No abstract

Ventricular noncompaction is a rare unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. It is characterized by markedly irregular endocardial surfaces with prominent trabeculations and intertrabecular recesses. Patients are asymptomatic or present with depressed ventricular function, ventricular arrhythmia, and systemic embolism. Areas of restricted myocardial perfusion have been previously documented by scintigraphy and positron emission tomography. This report documents a case of isolated left ventricular noncompaction with subendocardial perfusion deficits on MRI.

No abstract

The aim of this study was to define a system for scoring hemophilic arthropathy (HA) based on MR findings, providing for objective evaluation of the degree of joint involvement and evolution after on-demand administration of FVIII substitutional therapy or prophylactic treatment. A total of 133 MR examinations (89 basal and 44 during follow-up) were performed in 25 patients. Patients were divided in two groups depending on the therapy received and the length of time that the disease had been evolving at the start of the study. Group I was composed of 10 patients with secondary prophylaxis and group II was composed by 15 treated on demand. T1-weighted and T2*-weighted images performed on a 0.5-T unit were prospectively evaluated. The joint involvement was established on a scale of 0 (no abnormalities), I (minimal amount of hemosiderin), II (large amount of hemosiderin and isolated cartilaginous erosion), III (cartilage destruction, bone erosions, and subchondral cysts) to IV (large internal joint derangement, secondary osteoarthritis and/or ankylosis). At basal MR examination, patients of group I showed no abnormalities ( n=28, 75.6%), grade I ( n=3), and grade II ( n=6) of HA. Patients of group II corresponded to MR grades III ( n=21) and IV ( n=11) of HA. The MR follow-up showed improvement in three joints of group I and worsening in 5 joints in group I and 2 joints in group II. Early signs of HA were detected in 10 joints with MR imaging but were underestimated on plain radiographs. Advanced degrees of HA were classified as severe under both imaging techniques. Magnetic resonance is the preferred imaging technique to assess HA. The earliest signs of joint damage, detected by MR, are overlooked by plain radiography. The MR scoring system can be used for evaluating HA. Follow-up MR should be performed to evaluate regression or worsening of the abnormalities, primarily in the case of patients with prophylaxis who usually suffer normal or early joint involvement not detected by other means.

CMR data are associated to exercise WMAs in patients with HCM. ExE and CMR may help to predict outcome in them.