복합심장기형과 동반된 폐의 얼기형성동맥병증 - 2예 보고 -
Plexogenic Pulmonary Arteriopathy in Congenital Heart Disease: A Report of Two Cases
하승연, 박국양, 조현이, 오영하, 정재걸, 정동해, 김정열, 김한겸,
소속 상세정보
하승연 ( Ha Seung-Yeon )
가천의대 길병원 해부병리학과
박국양 ( Park Kook-Yang )
가천의과학대학교 길병원 흉부외과
조현이 ( Cho Hyun-Yee )
가천의대 길병원 해부병리학과
오영하 ( Oh Young-Ha )
가천의대 길병원 해부병리학과
정재걸 ( Chung Jae-Gul )
가천의대 길병원 해부병리학과
정동해 ( Chung Dong-Hae )
가천의대 길병원 해부병리학과
김정열 ( Kim Chung-Yeul )
고려대학교 의과대학 병리학교실
김한겸 ( Kim Han-Kyeom )
고려대학교 의과대학 병리학교실
KMID : 0357920020360060412
Abstract
Hypertensive pulmonary vascular disease can develop in those cases of congenital cardiac shunt in which critical levels of pulmonary artery pressure and flow are reached and exceeded. We have experienced two cases of plexogenic arteriopathy in complex congenital heart disease and tried to evaluate of distribution of arterial lesions by total mapping of the explanted lung. Case 1 and 2 were 12-year-old boy and 36 year-old man. They were treated with combined heart-lung transplantation. Mapping of the both lungs was done, and graded according to Heath and Edward´s grading scheme. The elastic pulmonary artery was tortuous, dilated and aortic configuration. Both lungs showed mostly grade 3. Plexiform lesion or veinlike branches of hypertrophied muscular arteries arosed in a lateral branch of a muscular artery that might be proximal to an area of occlusion. Comprising the right and left lung, the right was more severe than the left. By getting closer to the distal part, the grade tended to increase to 4 to 5. By analyzing the pulmonary lobe, severe pulmonary hypertension of grade 4 or 5 was comparatively disseminated throughout the right lung. On the other hand, in the left lung, the grade of the lower lobe was higher than that of the upper lobe, and within the upper lobe, there was a tendency for the grade of inferior segment to be higher than that of the corresponding apical segment.
키워드
Hypertension;Pulmonary;Congenital Heart Disease
원문 및 링크아웃 정보
등재저널 정보