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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Introduction</span><p id="par0065" class="elsevierStylePara elsevierViewall">Ebstein&#39;s anomaly &#40;EA&#41; is a rare complex congenital heart defect &#40;CHD&#41; of the tricuspid valve &#40;TV&#41; first described by Wilhelm Ebstein in 1866&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">1</span></a> It occurs in about 1 per 200<span class="elsevierStyleHsp" style=""></span>000 live births and accounts for less than 1&#37; of all CHDs&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#8211;8</span></a> The anatomical basis of EA consists in displacement by more than 8 mm&#47;m<span class="elsevierStyleSup">2</span> body surface area of the septal and posterior leaflets of the TV in relation to the position of the mitral valve anterior leaflet&#46;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">4&#44;6</span></a> The genetic etiology is unknown&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">5</span></a> The usual clinical presentation is cyanosis&#44; heart failure&#44; arrhythmia and sudden death&#46;<a class="elsevierStyleCrossRefs" href="#bib0120"><span class="elsevierStyleSup">3&#44;8</span></a> The severity and onset of symptoms depend on the degree of displacement of the TV leaflets&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a> Transthoracic echocardiography is the gold standard for diagnosis of this entity&#46;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">4&#44;6</span></a> EA may coexist with other CHDs&#44; genetic syndromes&#44; or Wolff-Parkinson-White &#40;WPW&#41; syndrome&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#8211;4&#44;9</span></a> Surgical management of EA depends on the patient&#39;s age&#44; the clinical presentation and association with other CHDs&#44; and therefore the surgical approach should be individualized&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#44;10</span></a> Ebstein&#39;s anomaly is classified among the category of CHDs that requires early and specific health care&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">7&#44;8</span></a> In this context&#44; the present study aimed to describe the experience of a tertiary center in a developing country in managing this complex disease in patients less than 18 years old&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Methods</span><p id="par0070" class="elsevierStylePara elsevierViewall">Based on the records of the Cardiothoracic Center of Cl&#237;nica Girassol&#44; previously described&#44;<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">11</span></a> a retrospective study was performed that included all patients with CHDs over a period of 37 months &#40;March 29&#44; 2011 to April 29&#44; 2014&#41;&#46; Data on clinical presentation&#44; chest X-ray&#44; electrocardiography&#44; echocardiographic studies and surgical reports were analyzed&#46; Transthoracic echocardiography and Doppler studies was performed by pediatric cardiologists using a GE Vivid 7 system &#40;GE Healthcare&#44; Milwaukee&#44; WI&#44; USA&#41; with multiple frequency probes &#40;3S-RS&#44; 4C-RS and RS-6S&#41; in accordance with the recommendations of the American College of Cardiology&#47;American Heart Association&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">12</span></a> Only the first echocardiogram was validated&#46; A diagnosis of EA was made when a displacement of the septal and posterior leaflets of the TV was observed of more than 8 mm&#47;m<span class="elsevierStyleSup">2</span> body surface area in relation to the position of the mitral valve anterior leaflet&#46;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">4&#44;6</span></a> The surgical indications were the conventional ones&#58; NYHA functional class III or IV&#59; NYHA class I or II with cardiothoracic index &#40;CTI&#41; of 0&#46;65 or greater&#59; progressive right ventricular &#40;RV&#41; dilation&#59; significant cyanosis and polycythemia&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#8211;11</span></a> In patients with favorable anatomy cone reconstruction &#40;CR&#41; of the TV was performed as described by Da Silva et al&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> Atrial septal defect or patent foramen ovale&#44; if present&#44; was closed at the time of repair&#46; Cavopulmonary shunt was reserved for patients less than six months old and for those with significant RV dysfunction&#46; The modified Blalock-Taussig &#40;MBT&#41; shunt was reserved for patients with pulmonary artery hypoplasia &#40;PAH&#41;&#46; Follow-up was in the first half of June 2014&#44; when all patients underwent clinical&#44; radiological and echocardiography assessment&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">A descriptive statistical analysis was performed &#40;absolute and relative frequencies&#44; mean and standard deviation&#41;&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Results</span><p id="par0080" class="elsevierStylePara elsevierViewall">During the study period&#44; 1362 patients under the age of 19 years with a diagnosis of CHD were analyzed&#44; eight &#40;0&#46;6&#37;&#41; of them with Ebstein&#39;s anomaly&#46; Demographic data&#44; clinical presentations&#44; and NYHA functional class of the study population are shown in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46; Mean age was 69&#177;59 months&#44; ranging from 4 to 168 months&#46; Female gender was predominant&#44; with a female&#58;male ratio of 3&#58;1&#46; Regarding clinical presentation&#44; five patients &#40;62&#46;5&#37;&#41; had signs and symptoms of heart failure&#44; three of them in NYHA functional class IV and two in class III&#44; and four also had cyanosis&#46; Two patients &#40;25&#37;&#41; were referred for a heart murmur and one for cyanosis&#59; these were in NYHA functional class I&#46; Data on X-ray&#44; echocardiography&#44; indications for surgery&#44; type of surgical techniques and complications are presented in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0085" class="elsevierStylePara elsevierViewall">Mean CTI was 0&#46;72&#44; ranging from 0&#46;56 to 0&#46;95&#46; Seven patients &#40;87&#46;5&#37;&#41; had severe tricuspid regurgitation &#40;TR&#41; and five &#40;62&#46;5&#37;&#41; had other CHDs associated with EA&#46; Atrial septal defect &#40;ASD&#41; was the most frequent CHD &#40;n&#61;3&#59; 37&#46;5&#37;&#41;&#46; In two cases &#40;25&#37;&#41; EA was associated with PAH&#46; In one of these cases an extremely rare association was found &#40;ostium primum ASD&#44; pulmonary atresia and patent ductus arteriosus &#91;PDA&#93;&#41;&#46; No patients with a genetic syndrome or WPW syndrome were found&#46; All patients underwent surgical intervention&#46; The indications for surgery were&#58; heart failure&#44; one patient&#59; heart failure and cyanosis&#44; four patients&#59; cyanosis&#44; one patient&#59; and cardiothoracic index &#62;0&#46;65&#44; in two asymptomatic patients&#46; In five patients CR of the tricuspid valve was performed with 100&#37; immediate success&#46; In these patients&#44; the mean bypass time was 82&#46;8&#177;42&#46;2 minutes &#40;46&#8211;148 minutes&#41; with mean aortic cross-clamp time of 56&#46;4&#177;32&#46;3 minutes &#40;27&#8211;106 minutes&#41;&#46; De Vega annuloplasty was performed concomitantly in two &#40;25&#37;&#41; patients&#46; At the time of CR&#44; ASD closure was performed in two patients&#46; Two patients &#40;25&#37;&#41; had post-operative complications&#58; one wound infection and the other complete atrioventricular block&#44; and a DDD permanent pacemaker was implanted&#46; Mean hospital stay was 10&#46;6&#177;2&#46;7 days&#46; At discharge&#44; in this group two patients had no TR and three had mild TR&#46; The survival rate was 100&#37;&#46; The remaining three patients&#44; in two with PAH an MBT shunt was performed&#44; and in one patient with unfavorable anatomy and severe EA a Glenn operation was performed along with CR of the TV&#46; The patient with the rare association of ostium primum ASD&#44; pulmonary valve atresia and PDA&#44; who was treated by an MBT shunt&#44; died five hours after surgery&#46; The mean follow-up was 1&#46;22&#177;0&#46;6 years&#46; In this period&#44; another patient who underwent an MBT shunt died &#40;12&#46;5&#37;&#41;&#46; At the end of the study four patients &#40;80&#37;&#41; were in NYHA functional class I &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; CTI decreased in all patients&#44; by a mean of 0&#46;72&#8211;0&#46;64 &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; The third patient showed the greatest improvement &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; Three patients &#40;60&#37;&#41; had mild and two &#40;40&#37;&#41; had moderate TR on the last echocardiogram&#46; The patient with a permanent pacemaker was readmitted five months after surgery for pericardial effusion and minimal right pleural effusion and was discharged in NYHA class II seven days after readmission&#46; The patient who underwent a Glenn procedure along with CR of the TV at the end of the follow-up period was in NYHA class I&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Discussion</span><p id="par0090" class="elsevierStylePara elsevierViewall">This study revealed that 0&#46;6&#37; of patients with CHDs referred to our center had EA&#44; a similar finding to those reported by other authors&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#8211;8</span></a> However&#44; it contrasts with the high frequency of EA &#40;2&#37;&#41; described in a study carried in the Sudanese population&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">13</span></a> In the present study&#44; females had an almost three-fold higher probability of having EA&#46; This result contrasts with those of Anderson<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">9</span></a> and Da Silva&#44;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> who report a slight predominance of males&#44; and with other studies that described no predominance of either gender&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#8211;6&#44;8&#44;13&#44;14</span></a> In developed countries&#44; the diagnosis of CHD is made in the fetal or neonatal period&#44; enabling an early approach and improving the prognosis of these patients&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#44;3&#44;8&#44;14</span></a> In Africa&#44;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">13</span></a> particularly in Angola&#44; CHDs still tend to be diagnosed later&#44;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">16</span></a> as shown by the mean age &#40;29&#177;36 years&#41; found in the present series&#46; Access to health care services are still a serious problem in our continent&#44; from the prenatal period to old age&#44; which is why we have a small sample without neonates with severe forms of EA and older patients with good functional status&#46; In the present study more than half of the patients were in NYHA functional class III or IV&#46; This result is similar to that reported in a study conducted in Sudan&#44; in which the authors found that half of the patients were symptomatic&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">13</span></a> The mean CTI found in this series was higher than that reported by Arizmendi et al&#46; and Ali and Nimeri&#44; 0&#46;65 and 0&#46;64&#44; respectively&#46;<a class="elsevierStyleCrossRefs" href="#bib0120"><span class="elsevierStyleSup">3&#44;13</span></a> It should be noted that CTI &#62;0&#46;65 is consistently associated with a worse prognosis&#44;<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">11</span></a> and the mean CTI in this study was greater than this cut-off value&#44; suggesting that our patients were at high risk&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Transthoracic echocardiography is the most important diagnostic test for EA&#44; to determine TV morphology and physiology and to assess the cardiac chambers and the presence of other CHD&#46;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">4&#44;6&#44;14</span></a> Severe tricuspid regurgitation in our patients was almost twice as frequent as that reported by Ali and Nimeri &#40;33&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">13</span></a> Dilation of the right cardiac chambers and therefore of the tricuspid annulus is the most likely cause of the severity of tricuspid regurgitation found in the population studied&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">6&#44;9&#44;17</span></a> EA is frequently associated with other CHDs&#44; in more than 50&#37; of cases&#44; most often ASD&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">2&#44;3&#44;6&#44;10&#44;14&#44;15</span></a> Although in smaller proportions&#44; the results of this study reflect this frequency&#46; On the other hand&#44; Arizmendi et al&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a> reported pulmonary valve stenosis as the most frequent CHD &#40;39&#37;&#41;&#46; Although associations between EA and certain genetic syndromes have been described&#44; mainly trisomy 21 and WPW syndrome&#44;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#44;14</span></a> these syndromes were not found in the present series&#46; Finally&#44; it is noteworthy that one patient in our series had an extremely rare association &#40;ostium primum ASD&#44; pulmonary atresia and PDA&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">18</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">The conventional surgical indications for the correction of EA are NYHA functional class III or IV&#59; NYHA functional class I or II with CTI of 0&#46;65 or greater&#59; progressive RV dilatation&#59; cyanosis &#40;oxygen saturation &#60;90&#37;&#41; and polycythemia&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#8211;11</span></a> In the present series the indications for surgery were heart failure and&#47;or cyanosis in six patients and CTI &#62;0&#46;65 in two asymptomatic patients&#46; The complexity of the anatomical and functional changes in EA and the possibility of association with other CHDs mean that the surgical approach should be individualized&#46; Cavopulmonary shunt is reserved for patients with severe RV dysfunction&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#44;15</span></a> The MBT shunt is generally reserved for patients with PAH&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">19</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Various techniques for tricuspid valve reconstruction have been developed in the last 35 years&#44; with different results&#46;<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">20&#44;21</span></a> The most recent technique is CR&#44; developed by da Silva et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">16</span></a> which we use in our center&#46; Although the number of patients is