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1 대한내과학회지 : 제 77 권부록 1 호 2009 증례 Inverted Takotsubo cardiomyopathy로발현된갈색세포종 원광대학교의과대학 1 내과학교실, 2 병리학교실 조미영 1 신익상 1 노혜정 1 김헌수 2 김하영 1 박병현 1 조정구 1 case of pheochromocytoma presenting as inverted Takotsubo cardiomyopathy Meyoung Cho, M.D. 1, Ik Sang Shin, M.D. 1, Hye Jung Noh, M.D. 1, Hun Soo Kim, M.D. 2, Ha Young Kim, M.D. 1, young Hyun Park, M.D. 1 and Chung Gu Cho, M.D. 1 Departments of 1 Internal Medicine and 2 Pathology, Wonkwang University School of Medicine, Iksan, Korea 61-year-old woman was admitted to the emergency department with acute chest pain. Echocardiography showed transient cardiomyopathy with akinesia of the basal and midportions of the left ventricle and hyperkinesia of the apex. No evidence of ischemic cardiomyopathy on myocardial SPECT or ergonovine stress echocardiography was observed. The patient s condition at discharge had improved, but she later was diagnosed as having pheochromocytoma. The findings of transient cardiomyopathy revealed inverted Takotsubo cardiomyopathy related to pheochromocytoma. The recognition of such a rare cardiac manifestation should be considered in the diagnosis of pheochromocytoma. (Korean J Med 77:S116-S121, 2009) Key Words: Inverted Takotsubo cardiomyopathy; Pheochromocytoma 서론갈색세포종은부신수질에서기원하는카테콜라민분비종양으로, 두통, 심계항진, 발한등은물론, 발작성고혈압이특징적으로나타나며드물게는심부전, 쇼크, 심근경색, 협심증, 부정맥과확장성심근병증등의심혈관질환을보일수있다 1-3). 갈색세포종과관련된카테콜라민유발성심근병증은급성심근경색과유사한스트레스성심근병증이나타날수 있는데 4), 이는관상동맥의병변없이가역적인좌심실첨부의운동저하에의한풍선형확장과좌심실벽기저부의과운동성이특징이다. Inverted Takotsubo cardiomyopathy는스트레스성심근병증과유사한유형으로좌심실의기저부및중간부위의운동저하그리고좌심실첨부의과운동성을특징으로하며, 갈색세포종과의연관성이보고되고있다 5-10). 이제까지갈색세포종과관련된 inverted Takotsubo cardiomyopathy는 8예가보고된바있으며 5-10), 이중 1예는국내에서보고한예로부신외갈색세포종을동반한경우였다 7). Received: ccepted: Correspondence to young Hyun Park, M.D., Department of Internal Medicine, Wonkwang University School of Medicine, Shinyong-dong, Iksan, Jeollabuk-do , Korea parkbhmd@wonkwang.ac.kr * This text is written with the support of research expenses of Wonkwang University in S 116 -

- Meyoung Cho, et al. Inverted Takotsubo cardiomyopathy on pheochromocytoma - Figure 1. () The electrocardiogram revealed significant ST depression in leads V 2~V 6.

증례환자 : 김, 여자, 61세주소 : 흉통및빈맥현병력 : 내원 1일전새벽 5시경밥을짓던중발생한 5분간격의 2~3차례꽉조이는흉통으로본원응급실에내원하였다. 과거력 : 6개월전당뇨병진단후투약중이었다. 가족력 : 특이사항없음. 신체검진소견 : 내원시혈압은촉지되지않았고, 맥박수 134회 / 분, 호흡수 24회 / 분, 체온 36.0 였다.

