array:24 [
  "pii" => "S0870255121004522"
  "issn" => "08702551"
  "doi" => "10.1016/j.repc.2021.10.008"
  "estado" => "S300"
  "fechaPublicacion" => "2022-02-01"
  "aid" => "1853"
  "copyright" => "Sociedade Portuguesa de Cardiologia"
  "copyrightAnyo" => "2021"
  "documento" => "simple-article"
  "crossmark" => 1
  "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
  "subdocumento" => "dis"
  "cita" => "Rev Port Cardiol. 2022;41:179.e1-179.e2"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:1 [
    "total" => 0
  ]
  "itemSiguiente" => array:19 [
    "pii" => "S0870255121004455"
    "issn" => "08702551"
    "doi" => "10.1016/j.repc.2021.05.012"
    "estado" => "S300"
    "fechaPublicacion" => "2022-02-01"
    "aid" => "1846"
    "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. 2022;41:181-2"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "en" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Image in Cardiology</span>"
      "titulo" => "Arterial waveform during extracorporeal cardiopulmonary resuscitation"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "181"
          "paginaFinal" => "182"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "pt" => array:1 [
          "titulo" => "Curva de linha arterial durante ressuscita&#231;&#227;o cardiopulmonar"
        ]
      ]
      "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" => 302
              "Ancho" => 1750
              "Tamanyo" => 52600
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Electrocardiographic and arterial line waveforms during resuscitation&#46; &#40;a&#41; Pulseless electrical activity cardiac arrest with an almost flat arterial waveform at 0-5 mmHg and start of manual compression generating a pulsatile blood flow with a low mean arterial pressure&#59; &#40;b&#41; compressions using a chest compression system generating a pulsatile blood flow with a mean arterial pressure of 50 mmHg&#59; &#40;c&#41; arterial line waveform showing an almost flat arterial wave form with a mean arterial pressure of 60 mmHg during initial venoarterial extracorporeal membrane oxygenation&#59; &#40;d&#41; arterial line waveform showing recovery of pulsatile blood pressure with a pulse pressure of 10 mmHg after 5 min of venoarterial extracorporeal membrane oxygenation&#59; &#40;e&#41; arterial line waveform showing full recovery of pulsatile blood pressure after percutaneous coronary intervention&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Jo&#227;o Jo&#227;o Mendes, Ruben Ramos, Paulo Franco, Pedro Ponce"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "Jo&#227;o Jo&#227;o"
              "apellidos" => "Mendes"
            ]
            1 => array:2 [
              "nombre" => "Ruben"
              "apellidos" => "Ramos"
            ]
            2 => array:2 [
              "nombre" => "Paulo"
              "apellidos" => "Franco"
            ]
            3 => array:2 [
              "nombre" => "Pedro"
              "apellidos" => "Ponce"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255121004455?idApp=UINPBA00004E"
    "url" => "/08702551/0000004100000002/v1_202202160601/S0870255121004455/v1_202202160601/en/main.assets"
  ]
  "itemAnterior" => array:19 [
    "pii" => "S0870255121004479"
    "issn" => "08702551"
    "doi" => "10.1016/j.repc.2018.10.016"
    "estado" => "S300"
    "fechaPublicacion" => "2022-02-01"
    "aid" => "1848"
    "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. 2022;41:177&#46;e1-177&#46;e4"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "en" => array:13 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Case report</span>"
      "titulo" => "Ruptured sinus of Valsalva aneurysm&#58; A reversible cause of suprasystemic pulmonary hypertension"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "tieneResumen" => array:2 [
        0 => "en"
        1 => "pt"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "177&#46;e1"
          "paginaFinal" => "177&#46;e4"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "pt" => array:1 [
          "titulo" => "<span class="elsevierStyleItalic">Shunt</span> card&#237;aco e hipertens&#227;o pulmonar&#58; uma associa&#231;&#227;o preocupante mas corrig&#237;vel"
        ]
      ]
      "contieneResumen" => array:2 [
        "en" => true
        "pt" => true
      ]
      "contieneTextoCompleto" => array:1 [
        "en" => true
      ]
      "contienePdf" => array:1 [
        "en" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0010"
          "etiqueta" => "Figure 2"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr2.jpeg"
              "Alto" => 954
              "Ancho" => 2175
              "Tamanyo" => 250168
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Fluoroscopic and echocardiographic images of the ruptured sinus of Valsalva aneurysm &#40;arrow&#41; during transient percutaneous occlusion testing &#40;A&#41; and after definitive closure &#40;B&#41; with the 12-mm Amplatzer Muscular VSD device&#44; with trace peri-device leak and trivial left-to-right shunt through the VSD&#59; &#40;C&#41; one-year echocardiographic follow-up showing appropriate apposition and sealing of the device and absence of pulmonary hypertension&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Lluis Asmarats, Armando Bethencourt, Antonio Rodr&#237;guez, Marta Noris, Jaume Maristany, Andr&#233;s Grau, Alfredo G&#243;mez-Jaume, Vicente Peral"
          "autores" => array:8 [
            0 => array:2 [
              "nombre" => "Lluis"
              "apellidos" => "Asmarats"
            ]
            1 => array:2 [
              "nombre" => "Armando"
              "apellidos" => "Bethencourt"
