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<article xmlns:xlink="http://www.w3.org/1999/xlink" specific-use="sps-1.9" dtd-version="1.1" xml:lang="pt" article-type="case-report">
  <front>
    <journal-meta>
      <journal-id journal-id-type="publisher-id">abc</journal-id>
      <journal-title-group>
        <journal-title>Arquivos Brasileiros de Cardiologia</journal-title>
        <abbrev-journal-title abbrev-type="publisher">Arq. Bras. Cardiol.</abbrev-journal-title>
      </journal-title-group>
      <issn pub-type="ppub">0066-782X</issn>
      <issn pub-type="epub">1678-4170</issn>
      <publisher>
        <publisher-name>Sociedade Brasileira de Cardiologia - SBC</publisher-name>
        <publisher-loc>S&#227;o Paulo, SP, Brazil</publisher-loc>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">T97nnn36Q98HcqfbkFFNfpJ</article-id>
      <article-id pub-id-type="other">00009</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2004000200009</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(04)08200209</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2004000200009</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relatos de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Crise de feocromocitoma simulando um infarto agudo do mioc&#225;rdio em paciente com art&#233;rias coron&#225;rias normais</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Darz&#233;</surname>
            <given-names>Eduardo S.</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Von Sohsten</surname>
            <given-names>Roberto L.</given-names>
          </name>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">Salvador</named-content>
          <named-content content-type="state">BA</named-content>
        </addr-line>
        <institution content-type="orgname">Hospital Alian&#231;a</institution>
        <institution content-type="orgdiv1">Servi&#231;o de Cardiologia/Ecocardiografia </institution>
        <email>esdarze@ufba.br</email>
      </aff>
      <aff id="aff02">
        <addr-line>
          <named-content content-type="state"/>
        </addr-line>
        <institution content-type="orgname">John F. Kennedy Medical Center Atlantis</institution>
        <institution content-type="orgdiv1">Cardiology Division </institution>
        <country country="US">United States</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>02</month>
        <year>2004</year>
      </pub-date>
      <volume>82</volume>
      <issue>2</issue>
      <fpage>175</fpage>
      <lpage>177</lpage>
      <history>
        <date date-type="accepted">
          <day>26</day>
          <month>05</month>
          <year>2003</year>
        </date>
        <date date-type="received">
          <day>24</day>
          <month>02</month>
          <year>2003</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/4.0/" xml:lang="en">
          <license-p>This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.</license-p>
        </license>
      </permissions>
      <abstract>
        <p>Relatamos o caso de um paciente com altera&#231;&#245;es eletrocardiogr&#225;ficas e disfun&#231;&#227;o mioc&#225;rdica segmentar induzidas por feocromocitoma, simulando infarto agudo do mioc&#225;rdio. A angiografia coronariana foi normal e houve normaliza&#231;&#227;o completa do eletrocardiograma e ecocardiograma, ap&#243;s terapia com um bloqueador alfa-adren&#233;rgico e ressec&#231;&#227;o do tumor. Espasmo coronariano foi o prov&#225;vel mecanismo envolvido na produ&#231;&#227;o dessas altera&#231;&#245;es, ilustrando a import&#226;ncia de manter um alto grau de suspei&#231;&#227;o cl&#237;nica em pacientes com evento mioc&#225;rdico inesperado em meio a uma crise hipertensiva.</p>
      </abstract>
      <pub-date publication-format="electronic" date-type="pub">
        <day>16</day>
        <month>03</month>
        <year>2004</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p>Crise de feocromocitoma simulando um infarto agudo do mioc&#225;rdio em paciente com art&#233;rias coron&#225;rias normais</p>
    <p>
      <bold>Eduardo S. Darz&#233;; Roberto L. Von Sohsten</bold>
    </p>
    <p>Servi&#231;o de Cardiologia/Ecocardiografia - Hospital Alian&#231;a - Salvador, BA e Cardiology Division - John F. Kennedy Medical Center Atlantis, FL - USA - Salvador, BA e Atlantis, FL</p>
    <p>