small&#44; the present series showed an immediate success rate of 100&#37; for CR&#44; with clinical and echocardiographic improvement in short-term follow-up&#44; in agreement with the good results for this technique reported by other authors&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9&#44;15</span></a> Da Silva et al&#46; reported that CR was associated with low in-hospital mortality and was an effective and long-lasting repair of tricuspid regurgitation&#44; restoring the functional area of the right ventricle and bringing clinical improvement in most patients in the long term&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> Anderson et al&#46;&#44; describing the Mayo Clinic experience with CR&#44; reported difficulties in reconstructing the sepal portion of the TV&#46; Several techniques have been described to avoid this complication&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> Finally&#44; in relation to ASD closure&#44; partial closure of the foramen ovale enables preservation of right-to-left flow&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">The low in-hospital mortality and long-term clinical improvement reported by da Silva et al&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> are supported by the Mayo Clinic experience&#44; in which CR in 84 patients with EA aged under 21 years showed a success rate of 98&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">9</span></a> In our present series also&#44; there was an immediate success rate of 100&#37;&#44; with short-term clinical and echocardiographic improvement&#46; However&#44; the technique could not be applied in two patients for the reasons described above&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Conclusions</span><p id="par0115" class="elsevierStylePara elsevierViewall">The prevalence of EA found in this series was similar to other centers&#46; Our patients had access to medical care at a critical stage of the disease&#46; CR was performed in the majority of patients with excellent immediate and short-term results&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Ethical disclosures</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Protection of human and animal subjects</span><p id="par0120" class="elsevierStylePara elsevierViewall">The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association &#40;Declaration of Helsinki&#41;&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Confidentiality of data</span><p id="par0125" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Right to privacy and informed consent</span><p id="par0130" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Conflicts of interest</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
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          "identificador" => "xres817819"
          "titulo" => "Abstract"
          "secciones" => array:4 [
            0 => array:2 [
              "identificador" => "abst0005"
              "titulo" => "Introduction and Objective"
            ]
            1 => array:2 [
              "identificador" => "abst0010"
              "titulo" => "Methods"
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            2 => array:2 [
              "identificador" => "abst0015"
              "titulo" => "Results"
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            3 => array:2 [
              "identificador" => "abst0020"
              "titulo" => "Conclusion"
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        1 => array:2 [
          "identificador" => "xpalclavsec814932"
          "titulo" => "Keywords"
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          "titulo" => "Resumo"
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              "titulo" => "Introdu&#231;&#227;o e Objectivos"
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              "titulo" => "M&#233;todos"
            ]
            2 => array:2 [
              "identificador" => "abst0035"
              "titulo" => "Resultados"
            ]
            3 => array:2 [
              "identificador" => "abst0040"
              "titulo" => "Conclus&#227;o"
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          "titulo" => "Palavras-chave"
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        4 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Introduction"
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        5 => array:2 [
          "identificador" => "sec0010"
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          "identificador" => "sec0015"
          "titulo" => "Results"
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          "identificador" => "sec0020"
          "titulo" => "Discussion"
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        8 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Conclusions"
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        9 => array:3 [
          "identificador" => "sec0030"
          "titulo" => "Ethical disclosures"
          "secciones" => array:3 [
            0 => array:2 [
              "identificador" => "sec0035"
              "titulo" => "Protection of human and animal subjects"
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            1 => array:2 [
              "identificador" => "sec0040"
              "titulo" => "Confidentiality of data"
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            2 => array:2 [
              "identificador" => "sec0045"
              "titulo" => "Right to privacy and informed consent"
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          "titulo" => "Conflicts of interest"
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          "titulo" => "References"
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    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2015-02-06"
    "fechaAceptado" => "2015-03-21"
    "PalabrasClave" => array:2 [
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec814932"
          "palabras" => array:5 [
            0 => "Congenital heart disease"
            1 => "Congenital heart surgery"
            2 => "Valve lesion"
            3 => "Tricuspid valve"
            4 => "Cone reconstruction"
          ]
        ]
      ]
      "pt" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palavras-chave"
          "identificador" => "xpalclavsec814933"
          "palabras" => array:5 [
            0 => "Cardiopatia cong&#233;nita"
            1 => "Cirurgia card&#237;aca cong&#233;nita"
            2 => "Les&#227;o valvular"
            3 => "V&#225;lvula tric&#250;spide"
            4 => "Reconstru&#231;&#227;o em cone"
          ]
        ]
      ]
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    "resumen" => array:2 [
