A systematic review and meta-analysis of the prevalence of transthyretin amyloidosis in heart failure with preserved ejection fraction.

BACKGROUND: Heart failure with preserved ejection fraction is a complex clinical syndrome marked by different phenotypes and related comorbidities. Transthyretin amyloidosis is an underestimated phenotype. We aim to evaluate the prevalence of transthyretin amyloidosis in heart failure with preserved ejection fraction. METHODS: This meta-analysis was conducted according to PRISMA guidelines. A search strategy was designed to utilize PubMed/Medline, EMBASE, and Google scholar to locate studies whose primary objective was to analyze the prevalence of transthyretin amyloidosis in heart failure preserved ejection fraction. RESULTS: Of 271 studies initially identified, 5 studies comprising 670 patients were included in the final analysis. The prevalence of transthyretin amyloidosis was 11%. Patients with transthyretin amyloid cardiomyopathy were more likely to be males (RR 1.38; 95% CI 1.09 to 1.75; P<0.01; I2=37%), and more likely to have low voltage criteria on ECG (RR 2.98; 95% CI 1.03 to 8.58; P=0.04; I2=75%) compared with transthyretin negative group. They also have higher SMD of age (SMD 0.73; 95% CI 0.48 to 0.97; P<0.01; I2=0%), and NT-proBNP (SMD 0.48; 95% CI 0.02 to 0.93; P=0.04; I2=36%) compared with transthyretin negative group. On reported echocardiogram, they have higher SMD of mass index (SMD 0.77; 95% CI 0.27 to 1.27; P<0.01; I2=65%), posterior wall thickness (SMD 0.92; 95% CI 0.62 to 1.21; P<0.01; I2=0%), and septal wall thickness (SMD 1.49; 95% CI 0.65 to 2.32; P<0.01; I2=87%) compared with transthyretin negative group. CONCLUSION: Transthyretin amyloidosis affects 11% of HFpEF patients. Therefore, screening HFpEF patients at risk of cardiac amyloidosis is warranted.

Incremental value of P-wave indices for predicting left atrial dysfunction in patients with primary mitral regurgitation using speckle tracking echocardiography.

The correlation of P wave indices on surface ECG and phasic LA dysfunction in patients with significant primary mitral regurgitation (MR) due to the adverse LA adaptive structural and functional changes needs to be more studied. This study aims to investigate the diagnostic value of P wave indices to predict LA function assessed both by volumetric analysis using 3-dimensional (3D)echocardiography, and by strain analysis using speckle tracking echocardiography. (STE). The study included 107 subjects, we measured maximum P-duration (Pmax), P dispersion (PD), and V1 negative terminal force (V1-NTF) (negative duration x negative amplitude) on surface ECG. Both Basic and Dynamic LA volumes (LAV) during reservoir, conduit, and contractile phases were measured. The global LA strain and strain rate parameters were calculated By STE. LA ejection fraction (LAEF) and ejection force were also calculated.V1-NTF showed a significant positive correlation while P-max a significant negative correlation with global peak atrial longitudinal strain (GPALS) (r = 0.75; P < 0.001 and r = - 0.72; P < 0.001 respectively). Using ROC curve analysis, Pmax > 110 ms, 1-NTF ≥ 4 ms.mV and P notching > 40 ms had a sensitivity of 90%, 95% and 50% and a specificity of 87.4%, 94.3% and 100% respectively in predicting GPALS ≤ 30%. P notching > 40 ms was associated with severe LA dysfunction. ECG P wave indices represent a simple bedside tool that could have an incremental role in predicting LA dysfunction as well as size in patients with significant primary MR.

Novel concepts and early results of repairing common arterial trunk.

OBJECTIVES: Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the correction of all components of the anomaly, using autologous arterial tissue. This aims to enhance results, availability worldwide, and importantly to avoid the need for repeated reoperations. METHODS: From January 2019 to 4 January 2021, all patients with isolated CAT had repair of the defect using autologous arterial trunk tissue with direct right ventricle (RV) to pulmonary artery (PA) connection. Clinical outcomes, follow-up which included multi-slice computed tomography 3D segmentation and 4D cardiovascular magnetic resonance flow, are presented. RESULTS: Twenty patients were included in the study (median age 4.5 months). There were 2 hospital deaths due to systemic infection and pulmonary hypertensive crisis, respectively. Following discharge all patients remained asymptomatic with no signs of heart failure and improved pattern of growth (median follow-up: 8 months). Early postoperative 3D segmentation showed a conical shaped neo-right ventricular outflow chamber connecting the body of the RV to the main PA through a valveless ostium, and normal crossing of PA and neo-aorta. 4D cardiovascular magnetic resonance pattern of flow showed normal rapid laminar flow through the atrioventricular valves followed by a vortex towards the outflow tracts. There was laminar flow through the neo-aorta and neo-PA with velocity not exceeding 2.5 m/s. The PA regurgitant fraction was 25 ± 5% and was limited to early diastole. CONCLUSIONS: The initial results of utilizing the key concepts, using autologous arterial tissue for the repair of CAT, are encouraging, both clinically and by multimodality imaging.

