Left dominant arrhythmogenic cardiomyopathy: A morbid association of ventricular arrhythmias and unexplained infero-lateral T-wave inversion

Biventricular arrhythmogenic cardiomyopathy: a paradigmatic case

“…This seems to be in line with other recent findings: a novel truncated DSP mutation has been described as associated with LDAC with primary LV involvement and aggressive phenotype [13]. The lack of specific criteria for AC with primary left-or biventricular involvement, nowadays recognized as a clinical entity per se [4,[11][12][13], does not allow a correct interpretation of the early signs of complex clinical entities such as these. The present case is a stark reminder of the need to consider more than a single diagnostic option when facing arrhythmic presentations in young patients and illustrates well the growing contribution provided by the genetic laboratory and contrast CMR to clinical management.…”

“…They are, however, generally interpreted as highly probable disease-causing mutations, as they are either absent or infrequently found in controls [10]. DSP mutations are known to cause ARVC with both primary RV and LV involvement [4,11,12]. This seems to be in line with other recent findings: a novel truncated DSP mutation has been described as associated with LDAC with primary LV involvement and aggressive phenotype [13].…”

“…This clinical entity was first described at postmortem examination in SD victims. Subsequently, LDAC has been observed in families with DSP mutations [4,11,12]. We consider that arrhythmogenic cardiomyopathy was likely responsible of the patient's son SD while sleeping.…”

“…Only the contrast CMR was able to completely unveil the extensive fibrous replacement of myocardial tissue in both ventricles. Nowadays we recognize three patterns of disease expression in patients and families with arrhythmogenic cardiomyopathy: the "classic right ventricular" subtype (ARVC), the "left dominant" variant (LDAC), characterized by early and predominant LV involvement, and the "biventricular" variant, defined by parallel involvement of both ventricles [4,11,12]. This clinical entity was first described at postmortem examination in SD victims.…”

Arrhythmogenic right ventricular cardiomyopathy: Electroarchitecture of the substrate

“…Although the RV is the predominant chamber involved, left ventricular (LV) involvement has also been documented. 1 , 2 , 3 , 4 Electrophysiological correlations of histopathologic findings in nonischemic cardiomyopathy, and especially in ARVC, are limited. 5 We report a case of ARVC that presented with ventricular tachycardia (VT) and diffuse fibrofatty involvement of both the RV and LV.…”