Gadolinium-enhanced cardiovascular magnetic resonance and exercise capacity in hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Using gadolinium-enhanced cardiovascular magnetic resonance, it is possible to evaluate the presence of myocardial fibrosis in hypertrophic cardiomyopathy. Classical disease markers are weak predictors of functional disability in affected patients. Our objective was to study the relationship between the degree of myocardial fibrosis observed by cardiac magnetic resonance and exercise capacity. METHODS: We performed cardiac magnetic resonance, echocardiography, exercise testing and Holter monitoring, along with the usual clinical assessments, in 98 patients (age, 46.3+/-15.4 years, 71.4% male) referred from two specialist hypertrophic cardiomyopathy clinics. Cardiac magnetic resonance assessment included quantifying the degree of fibrosis (i.e., the percentage of the myocardium showing enhancement) 10 min after gadolinium infusion. Symptom-limited exercise testing was used to determine exercise capacity (in metabolic equivalent [MET] units). In 71 patients, the basal N-terminal probrain natriuretic peptide (NT-proBNP) level was also measured. RESULTS: Late enhancement was observed on cardiac magnetic resonance in 67 (68.4%) patients. These patients had a lower exercise capacity (8.04+/-3.56 MET vs. 10.41+/-3.57 MET; P=.003). There was an inverse correlation between the percentage of fibrosis and exercise capacity (r=-0.21; P=.044). The best predictor of exercise capacity was the logarithm of the NT-proBNP level (r=-0.5; P< .0001). Multivariate analysis confirmed that age, a history of atrial fibrillation, the basal NT-proBNP level and the presence of fibrosis were independent predictors of exercise capacity (r2 for the model=0.47). CONCLUSIONS: The observation of areas of late gadolinium enhancement on cardiac magnetic resonance was independently associated with poor exercise capacity in patients with hypertrophic cardiomyopathy.

Cardiorresonancia magnética con gadolinio y capacidad de esfuerzo en la miocardiopatía hipertrófica / Gadolinium-Enhanced Cardiovascular Magnetic Resonance and Exercise Capacity in Hypertrophic Cardiomyopathy

Introducción y objetivos. La cardiorresonancia magnéticacon gadolinio permite estudiar la fibrosis miocárdicaen la miocardiopatía hipertrófica. Los marcadores clásicos de la enfermedad predicen débilmente la limitación funcional de estos pacientes. Nuestro objetivo es estudiar la relación entre la fibrosis en la cardiorresonancia magnética y la capacidad de ejercicio.Métodos. Se realizó cardiorresonancia magnética, ecocardiografía, ergometría y Holter, junto con la valoración clínica habitual, a 98 pacientes procedentes de dos consultas especializadas de miocardiopatía hipertrófica (edad, 46,3 ± 15,4 años; el 71,4% varones). El estudio de cardiorresonancia magnética incluyó cuantificación de fibrosis (porcentaje de miocardio realzado) 10 min tras la inyección de gadolinio. Mediante ergometría limitada por síntomas, se determinó la capacidad de esfuerzo (MET). En 71 pacientes se obtuvieron además concentraciones plasmáticas basales de NT-proBNP.Resultados. Presentaron áreas de realce tardío en lacardiorresonancia magnética 67 (68,4%) pacientes. Estospacientes tenían peor capacidad de esfuerzo (8,04 ± 3,56frente a 10,41 ± 3,57 MET; p = 0,003). Se observó correlación negativa entre el porcentaje de fibrosis y los MET alcanzados (r = –0,21; p = 0,044). El mejor predictor de capacidad de esfuerzo fue el logNT-proBNP (r = –0,5; p <0,0001). El análisis multivariado confirmó que la edad, los antecedentes de fibrilación auricular, las concentraciones basales de NT-proBNP y la fibrosis son predictores independientes de la capacidad de esfuerzo (r2 del modelo = 0,47).Conclusiones. La presencia de áreas de realce tardíocon gadolinio en la cardiorresonancia magnética se asociade forma independiente con una peor capacidad deesfuerzo en pacientes con miocardiopatía hipertrófica

