In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery or lung vasculature. Depending on the cause, it can be a severe disease with a markedly decreased exercise tolerance and right-sided heart failure. It was first identified by Dr Ernst von Romberg in 1891^[1]. It can be one of five different types, arterial, venous, hypoxic, thromboembolic, or miscellaneous. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Medicine is the branch of health science and the sector of public life concerned with maintaining or restoring human health through the study, diagnosis, treatment and possible prevention of disease and injury. ... The pulmonary arteries carry blood from the heart to the lungs. ... The lungs flank the heart and great vessels in the chest cavity. ... Pulmonary circulation is the portion of the cardiovascular system which carries oxygen-depleted blood away from the heart, to the lungs, and returns oxygenated blood back to the heart. ...

Signs and symptoms

A history usually reveals gradual onset of shortness of breath, fatigue, non-productive cough, angina pectoris, syncope (fainting), peripheral edema, and rarely hemoptysis. Pulmonary arterial hypertension (PAH) typically does not present with orthopnea or paroxysmal nocturnal dyspnea, while pulmonary venous hypertension typically does. Dyspnea (Latin dyspnoea, Greek dyspnoia from dyspnoos - short of breath) or shortness of breath (SOB) is perceived difficulty breathing or pain on breathing. ... Fatigue may refer to: Fatigue (physical) - tiredness in humans Fatigue (material) - failure by repeated stress in materials Fatigues (uniform) - military uniform (BDU or ACU) Chronic Fatigue Syndrome - a medical condition Battle fatigue - also known as Post-traumatic stress disorder Readers fatigue - a side-effect of parsing poorly formatted textual... Syncope has two distinct and apparantly unrelated meanings, one in linguistics and another in medicne. ... Edema (BE: oedema, formerly known as dropsy) is swelling of any organ or tissue due to accumulation of excess fluid. ... Hemoptysis (US English) or haemoptysis (International English) is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs (e. ... orthopnea ... kjkjk ...

In order to establish the cause, the physician will generally conduct a thorough medical history. A detailed family history is taken to determine whether the disease might be familial. Physical examination is performed to look for typical signs of pulmonary hyertension including a loud P2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal edema, ascites, hepatojugular reflux, etc. In medicine, the physical examination or clinical examination is the process by which the physician investigates the body of a patient for signs of disease. ... Edema (BE: oedema, formerly known as dropsy) is swelling of any organ or tissue due to accumulation of excess fluid. ... This article needs to be wikified. ...

Causes

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. Systolic is the adjective form of systole, typically referring to the contraction activity of the heart. ... Diastolic is the adjective form of diastole referring to relaxation of the heart, between muscle contractions. ... In anatomy, a ventricle is a part of the body filled with fluid. ... Mitral regurgitation (MR), also known as mitral insufficiency, is the abnormal leaking of blood through the mitral valve, from the left ventricle into the left atrium of the heart. ... Mitral stenosis is the incomplete opening of the mitral valve of the heart. ... Pulmonary edema is swelling and/or fluid accumulation in the lungs. ...

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, thyroid diseases^[3], 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^[4]. 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). Human immunodeficiency virus (commonly known as HIV, and formerly known as HTLV-III and lymphadenopathy-associated virus[1][2]) is a retrovirus that is the cause of the disease known as AIDS (Acquired Immunodeficiency Syndrome), a syndrome where the immune system begins to fail, leading to many life-threatening opportunistic... Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein and its branches. ... ... Cross-section of a healthy heart. ... Fen-phen was an anti-obesity medication (an anorectic) which consisted of two drugs: fenfluramine and phentermine. ... Wikipedia does not yet have an article with this exact name. ... Fenfluramine is a drug that was part of the Fen-Phen anti-obesity (the other drug being phentermine). ... Phentermine is a phenethylamine, more importantly a derivitive of amphetamine, primarily used as an appetite suppressant. ... Though histiocytosis can refer to any of several specific diseases, the term is generally used to refer to a rare blood disease that is caused by an excess of white blood cells called histiocytes. ...

