From the Guidelines
Pulmonary hypertension (PH) is typically classified into four stages based on the World Health Organization (WHO) functional classification system, which is also known as the New York Heart Association (NYHA) functional classification system 1. The stages are as follows:
Stages of Pulmonary Hypertension
- Stage I: Patients with PH who have no symptoms and no limitation of physical activity.
- Stage II: Patients with PH who have mild symptoms, such as shortness of breath with ordinary physical activity, but no limitation of physical activity.
- Stage III: Patients with PH who have marked limitation of physical activity due to symptoms, such as shortness of breath, even during less-than-ordinary activity.
- Stage IV: Patients with PH who have severe symptoms, such as shortness of breath at rest, and are unable to carry out any physical activity without discomfort.
The clinical classification of PH has been updated over the years, with the most recent update being in 2018, which includes five groups:
Clinical Classification of PH
- Group 1: Pulmonary arterial hypertension (PAH)
- Group 2: PH due to left heart disease
- Group 3: PH due to lung diseases and/or hypoxia
- Group 4: PH due to pulmonary artery obstructions
- Group 5: PH with unclear and/or multifactorial mechanisms 1.
It is essential to note that the diagnosis and treatment of PH depend on the underlying cause and severity of the disease, and treatment options may include medications such as phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and prostacyclin analogs, as well as lung transplantation in severe cases 1.
From the Research
Classification of Pulmonary Hypertension
The classification of Pulmonary Hypertension (PH) is based on the underlying cause and is divided into five clinical subgroups:
- Pulmonary arterial hypertension (PAH)
- PH due to left-sided heart disease
- PH due to chronic lung disease
- Chronic thromboembolic PH (CTEPH)
- PH with an unclear and/or multifactorial mechanisms 2
Stages of Pulmonary Hypertension
While the provided studies do not explicitly outline the stages of Pulmonary Hypertension, they discuss the importance of accurate diagnosis and classification of PH to establish effective treatment 3, 2, 4, 5, 6. The studies also highlight the need for a multidisciplinary approach to manage PH and the importance of early referral to specialized PH centers 2, 4, 6.
Diagnosis and Management
The diagnosis of PH involves a range of tests, including electrocardiography, chest radiography, pulmonary function tests, and transthoracic echocardiography 2, 6. Right-sided heart catheterization is essential for accurate diagnosis and classification 2, 4, 5. The management of PH depends on the underlying cause and severity of the disease, and may involve targeted treatments, such as phosphodiesterase type 5 inhibitors and endothelin receptor antagonists 2, 4.
Key Considerations
Key considerations in the diagnosis and management of PH include: