Formada pela Universidade de Pernambuco. Mestre em Ciências da Saúde pela Univerdiade de Pernambuco. Especialista em Cardiologia e Ecocardiografia pela SBC. Especialista em Imagem Cardíaca (DASA/Unifesp).
This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant per... more This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant peripheral nerve sheath tumour. The 18 F-fluorodeoxyglucosis positron emission tomography associated to computed tomography (18 F-FDG PET/CT) accesses the tumour anatomy and metabolic activity, thereby making it possible to characterize a malignant neoplasm noninvasively. The diagnostic approach with 18 F-FDG PET/CT spared the heart from a likely futile invasive procedure when detecting a distant metastasis and changed the biopsy site and therapeutic planning.
As melhorias na aquisição e no processamento de imagens na
última década permitiram uma crescent... more As melhorias na aquisição e no processamento de imagens na
última década permitiram uma crescente evolução do papel
da ressonância cardíaca e da tomografia computadorizada na
avaliação das doenças do coração. Uma série de patologias
cardíacas puderam ser melhor avaliadas, dentre elas as neo
plasias
cardíacas. Os tumores cardíacos são encontrados em
0,002%-0,3% das autópsias e, embora sejam raros, apresentam
morbidade e mortalidade elevadas, especialmente quando
malignos. A grande maioria dos tumores cardíacos são se
cundários,
metastáticos, com acometimento preferencial das
câmaras direitas e comprometimento do pericárdio. Dentre os
tumores cardíacos primários, os mais comuns são os de origem
benigna e, dentre estes, os mais frequentes são os mixomas,
os lipomas e os fibroelastomas, necessariamente nessa ordem.
A melhor resolução espacial e o maior campo de visão destes
novos métodos também os colocam em vantagem em relação às
limitações de janela acústica da ecocardiografia transtorácica.
A caracterização tecidual realizada, sobretudo, pela ressonância
pode auxiliar na distinção entre o grau de malignidade de um
tumor, ao mesmo tempo que permite a avaliação da função ven
tricular
e do comprometimento hemodinâmico. O diagnóstico
diferencial com cistos, trombos ou achados da anatomia normal
pode ser realizado permitindo, em alguns casos, prescindir de
biópsia miocárdica. A ressonância magnética e a tomografi
computadorizada tornaram-se ferramentas importantes na
decisão terapêutica e no acompanhamento desses pacientes
Background Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in th... more Background Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the alpha galactosidase A gene (GLA) that lead to the enzymatic deficiency of alpha galactosidase (α-Gal A), resulting in the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), causing multiple organ dysfunctions. Objective To perform GLA gene screening in a group of patients with echocardiographic diagnosis of hypertrophic cardiomyopathy (HCM). Methods a cross-sectional study was conducted with HCM patients from a university hospital. Patients with coronary artery disease and valvulopathies were excluded. Mutation analysis of the GLA gene was performed. In male subjects, the analysis was performed after evidence of low α-Gal A activity. Results 60 patients with echocardiographic diagnosis of HCM were included. Age ranged from 12 to 85 years and 60% were women. Mean myocardial fibrosis percentage on MRI was 10.7 ± 13.1% and mean ventricular thickness was18...
BACKGROUND Apical hypertrophic cardiomyopathy (AHCM) is a rare cardiomyopathy, in which hypertrop... more BACKGROUND Apical hypertrophic cardiomyopathy (AHCM) is a rare cardiomyopathy, in which hypertrophy occurs predominantly in the ventricular apex, and in some cases with a high risk of sudden cardiac death. OBJECTIVE The aim of this paper is to present a case series of patients with AHCM and describe their main clinical, echocardiographic and electrocardiographic characteristics, the recommendation for an implantable cardioverter-defibrillator (ICD) and the frequency of sudden cardiac death (SCD). METHODS A retrospective case series was conducted at the referral center of a federal teaching hospital, between the years 2005 to 2020, involving patients with an echocardiographic diagnosis of AHCM. The parameters of the American College of Cardiology and the European Society of Cardiology were used to assess the risk of SCD. RESULTS A total of 11 individuals were assessed with a mean age of 55.3 years, mean follow-up of 41.2 months, most of whom were symptomatic at diagnosis (72.7%). The most frequent symptom was dyspnea (27.3%). A family history of SCD was described in 45.5% of cases. Due to a high risk of SCD, four patients received ICDs. One patient presented sudden cardiac death after having refused the ICD. CONCLUSIONS Symptoms and alterations in the imaging exams are significant factors in the clinical and prognostic assessment of patients with AHCM.
