Luis Francisco Ávila

Evaluation of subclinical atherosclerosis by computed tomography coronary angiography and its association with risk factors in familial hypercholesterolemia

BACKGROUND Increasing age and cholesterol levels, male gender, and family history of early coronary heart disease (CHD) are associated with early onset of CHD in familial hypercholesterolemia (FH). OBJECTIVE Assess subclinical atherosclerosis by computed tomography coronary angiography (CTCA) and its association with clinical and laboratorial parameters in asymptomatic FH subjects. METHODS 102 FH subjects (36% male, 45 ± 13 years, LDL-c 280 ± 54 mg/dL) and 35 controls (40% male, 46 ± 12 years, LDL-c 103 ± 18 mg/dL) were submitted to CTCA. Plaques were divided into calcified, mixed and non-calcified; luminal stenosis was characterized as >50% obstruction. RESULTS FH had a greater atherosclerotic burden represented by higher number of patients with: plaques (48% vs. 14%, p=0.0005), stenosis (19% vs. 3%, p=0.015), segments with plaques (2.05 ± 2.85 vs.0.43 ± 1.33, p=0.0016) and calcium scores (55 ± 129 vs. 38 ± 140, p=0.0028). After multivariate analysis, determinants of plaque presence were increasing age (OR=2.06, for age change of 10 years, CI95%: 1.38-3.07, p<0.001) and total cholesterol (OR=1.86, for cholesterol change by 1 standard deviation, CI95%: 1.09-3.15, p=0.027). Coronary calcium score was associated with the presence of stenosis (OR=1.54; CI95%: 1.27-1.86, p<0.001, for doubling the calcium score). Male gender was directly associated with the presence of non-calcified plaques (OR: 15.45, CI95% 1.72-138.23, p=0.014) and inversely with calcified plaques (OR=0.21, CI95%: 0.05-0.84, p=0.027). Family history of early CHD was associated with the presence of mixed plaques (OR=4.90, CI95%: 1.32-18.21, p=0.018). CONCLUSIONS Patients with FH had an increased burden of coronary atherosclerosis by CTCA. The burden of atherosclerosis and individual plaque subtypes differed with the presence of other associated risk factors, with age and cholesterol being most important. A coronary calcium score of zero ruled out obstructive disease in this higher risk population.

Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

FUNDAMENTO: A estratificacao de risco para morte subita na cardiomiopatia hipertrofica (CMH), continua a ser um verdadeiro desafio devido a grande heterogeneidade da sua apresentacao, em que a maioria dos individuos permanecem assintomaticos por toda sua vida e outros apresentam a morte subita como primeiro sintoma. Recentes trabalhos vem sugerindo que a fibrose miocardica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsaveis pela morte subita nesta doenca. OBJETIVO: Avaliacao da prevalencia e quantificacao da fibrose miocardica (FM), em pacientes com CMH com alto risco ou recuperados de morte subita, portadores de cardiodesfibrilador implantavel (CDI). METODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos a tomografia computadorizada com multiplos detectores, para realizacao da tecnica de realce tardio, e avaliacao da fibrose miocardica. RESULTADOS: 96% dos pacientes apresentavam fibrose miocardica (20,38 ± 15,55 gramas) correspondendo a 15,96 ± 10,20% da massa miocardica total. A FM foi significativamente mais prevalente que os demais fatores de risco classicos para morte subita. CONCLUSAO: Concluimos que existe uma alta prevalencia de fibrose miocardica em pacientes com cardiomiopatia hipertrofica de alto risco ou recuperados de morte subita, como neste grupo - portadores de cardiodesfibrilador implantavel. A maior prevalencia da fibrose miocardica comparada aos fatores de risco de pior prognostico levantam a hipotese de que a fibrose miocardica possa ser um importante substrato potencialmente necessario na genese das arritmias desencadeadoras da morte subita.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 diseases 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% of these HCM patients with (20.38 +/- 15.55 g) comprising 15.96 +/- 10.20% 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.