            ]
            2 => array:2 [
              "nombre" => "Antonio"
              "apellidos" => "Rodr&#237;guez"
            ]
            3 => array:2 [
              "nombre" => "Marta"
              "apellidos" => "Noris"
            ]
            4 => array:2 [
              "nombre" => "Jaume"
              "apellidos" => "Maristany"
            ]
            5 => array:2 [
              "nombre" => "Andr&#233;s"
              "apellidos" => "Grau"
            ]
            6 => array:2 [
              "nombre" => "Alfredo"
              "apellidos" => "G&#243;mez-Jaume"
            ]
            7 => array:2 [
              "nombre" => "Vicente"
              "apellidos" => "Peral"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255121004479?idApp=UINPBA00004E"
    "url" => "/08702551/0000004100000002/v1_202202160601/S0870255121004479/v1_202202160601/en/main.assets"
  ]
  "en" => array:12 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Editorial comment</span>"
    "titulo" => "Congenital heart disease&#58; When simple becomes complex"
    "tieneTextoCompleto" => true
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "179&#46;e1"
        "paginaFinal" => "179&#46;e2"
      ]
    ]
    "autores" => array:1 [
      0 => array:3 [
        "autoresLista" => "S&#233;rgio Madeira"
        "autores" => array:1 [
          0 => array:3 [
            "nombre" => "S&#233;rgio"
            "apellidos" => "Madeira"
            "email" => array:1 [
              0 => "serg.lou.madeira@gmail.com"
            ]
          ]
        ]
        "afiliaciones" => array:1 [
          0 => array:2 [
            "entidad" => "Centro Hospitalar de Lisboa Ocidental-Hospital de Santa Cruz&#44; Carnaxide&#44; Portugal"
            "identificador" => "aff0005"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "pt" => array:1 [
        "titulo" => "Cardiopatia cong&#233;nita &#8211; quando o simples se torna complexo"
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital heart disease &#40;CHD&#41; comprises a large range of cardiac defects&#44; repaired or unrepaired&#44; which determine the pathophysiological evolution and clinical presentation in adulthood&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">1</span></a> Pulmonary arterial hypertension &#40;PAH&#41;&#44; characterized by proliferative obstructive small vessel vasculopathy&#44; is not uncommon &#40;estimated prevalence 4&#46;2-28&#37;&#41; in adult CHD &#40;ACHD&#41; patients&#44; although other forms &#40;types 2&#44; 4 and 5&#44; see below&#41; of pulmonary hypertension &#40;PH&#41; may also develop&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">PAH-CHD is usually associated with the presence of a shunt and encompasses four clinical groups&#58; &#40;1&#41; Eisenmenger syndrome&#59; &#40;2&#41; PAH associated with predominant systemic-to-pulmonary shunt&#59; &#40;3&#41; PAH associated with a small defect&#59; and &#40;4&#41; PAH associated with a repaired defect&#44; with the latter two&#44; carrying the worst prognosis&#44; resembling idiopathic PAH&#46; Other forms of CHD may be associated with different types of PH&#44; namely&#58; &#40;1&#41; congenital left heart valvular disease&#44; congenital left ventricular outflow tract obstruction and congenital pulmonary vein stenosis &#40;type 2 PH&#41;&#59; &#40;2&#41; congenital peripheral pulmonary arterial stenosis &#40;type 4 PH&#41;&#59; and &#40;3&#41; segmental PH &#40;type 5 PH&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">1&#8211;4</span></a> Furthermore&#44; PH may also develop due to comorbid acquired left heart disease&#46; The highly heterogeneous anatomical and functional milieu and dynamic competing mechanisms make PH classification&#44; interpretation and management challenging in ACHD patients&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">In this issue of the <span class="elsevierStyleItalic">Journal</span>&#44; Asmarats et al&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> report a rare case of severe PH and heart failure induced by systemic-to-pulmonary shunting through a right sinus of Valsalva aneurysm &#40;SVA&#41; ruptured into the right ventricle in a patient with a restrictive perimembranous ventricular septal defect&#46; The patient was successfully treated by percutaneous SVA closure after assessment of PH reversibility with occlusion testing&#46; Regarding the clinical case&#44; despite the possible presence of PAH associated with a small VSD&#44; the most probable cause of PH in this clinical setting was subacute pressure and volume overload imposed on the pulmonary circulation without elevation of pulmonary vascular resistance&#46; The present case reveals the complexity and the somewhat unpredictable course of a simple congenital defect&#44; creating a clinical dilemma concerning the etiology&#44; duration and management of PH&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The key messages that can be drawn from this case are&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">&#8211;</span><p id="par0025" class="elsevierStylePara elsevierViewall">All patients with ACHD should be seen at least once in a specialized clinic&#44; as even mild defects can develop serious complications&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">&#8211;</span><p id="par0030" class="elsevierStylePara elsevierViewall">An unknown percentage of patients with small VSDs develop problems later in life&#44; including SVA&#44; aortic regurgitation&#44; double-chambered right ventricle&#44; or endocarditis&#44; and require life-long follow-up &#40;intervals