Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension
CTEPH_pulmonary_artery_angiogram_(PA_angiogram).tif
Specialty	Pulmonology, cardiology
Usual onset	63 years (median)
Duration	Long term
Risk factors	Splenectomy, inflammatory bowel disease, chronic thyroid hormone replacement, blood types other than O, malignancy, infected ventriculo-atrial shunt, permanent intravenous leads
Diagnosis	Invasively measured mean pulmonary arterial pressure combined with specialist imaging
Treatment	Pulmonary endarterectomy, Balloon pulmonary angioplasty, medical treatment
Frequency	5 cases per million

PEA specimen

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a form of pulmonary hypertension caused by chronic obstruction of the pulmonary arteries due to organized blood clots. This condition is a serious complication that can arise after an episode of pulmonary embolism, where blood clots travel to the lungs and block the pulmonary arteries.

Pathophysiology

CTEPH occurs when blood clots that have traveled to the lungs do not dissolve completely and instead become organized into fibrotic tissue. This leads to persistent obstruction of the pulmonary arteries, resulting in increased pulmonary vascular resistance and elevated pulmonary artery pressure. Over time, this increased pressure can lead to right ventricular hypertrophy and eventually right heart failure.

Symptoms

The symptoms of CTEPH are often similar to those of other forms of pulmonary hypertension and can include:

• Dyspnea (shortness of breath), especially during exertion
• Fatigue
• Chest pain
• Syncope (fainting)
• Edema (swelling) in the legs and ankles
• Cyanosis (bluish discoloration of the skin)

Diagnosis

The diagnosis of CTEPH involves a combination of clinical evaluation, imaging studies, and hemodynamic measurements. Key diagnostic tools include:

• Ventilation/perfusion scan (V/Q scan) to detect mismatched perfusion defects
• Computed tomography pulmonary angiography (CTPA) to visualize the pulmonary arteries
• Right heart catheterization to measure pulmonary artery pressures
• Pulmonary angiography to confirm the presence of organized thrombi

Treatment

The primary treatment for CTEPH is pulmonary endarterectomy (PEA), a surgical procedure that removes the organized clots from the pulmonary arteries. This surgery can significantly improve symptoms and hemodynamics in eligible patients. For patients who are not candidates for surgery, medical therapies such as riociguat or balloon pulmonary angioplasty may be considered.

Prognosis

The prognosis for patients with CTEPH varies depending on the severity of the disease and the success of treatment. Surgical intervention with PEA can be curative in many cases, leading to significant improvements in quality of life and survival. However, untreated CTEPH can lead to progressive right heart failure and increased mortality.

Related Pages

• Pulmonary hypertension
• Pulmonary embolism
• Right heart failure
• Pulmonary endarterectomy