Case report
Unusual cause of left ventricular dysfunction in a child
Causa rara de disfunção ventricular esquerda em idade pediátrica
Read
3811
Timeswas read the article
1510
Total PDF
2301
Total HTML
Share statistics
array:25 [ "pii" => "S2174204919300509" "issn" => "21742049" "doi" => "10.1016/j.repce.2017.07.015" "estado" => "S300" "fechaPublicacion" => "2019-02-01" "aid" => "1213" "copyright" => "Sociedade Portuguesa de Cardiologia" "copyrightAnyo" => "2018" "documento" => "simple-article" "crossmark" => 1 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "crp" "cita" => "Rev Port Cardiol. 2019;38:159.e1-5" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 501 "formatos" => array:3 [ "EPUB" => 76 "HTML" => 292 "PDF" => 133 ] ] "Traduccion" => array:1 [ "pt" => array:20 [ "pii" => "S0870255117300100" "issn" => "08702551" "doi" => "10.1016/j.repc.2017.07.017" "estado" => "S300" "fechaPublicacion" => "2019-02-01" "aid" => "1213" "copyright" => "Sociedade Portuguesa de Cardiologia" "documento" => "simple-article" "crossmark" => 1 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "crp" "cita" => "Rev Port Cardiol. 2019;38:159.e1-159.e5" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 2398 "formatos" => array:3 [ "EPUB" => 145 "HTML" => 1762 "PDF" => 491 ] ] "pt" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Caso Clínico</span>" "titulo" => "Causa rara de disfunção ventricular esquerda em idade pediátrica" "tienePdf" => "pt" "tieneTextoCompleto" => "pt" "tieneResumen" => array:2 [ 0 => "pt" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "159.e1" "paginaFinal" => "159.e5" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Unusual cause of left ventricular dysfunction in a child" ] ] "contieneResumen" => array:2 [ "pt" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "pt" => true ] "contienePdf" => array:1 [ "pt" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figura 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 911 "Ancho" => 1400 "Tamanyo" => 112736 ] ] "descripcion" => array:1 [ "pt" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Tomografia Axial Computorizada cardíaca que mostra a coronária direita com origem no seio de Valsalva direito (seta vermelha) e a esquerda com origem na face póstero‐lateral esquerda do tronco da artéria pulmonar (seta azul).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Maria Emanuel Amaral, Pedro Epifânio, Natália Noronha, António Pires, Paula Martins, Vanda Azevedo, Eduardo Castela, Manuel J. Antunes" "autores" => array:8 [ 0 => array:2 [ "nombre" => "Maria Emanuel" "apellidos" => "Amaral" ] 1 => array:2 [ "nombre" => "Pedro" "apellidos" => "Epifânio" ] 2 => array:2 [ "nombre" => "Natália" "apellidos" => "Noronha" ] 3 => array:2 [ "nombre" => "António" "apellidos" => "Pires" ] 4 => array:2 [ "nombre" => "Paula" "apellidos" => "Martins" ] 5 => array:2 [ "nombre" => "Vanda" "apellidos" => "Azevedo" ] 6 => array:2 [ "nombre" => "Eduardo" "apellidos" => "Castela" ] 7 => array:2 [ "nombre" => "Manuel J." "apellidos" => "Antunes" ] ] ] ] ] "idiomaDefecto" => "pt" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2174204919300509" "doi" => "10.1016/j.repce.2017.07.015" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204919300509?idApp=UINPBA00004E" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255117300100?idApp=UINPBA00004E" "url" => "/08702551/0000003800000002/v6_201912041757/S0870255117300100/v6_201912041757/pt/main.assets" ] ] "itemSiguiente" => array:20 [ "pii" => "S2174204919300637" "issn" => "21742049" "doi" => "10.1016/j.repce.2017.08.012" "estado" => "S300" "fechaPublicacion" => "2019-02-01" "aid" => "1260" "copyright" => "Sociedade Portuguesa de Cardiologia" "documento" => "article" "crossmark" => 1 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "sco" "cita" => "Rev Port Cardiol. 2019;38:161-2" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 352 "formatos" => array:3 [ "EPUB" => 53 "HTML" => 186 "PDF" => 113 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Image in Cardiology</span>" "titulo" => "Acute myocardial infarction complicated with cardiogenic shock as a late onset of Kawasaki disease" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "161" "paginaFinal" => "162" ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Enfarte agudo do miocárdio complicado por choque cardiogénico devido a início tardio da doença de Kawasaki" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 965 "Ancho" => 2167 "Tamanyo" => 201822 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Right coronary angiogram.