depending on the associated lesions&#41;&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">&#8211;</span><p id="par0035" class="elsevierStylePara elsevierViewall">Any change in clinical status in patients with ACHD should prompt a comprehensive assessment&#46;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">&#8211;</span><p id="par0040" class="elsevierStylePara elsevierViewall">PH in the context of CHD may have several overlapping etiologies&#44; and thorough assessment and elucidation of the pathophysiological mechanism are of paramount importance for appropriate management&#44; with emphasis on the role of careful invasive hemodynamic assessment and interpretation&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">&#8211;</span><p id="par0045" class="elsevierStylePara elsevierViewall">All patients with PH and unrepaired CHD should undergo thorough assessment of the etiology&#44; mechanism and reversibility of pulmonary vascular disease before it is deemed unrepairable&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">&#8211;</span><p id="par0050" class="elsevierStylePara elsevierViewall">Percutaneous shunt closure is a valid therapeutic option in appropriately selected patients&#46;</p></li></ul></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
      "secciones" => array:2 [
        0 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Conflicts of interest"
        ]
        1 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:4 [
            0 => array:3 [
              "identificador" => "bib0025"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "2020 ESC guidelines for the management of adult congenital heart disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "B&#46; Baumgartner"
                            1 => "J&#46; De Backer"
                            2 => "V&#46;S&#46; Babu-Narayan"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1093/eurheartj/ehaa554"
                      "Revista" => array:6 [
                        "tituloSerie" => "Eur Heart J"
                        "fecha" => "2021"
                        "volumen" => "42"
                        "paginaInicial" => "563"
                        "paginaFinal" => "645"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32860028"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0030"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Pulmonary arterial hypertension associated with congenital heart disease&#58; classification and pathophysiology"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "R&#46; Condliffe"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "J Congenital Cardiol"
                        "fecha" => "2020"
                        "volumen" => "4"
                        "numero" => "Suppl&#46; 1"
                        "paginaInicial" => "16"
                        "itemHostRev" => array:3 [
                          "pii" => "S1699258X14000412"
                          "estado" => "S300"
                          "issn" => "1699258X"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "2015 ESC&#47;ERS guidelines for the diagnosis and treatment of pulmonary hypertension&#58; the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology &#40;ESC&#41; and the European Respiratory Society &#40;ERS&#41;&#58; endorsed by&#58; Association for European Paediatric and Congenital Cardiology &#40;AEPC&#41;&#44; International Society for Heart and Lung Transplantation &#40;ISHLT&#41;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "N&#46; Galie"
                            1 => "M&#46; Humbert"
                            2 => "J&#46;L&#46; Vachiery"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1093/eurheartj/ehv317"
                      "Revista" => array:6 [
                        "tituloSerie" => "Eur Heart J"
                        "fecha" => "2016"
                        "volumen" => "37"
                        "paginaInicial" => "67"
                        "paginaFinal" => "119"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26320113"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0040"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cardiac shunts and pulmonary hypertension&#58; a worrying but correctable association"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "L&#46; Asmarats"
                            1 => "A&#46; Bethencourt"
                            2 => "A&#46; Rodriguez"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:3 [
                        "tituloSerie" => "Rev Port Cardiol"
                        "fecha" => "2022"
                        "volumen" => "41"
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/08702551/0000004100000002/v1_202202160601/S0870255121004522/v1_202202160601/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "91923"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Coment&#225;rio  Editorial"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/08702551/0000004100000002/v1_202202160601/S0870255121004522/v1_202202160601/en/main.pdf?idApp=UINPBA00004E&text.app=https://revportcardiol.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255121004522?idApp=UINPBA00004E"
]
Partilhar
Informação da revista
Vol. 41. Núm. 2.
Páginas 179.e1-179.e2 (fevereiro 2022)
Partilhar
Partilhar
Baixar PDF
Mais opções do artigo
Vol. 41. Núm. 2.
Páginas 179.e1-179.e2 (fevereiro 2022)
Editorial comment
Open Access
Congenital heart disease: When simple becomes complex
Cardiopatia congénita – quando o simples se torna complexo
Visitas
2450
Sérgio Madeira
Centro Hospitalar de Lisboa Ocidental-Hospital de Santa Cruz, Carnaxide, Portugal
Este item recebeu

