Original Articles 27 5 1997 자기공명영상에의한중복위 (Situs Ambiguus) 복합심 장기형환자의전신정맥및폐정맥환류이상 원제환 홍용국 박영환 * 설준희 ** 이승규 ** 최규옥 조범구 * Abstract MR Imaging of Systemic and Pulmonary Venous Return in Congenital Cardiac Defects with Situs Ambiguus Je Hwan Won, M.D., Yong Kook Hong, M.D., Young Hwan Park, M.D.,* Jun Hee Sul, M.D.,** Sung Kyu Lee, M.D.,** Kyu Ok Choe, M.D., Bum Koo Cho, M.D.* Department of Diagnostic Radiology, Cardiovascular Center, Cardiovascular surgery,* Pediatric Cardiology,** College of Medicine, Yonsei University, Seoul, Korea BackgroundPreoperative identification of systemic and pulmonary venous return is essential for surgical design in situs ambiguus. This study was carried out to evaluate anatomy of systemic and pulmonary venous return and to assess clinical efficacy of magnetic resonance imagingmr by comparing with results of cardiac catheterizationcath and echocardiographyecho. Materials and MethodsMR performed on 22 patients with cardiac situs ambiguusright isomerism13, left isomerism9. MR findings were compared with the findings of Cath and Echo for the assessment of diagnostic accuracy of MR in 19 patients. Results 1 Interruptions of IVC with azygous continuation were found in all patients of left isomerism. But IVC was drained to right or left sided atria in right isomerism. 2 Bilateral SVC were found in 13 of 22 situs ambiguusleft isomerism5, right isomerism8. 3 Total anomalous pulmonary venous returnstapvr were found in 7 of 13 right isomerism. Location of vertical veins were as followsprearterialn1, retroarterial-prebronchialn3, retrobronchialn3. In 5 patients of remained 6 right isomerism, pulmonary veins formed common pulmonary vein and then entered to atria. Partial anomalous pulmonary venous returnspapvr in which right and left pulmonary veins entered to right and left atrium respectively, were found in 5 of 9 left isomerism. 4 Compared with Cath and Echo findingsn19 in which MR, Cath and Echo were performed simultaneously, TAPVR were found in 6 cases on MR but 2 cases on Cath and Echo. The cases that were not detected by Cath showed severe decrement of pulmonary flow due to hypoplasia of pul- 514
monary artery or obstruction of pulmonary vein. On MR, accurate anatomy of PAPVR were found only in 4 cases. ConclusionsMR can provide accurate and complete imaging of systemic and pulmonary venous return in situs ambiguus. Especially, MR is superior to Cath or Echo in depiction of TAPVR with severe decrement of pulmonary flow or obstruction of pulmonary vein, PAPVR and bilateral SVC. KEY WORDSHeart Abnormalities Heart MR. 서 론 대상및방법 515
결과 1. 전신정맥환류 1) 하대정맥 2) 상대정맥 2. 폐환류 Fig. 1. Schematic drawings of type of pulmonary venous return in patients with situs ambiguus. TAPVRtotal anomalous pulmonary venous return, PAPVRpartial anomalous pulmonary venous return, CAcommon atrium. Fig. 2. Coronal MR image obtained in patient with left isomerism. IVC interruption with azygous connectionarrows is demonstrated. Arrowheadsdescending thoracic aorta. 516