      <xref ref-type="corresp" rid="back10">Endere&#231;o para correspond&#234;ncia</xref>
    </p>
    <p>
      <bold>RESUMO</bold>
    </p>
    <p>Relatamos o caso de um paciente com altera&#231;&#245;es eletrocardiogr&#225;ficas e disfun&#231;&#227;o mioc&#225;rdica segmentar induzidas por feocromocitoma, simulando infarto agudo do mioc&#225;rdio. A angiografia coronariana foi normal e houve normaliza&#231;&#227;o completa do eletrocardiograma e ecocardiograma, ap&#243;s terapia com um bloqueador alfa-adren&#233;rgico e ressec&#231;&#227;o do tumor. Espasmo coronariano foi o prov&#225;vel mecanismo envolvido na produ&#231;&#227;o dessas altera&#231;&#245;es, ilustrando a import&#226;ncia de manter um alto grau de suspei&#231;&#227;o cl&#237;nica em pacientes com evento mioc&#225;rdico inesperado em meio a uma crise hipertensiva.</p>
    <p>Feocromocitomas s&#227;o tumores que secretam catecolaminas e t&#234;m origem no tecido cromafin do sistema nervoso simp&#225;tico. As manifesta&#231;&#245;es cl&#237;nicas desse tumor, comumente, incluem epis&#243;dios de palpita&#231;&#227;o, sudorese, cefal&#233;ia e hipertens&#227;o parox&#237;stica.</p>
    <p>Al&#233;m dos sintomas cl&#225;ssicos, os feocromocitomas t&#234;m sido raramente associados &#224; ocorr&#234;ncia de infarto agudo do mioc&#225;rdio e outras complica&#231;&#245;es cardiovasculares<sup>1</sup>. Relatamos caso de um paciente com feocromocitoma adrenal e art&#233;rias coron&#225;rias normais, cujos achados eletrocardiogr&#225;ficos e ecocardiogr&#225;ficos eram compat&#237;veis com infarto agudo de parede anterior e lateral, e que apresentou revers&#227;o completa dessas anormalidades ap&#243;s terapia com bloqueador alfa-adren&#233;rgico.</p>
    <p>
      <bold>Relato do Caso</bold>
    </p>
    <p>Mulher de 46 anos de idade foi hospitalizada com n&#225;usea, v&#244;mitos e sudorese por 2 dias. Ao exame f&#237;sico sua press&#227;o arterial inicial era de 230/130 mmHg, frequ&#234;ncia card&#237;aca de 132 bpm. O resto do exame segmentar era normal. O eletrocardiograma (<xref ref-type="fig" rid="f1">fig. 1</xref>) mostrava eleva&#231;&#227;o do segmento ST e invers&#227;o profunda e sim&#233;trica da onda T de V2 a V6. A onda T tamb&#233;m estava invertida nas deriva&#231;&#245;es I, aVL, II e aVF e o intervalo QT bastante prolongado, com ondas Q nas deriva&#231;&#245;es II, III e aVF.</p>
    <p content-type="asset">
      <fig id="f1">
        <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf01.jpg"/></fig>
    </p>
    <p>Encaminhada ao laborat&#243;rio de cateterismo, a angiografia coronariana n&#227;o mostrou doen&#231;a coronariana significativa. A ventriculografia contrastada (<xref ref-type="fig" rid="fig2">fig. 2</xref>) revelou hipocinesia severa das paredes anterolateral e apical. Durante o procedimento a paciente necessitou de ventila&#231;&#227;o mec&#226;nica e sua press&#227;o arterial tornou-se muito l&#225;bil, atingindo n&#237;veis elevados de 320/240 mmHg, e baixos de 70/30 mmHg. Feita a suspeita de feocromocitoma instalou-se terapia com um bloqueador alfa-adren&#233;gico, obtendo-se controle progressivo da press&#227;o arterial. Os n&#237;veis de &#225;cido vanilmand&#233;lico, metanefrinas, adrenalina e noradrenalina em urina de 24h foram, respectivamente, 313 mg (normal: 2-10), 76 (normal: 0,3-0,9), 12339 ng (normal: 0-16) e 28316 ng (normal: 11-86). Outros exames laboratoriais inclu&#237;ram: ur&#233;ia 26 mg/dl, creatinina 2,0 mg/dl e um valor m&#225;ximo de CPK de 951 IU/L com uma fra&#231;&#227;o MB normal. A tomografia computadorizada de abdomen revelou massa na gl&#226;ndula adrenal esquerda. Com o retorno da press&#227;o arterial a n&#237;veis normais, a paciente foi extubada e a fun&#231;&#227;o renal normalizada. Ecocardiogramas seriados foram realizados no 1&#176; e 10&#176; dias de hospitaliza&#231;&#227;o. O primeiro confirmou os achados da ventriculografia de hipocinesia anterior e apical, enquanto que o seguinte mostrou resolu&#231;&#227;o completa das altera&#231;&#245;es segmentares. Ap&#243;s 10 dias de tratamento, a paciente foi submetida a uma laparotomia quando um feocromocitoma medindo 11,2 x 11,8 x 7,2 cm foi removido sem complica&#231;&#245;es. Um eletrocardiograma obtido aproximadamente 3 meses ap&#243;s o evento agudo mostrou resolu&#231;&#227;o completa das anormalidades pr&#233;vias (<xref ref-type="fig" rid="f3">fig. 3</xref>).</p>