Fibrose miocárdica em pacientes com cardiomiopatia hipertrófica com alto risco para morte súbita cardíaca

FUNDAMENTO: A estratificacao de risco para morte subita na cardiomiopatia hipertrofica (CMH), continua a ser um verdadeiro desafio devido a grande heterogeneidade da sua apresentacao, em que a maioria dos individuos permanecem assintomaticos por toda sua vida e outros apresentam a morte subita como primeiro sintoma. Recentes trabalhos vem sugerindo que a fibrose miocardica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsaveis pela morte subita nesta doenca. OBJETIVO: Avaliacao da prevalencia e quantificacao da fibrose miocardica (FM), em pacientes com CMH com alto risco ou recuperados de morte subita, portadores de cardiodesfibrilador implantavel (CDI). METODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos a tomografia computadorizada com multiplos detectores, para realizacao da tecnica de realce tardio, e avaliacao da fibrose miocardica. RESULTADOS: 96% dos pacientes apresentavam fibrose miocardica (20,38 ± 15,55 gramas) correspondendo a 15,96 ± 10,20% da massa miocardica total. A FM foi significativamente mais prevalente que os demais fatores de risco classicos para morte subita. CONCLUSAO: Concluimos que existe uma alta prevalencia de fibrose miocardica em pacientes com cardiomiopatia hipertrofica de alto risco ou recuperados de morte subita, como neste grupo - portadores de cardiodesfibrilador implantavel. A maior prevalencia da fibrose miocardica comparada aos fatores de risco de pior prognostico levantam a hipotese de que a fibrose miocardica possa ser um importante substrato potencialmente necessario na genese das arritmias desencadeadoras da morte subita.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 diseases 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% of these HCM patients with (20.38 +/- 15.55 g) comprising 15.96 +/- 10.20% 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.

Compensatory enlargement of human coronary arteries identified by magnetic resonance imaging

The aim of the present study was to evaluate the role of magnetic resonance imaging (MRI) for the non-invasive detection of coronary abnormalities and specifically the remodeling process in patients with coronary artery disease (CAD). MRI was performed in 10 control healthy subjects and 26 patients with angiographically proven CAD of the right coronary (RCA) or left anterior descending (LAD) artery; 23 patients were within two months of acute coronary syndromes, and 3 had stable angina with a positive test for ischemia. Wall thickness (WT), vessel wall area (VWA), total vessel area (TVA), and luminal area (LA) were measured. There were significant increases in WT (mean +/- SEM, RCA: 2.62 +/- 0.75 vs 0.53 +/- 0.15 mm; LAD: 2.21 +/- 0.69 vs 0.62 +/- 0.24 mm) and in VWA (RCA: 30.96 +/- 17.57 vs 2.1 +/- 1.2 mm(2); LAD: 19.53 +/- 7.25 vs 3.6 +/- 2.0 mm(2)) patients compared to controls (P < 0.001 for each variable). TVA values were also greater in patients compared to controls (RCA: 44.56 +/- 21.87 vs 12.3 +/- 4.2 mm(2); LAD: 31.89 +/- 11.31 vs 17.0 +/- 6.2 mm(2); P < 0.001). In contrast, the LA did not differ between patients and controls for RCA or LAD. When the LA was adjusted for vessel size using the LA/TVA ratio, a significant difference was found: 0.33 +/- 0.16 in patients vs 0.82 +/- 0.09 in controls (RCA) and 0.38 +/- 0.13 vs 0.78 +/- 0.06 (LAD) (P < 0.001). As opposed to normal controls, positive remodeling was present in all patients with CAD, as indicated by larger VWA. We conclude that MRI detected vessel wall abnormalities and was an effective tool for the noninvasive evaluation of the atherosclerotic process and coronary vessel wall modifications, including positive remodeling that frequently occurs in patients with acute coronary syndromes.

