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선천성 폐동맥관 협착증의 임상적 고찰

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 (A) clinical study of isolated congenital pulmonary stenosis 
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[한글] 선천성 심장병의 진단이 심도자술과 심조영술의 발달로 용이하게 됨에 따라 선천성 폐동맥판협착증의 진단과 병태생리가 잘 알려지게 되었다. 저자는 1963년 12월부터 1977년 6월까지 13년 7개월간 연세대학교의과대학 부속 세브란스병원에서 심도자술로 확진된 폐동맥판협착증 47예를 대상으로 심전도, 심음도, 흉부 X-선 및 협착의 유형과 혈역학과의 상호관계를 연구분석하여 다음과 같은 결과를 얻었다. 1. 심전도와 혈역학과의 상호관계 ① 우심실수축기압(Right Ventricular Systolic Pressure; RVSP)과 RV, 크기 사이에는 RVSP=3.32RV^^1 + 48.2의 관계가 있었고 상관계수는 0.818로 통계학적 의의(P=0.000)가 있었다. ② RVSP와 QRS의 전평면전기축과의 관계에서는 RVSP가 높을수록 우측변위를 나타내었다. (r=0.55, P=0.001) ③ 심전도의 폐성P, 우심실비대나 우심견이 (right ventrioular strain)이 있는 군의 RVSP의 평균치는 없는 군의 평균치보다 의의(P=0.032, 0.000, 0.000)있게 높았다. ④ RV^^1 이 20mm이상인 군에서 RVSP의 평균치가 이하 군의 평균치보다 높았다. (P=0.0000). ⑤ RVSP에 따라 49mmHg 이하, 50-99mmHg 및 100mmHg 이상 3군으로 나눌 때 심전도 소견, 폐성P, 우심실비대나 우심견인의 양성율은 압력증가에 따라 유의성(x**2 = 8.96, 26.69, 1906 ; P=0.0114, 0.0000, 0.0001)있게 증가하였다. 2. 심음도와 혈역학과의 상호관계는 방출성수축기심잡음의 최고봉이 수축기 중앙 뒤에 오는 군의 RVSP의 평균치가 중앙 이전에 오는 군에 비해 높았다 (P=0.014). 3. 흉부 X-선과 혈역학과의 상호관계에서는 폐동맥음영이 감소하는 군, 협착후부확대나 우심실비대가 있는 군의 RVSPD의 평균치가 정상이거나 없는군에 비해 높았다(P=0.000, 0.005, 0.015). RVSP의 상기 세 압력군의 변화와 흉부 X-선 소견 사이에는 압력이 높은 군 에서 의의(X**2 =7.55, 10.94, 13.36 ; P=0.0229, 0.0042, 0.0013)있게 많은 X-선의 양성율을 보였다. 4. 협착의 유형과 혈역학의 상호관계에서는 복합형이 단독형(판막형이나 누두형)보다 RVSP의 평균치가 높았다(P=0.0003).
[영문] Isolated pulmonary stenosis, a relatively common congenital anomaly that accounts for about 10 percent of all congenital heart disease, is characterized by stenosis of pulmonary valve itself, infundibulum or both of them, Since cardiac catheterizatin was applied to man by Cournand and Ranges, pulmonary stenosis has been easily diagnosed and may clinical studies were investigated. It has a wide clinical spectrum depending on the degree of stenosis. The electrocardiogram, phonocadiogram, and chest X-ray have proved useful in estimating the severity of hemodynamic factors in individual cases. This series comprises 47 cases in whom the clinical disgnosis of isolated pulmonary stenosis was confirmed by right heart catheterization with cardiac cineangiography at Severance Hospital, Yonsel University. An attempt was made to correlate the electrocardiographic, phonocardiographic and chest X-ray findings, and types of stenosis with the hemodynamics data in these cases. 1. Of 47 patients, 33 were male and 14 female. Their ages ranged from 2 to 42 years: The mean age was 19.1 years. 2. The incidence was 5.9 percent of all 797 catheterized congenital heart disease cases. The pulmonary valvular stenosis was 30(68.3%), infundibular 7(14.6%) and combined 10(21.6%). 3. The correlation between electrocadiorgram and hemodynamic data were as follows. ⅰ) The regression equation between right ventricular systolic pressure(RVSP) and height of R wave in V^^1 lead(RV^^1) was RVSP=3.32RV^^1 +48.2; its correlation coefficient was 0.818 and it was very significant(p=0.000). ⅱ) The higher the RVSP, the more the frontal axis of QRS complex shifted to the right side(r=0.55, p=0.001). ⅲ) The RVSP of the groups with positive EKG findings such as p-pulmonale, right ventricular hypertrophy or right ventricular strain were much higher than the RVSP of the groups without such findings(p=0.032, 0.000, 0.000). ⅳ) The group with RV^^1 higher than 20㎜ showed much more elevated mean of RVSP than the group with lower RV^^1 (p=0.000). ⅴ) The groups with the above mentioned positive EKG findings showed good correlations with the severity of RVSP which was arbitrarily classified as 49 or less, 50-99, 100mmHg or more (X**2 =8.96, 26.69, 19.06; p=0.0114, 0.0000, 0.0001). 4. The group with late occurrence of the maximum intensity of the ejection systolic murmur showed higher mean of RVSP than the group with early peak of the murmur(p=0.014). 5. The mean of RVSP of the groups with chest X-ray findings such as decreased pulmonary vascularity, post-stenotic dilatation or right ventricular hypertrophy were much higher than the means of RVSP of the groups withput such findings(p=0.000, 0.005, 0.015). The groups with above mentioned positive chest X-ray findings showed good correlations with the severity of RVSP which was classified as above limits(X**2 =7.55, 10.94, 13.36; p=0.0229, 0.0042, 0.0013). 6. Combined pulmonary valvular and infundibular stenosis showed more severe systolic pressure gradient and higher mean of RVSP than the isolated types(p=0.0003).
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