Prevalence and pattern of myocardial involvement on cardiac magnetic resonance imaging in Takayasu arteritis and its relationship with disease activity / Prevalencia y patrón de afectación miocárdica en la resonancia magnética cardíaca en la arteritis de Takayasu y su relación con la actividad de la enfermedad

Background: Cardiac involvement in Takayasu arteritis (TA) is the major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) is an excellent modality for the assessment of myocardial involvement. Studies have shown myocardial involvement in 25%–27% of patients. Objectives: To evaluate the prevalence and pattern of myocardial involvement in TA on CMR. We also evaluated any correlation between CMR changes and disease activity score (ITAS 2010 and ITAS-A) assessed at the time of CMR. Methods: Patients classified as Takayasu arteritis according to Sharma et al. criteria were enrolled in the study. Demographic, clinical, and laboratory data were documented in the predesigned proforma. CMR was performed on a dedicated cardiac 3Tesla MR machine. Disease activity was recorded by ITAS2010 and ITAS-A. Results: A total of 37 TA patients were included. Mean (±SD) age was 29±11 years. Female to male ratio was 3:1. Five patients (14%) had myocardial involvement on CMR. Two (2/5) had myocarditis and three (3/5) patients had features of ischaemic myocardial fibrosis. Conclusion: The myocardium is affected in TA, however the prevalence of subclinical myocardial involvement in our study was less (8% vs. 25%–27%) compared to the previous studies. Myocardial involvement trends towards early age of onset, less disease duration, lack of classical risk factors, and more with disease activity.(AU)

Antecedentes: La afectación cardíaca en la arteritis de Takayasu (AT) es la principal causa de morbmortalidad. La resonancia magnética cardiovascular (RMC) es una modalidad excelente para la evaluación de la afectación miocárdica. Los estudios han demostrado afectación del miocardio en el 25-27% de los pacientes. Objetivos: Evaluar la prevalencia y patrón de afectación miocárdica en AT en RMC. También se evaluó cualquier correlación entre los cambios de RMC y la puntuación de actividad de la enfermedad (ITAS 2010 e ITAS-A) evaluada en el momento de la RMC. Métodos: Pacientes clasificados como arteritis de Takayasu según los criterios de Sharma et al. se inscribieron en el estudio. Los datos demográficos, clínicos y de laboratorio se documentaron en el formulario prediseñado. La RMC se realizó en una máquina de RM cardíaca de 3 Tesla dedicada. La actividad de la enfermedad fue registrada por ITAS 2010 e ITAS-A. Resultados: Se incluyeron un total de 37 pacientes con AT. La edad media (±DE) fue de 29±11 años. La proporción de mujeres a hombres fue de 3:1. Cinco pacientes (14%) tenían afectación miocárdica en la RMC. Dos (2/5) tenían miocarditis y 3 (3/5) pacientes tenían características de fibrosis miocárdica isquémica. Conclusión: El miocardio es afectado en la AT, sin embargo, la prevalencia de afectación miocárdica subclínica en nuestro estudio fue menor (8% vs. 25-27%) en comparación con los estudios previos. La afectación miocárdica tiende hacia una edad de inicio temprana, menor duración de la enfermedad, falta de factores de riesgo clásicos y más con la actividad de la enfermedad.(AU)

Prevalence and pattern of myocardial involvement on cardiac magnetic resonance imaging in Takayasu arteritis and its relationship with disease activity.

BACKGROUND: Cardiac involvement in Takayasu arteritis (TA) is the major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) is an excellent modality for the assessment of myocardial involvement. Studies have shown myocardial involvement in 25%-27% of patients. OBJECTIVES: To evaluate the prevalence and pattern of myocardial involvement in TA on CMR. We also evaluated any correlation between CMR changes and disease activity score (ITAS 2010 and ITAS-A) assessed at the time of CMR. METHODS: Patients classified as Takayasu arteritis according to Sharma et al. criteria were enrolled in the study. Demographic, clinical, and laboratory data were documented in the predesigned proforma. CMR was performed on a dedicated cardiac 3Tesla MR machine. Disease activity was recorded by ITAS2010 and ITAS-A. RESULTS: A total of 37 TA patients were included. Mean (±SD) age was 29±11 years. Female to male ratio was 3:1. Five patients (14%) had myocardial involvement on CMR. Two (2/5) had myocarditis and three (3/5) patients had features of ischaemic myocardial fibrosis. CONCLUSION: The myocardium is affected in TA, however the prevalence of subclinical myocardial involvement in our study was less (8% vs. 25%-27%) compared to the previous studies. Myocardial involvement trends towards early age of onset, less disease duration, lack of classical risk factors, and more with disease activity.