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]. Hypoxia is a pathological condition in which the body as a whole (generalised hypoxia) or region of the body (tissue hypoxia) is deprived of adequate oxygen supply. ... Chronic obstructive pulmonary disease (COPD) is an umbrella term for a group of respiratory tract diseases that are characterised by airflow obstruction or limitation. ... Diffuse parenchymal lung disease (DPLD), also known as interstitial lung disease, refers to a group of lung diseases, affecting the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. ... The Pickwickian syndrome, also known as obesity hypoventilation syndrome, is the combination of severe obesity and hypoventilation. ... Sleep apnea (alternatively sleep apnoea) is a common sleep disorder characterized by brief interruptions of breathing during sleep. ... Kaposis sarcoma-associated herpesvirus (KSHV) is the eighth human herpesvirus; its formal name according to the International Committee on Taxonomy of Viruses is HHV-8. ...

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 morphogenic proteins^[6], as well as the 5-HT(2B) gene, which codes for a serotonin receptor^[7]. A genetic disorder, or genetic disease is a disease caused by abnormal expression of one or more genes in a person causing a clinical phenotype. ... In biochemistry, a receptor is a protein on the cell membrane or within the cytoplasm or cell nucleus that binds to a specific molecule (a ligand), such as a neurotransmitter, hormone, or other substance, and initiates the cellular response to the ligand. ... Serotonin (5-hydroxytryptamine, or 5-HT) is a monoamine neurotransmitter synthesized in serotonergic neurons in the central nervous system and enterochromaffin cells in the gastrointestinal tract. ...

Diagnosis

Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12–16 mm Hg (1600–2100 Pa). Definite pulmonary hypertension is present when mean pressures at rest exceed 25 mm Hg (3300 Pa). If mean pulmonary artery pressure rises above 30 mm Hg (4000 Pa) with exercise, that is also considered pulmonary hypertension.

Diagnosis of PAH requires the presence of pulmonary hypertension with two other conditions. Pulmonary artery occlusion pressure (PAOP or PCWP) must be less than 15 mm Hg (2000 Pa) and pulmonary vascular resistance (PVR) must be greater than 3 Wood units (240 dyn•s•cm^-5 or 2.4 mN•s•cm^-5).

Although pulmonary arterial pressure can be estimated on the basis of echocardiography, pressure sampling with a Swan-Ganz catheter provides the most definite measurement. PAOP and PVR can not be measured directly with echocardiography. Therefore diagnosis of PAH requires a cardiac catheterization. A Swan-Ganz catheter can also measure the cardiac output, which is far more important in measuring disease severity than the pulmonary arterial pressure. It has been suggested that Transesophageal_echocardiogram be merged into this article or section. ... In medicine pulmonary artery catheterization is the insertion of a catheter into a pulmonary artery. ... It has been suggested that Transesophageal_echocardiogram be merged into this article or section. ... Cardiac catheterization (heart cath) is the insertion of a catheter into a chamber or vessel of the heart. ... In medicine pulmonary artery catheterization is the insertion of a catheter into a pulmonary artery. ... Cardiac output is the volume of blood being pumped by the heart in a minute. ...

Other diagnostic tests generally include pulmonary function tests, blood tests, electrocardiography (ECG), arterial blood gas measurements, X-rays of the chest (followed by high-resolution CT scanning if interstitial lung disease is suspected), and ventilation-perfusion or V/Q scanning to exclude chronic thromboembolic pulmonary hypertension. Biopsy of the lung is usually not indicated unless the pulmonary hypertension is thought to be secondary to an underlying interstitial lung disease, but lung biopsies are fraught with risks of bleeding due to the high intrapulmonary blood pressure. Clinical improvement is often measured by a "six-minute walk test", i.e. the distance a patient can walk in six minutes. Stability and improvement in this measurement correlate with reduced mortality. Spirometry, also known as Pulmonary Function Testing (PFT), is the measurement of lung function, specifically by measuring the volume and speed of air that can be inhaled and exhaled. ... Blood tests are laboratory tests done on blood to gain an appreciation of disease states and the function of organs. ... ECG may also refer to the East Coast Greenway Lead II An Electrocardiogram (ECG or EKG, abbreviated from the German Elektrokardiogramm) is a graphic produced by an electrocardiograph, which records the electrical voltage in the heart in the form of a continuous strip graph. ... Arterial blood gas measurement is a blood test that is performed to determine the concentration of oxygen, carbon dioxide and bicarbonate, as well as the pH, in the blood. ... In the NATO phonetic alphabet, X-ray represents the letter X. An X-ray picture (radiograph) taken by Röntgen An X-ray is a form of electromagnetic radiation with a wavelength approximately in the range of 5 pm to 10 nanometers (corresponding to frequencies in the range 30 PHz... CAT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... Diffuse parenchymal lung disease (DPLD), also known as interstitial lung disease, refers to a group of lung diseases, affecting the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. ...

