Aims  Myocardial scar detected by cardiovascular magnetic resonance has been associated with sudden cardiac death in dilated cardiomyopathy (DCM). Certain genetic causes of DCM may cause a malignant arrhythmogenic phenotype. The concepts of arrhythmogenic left ventricular (LV) cardiomyopathy (ALVC) and arrhythmogenic DCM are currently ill-defined. We hypothesized that a distinctive imaging phenotype defines ALVC. Methods and results  Eighty-nine patients with DCM-associated mutations [desmoplakin (DSP) n = 25, filamin C (FLNC) n = 7, titin n = 30, lamin A/C n = 12, bcl2-associated athanogene 3 n = 3, RNA binding motif protein 20 n = 3, cardiac sodium channel NAv1.5 n = 2, and sarcomeric genes n = 7] were comprehensively phenotyped. Clustering analysis resulted in two groups: ‘DSP/FLNC genotypes’ and ‘non-DSP/FLNC’. There were no significant differences in age, sex, symptoms, baseline electrocardiography, arrhythmia burden, or ventricular volumes between the two groups. Subepicardial LV late gadolinium enhancement with ring-like pattern (at least three contiguous segments in the same short-axis slice) was observed in 78.1% of DSP/FLNC genotypes but was absent in the other DCM genotypes (P < 0.001). Left ventricular ejection fraction (LVEF) and global longitudinal strain were lower in other DCM genotypes (P = 0.053 and P = 0.015, respectively), but LV regional wall motion abnormalities were more common in DSP/FLNC genotypes (P < 0.001). DSP/FLNC patients with non-sustained ventricular tachycardia (NSVT) had more LV scar (P = 0.010), whereas other DCM genotypes patients with NSVT had lower LVEF (P = 0.001) than patients without NSVT. Conclusion DSP/FLNC genotypes cause more regionality in LV impairment. The most defining characteristic is a subepicardial ring-like scar pattern in DSP/FLNC, which should be considered in future diagnostic criteria for ALVC.

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Aims Mitral valve prolapse (MVP) causes left ventricular (LV) remodelling even in the absence of significant mitral regurgitation. To evaluate whether apical insertion of the papillary muscle (PM) influences the pattern and severity of MVP-related LV remodelling. Methods and results All MVP patients who underwent CMR at our institution between December 2008 and December 2019 were included, thoroughly reviewed and grouped according to apical/non-apical PM insertion. Apical PM insertion was found in 53/92 patients (58%) and associated with mitral leaflet thickening (P &lt; 0.01) and a trend towards higher prevalence of mitral annular disjunction (P = 0.05). Whereas no differences in ventricular volumes or ejection fraction were found, patients with apical PM insertion showed more lateral wall remodelling with mid lateral wall thinning [2.1 (1.8–2.5) vs. 4.0 (3.5–5.0) mm, P &lt; 0.01], increased LV eccentricity and a lower GCS at this level (15 ± 3% vs. 20 ± 3%, P &lt; 0.01). In long-axis direction, increased end-diastolic mid lateral wall angulation was found (i.e. angle &lt;155° measured in the thinnest point of the mid lateral wall in four-chamber view) with a higher angle variation during systole (25 ± 11° vs. 17 ± 8°, P &lt; 0.01). Remarkably, PM fibrosis was significantly more frequent in patients with apical PM insertion (i.e. 66% vs. 28%, P &lt; 0.01). Finally, a higher burden of premature ventricular complexes (&gt;5%) and non-sustained ventricular tachyarrhythmias was found in patients with apical PM insertion: 53% vs. 25% (P = 0.04) and 38% vs. 18% (P = 0.04), respectively. Conclusion Apical PM insertion is part of the phenotypic spectrum of MVP, impacts significantly LV remodelling, and potentially may be related to increased ventricular arrhythmogenicity.

Mitral valve cleft is a rare entity. However, it must be recognized as a differential mechanism for mitral valve regurgitation. 3D transesophageal echocardiography plays an essential role in the evaluation of single and multiple mitral valve clefts, improving morphological and functional assessment of the valve, with potential implications on surgical planning. The authors present four rare cases of mitral valve cleft presenting during adulthood, followed by a mini‐review about the role of 3D echocardiography, as well as its pitfalls, in the assessment of this complex congenital mitral valve disease.

Percutaneous coronary intervention (PCI) has been increasingly performed in patients with severely depressed left ventricular function and complex coronary lesions, including multivessel disease. Mechanical ventricular assist devices play an increasingly important role in high-risk PCI. Impella CP (Abiomed, Inc.) is a new percutaneous left ventricular assist device, designed for short-term circulatory support. It is a promising option for hemodynamic support in high-risk procedures and can potentially reduce PCI-related complications. The authors present two case reports of high-risk PCI using the Impella CP device. In the setting of low coronary flow reserve, severely depressed left ventricular function and potential hemodynamic instability, the Impella CP device has made it possible to maintain hemodynamic stability during procedures, without being associated with vascular complications.