Context Bicuspid aortic valve is the most common congenital cardiac anomaly in the adult population. Cardiac outcomes in a contemporary population of adults with bicuspid aortic valve have not been systematically determined. Objective To determine the frequency and predictors of cardiac outcomes in a large consecutive series of adults with bicuspid aortic valve. Design, Setting, and Participants Cohort study examining cardiac outcomes in 642 consecutive ambulatory adults (mean [SD] age, 35 [16] years; 68% male) with bicuspid aortic valve presenting to a Canadian congenital cardiac center from 1994 through 2001 and followed up for a mean (SD) period of 9 (5) years. Frequency and predictors of major cardiac events were determined by multivariate analysis. Mortality rate in the study group was compared with age-and sex-matched population estimates. Main Outcome Measures Mortality and cause of death were determined. Primary cardiac events were defined as the occurrence of any of the following complications: cardiac death, intervention on the aortic valve or ascending aorta, aortic dissection or aneurysm, or congestive heart failure requiring hospital admission during the follow-up period. Results During the follow-up period, there were 28 deaths (mean [SD], 4% [1%]). One or more primary cardiac events occurred in 161 patients (mean [SD], 25% [2%]), which included cardiac death in 17 patients (mean [SD], 3% [1%]), intervention on aortic valve or ascending aorta in 142 patients (mean [SD], 22% [2%]), aortic dissection or aneurysm in 11 patients (mean [SD], 2% [1%]), or congestive heart failure requiring hospital admission in 16 patients (mean [SD], 2% [1%]). Independent predictors of primary cardiac events were age older than 30 years (hazard ratio [HR], 3.01; 95% confidence interval [CI], 2.15-4.19; PϽ.001), moderate or severe aortic stenosis (HR, 5.67; 95% CI, 4.16-7.80; PϽ.001), and moderate or severe aortic regurgitation (HR, 2.68; 95% CI, 1.93-3.76; PϽ.001). The 10-year survival rate of the study group (mean [SD], 96% [1%]) was not significantly different from population estimates (mean [SD], 97% [1%]; P=.71). At last follow-up, 280 patients (mean [SD], 45% [2%]) had dilated aortic sinus and/or ascending aorta. Conclusions In this study population of young adults with bicuspid aortic valve, age, severity of aortic stenosis, and severity of aortic regurgitation were independently associated with primary cardiac events. Over the mean follow-up duration of 9 years, survival rates were not lower than for the general population.

In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.

Background Emerging reports raise concerns on the potential association between the COVID-19 vaccines and cardiac manifestations. We sought to evaluate cardiac complications associated with COVID-19 vaccination in a pooled analysis from our institution’s cohort study and systematic review. Methods Consecutive patients admitted in a tertiary hospital in Singapore between 1 January 2021 and 31 March 2021, with onset of cardiac manifestations within 14 days following COVID-19 vaccination were studied. Furthermore, a systematic review was performed, with PubMed, Embase, Research Square, MedRxiv, and LitCovid databases accessed from inception up to 29 June 2021. Relevant manuscripts reporting individual patient data on cardiac complications following COVID-19 vaccination were included. Results Thirty patients were included in the study cohort, with 29 diagnosed with acute myocardial infarction (AMI) and 1 with myocarditis. Five patients developed heart failure, two had cardiogenic shock, three intubated, and one had cardiovascular-related mortality. In the systematic review, 16 studies were included with 41 myocarditis and six AMI cases. In the pooled analysis of the study cohort and the systematic review, 35 patients had AMI and 42 had myocarditis. Majority were men, and myocarditis patients were younger than AMI patients. Myocarditis patients tended to present 72 hours post-vaccination, while AMI patients were older and typically presented 24 hours post-vaccination. Majority with AMI or myocarditis developed symptoms after the first and second vaccination dose respectively. Conclusions This pooled analysis of patients presenting with cardiac manifestations following COVID-19 vaccination highlights the differences between myocarditis and AMI presentations in temporal association with the vaccination.

Background The transition of right ventricle (RV) from a compensated to decompensated state contributes to survival in pulmonary arterial hypertension (PAH). This study investigates the significance of right atrial (RA) dysfunction on disease progression in PAH. Methods Eighty patients with PAH, including 58 with hemodynamically compensated RV function (PAH-C) and 22 with decompensated RV function (PAH-D), were compared with 80 age-matched and sex-matched normal controls. RA longitudinal strain and strain rate (SR) parameters corresponding to reservoir (total strain ε s and strain rate SR s ), conduit (passive strain ε e and strain rate SR e ), and booster pump (active strain ε a and strain rate SR a ) phases were derived by a rapid semiautomated method on cine cardiovascular magnetic resonance. Results In PAH compared with controls, significantly reduced RA strains and SRs were observed. Among patients with PAH, PAH-D had significantly impaired RA strains and SRs compared with PAH-C. RA total strain and passive strain were the best parameters for differentiating PAH-D from PAH-C. Lower RA strain correlated with increased RA pressure ( r =−0.57; P <0.0001), RV volume ( r =−0.37; P =0.002) and biomarker ( r =−0.53; P <0.0001), impaired RV function ( r =0.46–0.72; P <0.0001), and lower exercise capacity ( r =0.41; P <0.0001). Reduced RA strains were significantly associated with higher risk of clinical worsening in PAH. RA passive strain was the best predictor of a composite adverse event end point (Harrell’s C statistic,0.75; hazard ratio,0.84; P =0.019) compared with other conventional RA and RV functional measurements. Conclusions RA phasic functions are impaired in PAH. Among patients with PAH, impaired RA strains reflect RV decompensation and higher risks and predict adverse clinical outcomes. Clinical Trial Registration https://www.clinicaltrials.gov . Unique identifier: NCT02790918.