Arrhythmogenic Right Ventricular Cardiomyopathy Accompanied by Chronic Myocarditis.

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) classically present with ventricular arrhythmias and less commonly heart failure. ARVC is an inherited cardiomyopathy and generally based on a variant of desmosomal genes. Recently, the association between myocardial inflammation and ARVC has been a matter of great concern. We encountered a patient with ARVC who had a desmoglein-2 mutation with advanced right ventricular failure accompanying a preserved left ventricular function. Concomitant right ventricular myocarditis was detected four years after the diagnosis of ARVC. ARVC and myocarditis might have a deep pathophysiological association, at least in some cases.

Successful Management of Pheochromocytoma Crisis with Cardiogenic Shock by Percutaneous Left Ventricular Assist Device.

Therapeutic strategy utilizing mechanical circulatory supports in patients with pheochromocytoma-related cardiogenic shock remains unestablished. We had a 51-year-old man with acute decompensated heart failure due to pheochromocytoma crisis. He received a percutaneous left ventricular assist device-supported alpha-blocker and intensive fluid infusion therapy, which ameliorated impaired end-organ dysfunction, maintaining hemodynamics and achieving cardiac recovery, followed by the successfully scheduled adrenalectomy. Early suspicion of pheochromocytoma and Impella-supported disease-specific medical management might be a promising bridge to surgery strategy.

Validation of Inter-Rater and Intra-Rater Reliability of Remote Dielectric Sensing Measurement.

Remote dielectric sensing (ReDS) is a recently introduced non-invasive electromagnetic-based device used to quantify lung fluid levels. Nevertheless, its inter-rater and intra-rater reliability remain uncertain. In 10 healthy volunteers, ReDS values were measured three times successively by the officially trained expert examiner to validate intra-rater reliability. Similar measures were performed by a total of three examiners to validate inter-rater reliability. Intra-class correlation (ICC) was applied to validate each reliability. Ten healthy volunteers [median 34 (32, 40) years old, 10 men, body mass index 23.0 (21.2, 23.9) ] were included. Median ReDS value was 28% (25%, 31%). For the intra-rater reliability, ICC (1, 1) and ICC (1, 3) were 0.966 and 0.988, respectively (P < 0.001). For the inter-rater reliability, ICC (2, 1) and ICC (2, 3) were 0.683 and 0.866, respectively (P < 0.001). Given almost perfect intra-rater reliability, an examiner does not need to repeat ReDS measurement. Given substantial inter-rater reliability, ReDS measurements had better be measured by multiple examiners if possible.

Relationship Between Body Posture and Lung Fluid Volume Assessed Using a Novel Noninvasive Remote Dielectric Sensing System.

Background: The relationship between body posture and lung fluid level has not been quantified thus far. Remote dielectric sensing (ReDSTM) is a recently introduced non-invasive electromagnetic-based technology to quantify lung fluid percentage. Methods and Results: ReDS values were measured at different body postures (i.e., sitting, supine, and supine with legs elevated) in a healthy volunteer cohort (n=16; median age 39 years, 69% men, median [interquartile range {IQR}] body mass index 23.3 kg/m2 [21.0-26.2 kg/m2]). In the sitting position, the median ReDS value was 27% (IQR 25-29%). The ReDS value increased significantly in the supine position (median 28%; IQR 27-30%; P=0.009), and increased further upon leg elevation (median 29%; IQR 28-32%; P=0.001). Conclusions: In this proof-of-concept study, the relationship between body posture and lung fluid level was quantitatively validated in a healthy cohort.

Transcatheter closure of patent foramen ovale accompanying right-to-left shunt during Impella support.

We experienced a 65-year-old woman who was diagnosed as fulminant myocarditis and transferred on mechanical ventilator and veno-arterial extracorporeal membrane oxygenation (ECMO) supports. Impella 5.0 support was additionally initiated to improve pulmonary edema and unload left ventricle. We found a patent foramen ovale (PFO) at the time of Impella insertion by transesophageal echocardiography. Follow-up transesophageal echocardiography found a development of right-to-left shunt flow through PFO accompanying central hypoxia during Impella P8 support. Veno-arterial ECMO was converted to veno-arterio-venous ECMO and PFO was occluded percutaneously on the next day, which stabilized hemodynamics and systemic oxygen supply. In case of Impella 5.0 support, the existence of PFO and the development of right-to-left shunt flow should be carefully surveyed and closed immediately to maintain hemodynamics and systemic oxygen supply.

How to Estimate the Optimal Heart Rate in Patients with Heart Failure with Preserved Ejection Fraction.

Ideal heart rate (HR), particularly for those with heart failure with preserved ejection fraction (HFpEF), remains unknown. We hypothesized that cardiac output would be maximum when the overlap between E-wave and A-wave at the trans-mitral flow is "zero" in the Doppler echocardiography. We retrospectively investigated the association among the overlap length between two waves, actual HR, and other echocardiographic parameters to construct a formula for estimating theoretically ideal HR among those with HFpEF. In total, 48 HFpEF patients were included (70-year-olds, 18 males). Given the results of multivariate linear regression analyses, the overlap length was estimated as follows: -1,050 + 8.4 × (HR [bpm]) + 0.6 × (deceleration time [millisecond]) + 1.7 × (A-width [millisecond]), which had a strong agreement with the actually measured overlap length (r = 0.86, P < 0.001). Theoretically ideal HR was calculated by substituting zero into the estimated overlap length as follows: 125 - 0.07 × (deceleration time [millisecond]) - 0.20 × (A-width [millisecond]). In the validation cohort including another 143 HFpEF patients, the estimated overlap using the formula again had a strong agreement with the actually measured overlap (r = 0.72, P < 0.001). In this study, we proposed a novel formula for calculating theoretically ideal HR, consisting of deceleration time and A-width, in the HFpEF cohort. Clinical implication to optimize the HR targeting the theoretically ideal HR should be investigated in prospective studies.

Impact of the angle between aortic and mitral annulus on the occurrence of hemolysis during Impella support.

Despite optimal management, we sometimes experience refractory hemolysis requiring extensive device speed reduction or continuous hemodiafiltration following Impella implantation. However, pre-procedural predictors of such a refractory hemolysis remain unknown. In this study, we investigated the pre-procedural factors, including the echocardiographic narrow angle between aortic and mitral annulus, associating with the occurrence of refractory hemolysis following Impella insertion. We enrolled 26 patients (71 years, 65% male) who received Impella insertion between March 2018 and November 2019. Among baseline characteristics, the angle between aortic and mitral annulus, < 126.5°, was an independent risk factor of refractory hemolysis with an adjusted hazard ratio of 7.840 (95% confidence interval 0.925-66.44) and was associated with lower 30-day survival (64% vs. 100%, p = 0.0116). The narrow angle between aortic and mitral annulus might be a useful tool to risk-stratify the occurrence of refractory hemolysis following Impella insertion.

Impella support as a bridge to scheduled surgical repair of ventricular septal rupture.

We studied the experience of a 69-year-old woman with acute decompensated heart failure due to post-infarction ventricular septal rupture. She received mechanical hemodynamic support by Impella 2.5 (Abiomed, Danvers, MA, USA), percutaneous axial-flow left ventricular assist device, which improved intra-cardiac pressure, maintaining end-organ function. She was successfully bridged to the scheduled surgical repair of ventricular septal rupture following the 9-day Impella support and discharged on foot on the 32nd day. Impella might be a useful mechanical circulatory support device as a bridge tool to the scheduled surgical repair of ventricular septal rupture if used with careful daily management to prevent device-related comorbidities.