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ObjectiveTo compare multi-detector computed tomography (MDCT) with cardiac catheterization and transthoracic echocardiography (TTE) in comprehensive evaluation of the global cardiovascular anatomy in patients with pulmonary atresia with ventricular septal defect (PA-VSD).MethodsThe clinical and imaging data of 116 patients with PA-VSD confirmed by surgery were reviewed. Using findings at surgery as the reference standard, data from MDCT, TTE and catheterization were reviewed for assessment of native pulmonary vasculature and intracardiac defects.ResultsMDCT was more accurate than catheterization and TTE in identification of native pulmonary arteries. MDCT is also the most accurate test for delineation of the major aortopulmonary collateral arteries. The inter-modality agreement for evaluation of overriding aorta and VSD were both excellent. In the subgroup with surgical correlation, excellent agreement was found between TTE and surgery, and substantial agreement was also found at MDCT.ConclusionMDCT can correctly delineate the native pulmonary vasculatures and intracardiac defects and may be a reliable method for noninvasive assessment of global cardiovascular abnormalities in patients with PA-VSD.

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of heart muscle disease characterized by the gradual replacement of the right ventricular myocardium with fibrous tissue and fat. It could be the major cause of sudden cardiac death with ventricular tachycardia, and there is a variation in the history of the disease. We reported an unusual case of ARVC in a middle-aged woman with congestive heart failure as her first presentation for a long time.