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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" article-type="case-report" dtd-version="1.1" specific-use="sps-1.8" xml:lang="en">
  <front>
    <journal-meta>
      <journal-id journal-id-type="nlm-ta">Arq Bras Cardiol</journal-id>
      <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>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">CgXt43bnTbJyQTzVn4xmnsF</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2018001600629</article-id>
      <article-id pub-id-type="doi">10.5935/abc.20180205</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Anatomopathological Correlation</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Case 5 / 2018 - Acute Respiratory Failure and Cardiogenic Shock in a Patient in the First Trimester of Pregnancy with Mechanical Mitral Valve Prosthesis Implant</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>&#193;vila</surname>
            <given-names>Walk&#237;ria Samuel</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Caldas</surname>
            <given-names>Vin&#237;cius Ara&#250;jo de Freitas Chagas</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Batista</surname>
            <given-names>Daniel Valente</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Gutierrez</surname>
            <given-names>Paulo Sampaio</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
        </contrib>
        <aff id="aff1">
          <institution content-type="orgname">Universidade de S&#227;o Paulo</institution>
          <institution content-type="orgdiv1">Faculdade de Medicina</institution>
          <institution content-type="orgdiv2">Hospital das Cl&#237;nicas</institution>
          <addr-line>
            <city>S&#227;o Paulo</city>
            <state>SP</state>
          </addr-line>
          <country country="BR">Brazil</country>
          <institution content-type="original">Instituto do Cora&#231;&#227;o (InCor) HC-FMUSP, S&#227;o Paulo, SP - Brazil</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Vera Demarchi Aiello,</bold> Avenida Dr. En&#233;as de Carvalho Aguiar, 44, subsolo, bloco I, Cerqueira C&#233;sar. Postal Code 05403-000, S&#227;o Paulo, SP - Brasil. E-mail: <email>demarchi@cardiol.br</email>, <email>anpvera@incor.usp.br</email>
				</corresp>
      </author-notes>
      <pub-date pub-type="epub-ppub">
        <month>10</month>
        <year>2018</year>
      </pub-date>
      <volume>111</volume>
      <issue>4</issue>
      <fpage>629</fpage>
      <lpage>634</lpage>
      <history>
        <date date-type="received">
          <day>06</day>
          <month>09</month>
          <year>2018</year>
        </date>
        <date date-type="rev-recd">
          <day>19</day>
          <month>09</month>
          <year>2018</year>
        </date>
        <date date-type="accepted">
          <day>20</day>
          <month>09</month>
          <year>2018</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by/4.0/" xml:lang="en">
          <license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Respiratory Insufficiency</kwd>
        <kwd>Heart Defects, Congenital</kwd>
        <kwd>Heart Valve Prosthesis</kwd>
        <kwd>Shock, Cardiogenic</kwd>
        <kwd>Pregnancy.</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <p>This case describes a 36-year-old female patient born in the state of Alagoas, and residing in the municipality of Guarulhos, state of S&#227;o Paulo, Brazil, married, illiterate, admitted at the Gynecology and Obstetric Service after clinical diagnosis of upper airway infection at the 9<sup>th</sup> week of the 1<sup>st</sup> pregnancy.</p>
    <p>She was followed up at the outpatient clinic specialized in congenital heart defects due to complex congenital heart disease, which included interatrial defect associated with patent ductus arteriosus and interventricular septal defect, as well as a left atrioventricular septal defect. She underwent surgery at the age of eight, consisting of atrioseptoplasty, ventriculoseptoplasty and mitral valve replacement by a mechanical prosthesis. She had paroxysmal atrial fibrillation, with a previous thromboembolic event, left hemisphere ischemic stroke, without neurological sequelae, being asymptomatic from the cardiovascular point of view, in functional class I (NYHA classification) at the last consultation in April 2018. She used only warfarin, undergoing regular follow-up of prothrombin time control/INR, having maintained it between 2-3 in the last controls.</p>
    <p>During hospitalization in the Obstetrics Service, warfarin was replaced by enoxaparin 1mg / kg, subcutaneously, every 12 hours, and during the evolution she had atrial fibrillation with high ventricular response accompanied by dyspnea at rest and orthopnea, being subsequently referred to the Emergency Service of the Cardiology Hospital.</p>
    <p>The physical examination at admission (May 30, 2018) showed regular overall health status, normal skin color, hydrated, anicteric, conscious, oriented, without alterations at the neurological examination. Cardiovascular examination showed regular heart rhythm, with heart rate at 115 beats per minute, holosystolic murmur, with prosthesis profile, at the superior left sternal border 2 + / 6 +, good peripheral perfusion. The respiratory system showed crackling rales on the left lung base, and mild dyspnea at rest. Gravid abdomen, with no signs of hepatic congestion. Extremities without edema, with no discomfort or pain in the calves.</p>
