Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of inherited cardio-myopathy, which is characterized by fibro-fatty replacement of right ventricular myocardium, leading to ventricular arrhythmia. However, rapid atrial arrhythmias are also common, including atrial fibrillation, atrial flutter and atrial tachycardia. Long term rapid atrial arrhythmia can lead to further deterioration of cardiac function. This case is a 51-year-old male. He was admitted to Department of Cardiology, Peking University Third Hospital with palpitation and fatigue after exercise. Electrocardiogram showed incessant atrial tachycardia. Echocardiography revealed dilation of all his four chambers, especially the right ventricle, with the left ventricular ejection fraction of 40% and the right ventricular hypokinesis. Cardiac magnetic resonance imaging found that the right ventricle was significantly enlarged, and the right ventricular aneurysm had formed; the right ventricular ejection fraction was as low as 8%, and the left ventricular ejection fraction was 35%. The patients met the diagnostic criteria of ARVC, and both left and right ventricles were involved. His physical activities were restricted, and metoprolol, digoxin, spironolactone and ramipril were given. Rivaroxaban was also given because atrial tachycardia could cause left atrial thrombosis and embolism. His atrial tachycardia converted spontaneously to normal sinus rhythm after these treatments. Since the patient had severe right ventricular dysfunction, frequent premature ventricular beats and non-sustained ventricular tachycardia on Holter monitoring, indicating a high risk of sudden death, implantable cardioverter defibrillator (ICD) was implanted. After discharge from hospital, physical activity restriction and the above medicines were continued. As rapid atrial arrhythmia could lead to inappropriate ICD shocks, amiodarone was added to prevent the recurrence of atrial tachycardia, and also control ventricular arrhythmia. After 6 months, echocardiography was repeated and showed that the left ventricle diameter was reduced significantly, and the left ventricular ejection fraction increased to 60%, while the size of right ventricle and right atrium decreased slightly. According to the clinical manifestations and outcomes, he was diagnosed with ARVC associated with arrhythmia induced cardiomyopathy. According to the results of his cardiac magnetic resonance imaging, the patient had left ventricular involvement caused by ARVC, and the persistent atrial tachycardia led to left ventricular systolic dysfunction.
Arrhythmogenic Right Ventricular Dysplasia/complications* ; Atrial Fibrillation ; Humans ; Male ; Middle Aged ; Stroke Volume ; Ventricular Function, Left ; Ventricular Function, Right

Arrhythmogenic Right Ventricular Dysplasia ; complications ; Autopsy ; Cardiomyopathy, Hypertrophic ; complications ; Cause of Death ; Coronary Disease ; complications ; Coronary Vessel Anomalies ; complications ; Death, Sudden, Cardiac ; etiology ; pathology ; Humans

OBJECTIVETo study the application of abnormal electrophysiological substrate mapping for guiding ablation of ventricular tachycardias in arrhythmogenic right ventricular cardiomyopathy (ARVC-VTs) using a non-contact mapping system.

METHODSDynamic substrate mapping was performed in three male ARVC patients during sinus rhythm. The sites of the earliest activation, exit point and activation sequence were mapped for each induced VT.

RESULTSThree different patterns of substrates were determined in 3 patients, which located in right ventricular outflow tract, anterior right ventricular wall, and anterolateral right ventricular wall, respectively. Five different clinical VTs [mean CL (348 +/- 65) ms] were induced. Of 5 VTs, three were originated from substrate or boundary of substrate, and two had a remote origin. One VT conducted through the substrate. Linear ablations were created between the sites of the earliest ventricular activation and the VT exit point, or across the critical isthmus. The five clinical VTs were successfully ablated. There were no VT recurrences during 20 months of follow-up.

CONCLUSIONSDefining the abnormal electrophysiologic VT substrates is useful for understanding the mechanisms of ARVC-VTs and determining an ablation strategy. Linear ablation across a critical isthmus or between the earliest activation and the exit point can effectively cure these arrhythmias.

Adult ; Arrhythmogenic Right Ventricular Dysplasia ; etiology ; physiopathology ; therapy ; Catheter Ablation ; methods ; Electrophysiologic Techniques, Cardiac ; Humans ; Male ; Tachycardia, Ventricular ; complications ; physiopathology ; therapy

OBJECTIVESTo study the pathologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) in the phase of heart failure.