2 - Meyoung Cho, et al. Inverted Takotsubo cardiomyopathy on pheochromocytoma - Figure 1. () The electrocardiogram revealed significant ST depression in leads V 2~V 6. () The electrocardiogram revealed normal sinus rhythms with nonspecific ST segment changes. 저자들은 inverted Takotsubo cardiomyopathy를보인환자에서갈색세포종이진단된예를경험하였기에문헌고찰과함께보고하는바이다. 증례환자 : 김, 여자, 61세주소 : 흉통및빈맥현병력 : 내원 1일전새벽 5시경밥을짓던중발생한 5분간격의 2~3차례꽉조이는흉통으로본원응급실에내원하였다. 과거력 : 6개월전당뇨병진단후투약중이었다. 가족력 : 특이사항없음. 신체검진소견 : 내원시혈압은촉지되지않았고, 맥박수 134회 / 분, 호흡수 24회 / 분, 체온 36.0 였다. 급성병색을보였으며, 의식은명료하였다. 흉부청진시호흡음은약간감소되었고, 심잡음은청진되지않았다. 복부및사지진찰에서이상소견은없었다. 검사실소견 : 내원당시말초혈액검사는백혈구 21,920/ mm 3 ( 호중구 94%, 림프구 4%, 단핵구 2%, 호산구 0%), 혈색소 15.3 g/dl, 혈소판 163,000/mm 3 이었으며, 생화학검사에서총단백 8.04 g/dl, 알부민 4.15 g/dl, ST 58.3 IU/L, LT 35.4 IU/L, 총빌리루빈 1.80 mg/dl, LP 262 IU/L, r-gtp 23.6 IU/L, LDH 1,614 IU/L, 총칼슘 mg/dl, UN 47.43mg/dL, 크레아티닌 0.89 mg/dl이었다. 심장근육효소는 CK-M 102 IU/L, Troponin T ng/ml이었으며, 동맥혈가스검사는 ph 7.422, PaCO mmhg, PaO mmHg, SaO 2 81% 였다. 소변검사에서 glucose ++++, protein ++ Ketone + 를보였다. 단순흉부방사선검사 : 특이소견없음. 심전도 : 내원시분당 103회의빈맥과함께 QRS 축은정상이면서 V 2~V 6 유도에서 ST 분절의하강이관찰되었다 ( 그림 1). 심초음파검사 : 내원시좌심실확장기말직경이 71 mm, 수축기말직경이 63 mm로확장되어있었고, 심첨부의과다운동과나머지좌심실벽의심한운동저하가관찰되었으며 ( 그림 2, 2) 좌심실구혈률은 24% 였다. 그밖에중등도의대동맥판막과승모판의역류가관찰되었다. - S 117 -

- 대한내과학회지 : 제 77 권부록 1 호 2009 - C Figure 2. () On diastole. () On systole. The transthoracic echocardiogram showed severe LV dysfunction with apical hyperkinesia. (C) On diastole. (D) On systole.

D 임상경과 : 내원시흉통은니트로글리세린으로호전되었으며, 심인성쇼크로생각하고, 수액요법과도파민정주를실시한후혈압은 100/60 mmhg 로촉지되어도파민을감량하였고, 심전도모니터에서 190회 / 분이상의상심실성빈맥이있었으나 adenosine 투여후호전되었다.

3 - 대한내과학회지 : 제 77 권부록 1 호 C Figure 2. () On diastole. () On systole. The transthoracic echocardiogram showed severe LV dysfunction with apical hyperkinesia. (C) On diastole. (D) On systole. ased on the transthoracic echocardiogram, left ventricular abnormal wall movement with akinesia in the basal and mid-ventricle and hyperkinesis in the apical area had improved on day 7. D 임상경과 : 내원시흉통은니트로글리세린으로호전되었으며, 심인성쇼크로생각하고, 수액요법과도파민정주를실시한후혈압은 100/60 mmhg 로촉지되어도파민을감량하였고, 심전도모니터에서 190회 / 분이상의상심실성빈맥이있었으나 adenosine 투여후호전되었다. 입원 4일째혈역학적상태가안정되어도파민을중단하였으며, 분당 80회의정상동율동과함께내원시보였던 ST 분절하강소견은보이지않아 ( 그림 1) 도부타민심근관류검사를실시하였으나심근허혈은보이지않았다 ( 그림 3). 입원 7일째시행한추적심초음파에서좌심실의확장기말직경이 51 mm, 수축기말직경이 35 mm, 좌심실구혈률이 60% 로증가하였고, 심근벽의운동은정상이었다 ( 그림 2C, 2D). 입원 10일째실시한에르고노빈부하심초음파검사에서이상소견이보이지않아관상동맥조영술을실시할예정이었으나, 경제적인이유로더이상의검사를거부하고자의로 퇴원하였다. 퇴원후환자는 3년동안순환기내과외래에서경과관찰중두통과홍조가지속되어시행한복부전산화단층촬영에서 3 cm 크기의좌측부신종괴가발견되었다 ( 그림 3). 6개월간추적관찰중어지럼증으로인근지역병원에입원하여시행한복부전산화단층촬영에서좌측부신의종괴는 3.5 cm로크기가증가되었고, 간헐적으로혈압이 160/90 mmhg 으로상승하여본원으로전원되었다. 재입원 2일째부신자기공명영상촬영에서좌측부신에직경약 cm 크기의종괴가있고내부에 T1 강조영상에서저신호강도, T2 강조영상에서고신호강도를보여갈색세포종을의심할수있었다. 입원 4일째부신종괴의기능평가를위해시행한 24시간요검사에서노르에피네프린 μg/day ( 정상범위 : 15~80 μg/day), 에피네프린 μg/day ( 정상범위 : 20 μg/day 이하 ), 바닐만델산 6.48 mg/day ( 정상범위 : 8 mg/day 이하 ), 메타네 - S 118 -