Introduction and objectives. Using gadoliniumenhancedcardiovascular magnetic resonance, it ispossible to evaluate the presence of myocardial fibrosis in hypertrophic cardiomyopathy. Classical disease markersare weak predictors of functional disability in affectedpatients. Our objective was to study the relationshipbetween the degree of myocardial fibrosis observed bycardiac magnetic resonance and exercise capacity.Methods. We performed cardiac magnetic resonance,echocardiography, exercise testing and Holter monitoring,along with the usual clinical assessments, in 98 patients(age, 46.3±15.4 years, 71.4% male) referred from twospecialist hypertrophic cardiomyopathy clinics. Cardiacmagnetic resonance assessment included quantifying thedegree of fibrosis (i.e., the percentage of the myocardium showing enhancement) 10 min after gadolinium infusion. Symptom-limited exercise testing was used to determine exercise capacity (in metabolic equivalent [MET] units). In 71 patients, the basal N-terminal probrain natriuretic peptide (NT-proBNP) level was also measured.Results. Late enhancement was observed on cardiacmagnetic resonance in 67 (68.4%) patients. Thesepatients had a lower exercise capacity (8.04±3.56 METvs. 10.41±3.57 MET; P=.003). There was an inversecorrelation between the percentage of fibrosis andexercise capacity (r=–0.21; P=.044). The best predictor of exercise capacity was the logarithm of the NT-proBNPlevel (r=–0.5; P<.0001). Multivariate analysis confirmedthat age, a history of atrial fibrillation, the basalNT-proBNP level and the presence of fibrosis wereindependent predictors of exercise capacity (r2 for themodel=0.47).Conclusions. The observation of areas of lategadolinium enhancement on cardiac magnetic resonancewas independently associated with poor exercise capacityin patients with hypertrophic cardiomyopathy

Variables associated with contrast-enhanced cardiovascular magnetic resonance in hypertrophic cardiomyopathy: clinical implications.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) shows increased myocardial collagen and disarray. Late gadolinium enhancement in cardiovascular magnetic resonance (CMR) is observed in regions of increased myocardial collagen. The extent of late gadolinium enhancement has been associated with higher prevalence of risk factors of sudden death. The aim of the present study was to describe the clinical characteristics and the presence of risk factors for sudden death in a series of patients from 2 referral centers for HCM in relation to late gadolinium enhancement in CMR. METHODS AND RESULTS: A total of 120 patients (47 +/- 16 years) were included. All patients fulfilled conventional criteria for HCM. A complete history and clinical examination were performed. Risk factors for sudden death were evaluated. A blinded CMR was performed with late gadolinium enhancement in the left ventricular short-axis orientation. NT pro B-type natriuretic protein (BNP) and C-reactive protein were determined in serum samples. A total of 83 patients (69%) showed late gadolinium enhancement. These patients had higher maximal left ventricular wall thickness (22 +/- 5 versus 17 +/- 3 mm, P < .001), showed more frequently obstruction (42% versus 16%, P = .006), nonsustained ventricular tachycardia (38% versus 8%, P = .001), worse exercise capacity (8 +/- 4 versus 10 +/- 4 METs, P = .003) and increased levels of NT BNP (656 [300-1948] versus 290 [122-948] pg/mL, P = .020). On multivariate analysis, maximal left ventricular wall thickness (P < .001) and nonsustained ventricular tachycardia (P = .011) remained associated with gadolinium-enhanced imaging. Number of risk factors for sudden death was associated with late gadolinium enhancement (OR 2.18, 95%CI 1.45-3.20, P < .001). CONCLUSIONS: Late gadolinium enhancement in CMR is a common finding in HCM. Increased maximal left ventricular wall thickness and nonsustained ventricular tachycardia are associated with late gadolinium enhancement. Associations with risk factors for sudden death and functional status are observed.

Prevalence of fabry disease in a cohort of 508 unrelated patients with hypertrophic cardiomyopathy.

