儿童肺动脉高压的诊治_傅立军.pptVIP

31 Idiopathic PAH: Survival Untreated - survival worse in children Adult median survival: 2.8 years (n=194) Pediatric median survival: 0.8 years (n=16) 0 20 40 60 80 100 0 0.5 1 1.5 2 2.5 3 3.5 4 4.5 5 Years of Follow-up % S u r v i v a l DAlonzo, et al. Ann Internal Med 1991 (n=194) 68% 48% 34% 32 Pathology and Pathophysiology ? Pediatric PH is similar to adult disease in pathobiology, pathophysiology and mechannisms even if the lungs are still developing. ? Adults with IPAH often have “fixed” obstructive vascular changes. In contrast, children with IPAH have more pulmonary vascular medial hypertrophy and less intimal fibrosis and fewer plexiform lesions. Postmortem studies suggest medial hypertrophy was severe in patients younger than 15 years of age, and it was usually the only abnormality seen in infants. ? Younger children appear to have a more reactive pulmonary vascular bed relative to both vasodilatation and vasoconstriction. Severe acute pulmonary hypertensive crises occur in response to pulmonary vasoconstrictor ‘‘triggers more often than in older children or adults. 33 PAH: Diagnostic Evaluation ? History* ? Physical ? Blood ? EKG ? ECHO ? Physiologic* - Lung function - Exercise testing ? Imaging ? Sleep study ? Right Heart Catheterization * Difficult in young children 34 Clinical Features ? Many of the symptoms of pediatric PH are nonspecific. ? Infants with IPAH often present with signs of low cardiac outpu. Some infants have crying spells, presumably as a result of chest pain. In addition, infants may develop cyanosis with exertion, due to right-to-left shunting through a patent foramen ovale. ? After early childhood, children present with similar symptoms as adults. In older children, the most common symptoms are exertional dyspnea and, occasionally, chest pain. ? Peripheral edema is generally a reflection of right ventricular failure, but overt RV failure is a late event and the child may die suddenly before the appearance o