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1.
Heart ; 2019 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-31471463

RESUMO

OBJECTIVE: The current guidelines suggest alcohol septal ablation (ASA) is less effective in hypertrophic obstructive cardiomyopathy (HOCM) patients with severe left ventricular hypertrophy, despite acknowledging that systematic data are lacking. Therefore, we analysed patients in the Euro-ASA registry to test this statement. METHODS: We compared the short-term and long-term outcomes of patients with basal interventricular septum (IVS) thickness <30 mm Hg to those with ≥30 mm Hg treated using ASA in nine European centres. RESULTS: A total of 1519 patients (57±14 years, 49% women) with symptomatic HOCM were treated, including 67 (4.4%) patients with IVS thickness ≥30 mm. The occurrence of short-term major adverse events were similar in both groups. The mean follow-up was 5.4±4.3 years and 5.1±4.1 years, and the all-cause mortality rate was 2.57 and 2.94 deaths per 100 person-years of follow-up in the IVS <30 mm group and the IVS ≥30 mm group (p=0.047), respectively. There were no differences in dyspnoea (New York Heart Association class III/IV 12% vs 16%), residual left ventricular outflow tract gradient (16±20 vs 16±16 mm Hg) and repeated septal reduction procedures (12% vs 18%) in the IVS <30 mm group and IVS ≥30 mm group, respectively (p=NS for all). CONCLUSIONS: The short-term results and the long-term relief of dyspnoea, residual left ventricular outflow obstruction and occurrence of repeated septal reduction procedures in patients with basal IVS ≥30 mm is similar to those with IVS <30mm. However, long-term all-cause and cardiac mortality rates are worse in the ≥30 mm group.

2.
Eur Heart J ; 40(21): 1681-1687, 2019 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-31152553

RESUMO

AIMS: Based on European guidelines, alcohol septal ablation (ASA) for hypertrophic obstructive cardiomyopathy (HOCM) is indicated only in patients with interventricular septum (IVS) thickness >16 mm. The aim of this study was to evaluate the short- and long-term outcomes in ASA patients with mild hypertrophy (IVS ≤ 16 mm). METHODS AND RESULTS: We retrospectively evaluated 1505 consecutive ASA patients and used propensity score to match 172 pairs (344 patients) in groups IVS ≤ 16 mm or IVS > 16 mm. There was no occurrence of post-ASA ventriculoseptal defect in the whole cohort (n = 1505). Matched patients had 30-day mortality rate 0% in IVS ≤ 16 mm group and 0.6% in IVS > 16 mm group (P = 1). Patients in IVS ≤ 16 mm group had more ASA-attributable early complications (16% vs. 9%; P = 0.049), which was driven by higher need for pacemaker implantation (13% vs. 8%; P = 0.22). The mean follow-up was 5.4 ± 4.3 years and the annual all-cause mortality rate was 1.8 and 3.2 deaths per 100-patient-years in IVS ≤ 16 group and IVS > 16 group, respectively (log-rank test P = 0.04). There were no differences in symptom relief and left ventricular (LV) gradient reduction. Patients with IVS ≤ 16 mm had less repeated septal reduction procedures (log-rank test P = 0.03). CONCLUSION: Selected patients with HOCM and mild hypertrophy (IVS ≤ 16 mm) had more early post-ASA complications driven by need for pacemaker implantation, but their long-term survival is better than in patients with IVS >16 mm. While relief of symptoms and LV obstruction reduction is similar in both groups, a need for repeat septal reduction is higher in patients with IVS > 16 mm.