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(A) Calcified giant aneurysm of the right coronary artery is easier to see on the fluoroscopy; (B) total thrombotic occlusion of the proximal right coronary artery.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Rocío González Ferreiro, Diego López Otero, Alfredo Redondo Diéguez, José Luis Martínez Sande, Ramiro Trillo Nouche, José Ramón González Juanatey" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Rocío González" "apellidos" => "Ferreiro" ] 1 => array:2 [ "nombre" => "Diego López" "apellidos" => "Otero" ] 2 => array:2 [ "nombre" => "Alfredo Redondo" "apellidos" => "Diéguez" ] 3 => array:2 [ "nombre" => "José Luis Martínez" "apellidos" => "Sande" ] 4 => array:2 [ "nombre" => "Ramiro Trillo" "apellidos" => "Nouche" ] 5 => array:2 [ "nombre" => "José Ramón González" "apellidos" => "Juanatey" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S0870255117305310" "doi" => "10.1016/j.repc.2017.08.011" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255117305310?idApp=UINPBA00004E" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204919300637?idApp=UINPBA00004E" "url" => "/21742049/0000003800000002/v1_201904100624/S2174204919300637/v1_201904100624/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2174204919300789" "issn" => "21742049" "doi" => "10.1016/j.repce.2018.05.016" "estado" => "S300" "fechaPublicacion" => "2019-02-01" "aid" => "1354" "copyright" => "Sociedade Portuguesa de Cardiologia" "documento" => "article" "crossmark" => 1 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "rev" "cita" => "Rev Port Cardiol. 2019;38:143-58" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 592 "formatos" => array:3 [ "EPUB" => 73 "HTML" => 268 "PDF" => 251 ] ] "en" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review article</span>" "titulo" => "Frailty in cardiovascular disease: Screening tools" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "pt" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "143" "paginaFinal" => "158" ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Fragilidade nas doenças cardiovasculares: instrumentos de rastreio" ] ] "contieneResumen" => array:2 [ "en" => true "pt" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Ana Zão, Sandra Magalhães, Mário Santos" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Ana" "apellidos" => "Zão" ] 1 => array:2 [ "nombre" => "Sandra" "apellidos" => "Magalhães" ] 2 => array:2 [ "nombre" => "Mário" "apellidos" => "Santos" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204919300789?idApp=UINPBA00004E" "url" => "/21742049/0000003800000002/v1_201904100624/S2174204919300789/v1_201904100624/en/main.assets" ] "en" => array:21 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case report</span>" "titulo" => "Unusual cause of left ventricular dysfunction in a child" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "159.e1" "paginaFinal" => "159.e5" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Maria Emanuel Amaral, Pedro Epifânio, Natália Noronha, António Pires, Paula Martins, Vanda Azevedo, Eduardo Castela, Manuel J. Antunes" "autores" => array:8 [ 0 => array:4 [ "nombre" => "Maria Emanuel" "apellidos" => "Amaral" "email" => array:1 [ 0 => "mariaemanuelamaral@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Pedro" "apellidos" => "Epifânio" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Natália" "apellidos" => "Noronha" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "António" "apellidos" => "Pires" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "Paula" "apellidos" => "Martins" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 5 => array:3 [ "nombre" => "Vanda" "apellidos" => "Azevedo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 6 => array:3 [ "nombre" => "Eduardo" "apellidos" => "Castela" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 7 => array:3 [ "nombre" => "Manuel J." "apellidos" => "Antunes" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Serviço de Cardiologia Pediátrica – Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Serviço de Cardiologia - Hospital Agostinho Neto, Cidade da Praia, Cape Verde" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Centro de Cirurgia Cardiotorácica, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Causa rara de disfunção ventricular esquerda em idade pediátrica" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1903 "Ancho" => 2667 "Tamanyo" => 415258 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiogram with color Doppler. Short-axis and long-axis parasternal views show interventricular systolic and diastolic flows due to the extensive collateral network (A and B); short-axis parasternal view reveals anterograde flow in the right coronary artery, which is dilated in its proximal portion (C), and retrograde flow in the main pulmonary artery (arrow), corresponding to runoff from the left coronary artery (D).