      "en" => array:3 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction and Objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ebstein&#39;s anomaly is a rare complex congenital heart defect of the tricuspid valve&#46; We aimed to describe the frequency&#44; clinical profile&#44; and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A retrospective cross-sectional study was conducted over a period of 37 months&#46; We analyzed all patients diagnosed with congenital heart defects&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Of the 1362 patients studied&#44; eight &#40;0&#46;6&#37;&#41; had Ebstein&#39;s anomaly&#59; six patients &#40;75&#37;&#41; were female&#46; Mean age was 69&#177;59 months&#46; Five patients were in NYHA functional class III or IV&#46; Mean cardiothoracic index was 0&#46;72&#46; Seven patients &#40;87&#46;5&#37;&#41; had severe tricuspid regurgitation and five &#40;62&#46;5&#37;&#41; had another associated congenital heart defect&#46; All patients were operated&#58; two had complications and one &#40;12&#46;5&#37;&#41; died in the early post-operative period&#46; The mean follow-up time was 1&#46;22&#177;0&#46;6 years&#44; and mortality during follow-up was 12&#46;5&#37; &#40;n&#61;1&#41;&#46; At the end of the study&#44; of the five patients in whom cone reconstruction was performed&#44; four &#40;80&#37;&#41; were in functional class I&#46; Mean cardiothoracic index decreased to 0&#46;64&#46; Three patients had mild and two had moderate tricuspid regurgitation&#46; The patient who underwent cone reconstruction and a Glenn procedure was in functional class I&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">The frequency of Ebstein&#39;s anomaly was similar to that in other centers&#46; Cone reconstruction was viable in the majority of patients&#44; with good early and short-term results&#46;</p></span>"
        "secciones" => array:4 [
          0 => array:2 [
            "identificador" => "abst0005"
            "titulo" => "Introduction and Objective"
          ]
          1 => array:2 [
            "identificador" => "abst0010"
            "titulo" => "Methods"
          ]
          2 => array:2 [
            "identificador" => "abst0015"
            "titulo" => "Results"
          ]
          3 => array:2 [
            "identificador" => "abst0020"
            "titulo" => "Conclusion"
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      "pt" => array:3 [
        "titulo" => "Resumo"
        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introdu&#231;&#227;o e Objectivos</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A anomalia de Ebstein &#233; uma cardiopatia cong&#233;nita complexa e rara da v&#225;lvula tric&#250;spide&#46; Descrever a frequ&#234;ncia&#44; o perfil cl&#237;nico e os resultados p&#243;s operat&#243;rios imediatos desta anomalia em crian&#231;as e adolescentes&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todos</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Estudo transversal retrospetivo no &#250;nico centro com tratamento integrado em cardiopatias cong&#233;nitas em rec&#233;m nascidos&#44; crian&#231;as e adolescentes &#40;&#60;18 anos de idade&#41; em Angola&#44; num per&#237;odo de 37 meses&#46; Foram analisados todos os doentes com diagn&#243;stico de cardiopatia cong&#233;nita&#46; O diagn&#243;stico foi feito com base no exame cl&#237;nico e dados da ecocardiografia transtor&#225;cica e Doppler&#46; Os ecocardiogramas foram realizados pelos Cardiologistas Pedi&#225;tricos numa m&#225;quina Vivid 7 G&#46;E&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Foram analisados 1362 ecocardiogramas sequenciais&#46; Oito doentes &#40;0&#44;6&#37;&#41; tinham anomalia de Ebstein&#44; destes 6 &#40;75&#37;&#41; eram do g&#233;nero feminino&#46; As idades variaram entre 4 a 168 meses&#44; a m&#233;dia foi de 69 &#177; 59 meses&#46; Cinco estavam em classe funcional III ou IV da NYHA&#46; A m&#233;dia do &#237;ndice cardiotor&#225;cico foi de 0&#44;72&#46; Sete doentes &#40;87&#44;5&#37;&#41; tinham regurgita&#231;&#227;o tric&#250;spide grave e 5 &#40;62&#44;5&#37;&#41; tinham outra cardiopatia cong&#233;nita associada&#46; Todos os doentes foram submetidos a interven&#231;&#227;o cir&#250;rgica&#46; Dois tiveram complica&#231;&#245;es no p&#243;s-operat&#243;rio imediato&#46; A mortalidade p&#243;s operat&#243;ria foi de 12&#44;5&#37; &#40;n&#61;1&#41;&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclus&#227;o</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">A frequ&#234;ncia da Anomalia de Ebstein encontrada foi semelhante a de outros Centros&#46; A t&#233;cnica de Cone foi vi&#225;vel na maior parte dos pacientes&#46; A mortalidade p&#243;s operat&#243;ria imediata foi baixa&#46;</p></span>"
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            "titulo" => "Introdu&#231;&#227;o e Objectivos"
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      0 => array:3 [
        "identificador" => "nom0005"
        "titulo" => "<span class="elsevierStyleSectionTitle" id="sect0065">List of abbreviations</span>"
        "listaDefinicion" => array:1 [
          0 => array:1 [
            "definicion" => array:12 [
              0 => array:2 [
                "termino" => "ASD"
                "descripcion" => "<p id="par0005" class="elsevierStylePara elsevierViewall">atrial septal defect</p>"
              ]
              1 => array:2 [
                "termino" => "CHD"
                "descripcion" => "<p id="par0010" class="elsevierStylePara elsevierViewall">congenital heart defect</p>"
              ]
              2 => array:2 [
                "termino" => "CR"
                "descripcion" => "<p id="par0015" class="elsevierStylePara elsevierViewall">cone reconstruction</p>"
              ]
              3 => array:2 [
                "termino" => "CTI"
                "descripcion" => "<p id="par0020" class="elsevierStylePara elsevierViewall">cardiothoracic index</p>"
              ]
              4 => array:2 [
                "termino" => "EA"
                "descripcion" => "<p id="par0025" class="elsevierStylePara elsevierViewall">Ebstein&#39;s anomaly</p>"
              ]
              5 => array:2 [
                "termino" => "MBT"
                "descripcion" => "<p id="par0030" class="elsevierStylePara elsevierViewall">modified Blalock-Taussig</p>"
              ]
              6 => array:2 [
                "termino" => "NYHA"
                "descripcion" => "<p id="par0035" class="elsevierStylePara elsevierViewall">New York Heart Association</p>"
              ]
              7 => array:2 [
                "termino" => "PAH"
                "descripcion" => "<p id="par0040" class="elsevierStylePara elsevierViewall">pulmonary artery hypoplasia</p>"
              ]
              8 => array:2 [
                "termino" => "PDA"
                "descripcion" => "<p id="par0045" class="elsevierStylePara elsevierViewall">patent ductus arteriosus</p>"
              ]
              9 => array:2 [
                "termino" => "RV"
                "descripcion" => "<p id="par0050" class="elsevierStylePara elsevierViewall">right ventricular</p>"
              ]
              10 => array:2 [
                "termino" => "TV"
                "descripcion" => "<p id="par0055" class="elsevierStylePara elsevierViewall">tricuspid valve</p>"
              ]
              11 => array:2 [
                "termino" => "WPW"
                "descripcion" => "<p id="par0060" class="elsevierStylePara elsevierViewall">Wolff-Parkinson-White</p>"
              ]
            ]
          ]
        ]
      ]
    ]
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        "tipo" => "MULTIMEDIAFIGURA"
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        "descripcion" => array:1 [
          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Evolution of functional class in the five patients undergoing cone reconstruction&#46; NYHA&#58; New York Heart Association&#46;</p>"
        ]
      ]
      1 => array:7 [
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        "etiqueta" => "Figure 2"
        "tipo" => "MULTIMEDIAFIGURA"
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        "descripcion" => array:1 [