3.
Eur J Heart Fail ; 21(5): 553-576, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30989768

RESUMO

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

5.
Dtsch Arztebl Int ; 116(4): 47-53, 2019 01 25.
Artigo em Inglês | MEDLINE | ID: mdl-30855006

RESUMO

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is caused by mutations in a number of genes. Its prevalence is 0.2% to 0.6%. METHODS: This review is based on publications retrieved by a selective literature search and on the authors' clinical experi- ence. RESULTS: 70% of patients with HCM suffer from the obstructive type of the condition, clinically characterized by highly dynamic and variable manifestations in the form of dyspnea, angina pectoris, and stress-dependent presyncope and syn- cope. Younger patients are at particular risk of sudden cardiac death; thus, all patients need not only symptomatic treatment, but also risk assessment, which can be difficult in individual cases. Left ventricular obstruction, which usually causes symptoms, is treated medically at first, with either a beta- blocker or verapamil. If medical treatment fails, two invasive treatments are available, surgical myectomy and percu- taneous septum ablation. Both of these require a high level of expertise. If performed successfully, they lead to sustained gradient reduction and clinical improvement. Septum ablation is associated with low perioperative and peri-interventional mortality but necessitates permanent pacemaker implantation in 10-20% of patients. CONCLUSION: In the absence of evidence from randomized comparison trials, a suitable method of reducing the gradient should be determined by an HCM team in conjunction with each individual patient. Important criteria for decision-making include the anatomical findings and any accompanying illnesses.

6.
J Am Coll Cardiol ; 72(24): 3087-3094, 2018 12 18.
Artigo em Inglês | MEDLINE | ID: mdl-30545446

RESUMO

BACKGROUND: Alcohol-induced infarction for treatment of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) was discussed as a risk factor for increased cardiac mortality during follow-up. OBJECTIVES: This study sought to report on long-term survival after echo-guided alcohol septal ablation (percutaneous transluminal septal myocardial ablation [PTSMA]) in symptomatic patients with HOCM. METHODS: Between May 2000 and June 2017, PTSMA with alcohol injection was performed in 952 patients (age 55.7 ± 14.9 years; 59.2% men; 73.3% New York Heart Association functional class III or IV; 50.3% syncope; 10.3% sudden cardiac death in family). Clinical follow-up after 6.0 ± 5.0 years was achieved in all patients. RESULTS: We injected 2.1 ± 0.4 cc of alcohol. Maximal creatine kinase rise was 872 ± 489 U/l. Two (0.21%) patients died 3 and 33 days after ablation. Permanent pacemaker was implanted in 100 (10.50%) patients. Echo gradients were acutely reduced from 63.9 ± 38.2 mm Hg to 33.6 ± 29.8 mm Hg at rest and from 104.6 ± 44.0 mm Hg to 56.5 ± 41.0 mm Hg at Valsalva (p < 0.0001, each). During follow-up, 164 (17.2%) patients underwent reablation due to the planned staged procedure, 18 (1.9%) patients underwent surgical myectomy, and 49 (5.10%) patients underwent cardioverter-defibrillator implantation. Seventy patients died: causes of death were identified as noncardiovascular in 50, stroke related in 6, and cardiac in 14 patients. Estimated 5-year survival was 95.8%, estimated 5-year survival free of cardiovascular events was 98.6%, and an estimated 5-year survival free of cardiac events was 98.9%. Corresponding values at 10 years were 88.3%, 96.5%, and 97.0%, and at 15 years were 79.7%, 92.3%, and 96.5%. CONCLUSIONS: In this study, PTSMA could be proofed as a safe procedure with ongoing symptomatic improvement and excellent long-term survival. Therefore, PTSMA is a reasonable alternative to surgical myectomy in HOCM.