    <p>The laboratory results at admission (May 30, 2018) were: hemoglobin 12.4 g / dL; leukocytes 13,050/mm&#179; (band cells 1%, segmented 79%, eosinophils 1%); platelets 120,000/mm&#179;; C-reactive protein: 74.6mg / dL; Urinalysis: Leukocytes 16,000/mL, negative nitrite test, bacteria 1+/4+, Urinary culture at the hospital of origin with multisensitive <italic>E.coli</italic>.</p>
    <p>The admission electrocardiogram (May 30, 2018) (<xref ref-type="fig" rid="f1">Figure 1</xref>) showed sinus rhythm, heart rate of 115 bpm, indirect signs of right atrial overload.</p>
    <p>
      <fig id="f1">
        <label>Figure 1</label>
        <caption>
          <title>Admission ECG: sinus rhythm with indirect signs of left atrial overload and right atrial overload (Pe&#241;aloza-Tranquesi).</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf01.jpg"/></fig>
    </p>
    <p>The admission chest x-ray (May 30, 2018) (<xref ref-type="fig" rid="f2">Figure 2</xref>) disclosed indirect signs of pulmonary congestion ("cottony" infiltrate, predominantly bibasal), peri-hilar air bronchogram on the right and image compatible with mechanical prosthesis in the mitral position.</p>
    <p>
      <fig id="f2">
        <label>Figure 2</label>
        <caption>
          <title>Admission chest x-ray: signs of congestion and pulmonary infection (air bronchogram).</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf02.jpg"/></fig>
    </p>
    <p>The initial diagnosis at hospitalization was bronchopneumonia, pulmonary congestion, atrial fibrillation with high ventricular response, and a single, nine-week non-ectopic pregnancy, and she was prescribed: Ceftriaxone, Clarithromycin, Oseltamivir, Furosemide and Sotalol. The requested exams included blood culture, H1N1 virus screening, transthoracic echocardiography, and Anti-Xa factor.</p>
    <p>During the evolution she showed signs and symptoms of pulmonary infectious disease (cough, dyspnea, leukocytosis with left shift, high PCR, with negative H1N1), and it was decided to discontinue Oseltamivir and implement empirical antibiotic therapy with Meropenem.</p>
    <p>Compared with the patient&#8217;s last transthoracic echocardiogram, the transthoracic echocardiogram carried out on June 4, 2018 disclosed a marked increase in the mitral transvalvular gradient (maximum diastolic gradient of 39mmHg and mean of 25mmHg), in addition to an increase of pressures in the right chambers, with right ventricular systolic pressure of 75 mmHg, with no evidence of thrombi or vegetation (<xref ref-type="table" rid="t1">Table 1</xref>).</p>
    <table-wrap id="t1">
      <label>Table 1</label>
      <caption>
        <title>Echocardiographic evolution</title>
      </caption>
      <table frame="hsides" rules="groups">
        <colgroup>
          <col width="25%"/>
          <col width="25%"/>
          <col width="25%"/>
          <col width="25%"/>
        </colgroup>
        <thead>
          <tr>
            <th align="left" rowspan="2">Echocardiographic measures</th>
            <th align="center" colspan="3" style="border-bottom-width:thin;border-bottom-style:solid">Date</th>
          </tr>
          <tr>
            <th align="center">Pre-admission</th>
            <th align="center">June 04, 2018</th>
            <th align="center">June 14, 2018</th>
          </tr>
        </thead>
        <tbody>
          <tr>
            <td align="left">Aorta (mm)</td>
            <td align="center">24</td>
            <td align="center">28</td>
            <td align="center">28</td>
          </tr>
          <tr>
            <td align="left">Left atrium (mm)</td>
            <td align="center">45</td>
            <td align="center">55</td>
            <td align="center">57</td>
          </tr>
          <tr>
            <td align="left">Right ventricle (mm)</td>
            <td align="center">24</td>
            <td align="center">26</td>
            <td align="center">41</td>
          </tr>
          <tr>
            <td align="left">Septum (mm)</td>
            <td align="center">10</td>
            <td align="center">9</td>
            <td align="center">9</td>
          </tr>
          <tr>
            <td align="left">Posterior wall (mm)</td>
            <td align="center">9</td>
            <td align="center">10</td>
            <td align="center">9</td>
          </tr>
          <tr>
            <td align="left">Left Ventricle Diast./Syst. (mm)</td>
            <td align="center">53/33</td>
            <td align="center">40/28</td>
            <td align="center">40/25</td>
          </tr>
          <tr>
            <td align="left">LVEF (%)</td>
            <td align="center">Normal</td>
            <td align="center">Normal</td>
            <td align="center">Normal</td>
          </tr>
          <tr>
            <td align="left">Max. trans-mitral gradient (mm Hg) </td>
            <td align="center">16</td>
            <td align="center">39</td>
            <td align="center">45</td>
          </tr>
          <tr>
            <td align="left">Mean trans-mitral gradient (mm Hg)</td>
            <td align="center">6</td>
            <td align="center">25</td>
            <td align="center">30</td>
          </tr>
          <tr>
            <td align="left">Mitral prosthesis (mobility)</td>
            <td align="center">Good</td>