OBJECTIVES: We aimed to study the prevalence of Fabry disease (FD) in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: There are limited and controversial data about the prevalence of FD in patients with HCM. METHODS: We screened the plasma alpha-galactosidase A activity from 508 unrelated patients with HCM (328 men, 180 women, ages 58 +/- 16 years). Patients with low activity (0% to 30% of the normal control in men, and 0% to 50% in women) underwent genetic study of the GLA gene. RESULTS: We found low plasma activity in 15 patients (3%). Three men had GLA mutations (0.9%): S238N (novel) in 2 and E358del (described) in 1. Two women had described mutations (1.1%): L89P and A143T. Three unrelated men had the D313Y variant previously associated with enzyme pseudo-deficiency. Two women had polymorphisms that did not segregate with the disease in their families. Five women (activity 39% to 47%) had no sequence variants. The familial studies allowed the diagnosis of 14 carriers: 6 women without Fabry manifestations, 3 women with cardiomyopathy, 2 men with renal and cardiac disease, 1 man with microhematuria, 1 woman with first-degree atrioventricular block, and a 32-year-old woman with only renal disease. CONCLUSIONS: By means of a screening based on genotyping of patients with low plasma enzymatic activity, the prevalence of FD in our population of HCM is 1% (0.9% in men and 1.1% in women). This diagnosis is relevant, because it allows the identification of disease carriers that might benefit from enzyme replacement therapy.

[The implantable cardioverter-defibrillator and hypertrophic cardiomyopathy. Experience at three centers]. / Desfibrilador automático en la miocardiopatía hipertrófica. Experiencia de 3 centros.

INTRODUCTION AND OBJECTIVES: Although implantable cardioverter-defibrillators (ICDs) are recommended for high-risk patients with hypertrophic cardiomyopathy (HCM), there is no agreement on their general use. Moreover, little information is available on ICD use in this setting in Spain. Our aims were to describe the characteristics of HCM patients who received ICDs at three hospitals in Spain, and to study indications for device implantation and the results of follow-up in device users. METHODS: We evaluated risk factors for sudden death in HCM patients with ICDs, including family history of sudden death, recurrent syncope, maximum wall thickness > or =30 mm, left ventricular outflow pressure gradient >30 mmHg, abnormal blood pressure response to exercise, and nonsustained ventricular tachycardia. During regular follow-up, appropriate and inappropriate administration of ICD therapy was recorded. RESULTS: Of 726 HCM patients, 45 (6.2%) had an ICD (mean age 43 [20] years). The proportion of patients with ICDs at the three centers studied was highly variable despite patients' clinical characteristics being similar. The indication for implantation was primary prevention in 27 patients and secondary prevention in 18. During follow-up (median 32 months), ICD therapy was administered appropriately in 10 (22.0%) patients (in nine, as secondary prevention and, in one, as primary prevention). The annual appropriate ICD therapy rate was 11.1% for secondary prevention and 1.6% for primary prevention. Two patients received an ICD to treat ventricular fibrillation and eight, to treat sustained ventricular tachycardia. The only significant predictor of appropriate ICD therapy was a history of sustained ventricular tachycardia or ventricular fibrillation (hazard ratio =13.3, P=.014). CONCLUSIONS: The percentage of HCM patients undergoing ICD implantation at Spanish hospitals was highly variable, possibly due to different selection criteria. When used as secondary prevention, ICD therapy was administered appropriately in a high proportion of cases (50% in 3 years).

Desfibrilador automático en la miocardiopatía hipertrófica. Experiencia de 3 centros / The implantable cardioverter-defibrillator and hypertrophic cardiomyopathy. Experience at three centers

Introducción y objetivos. El desfibrilador automático implantable (DAI) es el tratamiento recomendado en la miocardiopatía hipertrófica (MCH) de alto riesgo, aunque no hay acuerdo en sus indicaciones. Hay pocos datos sobre su utilización en nuestro país. El objetivo es describir las características de los pacientes con MCH a los que se les implantó un DAI y analizar los resultados de esta terapia. Métodos. Se analizaron los factores de riesgo de muerte súbita en los pacientes portadores de DAI de 3 centros con consultas dedicadas a la MCH (antecedentes personales y familiares de muerte súbita, síncope recurrente, grosor ≥ 30 mm y gradiente subaórtico > 30 mmHg, respuesta anormal de la presión al esfuerzo y taquicardia ventricular no sostenida) y la indicación del implante. Se realizó un seguimiento periódico y se registraron las terapias adecuadas e inadecuadas. Resultados. De 726 pacientes, 45 (6,2%) eran portadores de DAI (edad de 43 ± 20 años). La proporción de pacientes con DAI en los 3 centros fue muy variable, a pesar de que las características de los pacientes eran similares. La indicación fue prevención primaria en 27 pacientes y secundaria en 18. Con un seguimiento de 32 meses, 10 pacientes (22%) recibieron tratamiento adecuado (9 de prevención secundaria y uno de prevención primaria). La tasa anual de tratamientos adecuados fue del 11,1% en prevención secundaria y del 1,6% en prevención primaria. El único factor asociado con el tratamiento adecuado fue el antecedente de taquicardia ventricular sostenida o fibrilación ventricular (riesgo relativo [RR] = 13,3; p = 0,014). Conclusiones. En consultas dedicadas a la MCH, el porcentaje de pacientes portadores de DAI varía en función del grado de selección de la población de origen. La incidencia de terapias adecuadas es elevada en prevención secundaria (el 50% en 3 años)