7.
Artigo em Inglês | MEDLINE | ID: mdl-30304395

RESUMO

Aims: We analysed the impact of bundle branch block (BBB) and pacemaker (PM) implantation on symptoms and survival after alcohol septal ablation (ASA) in patients with hypertrophic cardiomyopathy (HCM). Methods and Results: Among 1416 HCM patients from the Euro-ASA registry 58 (4%) patients had a PM and 64 (5%) patients had an implantable cardioverter defibrillator (ICD) before ASA. At latest follow-up (5.0±4.0 years) after ASA, 118 (8%) patients had an ICD and 229 (16%) patients had a PM. In patients without an implantable device prior to ASA 13% had a PM and 5% had an ICD implanted following ASA. New onset BBB was present in 44% (right BBB in 31%) of patients without previous BBB. At latest follow-up, we found no associations between BBB and New York Heart Association (NYHA) class 3-4 (OR 0.98, CI 0.63-1.51, p = 0.91) or Canadian Cardiovascular Society (CCS) class 3-4 (OR 1.5, CI 0.32-6.7, p = 0.62), respectively, and no associations between PM and NYHA class 3-4 (OR 1.2, CI 0.70-2.0, p = 0.52) or CCS 3-4 (OR 1.3, CI 0.24-6.6, p = 0.79), respectively. The survival after ASA was not reduced in patients with BBB (HR 0.73, CI 0.53-1.01, p = 0.06) or PM (HR 0.78, CI 0.52-1.17, p = 0.24). Conclusions: Development of BBB or need for a PM after ASA in patients with obstructive HCM was not associated with inferior symptomatic outcome or reduced survival, thus concerns for the negative impact of impaired cardiac conduction on the clinical outcome after ASA were not confirmed.

9.
Artigo em Alemão | MEDLINE | ID: mdl-29761339

RESUMO

Hypertrophic cardiomyopathy is the most common genetic cardiac disease. The most important pathophysiological finding is dynamic (outflow tract) obstruction of the left ventricle in about 70% of affected patients. Especially in younger patients, an increased risk of sudden cardiac death has been observed. Syncope and presyncope-in addition to extremely variable cardiac symptoms (dyspnea and angina)-are common. The etiology of syncope is complex. The most important aspect for diagnosis is a detailed history regarding the accompanying circumstances of the syncope. In principle, an attempt must be made to distinguish between rhythmogenic and hemodynamic causes. Diagnostic work-up should be performed under the criteria of a possible prognostic-with implantation of an implantable cardioverter-defibrillator (ICD) in patients at increased risk of sudden cardiac death-and symptomatic therapy. Depending on the underlying morphology and the experience of the surgeon, percutaneous septal ablation and operative myectomy are complementary options for symptomatic treatment if medical therapy with beta-blockers and/or verapamil is inadequate.

10.
Clin Res Cardiol ; 107(6): 479-486, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29453593

RESUMO

BACKGROUND AND STUDY OBJECTIVE: Different studies have shown a good long-term survival with improvement of symptoms and hemodynamics after percutaneous septal ablation (PTSMA), similar to myectomy considered to be the therapeutic "gold standard" for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Furthermore, desobliteration of left ventricular (LV) outflow may modify features of the disease considered to be risk-relevant, such as magnitude of LV hypertrophy, outflow gradient, and left atrial (LA) size. The aim oft this study was to examine predictors of long-term mortality in a large cohort of patients with HOCM treated with PTSMA. METHODS AND RESULTS: 497 consecutive patients with symptomatic HOCM (mean age 55.4 ± 14.4 years, NYHA class: 2.9 ± 0.4) who underwent PTSMA between 1996 and 2014 were studied. Periprocedural mortality was 1% (five patients). During a follow-up time of 64.5 ± 53.2 months (range 0.1-207), 51 patients died, of these 25 due to cardiovascular problems, while 26 deaths were attributed to non-cardiac causes. Overall survival rates were 89.4% (± 1.7) after 5 and 80.6% (± 2.7) after 10 years. Among the baseline variables, advanced age, recurrent syncope, and magnitude of LV thickening were risk markers correlated to long-term post-procedural survival. The only mortality predictor that could be identified during follow-up was post-procedural NYHA class. Neither the other classical HCM risk markers nor other clinical or echocardiographic parameters predicted cardiac or all-cause mortality in this cohort. CONCLUSIONS: Our findings suggest that the risk profile after septal ablation may differ from that of HOCM patients without an outflow desobliteration.