            <td align="center">Low</td>
            <td align="center">Low </td>
          </tr>
          <tr>
            <td align="left">Thrombus</td>
            <td align="center">No</td>
            <td align="center">No</td>
            <td align="center">Yes</td>
          </tr>
          <tr>
            <td align="left">RV Syst pressure (mm Hg)</td>
            <td align="center">46 </td>
            <td align="center">75 </td>
            <td align="center">73</td>
          </tr>
        </tbody>
      </table>
      <table-wrap-foot>
        <fn id="TFN1">
          <p>Diast.: diastolic; Syst.: systolic; LVEF: left ventricular ejection fraction; RV: right ventricle.</p>
        </fn>
      </table-wrap-foot>
    </table-wrap>
    <p>Furosemide and metoprolol were added to the antibiotics aiming at heart rate control, in addition to anticoagulation maintenance with enoxaparin with adequate levels of Anti-Xa factor (between 0.8 and 1U/mL) with improvement of clinical status. A transesophageal echocardiogram was requested for a more adequate assessment of the valve prosthesis (June 14, 2018). This examination showed the reduction in the mobility of the mitral prosthesis components, with a high mean transvalvular gradient (30 mmHg and a hypoechogenic image occupying the central region of the atrial face of the prosthesis, compatible with a thrombus). Its measurements, even underestimated, since it was difficult to determine its full extent using the two-dimensional methodology, reached values of 0.9 x 1.3 cm, resulting in an area of 1.17 cm&#178; (important when &gt; 0.8 cm&#178;) and, thus, the surgical intervention was indicated, since it was available at the service (Table 1). Given the echocardiographic diagnosis of mitral valve prosthesis thrombosis, surgical treatment of the mitral valve was indicated, despite the gestational age, due to the high risk of maternal death. Intravenous unfractionated heparin was then started in an infusion pump while awaiting the surgical procedure.</p>
    <p>During this period, the patient developed a new picture of marked dyspnea, with marked congestion (<xref ref-type="fig" rid="f3">Figure 3</xref>), tachycardia, and fever, requiring invasive ventilatory support with orotracheal intubation, and hypotension requiring vasopressor agent (noradrenaline). She went into cardiorespiratory arrest for 6 minutes, with spontaneous circulation and frank shock, requiring high doses of noradrenaline, adrenaline and vasopressin. She remained in shock and, despite the measures, presented with bradycardia and asystole and died (7h22; June 18, 2018). (Dr. <italic>Walk&#237;ria Samuel &#193;vila)</italic></p>
    <p>
      <fig id="f3">
        <label>Figure 3</label>
        <caption>
          <title>Chest X-ray showing significant pulmonary congestion.</title>
        </caption>
        <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf03.jpg"/></fig>
    </p>
    <sec>
      <title>Clinical aspects</title>
      <p>The case reported is of a 36-year-old pregnant woman with repaired complex congenital heart disease, with a mechanical mitral valve prosthesis implanted 28 years before, paroxysmal atrial fibrillation, and a history of thromboembolism, a triad that characterizes a high thromboembolic risk.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
      <p>Notwithstanding, the patient maintained the adequate anticoagulation goal (INR = 3) until the pregnancy diagnosis, when the anticoagulation regimen of warfarin was replaced by enoxaparin due to the risk of fetal warfarin syndrome, which occurs between the 6<sup>th</sup> and 12<sup>th</sup> weeks of gestation (characterized by nasal hypoplasia, dysplasia of the bony epiphyses, limb deformities, neurological and respiratory problems).<xref ref-type="bibr" rid="B1">1</xref> However, there is less evidence of the erratic bioavailability and distribution of enoxaparin during pregnancy,<xref ref-type="bibr" rid="B2">2</xref> although it constitutes a current challenge to define the best anticoagulation strategy in this population with high thromboembolic risk.</p>
      <p>As a therapeutic option for the treatment of prosthesis thrombosis, the thrombolysis with streptokinase or alteplase, guided by serial transesophageal echocardiography, was shown to be safe and effective.<xref ref-type="bibr" rid="B3">3</xref> However, considering the clinical situation of the patient, such as NYHA functional class IV, the need for intensive care, mechanical mitral prosthesis with a thrombus size &gt; 0.8 cm&#178;, the surgical treatment was chosen.<xref ref-type="bibr" rid="B4">4</xref><sup>-</sup><xref ref-type="bibr" rid="B6">6</xref><sup>)</sup> Despite the established supportive care, while waiting for the previously indicated definitive surgical therapy, the patient showed clinical deterioration and died, alerting us to the potential severity of a prosthesis thrombosis picture, which requires an emergency procedure (surgical or thrombolysis), regardless of aggravating factors such as the pregnancy itself or associated infections. (Dr. Vin&#237;cius Ara&#250;jo de Freitas Chagas Caldas and Dr. Daniel Valente Batista)</p>
      <p><bold>Diagnostic hypotheses:</bold> cardiogenic shock, acute pulmonary edema, thrombosis of the mechanical mitral prosthesis, systemic inflammatory response syndrome with possible pulmonary infectious focus. (Dr. Vin&#237;cius Ara&#250;jo de Freitas Chagas Caldas and Dr. Daniel Valente Batista)</p>