Introduction and objectives. Although implantable cardioverter-defibrillators (ICDs) are recommended for high-risk patients with hypertrophic cardiomyopathy (HCM), there is no agreement on their general use. Moreover, little information is available on ICD use in this setting in Spain. Our aims were to describe the characteristics of HCM patients who received ICDs at three hospitals in Spain, and to study indications for device implantation and the results of follow-up in device users. Methods. We evaluated risk factors for sudden death in HCM patients with ICDs, including family history of sudden death, recurrent syncope, maximum wall thickness ≥30 mm, left ventricular outflow pressure gradient >30 mmHg, abnormal blood pressure response to exercise, and nonsustained ventricular tachycardia. During regular follow-up, appropriate and inappropriate administration of ICD therapy was recorded. Results. Of 726 HCM patients, 45 (6.2%) had an ICD (mean age 43 [20] years). The proportion of patients with ICDs at the three centers studied was highly variable despite patients' clinical characteristics being similar. The indication for implantation was primary prevention in 27 patients and secondary prevention in 18. During follow-up (median 32 months), ICD therapy was administered appropriately in 10 (22.0%) patients (in nine, as secondary prevention and, in one, as primary prevention). The annual appropriate ICD therapy rate was 11.1% for secondary prevention and 1.6% for primary prevention. Two patients received an ICD to treat ventricular fibrillation and eight, to treat sustained ventricular tachycardia. The only significant predictor of appropriate ICD therapy was a history of sustained ventricular tachycardia or ventricular fibrillation (hazard ratio =13.3, P=.014). Conclusions. The percentage of HCM patients undergoing ICD implantation at Spanish hospitals was highly variable, possibly due to different selection criteria. When used as secondary prevention, ICD therapy was administered appropriately in a high proportion of cases (50% in 3 years)

[Reinfarction as a complication of acute myocardial infarction. PRIMVAC Registry data]. / El reinfarto como complicación del infarto agudo de miocardio. Datos del registro PRIMVAC.

INTRODUCTION AND OBJECTIVES: The clinical profile of patients with acute myocardial infarction (AMI) who have reinfarction (REAMI) during their stay in the intensive cardiologic care unit (ICCU) is not well known. The aim of this study was to identify factors predictive of REAMI, as well as its global incidence and mortality. PATIENTS AND METHOD: All patients with AMI admitted to the ICCU of 17 hospitals in the Comunidad de Valencia (Spain) in the period 1995-2000 (PRIMVAC Registry) were included. Differential characteristics between patients with or without REAMI were determined, and odds ratios (OR) for possible predictive factors were estimated with their 95% confidence intervals by logistic regression. RESULTS: A total of 12,071 patients were included. Mean age of the patients was of 65.5 years, the percentage of women was 23.8%, and the incidence of REAMI was 2.8%. The REAMI group was significantly older than the non-REAMI group. Female sex was significantly more common in the REAMI group. More diagnostic and therapeutic procedures were carried out, more drugs were used and there were more complications in the REAMI group. Mortality was significantly higher in the REAMI group (37.8% vs 12.6%). Only age, diabetes mellitus, previous myocardial infarction and the appearance of Q waves in the electrocardiogram were independently associated with the presence of REAMI. CONCLUSIONS: REAMI in the ICCU was associated with high mortality. Some clinical factors present during the first few hours after AMI were associated independently with the appearance of REAMI.