- 조미영외 6 인. Inverted Takotsubo cardiomyopathy - Figure 3.

() bdominal computed tomography showed a well enhanced left adrenal mass. Figure 4.

() Microscopic findings of the left adrenal mass confirmed a diagnosis of pheochromocytoma. 프린 1.

입원 11일째 MIG 스캔에서좌측부신에국한되어방사성동위원소섭취의증가가관찰되었다. 환자는복강경을통한좌측부신절제술을받았으며, 3 1.

4 - 조미영외 6 인. Inverted Takotsubo cardiomyopathy - Figure 3. () Myocardial SPECT with dobutamine revealed no perfusion defects. () bdominal computed tomography showed a well enhanced left adrenal mass. Figure 4. () Gross findings of the resected left adrenal mass. () Microscopic findings of the left adrenal mass confirmed a diagnosis of pheochromocytoma. 프린 mg/day ( 정상범위 : 1.3 mg/day 이하 ) 로측정되어갈색세포종을확진할수있었다. 입원 11일째 MIG 스캔에서좌측부신에국한되어방사성동위원소섭취의증가가관찰되었다. 환자는복강경을통한좌측부신절제술을받았으며, cm의경계가분명한부신종괴 ( 그림 4) 와함께, 이종괴의병리조직검사를통해갈색세포종을확진할수있었다 ( 그림 4). 환자는수술후내원시보였던두통, 홍조가호전되었으 며현재외래에서경과관찰중이다. 고찰 Takotubo cardiomyopathy나 apical ballooning syndrome, broken heart, reversible left ventricular dysfunction 등으로불리는스트레스성심근병증은정서적또는신체적스트레스등에의해유발되며급성관상동맥증후군과유사한임상양상을보인다. 심초음파검사나좌심실조영술에서특징적 - S 119 -