    <p content-type="asset">
      <fig id="f3">
        <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf02.jpg"/></fig>
    </p>
    <fig id="fig2">
      <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf03.jpg"/></fig>
    <p>Antes da alta hospitalar, a paciente reconheceu que os sintomas que precipitaram sua hospitaliza&#231;&#227;o, vinham acontecendo de forma intermitente desde que fora diagnosticada com hipertens&#227;o arterial h&#225; 5 anos. At&#233; 1 ano ap&#243;s alta hospitalar, a paciente encontrava-se muito bem, com a press&#227;o arterial controlada e sem recorr&#234;ncia dos sintomas.</p>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>Os feocromocitomas podem induzir altera&#231;&#245;es mioc&#225;rdicas sob a forma de hipertrofia ventricular, devido &#224; hipertens&#227;o cr&#244;nica<sup>2</sup>, miocardiopatia dilatada como resultado de exposi&#231;&#227;o prolongada e persistente a n&#237;veis altos de catecolaminas<sup>3</sup>, ou, raramente, simular infarto agudo do mioc&#225;rdio<sup>4-9</sup>.</p>
    <p>Relatamos um caso raro da associa&#231;&#227;o entre feocromocitoma e disfun&#231;&#227;o ventricular revers&#237;vel em paciente com art&#233;rias coron&#225;rias normais. Somente alguns casos foram relatados na literatura de crise de feocromocitoma, causando ou simulando infarto agudo do mioc&#225;rdio. A apresenta&#231;&#227;o cl&#237;nica desses casos varia significativamente, desde um infarto silencioso, um dos primeiros relatos da literatura<sup>4</sup>, at&#233; choque cardiog&#234;nico franco<sup>5</sup>. Altera&#231;&#245;es eletrocardiogr&#225;ficas acentuadas, sugerindo isqu&#234;mia mioc&#225;rdica, est&#227;o comumente presentes em pacientes com feocromocitomas, e incluem prolongamento do intervalo QT, invers&#227;o profunda e sim&#233;trica da onda T e altera&#231;&#245;es do segmento ST<sup>10</sup>. Apesar desses achados serem muito sugestivos de isqu&#234;mia mioc&#225;rdica, alguns autores descreveram altera&#231;&#245;es similares em pacientes com hemorragia subaracn&#243;ide ou em uso de certas drogas<sup>10</sup>. Nenhuma dessas situa&#231;&#245;es parece prov&#225;vel em nosso caso. O padr&#227;o de disfun&#231;&#227;o ventricular observado em pacientes com feocromocitoma n&#227;o tem sido bem caracterizado. No entanto, h&#225; relatos de disfun&#231;&#227;o global<sup>7</sup> e tamb&#233;m segmentar<sup>6</sup>. Apesar de comumente associadas, as altera&#231;&#245;es isqu&#234;micas no eletrocardiograma, n&#227;o s&#227;o necessariamente acompanhadas de altera&#231;&#245;es de contratilidade<sup>8</sup>. Ademais, a exemplo do nosso caso, os marcadores de necrose mioc&#225;rdica podem n&#227;o estar elevados, n&#227;o obstante a presen&#231;a de altera&#231;&#245;es isqu&#234;micas ao eletrocardiograma e disfun&#231;&#227;o ventricular<sup>7</sup>. A eleva&#231;&#227;o significativa dos n&#237;veis totais de CPK encontrada no caso presente, &#233; provavelmente de origem muscular esquel&#233;tica, uma vez que a fra&#231;&#227;o MB permaneceu normal em todas as medidas.</p>
    <p>A fisiopatologia da disfun&#231;&#227;o ventricular em pacientes com feocromocitoma est&#225; associada a efeito t&#243;xico direto das catecolaminas<sup>3</sup>, ou a <italic>stunning</italic> mioc&#225;rdico causado por espasmo das art&#233;rias coron&#225;rias<sup>9</sup>. A disfun&#231;&#227;o ventricular, assim como as anormalidades no eletrocardiograma, s&#227;o comumente revers&#237;veis ap&#243;s terapia com bloqueadores alfa-adren&#233;rgicos e ressec&#231;&#227;o do tumor<sup>5,7</sup>. Apesar dos betabloqueadores serem recomendados no tratamento do infarto agudo do mioc&#225;rdio, eles podem exacerbar o espasmo coronariano nos casos de feocromocitomas, devido &#224; estimula&#231;&#227;o alfa-adren&#233;rgica sem oposi&#231;&#227;o, e, portanto, s&#227;o contra-indicados nessa situa&#231;&#227;o.</p>