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) accounts for 0.24-0.46% of congenital heart defects.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Although rare, it is the most common congenital coronary artery anomaly. Without treatment, only 10-15% of cases reach adulthood; survival depends on the development of an intercoronary collateral network.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Echocardiography is the first-line diagnostic method, but other imaging exams may be required to obtain better definition of the coronary anatomy. Computed tomography angiography (CTA) can identify the origin of the coronary arteries and their relation with adjacent structures, and these measurements are crucial to planning the surgical approach. However, for many authors, selective coronary angiography remains the gold standard for surgical planning.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Surgery is the treatment of choice for patients with ALCAPA and in severely symptomatic children may have to be performed on an emergency basis. Ideally a dual coronary perfusion system should be created. In infants when the anatomy is favorable, the best option is for the most anatomical correction, the direct implantation of the LCA into the aorta. Alternatively, an intrapulmonary artery baffle can be created from the left coronary ostium to the aorta (Takeuchi procedure). However, this solution is subject to late complications including pulmonary stenosis, intrapulmonary leak, and aortic stenosis and regurgitation.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">In adults, coronary artery bypass grafting of the LCA is usually performed following ligation of its origin in the pulmonary artery. The advantage of this technique is that it avoids manipulation of the aortic root, but this must be weighed against the risk associated with a less durable graft.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The clinical course following surgical correction is generally favorable.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors present the case of an eight-year-old black girl transferred from Cape Verde in order to clarify heart disease.</p><p id="par0035" class="elsevierStylePara elsevierViewall">She had been suffering from heart failure since the age of three months, and at 11 months was diagnosed by echocardiography with dilated cardiomyopathy. Anticongestive therapy was begun, resulting in clinical improvement.</p><p id="par0040" class="elsevierStylePara elsevierViewall">She was transferred at the age of eight years to our center for further diagnostic clarification and treatment. Although asymptomatic, she presented a continuous murmur at the left upper parasternal border.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The chest X-ray revealed mild cardiomegaly and the electrocardiogram showed criteria for left ventricular hypertrophy, with signs of subendocardial ischemia of the lateral and septal wall but no evidence of necrosis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Echocardiography revealed dilatation of the left ventricle with a hypokinetic and aneurysmal apical region, and increased echogenicity of the subvalvular mitral apparatus compatible with fibroelastosis secondary to ischemia of the papillary muscle. Doppler study identified multiple interventricular systolic and diastolic flows with maximum velocity <1 ms (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>A and B). There was no mitral regurgitation and ejection fraction by Simpson's method was 48%. Right coronary artery (RCA) ectasia was observed (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>C) and the origin of the LCA could not be identified. Retrograde flow in the main pulmonary artery (MPA) immediately above the pulmonary valve was seen in short-axis parasternal view, suggestive of runoff from the LCA (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>D, arrow).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">To confirm the diagnosis, CTA was performed, which showed the left main coronary artery originating from the left lower aspect of the MPA, about 10 mm from the aortic root (<a class="elsevierStyleCrossRefs" href="#fig0010">Figures 2 and 3</a>). This distance raised doubts concerning the feasibility of direct implantation of the LCA into the aorta. The patient's periods of higher heart rate despite negative chronotropic medication and motion-induced artefacts meant that only the proximal portions of the coronary arteries could be defined.