新生児 Ebstein 奇形に対する一期的両心室修復として cone 手術を行った1例
新生児Ebstein奇形に対する一期的両心室修復としてのcone手術の報告はほとんどない．症例は日齢14の女児，胎児エコーで重症Ebstein奇形と診断され帝王切開で出生した．プロスタグランジンが開始され当院に搬送となった．CTRは74%，三尖弁逆流はsevere，肺動脈弁は機能的閉鎖であった．プロスタグランジンを離脱しても動脈管は縮小せず，むしろ肺血管抵抗低下に伴い心不全が悪化したため新生児期での手術介入となった．三尖弁は前尖も高度にplasteringしており，弁の形態的にはCarpentier type Cに相当するものであった．しかしcone手術により三尖弁逆流は制御され，右心機能は改...
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| Published in | 日本心臓血管外科学会雑誌 Vol. 45; no. 6; pp. 262 - 266 |
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| Main Authors | , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
特定非営利活動法人 日本心臓血管外科学会
2016
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0285-1474 1883-4108 |
| DOI | 10.4326/jjcvs.45.262 |
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| Abstract | 新生児Ebstein奇形に対する一期的両心室修復としてのcone手術の報告はほとんどない．症例は日齢14の女児，胎児エコーで重症Ebstein奇形と診断され帝王切開で出生した．プロスタグランジンが開始され当院に搬送となった．CTRは74%，三尖弁逆流はsevere，肺動脈弁は機能的閉鎖であった．プロスタグランジンを離脱しても動脈管は縮小せず，むしろ肺血管抵抗低下に伴い心不全が悪化したため新生児期での手術介入となった．三尖弁は前尖も高度にplasteringしており，弁の形態的にはCarpentier type Cに相当するものであった．しかしcone手術により三尖弁逆流は制御され，右心機能は改善し，肺血流は右室の順行性拍出のみで維持できた．術後6日目に抜管し，術後8日目に集中治療室を退室した．CTRは58%と縮小し，三尖弁逆流はmoderate以下であった．右室機能は良好であり，心房間交通は右左シャントではあるがSpO2 は90%以上であった．Cone手術は三尖弁前尖が低形成で，両心室修復の可否がborder lineであるような新生児Ebstein奇形に対しても有効であった． |
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| AbstractList | 新生児Ebstein奇形に対する一期的両心室修復としてのcone手術の報告はほとんどない．症例は日齢14の女児，胎児エコーで重症Ebstein奇形と診断され帝王切開で出生した．プロスタグランジンが開始され当院に搬送となった．CTRは74%，三尖弁逆流はsevere，肺動脈弁は機能的閉鎖であった．プロスタグランジンを離脱しても動脈管は縮小せず，むしろ肺血管抵抗低下に伴い心不全が悪化したため新生児期での手術介入となった．三尖弁は前尖も高度にplasteringしており，弁の形態的にはCarpentier type Cに相当するものであった．しかしcone手術により三尖弁逆流は制御され，右心機能は改善し，肺血流は右室の順行性拍出のみで維持できた．術後6日目に抜管し，術後8日目に集中治療室を退室した．CTRは58%と縮小し，三尖弁逆流はmoderate以下であった．右室機能は良好であり，心房間交通は右左シャントではあるがSpO2 は90%以上であった．Cone手術は三尖弁前尖が低形成で，両心室修復の可否がborder lineであるような新生児Ebstein奇形に対しても有効であった． |
| Author | 夷岡, 徳彦 新井, 洋輔 浅井, 英嗣 橘, 剛 |
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| References | 4) Davies, R.R., Pasquali, S.K., Jacobs, M.L. et al. : Current spectrum of surgical procedures performed for Ebstein's malformation : an analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann. Thorac. Surg. 96 : 1703-1710, 2013. 12) Mizuno, M., Shiraishi, I., Ichikawa, H. et al. : Application of cone reconstruction for neonatal Ebstein anomaly or tricuspid valve dysplasia. Ann. Thorac. Surg. 101 : 1811-1817, 2016. 9) Knott-Craig, C.J., Goldberg, S.P., Kirklin, J.K. et al. : Repair of neonates and young infants with Ebstein's anomaly and related disorders. Ann. Thorac. Surg. 84 : 587-593, 2007. 6) Reemtsen, B.L., Fagan, B.T., Starnes, V.A. et al. : Current surgical therapy for Ebstein anomaly in neonates. J. Thorac. Cardiovasc. Surg. 132 : 1285-1289, 2006. 7) Reemtsen, B.L., Polimenakos, A.C., Starnes, V.A. et al. : Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J. Thorac. Cardiovasc. Surg. 134 : 1406-1412, 2007. 3) Shinkawa, T., Polimenakos, A.C., Gomez-Fifer, C.A. et al. : Management and long-term outcome of neonatal Ebstein's anomaly. J. Thorac. Cardiovasc. Surg. 139 : 354-358, 2010. 1) Carpentier, A., Chauvaud, S., Mace, L. et al. : A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J. Thorac. Cardiovasc. Surg. 96 : 92-101, 1988. 10) Boston, U.S., Goldberg, S.P., Knott-Craig, C.J. et al. : Complete repair of Ebstein anomaly in neonates and young infants : a 16-year follow-up. J. Thorac. Cardiovasc. Surg. 141 : 1163-1169, 2011. 8) Knott-Craig, C.J., Overholt, E.D., Ward, K.E. et al. : Neonatal repair of Ebstein's anomaly : indications, surgical technique, and medium-term follow-up. Ann. Thorac. Surg. 69 : 1505-1510, 2000. 11) Sano, S., Fujii, Y., Kasahara, S. et al. : Repair of Ebstein's anomaly in neonates and small infants : impact of right ventricular exclusion and its indications. Eur. J. Cardiothorac. Surg. 45 : 549-555, 2014. 2) Celermajer, D.S., Cullen, S., Sullivan, I.D. et al. : Outcome in neonates with Ebstein's anomaly. J. Am. Coll. Cardiol. 