Right ventricular functions in subphenotypes of sickle cell disease.

OBJECTIVES: Sickle cardiomyopathy is the most important cause of death in patients with sickle cell disease (SCD). Based on recent evidence, SCD can be divided into two subphenotypes, namely, the viscosity vasoocclusion (VVO) subphenotype and the hemolysis endothelial dysfunction (HED) subphenotype. The aim of our series is to study right ventricular (RV) functions in both subphenotypes. METHODS: Echocardiography including conventional and tissue Doppler imaging as well as speckle tracking echocardiography was performed in 50 patients (23 from the VVO subgroup and 27 from the HED subgroup) based on a serum lactate dehydrogenase (LDH) level below or above 270 U/L, respectively, and in 50 controls. Reticulocyte count and hemoglobin levels were assessed in different groups of patients. RESULTS: The HED subgroup showed RV dysfunction. Patients in this subgroup also showed systolic and diastolic functions similar to those seen in the VVO subgroup and controls. In addition, a tight correlation exists between LDH and both RV global longitudinal strain (-0.68) and RV E/E' ratio (0.9), defined as the ratio of early diastolic tricuspid inflow velocity to tricuspid annular early diastolic velocity. CONCLUSIONS: Results reveal a marked discrepancy in RV functions between HED and VVO subphenotypes of SCD, with patients in the former subgroup being more prone to RV dysfunction. This warrants early screening of such patients in daily practice.

Journey to the center of the heart: Bilateral absent superior vena cava with heart block.

Bilaterally absent superior vena cava (SVC) is extremely rare anomaly with a few case reports in the literature. Without associated congenital cardiac disease, these anomalies are asymptomatic. This report describes an adult patient with bilaterally absent SVC presenting with Mobitz type II heart block and a structurally normal heart.

Revival and modification of the Mustard operation.

OBJECTIVE: The neonatal arterial switch operation is currently the procedure of choice for patients with transposition of the great arteries. However, a large number of patients present too late for the arterial switch operation and are best managed with the atrial switch operation. METHODS: We have used the Mustard operation in its original form or following a new modification designed to enhance the atrial functions and filling of the left ventricle in an attempt to improve long-term results. RESULTS: Between July 2013 and November 2018, a total of 101 patients underwent the Mustard operation, 86 with the new modification. The median age at operation was 16 months (6 months to 27 years). A total of 75 patients (74.3%) were male. Median preoperative oxygen saturation was 71%. There were no early deaths and there were 3 late deaths during a median follow-up period of 24.2 months (all in patients with large ventricular septal defect and established pulmonary vascular disease). At the latest follow-up, all patients were in stable sinus rhythm. There were no baffle leaks. Seven patients had asymptomatic narrowing of the superior baffle, and 1 patient required balloon dilatation. Follow-up is 100% complete and includes computed tomography and magnetic resonance imaging at regular intervals (75 patients to date). Computerized analysis of representative subsets showed enhanced rate and pattern of filling of the left ventricle in the modified operation compared with the classic operation. CONCLUSIONS: The use of the Mustard operation, particularly the modified technique should play an important role in treating late-presenting patients with transposition of the great arteries. Improving the pattern of filling of the left ventricle could enhance the long-term results of the Mustard operation.

Effect of live oud music on physiological and psychological parameters in patients undergoing cardiac surgery.

Background. Music therapy has emerged as a promising evidence-based adjuvant method of intervention. This study aims to assess the effect of live oud music on physiological and psychological parameters in patients undergoing cardiac surgery, pre- and post-operatively. Methods. Twelve patients undergoing cardiac operations were randomly allocated into either intervention group or control group, six patients in each group. Patients in the intervention group listened to 20 minutes of improvised and personally customized live oud music before and after surgery while patients in the control group heard the normal hospital sounds. While anxiety scores were assessed preoperatively, vital signs and pain scores were assessed postoperatively together with serum levels of cortisol, which was used as a surrogate marker of the stress response. Results. In the intervention group, pain scores and respiratory rates showed statistically significant reduction after listening to music (P values of 0.043 and 0.034 respectively). Additionally, heart rates, anxiety scores and serum cortisol levels showed borderline significant reduction in patients who listened to music with P values, 0.063, 0.066 and 0.068 respectively. These changes were not found in the control group. Conclusions. This preliminary study suggests a role of live oud music therapy in decreasing stress response of the patients undergoing cardiac surgery, in addition to its positive effects on pain perception and anxiety scores.