          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Evolution of cardiothoracic index in the five patients undergoing cone reconstruction&#46;</p>"
        ]
      ]
      2 => array:7 [
        "identificador" => "fig0015"
        "etiqueta" => "Figure 3"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
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          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Chest X-rays showing an impressive reduction in cardiothoracic index after cone reconstruction&#46;</p>"
        ]
      ]
      3 => array:7 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:2 [
          "leyenda" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">CHF&#58; congestive heart failure&#59; F&#58; female&#59; M&#58; male&#59; NYHA&#58; New York Heart Association&#46;</p>"
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Patient no&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Year&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gender&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age &#40;months&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Symptoms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">NYHA class&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">168&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">I&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">M&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">120&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">IV&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">F&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">108&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">III&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab1374656.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Distribution of demographic data&#44; year of diagnosis&#44; clinical presentation and NYHA functional class&#46;</p>"
        ]
      ]
      4 => array:7 [
        "identificador" => "tbl0010"
        "etiqueta" => "Table 2"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:2 [
          "leyenda" => "<p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">ASD&#58; atrial septal defect&#59; AVB&#58; atrioventricular block&#59; CHD&#58; congenital heart defect&#59; CHF&#58; congestive heart failure&#59; CR&#58; cone reconstruction&#59; CTI&#58; cardiothoracic index&#59; MBT&#58; modified Blalock-Taussig&#59; PA&#58; pulmonary atresia&#59; OPASD&#58; ostium primum atrial septal defect&#59; OSASD&#58; ostium secundum atrial septal defect&#59; PAH&#58; pulmonary artery hypoplasia&#59; PDA&#58; persistent ductus arteriosus&#59; RA&#58; right atrium&#59; RV&#58; right ventricle&#59; TR&#58; tricuspid regurgitation&#46;</p>"
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Patient no&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">CTI&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">TR&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Other CHD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Surgical indication&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Surgery&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Early post-operative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;66&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Moderate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">OSASD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CTI &#62;0&#46;65&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR and ASD closure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">No complications&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;95&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">PA&#44; PDA&#44; OPASD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF&#44; cyanosis&#44; NYHA IV and CTI &#62;0&#46;95&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MBT shunt&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Died five hours after surgery&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;63&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">PAH&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">MBT shunt&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">No complications&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CTI &#62;0&#46;72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">No complications&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;76&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and CTI &#62;0&#46;76&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Wound infection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF&#44; cyanosis and CTI &#62;0&#46;72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">No complications&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;77&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">OPASD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF&#44; cyanosis and CTI &#62;0&#46;77&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR and ASD closure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Complete AVB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="char" valign="top">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="char" valign="top">0&#46;56&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Severe&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">PAH&#44; OPASD&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CHF and cyanosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CR&#44; ASD closure and Glenn operation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">No complications&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
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                0 => "xTab1374655.png"
              ]
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          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Cardiothoracic index&#44; echocardiographic findings&#44; surgical indications&#44; surgery performed and early post-operative evaluation&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:21 [
            0 => array:3 [
              "identificador" => "bib0110"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "On a very rare case of insufficiency of the tricuspid valve caused by a severe congenital malformation of the same"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "W&#46; Ebstein"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:3 [
                        "tituloSerie" => "Arch F Anat Physiol Wissensch Med Leipz"
                        "fecha" => "1866"
                        "paginaInicial" => "238"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0115"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Ebstein&#39;s malformation of the tricuspid valve&#58; genetic and environmental factors"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "A&#46; Correa-Villase&#241;or"
                            1 => "C&#46; Ferencz"
                            2 => "C&#46;A&#46; Neill"
                          ]
                        ]
                      ]
                    ]
                  ]
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Vol. 34. Issue 10.
Pages 607-612 (October 2015)
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Vol. 34. Issue 10.
Pages 607-612 (October 2015)
Original Article
Open Access
Ebstein's anomaly in children: A single-center study in Angola
Anomalia de Ebstein em crianças: estudo unicêntrico em Angola
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Valdano Manuela,
Corresponding author
valdanympub@gmail.com

