From the Guidelines
Pulmonary hypertension (PH) is a complex condition characterized by high blood pressure in the arteries of the lungs, with various causes and symptoms, including idiopathic, heritable, and associated forms, as outlined in the 2015 ESC/ERS guidelines 1. The causes of PH can be classified into several groups, including:
- Pulmonary arterial hypertension (PAH)
- Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
- Pulmonary hypertension due to left heart disease
- Pulmonary hypertension due to lung diseases and/or hypoxia
- Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions
- Pulmonary hypertension with unclear and/or multifactorial mechanisms Symptoms of PH may include shortness of breath, fatigue, chest pain, and swelling in the legs and ankles, and can be assessed through right heart catheterization, which measures mean pulmonary arterial pressure ≥25 mmHg at rest 1. The diagnosis and treatment of PH should be guided by the comprehensive clinical classification outlined in the 2015 ESC/ERS guidelines 1, which provides a framework for understanding the various forms of PH and their underlying causes. Key considerations in the diagnosis and treatment of PH include:
- Identifying the underlying cause of PH, such as idiopathic, heritable, or associated forms
- Assessing the severity of PH through right heart catheterization and other diagnostic tests
- Selecting appropriate treatment options, such as medications, oxygen therapy, and lifestyle modifications, to reduce symptoms, improve quality of life, and slow disease progression 1.
From the Research
Causes of Pulmonary Hypertension (PH)
- Endothelin 1, a powerful endogenous vasoconstrictor and mitogen, is thought to be a cause of pulmonary hypertension 2
- Pulmonary vascular remodelling, thrombosis, and vasoconstriction are also involved in increasing pulmonary vascular resistance (PVR) 3
Symptoms of Pulmonary Hypertension (PH)
- Shortness of breath (dyspnoea) 2, 3
- Fatigue 2
- Clinical worsening, including right ventricular failure and death if left untreated 3
- Decreased exercise capacity, as measured by the 6-minute walk distance (6mwd) 2, 3
Pathophysiology of Pulmonary Hypertension (PH)
- Endothelin-1 (ET-1) plays a significant role in the pathophysiology and prognosis of pulmonary hypertension (PHT) 4
- Multiple pathways are involved in the pathophysiology of PH, including endothelin receptor antagonism, phosphodiesterase type 5 inhibition, and prostacyclin analogs 5, 6, 3
Treatment of Pulmonary Hypertension (PH)
- Endothelin receptor antagonists, such as bosentan, sitaxsentan, and ambrisentan, are used to treat PH 4, 6, 3
- Phosphodiesterase type 5 inhibitors, such as sildenafil, are also used to treat PH 6, 3
- Prostacyclin analogs, such as epoprostenol and treprostinil, are used to treat PH, particularly in severe cases 5, 6