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Since that time much has changed in our knowledge of hypertrophic cardiomyopathy (HCM).</p><p id="par0010" class="elsevierStylePara elsevierViewall">With regard to phenotypes, unlike what was previously believed, the hallmark of the disease (septal hypertrophy) is only seen in about two-thirds of patients.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">2</span></a> Even fibrosis can now be quantified by cardiac magnetic resonance imaging.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Information on hemodynamics in HCM has advanced from the first descriptions of abnormal systolic anterior motion (SAM) of the mitral valve and resulting mitral regurgitation on angiography in the mid-1960s<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> to the ability to quantify obstruction at rest by provocative maneuvers or exercise testing.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The view of the importance of genomics in HCM has also changed, moving toward broader, more inclusive models that include both biological risk and acquired factors to explain the disease. Mutations in a single sarcomere gene are no longer considered an adequate driving force for the disease, since they do not properly explain regional left ventricular hypertrophy and myocardial fibrosis, as well as structurally abnormal elongated mitral valve leaflets and remodeled intramural coronary arterioles, which involve tissue types that do not express cardiomyocyte sarcomere proteins.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">In terms of outcomes, in contrast to what was initially thought, most individuals with HCM have near-normal life expectancy, and many remain asymptomatic throughout life, only some patients develop heart failure, angina, syncope or even sudden cardiac death, which may be caused by different mechanisms.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">7</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">There is nowadays a range of treatments available for HCM, including behavior modification, devices, drugs, and surgical or percutaneous intervention.<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">7,8</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Nearly two-thirds of patients with HCM have a significant gradient across the left ventricular outflow tract (LVOT) at rest or during provocative maneuvers or exercise. LVOT obstruction results from the combined effects of septal hypertrophy and abnormalities of the mitral valve apparatus (systolic flow drags the elongated and abnormally positioned anterior mitral leaflet into the LVOT). Coaptation of the mitral leaflet is distorted, resulting in dynamic mitral regurgitation, which plays an important role in symptoms. LVOT obstruction has several pathophysiological consequences, including reduction of cardiac output, diastolic dysfunction, secondary mitral regurgitation, and myocardial ischemia.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">9</span></a> A resting LVOT gradient of ≥30 mmHg is a predictor of both all-cause mortality and arrhythmic events.<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">7,8</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The main treatment in these patients is negative inotropic drugs (beta-blockers, calcium channel blockers and disopyramide), but 5-10% remain symptomatic and need additional therapy, such as pacemaker implantation, surgical septal myectomy or alcohol septal ablation (ASA).<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The 2011 AHA/ACC guidelines<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">8</span></a> consider septal myectomy the gold standard technique for septal reduction therapy, and advise against performing ASA in patients who are younger or who present marked septal hypertrophy (>30 mm) or concomitant cardiac disease. ASA can be offered to elderly patients, those with high surgical risk, and those who refuse open-heart surgery. As there have been no randomized trials comparing surgery and ASA, the guidelines are based on observational studies, but current evidence<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">11</span></a> is closer to the European guidelines, which accept both approaches, and recommend individual assessment based on a heart team discussion.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Three meta-analyses – Agarwal et al.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">12</span></a> (2010), Leonardi et al.<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">13</span></a> (2010), and Liebregts et al.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">14</span></a> (2015) – have confirmed that both procedures are efficacious, with no differences in symptom relief, safety, or mortality, only a more frequent need for pacemaker implantation with ASA. The fear that additional scar tissue secondary to ablation could result in an arrhythmic substrate, sudden cardiac death or evolution to systolic dysfunction is now a thing of the past.<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">15,16</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Regarding treatment with ASA of LVOT gradients refractory to medical therapy, the study by Rosa et al.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">17</span></a> published in this issue of the <span class="elsevierStyleItalic">Journal</span> adds robustness to previous data. The authors report a mean 50% reduction in LVOT gradient within a year of the procedure in 85.7% of patients, improved New York Heart Association functional class in 77%, permanent pacemaker implantation in 8.8%, redo ASA in 10%, myectomy in 2.5%, and cardiac death in 2.7% (two patients), similar figures to the Euro-ASA registry<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> and high-volume centers (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">Concerns about differences in outcomes between high- and low-volume centers have been voiced on both sides of the Atlantic. In the US Nationwide Inpatient Database,<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">11</span></a> myectomy had different outcomes in high- and low-volume centers, whereas this was not seen with ASA. Veselka at al.<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">20</span></a> showed that in the Euro-ASA Registry, the first consecutive 50 patients treated in each center had worse outcomes than patients treated thereafter. Although Rosa et al.