Diagnostic classification |
1. Pulmonary arterial hypertension |
1.1 Primary pulmonary hypertension |
(a) Sporadic |
(b) Familial |
1.2 Related to: |
(a) Collagen vascular disease |
(b) Congenital systemic to pulmonary shunts |
(c) Portal hypertension |
(d) HIV infection |
(e) Drugs/toxins |
(1) Anorexigens |
(2) Other |
(f) Persistent pulmonary hypertension of the newborn |
(g) Other |
2. Pulmonary venous hypertension |
2.1 Left sided atrial or ventricular heart disease |
2.2 Left sided valvular heart disease |
2.3 Extrinsic compression of central pulmonary veins |
(a) Fibrosing mediastinitis |
(b) Adenopathy/tumours |
2.4 Pulmonary veno-occlusive disease |
2.5 Other |
3. Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxaemia |
3.1 Chronic obstructive pulmonary disease |
3.2 Interstitial lung disease |
3.3 Sleep disordered breathing |
3.4 Alveolar hypoventilation disorders |
3.5 Chronic exposure to high altitude |
3.6 Neonatal lung disease |
3.7 Alveolar-capillary dysplasia |
3.8 Other |
4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease |
4.1 Thromboembolic obstruction of proximal pulmonary arteries |
4.2 Obstruction of distal pulmonary arteries |
(a) Pulmonary embolism (thrombus, tumour, ova and/or parasites, foreign material) |
(b) In situ thrombosis |
(c) Sickle cell disease |
5. Pulmonary hypertension due to disorders directly affecting the pulmonary vasculature |
5.1 Inflammatory |
(a) Schistosomiasis |
(b) Sarcoidosis |
(c) Other |
5.2 Pulmonary capillary haemangiomatosis |
Functional assessment1-150 |
(A) Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain or near syncope. |
(B) Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain or near syncope. |
(C) Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain or near syncope. |
(D) Class IV: Patients with pulmonary hypertension with inablility to carry out any physical acitivity without symptoms. These patients manifest signs of right heart failure. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. |
↵1-150 Modified after the New York Heart Association functional classification.1