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">Cardiac catheterization confirmed the diagnosis of ALCAPA as well as retrograde perfusion of the LCE by the RCA via an extensive collateral network, followed by drainage into the MPA. It also detected reduced luminal diameter of the distal portion of the anterior descending artery (<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>A and C). Left ventriculography showed aneurysmal dilatation of the left ventricular apical region (<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>A) and mild systolic dysfunction. Left ventricular end-diastolic pressure was elevated but pulmonary vascular pressure and resistance were normal. Attempted selective catheterization of the LCA via the MPA was unsuccessful, and angiography of the MPA showed no opacification of the LCA.</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">Following discussion with the surgical team, the patient underwent successful direct implantation of the LCA into the aorta. Her postoperative course was favorable.</p><p id="par0070" class="elsevierStylePara elsevierViewall">The electrocardiographic alterations observed at admission persisted. Before return to her country of origin, in order to exclude perfusion deficits following coronary implantation, she underwent exercise testing according to the Bruce protocol, which excluded arrhythmia or worsening of septal and lateral wall repolarization abnormalities with exercise. Echocardiographic assessment showed adequate flow in the reimplanted coronary artery and normal left chamber dimensions, with improvement in left ventricular systolic dysfunction.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0075" class="elsevierStylePara elsevierViewall">ALCAPA can be classified into two types, infant and adult, according to its clinical expression. In fetal life, elevated pulmonary pressures lead to antegrade flow in the LCA, enabling good adaptation in utero. Following birth, the gradual lowering of pulmonary pressure and vascular resistance results in progressively reduced coronary perfusion, and eventually to flow reversal in the LCA.</p><p id="par0080" class="elsevierStylePara elsevierViewall">In the more common infant type, a collateral network does not form after flow reversal in the LCA and the diameter of the coronary arteries remains normal. The reduced blood supply to the myocardium in the LCA territory results in ischemic heart disease that leads to 90% mortality in the first year of life.</p><p id="par0085" class="elsevierStylePara elsevierViewall">Flow reversal in the LCA in the adult type triggers the formation of an intercoronary collateral circulation and gradual dilatation of the coronaries, enabling adequate filling pressure in the LCA from the RCA. Progressive coronary dilatation means that blood flows preferentially to the pulmonary artery rather than to the high-resistance myocardial system, leading to the phenomenon known as coronary steal. These patients develop chronic ischemic heart disease and are at high risk for sudden death due to arrhythmias.</p><p id="par0090" class="elsevierStylePara elsevierViewall">It should be borne in mind that ventricular function depends more on perfusion pressure in the coronaries than on the oxygen saturation of the blood they transport. Thus, unlike in ALCAPA, in simple transposition of the great arteries, which is characterized by low oxygen saturation in the coronaries but normal coronary perfusion pressure, left ventricular function is preserved.</p><p id="par0095" class="elsevierStylePara elsevierViewall">In the case presented, a child with ALCAPA survived due to the development of an extensive right-to-left coronary collateral circulation. The onset of symptoms at the age of three months may have coincided with the progressive fall in pulmonary vascular resistance and closure of the ductus arteriosus. In her country of origin, she had been diagnosed echocardiographically with dilated cardiomyopathy of uncertain cause. The diagnosis of ALCAPA does in fact require a high index of clinical suspicion and a range of diagnostic exams.</p><p id="par0100" class="elsevierStylePara elsevierViewall">The improvement in ventricular function was probably due to the extensive collateral network that developed during the most critical phase, maintaining adequate coronary perfusion pressure and oxygen supply to the at-risk myocardium.