    </sec>
    <sec>
      <title>Necropsy</title>
      <p>The gravid uterus contained an apparently well-formed fetus. The mother had a mild degree of pulmonary emphysema and significant alterations in the cardiovascular system, with a patent ductus arteriosus (<xref ref-type="fig" rid="f4">Figure 4</xref>) measuring 2 mm in diameter; small interventricular septal defect (<xref ref-type="fig" rid="f5">Figure 5</xref>); surgical sutures in the atrial septum, possibly corresponding to the defect closure; embolism (or thrombosis) of the left subclavian vein; and mechanical valve prosthesis in the mitral position, occluded by the presence of a thrombus-like mass in the two faces (<xref ref-type="fig" rid="f6">Figure 6</xref>). Microscopic study confirmed the nature of this mass, with absence of microorganisms (<xref ref-type="fig" rid="f7">Figure 7</xref>). There were small infarcts in the right kidney, possibly due to embolism caused by the prosthesis thrombus, and in the subendocardial region of the left ventricle. The lungs showed many alterations, almost in their entirety, with a histopathological pattern of organizing pneumonia (<xref ref-type="fig" rid="f8">Figure 8</xref>). Furthermore, demonstrating congestion, there were macrophages containing hemosiderin, but not in large numbers; and dilation of lymphatic vessels. (Paulo Sampaio Gutierrez)</p>
      <p>
        <fig id="f4">
          <label>Figure 4</label>
          <caption>
            <title>Great arteries of the heart showing patent ductus arteriosus (between the arrows).</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf04.jpg"/></fig>
      </p>
      <p>
        <fig id="f5">
          <label>Figure 5</label>
          <caption>
            <title>Transversal section of the heart at the region of the ventricles showing a muscular ventricular septal defect (arrow). RV- right ventricle; LV- left ventricle.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf05.jpg"/></fig>
      </p>
      <p>
        <fig id="f6">
          <label>Figure 6</label>
          <caption>
            <title>The mechanical valve prosthesis is seen from the opened left atrium. The left panel shows a massive thrombus occluding almost completely the valvar orifice. After removal of the thrombus, (right panel), it is demonstrated that the prosthesis shows adequate opening.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf06.jpg"/></fig>
      </p>
      <p>
        <fig id="f7">
          <label>Figure 7</label>
          <caption>
            <title>Histological section of the mass adhered to the valve prosthesis, constituted by thrombus, with fibrin and moderate amounts of inflammatory cells, without microorganisms. Hematoxylin &amp; eosin staining; objective magnification: 10x.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf07.jpg"/></fig>
      </p>
      <p>
        <fig id="f8">
          <label>Figure 8</label>
          <caption>
            <title>Histological section of the lung showing alveoli filled by mononuclear cells and presence of collagen. Hematoxylin &amp; eosin staining; objective magnification: 10x.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf08.jpg"/></fig>
      </p>
      <p><bold>Anatomopathological diagnoses:</bold> Congenital heart disease with interatrial defect, interventricular defect, patent ductus arteriosus, and thrombosis of the mechanical mitral valve.</p>
      <p><bold>Cause of death:</bold> Mitral valve obstruction / organizing pneumonia (Dr. Paulo Sampaio Gutierrez)</p>
    </sec>
    <sec sec-type="conclusions">
      <title>Comment</title>
      <p>Although the main problem that led to the death of the patient was thrombosis of the mitral valve prosthesis - emphasizing the difficulty of managing the coagulation system during pregnancy - it is worth mentioning that the lungs were also very affected, with a pattern of organizing pneumonia. It is important to emphasize that the diagnosis of &#8220;organizing pneumonia&#8221; refers to a picture that may follow not only classical bacterial pneumonia, but also several other situations, such as viral infections, exposure to toxic inhalants and others.<xref ref-type="bibr" rid="B7">7</xref> However, marked congestion, albeit sudden, is not listed among the possible causes of this process. Therefore, in the present case, organizing pneumonia must have been due to the respiratory picture, possibly an infectious one, whether viral or bacterial, which was already present when the patient was admitted at the institution.</p>
    </sec>
  </body>
  <back>
    <fn-group>
      <fn fn-type="other">
        <p><bold>Section Editor:</bold> Alfredo Jos&#233; Mansur (<email>ajmansur@incor.usp.br</email>)</p>
      </fn>
      <fn fn-type="other">
        <p><bold>Associated Editors:</bold> Desid&#233;rio Favarato (<email>dclfavarato@incor.usp.br</email>)</p>
        <p> Vera Demarchi Aiello (<email>anpvera@incor.usp.br</email>)</p>
      </fn>
    </fn-group>
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  </back>
  <sub-article article-type="translation" id="s1" xml:lang="pt">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Correla&#231;&#227;o Anatomocl&#237;nica</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Caso 5 /2018 - Insufici&#234;ncia Respirat&#243;ria Aguda e Choque Cardiog&#234;nico em Mulher no Primeiro Trimestre de Gravidez e Portadora de Pr&#243;tese Mitral Mec&#226;nica</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>&#193;vila</surname>
