Chronic thromboembolic pulmonary hypertension

Synonyms

CTEPH,

Overview

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious, progressive form of pulmonary hypertension . It occurs when old blood clots in the lungs do not dissolve completely, but instead turn into scar tissue that narrows and blocks the pulmonary arteries. This restriction forces the right side of the heart to work much harder, eventually leading to heart failure if left untreated.

Symptoms

Symptoms frequently mimic other cardiopulmonary diseases (like asthma or COPD) and include unexplained shortness of breath, extreme fatigue, and chest discomfort.

Causes

CTEPH is caused by unresolved or organized blood clots (thrombi) in the pulmonary arteries that fail to dissolve over time. Instead, these clots turn into scar tissue, narrowing the arteries and forcing the heart to work much harder to pump blood into the lungs.

Prevention

Prevention centers on the prompt, effective management of acute pulmonary embolisms (PE) and minimizing risk factors for blood clots. Because CTEPH occurs when blood clots fail to dissolve and instead scar the pulmonary arteries, prevention primarily requires ensuring initial clots are fully resolved.

Diagnosis

Diagnosis involves evaluating high blood pressure in the lungs and identifying unresolved, organized blood clots as the cause . Because symptoms like shortness of breath and fatigue mimic other conditions, diagnosis typically follows a specific, multi-step process.

Prognosis

Chronic thromboembolic pulmonary hypertension (CTEPH) prognosis has improved dramatically. Once considered almost universally fatal within a few years , modern multimodal treatments yield excellent survival rates exceeding 90% at 3 years . Prognosis is largely dictated by treatment approach, operability, and early multidisciplinary care .

Treatment

Chronic thromboembolic pulmonary hypertension (CTEPH) treatment primarily involves a potentially curative open-chest surgery called pulmonary thromboendarterectomy (PTE). For inoperable cases or patients with residual pulmonary hypertension, minimally invasive balloon pulmonary angioplasty (BPA), targeted medications, and lifelong anticoagulation are used.