    <p>Nosso caso ilustra apresenta&#231;&#227;o incomum de uma crise de feocromocitoma com altera&#231;&#245;es eletrocardiogr&#225;ficas e ecocardiogr&#225;ficas, simulando infarto agudo do mioc&#225;rdio. A aus&#234;ncia de eleva&#231;&#227;o dos marcadores de necrose mioc&#225;rdica associada &#224; revers&#227;o completa da disfun&#231;&#227;o ventricular sugerem que <italic>stunning</italic> devido ao espasmo coronariano foi o mecanismo subjacente a todas as altera&#231;&#245;es. Nosso relato tamb&#233;m refor&#231;a a import&#226;ncia de manter um alto grau de suspei&#231;&#227;o cl&#237;nica em pacientes que se apresentam com infarto do mioc&#225;rdio inesperado, associado a uma crise hipertensiva. Nesses casos, uma anamnese cuidadosa &#233; fundamental para o diagn&#243;stico, j&#225; que em nosso caso e, tamb&#233;m, em outros pacientes relatados na literatura, sintomas bem sugestivos de feocromocitoma est&#227;o comumente presentes por alguns anos antes do evento agudo<sup>1</sup>. Este tipo de paciente deve ser investigado para presen&#231;a de feocromocitoma, uma vez que o tratamento precoce previne morbidade e mortalidade.</p>
    <p>
      <fn id="back10" fn-type="corresp">
        <label>
          <bold> E</bold>
        </label>
        <p>
          <bold>ndere&#231;o para correspond&#234;ncia</bold>
        </p>
        <p> Eduardo S. Darz&#233; </p>
        <p> Servi&#231;o de Cardiologia/Ecocardiografia - Hospital Alian&#231;a </p>
        <p> Avenida Juracy Magalh&#227;es Jr. 2096 </p>
        <p> Salvador, BA - Cep 41920-000 </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="esdarze@ufba.br">esdarze@ufba.br</email>
        </p>
      </fn>
    </p>
    <p>Recebido para publica&#231;&#227;o em 24/2/03 </p>
    <p>Aceito em 26/5/03</p>
  </body>
  <back>
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        <mixed-citation>9. Sheikhzadeh    A, Fatourechi V, Paydar D, et al. Unusual cardiovascular manifestation in a    case of pheochromocytoma. Clin Cardiol 1983; 6: 136-42.</mixed-citation>
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          <source>Clin Cardiol</source>
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            <year>1983</year>
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          <fpage>136</fpage>
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              <surname>Sheikhzadeh</surname>
              <given-names>A</given-names>
            </name>
            <name>
              <surname>Fatourechi</surname>
              <given-names>V</given-names>
            </name>
            <name>
              <surname>Paydar</surname>
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            </name>
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        <mixed-citation>10. Cheng    TO, Bashour TT. Striking electrocardiographic changes associated with pheochromocytoma.    Chest 1976; 70: 397-9.</mixed-citation>
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          <article-title>Striking electrocardiographic changes associated with pheochromocytoma</article-title>
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            <year>1976</year>
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      </ref>
    </ref-list>
  </back>
  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Case Reports</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Pheochromocytoma-induced segmental myocardial dysfunction mimicking an acute myocardial infarction in a patient with normal coronary arteries</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>We report a case of pheochromocytoma-induced segmental myocardial dysfunction and electrocardiographic abnormalities mimicking an acute anterior myocardial infarction, probably due to coronary spasm. Coronary angiography showed normal coronaries, and the electrocardiographic and echocardiographic changes resolved completely after therapy with an alpha-adrenergic blocker and tumor removal. Our case illustrates the importance of maintaining a high index of suspicion in patients presenting with an unexpected myocardial event and a hypertensive crisis.</p>
      </abstract>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p>Pheochromocytoma-induced segmental myocardial dysfunction mimicking an acute myocardial infarction in a patient with normal coronary arteries</p>
      <p>
        <bold>Eduardo S. Darz&#233;; Roberto L. Von Sohsten</bold>
      </p>
      <p>Servi&#231;o de Cardiologia/Ecocardiografia - Hospital Alian&#231;a, Salvador, BA and Cardiology Division - John F. Kennedy Medical Center - Salvador, BA, Brazil - Atlantis, FL, USA</p>
      <p>
        <xref ref-type="corresp" rid="back10-body2">Correspondence</xref>
      </p>
      <p>
        <bold>ABSTRACT</bold>