            <given-names>Walk&#237;ria Samuel</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Caldas</surname>
            <given-names>Vin&#237;cius Ara&#250;jo de Freitas Chagas</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Batista</surname>
            <given-names>Daniel Valente</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Gutierrez</surname>
            <given-names>Paulo Sampaio</given-names>
          </name>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <aff id="aff2">
          <institution content-type="original">Instituto do Cora&#231;&#227;o (InCor) HC-FMUSP, S&#227;o Paulo, SP - Brasil</institution>
        </aff>
      </contrib-group>
      <author-notes>
        <corresp id="c2"><bold>Correspond&#234;ncia: Vera Demarchi Aiello,</bold> Avenida Dr. En&#233;as de Carvalho Aguiar, 44, subsolo, bloco I, Cerqueira C&#233;sar. CEP 05403-000, S&#227;o Paulo, SP - Brasil. E-mail: <email>demarchi@cardiol.br</email>, <email>anpvera@incor.usp.br</email>
				</corresp>
      </author-notes>
      <kwd-group xml:lang="pt">
        <title>Palavras-chave</title>
        <kwd>Insufici&#234;ncia Respirat&#243;ria</kwd>
        <kwd>Cardiopatias Cong&#234;nitas</kwd>
        <kwd>Pr&#243;teses Valvulares Card&#237;acas</kwd>
        <kwd>Gravidez</kwd>
        <kwd>Choque Cardiog&#234;nico.</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>Trata-se de paciente do sexo feminino, 36 anos, natural do estado de Alagoas, e procedente da regi&#227;o de Guarulhos, grande S&#227;o Paulo, casada, analfabeta, admitida no Servi&#231;o de Ginecologia e Obstetr&#237;cia ap&#243;s diagn&#243;stico cl&#237;nico de infec&#231;&#227;o de vias a&#233;reas superiores no curso da 9&#170; semana da 1&#170; gesta&#231;&#227;o.</p>
      <p>Fazia acompanhamento no ambulat&#243;rio especializado em cardiopatias cong&#234;nitas por cardiopatia cong&#234;nita complexa que inclu&#237;a comunica&#231;&#227;o interatrial associada &#224; persist&#234;ncia do canal arterial e comunica&#231;&#227;o interventricular, al&#233;m de defeito do septo atrioventricular esquerdo. Operada aos oito anos de idade com realiza&#231;&#227;o de atriosseptoplastia, ventriculosseptoplastia e troca valvar mitral por pr&#243;tese mec&#226;nica. Apresentava fibrila&#231;&#227;o atrial parox&#237;stica, com evento tromboemb&#243;lico pr&#233;vio, acidente vascular cerebral isqu&#234;mico em hemisf&#233;rio esquerdo, sem sequelas neurol&#243;gicas, mantendo-se assintom&#225;tica do ponto de vista cardiovascular, em classe funcional I (classifica&#231;&#227;o NYHA) na &#250;ltima consulta em abril de 2018. Fazia uso apenas de varfarina, em acompanhamento regular do controle de tempo de protrombina/INR, mantido em &#250;ltimos controles entre 2-3.</p>
      <p>Durante interna&#231;&#227;o no servi&#231;o de obstetr&#237;cia, a varfarina foi substitu&#237;da por enoxaparina 1mg/kg, via subcut&#226;nea, de 12/12 horas, e na evolu&#231;&#227;o apresentou fibrila&#231;&#227;o atrial de alta resposta ventricular acompanhada de dispneia ao repouso e ortopneia, sendo ent&#227;o encaminhada ao servi&#231;o de emerg&#234;ncia do hospital de cardiologia.</p>
      <p>O exame f&#237;sico de entrada (30 de maio de 2018) mostrava paciente em regular estado geral, corada, hidratada, anict&#233;rica, vigil, orientada, sem altera&#231;&#245;es ao exame neurol&#243;gico. Exame cardiovascular em ritmo card&#237;aco regular, com frequ&#234;ncia card&#237;aca em 115 batimentos por minuto, sopro holossist&#243;lico, com perfil de pr&#243;tese, em borda esternal esquerda alta 2+/6+, boa perfus&#227;o perif&#233;rica. Aparelho respirat&#243;rio com estertores crepitantes em base pulmonar esquerda, dispneia leve ao repouso. Abdome grav&#237;dico, sem sinais de congest&#227;o hep&#225;tica. Extremidades sem edema, panturrilhas livres.</p>
      <p>Os resultados dos exames laboratoriais de entrada (30 de maio de 2018): hemoglobina 12,4 g/dL; leuc&#243;citos 13050/mm&#179; (bast&#245;es 1%; segmentados 79%; eosin&#243;filos 1%); plaquetas 120mil/mm&#179;; Prote&#237;na C reativa: 74,6mg/dL; Urina I: Leuc&#243;citos 16mil/mL, nitrito negativo, bact&#233;rias 1+/4+, Urocultura do hospital de origem com E.coli multissens&#237;vel.</p>
      <p>O eletrocardiograma de entrada (30 de maio de 2018) (<xref ref-type="fig" rid="f9">Figura 1</xref>) mostrava ritmo sinusal, frequ&#234;ncia card&#237;aca de 115 bpm, sinais indiretos de sobrecarga atrial direita.</p>
      <p>
        <fig id="f9">
          <label>Figura 1</label>
          <caption>
            <title>ECG admiss&#227;o: ritmo sinusal com sinais indiretos de sobrecarga atrial esquerda e sobrecarga atrial direita (Pe&#241;aloza-Tranquesi).</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf01-pt.jpg"/></fig>
      </p>
      <p>A radiografia de t&#243;rax de admiss&#227;o (30 de maio 2018) (<xref ref-type="fig" rid="f10">Figura 2</xref>) revelou sinais indiretos de congest&#227;o pulmonar (infiltrado &#8220;algodonoso&#8221; predom&#237;nio bibasal), broncograma a&#233;reo peri-hilar &#224; direita e imagem compat&#237;vel com pr&#243;tese mec&#226;nica em posi&#231;&#227;o mitral.</p>
      <p>
        <fig id="f10">
          <label>Figura 2</label>
          <caption>
