CTEPH - Chronic Thromboembolic Pulmonary Hypertension Thoughts on Diagnosis and Treatment

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is caused by blood clots in the lung arteries and may be a common cause of pulmonary hypertension that can in turn cause heart failure. However, due to its nonspecific symptoms and the fact that such symptoms often don’t appear until 3-6 months after an acute event – if ever – CTEPH often goes undiagnosed. In this discussion, we explore the challenges of diagnosis, the elements of a thorough workup, and potential treatments including surgical pulmonary thromboendarterectomy (PTE). In this discussion, we highlight:

● Why non-specific symptoms including fatigue and shortness of breath often lead practitioners to incorrectly suspect Chronic Obstructive Pulmonary Disease (COPD), when these symptoms in patients with a history of pulmonary emboli may actually indicate CTEPH.
● How to correctly evaluate patients for CTEPH through echocardiograms, V/Q scans, CT scans, pulmonary angiographs and right heart catheterization.
● Why it is important to refer likely patients to a pulmonologist or cardiologist with specific experience in CTEPH, and to surgical centers with the necessary expertise to treat it.
● Video of a successful Pulmonary Thromboendarterectomy (PTE), in which surgeons surgically remove scar tissue and any residual blood clots from the patient's pulmonary arteries – a procedure that can cure the patient of Pulmonary Hypertension.

Ani Anyanwu, MD
Professor and Vice Chair
Department of Cardiovascular Surgery
Icahn School of Medicine at Mount Sinai

Randolph P. Martin, MD
Clinical Professor
Department of Cardiovascular Surgery
Icahn School of Medicine at Mount Sinai