Successful Removal of Left Ventricular Rhabdomyoma : A Rare Cause of Left Ventricular Outflow Obstruction in the Newborn Infant -1 case report

신생아에서 좌심실유출로 폐쇄를 동반한 심장 횡문근종 치험 1례

  • 안병희 (전남대학교병원 흉부외과학교실, 전남대학교 의과대학 흉부외과학교실) ;
  • 문형선 (전남대학교병원 흉부외과학교실, 전남대학교 의과대학 흉부외과학교실)
  • Published : 1997.02.01

Abstract

Cardiac rhabdomyomas are the moil common primary tumor in infancy and childhood and are frequently associated with tuberous sclerosis. Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis of this tumor associated with subaortic stenosis is still considered to be poor and surgery continues to be indicated. A 4-day-old female was admitted due to tachypnea and cyanosis. Single rhabdomyoma arising from the interventricular septum associated with severe subaortic stcnosls as partially removed under cardiopulmonary bypass. Excised tumor sizc was 0.7$\times$0.9$\times$0.4cm in dimension. The postoperative course was uneventful and the infant discharged on the 14th postoperative day.

심장 횡문근종은 영아나 소아기에서 가장 발생빈도가 높은 원발성 심장종양으로서 결절성 경화증을 빈번히 동반하고, 자연 퇴화가 보고된다 할지라도 좌심실 유출로 폐쇄를 동반한 종양은 아직까지는 예후가 불량하고 수술적 치료가 적응이 된다. 생후 4 일된 신생아가 청색증과 빈 호흡을 주소로 내원하여 생후 4일째에 정중흉골절개를 통한 체외 순환하에 수술을 시행하여 좌심실 유출로를 막고있는 0.7$\times$0.9$\times$0.4cm크기의 종괴를 제거하였다. 수술 후 합병증은 발생하지 않았으며 수술후 14일째에 건강하게 퇴원하였다.

Keywords

References

  1. Pathol Annu v.14 Primary tumors of the heart and pericardium McAllister HA Jr
  2. Am J Cardiol v.21 Cardiac tumors in infancy Nadas A;Ellison R
  3. Am J Cardiol v.66 Natural History of Cardiac Rhabdomyoma in Infancy and Childhood Smythe JF;Jeffrey DD;Smallhorn JF;Freedom RM
  4. J Am Coll Cardiol v.2 Investigation and management of primary cardiac tumor in infants and children Bini R;Westaby S;Bargeron L;Pacifico A;Kirklin J
  5. 대흉외지 v.26 태아에서 발생한 Cardiac Rhabdomyoma 치험 1례 박성동;박재홍;문준호;안욱수;허웅;김병철
  6. Am J Cardiol v.38 Cardiac rhabdomyoma : A clinicopathologic and electron microscopic study Fenoglio J;J McAllister HA;Ferrans VJ
  7. Am J Cardiol v.67 Spontaneous regression of cardiac rhabdomyoma Farooki ZQ;Ross RD;Paridon SM;Humes RA;Karpawich PP;Pinsky WW
  8. Am J Cardiol v.55 Echocardiographic incidence of cardiac rhabdomyoma in tuberous sclerosis Bass JL;Breninstall GN;Swaiman KF