Late gadolinium enhancement location assessed by magnetic resonance and arrhythmogenic risk in hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Late gadolinium enhancement (LGE) extent has emerged as a predictor of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM), however little is known about the arrhythmogenic relevance of its specific location in the left ventricle. Our aim was to analyze the influence of LGE location on the occurrence of ventricular arrhythmias (VA) and SCD in patients with HCM. METHODS: We performed a retrospective analysis of clinical and Holter records of HCM patients who underwent cardiac magnetic resonance at our center. LGE extent and distribution were assessed using the American Heart Association 17-segment model. VA was defined as non-sustained or sustained ventricular tachycardia, ventricular fibrillation or SCD. RESULTS: Sixty-one patients (age 57.0±16.7 years) were included and VA occurred in 24.6% (n=15). Patients with VA showed greater LGE extent than those without (7.40±5.3 vs. 3.52±3.0 segments, p=0.007). Analyzing the distribution of LGE, a set of arrhythmogenic segments (apex/basal inferior/basal anterolateral/mid inferoseptal) was found. The extent of LGE involvement in these segments was also greater in patients with VA (2.07±1.03 vs. 0.65±0.71 segments, p<0.001; area under the curve 0.861 for VA) and this difference remained significant after adjustment for potentially confounding variables. CONCLUSIONS: The extent of LGE involvement of a set of segments with an apparent relation to cardiac areas of increased mechanical stress was significantly and independently associated with the occurrence of VA, suggesting that not only the extent but also the location of LGE is important for the assessment of SCD risk in HCM patients.

Importance of cardiac magnetic resonance findings in the diagnosis of left dominant arrythmogenic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Left dominant arrhythmogenic cardiomyopathy (LDAC) has recently been recognized as falling on the spectrum of arrhythmogenic cardiomyopathy. It is characterized by fibroadipose replacement of the left ventricle. The aim of this study was to describe the most frequent forms of clinical presentation of LDAC, imaging findings, and events at follow-up, highlighting the importance of cardiac magnetic resonance (CMR). METHODS: Prospective registry of patients with findings compatible with LDAC. CMR image analysis and clinical follow-up was performed. The primary endpoint was the appearance of major adverse cardiovascular events (MACE) during follow-up, defined as sudden cardiac death, sustained ventricular arrhythmias, and heart transplant. RESULTS: We included 74 consecutive patients (mean age, 48.6 years; 50 men [67.6%]). The most frequent CMR indications were chest pain with normal coronary angiography, ventricular arrhythmias, and suspicion of cardiomyopathies. The main CMR findings were midwall and/or subepicardial pattern of late gadolinium enhancement (91.9%), fatty epicardial infiltration (83.8%), and left ventricle segmental contractility abnormalities (47.9%). At a mean follow-up of 3.74 years, 24 patients (32.4%) had a MACE (sudden cardiac death 8.1%, sustained ventricular arrhythmias 21.6%, and heart transplant 4.1%). Independent predictors for the appearance for MACE were a CMR study showing severe late gadolinium enhancement, male sex, and practicing sports. CONCLUSIONS: CMR is a key tool for diagnosing LDAC. Characteristic findings are subepicardial fatty infiltration and midwall-subepicardial late gadolinium enhancement. The prognosis of this population is poor with a high incidence of sudden cardiac death and ventricular arrhythmias.

Resonancia Magnética Cardíaca en el diagnóstico de miocarditis / Cardiac Magnetic Resonance in the diagnosis of myocarditis

El uso de la Resonancia Magnética Cardiaca (RMC), se ha convertido en los últimos años en una herramienta fundamental para el diagnóstico de diversas miocardiopatías, entre las cuales sobresale la miocarditis, entidad que cursa con manifestaciones y signos clínicos muy variables y poco específicos en la mayor parte de los casos, lo que la convierte en una patología difícil de reconocer sobre todo en sus episodios de presentación inicial, lo que probablemente conlleve a que sea subdiagnosticada. El desarrollo de nuevas modalidades de imagen ha permitido que ante la sospecha de esta entidad el uso de la RMC se convierta en la técnica ideal para el diagnóstico temprano. A continuación se presenta el caso clínico de un paciente masculino que acude con cuadro de dolor torácico agudo, enzimas cardiacas elevadas, electrocardiograma no concluyente y coronarias normales, mostrando el curso insidioso de esta patología así como de manera resumida el rol de la RMC en su diagnóstico y pronóstico.

