좌심형성부전증후군의 외과적 치료 및 폰탄수술의 결과

The Surgical Management of Hypoplastic Left Heart Syndrome and the Results of a Fontan Operation

  • 정의석 (서울대학교 의과대학 분당서울대학교병원 흉부외과학교실) ;
  • 김웅한 (서울대학교 의과대학 서울대학교어린이병원 흉부외과학교실) ;
  • 전재현 (서울대학교 의과대학 서울대학교어린이병원 흉부외과학교실) ;
  • 최창휴 (가천의과학대학교 길병원 흉부외과학교실) ;
  • 이창하 (부천세종병원 흉부외과) ;
  • 이영탁 (성균관대학교 의과대학 삼성서울병원 흉부외과학교실)
  • Chung, Eui Suk (Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine) ;
  • Kim, Woong-Han (Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Jeon, Jae-Hyun (Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Choi, Chang-Hyu (Department of Thoracic and Cardiovascular Surgery, Gil Medical Center, Gachon University of Medicine and Science) ;
  • Lee, Chang-Ha (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lee, Young-Tak (Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2008.07.25
  • 심사 : 2008.10.10
  • 발행 : 2009.02.05

초록

서론: 좌심형성부전증후군은 사망률이 높은 질환이다. 이번 연구는 노우드(Norwood) 수술을 거쳐 최종적으로 폰탄 수술을 시행한 좌심형성부전증후군 환자에서 외과적 치료 및 폰탄 수술 후 결과를 알아 보기 위한 것이다. 대상 및 방법: 1997년 10월부터 2005년 5월까지 좌심형성부전증후군으로 노우드 수술을 시행한 21명의 환자 중 최종적으로 폰탄(Fontan) 수술을 시행한 6명의 환자를(M : F=4 : 2) 대상으로 역행적 조사를 하였다. 노우드 수술 시 평균 나이는 $17.3{\pm}10.8$일(9~36일)이었고, 양방향성 글렌수술은 $8.9{\pm}7.1$개월(4.6~23.3개월), 그리고 폰탄 수술은 $32.4{\pm}9.8$개월(20.9~43.9개월)에 시행하였다. 한 명의 환자에서 삼첨판 역류가 보여, 2차 수술 시 판막 성형술을 시행하였다. 모든 환자에서 고어텍스 도관을 이용한 심장외 폰탄 수술(20 mm 2명, 18 mm 4명)을 하였으며 1명에서 개방창(fenestration)을 두었다. 결과: 노우드 수술 사망률은 30% (7/21)이며, 4명의 중간단계사망이 있어, 10명만이 양방향성 글렌수술을 시행 하였다. 10명의 환자 중 1명의 만기 사망과 1명의 외래 관찰 누락이 있었으며, 6명의 환자는 현재 폰탄 수술을 시행한 후 외래 관찰 중이고, 2명은 폰탄 수술을 위해 대기 중이다. 폰탄 수술 후에는 1명에서 출혈로 재수술을 한 이외의 합병증은 없었다. 모든 환자가 정상 동성 리듬을 보였다. 수술 후 시행한 초음파에서 모든 환자에서 좋은 심실기능을 관찰할 수 있었으며, 삼첨판 역류는 2명에서만 Grade I으로 관찰되었다. 환자들은 현재 평균 $19.6{\pm}14.9$개월(1.5~39.1개월)간 외래 관찰 중이며, 현재 모든 환자에서 항 혈소판제(아스피린) 및 심장약을 투약하고 있으며, 모두 NYHA functional class I의 상태이다. 결론: 좌심형성부전증후군으로 Norwood 술식을 거쳐 최종적으로 폰탄 수술을 시행한 환자에서 1차 수술 후 높은 사망률을 보임에도 불구하고 만족할만한 혈류역학과 임상상태를 확인할 수 있었고, 단계적 수술 교정법은 이 질환에서, 보다 적극적으로 고려되어야 된다고 사료된다.

Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

키워드

참고문헌

  1. Ruckman RN, Van praagh R. Anatomic type of congenital mitral stenosis: report of 49 autopsy cases with consideration of diagnosis and surgical implication. Am J Cardiol 1978; 42:592 https://doi.org/10.1016/0002-9149(78)90629-X
  2. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. New Engl J Med 1983;308:23-6 https://doi.org/10.1056/NEJM198301063080106
  3. Lev M. Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes. Lab Invest 1952;1: 61-70
  4. Noonan JA, Nadas AS. The hypoplastic left heart syndrome: an analysis of 101 cases. Pediatr Clin N Am 1958;5: 1029-56 https://doi.org/10.1016/S0031-3955(16)30727-1
  5. Checchia PA, Larsen R, Sehra R, et al. Effect of a selection and postoperative care protocol on survival of infants with hypoplastic left heart. Ann Thorac Surg 2004;77:477-83 https://doi.org/10.1016/S0003-4975(03)01596-0
  6. Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg 1984;88:97
  7. Natowicz M, Chatten J, Clancy R, et al. Genetic disorder and major extracardiac anomalies associated with hypoplastic left heart syndrome. Pediatrics 1988;82:698
  8. Marvroudis C, Backer CL. Pediatric cardiac surgery. 3rd ed. Philadelphia: Mosby. 2003:562
  9. Jobes DR, Nicolson SC, Steven JM, et al. Carbondioxide prevents pulmonary overcirculation in hypoplastic left heart syndrome. Ann Thorac Surg 1992;54:150-1 https://doi.org/10.1016/0003-4975(92)91166-7
  10. Sinha SN, Rusnak SL, Sommers HM, et al. Hypoplastic left heart syndrome: analysis of thirty autopsy cases in infants with surgical considerations. Am J Cardiol 1968;21:166-73 https://doi.org/10.1016/0002-9149(68)90316-0
  11. Marvroudis C, Backer CL. Pediatric cardiac surgery. 3rd ed. Philadelphia: Mosby. 2003:563
  12. Mahle WT, Spray TL, Wernovsky G, et al. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 2000;102(suppl 3):136-41
  13. Shburn DA, McCrindle BW, Tchervenkov CI, et al. Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 2003;125:1070-82 https://doi.org/10.1067/mtc.2003.183
  14. Murdison KA, Baffa JM, Farrell PE, et al. Hypoplastic left heart syndrome: outcome after initial reconstruction and before modified Fontan procedure. Circulation 1990:82: 199-207.
  15. Mosca S, Bove EL, Crowley DC, et al, Hemodynamic characteristics of neonates following first stage palliation for hypoplastic left heart syndrome. Circulation 1995;92:267-71 https://doi.org/10.1161/01.CIR.92.9.267
  16. Canter CE, Moorhead S, Huddleston CB, et al. Restrictive atrial septal communication as a determinant of outcome of cardiac transplantation for hypoplastic left heart syndrome. Circulation 1993;88:456-60
  17. Scheurer MA, Salvin JW, Vida VL, et al. Survival and clinical course at Fontan after stage one palliation with either a modified Blalock-Taussig shunt or a right ventricle to pulmonary artery conduit. J Am Coll Cardiol 2008;52: 52-9 https://doi.org/10.1016/j.jacc.2008.03.034
  18. Ghanayem NS, Cava JR, Jaquiss RDB, Tweddell JS. Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004;7:32-8 https://doi.org/10.1053/j.pcsu.2004.02.017
  19. Jaquiss RD, Siehr SL, Fedderly RT, Cava JR, Tweddell JS. Early cavopulmonary anastomosis after norwood procedure results in excellent fontan outcome. Ann Thorac Surg 2006; 82:1260-6 https://doi.org/10.1016/j.athoracsur.2006.04.095
  20. Brawn WJ, Barron DJ. Management and outcome in hypoplastic left heart syndrome current paediatrics 2004;14: 26-32