</p><p id="par0105" class="elsevierStylePara elsevierViewall">On admission to our center, the patient was asymptomatic, reflecting the good adaptation provided by natural revascularization. The continuous murmur heard on auscultation may have been caused by runoff from the LCA to the MPA. Echocardiography revealed the typical alterations of the adult type of ALCAPA: intraventricular systolic and diastolic flows (due to the collateral network supplying the LCA); left ventricular dilatation and dysfunction secondary to myocardial ischemia; and the LCA not originating from the aorta. However, false images of the LCA emerging from the aorta have been reported. In such cases the anomalous retrograde direction of flow in the LCA indicates the correct diagnosis.</p><p id="par0110" class="elsevierStylePara elsevierViewall">CTA is hampered by technical difficulties at pediatric ages, and so investigation may need to be supplemented with coronary angiography, as in our patient.</p><p id="par0115" class="elsevierStylePara elsevierViewall">Cardiac catheterization was used in this case to characterize coronary flow and the entire coronary anatomy. It demonstrated reduced luminal diameter in the distal portion of the anterior descending artery leading to inadequate perfusion in its distal territory, with an aneurysmal region and apical hypokinesis.</p><p id="par0120" class="elsevierStylePara elsevierViewall">Surgical correction should aim to establish a dual coronary perfusion system, and this was the option chosen by our center's surgical team.</p><p id="par0125" class="elsevierStylePara elsevierViewall">Following intervention, left ventricular dysfunction and dilatation can be expected to improve. In the case reported, although postoperative follow-up has been short, normalization of left ventricular dimensions and improvement in systolic function have been observed.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0130" class="elsevierStylePara elsevierViewall">ALCAPA is a rare heart defect associated with high mortality. The natural history of the few cases that have survived un-diagnosed after the first year of life includes chronic ischemia, ventricular dysfunction, and potentially lethal arrhythmias. Multimodality diagnostic exams are important to obtain an accurate diagnosis and to provide essential information to guide surgery. Surgical correction should be considered in all patients, with preference for the establishment of a dual coronary perfusion system. Prognosis after surgery is generally favorable.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of interest</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:11 [ 0 => array:3 [ "identificador" => "xres1178802" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1100256" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1178803" "titulo" => "Resumo" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1100255" "titulo" => "Palavras-chave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of interest" ] 9 => array:2 [ "identificador" => "xack402469" "titulo" => "Acknowledgments" ] 10 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-01-08" "fechaAceptado" => "2017-07-09" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1100256" "palabras" => array:6 [ 0 => "Anomalous left coronary artery from the pulmonary artery" 1 => "Bland-White-Garland Syndrome" 2 => "Coronary anomalies" 3 => "Inter-coronary collateral circulation" 4 => "Congenital heart disease" 5 => "Takeuchi procedure" ] ] ] "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec1100255" "palabras" => array:6 [ 0 => "ALCAPA" 1 => "Síndrome de <span class="elsevierStyleItalic">Bland-White-Garland</span>" 2 => "Anomalia das coronárias" 3 => "Circulação colateral inter-coronária" 4 => "Cardiopatia congénita" 5 => "Procedimento de <span class="elsevierStyleItalic">Takeuchi</span>" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital heart defect and a cause of myocardial ischemia during childhood. Most undiagnosed cases die in the first year of life as an extensive collateral network is essential for survival. The diagnosis requires a high index of clinical suspicion. The authors present the case of an asymptomatic eight-year-old black girl referred from Cape Verde in order to clarify left ventricular dilatation and dysfunction with turbulent systolic and diastolic flows observed at the interventricular septum. At the age of three months, she was diagnosed with heart failure, subsequently classified as dilated cardiomyopathy. She improved clinically following anticongestive therapy, which was ongoing at the time of admission to our center. The echocardiogram suggested anomalous origin of the left coronary artery from the pulmonary artery, and the diagnosis was confirmed by computed tomography angiography and cardiac catheterization. The patient underwent successful direct implantation of the left coronary artery into the aorta, creating a dual coronary perfusion system. This case illustrates an unusual presentation of a rare heart condition that survived without a diagnosis after the first year of life. It also highlights the importance of multimodality imaging in these cases.