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Primary pulmonary hypertension

In medicine, pulmonary hypertension (PH) or pulmonary artery hypertension (PAH) is an increase in blood pressure in the pulmonary artery or lung vasculature. more...

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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.

Signs and symptoms

A history usually reveals gradual onset of shortness of breath, fatigue, angina pectoris, syncope (fainting) and peripheral edema.

In order to establish the cause, the physician will generally conduct a thorough medical history and physical examination. A detailed family history is taken to determine whether the disease might be familial.

Diagnosis

Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12-16mmHg. Definite pulmonary hypertension is present when mean pressures at rest exceed 25 mmHg. Although pulmonary arterial pressure can be estimated on the basis of echocardiography, pressure sampling with a Swan-Ganz catheter provides the most definite measurement.

Diagnostic tests generally involve blood tests, electrocardiography, arterial blood gas measurements, X-rays of the chest (generally followed by high-resolution CT scanning). Biopsy of the lung is usually not indicated unless the pulmonary hypertension is thought to be secondary to an underlying intrinsic lung disease. Clinical improvement is often measured in a "six-minute walking test", i.e. the distance a patient can walk in six minutes, and stability and improvements in this measurement correlate with reduced mortality.

Causes and mechanisms

Pulmonary hypertension can be primary (occurring without an obvious cause) or secondary (a result of other disease processes.)

Primary PH

Primary pulmonary hypertension (PPH) is considered a genetic disorder. Certain forms of PPH have been linked to mutations in the BMPR2 gene, which encodes a receptor for bone morphogenic proteins, as well as the 5-HT(2B) gene, which codes for a serotonin receptor. Recently, characteristic proteins of human herpesvirus 8 (also known for causing Kaposi sarcoma) were identified in vascular lesions of PPH patients. However, it is not understood what roles these genes and viral particles play in PPH. PPH has also been associated to the use of appetite suppressants (e.g. Fen-phen). While genetic susceptibility to adverse drug reactions is suspected, the cause of the disease is still largely unknown.

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Hyperplasia of pulmonary artery smooth muscle cells is causally related to overexpression of the serotonin transporter in primary pulmonary hypertension
From CHEST, 3/1/02 by Saadia Eddahibi

Abbreviations: L = long; PA-SMC = pulmonary artery smooth muscle cell; PPH = primary pulmonary hypertension

Hyperplasia of pulmonary artery smooth muscle cells (PA-SMCs) is a hallmark pathologic feature of primary pulmonary hypertension (PPH). Herein, we found that PA-SMCs from patients with PPH grow faster than those from control subjects when stimulated by serotonin or serum and that these effects are due to the increased expression of the serotonin transporter, which mediates the internalization of indolamine. The increased growth responsiveness of PA-SMCs from patients with PPH was selective to serotonin since it did not occur with growth factors such as epidermal growth factor, transforming growth factor-[beta], fibroblast growth factor, or platelet-derived growth factor. In the presence of 5-HTT inhibitors, the growth stimulatory effects of serum and serotonin were markedly reduced, and the difference between the growth of PA-SMCs between patients and control subjects was no longer observed. The expression of 5-HTT was increased in cultured cells from patients with PPH compared to those from control subjects, with an increase of two to three times in 5-HTT messenger BNA, 5-HTT protein, and 5-HTT uptake activity. Increased 5-HTT expression also was found in the platelets and lungs of patients with PPH in whom it predominated in the media of thickened pulmonary arteries and in onion-bulb lesions. Because a 5-HTT-linked promoter region insertion/ deletion polymorphism with long (L) and short forms affects 5-HTT expression and function, we additionally examined the role of this polymorphism in PPH. Cells homozygous for the L variant showed increased 5-HTT expression and increased growth responses to 5-HT and serum. In a population of 89 patients with PPH and 84 control subjects, we found that the biallelie LL condition was present in 65% of the patients with PPH compared to 27% of the control subjects. These results indicate that 5-HTT plays a key role in the pathogenesis of PA-SMC proliferation in patients with PPH and that the L variant of the 5-HTT gene polymorphism represents a genetic susceptibility for developing PPH.

* From INSERM U492 et Departement de Physiologie (Drs. Eddahibi, Raffestin, and Adnot), Creteil, France; Service de Pneumologie (Drs. Humbert, Capron, and Simonneau), Clamart France; INSERM U288 (Drs. Darmon and Hamon), Paris, France; and Service de Chirurgie Thoracique (Drs. Fadel and Dartevelle), Le Plessis Robinson, France.

Correspondence to: Serge Adnot, MD, PhD, INSERM Unite 492, Faculte de Medecine, 8, rue du General Sarrail, 94010 Creteil Cedex, France; e-mail: secr.u492@im3.inserm.fr

COPYRIGHT 2002 American College of Chest Physicians
COPYRIGHT 2002 Gale Group

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