Letter to the Editor
Reply to the Letter to the Editor entitled “Acute heart failure from noncompaction requiring emergency heart transplantation”
Resposta à Carta ao Editor intitulada “Insuficiência cardíaca aguda associada a não compactação requerendo transplantação cardíaca emergente”
Rafael Alexandre Meneguz-Moreno
, Marco Aurélio Finger, Carolina Casadei, João Manoel Rossi Neto
Corresponding author
Seção Médica de Transplantes, Ambulatório de Disfunção Ventricular e Transplante Cardíaco, Instituto Dante Pazzanese de Cardiologia, Brazil
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array:4 [ "autoresLista" => "Rafael Alexandre Meneguz-Moreno, Marco Aurélio Finger, Carolina Casadei, João Manoel Rossi Neto" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Rafael Alexandre" "apellidos" => "Meneguz-Moreno" "email" => array:1 [ 0 => "rafael.meneguz@yahoo.com.br" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Marco Aurélio" "apellidos" => "Finger" ] 2 => array:2 [ "nombre" => "Carolina" "apellidos" => "Casadei" ] 3 => array:2 [ "nombre" => "João Manoel" "apellidos" => "Rossi Neto" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Seção Médica de Transplantes, Ambulatório de Disfunção Ventricular e Transplante Cardíaco, Instituto Dante Pazzanese de Cardiologia, Brazil" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Resposta à Carta ao Editor intitulada “Insuficiência cardíaca aguda associada a não compactação requerendo transplantação cardíaca emergente”" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We appreciate the interest of Josef Finsterer and Sinda Zarrouk-Mahjoub<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> in our recent original article entitled “Isolated left ventricular noncompaction causing refractory heart failure”,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> and would like to thank them for their pertinent comments.</p><p id="par0010" class="elsevierStylePara elsevierViewall">First of all, with regard to the concern about the patient's father, we agree that it would be important to have information about his echocardiographic exams or even an autopsy study. However, his father had been treated from disease onset in another hospital and we did not have access to these exams. Besides, the family did not keep the father's previous exams, so it was not possible to analyze them. The family had been contacted and insisted he had no episodes of atrial fibrillation, stroke, cardioembolism, arrhythmias or epilepsy.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Second, concerning the index patient's seizures, these occurred during use of high doses of vasoactive drugs, which could have contributed to atrial fibrillation episodes (recorded on ECG). The seizures really occurred, but only once (with no previous episode in the patient's life), and were considered to be a sign of low cardiac output (LCO) causing cerebral hypoxia due to cerebral hypoperfusion. They were tonic-clonic, lasted about two minutes, and ceased after intravenous benzodiazepine administration; at this time, an electroencephalogram was not recorded. She was then treated prophylactically with phenytoin. An intracardiac thrombus 17 mm×14 mm in size was detected in the left atrium, as reported in the main article.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> Thrombocytopenia and hepatopathy developed previous to the seizures and were attributed to the use of prophylactic heparin and systemic inflammatory response syndrome (SIRS), in combination with congestive heart failure and LCO.</p><p id="par0020" class="elsevierStylePara elsevierViewall">It is important to note that after heart transplantation and hospital discharge, the patient presented in outpatient care complaining of loss of consciousness, not sudden, but progressively and constantly. Psychological and physical (cerebral magnetic resonance imaging and EEG) investigation revealed nothing. She then underwent tilt testing; a diagnosis was made of vasovagal syndrome and she was advised to make changes in her behavior in order to prevent syncope episodes.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Furthermore, considering chromosomal defects, we understand the role of genetics in this disease<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> and that it is inherited in at least 30-50% of patients, and several genes that cause left ventricular non-compaction (LVNC) have been identified. These genes seem generally to encode sarcomere (contractile apparatus) or cytoskeletal proteins.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> However, the patient was not investigated for gene mutations and it was presumed to be a congenital abnormality, as reported.