Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Humans ; Male

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Electrocardiography ; Humans ; Male ; Middle Aged

The clinical diagnosis of right ventricular (RV) cardiomyopathies is often challenging. It is difficult to differentiate the isolated left ventricular (LV) noncompaction cardiomyopathy (NC) from biventricular NC or from coexisting arrhythmogenic ventricular cardiomyopathy (AC). There are currently few established morphologic criteria for the diagnosis other than RV dilation and presence of excessive regional trabeculation. The gross and microscopic changes suggest pathological similarities between, or coexistence of, RV-NC and AC. Therefore, the term arrhythmogenic right ventricular cardiomyopathy is somewhat misleading as isolated LV or biventricular involvement may be present and thus a broader term such as AC should be preferred. We describe an unusual case of AC associated with a NC in a 27-year-old man who had a history of permanent pacemaker 7 years ago due to second-degree atrioventricular block.
Adult ; Arrhythmogenic Right Ventricular Dysplasia ; Atrioventricular Block ; Cardiomyopathies* ; Diagnosis ; Echocardiography* ; Humans

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Death, Sudden ; Humans ; Male ; Middle Aged

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; therapy ; Child, Preschool ; Diagnostic Errors ; Fatal Outcome ; Female ; Humans

Differentiating arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) from other cardiomyopathies is clinically important but challenging. Although the modified Task Force Criteria can facilitate diagnosis of ARVD/C according to clinical manifestations, histopathological examination plays a pivotal role in excluding other diseases that can mimic ARVD/C. Here, we report a patient with amyloidosis that initially presented similarly to ARVD/C. The diagnosis was confirmed by endomyocardial biopsy, and catheter ablation eliminated the ventricular tachyarrhythmias through an epicardial approach.
Advisory Committees ; Amyloidosis* ; Arrhythmogenic Right Ventricular Dysplasia ; Biopsy ; Cardiomyopathies ; Catheter Ablation* ; Catheters* ; Diagnosis ; Humans ; Tachycardia ; Tachycardia, Ventricular

OBJECTIVETo summarize the experience in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC) in children.

METHODSA total of 14 children (7 females and 7 males) with ARVC were involved. The cases underwent electrocardiography, echocardiography, cardiac CT or MRI examinations. All cases were treated with sotalol or amiodarone in combination with propranolol. In 2 cases with drug-refractory ventricular extrasystoles, catheter ablation treatment was performed. In 6 cases with obvious impaired ventricular function, additional pharmacological therapy including vasodilators, diuretics, and digitalis were given.

RESULTSVentricular extrasystoles occurred in all 14 cases and ventricular tachycardia in 8 cases. Ten cases showed Epsilon wave on electrocardiography. All 14 cases had enlarged right ventricle and reduced right ventricular ejection fraction. CT or MRI examination showed right ventricular dilatation and a thinned wall of right ventricle in 10 cases. Ventricular extrasystoles or tachycardia disappeared in 7 cases and was reduced in 4 cases after treatment. The two children receiving catheter ablation treatment did not present ventricular extrasystoles or tachycardia in a 3-month follow-up. The heart function was improved in the 6 children with obvious impaired ventricular function after pharmacological therapy.

CONCLUSIONSThe clinical manifestations are diverse in children with ARVC. A definite diagnosis of ARVC should be based on a combination of electrocardiography and echocardiography examinations. Pharmacological therapy is effective partially. Catheter ablation treatment appears to be a promising option in patients with drug-refractory ventricular extrasystoles.

Adolescent ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; drug therapy ; Child ; Child, Preschool ; Echocardiography ; Electrocardiography ; Female ; Humans ; Male ; Tomography, X-Ray Computed

PURPOSE: To evaluate the usefulness of cardiac MRI in the diagnosis of clinically suspected arrhythmogenic right ventricular dysplasia (ARVD). MATERIALS AND METHODS: Between February 1991 and January 1999, 15 patients [M:F=13:2, aged 2 -60 (mean, 37 -7) years] with clinically suspected ventricular arrhythmia due to unknown causes underwent MR imaging. Using a CP body array coil and the single slice breath hold technique, ECG-gated T1-weighted images were obtained. In all patients, these were acquired transaxially from the diaphragm to the aortic arch and along the true short and long axis, and in two, coronal images were obtained. On the basis of clinical and MRI diagnostic criteria, ARVD was classified as one of four types. The significance of differences in diagnostic grades between clinical and MRI criteria was determined using Wilcoxon's signed rank test. RESULTS: According to both clinical and MRI criteria, it was highly probable that three of the 15 patients had ARVD. In eleven, both sets of criteria indicated the same diagnostic grade. Wilcoxon's signed rank test indicated no significant differences in diagnostic grades between clinical and MRI criteria (p > 0.05). CONCLUSION: For the diagnosis or exclusion of ARVD, MR imaging is a useful modality.
Aorta, Thoracic ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; Axis, Cervical Vertebra ; Diagnosis* ; Diaphragm ; Humans ; Magnetic Resonance Imaging*

PURPOSE: To evaluate the usefulness of cardiac MRI in the diagnosis of clinically suspected arrhythmogenic right ventricular dysplasia (ARVD). MATERIALS AND METHODS: Between February 1991 and January 1999, 15 patients [M:F=13:2, aged 2 -60 (mean, 37 -7) years] with clinically suspected ventricular arrhythmia due to unknown causes underwent MR imaging. Using a CP body array coil and the single slice breath hold technique, ECG-gated T1-weighted images were obtained. In all patients, these were acquired transaxially from the diaphragm to the aortic arch and along the true short and long axis, and in two, coronal images were obtained. On the basis of clinical and MRI diagnostic criteria, ARVD was classified as one of four types. The significance of differences in diagnostic grades between clinical and MRI criteria was determined using Wilcoxon's signed rank test. RESULTS: According to both clinical and MRI criteria, it was highly probable that three of the 15 patients had ARVD. In eleven, both sets of criteria indicated the same diagnostic grade. Wilcoxon's signed rank test indicated no significant differences in diagnostic grades between clinical and MRI criteria (p > 0.05). CONCLUSION: For the diagnosis or exclusion of ARVD, MR imaging is a useful modality.
Aorta, Thoracic ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; Axis, Cervical Vertebra ; Diagnosis* ; Diaphragm ; Humans ; Magnetic Resonance Imaging*

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.
Arrhythmogenic Right Ventricular Dysplasia/*diagnosis ; Female ; Heart Ventricles/pathology ; Humans ; Lung/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Myocardium/pathology ; Sarcoidosis/*diagnosis ; Thyroid Gland/pathology ; Ventricular Fibrillation/diagnosis/*etiology