Corresponding author.
, Humberto Moraisb, Manuel Pedro Magalhãesa,c, Maria Ana Sampaio Nunesa, Gilberto Leona, Manuel Ferreiraa,c, António Pedro Filipe Júniora
a Cardio-Thoracic Center, Clinica Girassol, Luanda, Angola
b Cardiology Department, Hospital Militar Principal/Instituto Superior, Luanda, Angola
c Hospital Cruz Vermelha, Lisbon, Portugal
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Table 1. Distribution of demographic data, year of diagnosis, clinical presentation and NYHA functional class.
Table 2. Cardiothoracic index, echocardiographic findings, surgical indications, surgery performed and early post-operative evaluation.
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Abstract
Introduction and Objective

Ebstein's anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola.

Methods

A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects.

Results

Of the 1362 patients studied, eight (0.6%) had Ebstein's anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I.

Conclusion

The frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

Keywords:
Congenital heart disease
Congenital heart surgery
Valve lesion
Tricuspid valve
Cone reconstruction
Resumo
Introdução e Objectivos

A anomalia de Ebstein é uma cardiopatia congénita complexa e rara da válvula tricúspide. Descrever a frequência, o perfil clínico e os resultados pós operatórios imediatos desta anomalia em crianças e adolescentes.

Métodos

Estudo transversal retrospetivo no único centro com tratamento integrado em cardiopatias congénitas em recém nascidos, crianças e adolescentes (<18 anos de idade) em Angola, num período de 37 meses. Foram analisados todos os doentes com diagnóstico de cardiopatia congénita. O diagnóstico foi feito com base no exame clínico e dados da ecocardiografia transtorácica e Doppler. Os ecocardiogramas foram realizados pelos Cardiologistas Pediátricos numa máquina Vivid 7 G.E.

Resultados

Foram analisados 1362 ecocardiogramas sequenciais. Oito doentes (0,6%) tinham anomalia de Ebstein, destes 6 (75%) eram do género feminino. As idades variaram entre 4 a 168 meses, a média foi de 69 ± 59 meses. Cinco estavam em classe funcional III ou IV da NYHA. A média do índice cardiotorácico foi de 0,72. Sete doentes (87,5%) tinham regurgitação tricúspide grave e 5 (62,5%) tinham outra cardiopatia congénita associada. Todos os doentes foram submetidos a intervenção cirúrgica. Dois tiveram complicações no pós-operatório imediato. A mortalidade pós operatória foi de 12,5% (n=1).

Conclusão

A frequência da Anomalia de Ebstein encontrada foi semelhante a de outros Centros. A técnica de Cone foi viável na maior parte dos pacientes. A mortalidade pós operatória imediata foi baixa.

Palavras-chave:
Cardiopatia congénita
Cirurgia cardíaca congénita
Lesão valvular
Válvula tricúspide
Reconstrução em cone
List of abbreviations
ASD

atrial septal defect

CHD

congenital heart defect

CR

cone reconstruction

CTI

cardiothoracic index

EA

Ebstein's anomaly

MBT

modified Blalock-Taussig

NYHA

New York Heart Association

PAH

pulmonary artery hypoplasia

PDA

patent ductus arteriosus

RV

right ventricular

TV

tricuspid valve

WPW

Wolff-Parkinson-White

Full Text
Introduction

Ebstein's anomaly (EA) is a rare complex congenital heart defect (CHD) of the tricuspid valve (TV) first described by Wilhelm Ebstein in 1866.1 It occurs in about 1 per 200000 live births and accounts for less than 1% of all CHDs.2–8 The anatomical basis of EA consists in displacement by more than 8 mm/m2 body surface area of the septal and posterior leaflets of the TV in relation to the position of the mitral valve anterior leaflet.4,6 The genetic etiology is unknown.5 The usual clinical presentation is cyanosis, heart failure, arrhythmia and sudden death.3,8 The severity and onset of symptoms depend on the degree of displacement of the TV leaflets.3 Transthoracic echocardiography is the gold standard for diagnosis of this entity.4,6 EA may coexist with other CHDs, genetic syndromes, or Wolff-Parkinson-White (WPW) syndrome.2–4,9 Surgical management of EA depends on the patient's age, the clinical presentation and association with other CHDs, and therefore the surgical approach should be individualized.9,10 Ebstein's anomaly is classified among the category of CHDs that requires early and specific health care.7,8 In this context, the present study aimed to describe the experience of a tertiary center in a developing country in managing this complex disease in patients less than 18 years old.