</p></span>" ] "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A Origem Anómala da Artéria Coronária Esquerda da Artéria Pulmonar é uma cardiopatia congénita rara e uma causa de isquemia miocárdica em idade pediátrica. A maioria dos casos não diagnosticados morre no primeiro ano de vida, sendo necessária a formação de uma extensa rede de colaterais para permitir a sobrevivência. O diagnóstico não é linear exigindo elevada suspeição clínica. Os autores apresentam o caso de uma criança de oito anos de idade, de raça negra, assintomática, referenciada de Cabo Verde para esclarecimento de dilatação e disfunção do ventrículo esquerdo com fluxos turbulentos sistodiastólicos a nível do septo interventricular. Dos antecedentes destacava-se quadro de insuficiência cardíaca diagnosticada aos três meses de idade, com avaliação posterior compatível com miocardiopatia dilatada. Teve melhoria clínica após início de terapêutica anticongestiva que mantinha na altura da admissão no nosso Centro. Ecocardiograficamente suspeitou-se de Origem Anómala da Artéria Coronária Esquerda da Artéria Pulmonar, sendo este diagnóstico confirmado através de angiotomografia computorizada e cateterismo cardíaco. A doente foi submetida, com sucesso, a implantação direta da artéria coronária esquerda na aorta permitindo criar um sistema de perfusão coronário duplo. Este caso ilustra uma forma incomum de uma patologia rara que sobreviveu sem diagnóstico após o primeiro ano de vida. Reforça igualmente a importância da multimodalidade de imagem nestes casos.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Amaral ME, Epifânio P, Noronha N, Pires A, Martins P, Azevedo V, et al. Causa rara de disfunção ventricular esquerda em idade pediátrica. Rev Port Cardiol. 2019;38:159.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1903 "Ancho" => 2667 "Tamanyo" => 415258 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Transthoracic echocardiogram with color Doppler. Short-axis and long-axis parasternal views show interventricular systolic and diastolic flows due to the extensive collateral network (A and B); short-axis parasternal view reveals anterograde flow in the right coronary artery, which is dilated in its proximal portion (C), and retrograde flow in the main pulmonary artery (arrow), corresponding to runoff from the left coronary artery (D).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 911 "Ancho" => 1400 "Tamanyo" => 112788 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Cardiac computed tomography showing the right coronary artery originating in the right sinus of Valsalva (red arrow) and the left coronary artery arising from the left posterolateral aspect of the main pulmonary artery (blue arrow).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 480 "Ancho" => 1867 "Tamanyo" => 64813 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">64-slice ECG-gated computed tomography, curved multiplanar reformatting, showing the left main coronary artery originating from the lower left aspect of the main pulmonary artery. The left main coronary artery has a caliber of 2.6 mm and a length of around 3 mm, with regular contours and preserved patency. It presents a normal bifurcation into the anterior descending artery (A) and circumflex artery (non-dominant) (B). These vessels have preserved patency in their visible portions (proximal half).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 847 "Ancho" => 2042 "Tamanyo" => 141304 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Diagnostic cardiac catheterization. Left ventriculography in posteroanterior view shows an aneurysmal region of the left ventricular apex and filling of the aorta and the right coronary artery (A); right coronary angiography in posteroanterior and right anterior oblique views shows a dilated coronary artery communicating with and filling the left coronary artery via an extensive collateral network (B and C). The left main coronary artery originates from the main pulmonary artery, and the blood flow is from aorta to right coronary artery, to collaterals, to left coronary artery, to pulmonary artery. The distal portion of the anterior descending artery is poorly filled.