In pulmonary hypertension the mean pulmonary artery pressure (PAP) is > 25 mmHg at rest or > 30 mmHg with exercise.

Signs and symptoms

• Shortness of breath on exertion
• Non productive cough
• Swollen ankles or swollen feet
• Swollen abdomen
• Orthopnea or paroxysmal nocturnal dyspnea (PND)
• Hemoptysis
• Chest pain or discomfort
• Syncopal attacks or fainting
• Light headedness
• Dizziness
• Tiredness
• On examination the typical signs
• Loud P2 (heart sound)
• Parasternal heave
• Jugular vein distension
• Hepatojuglar reflex
• Clubbing
• Cyanosis
• Palpitations

Classification and causes of pulmonary hypertension

Pulmonary arterial hypertension

• Idiopathic pulmonary hypertension
• Familial pulmonary hypertension
• Associated with following conditions like connective tissue disease, congenital systemic pulmonary shunts, portal hypertension, HIV infection, drugs and toxins
• Other (hyperthyroid state, gaucher's disease, following splenectomy, myeloproliferative disorders)
• Associated with significant venous or capillary involvement like pulmonary veno- occulsive disease, pulmonary capillary hemangiomatosis
• Pulmonary hypertension associated with left heart disease left sided atrial or ventricular heart disease and left sided valvular heart disease
• Pulmonary hypertension associated with lung respiratory disease, COPD, interstitial lung disease, alveolar hypoventilation disorders, chronic exposure to high altitude, sleep apnea syndrome.
• Pulmonary hypertension due to chronic thrombotic and or embolic disorder thromboembolic obstruction of proximal and distal pulmonary arteries and non- thromboembolic pulmonary embolism
• Miscellaneous- sarcoidosis, histiocytosis, compression of pulmonary vessels.

Complications

• Right heart enlargement and failure
• Reduction in heart function
• Convulsions
• Cor pulmonale
• Tricuspid valve incompetence
• Right ventricular hypertrophy
• Pulmonary valve incompetence
• Respiratory failure
• Right bundle branch block
• Death due to heart failure

Tests and diagnosis

• ECG- Right ventricular hypertrophy, strain, and right atrial dilation. Right ventricular hypertrophy and right axis deviation.
• Chest radiograph- Central pulmonary arterial dilatation with pruning of peripheral blood vessels. Right atrial and ventricular enlargement.
• Trans thoracic Doppler echocardiography
• Arterial blood gas analysis
• HRCT of lung
• Contrast enhanced spiral or helical CT

Treatment options

Since pulmonary venous hypertension is synonymous with congestive heart failure, the treatment is to optimize left ventricular function.

• Vasodilators (blood vessel dilators)- Epoprostenol and iloprost
• Endothelin receptor antagonists- Bosentan and Sitaxsentan
• PDE- 5 inhibitors- Sildenafil
• High dose calcium channel blockers- Amlodipine, Diltiazem, and Nifedipine.
• Anticoagulants- Warfarin
• Diuretics- lasix and Toresamide
• Oxygen
• Balloon atrial septostomy
• Heart or heart lung transplant may be an option, especially for younger people who have idiopathic pulmonary hypertension.