Methods

Based on the records of the Cardiothoracic Center of Clínica Girassol, previously described,11 a retrospective study was performed that included all patients with CHDs over a period of 37 months (March 29, 2011 to April 29, 2014). Data on clinical presentation, chest X-ray, electrocardiography, echocardiographic studies and surgical reports were analyzed. Transthoracic echocardiography and Doppler studies was performed by pediatric cardiologists using a GE Vivid 7 system (GE Healthcare, Milwaukee, WI, USA) with multiple frequency probes (3S-RS, 4C-RS and RS-6S) in accordance with the recommendations of the American College of Cardiology/American Heart Association.12 Only the first echocardiogram was validated. A diagnosis of EA was made when a displacement of the septal and posterior leaflets of the TV was observed of more than 8 mm/m2 body surface area in relation to the position of the mitral valve anterior leaflet.4,6 The surgical indications were the conventional ones: NYHA functional class III or IV; NYHA class I or II with cardiothoracic index (CTI) of 0.65 or greater; progressive right ventricular (RV) dilation; significant cyanosis and polycythemia.9–11 In patients with favorable anatomy cone reconstruction (CR) of the TV was performed as described by Da Silva et al.15 Atrial septal defect or patent foramen ovale, if present, was closed at the time of repair. Cavopulmonary shunt was reserved for patients less than six months old and for those with significant RV dysfunction. The modified Blalock-Taussig (MBT) shunt was reserved for patients with pulmonary artery hypoplasia (PAH). Follow-up was in the first half of June 2014, when all patients underwent clinical, radiological and echocardiography assessment.

A descriptive statistical analysis was performed (absolute and relative frequencies, mean and standard deviation).

Results

During the study period, 1362 patients under the age of 19 years with a diagnosis of CHD were analyzed, eight (0.6%) of them with Ebstein's anomaly. Demographic data, clinical presentations, and NYHA functional class of the study population are shown in Table 1. Mean age was 69±59 months, ranging from 4 to 168 months. Female gender was predominant, with a female:male ratio of 3:1. Regarding clinical presentation, five patients (62.5%) had signs and symptoms of heart failure, three of them in NYHA functional class IV and two in class III, and four also had cyanosis. Two patients (25%) were referred for a heart murmur and one for cyanosis; these were in NYHA functional class I. Data on X-ray, echocardiography, indications for surgery, type of surgical techniques and complications are presented in Table 2.

Table 1.

Distribution of demographic data, year of diagnosis, clinical presentation and NYHA functional class.

Patient no.  Year  Gender  Age (months)  Symptoms  NYHA class 
2012  36  Asymptomatic 
2012  CHF and cyanosis  IV 
2013  Cyanosis 
2013  168  Asymptomatic 
2013  72  CHF  IV 
2013  36  CHF and cyanosis  III 
2014  120  CHF and cyanosis  IV 
2014  108  CHF and cyanosis  III 

CHF: congestive heart failure; F: female; M: male; NYHA: New York Heart Association.

Table 2.

Cardiothoracic index, echocardiographic findings, surgical indications, surgery performed and early post-operative evaluation.

Patient no.  CTI  TR  Other CHD  Surgical indication  Surgery  Early post-operative 
0.66  Moderate  OSASD  CTI >0.65  CR and ASD closure  No complications 
0.95  Severe  PA, PDA, OPASD  CHF, cyanosis, NYHA IV and CTI >0.95  MBT shunt  Died five hours after surgery 
0.63  Severe  PAH  Cyanosis  MBT shunt  No complications 
0.72  Severe  None  CTI >0.72  CR  No complications 
0.76  Severe  None  CHF and CTI >0.76  CR  Wound infection 
0.72  Severe  None  CHF, cyanosis and CTI >0.72  CR  No complications 
0.77  Severe  OPASD  CHF, cyanosis and CTI >0.77  CR and ASD closure  Complete AVB 
0.56  Severe  PAH, OPASD  CHF and cyanosis  CR, ASD closure and Glenn operation  No complications 

ASD: atrial septal defect; AVB: atrioventricular block; CHD: congenital heart defect; CHF: congestive heart failure; CR: cone reconstruction; CTI: cardiothoracic index; MBT: modified Blalock-Taussig; PA: pulmonary atresia; OPASD: ostium primum atrial septal defect; OSASD: ostium secundum atrial septal defect; PAH: pulmonary artery hypoplasia; PDA: persistent ductus arteriosus; RA: right atrium; RV: right ventricle; TR: tricuspid regurgitation.

Mean CTI was 0.72, ranging from 0.56 to 0.95. Seven patients (87.5%) had severe tricuspid regurgitation (TR) and five (62.5%) had other CHDs associated with EA. Atrial septal defect (ASD) was the most frequent CHD (n=3; 37.5%). In two cases (25%) EA was associated with PAH. In one of these cases an extremely rare association was found (ostium primum ASD, pulmonary atresia and patent ductus arteriosus [PDA]). No patients with a genetic syndrome or WPW syndrome were found. All patients underwent surgical intervention. The indications for surgery were: heart failure, one patient; heart failure and cyanosis, four patients; cyanosis, one patient; and cardiothoracic index >0.65, in two asymptomatic patients. In five patients CR of the tricuspid valve was performed with 100% immediate success. In these patients, the mean bypass time was 82.8±42.2 minutes (46–148 minutes) with mean aortic cross-clamp time of 56.4±32.3 minutes (27–106 minutes). De Vega annuloplasty was performed concomitantly in two (25%) patients. At the time of CR, ASD closure was performed in two patients. Two patients (25%) had post-operative complications: one wound infection and the other complete atrioventricular block, and a DDD permanent pacemaker was implanted. Mean hospital stay was 10.6±2.7 days. At discharge, in this group two patients had no TR and three had mild TR. The survival rate was 100%. The remaining three patients, in two with PAH an MBT shunt was performed, and in one patient with unfavorable anatomy and severe EA a Glenn operation was performed along with CR of the TV. The patient with the rare association of ostium primum ASD, pulmonary valve atresia and PDA, who was treated by an MBT shunt, died five hours after surgery. The mean follow-up was 1.22±0.6 years. In this period, another patient who underwent an MBT shunt died (12.5%). At the end of the study four patients (80%) were in NYHA functional class I (Figure 1). CTI decreased in all patients, by a mean of 0.72–0.64 (Figure 2). The third patient showed the greatest improvement (Figure 3). Three patients (60%) had mild and two (40%) had moderate TR on the last echocardiogram. The patient with a permanent pacemaker was readmitted five months after surgery for pericardial effusion and minimal right pleural effusion and was discharged in NYHA class II seven days after readmission. The patient who underwent a Glenn procedure along with CR of the TV at the end of the follow-up period was in NYHA class I.