Assuntos
Cardiomiopatia Hipertrófica/cirurgia , Ablação por Cateter/métodos , Medição de Risco/métodos , Septo Interventricular/cirurgia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/mortalidade , Ecocardiografia Doppler , Eletrocardiografia Ambulatorial , Feminino , Seguimentos , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento , Septo Interventricular/diagnóstico por imagem
11.
Europace ; 20(FI2): f198-f203, 2018 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-29016960

RESUMO

Aims: The HCM Risk-SCD model for prediction of sudden cardiac death (SCD) in hypertrophic cardiomyopathy recommended by the 2014 European Society of Cardiology (ESC) guidelines has not been validated after septal reduction therapy. The aim of this study was to validate the HCM Risk-SCD model in patients undergoing alcohol septal ablation (ASA) and to compare its performance to previous models. Methods and result: A total of 844 ASA patients without prior SCD event were included. The primary endpoint was a composite of SCD and appropriate implantable cardioverter defibrillator (ICD) therapy, identical to the HCM Risk-SCD endpoint. A distinction between periprocedural (≤30 days) and long-term (>30 days) SCD was made to discern procedure-related adverse arrhythmic events caused by the ASA-induced myocardial infarction from long-term SCD risk. Twenty patients reached the SCD endpoint within the first 30 days. During a follow-up of 6.5 ± 4.2 years, another 46 patients reached the SCD endpoint. The predicted 5-year SCD risk according to the HCM Risk-SCD model was 5.1%, and the observed 5-year SCD risk was 4.0%. The C-statistics for the use of the HCM Risk-SCD model was 0.61 (P = 0.02), the C-statistics for the use of the 2003 American College of Cardiology/ESC guidelines was 0.59 (P = 0.051), and the C-statistic for the use of the 2011 American College of Cardiology Foundation/American Heart Association guidelines was 0.58 (P = 0.054). Maximal left ventricular wall thickness, syncope after ASA, and fulfilling the 2014 ESC recommendations for primary ICD implantation according to the HCM Risk-SCD model, respectively, predicted SCD during long-term follow-up. Conclusion: The HCM Risk-SCD model can be used for SCD prediction in patients undergoing ASA.

12.
Can J Cardiol ; 34(1): 16-22, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29275877

RESUMO

BACKGROUND: The current American College of Cardiology Foundation/American Heart Association guidelines on hypertrophic cardiomyopathy state that institutional experience is a key determinant of successful outcomes and lower complication rates of alcohol septal ablation (ASA). The aim of this study was to evaluate the safety and efficacy of ASA according to institutional experience with the procedure. METHODS: We retrospectively evaluated 1310 patients with symptomatic obstructive hypertrophic cardiomyopathy who underwent ASA and were divided into 2 groups. The first-50 group consisted of the first consecutive 50 patients treated at each centre, and the over-50 group consisted of patients treated thereafter (patients 51 and above). RESULTS: In the 30-day follow-up, there was a significant difference in the occurrence of major cardiovascular adverse events (21% in the first-50 group vs 12% in the over-50 group; P < 0.01), which was driven by the occurrence of cardiovascular deaths (2.1% vs 0.4%; P = 0.01) and implanted pacemakers (15% vs 9%; P < 0.01). In the long-term follow-up (5.5 ± 4.1 years), the first-50 group was associated with a significantly higher occurrence of major adverse events (P < 0.01) and higher cardiovascular mortality (P < 0.01). Also, patients in the first-50 group were more likely to self-report dyspnea of New York Heart Association class III/IV (16% vs 10%), to have a left ventricular outflow gradient > 30 mm Hg (16% vs 10%) at the last clinical check-up (P < 0.01 for both), and a probability of repeated septal reduction therapy (P = 0.03). CONCLUSIONS: An institutional experience of > 50 ASA procedures was associated with a lower occurrence of ASA complications, better cardiovascular survival, better hemodynamic and clinical effect, and less need for repeated septal reduction therapy.