      </p>
      <p>We report a case of pheochromocytoma-induced segmental myocardial dysfunction and electrocardiographic abnormalities mimicking an acute anterior myocardial infarction, probably due to coronary spasm. Coronary angiography showed normal coronaries, and the electrocardiographic and echocardiographic changes resolved completely after therapy with an alpha-adrenergic blocker and tumor removal. Our case illustrates the importance of maintaining a high index of suspicion in patients presenting with an unexpected myocardial event and a hypertensive crisis.</p>
      <p>Pheochromocytoma is a catecholamine-secreting tumor that arises from chromaffin tissue of the sympathetic nervous system. The usual manifestations of this tumor include palpitations, diaphoresis, headache, and paroxysmal hypertension. In addition to the classic symptoms, pheochromocytomas have been rarely associated with acute myocardial infarction and other cardiovascular complications <sup>1</sup>. We report the case of a patient with an adrenal pheochromocytoma and normal coronary arteries who presented with electrocardiographic and echocardiographic findings consistent with an acute anterolateral myocardial infarction (MI), with complete reversal of these abnormalities after alpha-adrenergic treatment.</p>
      <p>
        <bold>Case Report</bold>
      </p>
      <p>A 46-year-old female with history of hypertension was hospitalized with nausea, vomiting, and diaphoresis for 2 days. On physical examination, her initial blood pressure was 230/130 mmHg; heart rate, 132 bpm; and her skin was mottled. The rest of the examination was unremarkable. The electrocardiogram (<xref ref-type="fig" rid="f1-body2">fig. 1</xref>) showed sinus tachycardia with ST segment elevation and deep, symmetric T wave inversion in leads V2 thru V6. The T waves were also inverted in leads I, aVL, II, and aVF. The QT interval was markedly prolonged and Q waves were present in leads II, III, aVF.</p>
      <p content-type="asset">
        <fig id="f1-body2">
          <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf01-en.jpg"/></fig>
      </p>
      <p>She was taken to the cardiac catheterization laboratory where a coronary angiogram showed no significant coronary disease. The left ventriculogram (<xref ref-type="fig" rid="fig2-body2">fig. 2</xref>) showed severe anterolateral and apical hypokinesis. During the procedure, the patient required mechanical ventilation, and the arterial blood pressure became very labile, going from as high as 320/240 mmHg to as low as 70/30 mmHg, warranting the use of vasodilators and, at times, vasopressors. The diagnosis of pheochromocytoma was suspected, and therapy with an alpha-adrenergic blocker was started, which slowly controlled the blood pressure.</p>
      <fig id="fig2-body2">
        <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf02-en.jpg"/></fig>
      <p>Laboratory data included 24-hour urinary vanillylmandelic acid, metanephrine, epinephrine, and norepinephrine levels that were, respectively, 313 mg (normal, 2-10), 76 (normal, 0.3-0.9), 12339 ng (normal, 0-16), and 28316 ng (normal, 11-86). The blood urea nitrogen was 26 mg/dL and creatinine was 2.0 mg/dL. The peak CPK level was 951 IU/L with a normal MB fraction. A computed tomography revealed a rounded mass in the left adrenal gland.</p>
      <p>Blood pressure returned to normal within 5 days of therapy with an intravenous alpha-adrenergic blocker. The patient was extubated, and renal function normalized. Serial echocardiograms were performed on days 1 and 10 of hospitalization. The first confirmed the findings on the left ventriculogram of severe hypokinesis of the anterior and apical walls, and the second demonstrated complete resolution of those abnormalities. After 10 days of therapy, the patient was sent to surgery where an 11.2 x 11.8 x 7.2 cm pheochromocytoma was removed uneventfully. A follow-up electrocardiogram obtained 3 months after the first one showed complete resolution of the ST segment and T wave changes (<xref ref-type="fig" rid="f3-body2">fig. 3</xref>).</p>
      <p content-type="asset">
        <fig id="f3-body2">
          <graphic xlink:href="1678-4170-abc-82-02-0175-00009-gf03-en.jpg"/></fig>
      </p>
