Pulmonary hypertension causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: Ralph Matar,
Overview
- The most common cause of pulmonary hypertension is left heart failure leading to pulmonary venous hypertension.
- Common causes of pulmonary arterial hypertension (PAH) include HIV, systemic sclerosis, portal hypertension, sickle cell disease,[1] and congenital heart disease
- Lung diseases that lower oxygen in the blood (hypoxia) are well known causes of pulmonary hypertension, including COPD, interstitial lung disease, Pickwickian syndrome or obesity-hypoventilation syndrome, and possibly sleep apnea.
- Other causes include sarcoidosis, histiocytosis X, genetic diseases linked to known mutations[2],[3],fibrosing mediastinitis, pulmonary embolism, and
thyroid disease.[4]However thyroid disease is only am association and is not regarded as causative.
- The use of weight loss pills has also led to the development of PAH in the past [5]
- Human herpesvirus 8, also associated with Kaposi's sarcoma, has been demonstrated in patients with PAH, suggesting that this virus may play a role in its development[6] Recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.
- There also seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and Trisomy 21.
- When none of these causes can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH).
Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(By Organ System)
Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(In Alphabetical Order)
- Altitude sickness, chronic
- Alveolar capillary dysplasia with misalignment of pulmonary veins
- Atrial Septal Defects
- Bronchiectasis
- Bronchopulmonary Dysplasia
- Cholesterol ester storage disease
- Chronic hemolytic anemia
- Chronic obstructive pulmonary disease
- Chronic renal failure on dialysis
- Churg-Strauss syndrome
- Coal worker pneumoconiosis
- Cor triatriatum
- Cystic fibrosis
- Diastolic left heart dysfunction
- Diethylpropion
- Fallot tetralogy
- Fetal circulation, persistent
- Fibrosing mediastinitis
- Gaucher disease
- Glycogen storage diseases
- Idiopathic pulmonary haemosiderosis
- Idiopathic spinal scoliosis
- Indian familial childhood cirrhosis
- Interstitial Lung Disease
- Langerhan cell Histiocytosis
- Mitral valve incompetence
- Mitral valve stenosis
- Monocrotaline poisoning
- Myeloproliferative disorders
- Neurofibromatosis
- Obstructive sleep apnoea
- Paroxysmal nocturnal haemoglobinuria
- Phentermine poisoning
- Pickwickian syndrome
- Portal hypertension
- Polycythemia vera
- Primary pulmonary hypertension
- Pulmonary alveolar microlithiasis
- Pulmonary capillary hemangiomatosis
- Pulmonary embolism
- Pulmonary fibrosis
- Pulmonary veno-occlusive disease
- Sarcoidosis
- Schistosoma japonicum
- Schistosoma mansoni
- Sickle cell disease
- Splenectomy
- Systemic Lupus Erythematosus
- Systolic Left heart dysfunction
- Tropical pulmonary eosinophilia
- Vasculitis
- Ventricular septal defect
Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(By Classes, Dana Point 2008)
Class 1: Pulmonary Aterial Hypertension
- Idiopathic PAH
- Heritable( BMPR2,ALK-1,Endogin...)
- Drug and toxin induced.
- Connective tissue diseases
- HIV
- Portal Hypertension
- Congenital heart diseases
- Schistosomiasis
- Chronic hemolytic anemia.
- Persistent Pulmonary Hypertension of the newborn
Class 2: Pulmonary Venous Hypertension or pulmonary hypertension owing to left heart disease
- Systolic Dysfunction.
- Diastolic dysfunction.
- Valvular disease.
Class 2: Pulmonary Hypertension associated with disorders of the respiratory system and/or Hypoxemia
- Chronic Obstructive Pulmonary Disease.
- Interstitial Lung Disease
- Pulmonary diseases with mixed restrictive and obstructive patterns.
- Obstructive sleep apnea.
- High Altitude(chronically).
- Developmental abnormalities.
Class 4: Pulmonary Hypertension due to chronic thrombotic and/or embolic disease
Class 5: Pulmonary Hypertension due to disorders directly affecting the pulmonary vasculature
- Hematologic disorders: Myeloproliferative disorders,splenectomy,polycythemia vera.
- Systemic disorders: Sarcoidosis,Langerhan cell Histiocytosis, Neurofibromatosis, Vasculitis.
- Metabolic disorders: Glycogen storage diseases, Gaucher disease, Thyroid disorders.
- Miscellaneous: Tumor obstruction, Fibrosing mediastinitis, chronic renal failure on dialysis.
- ↑ Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95
- ↑ Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44.
- ↑ Blanpain C, Le Poul E, Parma J, Knoop C, Detheux M, Parmentier M, Vassart G, Abramowicz MJ. Serotonin 5-HT(2B) receptor loss of function mutation in a patient with fenfluramine-associated primary pulmonary hypertension. Cardiovasc Res 2003;60(3):518-28.
- ↑ Curnock AL, Dweik RA, Higgins BH, Saadi HF, Arroliga AC. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999;318:289-292
- ↑ Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;335:609-16
- ↑ Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, Geraci MW, Brown KK, Routes JM, Tuder RM, Voelkel NF. Expression of Human Herpesvirus 8 in Primary Pulmonary Hypertension.N Engl J Med 2003;349:1113-22.