From the Guidelines
Pulmonary hypertension is characterized by clinical signs such as shortness of breath, fatigue, weakness, angina, and syncope, which are mainly related to progressive right ventricular dysfunction, as stated in the 2015 ESC/ERS guidelines 1. These symptoms are non-specific and often induced by exertion, with patients experiencing shortness of breath, fatigue, and weakness, as well as less common symptoms like dry cough and exercise-induced nausea and vomiting. As the disease progresses, patients may develop more severe symptoms, including abdominal distension and ankle edema, which are indicative of right ventricular failure. Some patients may also experience mechanical complications of pulmonary hypertension, such as haemoptysis, hoarseness, and wheeze, due to the abnormal distribution of blood flow in the pulmonary vascular bed. The physical signs of pulmonary hypertension include left parasternal lift, an accentuated pulmonary component of the second heart sound, an RV third heart sound, a pansystolic murmur of tricuspid regurgitation, and a diastolic murmur of pulmonary regurgitation, as outlined in the 2015 ESC/ERS guidelines 1. In advanced cases, patients may exhibit elevated jugular venous pressure, hepatomegaly, ascites, peripheral edema, and cool extremities, which are characteristic of right heart failure. Clinical examination may also suggest an underlying cause of pulmonary hypertension, such as telangiectasia, digital ulceration, and sclerodactyly in scleroderma, or inspiratory crackles in interstitial lung disease. Key clinical signs to look out for include:
- Shortness of breath (dyspnea)
- Fatigue
- Weakness
- Angina
- Syncope
- Abdominal distension
- Ankle edema
- Left parasternal lift
- Accentuated pulmonary component of the second heart sound
- RV third heart sound
- Pansystolic murmur of tricuspid regurgitation
- Diastolic murmur of pulmonary regurgitation
- Elevated jugular venous pressure
- Hepatomegaly
- Ascites
- Peripheral edema
- Cool extremities.
From the FDA Drug Label
Adverse events which may be related to the underlying disease include dyspnea, fatigue, chest pain, edema, hypoxia, right ventricular failure, and pallor. Withdrawal Effects Abrupt withdrawal (including interruptions in drug delivery) or sudden large reductions in dosage of epoprostenol may result in symptoms associated with rebound pulmonary hypertension, including dyspnea, dizziness, and asthenia.
- Clinical signs of having pulmonary HTN that may be related to the underlying disease include:
- Dyspnea
- Fatigue
- Chest pain
- Edema
- Hypoxia
- Right ventricular failure
- Pallor
- Rebound pulmonary hypertension symptoms include:
- Dyspnea
- Dizziness
- Asthenia 2
From the Research
Clinical Signs of Pulmonary Hypertension
The clinical signs of pulmonary hypertension (PH) can vary depending on the underlying cause and severity of the disease. Some common signs and symptoms include:
- Shortness of breath (dyspnea) 3
- Fatigue 3
- Chest pain or pressure 3
- Swelling in the legs and ankles (edema) 3
- Palpitations or irregular heartbeat 3
- Coughing up pink, frothy mucus 3
Diagnostic Tests
To diagnose PH, healthcare providers may use a combination of tests, including:
- Electrocardiography (ECG) to evaluate the heart's electrical activity 3
- Chest radiography (X-ray) to visualize the lungs and heart 3
- Pulmonary function tests (PFTs) to assess lung function 3
- Transthoracic echocardiography (TTE) to estimate the probability of PH 3
- Right-sided heart catheterization to measure pulmonary artery pressure and assess right heart function 3
Classification and Treatment
PH can be classified into five clinical subgroups, including:
- Pulmonary arterial hypertension (PAH) 4, 3
- PH due to left-sided heart disease 5, 3
- PH due to chronic lung disease 3
- Chronic thromboembolic PH (CTEPH) 3
- PH with an unclear and/or multifactorial mechanisms 3 Treatment options vary depending on the underlying cause and severity of PH, but may include:
- Oral anticoagulation 4
- Diuretics 4
- Oxygen supplementation 4
- Calcium channel blockers 4
- Phosphodiesterase type 5 inhibitors 5, 6, 7
- Endothelin receptor antagonists 5, 6, 7
- Prostacyclin analogues 4, 6
- Prostacyclin receptor agonists 3
- Soluble guanylate cyclase stimulators 3, 6