      <p>Before discharge, the patient admitted that she had been having frequent bouts of nausea, vomiting, and severe headaches since she was diagnosed with hypertension 5 years before. She continues to do well 1 year after discharge, with good blood pressure control and no recurrence of symptoms.</p>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>Pheochromocytoma-induced myocardial disease may take the form of ventricular hypertrophy due to long-standing hypertension <sup>2</sup>, dilated cardiomyopathy because of persistent and prolonged exposure to high levels of catecholamines <sup>3</sup>, or, rarely, it may mimic an acute myocardial infarction <sup>4-9</sup>.</p>
      <p>We report the case of a rare association between pheochromocytoma and reversible myocardial dysfunction in a patient with normal coronaries. Only a few cases of a pheochromocytoma crisis causing or mimicking an acute myocardial infarction have been reported in the literature. Their clinical presentations have varied significantly, from a silent myocardial infarction in one of the first cases reported in the literature <sup>4</sup>, to frank cardiogenic shock <sup>5</sup>. Striking electrocardiographic changes suggesting myocardial ischemia are often present in patients with pheochromocytoma and include marked prolongation of the QT interval, deep and symmetric T wave inversion, and ST segment changes <sup>10</sup>. Although these are very suggestive of myocardial ischemia, they have been described in other conditions such as subarachnoid hemorrhage and the use of certain drugs <sup>10</sup>. None of these situations is likely in the present case. Wall motion abnormalities have not been well characterized, but both segmental<sup>6</sup> and global <sup>7</sup> myocardial dysfunction have been reported. Although they are commonly associated, the ischemic ECG changes may not be accompanied by wall motion abnormalities <sup>8</sup>. Additionally, despite striking ECG changes and myocardial dysfunction, serum markers of myocyte necrosis may or may not be elevated <sup>7</sup>. In our case, the significant elevation in the CPK levels is probably of skeletal muscle origin and not myocardial injury because the MB fraction was normal.</p>
      <p>The pathophysiology of myocardial dysfunction associated with pheochromocytoma has been linked to either a direct toxic effect induced by catecholamines <sup>3</sup>, or myocardial stunning caused by coronary spasm <sup>9</sup>. These changes along with the ECG abnormalities are commonly reversible after treatment with alpha-adrenergic blockers and tumor removal <sup>5,7</sup>. Although beta-blockers are certainly recommended in patients with an acute MI, they can worsen coronary spasm in cases of pheochromocytomas as a result of unopposed alpha-adrenergic stimulation, and are, therefore, contraindicated in this circumstance.</p>
      <p>Our case illustrates an uncommon presentation of a pheochromocytoma crisis with ECG and wall motion abnormalities mimicking myocardial infarction. The lack of elevation of myocardial necrosis markers and complete reversal of segmental myocardial dysfunction suggest stunning due to coronary spasm as the underlying mechanism. Our report also reinforces the importance of maintaining a high index of suspicion in patients who present with an unexpected myocardial infarction associated with a hypertensive crisis. The relevance of a thorough history cannot be overemphasized, because in our case and in a number of cases reported in the literature, symptoms suggestive of pheochromocytoma are found to have been present for years prior to the acute event, and are frequently missed on presentation <sup>1</sup>. These patients ought to be screened for pheochromocytoma, as early treatment may prevent serious morbidity and mortality.</p>
      <p>
        <bold>References</bold>
      </p>
      <p>
        <fn id="back10-body2" fn-type="corresp">
          <p>Correspondence to</p>
          <p>Eduardo S. Darz&#233;</p>
          <p> Servi&#231;o de Cardiologia/Ecocardiografia - Hospital Alian&#231;a</p>
          <p> Av. Juracy Magalh&#227;es Jr., 2096</p>
          <p> Salvador, BA - Brazil - Cep 41920-000</p>
          <p> E-mail: </p>
          <p>
            <email xlink:href="esdarze@ufba.br">esdarze@ufba.br</email>
          </p>
        </fn>
      </p>
      <p>Received: 2/24/03</p>
      <p>Accepted: 5/26/03</p>
    </body>
  </sub-article>
</article>