Classification

Current classification

In 2003, the 3rd World Symposium on Pulmonary Arterial Hypertension was convened in Venice to modify the classification based on the new understanding of disease mechanisms. The revised system developed by this group provides the current framework for understanding pulmonary hypertension.

The system includes several improvements over the former 1998 Evian Classification system. Risk factor descriptions were updated, and the classification of congenital systemic-to pulmonary shunts was revised. A new classification of genetic factors in PH was recommended, but not implemented because available data were judged to be inadequate.

The Venice 2003 Revised Classification system can be summarized as follows^[8]:

• WHO Group I - Pulmonary arterial hypertension (PAH)
• WHO Group II - Pulmonary hypertension associated with left heart disease
• WHO Group III - Pulmonary hypertension associated with lung diseases and/or hypoxemia
• WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/or embolic disease
• WHO Group V - Miscellaneous

Previous terminology

The terms primary and secondary pulmonary hypertension (PPH and SPH) were formerly used to classify the disease. This led to the assumption that only the primary disease should be treated, and the secondary variety should be ignored. In fact all forms of pulmonary arterial hypertension are treatable. Unfortunately, this classification system still persists in the minds of many physicians, and probably leads to many patients with being denied treatment. This nihilistic approach to pulmonary arterial hypertension may also contribute to underdiagnosis. It is estimated that there are about 100,000 patients with PAH in the US, but only 15-20,000 have been diagnosed. Many others have been misdiagnosed as COPD, asthma, or congestive heart failure. Chronic obstructive pulmonary disease (COPD) is an umbrella term for a group of respiratory tract diseases that are characterised by airflow obstruction or limitation. ... Congestive heart failure (CHF), also called congestive cardiac failure (CCF) or just heart failure, is a condition that can result from any structural or functional cardiac disorder that impairs the ability of the heart to fill with or pump a sufficient amount of blood throughout the body. ...

The term primary pulmonary hypertension (PPH) has now been replaced with idiopathic pulmonary arterial hypertension (IPAH).

Epidemiology

IPAH is a rare disease with an annual incidence is about 1-10 in 1,000,000. Women are almost three times as likely to present with IPAH than men.

Other forms of PAH are far more common. In scleroderma the incidence has been estimated to be 6 to 60% of all patients, in rheumatoid arthritis up to 21%, in systemic lupus erythematosus 4 to 14%, in portal hypertension between 2 to 5%, in HIV about 0.5%, and in sickle cell disease ranging from 20 to 40%. Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. ... Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein and its branches. ...

Diet pills such as Fen-Phen produced an annual incidence of 25-50 per million. Fen-phen was an anti-obesity medication (an anorectic) which consisted of two drugs: fenfluramine and phentermine. ...

Treatment

Treatment is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. Since pulmonary venous hypertension is synonymous with congestive heart failure, the treatment is to optimize left ventricular function by the use of diuretics, beta blockers, ACE inhibitors, etc., or to repair/replace the mitral valve. Congestive heart failure (CHF), also called congestive cardiac failure (CCF) or just heart failure, is a condition that can result from any structural or functional cardiac disorder that impairs the ability of the heart to fill with or pump a sufficient amount of blood throughout the body. ... A diuretic (colloquially called a water pill) is any drug or herb that elevates the rate of bodily urine excretion (diuresis). ... Beta blockers or beta-adrenergic blocking agents are a class of drugs used to treat a variety of cardiovascular conditions and some other diseases. ... Captopril, the first ACE inhibitor ACE inhibitors, or inhibitors of Angiotensin-Converting Enzyme, are a group of pharmaceuticals that are used primarily in treatment of hypertension and congestive heart failure, in most cases as the drugs of first choice. ... The mitral valve, also known as the bicuspid valve, is a valve in the heart that lies between the left atrium (LA) and the left ventricle (LV). ...