5 - The Korean Journal of Medicine: Vol. 77, Suppl. 1, 으로가역적인좌심실벽기저부의과운동성과심첨부의운동저하를보이며, 심전도의흉부유도에서 ST 분절의상승과 T파역위, 그리고심근효소의상승이있을수있지만, 관상동맥조영술에서관상동맥의폐쇄는동반되지않는다 11,12). 스트레스성심근병증의병태생리는완전히밝혀지지않았으나, 카테콜라민매개에의한것으로여겨지고있다 13-15). 스트레스성심근병증은가역적인좌심실기능부전과대체로양호한원내사망률을보이는것으로알려져있지만 12,15), Lee 등은 30% 로높은사망률을보고하기도하였다 16). 유발인자, 증상, 임상양상이유사하나좌심실의움직임에서차이를보이는 Takotsubo cardiomyopathy의또다른유형으로 inverted Takotsubo cardiomyopathy가보고된바있다 17,18). 이는좌심실첨부의과운동성과좌심실기저부와중간부위의운동저하가특징이다. 이제까지갈색세포종과관련된 inverted Takotsubo cardiomyopathy는 8예가보고된바있다 5-10). 보고된증례들에서국내에서보고한부신외갈색세포종을동반한 1예 7) 와일본의 1예 11) 를제외한환자들의대부분은중년의여자였다. 환자들은흉통, 쇼크, 갑작스런고혈압위기혹은두통및심계항진같은갈색세포종과관련된증상을주소로내원하였다. 내원시심전도에서하벽또는측벽유도혹은하벽과측벽유도모두에서 ST 분절의하강을보였으며, 심근효소의상승과함께심초음파검사나심실조영술에서좌심실기저부의운동저하와심첨부의과운동성과같은심근운동의이상이있었지만관상동맥조영술에서관상동맥관류는정상이었다. 이후이어진검사를통해갈색세포종이진단되었고, 환자의임상증상이호전된후수술적절제를통해갈색세포종을치료하였다. 그리고관상동맥조영술중사망한 1예 6) 를제외하면환자들은비교적예후가양호하였다. 본증례는흉통을주소로내원하였고, ST 분절의하강을보이며, 심근효소의상승을동반하여급성관상동맥증후군이의심되었다. 비록관상동맥조영술을시행하지못하였지만진행된일련의검사에서관상동맥폐쇄의증거는찾을수없었고, 내원시보였던심부전은완전한가역성이었다. Inverted Takotsubo cardiomyopathy는비교적최근에기술된증후군 17,19) 이나갈색세포종에서카테콜라민유도심근병증의한양상으로발현될수있다. 따라서본증례와같이내원시심전도에서하벽또는측벽유도혹은하벽과측벽유도모두에서 ST 분절의하강이있고심근효소의상승과함께심초음파검사에서 inverted Takotsubo cardiomyopathy를보이는환자에서는반드시갈색세포종의감별을고려하여야 할것으로생각된다. 요 61세여자환자가급성흉통을주소로내원하여시행한심전도와심초음파에서급성심근경색증후군이의심되었다. 심초음파검사에서좌심실기저부의운동저하와심첨부의과운동성이보였다. 심근관류검사와에르고노빈부하심초음파검사에서심근허혈의증거는없었다. 환자는호전된상태로퇴원하였고, 이후갈색세포종을진단받았다. 이전의가역적인심근병증은갈색세포종과연관된 inverted Takostubo cardiomyopathy로진단되었다. Inverted Takostubo cardiomyopathy는갈색세포종의매우드문심장합병증이므로이질환을진단할때꼭갈색세포종을고려하여야할것으로생각된다. 중심단어 : Inverted Takostubo Cardiomyopathy; 갈색세포종 약 REFERENCES 1) Sardesai SH, Mourant J, Sivathandon Y, Farrow R, Gibbons DO. Phaeochromocytoma and catecholamine induced cardiomyopathy presenting as heart failure. r Heart J 63: , ) Lin PC, Hsu JT, Chung CM, Chang ST. Pheochromocytoma underlying hypertension, stroke, and dilated cardiomyopathy. Tex Heart Inst J 34: , ) rouwers FM, Lenders JW, Eisenhoffer G, Pacak K. Pheochromocytoma as an endocrine emergency. Rev Endocr Metab Disord 4: , ) Takizawa M, Kobayakawa N, Uozumi H, Yonemura S, Kodama T, Fukusima K, Takeuchi H, Kaneko Y, Kaneko T, Fujita K, Honma Y, oyagi T. case of transient left ventricular ballooning with pheochromocytoma, supporting pathogenetic role of catecholamines in stress-induced cardiomyopathy or takotsubo cardiomyopathy. Int J Cardiol 114:e15-e17, ) Sanchez-Recalde, Costero O, Oliver JM, Iborra C, Ruiz E, Sobrino J. Images in cardiovascular medicine: pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern. Circulation 113:e738-e739, ) Sanchez-Recalde, Iborra C, Costero O, Moreno R, López de Sá E, Sobrino J, López-Sendón JL. Isolated left ventricular basal ballooning in young women: inverted takotsubo pattern related to catecholamine-toxicity. m J Cardiol 100: , ) Kim HS, Chang WI, Kim YC, Yi SY, Kil JS, Hahn JY, Kang M, Lee MS, Lee SH. Catecholamine cardiomyopathy associated with paraganglioma rescued by percutaneous cardiopulmonary sup- - S 120 -

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<313520C1F5B7CA B0ADB9CEB1D42DB9DAC1A4B6FB D E687770> 대한내과학회지 : 제 82 권제 5 호 2012 http://dx.doi.org/10.3904/kjm.2012.82.5.609 영구형인공심박동기삽입술후발생한스트레스성심장근육병증 1예 경상대학교의학전문대학원내과학교실 강민규 김나영 박정랑 황석재 박용휘 곽충환 황진용 Stress-Induced Cardiomyopathy as a Complication of Permanent