            <title>Radiografia de t&#243;rax admiss&#227;o: sinais de congest&#227;o e infec&#231;&#227;o pulmonar (broncograma a&#233;reo).</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf02-pt.jpg"/></fig>
      </p>
      <p>O diagn&#243;stico inicial da interna&#231;&#227;o foi de broncopneumonia, congest&#227;o pulmonar, fibrila&#231;&#227;o atrial de alta resposta ventricular, gesta&#231;&#227;o &#250;nica t&#243;pica de nove semanas, sendo prescritos: Ceftriaxona, Claritromicina, Oseltamivir, Furosemida e Sotalol. Os exames solicitados inclu&#237;ram hemocultura, pesquisa de v&#237;rus H1N1, ecocardiograma transtor&#225;cico e fator Anti-Xa.</p>
      <p>A evolu&#231;&#227;o mostrava sinais e sintomas de quadro infeccioso pulmonar (tosse, dispneia, leucocitose com desvio &#224; esquerda, PCR elevado, com H1N1 negativo), sendo optado por suspender Oseltamivir e escalonar antibioticoterapia emp&#237;rica para Meropenem.</p>
      <p>Comparando com o &#250;ltimo ecocardiograma transtor&#225;cico ambulatorial da paciente, o ecocardiograma transtor&#225;cico de 04 de junho de 2018 revelou aumento acentuado de gradiente transvalvar mitral (gradiente diast&#243;lico m&#225;ximo de 39mmHg e m&#233;dio de 25mmHg), al&#233;m do aumento de press&#245;es em c&#226;maras direitas, com press&#227;o sist&#243;lica de ventr&#237;culo direito de 75mmHg, sem evid&#234;ncia de trombos ou vegeta&#231;&#227;o (<xref ref-type="table" rid="t2">Tabela 1</xref>).</p>
      <table-wrap id="t2">
        <label>Tabela 1</label>
        <caption>
          <title>Evolu&#231;&#227;o ecocardiogr&#225;fica</title>
        </caption>
        <table frame="hsides" rules="groups">
          <colgroup>
            <col width="25%"/>
            <col width="25%"/>
            <col width="25%"/>
            <col width="25%"/>
          </colgroup>
          <thead>
            <tr>
              <th align="left" rowspan="2">Medidas ecocardiogr&#225;ficas</th>
              <th align="center" colspan="3" style="border-bottom-width:thin;border-bottom-style:solid">Data</th>
            </tr>
            <tr>
              <th align="left">Pr&#233;-interna&#231;&#227;o</th>
              <th align="left">04 junho 2018</th>
              <th align="left">14 junho 2018</th>
            </tr>
          </thead>
          <tbody>
            <tr>
              <td align="left">Aorta (mm)</td>
              <td align="left">24</td>
              <td align="left">28</td>
              <td align="left">28</td>
            </tr>
            <tr>
              <td align="left">&#193;trio esquerdo (mm)</td>
              <td align="left">45</td>
              <td align="left">55</td>
              <td align="left">57</td>
            </tr>
            <tr>
              <td align="left">Ventr&#237;culo direito (mm)</td>
              <td align="left">24</td>
              <td align="left">26</td>
              <td align="left">41</td>
            </tr>
            <tr>
              <td align="left">Septo (mm)</td>
              <td align="left">10</td>
              <td align="left">9</td>
              <td align="left">9</td>
            </tr>
            <tr>
              <td align="left">Parede posterior (mm)</td>
              <td align="left">9</td>
              <td align="left">10</td>
              <td align="left">9</td>
            </tr>
            <tr>
              <td align="left">Ventr&#237;culo esquerdo Diast./Sist. (mm)</td>
              <td align="left">53/33</td>
              <td align="left">40/28</td>
              <td align="left">40/25</td>
            </tr>
            <tr>
              <td align="left">FEVE (%)</td>
              <td align="left">Normal</td>
              <td align="left">Normal </td>
              <td align="left">Normal </td>
            </tr>
            <tr>
              <td align="left">Gradiente trans-mitral max (mm Hg) </td>
              <td align="left">16</td>
              <td align="left">39</td>
              <td align="left">45</td>
            </tr>
            <tr>
              <td align="left">Gradiente trans-mitral m&#233;dio (mm Hg)</td>
              <td align="left">6</td>
              <td align="left">25</td>
              <td align="left">30</td>
            </tr>
            <tr>
              <td align="left">Pr&#243;tese mitral (mobilidade)</td>
              <td align="left">Boa</td>
              <td align="left">Baixa</td>
              <td align="left">Baixa </td>
            </tr>
            <tr>
              <td align="left">Trombo</td>
              <td align="left">N&#227;o</td>
              <td align="left">n&#227;o</td>
              <td align="left">Sim</td>
            </tr>
            <tr>
              <td align="left">Press&#227;o Sist VD (mm Hg)</td>
              <td align="left">46 </td>
              <td align="left">75 </td>
              <td align="left">73</td>
            </tr>
          </tbody>
        </table>
        <table-wrap-foot>
          <fn id="TFN2">
            <p>Diast.: diast&#243;lico; Sist.: sist&#243;lico; FEVE: fra&#231;&#227;o de eje&#231;&#227;o do ventr&#237;culo esquerdo; VD: ventr&#237;culo direito.</p>
          </fn>
        </table-wrap-foot>
      </table-wrap>
