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      Prenatal diagnosis of atrial isomerism in the Korean population = Prenatal diagnosis of atrial isomerism in the Korean population

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      https://www.riss.kr/link?id=A100014193

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      다국어 초록 (Multilingual Abstract)

      Objective To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. Methods A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institut...

      Objective To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. Methods A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. Results Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. Conclusion Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

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      참고문헌 (Reference) 논문관계도

      1 Berg C, "The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases" J Ultrasound Med 24 : 921 ~ 931, 2005

      2 이미영, "Technique of fetal echocardiography" Obstetrics & Gynecology Science 56 (04) : 217 ~ 226, 2013

      3 Rose V, "Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis" Br Heart J 37 : 840 ~ 852, 1975

      4 Taketazu M, "Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome" Am J Cardiol 97 : 720 ~ 724, 2006

      5 Waldman JD, "Sepsis and congenital asplenia" J Pediatr 90 : 555 ~ 559, 1977

      6 Yan YL, "Right atrial isomerism: preponderance in Asian fetuses. Using the stomachdistance ratio as a possible diagnostic tool for prediction of right atrial isomerism" Ann Acad Med Singapore 37 : 906 ~ 912, 2008

      7 Eronen M, "Right atrial isomerism in four siblings" Pediatr Cardiol 25 : 141 ~ 144, 2004

      8 Kaasinen E, "Recessively inherited right atrial isomerism caused by mutations in growth/differentiation factor 1 (GDF1)" Hum Mol Genet 19 : 2747 ~ 2753, 2010

      9 Ruano R, "Prenatal sonographic diagnosis of congenital hiatal hernia" Prenat Diagn 24 : 26 ~ 30, 2004

      10 Pepes S, "Prenatal diagnosis of left atrial isomerism" Heart 95 : 1974 ~ 1977, 2009

      1 Berg C, "The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases" J Ultrasound Med 24 : 921 ~ 931, 2005

      2 이미영, "Technique of fetal echocardiography" Obstetrics & Gynecology Science 56 (04) : 217 ~ 226, 2013

      3 Rose V, "Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis" Br Heart J 37 : 840 ~ 852, 1975

      4 Taketazu M, "Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome" Am J Cardiol 97 : 720 ~ 724, 2006

      5 Waldman JD, "Sepsis and congenital asplenia" J Pediatr 90 : 555 ~ 559, 1977

      6 Yan YL, "Right atrial isomerism: preponderance in Asian fetuses. Using the stomachdistance ratio as a possible diagnostic tool for prediction of right atrial isomerism" Ann Acad Med Singapore 37 : 906 ~ 912, 2008

      7 Eronen M, "Right atrial isomerism in four siblings" Pediatr Cardiol 25 : 141 ~ 144, 2004

      8 Kaasinen E, "Recessively inherited right atrial isomerism caused by mutations in growth/differentiation factor 1 (GDF1)" Hum Mol Genet 19 : 2747 ~ 2753, 2010

      9 Ruano R, "Prenatal sonographic diagnosis of congenital hiatal hernia" Prenat Diagn 24 : 26 ~ 30, 2004

      10 Pepes S, "Prenatal diagnosis of left atrial isomerism" Heart 95 : 1974 ~ 1977, 2009

      11 Celentano C, "Prenatal diagnosis of interrupted inferior vena cava as an isolated finding : A benign vascular malformation" Ultrasound Obstet Gynecol 14 : 215 ~ 218, 1999

      12 Berg C, "Prenatal diagnosis of cardiosplenic syndromes:a 10-year experience" Ultrasound Obstet Gynecol 22 : 451 ~ 459, 2003

      13 Bronshtein M, "Prenatal diagnosis and outcome of isolated interrupted inferior vena cava" Am J Obstet Gynecol 202 : 398.e1 ~ 398.e4, 2010

      14 Phoon CK, "Left atrial isomerism detected in fetal life" Am J Cardiol 77 : 1083 ~ 1088, 1996

      15 Ho SY, "Isomerism of the atrial appendages in the fetus" Pediatr Pathol 11 : 589 ~ 608, 1991

      16 Lin JH, "Intrauterine diagnosis of heterotaxy syndrome" Am Heart J 143 : 1002 ~ 1008, 2002

      17 Chang J, "Intestinal rotation and fixation abnormalities in heterotaxia: early detection and management" J Pediatr Surg 28 : 1281 ~ 1284, 1993

      18 Ota N, "Improving outcomes of the surgical management of right atrial isomerism" Ann Thorac Surg 93 : 832 ~ 838, 2012

      19 Freedom RM, "Hearts with isomerism of the right atrial appendages: one of the worst forms of disease in 2005" Cardiol Young 15 : 554 ~ 567, 2005

      20 Simpson J, "Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs" J Med Genet 10 : 303 ~ 304, 1973

      21 Ticho BS, "Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures" Am J Cardiol 85 : 729 ~ 734, 2000

      22 Nakada K, "Digestive tract disorders associated with asplenia/polysplenia syndrome" J Pediatr Surg 32 : 91 ~ 94, 1997

      23 Atkinson DE, "Diagnosis of heterotaxy syndrome by fetal echocardiography" Am J Cardiol 82 : 1147 ~ 1149, 1998

      24 Hsu JY, "Clinical implication of hiatal hernia in patients with right isomerism" Acta Paediatr 94 : 1248 ~ 1252, 2005

      25 Lim JS, "Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes" Circulation 112 : 2454 ~ 2461, 2005

      26 Vazquez J, "Biliary atresia and the polysplenia syndrome: its impact on final outcome" J Pediatr Surg 30 : 485 ~ 487, 1995

      27 Sheley RC, "Azygous continuation of the interrupted inferior vena cava: a clue to prenatal diagnosis of the cardiosplenic syndromes" J Ultrasound Med 14 : 381 ~ 387, 1995

      28 Zlotogora J, "Asplenia and polysplenia syndromes with abnormalities of lateralisation in a sibship" J Med Genet 18 : 301 ~ 302, 1981

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