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The rest of her family were investigated by echocardiography and screened for similar myocardial changes but no evidence of myocardial hypertrabeculation/non-compaction was found. There were also no clinical or laboratory indications of neuromuscular disease in the index case or any of her relatives, such as easy fatigability, exercise intolerance, muscle wasting or weakness, myotonia or adverse reactions to general anesthesia. We have not screened for chromosomal defects as this is not a routine procedure. We understand the importance of these disorders particularly in acquired hypertrabeculation, and that up to 80% of patients with LVNC suffer from a neuromuscular disorder.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">5–7</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Concerning anatomopathology, there were non-specific findings of hypertrophy and mild degeneration of myocardial fibers, with no fibrosis or subendocardial fibroelastosis.</p><p id="par0040" class="elsevierStylePara elsevierViewall">The patient currently has no new symptoms of heart failure and has not developed LVNC in the transplanted heart.</p><p id="par0045" class="elsevierStylePara elsevierViewall">To summarize, we believe that the genesis of isolated LVNC is still not completely understood; further investigation of the disease and of patients is required, and genetic studies in particular should be the aim of future research.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</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" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute heart failure from noncompaction requiring emergency heart transplantation" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. 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De Sutter" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.2143/AC.70.6.3120173" "Revista" => array:6 [ "tituloSerie" => "Acta Cardiol" "fecha" => "2015" "volumen" => "70" "paginaInicial" => "625" "paginaFinal" => "631" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26717209" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0065" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "C. Stollberger" 1 => "J. Finsterer" 2 => "G. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 6 | 12 |
| 2024 October | 25 | 25 | 50 |
| 2024 September | 33 | 26 | 59 |
| 2024 August | 40 | 33 | 73 |
| 2024 July | 24 | 29 | 53 |
| 2024 June | 31 | 21 | 52 |
| 2024 May | 25 | 17 | 42 |
| 2024 April | 20 | 18 | 38 |
| 2024 March | 24 | 29 | 53 |
| 2024 February | 26 | 44 | 70 |
| 2024 January | 18 | 41 | 59 |
| 2023 December | 20 | 22 | 42 |
| 2023 November | 25 | 18 | 43 |
| 2023 October | 17 | 12 | 29 |
| 2023 September | 15 | 21 | 36 |
| 2023 August | 20 | 13 | 33 |
| 2023 July | 16 | 9 | 25 |
| 2023 June | 28 | 13 | 41 |
| 2023 May | 31 | 25 | 56 |
| 2023 April | 11 | 4 | 15 |
| 2023 March | 21 | 22 | 43 |
| 2023 February | 19 | 20 | 39 |
| 2023 January | 17 | 5 | 22 |
| 2022 December | 23 | 19 | 42 |
| 2022 November | 28 | 19 | 47 |
| 2022 October | 21 | 10 | 31 |
| 2022 September | 25 | 23 | 48 |
| 2022 August | 18 | 29 | 47 |
| 2022 July | 20 | 23 | 43 |
| 2022 June | 16 | 20 | 36 |
| 2022 May | 16 | 31 | 47 |
| 2022 April | 17 | 20 | 37 |
| 2022 March | 19 | 26 | 45 |
| 2022 February | 12 | 18 | 30 |
| 2022 January | 17 | 16 | 33 |
| 2021 December | 13 | 27 | 40 |
| 2021 November | 22 | 35 | 57 |
| 2021 October | 20 | 42 | 62 |
| 2021 September | 18 | 21 | 39 |
| 2021 August | 35 | 38 | 73 |
| 2021 July | 27 | 26 | 53 |
| 2021 June | 23 | 24 | 47 |
| 2021 May | 28 | 43 | 71 |
| 2021 April | 48 | 49 | 97 |
| 2021 March | 49 | 20 | 69 |
| 2021 February | 57 | 21 | 78 |
| 2021 January | 35 | 13 | 48 |
| 2020 December | 39 | 11 | 50 |
| 2020 November | 20 | 9 | 29 |
| 2020 October | 26 | 13 | 39 |
| 2020 September | 35 | 19 | 54 |
| 2020 August | 19 | 9 | 28 |
| 2020 July | 30 | 7 | 37 |
| 2020 June | 32 | 10 | 42 |
| 2020 May | 24 | 5 | 29 |
| 2020 April | 29 | 6 | 35 |
| 2020 March | 29 | 6 | 35 |
| 2020 February | 22 | 10 | 32 |
| 2020 January | 20 | 8 | 28 |
| 2019 December | 29 | 2 | 31 |
| 2019 November | 22 | 6 | 28 |
| 2019 October | 18 | 8 | 26 |
| 2019 September | 26 | 6 | 32 |
| 2019 August | 18 | 3 | 21 |
| 2019 July | 30 | 10 | 40 |
| 2019 June | 21 | 8 | 29 |
| 2019 May | 26 | 14 | 40 |
| 2019 April | 20 | 17 | 37 |
| 2019 March | 57 | 9 | 66 |
| 2019 February | 43 | 10 | 53 |
| 2019 January | 14 | 10 | 24 |
| 2018 December | 55 | 13 | 68 |
| 2018 November | 51 | 8 | 59 |
| 2018 October | 75 | 7 | 82 |
| 2018 September | 35 | 8 | 43 |
| 2018 August | 26 | 6 | 32 |
| 2018 July | 14 | 4 | 18 |
| 2018 June | 29 | 5 | 34 |
| 2018 May | 38 | 9 | 47 |
| 2018 April | 54 | 3 | 57 |
| 2018 March | 55 | 5 | 60 |
| 2018 February | 25 | 2 | 27 |
| 2018 January | 36 | 3 | 39 |
| 2017 December | 41 | 13 | 54 |
| 2017 November | 24 | 15 | 39 |
| 2017 October | 28 | 14 | 42 |
| 2017 September | 18 | 10 | 28 |
| 2017 August | 33 | 10 | 43 |
| 2017 July | 21 | 10 | 31 |
| 2017 June | 23 | 7 | 30 |
| 2017 May | 30 | 8 | 38 |
| 2017 April | 15 | 3 | 18 |
| 2017 March | 25 | 31 | 56 |
| 2017 February | 28 | 11 | 39 |
| 2017 January | 15 | 3 | 18 |
| 2016 December | 26 | 6 | 32 |
| 2016 November | 29 | 9 | 38 |
| 2016 October | 48 | 20 | 68 |
| 2016 September | 51 | 18 | 69 |
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