Miocardiopatia preponderante de ventrículo direito por miocardite prévia ou por displasia arritmogênica

A clinical case of a 10-year-old male patient is reported. His dilated and prevailing right ventricular myocardiopathy shows diagnostic difficulties between previous myocarditis etiology and arrhythmogenic dysplasia. As the elements are not pathognomonic of one or other cause, the increase of cardiac enzymes in subacute stage maybe tends to the supposition of previous myocarditis. Hence, the questioning that many cases labeled as arrhythmogenic dysplasia can truly correspond to the possibility of evolutional myocarditis. The controversial clinic management is disputable.

Detection of myocardial inflammation in Chagas' disease by cardiac magnetic resonance

Methods Fifty-four patients with CD were analyzed, 16 patients in the indeterminate form (IND), 17 patients with the cardiac form without systolic dysfunction (CF), and 21 patients with the cardiac form with systolic dysfunction (CFSD). All patients underwent 1.5-T cardiac magnetic resonance (CMR) using three pulse sequences, previously described as useful for the diagnosis of viral myocarditis: the myocardial delayed enhancement (MDE) technique, Triple-IR FSE T2-weighted sequence and the T1 weighted global enhancement acquired before and after contrast injection, to identify fibrosis, edema and hyperemia of the myocardium, respectively. The parameters for all sequences followed precisely the recommendations for acute myocarditis published on JACC White Paper (Friedrich et al. J Am Coll Cardiol. 2009 Apr 28;53(17):1475-87).

Infecção de prótese vascular em cirurgia da aorta torácica: revisão da experiência e relato de caso tratado por técnica não convencional

We report the case of a 37-year-old-female patient who had undergone a Bentall procedure at our service and returned with intense chest pain and acute aortic dissection type III, which was diagnosed and clinically treated. One year after this episode, this dissection expanded, and the patient underwent surgery with interposition of a Dacron graft in the descending aorta. In the immediate postoperative period, the patient experienced left bronchopneumonia and was discharged afebrile and in good condition. One month after discharge, she returned with fever and toxemia. Pleural empyema was diagnosed, and she underwent an exploratory thoracotomy that did not confirm this diagnosis, but revealed intense effusion thickening. Four months after the exploratory thoracotomy, Klebsiella pneumoniae and Enterobacter sp were isolated in a blood culture. Magnetic resonance imaging revealed shapes compatible with perigraft infection. With this clinical and laboratory picture, graft removal was indicated as was axillo-bifemoral grafting. Surgery was successfully performed, the patient was discharged in good condition, and remains well after a 57-month follow-up without complications. The methods used for diagnosis and treatment of prosthesis infection in thoracic aorta surgery are discussed.

Utilidade clínica da angiografia coronária e da ressonância nuclear magnética no diagnóstico da cardiomiopatia isquêmica

BACKGROUND: The diagnosis of ischemic cardiomyopathy is frequently difficult. Coronary angiography (CA) is limited because it is invasive and the evaluation is exclusively anatomic. Cardiac magnetic resonance imaging (MRI) with late gadolinium enhancement (LGE) measures patterns of myocardial fibrosis caused by ischemia. However, LGE does not detect ischemia that does not result in fibrosis. Thus, a thorough clinical evaluation by a cardiologist seems to be the most effective option for diagnosis. The aim of this study was to evaluate CA and LGE as complementary methods for the diagnosis of ischemic cardiomyopathy in patients with systolic heart failure of unknown etiology. METHODS: Patients with systolic heart failure, left ventricle ejection fraction < 45% and unknown etiology after initial non-invasive evaluation were submitted to CA and MRI with LGE to define the etiology of the disease. Patient evaluation by two cardiologists was the gold standard for the diagnosis of ischemic cardiomyopathy. RESULTS: Twenty-four patients were included. The sensitivity to detect ischemic cardiomyopathy was 0.45 for CA vs. 0.81 for LGE. The specificity was 1.0 for CA vs. 0.84 for LGE. The positive predictive value was 1.0 vs. 0.81 and the negative predictive value was 0.68 vs. 0.84 for CA and LGE, respectively. LGE accuracy was superior to CA accuracy (0.83 vs. 0.75). CONCLUSIONS: LGE was more sensitive than CA to evaluate the etiology of ventricular dysfunction, whereas CA was more specific. The diagnosis of ischemic cardiomyopathy using each one of the methods separately presented limitations.