      <p>Aos antibi&#243;ticos associaram-se furosemida e metoprolol para controle de frequ&#234;ncia card&#237;aca, al&#233;m de manuten&#231;&#227;o da anticoagula&#231;&#227;o com enoxaparina com n&#237;veis adequados de fator Anti-Xa (entre 0,8 e 1U/mL) com melhora do quadro cl&#237;nico. Para avalia&#231;&#227;o mais adequada da pr&#243;tese valvar foi solicitado ecocardiograma transesof&#225;gico (14 de junho de 2018). Neste exame, foi observada a redu&#231;&#227;o de mobilidade dos elementos de sua pr&#243;tese mitral, com gradiente m&#233;dio transvalvar elevado (30 mmHg e imagem hipoecog&#234;nica ocupando a regi&#227;o central da face atrial da pr&#243;tese, compat&#237;vel com trombo). Suas medidas, mesmo subestimadas, dado que ficava dif&#237;cil determinar toda a sua extens&#227;o &#224; metodologia bidimensional, alcan&#231;ou valores de 0,9 x 1,3 cm, resultando em &#225;rea 1,17 cm&#178; (importante quando &gt; 0, 8 cm&#178;), sendo ent&#227;o decidido por indica&#231;&#227;o cir&#250;rgica, visto a disponibilidade desta no servi&#231;o em quest&#227;o. (<xref ref-type="table" rid="t2">Tabela 1</xref>)</p>
      <p>Dado o diagn&#243;stico ecocardiogr&#225;fico de trombose de pr&#243;tese mec&#226;nica mitral, foi indicado o tratamento cir&#250;rgico da valva mitral, a despeito da idade gestacional pelo alto risco de morte materna. Foi ent&#227;o iniciada a heparina n&#227;o fracionada intravenosa em bomba de infus&#227;o no aguardo do procedimento cir&#250;rgico.</p>
      <p>Neste per&#237;odo a paciente evoluiu com novo quadro de dispneia acentuada, com congest&#227;o acentuada (<xref ref-type="fig" rid="f11">Figura 3</xref>), taquicardia e febre sendo necess&#225;rias medidas de suporte ventilat&#243;rio invasivo com intuba&#231;&#227;o orotraqueal, hipotens&#227;o com necessidade de in&#237;cio de vasopressor (noradrenalina). Apresentou parada cardiorrespirat&#243;ria por 6 minutos, com retorno a circula&#231;&#227;o espont&#226;nea em franco choque e necessidade de doses altas de noradrenalina, adrenalina e vasopressina. Continuou em choque e, apesar das medidas, apresentou quadro de bradicardia e assistolia e faleceu (7h22; 18 jun 2018). (Dra. <italic>Walk&#237;ria Samuel &#193;vila)</italic></p>
      <p>
        <fig id="f11">
          <label>Figura 3</label>
          <caption>
            <title>Radiografia de t&#243;rax revelando congest&#227;o pulmonar importante.</title>
          </caption>
          <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf03-pt.jpg"/></fig>
      </p>
      <sec>
        <title>Aspectos cl&#237;nicos</title>
        <p>O caso relatado considera gestante primigesta de 36 anos de idade, portadora de cardiopatia cong&#234;nita complexa corrigida, com pr&#243;tese mec&#226;nica mitral com tempo de implante de 28 anos, fibrila&#231;&#227;o atrial parox&#237;stica e antecedentes de tromboembolismo, a triade que caracteriza alto risco tromboemb&#243;lico.&#185;</p>
        <p>Apesar disso, a paciente mantinha a meta de anticoagula&#231;&#227;o (INR = 3) adequada at&#233; o diagn&#243;stico da gesta&#231;&#227;o quando foi trocado o esquema de anticoagula&#231;&#227;o da varfarina por enoxaparina pelo risco de s&#237;ndrome varfar&#237;nica fetal que ocorre entre a 6&#170; e 12&#170; semana de gesta&#231;&#227;o (caracterizada por hipoplasia nasal, displasia das ep&#237;fises &#243;sseas, deformidades de membros, problemas neurol&#243;gicos e respirat&#243;rios).<xref ref-type="bibr" rid="B1">1</xref> Por&#233;m, existem evid&#234;ncias menores da distribui&#231;&#227;o e biodisponibilidade err&#225;tica da enoxaparina durante a gesta&#231;&#227;o,<sup>2</sup> ainda sendo um desafio atual definir a melhor estrat&#233;gia de anticoagula&#231;&#227;o nessa popula&#231;&#227;o de alto risco trombroemb&#243;lico.</p>
        <p>Como op&#231;&#227;o terap&#234;utica ao tratamento de trombose de pr&#243;tese, a tromb&#243;lise com estreptoquinase ou alteplase, guiado por ecocardiograma transesof&#225;gico seriado, mostrou-se segura e eficaz.<xref ref-type="bibr" rid="B3">3</xref> Por&#233;m, frente &#224; situa&#231;&#227;o cl&#237;nica da paciente tais como: classe funcional NYHA IV, necessidade de cuidados intensivos, pr&#243;tese mec&#226;nica mitral com o tamanho do trombo superior a 0,8cm&#178;, optou-se pelo tratamento cir&#250;rgico.<xref ref-type="bibr" rid="B4">4</xref><sup>-</sup><xref ref-type="bibr" rid="B6">6</xref>
				</p>
        <p>Apesar do tratamento de suporte institu&#237;do, em aguardo de terapia definitiva com cirurgia j&#225; indicada, a paciente deteriorou clinicamente e veio a &#243;bito, alertando-nos para a potencial gravidade do quadro de trombose de pr&#243;tese, que exige a conduta de urg&#234;ncia (cir&#250;rgica ou tromb&#243;lise), independente de agravantes ao quadro, como a pr&#243;pria gesta&#231;&#227;o ou infec&#231;&#245;es associadas. <bold>(Dr. Vin&#237;cius Ara&#250;jo de Freitas Chagas Caldas e Dr. Daniel Valente Batista)</bold></p>
        <p><bold>Hip&#243;teses diagn&#243;sticas:</bold> choque cardiog&#234;nico, edema agudo de pulm&#245;es, trombose de pr&#243;tese mec&#226;nica mitral, s&#237;ndrome da resposta inflamat&#243;ria sist&#234;mica com poss&#237;vel foco infeccioso pulmonar. <bold>(Dr. Vin&#237;cius Ara&#250;jo de Freitas Chagas Caldas e Dr. Daniel Valente Batista)</bold></p>
      </sec>
      <sec>
        <title>Necropsia</title>