El reinfarto como complicación del infarto agudo de miocardio. Datos del registro PRIMVAC / Reinfarction as a complication of acute myocardial infarction. PRIMVAC registry data

Introducción y objetivos. El perfil clínico de los pacientes con infarto agudo de miocardio (IAM) que presentan un reinfarto (REIAM) durante su estancia en la unidad de cuidados intensivos cardiológicos (UCIC) es poco conocido. El objetivo de este trabajo es determinar los factores predictores de REIAM, su incidencia global y su mortalidad. Pacientes y método. Se incluyó a todos los pacientes con IAM ingresados en las UCIC de 17 hospitales de la Comunidad Valenciana en el período 1995-2000. Se determinaron las características diferenciales de los pacientes con y sin REIAM, y se calcularon las odds ratio y sus intervalos de confianza del 95% mediante un análisis de regresión logística para los posibles factores predictores. Resultados. Se incluyó a 12.071 pacientes con IAM. La edad media fue de 65,5 años, la proporción de mujeres del 23,8% y la incidencia de REIAM del 2,8%. La edad fue significativamente mayor en el grupo con REIAM que en el grupo sin REIAM, al igual que el porcentaje de mujeres. Se realizaron más procedimientos, se utilizaron más fármacos y hubo más complicaciones en el grupo con REIAM. La mortalidad fue significativamente mayor en el grupo con REIAM (37,8 frente a 12,6%). La edad, la diabetes mellitus, el infarto de miocardio previo y el desarrollo de onda Q en el electrocardiograma se asociaron de forma independiente con la presencia de REIAM. Conclusiones. El REIAM en la UCIC conlleva una alta mortalidad. Algunos factores presentes en las primeras horas del IAM se asocian con la aparición de REIAM

Introduction and objectives. The clinical profile of patients with acute myocardial infarction (AMI) who have reinfarction (REAMI) during their stay in the intensive cardiologic care unit (ICCU) is not well known. The aim of this study was to identify factors predictive of REAMI, as well as its global incidence and mortality. Patients and method. All patients with AMI admitted to the ICCU of 17 hospitals in the Comunidad de Valencia (Spain) in the period 1995-2000 (PRIMVAC Registry) were included. Differential characteristics between patients with or without REAMI were determined, and odds ratios (OR) for possible predictive factors were estimated with their 95% confidence intervals by logistic regression. Results. A total of 12 071 patients were included. Mean age of the patients was of 65.5 years, the percentage of women was 23.8%, and the incidence of REAMI was 2.8%. The REAMI group was significantly older than the non-REAMI group. Female sex was significantly more common in the REAMI group. More diagnostic and therapeutic procedures were carried out, more drugs were used and there were more complications in the REAMI group. Mortality was significantly higher in the REAMI group (37.8% vs 12.6%). Only age, diabetes mellitus, previous myocardial infarction and the appearance of Q waves in the electrocardiogram were independently associated with the presence of REAMI. Conclusions. REAMI in the ICCU was associated with high mortality. Some clinical factors present during the first few hours after AMI were associated independently with the appearance of REAMI

[Mortality of myocardial infarction in the PRIMVAC registry. Prognostic factors]. / Mortalidad del infarto de miocardio en el registro PRIMVAC. Factores pronósticos.

BACKGROUND AND OBJECTIVE: The aim of this study was to determine the mortality due to acute myocardial infarction in the coronary units from Comunidad Valenciana (Spain) and the prognostic factors associated with a higher mortality. PATIENTS AND METHOD: Demographic characteristics, coronary risk factors, electrocardiographic ischemic signs, complications and mortality of patients with acute myocardial infarction admitted in the coronary units were collected. The study period comprised January 1995-December 1999. Death incidence was measured during coronary unit's stay. Factors associated with poor prognosis were analyzed. RESULTS: 10.213 patients entered into the study. Mean age at admission was 65 12 years. 23.8% were females (76.2% males). Global mortality in coronary units was 13.3%. Independent variables associated with higher mortality were (p < 0.05): advanced age (OR=1.06 [1.05-1.06]), female sex (OR=1.45 [1.26-1.66]), diabetes mellitus (OR=1.53 [1.35-1.74]), previous myocardial infarction (OR=1.46 [1.23-1.70]), previous angor pectoris (OR=1.29 [1.13-1.49]) and Q-wave infarction (OR=1.23 [1.03-1.43]). Factors associated with lower mortality were: hypercholesterolemia (OR=0.76 [0.66-0.78]), smoking (OR=0.65 [0.57-0.74]) and thrombolysis (OR=0.85 [0.78-0.92]). CONCLUSIONS: At present, in the reperfusion therapy era, acute myocardial infarction has a high mortality after coronary unit admission. Several clinical factors are associated with a worse prognosis.