Clinical Use of Coronary Angiography and Magnetic Nuclear Resonance Imaging in the Diagnosis of Ischemic Cardiomyopathy

ABSTRACT Background The diagnosis of ischemic cardiomyopathy is frequently difficult. Coronary angiography (CA) is limited because it is invasive and the evaluation is exclusively anatomic. Cardiac magnetic resonance imaging (MRI) with late gadolinium enhancement (LGE) measures patterns of myocardial fibrosis caused by ischemia. However, LGE does not detect ischemia that does not result in fibrosis. Thus, a thorough clinical evaluation by a cardiologist seems to be the most effective option for diagnosis. The aim of this study was to evaluate CA and LGE as complementary methods for the diagnosis of ischemic cardiomyopathy in patients with systolic heart failure of unknown etiology. Methods Patients with systolic heart failure, left ventricle ejection fraction Results Twenty-four patients were included. The sensitivity to detect ischemic cardiomyopathy was 0.45 for CA vs. 0.81 for LGE. The specificity was 1.0 for CA vs. 0.84 for LGE. The positive predictive value was 1.0 vs. 0.81 and the negative predictive value was 0.68 vs. 0.84 for CA and LGE, respectively. LGE accuracy was superior to CA accuracy (0.83 vs. 0.75). Conclusions LGE was more sensitive than CA to evaluate the etiology of ventricular dysfunction, whereas CA was more specific. The diagnosis of ischemic cardiomyopathy using each one of the methods separately presented limitations.

Fibrosis miocárdica en pacientes con cardiomiopatía hipertrófica con alto riesgo para muerte súbita cardíaca

FUNDAMENTO: A estratificacao de risco para morte subita na cardiomiopatia hipertrofica (CMH), continua a ser um verdadeiro desafio devido a grande heterogeneidade da sua apresentacao, em que a maioria dos individuos permanecem assintomaticos por toda sua vida e outros apresentam a morte subita como primeiro sintoma. Recentes trabalhos vem sugerindo que a fibrose miocardica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsaveis pela morte subita nesta doenca. OBJETIVO: Avaliacao da prevalencia e quantificacao da fibrose miocardica (FM), em pacientes com CMH com alto risco ou recuperados de morte subita, portadores de cardiodesfibrilador implantavel (CDI). METODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos a tomografia computadorizada com multiplos detectores, para realizacao da tecnica de realce tardio, e avaliacao da fibrose miocardica. RESULTADOS: 96% dos pacientes apresentavam fibrose miocardica (20,38 ± 15,55 gramas) correspondendo a 15,96 ± 10,20% da massa miocardica total. A FM foi significativamente mais prevalente que os demais fatores de risco classicos para morte subita. CONCLUSAO: Concluimos que existe uma alta prevalencia de fibrose miocardica em pacientes com cardiomiopatia hipertrofica de alto risco ou recuperados de morte subita, como neste grupo - portadores de cardiodesfibrilador implantavel. A maior prevalencia da fibrose miocardica comparada aos fatores de risco de pior prognostico levantam a hipotese de que a fibrose miocardica possa ser um importante substrato potencialmente necessario na genese das arritmias desencadeadoras da morte subita.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 diseases 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% of these HCM patients with (20.38 +/- 15.55 g) comprising 15.96 +/- 10.20% 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.