13.
Circ Res ; 121(7): 771-783, 2017 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-28912182

RESUMO

Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with ß-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated.


Assuntos
Técnicas de Ablação , Procedimentos Cirúrgicos Cardíacos/métodos , Cardiomiopatia Hipertrófica/cirurgia , Septos Cardíacos/cirurgia , Miocárdio/patologia , Técnicas de Ablação/efeitos adversos , Técnicas de Imagem Cardíaca , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/patologia , Eletrocardiografia , Predisposição Genética para Doença , Septos Cardíacos/patologia , Septos Cardíacos/fisiopatologia , Hemodinâmica , Humanos , Seleção de Pacientes , Fenótipo , Recuperação de Função Fisiológica , Fatores de Risco , Resultado do Tratamento , Função Ventricular
14.
JACC Cardiovasc Interv ; 10(11): 1134-1143, 2017 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-28595881

RESUMO

OBJECTIVES: The aim of this study was to describe the safety and outcomes of alcohol septal ablation (ASA) in younger patients with obstructive hypertrophic cardiomyopathy. BACKGROUND: The American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for older patients and patients with serious comorbidities. Data on long-term age-specific outcomes after ASA are scarce. METHODS: A total of 1,197 patients (mean age 58 ± 14 years) underwent ASA for obstructive hypertrophic cardiomyopathy. Patients were divided into young (≤50 years), middle-age (51 to 64 years), and older (≥65 years) groups. RESULTS: Thirty-day mortality and pacemaker implantation rates were lower in young compared with older patients (0.3% vs. 2% [p = 0.03] and 8% vs. 16% [p < 0.001], respectively). Ninety-five percent of young patients were in New York Heart Association functional class I or II at last follow-up. During a mean follow-up period of 5.4 ± 4.2 years, 165 patients (14%) died. Annual mortality rates of young, middle-age, and older patients were 1%, 2%, and 5%, respectively (p < 0.01). Annual adverse arrhythmic event rates were similar in the 3 age groups at about 1% (p = 0.90). Independent predictors of mortality in young patients were age, female sex, and residual left ventricular outflow tract gradient. Additionally, young patients treated with ≥2.5 ml alcohol had a higher all-cause mortality rate (0.6% vs. 1.4% per year in patients treated with <2.5 ml, p = 0.03). CONCLUSIONS: ASA in younger patients with obstructive hypertrophic cardiomyopathy was safe and effective for relief of symptoms at long-term follow-up. The authors propose that the indication for ASA can be broadened to younger patients.


Assuntos
Cardiomiopatia Hipertrófica/cirurgia , Etanol/uso terapêutico , Septos Cardíacos/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Técnicas de Ablação/efeitos adversos , Técnicas de Ablação/mortalidade , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Estimulação Cardíaca Artificial , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/mortalidade , Intervalo Livre de Doença , Etanol/efeitos adversos , Europa (Continente) , Feminino , Septos Cardíacos/diagnóstico por imagem , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Marca-Passo Artificial , Seleção de Pacientes , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/mortalidade , Adulto Jovem
15.
J Am Heart Assoc ; 6(5)2017 May 16.
Artigo em Inglês | MEDLINE | ID: mdl-28512112

