Characteristic _ general knowledge of the new categorised method of high pressure of the lung artery

Characteristic _ general knowledge of the new categorised method of high pressure of the lung artery
According to the artery high pressure (PAH) to the lung in recent years Pathologic physiology and diagnosing technology research, all kinds of PAH clinical manifestation, new therapeutic method and preventing the understanding of the characteristic, the third world PAH meeting circulates the high pressure (PH) to the lung Clinical criteria for classification carry on revise. This revision has maintained basic frame and spirit that Evian was classified of 1998, ones that upgraded PAH are apt to suffer from factor and relevant factor. Compared with old categorised method, more overall as PH new categorised method of the tool, operation is easier, help the clinician to assess the condition, make standardized treatment and precautionary measures, also more easy to popularize. The new categorised method has the following characteristics:
1. Stop using " Artery high pressure of primary lung (PPH) " Diagnosis name
Research of the past 20 years brief on, the intersection of appetite and inhibitor, desmosis, the intersection of door and the intersection of pulse and high pressure or HIV (HIV) It can also cause vascular pathological change of the lung to infect, its pathology changes and is similar to PPH with the clinical feature, in order to be different from PPH, PAH caused by these diseases is classify as then sending PAH. Because " then sending " The diagnosis name of PAH is not only helpless to diagnosing and treating, but also easy to cause obscuring, so out of use already in Evian that make classify at the second Meeting of world PAH; The time to use in clinic and scientific research in view of the diagnosis name of PPH is for more than more than 50 years, and is familiar with and accept medical circle extensively, so still kept. Have already found, the albumen receptor 2 (BMPR2) takes place in the bone shape in recent years It is important cause of disease of PPH, the intersection of PPH and obviously applicable already name this to be unusual. So in new classification, with specially taking place artery high pressure of lung (IPAH) Diagnosis name replace PPH.
2. Increase the family high-pressure classification of lung artery based on science of heredity
Nearly have 50% artery high pressure of lung (FPAH) of family nature Relate to gene mutation of code BMPR2 on the chromosome 2q33 with the foundation of science of heredity of 10% of the distributing IPAH, BMPR2 the intersection of kinase and structure make the intersection of receptor and the intersection of function and dominant inactivation, lung artery smooth muscle cell (PASMs) unusually Hyperplasia withers and dies to resist, has caused PAH. But the intersection of BMPR2 and gene mutation show rate to be high outside, carry BMPR2 in the crowd of gene mutation, only have PAH can happen in 15%~20%. Only there are reports that 70 example BMPR2 gene mutation caused IPAH in the world so far. PH that vascular pathological change of the lung causes still relates to other inherent causes and environmental factors, activate the plain receptor kind and surge the enzyme (ALK-1) Gene and 5 hydroxy color amine carrier the mutations of gene,too and IPAH and a few heredity bleeding the capillaries be had something to do to be lasted PAH where disease caused.
3. Stop up the disease (PVOD) to the pulmonary vein With the occuring frequently thin hemangioma (PCH) of mao of lung Classify again
In PH criteria for classification in 1998, PVOD and PCH were divided in two different classifications respectively, PVOD is classify as the high pressure of pulmonary veined, PCH is returned in order to be damaged PAH that disease of the lung blood vessel bed causes directly. Subsequent research suggestion, have hemosiderin of lung that is deposited in the lung essences of PVOD and PCH, among expand quality lungs edema, lymphatic vessel, increase membrane thick and changing at media smooth muscle hyperplasia and artery clump kind in lung artery, and while usually treating with the forefront ring, lung edema may happen in the two. This shows two kinds of perhaps overlap exist. Because the clinical manifestation of the two is similar to IPAH, so classified it again. PVOD and PCH prognostic difference, are made a definite diagnosis of, should consider the lung is transplanted.
4. Circulate the shunting further classification of disease to the inborn body - lung
And operation classify to intensity that defect correct according to the intersection of congenital heart disease and flawed or damaged position, size, belong to getting complicated, this can explain the condition development of congenital heart disease of different causes of disease and expand different responses of treating to the blood vessel. The time that PAH happens relates to flawed or damaged position, in simple heart defect, the artery conduit has not closed to happen with PAH of ventricular septal defect for time and should be earlier than atrial septal defect, unite the intersection of room and ventricular septal defect or the intersection of lung and the intersection of artery and aorta do together other complicated PAH of congenital heart disease take place time to be early. In addition, it divides flow to be relevant in size with defect still, diameter in ventricular septal defect less than or equal to 1.5 cm, divide little flow, the intersection of Eisenman and dose (eisenmenger) The incidence of syndrome is only 3%; And act as flawed or damaged gt; 1.5 Cm, when being large to divide into flow, Eisenman doses of incidence of syndrome up to 50%. So, can prevent the occurrence of PAH to pay correcting to the heart malformations in early days.
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