surgery. Balloon pulmonary angioplasty (BPA) also can be considered for patients who underwent endarterectomy with residual symptoms [2].…”

“…Computed tomographic pulmonary angiography (CTPA) shows clots in the pulmonary artery after three months of therapeutic anticoagulation therapy. Ring-like stenosis, webs, slits, and chronic total occlusion are the specific diagnostic signs of CTEPD which are appreciated on CTPA [1,2]. Presentation of pulmonary embolism (PE) can vary from chronic exertional dyspnea to syncope, hemoptysis, and lower extremity edema which can be due to acute PE or sub-acute PE or chronic PE [3].…”

A Case Report of Under-Recognized Conditions in Pulmonary Embolism: Patent Foramen Ovale and Right Ventricular Thrombus

“…We suspected PE with paradoxical embolism and performed a pulmonary CT angiography and a lower extremity venous CT angiography, which showed thrombi in the bilateral main pulmonary arteries (Figure 1B), deep vein thrombosis at the left femoral vein, RVT (Figure 2B), and contrast agent flow from the right atrium to the left atrium, suggesting a shunt through a PFO (Figure 2C). Based on the history of exertional dyspnea over the past few months, right ventricular enlargement, and an elevated tricuspid regurgitant pressure gradient (i.e., greater than 50 mmHg) [5], we made a diagnosis of acute-on-chronic PE complicated with renal infarctions through a PFO and RVT due to right ventricular dysfunction. After admission to the intensive care unit (ICU), he had hemodynamic stability and stable oxygen saturation with a high volume of supplemental oxygen.…”