Introduction: There is evidence that aldosterone plays a role in the pathogenesis of vascular cal... more Introduction: There is evidence that aldosterone plays a role in the pathogenesis of vascular calcification. The aim of this study was to evaluate the effect of spironolactone, a mineralocorticoid receptor antagonist, on the progression of coronary calcification (CC) in peritoneal dialysis patients and to identify the factors involved in this progression. Methods: Thirty-three patients with a coronary calcium score (CCS) ≥ 30, detected through multi-detector computed tomography (MDCT) and expressed in Agatston units, were randomly assigned to a group receiving 25mg spironolactone per day for 12 months (spironolactone group) and a control group not receiving this drug. The primary outcome was a percentage change in CCS from baseline to end of the study (relative progression), when a further MDCT was conducted. Patients who had progression of CC were compared with those who did not progress. Results: Sixteen patients, seven in the spironolactone group and nine in the control group, co...
This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant per... more This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant peripheral nerve sheath tumour. The 18 F-fluorodeoxyglucosis positron emission tomography associated to computed tomography (18 F-FDG PET/CT) accesses the tumour anatomy and metabolic activity, thereby making it possible to characterize a malignant neoplasm noninvasively. The diagnostic approach with 18 F-FDG PET/CT spared the heart from a likely futile invasive procedure when detecting a distant metastasis and changed the biopsy site and therapeutic planning.
As melhorias na aquisição e no processamento de imagens na
última década permitiram uma crescent... more As melhorias na aquisição e no processamento de imagens na
última década permitiram uma crescente evolução do papel
da ressonância cardíaca e da tomografia computadorizada na
avaliação das doenças do coração. Uma série de patologias
cardíacas puderam ser melhor avaliadas, dentre elas as neo
plasias
cardíacas. Os tumores cardíacos são encontrados em
0,002%-0,3% das autópsias e, embora sejam raros, apresentam
morbidade e mortalidade elevadas, especialmente quando
malignos. A grande maioria dos tumores cardíacos são se
cundários,
metastáticos, com acometimento preferencial das
câmaras direitas e comprometimento do pericárdio. Dentre os
tumores cardíacos primários, os mais comuns são os de origem
benigna e, dentre estes, os mais frequentes são os mixomas,
os lipomas e os fibroelastomas, necessariamente nessa ordem.
A melhor resolução espacial e o maior campo de visão destes
novos métodos também os colocam em vantagem em relação às
limitações de janela acústica da ecocardiografia transtorácica.
A caracterização tecidual realizada, sobretudo, pela ressonância
pode auxiliar na distinção entre o grau de malignidade de um
tumor, ao mesmo tempo que permite a avaliação da função ven
tricular
e do comprometimento hemodinâmico. O diagnóstico
diferencial com cistos, trombos ou achados da anatomia normal
pode ser realizado permitindo, em alguns casos, prescindir de
biópsia miocárdica. A ressonância magnética e a tomografi
computadorizada tornaram-se ferramentas importantes na
decisão terapêutica e no acompanhamento desses pacientes
Background Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in th... more Background Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the alpha galactosidase A gene (GLA) that lead to the enzymatic deficiency of alpha galactosidase (α-Gal A), resulting in the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), causing multiple organ dysfunctions. Objective To perform GLA gene screening in a group of patients with echocardiographic diagnosis of hypertrophic cardiomyopathy (HCM). Methods a cross-sectional study was conducted with HCM patients from a university hospital. Patients with coronary artery disease and valvulopathies were excluded. Mutation analysis of the GLA gene was performed. In male subjects, the analysis was performed after evidence of low α-Gal A activity. Results 60 patients with echocardiographic diagnosis of HCM were included. Age ranged from 12 to 85 years and 60% were women. Mean myocardial fibrosis percentage on MRI was 10.7 ± 13.1% and mean ventricular thickness was18...
BACKGROUND Apical hypertrophic cardiomyopathy (AHCM) is a rare cardiomyopathy, in which hypertrop... more BACKGROUND Apical hypertrophic cardiomyopathy (AHCM) is a rare cardiomyopathy, in which hypertrophy occurs predominantly in the ventricular apex, and in some cases with a high risk of sudden cardiac death. OBJECTIVE The aim of this paper is to present a case series of patients with AHCM and describe their main clinical, echocardiographic and electrocardiographic characteristics, the recommendation for an implantable cardioverter-defibrillator (ICD) and the frequency of sudden cardiac death (SCD). METHODS A retrospective case series was conducted at the referral center of a federal teaching hospital, between the years 2005 to 2020, involving patients with an echocardiographic diagnosis of AHCM. The parameters of the American College of Cardiology and the European Society of Cardiology were used to assess the risk of SCD. RESULTS A total of 11 individuals were assessed with a mean age of 55.3 years, mean follow-up of 41.2 months, most of whom were symptomatic at diagnosis (72.7%). The most frequent symptom was dyspnea (27.3%). A family history of SCD was described in 45.5% of cases. Due to a high risk of SCD, four patients received ICDs. One patient presented sudden cardiac death after having refused the ICD. CONCLUSIONS Symptoms and alterations in the imaging exams are significant factors in the clinical and prognostic assessment of patients with AHCM.
