Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by fibro-fatty replacement of the right ventricle.However,the feasibility and significance of myocardial fibrosis detec-ted by delaye...Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by fibro-fatty replacement of the right ventricle.However,the feasibility and significance of myocardial fibrosis detec-ted by delayed enhancement (DE) using 3.0T magnetic resonance imaging (MRI) in.ARVD /C is seldomly studied.Methods Twenty-seven consecutive patients were prospectively evaluated for ARVD /C.Magnetic reso-nance imaging was performed on a 3.0T scanner.Ten minutes after intravenous administration of 0.2 mmol /kg of gadodiamide,DE-MRI was obtained.Diagnosis of ARVD /C was based upon the Task Force criteria and in-cluded MRI findings.Results Seventeen(59% ) of 27 patients met the Task Force criteria for ARVD /C.Right ven-tricle DE was found in all (100% ) ARVD /C patients compared with none (0%) of the 10 patients without ARVD /C (P <0.001) .Additional left ventricular DE was found in 8/17 ARVD/C patients while without left ventricular mor-phological and functional abnormalities detected by echocardiography or MRI.Conclusions DE using 3.0T MRI could effectively detect myocardial fibrosis in the right and left ventricular myocardium in ARVD /C patients.Detection of myocardial fibrosis may have an important clinical significance in ARVD/C diagnosis.Histological left ventricle in-volvement may be easily missed by echocardiography.展开更多
Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden d...Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden death from ventricular arrhythmia in hereditary sarcomeric HCM. Patients and Methods: This is a retrospective and prospective descriptive study over 18 months (January 01, 2021, to July 31, 2022) on the records of patients over 18 with the diagnosis of hypertrophic sarcomeric cardiomyopathy with or without ventricular rhythm disorders and who have undergone myocardial scintigraphy. Results: Three patients were included. The average age of our patients was 66 years old. Dyspnea is the primary symptomatology found in our patients. One patient presented with syncope and unsustained ventricular tachycardia. His risk score for sudden death from ESC at five years is estimated at 6.45%, and the patient received an ICD in primary prevention. The average sudden death risk score of our patients was 3.78%. The mean LV wall thickness was 20 mm. The mean maximum left intraventricular gradient was 39 mmHg. Myocardial fibrosis was present in all our patients. Myocardial scintigraphy was normal in all cases. Conclusion: Hypertrophic cardiomyopathy is a very heterogeneous group of cardiomyopathies. The rhythmic risk is multifactorial and constitutes a significant prognostic factor.展开更多
文摘Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by fibro-fatty replacement of the right ventricle.However,the feasibility and significance of myocardial fibrosis detec-ted by delayed enhancement (DE) using 3.0T magnetic resonance imaging (MRI) in.ARVD /C is seldomly studied.Methods Twenty-seven consecutive patients were prospectively evaluated for ARVD /C.Magnetic reso-nance imaging was performed on a 3.0T scanner.Ten minutes after intravenous administration of 0.2 mmol /kg of gadodiamide,DE-MRI was obtained.Diagnosis of ARVD /C was based upon the Task Force criteria and in-cluded MRI findings.Results Seventeen(59% ) of 27 patients met the Task Force criteria for ARVD /C.Right ven-tricle DE was found in all (100% ) ARVD /C patients compared with none (0%) of the 10 patients without ARVD /C (P <0.001) .Additional left ventricular DE was found in 8/17 ARVD/C patients while without left ventricular mor-phological and functional abnormalities detected by echocardiography or MRI.Conclusions DE using 3.0T MRI could effectively detect myocardial fibrosis in the right and left ventricular myocardium in ARVD /C patients.Detection of myocardial fibrosis may have an important clinical significance in ARVD/C diagnosis.Histological left ventricle in-volvement may be easily missed by echocardiography.
文摘Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden death from ventricular arrhythmia in hereditary sarcomeric HCM. Patients and Methods: This is a retrospective and prospective descriptive study over 18 months (January 01, 2021, to July 31, 2022) on the records of patients over 18 with the diagnosis of hypertrophic sarcomeric cardiomyopathy with or without ventricular rhythm disorders and who have undergone myocardial scintigraphy. Results: Three patients were included. The average age of our patients was 66 years old. Dyspnea is the primary symptomatology found in our patients. One patient presented with syncope and unsustained ventricular tachycardia. His risk score for sudden death from ESC at five years is estimated at 6.45%, and the patient received an ICD in primary prevention. The average sudden death risk score of our patients was 3.78%. The mean LV wall thickness was 20 mm. The mean maximum left intraventricular gradient was 39 mmHg. Myocardial fibrosis was present in all our patients. Myocardial scintigraphy was normal in all cases. Conclusion: Hypertrophic cardiomyopathy is a very heterogeneous group of cardiomyopathies. The rhythmic risk is multifactorial and constitutes a significant prognostic factor.