The use of cardiac magnetic resonance (CMR) has become in recent years an essential tool for diagnosing various cardiomyopathies, among which stands myocarditis, an entity that presents with demonstrations and highly variable clinical signs and unspecific in most cases, which makes it a difficult disease to recognize especially in its initial filing episodes, which probably lead to be underdiagnosed. The development of new imaging modalities has allowed suspicion of this entity using the RMC becomes the ideal technique for early diagnosis. Next, the case of a male patient presenting with symptoms of acute chest pain, elevated cardiac enzymes inconclusive electrocardiogram and normal coronary showing insidious course of this disease and in summary the role of the RMC in its presented diagnosis and prognosis.

Resonancia magnética en el diagnóstico de amiloidosis cardíaca / Magnetic resonance imaging in the diagnosis of cardiac amyloidosis

La resonancia magnética cardíaca (RMC) es un método no invasivo que provee información acerca de la anatomía, función y caracterización tisular del miocardio, llegando a ser de gran utilidad en el diagnóstico y diferenciación de trastornos infiltrativos como la amiloidosis. La amiloidosis cardíaca (AC) es una miocardiopatía restrictiva resultado del depósito de amiloide en el corazón, que determina una semiología característica en la RM que permite establecer el diagnóstico en la mayoría de los casos. Los hallazgos por RMC incluyen hipertrofia miocárdica del ventrículo izquierdo (HVI) con realce tardío positivo en el ventrículo izquierdo (VI) y el resto de las cámaras cardíacas, asociado a alteración en la cinética del gadolinio con anulación del pool sanguíneo y hallazgos adicionales, como derrame pleural o pericárdico o ambos, que apoyan el diagnóstico1-3. Presentamos el caso de una paciente con diagnóstico de amiloidosis sometida a RMC en donde se demuestran los hallazgos característicos de esta patología. (AU)

Cardiac magnetic resonance imaging (MRI) is a noninvasive method of image that provides information about the anatomy, function and tissue characterization, becoming very useful in the diagnosis and differentiation of infiltrative disorders such as amyloidosis. Cardiac amyloidosis (CA) is a restrictive cardiomyopathy result of amyloid deposition in the heart. MRI findings include myocardial hypertrophy with positive late gadolinium enhancement (LGE) in the left ventricle (LV) associated with the altered kinetics of gadolinium and additional findings as pleural and pericardial effusion that support the diagnosis. We report the case of a patient diagnosed with amyloidosis showing the characteristic MR findings in this pathology. (AU)

Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance.

INTRODUCTION AND AIM: The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients. METHODS: From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed. RESULTS: Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality. CONCLUSIONS: The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events.

Clinical benefit of cardiac resynchronization therapy with a defibrillator in patients with an ejection fraction > 35% estimated by cardiac magnetic resonance.

INTRODUCTION AND OBJECTIVES: Cardiac resynchronization therapy with a defibrillator prolongs survival and improves quality of life in advanced heart failure. Traditionally, patients with ejection fraction > 35 estimated by echocardiography have been excluded. We assessed the prognostic impact of this therapy in a group of patients with severely depressed systolic function as assessed by echocardiography but with an ejection fraction > 35% as assessed by cardiac magnetic resonance. METHODS: We analyzed consecutive patients admitted for decompensated heart failure between 2004 and 2011. The patients were in functional class II-IV, with a QRS ≥ to 120 ms, ejection fraction ≤ 35% estimated by echocardiography, and a cardiac magnetic resonance study. We included all patients (n=103) who underwent device implantation for primary prevention. Ventricular arrhythmia, all-cause mortality and readmission for heart failure were considered major cardiac events. The patients were divided into 2 groups according to systolic function assessed by magnetic resonance. RESULTS: The 2 groups showed similar improvements in functional class and ejection fraction at 6 months. We found a nonsignificant trend toward a higher risk of all-cause mortality in patients with systolic function ≤ 35% at long-term follow-up. The presence of a pattern of necrosis identified patients with a worse prognosis for ventricular arrhythmias and mortality in both groups. CONCLUSIONS: We conclude that cardiac resynchronization therapy with a defibrillator leads to a similar clinical benefit in patients with an ejection fraction ≤ 35% or > 35% estimated by cardiac magnetic resonance. Analysis of the pattern of late gadolinium enhancement provides additional information on arrhythmic risk and long-term prognosis.