METHODSEight cases underwent heart transplantation in Fuwai Hospital during the period from May, 2004 to July, 2007 with pathologic diagnosis of ARVC were studied. The age of patients ranged from 15 to 54 years. They had history of palpitation and syncope for 1 to 22 years. Severe heart failure was diagnosed according to the New York Heart Association Classification System. The recipient hearts were examined and the following parameters were evaluated: weight of heart, presence of cardiac dilatation, myocardial hypertrophy, fatty infiltration, fibrosis, parietal thrombosis and myocarditis. The degree of left ventricular involvement was also analyzed.

RESULTSOf the 8 cases studied, 7 cases with prominent right ventricular lesion (fibrofatty replacement) were classified as classic type. One case with prominent left ventricle lesion and mild right ventricle involvement was classified as left predominant type. No biventricular type and no pure fatty infiltration were found. The cases of classic type showed moderate to severe dilatation of right ventricle, sometimes with aneurysm formation. Left ventricle was involved in 6 cases, which showed diffuse interstitial fibrosis, patchy fibrous replacement and subepicardial fatty infiltration. Mild to moderate dilatation of left ventricle, myocardial hypertrophy and vacuolation were also observed in these cases. The case of left predominant type had severe hypertrophy and dilatation of left ventricle, with prominent diffuse interstitial fibrosis and transmural fatty infiltration. Besides, 3 cases showed left ventricular hypertrophy and parietal thrombosis in both ventricles. Focal lymphocytic myocarditis was noted in 1 case.

CONCLUSIONSLeft ventricular involvement is common in the heart failure phase of ARVC. Extensive interstitial fibrosis, marked hypertrophy and degeneration of myocardial fibers, as well as severe cardiac dilatation with organized thrombi, represent the major pathologic changes which resembles dilated cardiomyopathy.

Adipose Tissue ; pathology ; Adolescent ; Adult ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; complications ; pathology ; physiopathology ; Cardiomyopathy, Dilated ; etiology ; Female ; Fibrosis ; etiology ; Heart Failure ; complications ; Humans ; Male ; Middle Aged ; Myocarditis ; etiology ; pathology ; Myocardium ; pathology ; Young Adult

OBJECTIVETo study the pathologic features of dilated heart in cardiac transplant recipients, with clinicoradiologic correlation.

METHODSSixty recipient hearts from cardiac transplantation performed in Fuwai Hospital were analyzed by gross examination, histologic observation and electron microscopy. Clinicoradiologic correlation was available in 40 cases.

RESULTSAmongst the 40 cases of dilated heart, 52.5% (21/40) were due to dilated cardiomyopathy, 22.5% (9/40) due to arrhythmogenic right ventricular cardiomyopathy, 15.0% (6/40) due to ischemic cardiomyopathy, and the remaining 10.0% (4/40) due to miscellaneous causes, including local noncompaction of ventricular myocardium, giant cell myocarditis, alcoholic cardiomyopathy and hypertensive cardiomyopathy. The discrepancy rate between clinical and pathologic diagnosis was 37.5% (15/40). The erroneous categories included arrhythmogenic right ventricular cardiomyopathy (7 cases), ischemic cardiomyopathy (5 cases), and giant cell myocarditis (1 case), which were all mistaken clinically as dilated cardiomyopathy. While ischemic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, noncompaction of ventricular myocardium and giant cell myocarditis have distinctive pathologic features, the diagnosis of alcoholic and hypertensive cardiomyopathies required clinicopathologic correlation. Dilated cardiomyopathy due to viral myocarditis was not identified in the cases studied.

CONCLUSIONPathologic examination is essential in analysis of transplant recipient heart and helps to rectify clinical diagnostic discrepancy.

Adolescent ; Adult ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; pathology ; Cardiomyopathy, Alcoholic ; diagnosis ; pathology ; Cardiomyopathy, Dilated ; diagnosis ; pathology ; Diagnostic Errors ; Dilatation, Pathologic ; diagnosis ; pathology ; Female ; Giant Cells ; pathology ; Heart Transplantation ; pathology ; Humans ; Hypertension ; complications ; Male ; Middle Aged ; Myocardial Ischemia ; diagnosis ; pathology ; Myocardium ; pathology