RESUMO

BACKGROUND: The long-term efficacy and safety of alcohol septal ablation (ASA) in patients with highly symptomatic hypertrophic obstructive cardiomyopathy has been demonstrated. The aim of this study was to evaluate the long-term outcomes of mildly symptomatic patients with hypertrophic obstructive cardiomyopathy treated with ASA. METHODS AND RESULTS: We retrospectively evaluated consecutive patients enrolled in the Euro-ASA registry (1427 patients) and identified 161 patients (53±13 years; 27% women) who were mildly symptomatic (New York Heart Association [NYHA] class II) pre-ASA. The median (interquartile range) follow-up was 4.8 (1.7-8.5) years. The clinical outcome was assessed and compared with the age- and sex-matched general population. The 30-day mortality after ASA was 0.6% and the annual all-cause mortality rate was 1.7%, which was similar to the age- and sex-matched general population (P=0.62). A total of 141 (88%) patients had resting left ventricular outflow tract gradient at the last clinical checkup ≤30 mm Hg. Obstruction was reduced from 63±32 to 15±19 mm Hg (P<0.01), and the mean NYHA class decreased from 2.0±0 to 1.3±0.1 (P<0.01); 69%, 29%, and 2% of patients were in NYHA class I, II, and III at the last clinical checkup, respectively. CONCLUSIONS: Mildly symptomatic hypertrophic obstructive cardiomyopathy patients treated with ASA had sustained symptomatic and hemodynamic relief with a low risk of developing severe heart failure. Their survival is comparable to the general population.


Assuntos
Técnicas de Ablação , Cardiomiopatia Hipertrófica/cirurgia , Etanol/administração & dosagem , Obstrução do Fluxo Ventricular Externo/cirurgia , Técnicas de Ablação/efeitos adversos , Técnicas de Ablação/mortalidade , Adulto , Idoso , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/mortalidade , Cardiomiopatia Hipertrófica/fisiopatologia , Etanol/efeitos adversos , Europa (Continente) , Feminino , Humanos , Estimativa de Kaplan-Meier , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Recuperação de Função Fisiológica , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Função Ventricular Esquerda , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/mortalidade , Obstrução do Fluxo Ventricular Externo/fisiopatologia
16.
Dtsch Med Wochenschr ; 142(4): 276-281, 2017 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-28235228

RESUMO

Since the first publication in 1995 percutaneous septal ablation (acronyms in alphabetical order: ASA, ESA, NSMR, PTSMA, TASH, among others) has made its way from a small case series into the international guidelines for patients with HOCM and symptoms not responding to medical treatment. We now report on the long-term course (20 years) in the first 6 cases who were successfully treated in our institution in early 1996, published in this Journal shortly thereafter.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/cirurgia , Ablação por Cateter/métodos , Septos Cardíacos/cirurgia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
17.
Clin Res Cardiol ; 105(11): 953-961, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27270758

RESUMO

INTRODUCTION: Percutaneous alcohol septal ablation (PTSMA) is an established treatment for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). However, there is concern of a higher risk for ventricular tachyarrhythmias and sudden death due to the myocardial scar created after PTSMA. We investigated the possibility of increased ventricular arrhythmias and risk of sudden death after PTSMA in a subgroup of patients with an already implanted ICD. METHODS AND RESULTS: Between 2009 and 2012, 239 PTSMAs were performed in 212 patients with HOCM. In 32 of those an ICD had already been implanted before PTSMA for primary (31 patients) or secondary (1 patient) prevention of sudden death. The maximum left ventricular outflow tract gradient (LVOTG) was reduced from 114 ± 39 mmHg before PTSMA to 23 ± 19 mmHg (P < 0.0001). Among clinical risk factors for sudden death, nonsustained ventricular tachycardia (VT), syncope and family history for sudden death were most common. After a median follow-up of 5.3 (IQR 4.3-5.7) years after PTSMA only one patient had ICD shocks (annual ICD discharge 0.6 %). In another 3 patients, with already documented nonsustained VTs as risk factor before ICD implantation, VT episodes that activated antitachycardic pacing were recorded. The annual appropriate ICD intervention including all events was 2.5 % and involved only patients with a very high estimated 5-year sudden death risk before PTSMA (>14.3 %). CONCLUSIONS: In a selected high-risk patient cohort with HOCM ominous arrhythmic events seem to be rare and predominantly occur in patients with a very high estimated risk of sudden death before PTSMA.