In PAH, lifestyle changes, digoxin, diuretics, oral anticoagulants, and oxygen therapy are considered conventional therapy, but have never been proven to be beneficial in a randomized, prospective manner. Digoxin is a cardiac glycoside extracted from the foxglove plant, digitalis. ... A diuretic (colloquially called a water pill) is any drug or herb that elevates the rate of bodily urine excretion (diuresis). ... An anticoagulant is a substance that prevents coagulation; that is, it stops blood from clotting. ...

High dose calcium channel blockers are useful in only 5% of IPAH patients who are vasoreactive by Swan-Ganz catheter. Unfortunately, calcium channel blockers have been largely misused, being prescribed to many patients with non-vasoreactive PAH, leading to excess morbidity and mortality. Calcium channel blockers are a class of drugs with effects on the muscle of the heart and the muscles of the rest of the body. ... In medicine pulmonary artery catheterization is the insertion of a catheter into a pulmonary artery. ...

Vasoactive substances

Three major pathways are involved in abnormal proliferation and contraction of the smooth-muscle cells of the pulmonary artery in patients with pulmonary arterial hypertension. These pathways correspond to important therapeutic targets in this condition and play a role in determining which of four classes of drugs — endothelin receptor antagonist, nitric oxide, phosphodiesterase type 5 inhibitors, and prostacyclin derivatives — will be used. A endothelin receptor antagonist (ERA) is a drug which blocks endothelin receptors. ... The chemical compound nitric oxide is a gas with chemical formula NO. It is an important signaling molecule in the body of mammals including humans, one of the few gaseous signaling molecules known. ... A phosphodiesterase (PDE) is an enzyme that catalyzes the hydrolysis of phosphodiester bonds. ...

Prostacyclin (prostaglandin I₂) is commonly considered the most effective treatment for PAH. Epoprostenol (synthetic prostacyclin, marketed as Flolan®) is given via continuous infusion that requires a semi-permanent central venous catheter. This delivery system can cause sepsis and thrombosis. Flolan® is unstable, and therefore has to be kept on ice during administration. Since it has a half-life of 3 to 5 minutes, the infusion has to be continuous (24/7), and interruption can be fatal. Other prostanoids have therefore been developed. Treprostinil (Remodulin®) can be given intravenously or subcutaneously, but the subcutaneous form can be very painful. Iloprost (Ilomedin®) is also used in Europe intravenously and has a longer half life. Iloprost (marketed as Ventavis®) is the only inhaled form of prostacyclin approved for use in the US and Europe. This form of administration has the advantage of selective deposition in the lungs with less systemic side effects. Prostacyclin is a member of the family of lipid molecules known as eicosanoids. ... Chemical structure of prostaglandin E1 (PGE1). ... Prostacyclin is a member of the family of lipid molecules known as eicosanoids. ... Prostacyclin is a member of the family of lipid molecules known as eicosanoids. ... In medicine, a central venous catheter (CVC or central (venous) line) is a catheter placed into a large vein in the chest or groin. ... Sepsis (in Greek Σήψις, putrefaction) is a serious medical condition, resulting from the immune response to a severe infection. ... Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... Prostanoid is the term used to describe three classes of eicosanoids: the prostaglandins (mediators of inflammatory and anaphylactic reactions), the thromboxanes (mediators of vasoconstriction) and the prostacyclins (active in the resolution phase of inflamation. ... Trepostinil is a synthetic analogue of prostacyclin, used to treat pulmonary hypertension. ... iloprost, an inhalation solution, is sold under the name Ventavis® and is used to treat pulmonary arterial hypertension (PAH). ... iloprost, an inhalation solution, is sold under the name Ventavis® and is used to treat pulmonary arterial hypertension (PAH). ...