Miocardiopatía arritmogénica del ventrículo derecho: Breve revisión de la literatura a propósito de un caso / Arrhythmogenic right ventricular cardiomyopathy: Case report and a brief literature review

Hombre de 51 años admitido en el hospital por presentar palpitaciones y mareos de 2 h de evolución. El electrocardiograma demostró taquicardia regular de QRS ancho y frecuencia cardíaca de 250 lpm, con eje superior y morfología de bloqueo completo de rama izquierda sin descompensación hemodinámica. Se administraron dosis de carga y mantenimiento con amiodarona, revirtiendo a ritmo sinusal. El estudio electrofisiológico demostró el origen ventricular de la taquicardia y su inducibilidad. En la angiografía coronaria no se observaron lesiones significativas en los vasos epicárdicos. Se realizó un ecocardiograma Doppler que presentó cavidades con diámetros y función sistólica y diastólica dentro de los parámetros normales. Ante la sospecha de enfermedad estructural miocárdica se llevó a cabo una resonancia magnética cardíaca contrastada con realce tardío que demostró alteración estructural del ventrículo derecho con incremento de la trabeculación e infiltración fibrograsa parietal y deterioro moderado de su función sistólica, y deterioro leve de la función sistólica del ventrículo izquierdo, lo cual permitió realizar el diagnóstico de miocardiopatía arritmogénica del ventrículo derecho por presentar 2 criterios mayores. Se decidió implantar un cardiodesfibrilador automático, para prevenir la muerte súbita. El paciente evolucionó de manera favorable y fue dado de alta.

A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revea- led systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications.

[Arrhythmogenic right ventricular cardiomyopathy. Case report and a brief literature review]. / Miocardiopatía arritmogénica del ventrículo derecho. Breve revisión de la literatura a propósito de un caso.

A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revealed systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications.

Fibrose miocárdica em pacientes com cardiomiopatia hipertrófica com alto risco para morte súbita cardíaca / Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

FUNDAMENTO: A estratificação de risco para morte súbita na cardiomiopatia hipertrófica (CMH), continua a ser um verdadeiro desafio devido à grande heterogeneidade da sua apresentação, em que a maioria dos indivíduos permanecem assintomáticos por toda sua vida e outros apresentam a morte súbita como primeiro sintoma. Recentes trabalhos vêm sugerindo que a fibrose miocárdica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsáveis pela morte súbita nesta doença. OBJETIVO: Avaliação da prevalência e quantificação da fibrose miocárdica (FM), em pacientes com CMH com alto risco ou recuperados de morte súbita, portadores de cardiodesfibrilador implantável (CDI). MÉTODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos à tomografia computadorizada com múltiplos detectores, para realização da técnica de realce tardio, e avaliação da fibrose miocárdica. RESULTADOS: 96 por cento dos pacientes apresentavam fibrose miocárdica (20,38 ± 15,55 gramas) correspondendo a 15,96 ± 10,20 por cento da massa miocárdica total. A FM foi significativamente mais prevalente que os demais fatores de risco clássicos para morte súbita. CONCLUSÃO: Concluímos que existe uma alta prevalência de fibrose miocárdica em pacientes com cardiomiopatia hipertrófica de alto risco ou recuperados de morte súbita, como neste grupo - portadores de cardiodesfibrilador implantável. A maior prevalência da fibrose miocárdica comparada aos fatores de risco de pior prognóstico levantam a hipótese de que a fibrose miocárdica possa ser um importante substrato potencialmente necessário na gênese das arritmias desencadeadoras da morte súbita.

BACKGROUND: The stratification of risk for sudden death in hypertrophic cardiomyopathy (HCM) continues to be a true challenge due to the great heterogeneity of this disease's presentation, as most individuals remain asymptomatic during their entire lives and others present sudden death as first symptom. Recent studies have suggested that myocardial fibrosis may represent an important substrate for the malignant ventricular arrhythmias, that are responsible for the cases of sudden death related to this disease. OBJECTIVE: To assess the prevalence and quantification of myocardial fibrosis (MF) in hypertrophic cardiomyopathy (HCM) patients with implantablecardioverter - defibrillator (ICD) indicated due to their high risk or recovered from cardiac sudden death. METHODS: Twenty-eight HCM patients with ICD were submitted to multidetector computed tomography to assess myocardial fibrosis by delayed enhancement technique. RESULTS: Myocardial fibrosis was present in 96 percent of these HCM patients with (20.38 ± 15.55 g) comprising 15.96 ± 10.20 percent of the total myocardial mass. MF was observed in a significantly higher prevalence as compared to other classical risk factors for sudden death. CONCLUSION: It is possible to conclude that there is a high prevalence of myocardial fibrosis in hypertrophic cardiomyopathy patients with high-risk or recovered from cardiac sudden death, like those with clinical indication to implantable cardioverter -defibrillator. The higher prevalence of myocardial fibrosis in comparison to classical risk factors of worse prognosis raise the hypothesis that the myocardial fibrosis may be an important substrate in the genesis of lifethreatening arrhythmias in these high risk HCM population.