Figure 1.

Evolution of functional class in the five patients undergoing cone reconstruction. NYHA: New York Heart Association.

(0.09MB).
Figure 2.

Evolution of cardiothoracic index in the five patients undergoing cone reconstruction.

(0.11MB).
Figure 3.

Chest X-rays showing an impressive reduction in cardiothoracic index after cone reconstruction.

(0.15MB).
Discussion

This study revealed that 0.6% of patients with CHDs referred to our center had EA, a similar finding to those reported by other authors.2–8 However, it contrasts with the high frequency of EA (2%) described in a study carried in the Sudanese population.13 In the present study, females had an almost three-fold higher probability of having EA. This result contrasts with those of Anderson9 and Da Silva,15 who report a slight predominance of males, and with other studies that described no predominance of either gender.2–6,8,13,14 In developed countries, the diagnosis of CHD is made in the fetal or neonatal period, enabling an early approach and improving the prognosis of these patients.2,3,8,14 In Africa,13 particularly in Angola, CHDs still tend to be diagnosed later,16 as shown by the mean age (29±36 years) found in the present series. Access to health care services are still a serious problem in our continent, from the prenatal period to old age, which is why we have a small sample without neonates with severe forms of EA and older patients with good functional status. In the present study more than half of the patients were in NYHA functional class III or IV. This result is similar to that reported in a study conducted in Sudan, in which the authors found that half of the patients were symptomatic.13 The mean CTI found in this series was higher than that reported by Arizmendi et al. and Ali and Nimeri, 0.65 and 0.64, respectively.3,13 It should be noted that CTI >0.65 is consistently associated with a worse prognosis,11 and the mean CTI in this study was greater than this cut-off value, suggesting that our patients were at high risk.

Transthoracic echocardiography is the most important diagnostic test for EA, to determine TV morphology and physiology and to assess the cardiac chambers and the presence of other CHD.4,6,14 Severe tricuspid regurgitation in our patients was almost twice as frequent as that reported by Ali and Nimeri (33%).13 Dilation of the right cardiac chambers and therefore of the tricuspid annulus is the most likely cause of the severity of tricuspid regurgitation found in the population studied.6,9,17 EA is frequently associated with other CHDs, in more than 50% of cases, most often ASD.2,3,6,10,14,15 Although in smaller proportions, the results of this study reflect this frequency. On the other hand, Arizmendi et al.3 reported pulmonary valve stenosis as the most frequent CHD (39%). Although associations between EA and certain genetic syndromes have been described, mainly trisomy 21 and WPW syndrome,9,14 these syndromes were not found in the present series. Finally, it is noteworthy that one patient in our series had an extremely rare association (ostium primum ASD, pulmonary atresia and PDA).18

The conventional surgical indications for the correction of EA are NYHA functional class III or IV; NYHA functional class I or II with CTI of 0.65 or greater; progressive RV dilatation; cyanosis (oxygen saturation <90%) and polycythemia.9–11 In the present series the indications for surgery were heart failure and/or cyanosis in six patients and CTI >0.65 in two asymptomatic patients. The complexity of the anatomical and functional changes in EA and the possibility of association with other CHDs mean that the surgical approach should be individualized. Cavopulmonary shunt is reserved for patients with severe RV dysfunction.9,15 The MBT shunt is generally reserved for patients with PAH.19

Various techniques for tricuspid valve reconstruction have been developed in the last 35 years, with different results.20,21 The most recent technique is CR, developed by da Silva et al.,16 which we use in our center. Although the number of patients is small, the present series showed an immediate success rate of 100% for CR, with clinical and echocardiographic improvement in short-term follow-up, in agreement with the good results for this technique reported by other authors.9,15 Da Silva et al. reported that CR was associated with low in-hospital mortality and was an effective and long-lasting repair of tricuspid regurgitation, restoring the functional area of the right ventricle and bringing clinical improvement in most patients in the long term.15 Anderson et al., describing the Mayo Clinic experience with CR, reported difficulties in reconstructing the sepal portion of the TV. Several techniques have been described to avoid this complication.15 Finally, in relation to ASD closure, partial closure of the foramen ovale enables preservation of right-to-left flow.

The low in-hospital mortality and long-term clinical improvement reported by da Silva et al.15 are supported by the Mayo Clinic experience, in which CR in 84 patients with EA aged under 21 years showed a success rate of 98%.9 In our present series also, there was an immediate success rate of 100%, with short-term clinical and echocardiographic improvement. However, the technique could not be applied in two patients for the reasons described above.

Conclusions

The prevalence of EA found in this series was similar to other centers. Our patients had access to medical care at a critical stage of the disease. CR was performed in the majority of patients with excellent immediate and short-term results.

Ethical disclosuresProtection of human and animal subjects

The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.

Conflicts of interest

The authors have no conflicts of interest to declare.

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