Under a Creative Commons license
Informação do artigo
Texto Completo
Bibliografia
Baixar PDF
Estatísticas
Texto Completo

Congenital heart disease (CHD) comprises a large range of cardiac defects, repaired or unrepaired, which determine the pathophysiological evolution and clinical presentation in adulthood.1 Pulmonary arterial hypertension (PAH), characterized by proliferative obstructive small vessel vasculopathy, is not uncommon (estimated prevalence 4.2-28%) in adult CHD (ACHD) patients, although other forms (types 2, 4 and 5, see below) of pulmonary hypertension (PH) may also develop.2

PAH-CHD is usually associated with the presence of a shunt and encompasses four clinical groups: (1) Eisenmenger syndrome; (2) PAH associated with predominant systemic-to-pulmonary shunt; (3) PAH associated with a small defect; and (4) PAH associated with a repaired defect, with the latter two, carrying the worst prognosis, resembling idiopathic PAH. Other forms of CHD may be associated with different types of PH, namely: (1) congenital left heart valvular disease, congenital left ventricular outflow tract obstruction and congenital pulmonary vein stenosis (type 2 PH); (2) congenital peripheral pulmonary arterial stenosis (type 4 PH); and (3) segmental PH (type 5 PH).1–4 Furthermore, PH may also develop due to comorbid acquired left heart disease. The highly heterogeneous anatomical and functional milieu and dynamic competing mechanisms make PH classification, interpretation and management challenging in ACHD patients.

In this issue of the Journal, Asmarats et al.4 report a rare case of severe PH and heart failure induced by systemic-to-pulmonary shunting through a right sinus of Valsalva aneurysm (SVA) ruptured into the right ventricle in a patient with a restrictive perimembranous ventricular septal defect. The patient was successfully treated by percutaneous SVA closure after assessment of PH reversibility with occlusion testing. Regarding the clinical case, despite the possible presence of PAH associated with a small VSD, the most probable cause of PH in this clinical setting was subacute pressure and volume overload imposed on the pulmonary circulation without elevation of pulmonary vascular resistance. The present case reveals the complexity and the somewhat unpredictable course of a simple congenital defect, creating a clinical dilemma concerning the etiology, duration and management of PH.

The key messages that can be drawn from this case are:

  • All patients with ACHD should be seen at least once in a specialized clinic, as even mild defects can develop serious complications.

  • An unknown percentage of patients with small VSDs develop problems later in life, including SVA, aortic regurgitation, double-chambered right ventricle, or endocarditis, and require life-long follow-up (intervals depending on the associated lesions).

  • Any change in clinical status in patients with ACHD should prompt a comprehensive assessment.

  • PH in the context of CHD may have several overlapping etiologies, and thorough assessment and elucidation of the pathophysiological mechanism are of paramount importance for appropriate management, with emphasis on the role of careful invasive hemodynamic assessment and interpretation.

  • All patients with PH and unrepaired CHD should undergo thorough assessment of the etiology, mechanism and reversibility of pulmonary vascular disease before it is deemed unrepairable.

  • Percutaneous shunt closure is a valid therapeutic option in appropriately selected patients.

Conflicts of interest

The author has no conflicts of interest to declare.

References
[1]
B. Baumgartner, J. De Backer, V.S. Babu-Narayan, et al.
2020 ESC guidelines for the management of adult congenital heart disease.
Eur Heart J, 42 (2021), pp. 563-645
[2]
R. Condliffe.
Pulmonary arterial hypertension associated with congenital heart disease: classification and pathophysiology.
J Congenital Cardiol, 4 (2020), pp. 16
[3]
N. Galie, M. Humbert, J.L. Vachiery, et al.
2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Eur Heart J, 37 (2016), pp. 67-119
[4]
L. Asmarats, A. Bethencourt, A. Rodriguez, et al.
Cardiac shunts and pulmonary hypertension: a worrying but correctable association.
Rev Port Cardiol, 41 (2022),
Copyright © 2021. Sociedade Portuguesa de Cardiologia
Baixar PDF
Idiomas
Revista Portuguesa de Cardiologia
Opções de artigo
Ferramentas
en pt

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

Ao assinalar que é «Profissional de Saúde», declara conhecer e aceitar que a responsável pelo tratamento dos dados pessoais dos utilizadores da página de internet da Revista Portuguesa de Cardiologia (RPC), é esta entidade, com sede no Campo Grande, n.º 28, 13.º, 1700-093 Lisboa, com os telefones 217 970 685 e 217 817 630, fax 217 931 095 e com o endereço de correio eletrónico revista@spc.pt. Declaro para todos os fins, que assumo inteira responsabilidade pela veracidade e exatidão da afirmação aqui fornecida.