        <p>No &#250;tero grav&#237;dico havia feto aparentemente bem formado. A m&#227;e tinha grau leve de enfisema pulmonar e altera&#231;&#245;es significativas do sistema cardiovascular, notando-se canal arterial persistente (<xref ref-type="fig" rid="f12">Figura 4</xref>), p&#233;rvio com di&#226;metro de 2 mm; pequena comunica&#231;&#227;o interventricular muscular (<xref ref-type="fig" rid="f13">Figura 5</xref>); pontos cir&#250;rgicos em septo atrial, possivelmente correspondendo a fechamento de comunica&#231;&#227;o; embolia (ou trombose) de veia subcl&#225;via esquerda; e pr&#243;tese valvar mec&#226;nica em posi&#231;&#227;o mitral, oclu&#237;da pela presen&#231;a em suas duas faces de massa com aspecto de trombo (<xref ref-type="fig" rid="f14">Figura 6</xref>). O estudo microsc&#243;pico confirmou a natureza desta massa, com aus&#234;ncia de microrganismos (<xref ref-type="fig" rid="f15">Figura 7</xref>). Havia pequenos infartos, no rim direito, possivelmente por embolia a partir do trombo da pr&#243;tese, e na regi&#227;o subendoc&#225;rdica do ventr&#237;culo esquerdo. Os pulm&#245;es mostravam-se em sua quase totalidade bastante alterados, com padr&#227;o histopatol&#243;gico de pneumonia em organiza&#231;&#227;o (<xref ref-type="fig" rid="f16">Figura 8</xref>). Al&#233;m disso, denotando congest&#227;o, havia macr&#243;fagos contendo hemossiderina, mas n&#227;o em grande quantidade; e dilata&#231;&#227;o de vasos linf&#225;ticos. (Paulo Sampaio Gutierrez)</p>
        <p>
          <fig id="f12">
            <label>Figura 4</label>
            <caption>
              <title>Grandes vasos da base mostrando canal arterial persistente (entre as setas).</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf04-pt.jpg"/></fig>
        </p>
        <p>
          <fig id="f13">
            <label>Figura 5</label>
            <caption>
              <title>Corte transversal do cora&#231;&#227;o na regi&#227;o dos ventr&#237;culos, mostrando comunica&#231;&#227;o interventricular (seta). VD: ventr&#237;culo direito; VE: ventr&#237;culo esquerdo.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf05-pt.jpg"/></fig>
        </p>
        <p>
          <fig id="f14">
            <label>Figura 6</label>
            <caption>
              <title>Foto &#224; esquerda: Pr&#243;tese valvar mec&#226;nica em posi&#231;&#227;o mitral vista pelo &#225;trio esquerdo, com oclus&#227;o quase total por trombo. Pequenas partes da pr&#243;tese podem ser vistas como &#225;reas negras sob o trombo. Foto &#224; direita: Ap&#243;s a retirada do trombo, a pr&#243;tese tem abertura adequada.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf06-pt.jpg"/></fig>
        </p>
        <p>
          <fig id="f15">
            <label>Figura 7</label>
            <caption>
              <title>Corte histol&#243;gico da massa aderida &#224; pr&#243;tese valvar, constitu&#237;da por trombo com fibrina e n&#250;mero moderado de c&#233;lulas inflamat&#243;rias, sem microrganismos. Colora&#231;&#227;o pela hematoxilina &amp; eosina; aumento da objetiva: 10x.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf07-pt.jpg"/></fig>
        </p>
        <p>
          <fig id="f16">
            <label>Figura 8</label>
            <caption>
              <title>Corte histol&#243;gico de pulm&#227;o, mostrando alv&#233;olos preenchidos por c&#233;lulas mononucleares e presen&#231;a de col&#225;geno. Colora&#231;&#227;o pela hematoxilina &amp; eosina; aumento da objetiva: 10x.</title>
            </caption>
            <graphic xlink:href="1678-4170-abc-111-04-0629-80205-gf08-pt.jpg"/></fig>
        </p>
        <p><bold>Diagn&#243;sticos anatomopatol&#243;gicos:</bold> Cardiopatia cong&#234;nita com comunica&#231;&#227;o interatrial, comunica&#231;&#227;o interventricular, persist&#234;ncia do canal arterial e trombose pr&#243;tese mec&#226;nica mitral.</p>
        <p><bold>&#8220;Causa mortis&#8221;:</bold> Obstru&#231;&#227;o valvar mitral/ pneumonia em organiza&#231;&#227;o <bold>(</bold>Dr. Paulo Sampaio Gutierrez)</p>
      </sec>
      <sec sec-type="conclusions">
        <title>Coment&#225;rio</title>
        <p>Ainda que o problema dominante que desencadeou a morte da paciente tenha sido a trombose da pr&#243;tese valvar mitral - real&#231;ando a dificuldade de manejo do sistema de coagula&#231;&#227;o durante a gesta&#231;&#227;o - vale comentar tamb&#233;m que os pulm&#245;es estavam muito alterados, com padr&#227;o de pneumonia em organiza&#231;&#227;o. &#201; importante frisar que o diagn&#243;stico de &#8220;pneumonia em organiza&#231;&#227;o&#8221; diz respeito a quadro que pode ser subsequente n&#227;o s&#243; a pneumonia bacteriana cl&#225;ssica, mas tamb&#233;m a v&#225;rias outras situa&#231;&#245;es, como infec&#231;&#245;es virais, exposi&#231;&#227;o a inalantes t&#243;xicos e outros.<xref ref-type="bibr" rid="B7">7</xref> No entanto, congest&#227;o acentuada, ainda que s&#250;bita, n&#227;o est&#225; listada entre as poss&#237;veis causas desse processo. Assim, no presente caso, a pneumonia em organiza&#231;&#227;o deve ser decorrente do quadro respirat&#243;rio, possivelmente infeccioso, seja ele viral ou bacteriano, com que a paciente deu entrada na institui&#231;&#227;o.</p>
      </sec>
    </body>
    <back>
      <fn-group>
        <fn fn-type="other">
          <p><bold>Editor da Se&#231;&#227;o:</bold> Alfredo Jos&#233; Mansur (<email>ajmansur@incor.usp.br</email>)</p>
        </fn>
        <fn fn-type="other">
          <p><bold>Editores Associados:</bold> Desid&#233;rio Favarato (dclfavarato@incor.usp.br) Vera Demarchi Aiello (<email>anpvera@incor.usp.br</email>)</p>
        </fn>
      </fn-group>
    </back>
  </sub-article>
</article>