Table 1. Summary of systemic venous return on MR Left isomerism(n=9) IVC Right isomerism(n=13) Interruption with azygous or hemiazygous To Lt. sided atrium(n=1) continuation(n=9) To Rt. sided atrium(n=2) Bilateral SVC(n=5) Bilater al SVC(n=8) To midline of common atrium(n=1) SVC Interconnecting vein(4/8) LSVC(n=4) RSVC(n=4) LSVC(n=1) IVC inferior vena cava, SVC superior vena cava, LSVC left superior vena cava, RSVC right superior vena cava Table 2. Summary of pulmonary venous return on MR Lt. isomerism(n=9) Rt. isomerism(n=13) TAPVR 7 PAPVR 5 One of two atria 3 5 1 1 Midline of common atrium TAPVR total anomalous pulmonary venous return PAPVR partial anomalous pulmonary venous return Fig. 4. Retroarterial-prebronchial vertical vein in patient with right isomerism. Axial MR image shows vertical vein(asterisk) that is located between pulmonary artery(p) and bronchus(arrows). Fig. 3. Axial MR image shows bilateral superior vena cava(s) and interconnecting vein(arrows in A) in caudad level. ao=rt.sided aortic arch. 공통폐정맥을 형성하였다(Table 4). 양측 폐정맥이 각 각 양측 심방으로 환류되는 부분폐정맥환류(PAPVR) 3)는 각각 좌측 상완두정맥, 반기정맥, 우측 상대정맥 는 좌이성체에서만 5예가 관찰되었다. 그 외의 좌이성 으로 연결되었다. 기관지 뒤에서 주행했던 경우(n 3) 체에서의 폐환류는 우측 심방으로 1예, 좌측심방으로 는 2예에서 기정맥으로, 1예에서 우측 상대정맥으로 2예 및 공통심방(common atrium)의 중앙부 1예 등 연결되었다. 그 외의 우이성체에서의 폐환류는 3예에 으로 환류되었으며 이 중 좌측심방으로 환류되었던 2 서 우측 심방으로, 2예에서는 좌측심방으로 환류되었 예와 공통심방으로 환류되었던 1예에서는 심방 뒤에 으며, 1예에서는 공통심방(common atrium)의 중앙 서 공통폐정맥을 형성한 후 심방으로 환류되는 것이 관 부로 환류되었고, 이들 중 좌측심방으로 연결된 1예를 찰되었다(Table 2, 4). 폐정맥 환류기형의 유무와 관 제외하고는 모두 심방으로 환류되기 전에 심방 뒤에서 계없이 위와 같이 공통폐정맥을 형성한 경우는 좌이성 517
Fig. 5. Retrobronchial vertical vein in patient with right isomerism. A, Both pulmonary veins(white arrows) enter common pulmonary vein(black arrow). B and C, The common pulmonary vein(arrow) courses vertically behind the bronchus(arrowheads). P=pulmonary artery. D, Finally, the common pulmonary vein(arrow) enters left sided superior vena cava(s). Table 3. Associated pulmonary venous or atrial anomaly Table 4. Comparison of MR findings with those of Cath and Echo findings in 19 patients Lt. isomerism(n=9) Rt. isomerism(n=13) CPV draining to MR Cath Echo 2 2 3 12 TAPVR 6 Lt. sided atrium (n=2) Midline of CA (n=1) Systemic vein (n=7) Midline of CA (n=1) Rt. or Lt. Sided atrium(n=4) PAPVR 4 CPV to Lt. or Rt. atrium 8 Bilateral SVC 1 7 8 Cor-triatriatum 2 CPV common pulmonary vein, CA common atrium 체에서 3예(3/9), 우이성체에서 12예(12/13)로 우이 성체에서 빈도가 월등히 많았다. 공통 심방내 폐정맥 이 환류되는 위치에 두개의 높은 신호강도의 격벽이 관 찰되는 삼심방(Cortriatriatum)은 좌이성체에서만 2 예가 관찰되었다(Table 3, Fig. 6). 그러나 격벽의 내 경이 커서 혈역학적 폐쇄는 없었다. 8 8 5 IVC interruption MR magnetic resonance imaging, Cath cardiac catheterization, Echo echocardiography, TAPVR total anomalous pulmonary venous return, PAPVR partial anomalous pulmonary venous return, CPV common pulmonary vein, IVC inferior vena cava, SVC superior vena cava 의 결과를 비교해 보면, TAPVR은 MR이 6예, 심혈 관조영술과 심초음파에서는 각각 2예에서 각각 관찰하 였다. 심장혈관조영술에서 관찰하지 못했던 4예는 폐 혈류가 감소되었거나 폐순환시간이 길어져 폐정맥폐 3. 심장혈관조영술, 심초음파 및 수술소견 쇄가 의심되었으며, MR에서는 폐동맥 직경의 감소소 견을 보이는 폐동맥 형성부전(pulmonary arteral hy- 과의 비교 MR과 심장혈관조영술을 함께 시행한 19명의 환자 poplasia) 혹은 폐정맥 및 공통폐정맥 내강의 고신호 518
Fig. 6. Cor triatriatum in patient with left isomerism. Axial image shows high signal septumlarge arrows in left sided atrium. Pulmonary veinssmall arrows is drained to accessory chamber. 고안 519
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요약 연구배경 : 방법 : 결과 : 521
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