The dual (ET_A and ET_B) endothelin receptor antagonist bosentan (marketed as Tracleer®) was approved in 2001. Two selective endothelin receptor antagonists (ET_A only) are in the final stages of approval: sitaxsentan and ambrisentan. Sildenafil, a selective inhibitor of cGMP specific phosphodiesterase type 5 (PDE5), was approved for the treatment of PAH in 2005. It is marketed for PAH as Revatio®. Endothelin is a 21-amino acid vasoconstricting peptide that plays a key part in vascular homeostasis. ... Bosentan is an endothelin receptor antagonist important in the treatment of pulmonary artery hypertension. ... Sitaxsentan or sitaxsentan sodium (to be marketed as Thelin®) is a small molecule sodium salt that blocks the action of endothelin on the endothelin-A receptor selectively (by a factor of 6000 compared to the ERB), and is undergoing FDA approval for treating pulmonary hypertension. ... Sildenafil citrate, sold under the names Viagra, Revatio and generically under various other names, is a drug used to treat male erectile dysfunction (impotence) and pulmonary arterial hypertension (PAH), developed by the pharmaceutical company Pfizer. ... 2005 (MMV) was a common year starting on Saturday of the Gregorian calendar. ...

Surgical

Atrial septostomy is a surgical procedure that creates a communication between the right and left atria. It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood (hypoxia). It is best performed in experienced centers. Lung transplantation cures pulmonary arterial hypertension, but leaves the patient with the complications of transplantation, and a survival of about 5 years. Atria may refer to: Atria is an alternative spelling for the Etruscan city that is now Adria in the Veneto region of Northern Italy. ...

Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension. It is the surgical removal of an organized thrombus (clot) along with the lining of the pulmonary artery; it is a large and very difficult procedure that is currently performed in San Diego, California and a few other centers. Case series show remarkable success in most patients. In thoracic surgery, a pulmonary thromboendarterectomy, PTE, is an operation that removes organized clotted blood (thrombus) from the pulmonary arteries. ... A thrombus, or blood clot, is the final product of the blood coagulation step in hemostasis. ... It has been suggested that Downtown San Diego be merged into this article or section. ...

Treatment for hypoxic and miscellaneous varieties of pulmonary hypertension have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.

Prognosis

The NIH IPAH registry from the 1980's showed an untreated median survival of 2-3 years from time of diagnosis, with the cause of death usually being right ventricular failure (cor pulmonale). Although this figure is widely quoted, it is probably irrelevant today. Outcomes have changed dramatically over the last two decades. This maybe because of newer drug therapy, better overall care, and earlier diagnosis (lead time bias). A recent outcome study of those patients who had started treatment with bosentan (Tracleer®) showed that 86% patients were alive at 3 years. With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently. It would not be unreasonable to expect median survival to extend past 10 years in the near future. Cor pulmonale is a medical term used to describe a change in structure and function of the right ventricle of the heart as a result of a respiratory disorder. ...

References

1. ^ Romberg E von. Über Sklerose der Lungenarterie. Dtsch Arch Klin Med 1891-1892;48:197-206.
2. ^ 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. PMID 14985486.
3. ^ 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. PMID 10555089.
4. ^ 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. PMID 8692238.
5. ^ 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. PMID 13679525.
6. ^ 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. PMID 10903931.
7. ^ 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. PMID 14659797.
8. ^ Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension. Venice, Italy, June 23-25, 2003. J Am Coll Cardiol 2004 Jun 16;43(12 Suppl S):1S-90S. PMID 15194171.

External links

• The Merck Manual of Diagnosis and Therapy: Pulmonary Hypertension
• PH Central - the internet resource for Pulmonary Arterial Hypertension
• The Pulmonary Hypertension Association Webpage provided by the Pulmonary Hypertension Association
• Facts About Primary Pulmonary Hypertension from the National Heart, Lung, and Blood Institute (NHLBI)
• Webcast: The Changing World of Pulmonary Arterial Hypertension Therapies - American College of CHEST Physicians