Assuntos
Técnicas de Ablação/efeitos adversos , Cardiomiopatia Hipertrófica/cirurgia , Etanol/efeitos adversos , Taquicardia Ventricular/etiologia , Obstrução do Fluxo Ventricular Externo/cirurgia , Adolescente , Adulto , Idoso , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/mortalidade , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Etanol/administração & dosagem , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidade , Taquicardia Ventricular/terapia , Fatores de Tempo , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/mortalidade , Adulto Jovem
18.
Eur Heart J ; 37(19): 1517-23, 2016 05 14.
Artigo em Inglês | MEDLINE | ID: mdl-26746632

RESUMO

AIMS: The first cases of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) were published two decades ago. Although the outcomes of single-centre and national ASA registries have been published, the long-term survival and clinical outcome of the procedure are still debated. METHODS AND RESULTS: We report long-term outcomes from the as yet largest multinational ASA registry (the Euro-ASA registry). A total of 1275 (58 ± 14 years, median follow-up 5.7 years) highly symptomatic patients treated with ASA were included. The 30-day post-ASA mortality was 1%. Overall, 171 (13%) patients died during follow-up, corresponding to a post-ASA all-cause mortality rate of 2.42 deaths per 100 patient-years. Survival rates at 1, 5, and 10 years after ASA were 98% (95% CI 96-98%), 89% (95% CI 87-91%), and 77% (95% CI 73-80%), respectively. In multivariable analysis, independent predictors of all-cause mortality were age at ASA (P < 0.01), septum thickness before ASA (P < 0.01), NYHA class before ASA (P = 0.047), and the left ventricular (LV) outflow tract gradient at the last clinical check-up (P = 0.048). Alcohol septal ablation reduced the LV outflow tract gradient from 67 ± 36 to 16 ± 21 mmHg (P < 0.01) and NYHA class from 2.9 ± 0.5 to 1.6 ± 0.7 (P < 0.01). At the last check-up, 89% of patients reported dyspnoea of NYHA class ≤2, which was independently associated with LV outflow tract gradient (P < 0.01). CONCLUSIONS: The Euro-ASA registry demonstrated low peri-procedural and long-term mortality after ASA. This intervention provided durable relief of symptoms and a reduction of LV outflow tract obstruction in selected and highly symptomatic patients with obstructive HCM. As the post-procedural obstruction seems to be associated with both worse functional status and prognosis, optimal therapy should be focused on the elimination of LV outflow tract gradient.


Assuntos
Cardiomiopatia Hipertrófica/terapia , Etanol/uso terapêutico , Solventes/uso terapêutico , Técnicas de Ablação/métodos , Técnicas de Ablação/mortalidade , Cardiomiopatia Hipertrófica/mortalidade , Intervalo Livre de Doença , Feminino , Septos Cardíacos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Resultado do Tratamento
19.
Curr Cardiol Rev ; 12(4): 285-296, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-25563291

RESUMO

Hypertrophic obstructive cardiomyopathy is the most common genetic cardiac disease and is generally characterised by asymmetric septal hypertrophy and intraventricular obstruction. Patients with severe obstruction and significant symptoms that persist despite optimal medical treatment are candidates for an invasive septal reduction therapy. Twenty years after its introduction, percutaneous transluminal alcohol septal ablation has been increasingly preferred for septal reduction in patients with drug refractory hypertrophic obstructive cardiomyopathy. Myocardial contrast echocardiography and injection of reduced alcohol volumes have increased safety, while efficacy is comparable to the surgical alternative, septal myectomy, which has for decades been regarded as the 'gold standard' treatment. Data on medium- and long-term survival show improved prognosis with survival being similar to the general population. Current guidelines have supported its use by experienced operators in centres specialised in the treatment of patients with hypertrophic obstructive cardiomyopathy.


Assuntos
Cateterismo Cardíaco/métodos , Cardiomiopatia Hipertrófica/terapia , Etanol/uso terapêutico , Septos Cardíacos , Fatores Etários , Cateterismo Cardíaco/instrumentação , Procedimentos Cirúrgicos Cardíacos , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia , Humanos , Seleção de Pacientes , Prognóstico , Fatores de Tempo
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