PH, PAH & CTEPH: Diagnoses & differences

In this video, Dr Hayley Barnes (Respiratory Physician, Alfred Health) answers commonly asked questions about Pulmonary Hypertension (PH), Pulmonary arterial hypertension (PAH) & Chronic thromboembolic pulmonary hypertension (CTEPH).

PAH is a condition characterised by high blood pressure in the arteries of the lungs. It can arise from various causes, including heart disease, lung disease, blood clots, or other rare conditions. Identifying the cause is crucial for determining the prognosis and treatment options.

To diagnose PAH, several tests are performed. An echocardiogram is a common initial test that assesses heart pressures and function. In PAH, the heart may not pump effectively, leading to increased blood flow and pressure in the lungs. Another essential test is a right heart catheterisation, where a wire is threaded through a vein to measure heart pressures directly. This test confirms the diagnosis, identifies the cause, and guides treatment.

CTEPH is a type of Pulmonary Hypertension caused by long-term blood clots in the lungs. Normally, the body dissolves blood clots over time, but in some patients, clots persist, blocking lung vessels and increasing pressure. Screening for CTEPH involves tests like a ventilation-perfusion (VQ) scan and a CT pulmonary angiogram to detect blood clots and assess lung vessel narrowing.

PH encompasses several conditions that increase lung pressure and strain the right side of the heart. Symptoms include breathlessness, fatigue, chest pain, dizziness, and coughing up blood. Treatment options for PAH include medications to reduce heart and lung pressures, improve symptoms, and enhance quality of life. These medications are typically prescribed by specialists.

In advanced cases, intravenous therapy or lung transplantation may be considered. Blood thinners are often used to prevent new clots. For CTEPH, treatment options include surgery to remove clots, balloon pulmonary angioplasty to open blocked vessels, and medications to reduce lung pressure.

Certain conditions, like scleroderma, increase the risk of PH. Scleroderma is an autoimmune disease affecting multiple body systems, including the lungs. Patients with scleroderma should be monitored for PH and pulmonary fibrosis through lung function tests and clinical exams to ensure early detection and treatment.