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">17</span></a> only performed around 10 ablations per year (80 patients in seven years), their excellent results provide reassurance that ASA can be a viable option for patients in Portugal to relieve obstruction without compromising safety even in relatively young patients (mean age 63.9±12.3 years). The safety of the procedure even in low-volume centers can be explained by the high degree of skill required to treat coronary total occlusion percutaneously, which interventional cardiologists who perform a large number of angioplasties achieve on a daily basis. The major challenges facing those who perform ASA are firstly patient selection (anatomy, gradient, previous medical therapy, comorbidities), secondly how much alcohol to infuse and into which septal branch(es), and lastly management of the cardiac conduction system. Unfortunately Rosa et al. could not add any data to help optimize patient selection, mainly because they were unable to find any markers that were correlated with success.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Since randomized trials comparing ASA and surgical myectomy are unlikely to occur, the report by Rosa at al. supports the latest consensus<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">21,22</span></a> that, if deemed suitable<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">23</span></a> after a heart team discussion (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>), both techniques should be proposed to patients, explaining the advantages and disadvantages of each, and taking the patient's wishes into account.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0070" class="elsevierStylePara elsevierViewall">Current evidence based on the latest knowledge, such as that provided in the report by Rosa et al. and others, sheds light on the options available for treating HCM, and should motivate physicians to fight inertia and remember in their daily practice that their patients could be relieved by alcohol.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The author has 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 "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1740 "Ancho" => 1500 "Tamanyo" => 162638 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Indication criteria for alcohol septal ablation (ASA) versus myectomy.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">T: tertile of hospital volume.</p>" "tablatextoimagen" => array:1 [ 0 => array:1 [ "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="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">No. of patients \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Improved symptoms \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Pacemaker implantation \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">In-hospital cardiovascular mortality \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Total cardiovascular mortality \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Rosa et al.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">17</span></a> (2019) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">80 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">77% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.8% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1.25% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2.5% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Batzner et al.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> (2019) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">952 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">94.3% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10.5% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0.21% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1.47% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">US Nationwide Inpatient Database<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">11</span></a> (2016) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4862 (248 centers) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">- \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Total: 11.9%1st T: 14.2%2nd T: 12.4%3rd T: 11.5% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Total: 0.7%1st T: 0.3%2nd T: 0.8%3rd T: 0.6% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">- \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Euro-ASA Registry<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> (2016) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1275 (10 centers) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">86% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2.4% \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Comparison between the results of Rosa et al.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">17</span></a> and previous data on alcohol septal ablation.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:23 [ 0 => array:3 [ "identificador" => "bib0125" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Asymmetrical hypertrophy of the heart in young adults" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "D. 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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 8 | 4 | 12 |
| 2024 Outubro | 50 | 28 | 78 |
| 2024 Setembro | 51 | 24 | 75 |
| 2024 Agosto | 44 | 29 | 73 |
| 2024 Julho | 47 | 32 | 79 |
| 2024 Junho | 47 | 24 | 71 |
| 2024 Maio | 49 | 26 | 75 |
| 2024 Abril | 50 | 28 | 78 |
| 2024 Maro | 62 | 24 | 86 |
| 2024 Fevereiro | 35 | 16 | 51 |
| 2024 Janeiro | 23 | 31 | 54 |
| 2023 Dezembro | 38 | 26 | 64 |
| 2023 Novembro | 40 | 32 | 72 |
| 2023 Outubro | 21 | 17 | 38 |
| 2023 Setembro | 20 | 18 | 38 |
| 2023 Agosto | 24 | 17 | 41 |
| 2023 Julho | 21 | 9 | 30 |
| 2023 Junho | 16 | 13 | 29 |
| 2023 Maio | 35 | 27 | 62 |
| 2023 Abril | 27 | 6 | 33 |
| 2023 Maro | 72 | 21 | 93 |
| 2023 Fevereiro | 37 | 21 | 58 |
| 2023 Janeiro | 11 | 14 | 25 |
| 2022 Dezembro | 54 | 24 | 78 |
| 2022 Novembro | 36 | 25 | 61 |
| 2022 Outubro | 68 | 26 | 94 |
| 2022 Setembro | 24 | 29 | 53 |
| 2022 Agosto | 32 | 32 | 64 |
| 2022 Julho | 22 | 39 | 61 |
| 2022 Junho | 26 | 26 | 52 |
| 2022 Maio | 17 | 35 | 52 |
| 2022 Abril | 24 | 30 | 54 |
| 2022 Maro | 37 | 45 | 82 |
| 2022 Fevereiro | 19 | 35 | 54 |
| 2022 Janeiro | 13 | 23 | 36 |
| 2021 Dezembro | 20 | 31 | 51 |
| 2021 Novembro | 21 | 40 | 61 |
| 2021 Outubro | 35 | 45 | 80 |
| 2021 Setembro | 18 | 27 | 45 |
| 2021 Agosto | 24 | 33 | 57 |
| 2021 Julho | 16 | 29 | 45 |
| 2021 Junho | 20 | 29 | 49 |
| 2021 Maio | 30 | 37 | 67 |
| 2021 Abril | 48 | 25 | 73 |
| 2021 Maro | 40 | 22 | 62 |
| 2021 Fevereiro | 31 | 13 | 44 |
| 2021 Janeiro | 25 | 22 | 47 |
| 2020 Dezembro | 23 | 24 | 47 |
| 2020 Novembro | 28 | 23 | 51 |
| 2020 Outubro | 13 | 11 | 24 |
| 2020 Setembro | 28 | 12 | 40 |
| 2020 Agosto | 26 | 12 | 38 |
| 2020 Julho | 14 | 22 | 36 |
| 2020 Junho | 23 | 21 | 44 |
| 2020 Maio | 19 | 4 | 23 |
| 2020 Abril | 34 | 24 | 58 |
| 2020 Maro | 28 | 15 | 43 |
| 2020 Fevereiro | 42 | 30 | 72 |
| 2020 Janeiro | 16 | 11 | 27 |
| 2019 Dezembro | 23 | 25 | 48 |
| 2019 Novembro | 54 | 20 | 74 |
| 2019 Outubro | 105 | 39 | 144 |
| 2019 Setembro | 27 | 23 | 50 |
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