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ダウン症候鮮(DS)と肺高血圧症(PH)には密接な関係がある。一般には先天性心疾患(CHD)を合併する症例にPHを認めるが1〜4),CHDを合併しないDS小児でもupper airway obstraction,pulmonary hypopla—sia9),primary pulmonary vascular disease2,3)などにより肺血管抵抗が増加しPHを生じることが報告されている。PHの存在を確認するためには心臓カテーテル検査が必要であるが,非観血的検査法である心エコー法を用いてPHを推定する方法もなされており,Mモード心エコー図によるRPEP/RVET (right pre-ejectionperiod/tright ventricular ejection time)の増加10),肺動脈弁"a"dipの消失11)として認められる。今回私達はこの心エコー法を用い,CHDを合併しないDS小児におけるPHの有無について検討を行ったので報告する。
We studied echocardiographic assessment of pul-monary hypertension in the patients with Down syndrome who have no congenital heart disease. Right ventricular pre-ejection period/right ventricu-lar ejection time (RPEP/RVET) were measured from pulmonary valve echo in 23 patients and 20 normal children. Absence of the "a" dip of pulmonary valve and presence of right ventricular dilatation were evaluated too. The mean -SD of RPEP/RVET was 0.24±0. 04 (range 0.16~0.35) in the patients and 0.21±0. 03 (range 0.15~4.26) in normal children. The mean RPEP/RVET was significantly greater in the patients than in normal children (p<0.05). In the patients, RPEP/RVET greater than 0.35 was found in one case, absence of the "a" dip in 3 cases, and right ventricular dilatation in one case. These data suggested that presence of pulmonary hyperten-sion should be considered in the patients with Down syndrome who have no congenital heart disease.
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