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:3 [ 0 => array:3 [ "identificador" => "bib0020" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anomalous origin of the left coronary artery from the pulmonary trunk" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "W. Wu" 1 => "J. Sun" 2 => "L. Ma" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ijcard.2015.08.041" "Revista" => array:6 [ "tituloSerie" => "International Journal of Cardiology" "fecha" => "2015" "volumen" => "201" "paginaInicial" => "165" "paginaFinal" => "167" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26298368" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0025" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anomalous left coronary artery from the pulmonary artery (ALCAPA) in adult age. A 5-case series successfully repaired with surgery" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "L. Yáñez-Gutiérrez" 1 => "D. López-Gallegos" 2 => "C.E. Cerrud-Sánchez" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:3 [ "tituloSerie" => "Rev Mex Cardiol" "fecha" => "2015" "volumen" => "26" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0030" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:3 [ "comentario" => "e3" "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Surgical treatment of an anomalous left coronary artery from the pulmonary artery in a sexagenarian woman" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "H. Abdallah" 1 => "I. Bouhout" 2 => "S. St-Onge" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Can J Cardiol" "fecha" => "2016" "volumen" => "32" "numero" => "1576." "paginaInicial" => "e1" "paginaFinal" => "e1576" ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack402469" "titulo" => "Acknowledgments" "texto" => "<p id="par0140" class="elsevierStylePara elsevierViewall">The authors thank Dr. Rui Catarino for his assistance in the production and choice of the images used in this case study.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/21742049/0000003800000002/v1_201904100624/S2174204919300509/v1_201904100624/en/main.assets" "Apartado" => array:4 [ "identificador" => "9919" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case Reports" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/file/21742049/0000003800000002/v1_201904100624/S2174204919300509/v1_201904100624/en/main.pdf?idApp=UINPBA00004E&text.app=https://revportcardiol.org/&text=false" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204919300509?idApp=UINPBA00004E" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 9 | 4 | 13 |
| 2024 October | 34 | 28 | 62 |
| 2024 September | 56 | 21 | 77 |
| 2024 August | 50 | 25 | 75 |
| 2024 July | 42 | 38 | 80 |
| 2024 June | 36 | 22 | 58 |
| 2024 May | 39 | 31 | 70 |
| 2024 April | 47 | 26 | 73 |
| 2024 March | 35 | 22 | 57 |
| 2024 February | 30 | 22 | 52 |
| 2024 January | 38 | 29 | 67 |
| 2023 December | 46 | 35 | 81 |
| 2023 November | 55 | 26 | 81 |
| 2023 October | 47 | 16 | 63 |
| 2023 September | 34 | 16 | 50 |
| 2023 August | 26 | 16 | 42 |
| 2023 July | 22 | 20 | 42 |
| 2023 June | 43 | 17 | 60 |
| 2023 May | 45 | 36 | 81 |
| 2023 April | 27 | 7 | 34 |
| 2023 March | 39 | 17 | 56 |
| 2023 February | 31 | 18 | 49 |
| 2023 January | 28 | 14 | 42 |
| 2022 December | 48 | 18 | 66 |
| 2022 November | 34 | 24 | 58 |
| 2022 October | 44 | 29 | 73 |
| 2022 September | 32 | 45 | 77 |
| 2022 August | 36 | 31 | 67 |
| 2022 July | 37 | 41 | 78 |
| 2022 June | 25 | 25 | 50 |
| 2022 May | 29 | 34 | 63 |
| 2022 April | 31 | 24 | 55 |
| 2022 March | 43 | 40 | 83 |
| 2022 February | 38 | 35 | 73 |
| 2022 January | 32 | 29 | 61 |
| 2021 December | 39 | 21 | 60 |
| 2021 November | 28 | 34 | 62 |
| 2021 October | 51 | 56 | 107 |
| 2021 September | 29 | 25 | 54 |
| 2021 August | 36 | 29 | 65 |
| 2021 July | 23 | 26 | 49 |
| 2021 June | 33 | 32 | 65 |
| 2021 May | 40 | 35 | 75 |
| 2021 April | 60 | 40 | 100 |
| 2021 March | 47 | 25 | 72 |
| 2021 February | 38 | 13 | 51 |
| 2021 January | 41 | 19 | 60 |
| 2020 December | 35 | 9 | 44 |
| 2020 November | 20 | 16 | 36 |
| 2020 October | 34 | 16 | 50 |
| 2020 September | 31 | 12 | 43 |
| 2020 August | 20 | 12 | 32 |
| 2020 July | 18 | 15 | 33 |
| 2020 June | 22 | 18 | 40 |
| 2020 May | 20 | 8 | 28 |
| 2020 April | 19 | 17 | 36 |
| 2020 March | 25 | 15 | 40 |
| 2020 February | 98 | 19 | 117 |
| 2020 January | 22 | 8 | 30 |
| 2019 December | 31 | 7 | 38 |
| 2019 November | 6 | 6 | 12 |
| 2019 October | 19 | 6 | 25 |
| 2019 September | 11 | 6 | 17 |
| 2019 August | 7 | 5 | 12 |
| 2019 July | 20 | 10 | 30 |
| 2019 June | 28 | 29 | 57 |
| 2019 May | 24 | 16 | 40 |
| 2019 April | 29 | 12 | 41 |
| 2019 March | 9 | 12 | 21 |
Show all