.jpg "Huda Asif, MD photo")
Huda Asif, MD
University of Miami Leonard M Miller School of Medicine
Atlantis, Florida
Disclosure information not submitted.
Travis Park, MD
Resident physician, Internal Medicine
University of Miami Leonard M Miller School of Medicine
Atlantis, Florida, United States
Disclosure information not submitted.
Elizabeth Valdes, DO
Resident physician, Internal Medicine
University of Miami Leonard M Miller School of Medicine
Atlantis, Florida, United States
Disclosure information not submitted.
Sunil Bali, MD
Pulmonary and critical care
JFK Medical Center
Atlantis, Florida, United States
Disclosure information not submitted.
Christopher Wood, DO
Pulmonary and critical care
JFK Medical Center
Atlantis, Florida, United States
Disclosure information not submitted.
Title: Unilateral chronic thromboembolic pulmonary hypertension presenting as left pulmonary embolism.
Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) represents uncommon sequelae of recurrent pulmonary embolism, prevalent in 0.5-5% of this cohort. We present a case of unilateral CTEPH presenting with chronic left pulmonary embolism.
Description: 57-year-old male presented to emergency department with one-year history of progressive exertional dyspnea worse for one week. Patient has a past medical history significant for recurrent bilateral lower extremity deep vein thrombosis status post thrombectomy and pulmonary embolism with failed multiple oral anti-coagulant therapies including warfarin, apixaban and dabigatran, currently on enoxaparin. On presentation, patient was hemodynamically stable. A CT angiogram showed left pulmonary embolism with right heart strain while a pulmonary angiogram was significant for complete occlusion of left main pulmonary artery by a likely chronic thrombus as evidenced by failed wire traversal test and so thrombectomy was not performed. No occlusion was noted in right pulmonary vasculature. Mean pulmonary artery pressure (mPAP) was 41mmHg, unchanged from 2-month old right heart catheterization with pulmonary capillary wedge pressure of 10mmHg. A hypercoagulable workup was negative. A diagnosis of chronic thromboembolic pulmonary hypertension was made. Patient later developed acute hypoxic respiratory failure. Intubation was deferred in favor of nitric oxide inhalation via high-flow nasal cannula with resultant improvement in oxygenation. Subsequently patient underwent left pulmonary artery endartectomy (PEA) with removal of a Jamieson class I thrombus consistent with complex clot on biopsy. Patient tolerated the procedure without complications and was subsequently discharged home.
Discussion: Unilateral CTEPH is a rare presentation of the disease reported only twice in literature as opposed to bilateral disease. PEA is surgical treatment of choice with >90% 5-year survival and has greater benefit in patients with unilateral obstruction as seen in our case. Early diagnosis is crucial to prevent right heart failure. In the event of respiratory failure awaiting surgical intervention, nitric oxide inhalation via high flow nasal cannula should be preferred over mechanical ventilation to prevent worsening pulmonary vascular resistance and right ventricular afterload.