Introduction: There is evidence that aldosterone plays a role in the pathogenesis of vascular cal... more Introduction: There is evidence that aldosterone plays a role in the pathogenesis of vascular calcification. The aim of this study was to evaluate the effect of spironolactone, a mineralocorticoid receptor antagonist, on the progression of coronary calcification (CC) in peritoneal dialysis patients and to identify the factors involved in this progression. Methods: Thirty-three patients with a coronary calcium score (CCS) ≥ 30, detected through multi-detector computed tomography (MDCT) and expressed in Agatston units, were randomly assigned to a group receiving 25mg spironolactone per day for 12 months (spironolactone group) and a control group not receiving this drug. The primary outcome was a percentage change in CCS from baseline to end of the study (relative progression), when a further MDCT was conducted. Patients who had progression of CC were compared with those who did not progress. Results: Sixteen patients, seven in the spironolactone group and nine in the control group, co...
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última década permitiram uma crescente evolução do papel
da ressonância cardíaca e da tomografia computadorizada na
avaliação das doenças do coração. Uma série de patologias
cardíacas puderam ser melhor avaliadas, dentre elas as neo
plasias
cardíacas. Os tumores cardíacos são encontrados em
0,002%-0,3% das autópsias e, embora sejam raros, apresentam
morbidade e mortalidade elevadas, especialmente quando
malignos. A grande maioria dos tumores cardíacos são se
cundários,
metastáticos, com acometimento preferencial das
câmaras direitas e comprometimento do pericárdio. Dentre os
tumores cardíacos primários, os mais comuns são os de origem
benigna e, dentre estes, os mais frequentes são os mixomas,
os lipomas e os fibroelastomas, necessariamente nessa ordem.
A melhor resolução espacial e o maior campo de visão destes
novos métodos também os colocam em vantagem em relação às
limitações de janela acústica da ecocardiografia transtorácica.
A caracterização tecidual realizada, sobretudo, pela ressonância
pode auxiliar na distinção entre o grau de malignidade de um
tumor, ao mesmo tempo que permite a avaliação da função ven
tricular
e do comprometimento hemodinâmico. O diagnóstico
diferencial com cistos, trombos ou achados da anatomia normal
pode ser realizado permitindo, em alguns casos, prescindir de
biópsia miocárdica. A ressonância magnética e a tomografi
computadorizada tornaram-se ferramentas importantes na
decisão terapêutica e no acompanhamento desses pacientes
última década permitiram uma crescente evolução do papel
da ressonância cardíaca e da tomografia computadorizada na
avaliação das doenças do coração. Uma série de patologias
cardíacas puderam ser melhor avaliadas, dentre elas as neo
plasias
cardíacas. Os tumores cardíacos são encontrados em
0,002%-0,3% das autópsias e, embora sejam raros, apresentam
morbidade e mortalidade elevadas, especialmente quando
malignos. A grande maioria dos tumores cardíacos são se
cundários,
metastáticos, com acometimento preferencial das
câmaras direitas e comprometimento do pericárdio. Dentre os
tumores cardíacos primários, os mais comuns são os de origem
benigna e, dentre estes, os mais frequentes são os mixomas,
os lipomas e os fibroelastomas, necessariamente nessa ordem.
A melhor resolução espacial e o maior campo de visão destes
novos métodos também os colocam em vantagem em relação às
limitações de janela acústica da ecocardiografia transtorácica.
A caracterização tecidual realizada, sobretudo, pela ressonância
pode auxiliar na distinção entre o grau de malignidade de um
tumor, ao mesmo tempo que permite a avaliação da função ven
tricular
e do comprometimento hemodinâmico. O diagnóstico
diferencial com cistos, trombos ou achados da anatomia normal
pode ser realizado permitindo, em alguns casos, prescindir de
biópsia miocárdica. A ressonância magnética e a tomografi
computadorizada tornaram-se ferramentas importantes na
decisão terapêutica e no acompanhamento desses pacientes