Left ventricular fibroma in childhood: 2-dimensional echocardiographic diagnosis and surgical treatment. Description of a case

We describe the case of a patient 4 months old transferred to our hospital for evaluation of occasionally detected cardiac alterations: ecg showed abnormal Q and negative T waves in D1 - aVL leads and chest x-ray was consistent with enlargement of the left ventricular cavity. After admission, 2D echocardiographic examination disclosed, in the left ventricular cavity, a large mass extending from the papillary muscles to the outflow tract. The mass was of uniform density and easily recognizable as a tumor. Although asymptomatic, the patient underwent operation 10 months later because of the risk of occurrence of severe conduction disturbances or arrhythmias. Histological examination demonstrated that the mass was a fibroma. At post-op echocardiographic and angiographic evaluation the left ventricle appeared abnormally dilated with a large aneurysm of the lateral wall and very poor pump function. Nevertheless the patient did well in the following months until a ventricular fibrillation occurred at home. He was immediately brought to hospital and resuscitation was attempted unsuccessfully. We discuss the role of 2D echocardiography in the diagnosis of cardiac tumors, the indication of the early surgical removal and the possible limitations of the technique we used in this case.     

Mechanical Synchrony: Role of Surgical Ventricular Restoration in Correcting LV Dyssynchrony During Chamber Rebuilding

Cardiac failure is frequently complicated by intra and or interventricular conduction delay that results in dyssynchronized cardiac contraction and relaxation. In contrast to an electrical intervention by biventricular pacing, this study tests the capacity of geometric rebuilding by surgical ventricular restoration (SVR) to restore a more synchronous contractile pattern through mechanical reconstruction without exogenous pacing input.Thirty patients (58 ± 8 years) undergoing SVR at the Cardiothoracic Center of Monaco were prospectively evaluated with a protocol which uses simultaneous measurements of ventricular volumes and pressure to construct pressure/volume (P/V) and pressure/length (P/L) loops. Mean QRS duration was within normal limits (100± 17 ms) preoperatively. Preoperative LV contraction was highly asynchronous. Endocardial time motion was either early or delayed at the end-systolic phase, yielding P/L loops with abnormal in size, shape, and orientation. Postoperatively, SVR resulted in leftward shifting of P/V loops and increased area; endocardial time motion and P/L loops almost normalized. The hemodynamic consequences of SVR included improved ejection fraction; reduced end-diastolic and end-systolic volume index; more rapid peak filling rate; peak ejection rate and mechanical efficiency resulting in mechanical intraventricular resynchronization that improves LV performance.     

Atypical myocardial delayed enhancement after surgical ventricle restoration

Objective

To retrospectively evaluate delayed enhancement after surgical ventricle restoration (SVR).

Materials and methods

Thirty patients with post-ischemic akinetic dilatation underwent cine and gadobenate dimeglumine-enhanced MRI before and 2–6 months after SVR. End-diastolic volume (EDV) normalized to body surface area (EDV index, EDVI), end-systolic volume index (ESVI), and ejection fraction (EF) of the left ventricle were obtained. Delayed enhancement involving subendocardial myocardium was considered typical (TDE) while enhancement apparently not involving subendocardium in its typical location was considered atypical (ADE). Wilcoxon signed-rank, Mann–Whitney U, and χ² tests were used.

Results

All 30 patients showed TDE prior to surgery but 16 of them (53%) showed ADE after SVR. Before SVR, EDVI (ml/m²) was 171 ± 101 in patients with ADE and 127 ± 41 in patients with TDE, ESVI (ml/m²) was 130 ± 105 and 94 ± 36, EF was 25 ± 6% and 24 ± 9%, respectively, without significant differences (P > 0.347). After SVR, EDVI (ml/m²) was 99 ± 24 and 89 ± 28 (P = 0.275), ESVI (ml/m²) 66 ± 24 and 65 ± 30, respectively, without significant differences (P > 0.275) while EF was 37 ± 12% and 29 ± 13%, respectively, with a near-significant difference (P = 0.077). The increase in EF before/after SVR was significant only for patients with ADE (P = 0.006). Of 20 patients in whom a patch had been used for SVR, 14 showed ADE and 6 TDE; of 10 patients without patch, 2 and 8, respectively (P = 0.010).

Conclusion

ADE was found in about half of SVR patients and was associated with the use of patch and EF improvement.     

MicroRNA dysregulation in diabetic ischemic heart failure patients

Increased morbidity and mortality associated with ischemic heart failure (HF) in type 2 diabetic patients requires a deeper understanding of the underpinning pathogenetic mechanisms. Given the implication of microRNAs (miRNAs) in HF, we investigated their regulation and potential role. miRNA expression profiles were measured in left ventricle biopsies from 10 diabetic HF (D-HF) and 19 nondiabetic HF (ND-HF) patients affected by non-end stage dilated ischemic cardiomyopathy. The HF groups were compared with each other and with 16 matched nondiabetic, non-HF control subjects. A total of 17 miRNAs were modulated in D-HF and/or ND-HF patients when compared with control subjects. miR-216a, strongly increased in both D-HF and ND-HF patients, negatively correlated with left ventricular ejection fraction. Six miRNAs were differently expressed when comparing D-HF and ND-HF patients: miR-34b, miR-34c, miR-199b, miR-210, miR-650, and miR-223. Bioinformatic analysis of their modulated targets showed the enrichment of cardiac dysfunctions and HF categories. Moreover, the hypoxia-inducible factor pathway was activated in the noninfarcted, vital myocardium of D-HF compared with ND-HF patients, indicating a dysregulation of the hypoxia response mechanisms. Accordingly, miR-199a, miR-199b, and miR-210 were modulated by hypoxia and high glucose in cardiomyocytes and endothelial cells cultured in vitro. In conclusion, these findings show a dysregulation of miRNAs in HF, shedding light on the specific disease mechanisms differentiating diabetic patients.