19 : 1041-1046, 1992. 13) da Silva, J.P., Baumgratz, J.F., Barbero-Marcial, M. et al. : The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation : early and midterm results. J. Thorac. Cardiovasc. Surg. 133 : 215-223, 2007. 14) Pizarro, C., Bhat, M.A. and Temple, J. : Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly. Multimed. Man. Cardiothorac. Surg., 2012. doi:10.1093/mmcts/mms014. 5) Sarris, G.E., Giannopoulos, N.M., Tsoutsinos, A.J. et al. : Results of surgery for Ebstein anomaly : a multicenter study from the European Congenital Heart Surgeons Association. J. Thorac. Cardiovasc. Surg. 132 : 50-57, 2006. 15) Sata, S., Murín, P. and Hraška, V. : Cone reconstruction of Ebstein's anomaly in a neonate. Ann. Thorac. Surg. 94 : e99-100, 2012. |
| References_xml | – reference: 1) Carpentier, A., Chauvaud, S., Mace, L. et al. : A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J. Thorac. Cardiovasc. Surg. 96 : 92-101, 1988. – reference: 14) Pizarro, C., Bhat, M.A. and Temple, J. : Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly. Multimed. Man. Cardiothorac. Surg., 2012. doi:10.1093/mmcts/mms014. – reference: 11) Sano, S., Fujii, Y., Kasahara, S. et al. : Repair of Ebstein's anomaly in neonates and small infants : impact of right ventricular exclusion and its indications. Eur. J. Cardiothorac. Surg. 45 : 549-555, 2014. – reference: 4) Davies, R.R., Pasquali, S.K., Jacobs, M.L. et al. : Current spectrum of surgical procedures performed for Ebstein's malformation : an analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann. Thorac. Surg. 96 : 1703-1710, 2013. – reference: 6) Reemtsen, B.L., Fagan, B.T., Starnes, V.A. et al. : Current surgical therapy for Ebstein anomaly in neonates. J. Thorac. Cardiovasc. Surg. 132 : 1285-1289, 2006. – reference: 12) Mizuno, M., Shiraishi, I., Ichikawa, H. et al. : Application of cone reconstruction for neonatal Ebstein anomaly or tricuspid valve dysplasia. Ann. Thorac. Surg. 101 : 1811-1817, 2016. – reference: 8) Knott-Craig, C.J., Overholt, E.D., Ward, K.E. et al. : Neonatal repair of Ebstein's anomaly : indications, surgical technique, and medium-term follow-up. Ann. Thorac. Surg. 69 : 1505-1510, 2000. – reference: 9) Knott-Craig, C.J., Goldberg, S.P., Kirklin, J.K. et al. : Repair of neonates and young infants with Ebstein's anomaly and related disorders. Ann. Thorac. Surg. 84 : 587-593, 2007. – reference: 3) Shinkawa, T., Polimenakos, A.C., Gomez-Fifer, C.A. et al. : Management and long-term outcome of neonatal Ebstein's anomaly. J. Thorac. Cardiovasc. Surg. 139 : 354-358, 2010. – reference: 5) Sarris, G.E., Giannopoulos, N.M., Tsoutsinos, A.J. et al. : Results of surgery for Ebstein anomaly : a multicenter study from the European Congenital Heart Surgeons Association. J. Thorac. Cardiovasc. Surg. 132 : 50-57, 2006. – reference: 10) Boston, U.S., Goldberg, S.P., Knott-Craig, C.J. et al. : Complete repair of Ebstein anomaly in neonates and young infants : a 16-year follow-up. J. Thorac. Cardiovasc. Surg. 141 : 1163-1169, 2011. – reference: 15) Sata, S., Murín, P. and Hraška, V. : Cone reconstruction of Ebstein's anomaly in a neonate. Ann. Thorac. Surg. 94 : e99-100, 2012. – reference: 2) Celermajer, D.S., Cullen, S., Sullivan, I.D. et al. : Outcome in neonates with Ebstein's anomaly. J. Am. Coll. Cardiol. 19 : 1041-1046, 1992. – reference: 7) Reemtsen, B.L., Polimenakos, A.C., Starnes, V.A. et al. : Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J. Thorac. Cardiovasc. Surg. 134 : 1406-1412, 2007. – reference: 13) da Silva, J.P., Baumgratz, J.F., Barbero-Marcial, M. et al. : The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation : early and midterm results. J. Thorac. Cardiovasc. Surg. 133 : 215-223, 2007. |
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| SubjectTerms | エプスタイン奇形 コーン手術 三尖弁 両心室修復 新生児 |
| Title | 新生児 Ebstein 奇形に対する一期的両心室修復として cone 手術を行った1例 |
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