Thursday 28 November 2013

Pulmonary vascular disease – Pulmonary arterial hypertension – The Risk factors

Pulmonary vascular disease is defined as a condition of blood flow to the lung’s artery is blocked suddenly due to a blood clot somewhere in the body, including pulmonary embolism, chronic thromboembolic disease, pulmonary arterial hypertension, pulmonary veno-occlusive disease, pulmonary arteriovenous malformations, pulmonary edema, etc.
Pulmonary arterial hypertension
Pulmonary arterial hypertension is a subgroup of a specific subgroup of pulmonary hypertension (PH) defined as a condition of slowly progressive disorder as a result of abnormally high blood pressure in the blood vessel, including pulmonary artery, pulmonary vein, or pulmonary capillaries, that carries blood from the heart to the lungs due to narrowing in diameter of most of the very small arteries throughout the lungs of that increased resistance to blood flow, leading to right heart failure and death. Because the phrase pulmonary arterial hypertension is long and pulmonary hypertension is a bit shorter the phrase. Pulmonary hypertension is often used in place of pulmonary arterial hypertension(a). According to statistic, approximately over 1,000 new cases of pulmonary arterial hypertension are diagnosed each year, in the United States alone.
B. Risk factors
1. Gender
If you women, you are at increased risk to develop Pulmonary arterial hypertension(8a)
2. Atrial septal defect (ASD)
In the study to analyze risk factors of pulmonary arterial hypertension (PAH) in patients with atrial septal defect (ASD) patients living at above 2000 m high altitude area, found that PAH prevalence was 68.4% (360/526) in ASD patients. The risk factors of developing PAH in these ASD patients were defect size (OR: 1.200, 95%CI: 1.156 – 1.246, P = 0.000), age (OR: 1.027, 95%CI: 1.003 – 1.052, P = 0.025) and altitude (OR: 1.389, 95%CI: 1.001 – 1.637, P = 0.043) while gender and nationality were not risk factors for PAH. The incidence of developing PAH increased with aging (P = 0.000). The standardized ratio of PAH at ≥ 3500 m was 74.8% which was significantly higher than that at 2000 – 2499 m altitude (66.2%, P = 0.005) and at 2500 – 3499 m altitude (66.9%, P = 0.005)(8).
3. Congenital heart disease
Patients with cardiac defects which result in left-to-right shunting are at risk of developing PAH, owing to the increased shear stress and circumferential stretch induced by increased pulmonary blood flow, which leads to endothelial dysfunction and progressive vascular remodelling and, thus, increased pulmonary vascular resistance, according to the study by the Royal Brompton Hospital, Imperial College(9).
4. Other risk factors
In the multicenter study included patients followed at 5 rheumatology units that were systematically assessed using a pretested questionnaire on clinical and immunological variables, focusing on PAH, showed that of a total of 349 patients with SSc, 61 (17%) met the criteria for PAH. Pulmonary fibrosis [adjusted odds ratio (AOR) 7.37, 95% CI 3.67-14.81, p < 0.0001], microstomia (AOR 3.3, 95% CI 1.70-6.28, p < 0.0001), gastroesophageal reflux (AOR 2.41, 95% CI 1.31-4.43, p = 0.005), dysphagia (AOR 2.7, 95% CI 1.49-4.77, p = 0.001), hyperpigmentation (AOR 2.15, 95% CI 1.11-4.16, p = 0.02), and hypopigmentation (AOR 2.4, 95% CI 1.26-4.64, p = 0.008) were the most prevalent clinical characteristics associated with PAH, while anemia (AOR 5.4, 95% CI 1.98-14.93, p = 0.001) was observed as the unique laboratory risk factor(10). Other study showed that increased incidence of the disease in young women, the familial cases, the association with autoimmune disorders, and the recent discovery that mutation of the PPH1 gene may not be restricted to familial PPH support the hypothesis that the development of pulmonary hypertension likely implies an individual susceptibility or predisposition, which is probably genetically determined. It is also now commonly believed that the development of pulmonary hypertension in some of these predisposed individuals could be hastened or precipitated by various expression factors (some of them yet unrecognized), such as ingestion of certain drugs or diets, portal hypertension, or HIV infection(11).
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Sources
(a) http://pulmonaryhypertensionrn.com/ph-vs-pah
/(8a) http://www.ncbi.nlm.nih.gov/pubmed/23625955
(9) http://www.ncbi.nlm.nih.gov/pubmed/20956136
(10) http://www.ncbi.nlm.nih.gov/pubmed/18203318
(11) http://www.ncbi.nlm.nih.gov/pubmed/11590841

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