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. This may be due to systolic or diastolic malfunction of the left ventricle or due to valvular dysfunction such as mitral regurgitation or mitral stenosis. It usually manifests as pulmonary edema. Because the malfunctioning heart does not pump efficiently, blood fails to leave the pulmonary circulation in a timely manner, leading to abnormally high pressure in the pulmonary vein. The increased pressure in the pulmonary vein can be carried through to the pulmonary artery,[1]
Common causes of pulmonary arterial hypertension (PAH) include HIV, scleroderma and other autoimmune disorders, cirrhosis and portal hypertension, sickle cell disease[2] congenital heart disease, and others. Use of weight loss pills such as Fen-Phen, Aminorex, fenfluramine (Pondimin), and phentermine led to the development of PAH in the past [3] Other causes include sarcoidosis, histiocytosis X, and fibrosing mediastinitis.
Pulmonary embolism also leads to pulmonary hypertension, acutely as well as chronically. When none of these causes can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH). There appears to be a link between IPAH and thyroid diseases[4], but this is not regarded as causative.
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. 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[5] Recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.
When a family history exists, the disease is termed familial pulmonary arterial hypertension (FPAH). IPAH and FPAH are now considered to be genetic disorders linked to mutations in the BMPR2 gene, which encodes a receptor for bone morphogenetic proteins[6] as well as the 5-HT(2B) gene, which codes for a serotonin receptor[7]
There also seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and Trisomy 21.
Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(By Organ System)
Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(In Alphabetical Order)
- Alveolar capillary dysplasia with misalignment of pulmonary veins
- Atrial Septal Defects
- Altitude sickness, chronic
- Bronchiectasis
- Bronchopulmonary Dysplasia
- Cholesterol ester storage disease
- Chronic obstructive pulmonary disease
- Cor triatriatum
- Cystic fibrosis
- Churg-Strauss syndrome
- Coal worker pneumoconiosis
- Chronic renal failure on dialysis
- Chronic hemolytic anemia
- 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
- Pulmonary alveolar microlithiasis
- Pickwickian syndrome
- Paroxysmal nocturnal haemoglobinuria
- Portal hypertension
- Polycythemia vera
- Pulmonary fibrosis
- Pulmonary embolism
- Pulmonary veno-occlusive disease
- Pulmonary capillary hemangiomatosis
- Phentermine poisoning
- Primary pulmonary hypertension
- Sarcoidosis
- Schistosoma japonicum
- Schistosoma mansoni
- 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.
- ↑ Hayes, Gail Boyer. Pulmonary Hypertension Patient's Survival Guide 3rd edition, p. 51
- ↑ 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
- ↑ 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
- ↑ 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
- ↑ 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.
- ↑ 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.