Management of acute pericarditis.

PURPOSE OF REVIEW: Provide an update on current management and most recent evidence in the treatment of pediatric pericarditis. RECENT FINDINGS: While treatment of acute pericarditis has not significantly changed over the last decade, management of recurrent acute pericarditis, with increased attention to autoinflammation as a causal mechanism, has evolved substantially. This includes clinical trial evidence that newer medications targeting interleukin-1 receptors are effective in recurrent forms of pericarditis. In addition, advanced imaging utilizing cardiac magnetic resonance has emerged as a particularly effective way to detect ongoing pericardial inflammation in support of more difficult-to-treat patients. SUMMARY: Recent advances in acute and recurrent pericarditis management have allowed for a more tailored approach to the individual patient. Yet, unresolved questions require further research.

Myopericarditis after messenger RNA Coronavirus Disease 2019 Vaccination in Adolescents 12 to 18 Years of Age.

OBJECTIVES: To characterize the clinical course and outcomes of children 12-18 years of age who developed probable myopericarditis after vaccination with the Pfizer-BioNTech (BNT162b2) coronavirus disease 2019 (COVID-19) messenger RNA (mRNA) vaccine. STUDY DESIGN: A cross-sectional study of 25 children, aged 12-18 years, diagnosed with probable myopericarditis after COVID-19 mRNA vaccination as per the Centers for Disease Control and Prevention criteria for myopericarditis at 8 US centers between May 10, 2021, and June 20, 2021. We retrospectively collected the following data: demographics, severe acute respiratory syndrome coronavirus 2 virus detection or serologic testing, clinical manifestations, laboratory test results, imaging study results, treatment, and time to resolutions of symptoms. RESULTS: Most (88%) cases followed the second dose of vaccine, and chest pain (100%) was the most common presenting symptom. Patients came to medical attention a median of 2 days (range, <1-20 days) after receipt of Pfizer mRNA COVID-19 vaccination. All adolescents had an elevated plasma troponin concentration. Echocardiographic abnormalities were infrequent, and 92% showed normal cardiac function at presentation. However, cardiac magnetic resonance imaging, obtained in 16 patients (64%), revealed that 15 (94%) had late gadolinium enhancement consistent with myopericarditis. Most were treated with ibuprofen or an equivalent nonsteroidal anti-inflammatory drug for symptomatic relief. One patient was given a corticosteroid orally after the initial administration of ibuprofen or an nonsteroidal anti-inflammatory drug; 2 patients also received intravenous immune globulin. Symptom resolution was observed within 7 days in all patients. CONCLUSIONS: Our data suggest that symptoms owing to myopericarditis after the mRNA COVID-19 vaccination tend to be mild and transient. Approximately two-thirds of patients underwent cardiac magnetic resonance imaging, which revealed evidence of myocardial inflammation despite a lack of echocardiographic abnormalities.

Non-sustained microvolt level T-wave alternans in congenital long QT syndrome types 1 and 2.

BACKGROUND: Patients with long QT syndrome (LQTS) are predisposed to polymorphic ventricular tachycardia (VT) during adrenergic stimulation. Microvolt T-wave alternans (MTWA) is linked to vulnerability to VT in structural heart disease. The prevalence of non-sustained MTWA (NS-MTWA) in LQTS is unknown. METHODS: 31 LQT1, 42 LQT2, and 80 controls underwent MTWA testing during exercise. MTWA tests were classified per standardized criteria, and re-analyzed according to the modified criteria to account for NS-MTWA. RESULTS: LQT1 and LQT2 patients had a significantly higher frequency of late NS-MTWA (26% and 12%) compared to controls (0%). There was no significant difference between the groups with respect to sustained and early NS-MTWA. Late NS-MTWA was significantly associated with QTc. CONCLUSION: LQT1 and LQT2 patients had a higher prevalence of late NS-MTWA during exercise than matched controls. NS-MTWA likely reflects transient adrenergically mediated dispersion of repolarization, and could be a marker of arrhythmic risk in LQTS.

Cardiac response to enzyme replacement therapy in infantile Pompe disease with severe hypertrophic cardiomyopathy.

Classic infantile-onset Pompe disease (IOPD), characterized by predominantly cardiac involvement, used to be considered uniformly lethal within months. The availability of enzyme replacement therapy (ERT) has transformed the course of the disease. Decrease in ventricular hypertrophy and improvement in ventricular function have been suggested as proof for efficacy. We report the cardiac response to ERT of a child with IOPD and severe hypertrophic cardiomyopathy. The myocardial hypertrophy resolved. Change in ejection fraction, however, was slow. We discuss the potential benefit of other parameters beyond ejection to assess cardiac function in IOPD, including speckle tracking-based strain.

Wearable Biosensors in Congenital Heart Disease: Needs to Advance the Field.

Traditional measures of clinical status and physiology have generally been based in health care settings, episodic, short in duration, and performed at rest. Wearable biosensors provide an opportunity to obtain continuous non-invasive physiologic data from patients with congenital heart disease (CHD) in the real-world setting, over longer durations, and across varying levels of activity. However, there are significant technical limitations to the use of wearable biosensors in CHD. Here, we review current applications of wearable biosensors in CHD; how clinical and research uses of wearable biosensors must consider various CHD physiologies; the technical challenges in developing wearable biosensors for CHD; and special considerations for digital biomarkers in CHD.

HeartWatch: Implementing a Pediatric Heart Center Program to Prevent Cardiac Arrests Outside the ICU.

Pediatric patients with cardiovascular disease are at increased risk of cardiopulmonary arrest. Despite utilization of Cardiac Pediatric Early Warning Scores to identify patients at risk of decompensation, our institution had a twofold increase in cardiac arrests (CAs) in the acute care cardiology unit (ACCU) over 2 years. Through a quality improvement initiative, we developed a watcher program, HeartWatch, to reduce the CA arrest rate in the ACCU by 50% over the first year of implementation. Methods: HeartWatch aims to identify patients not adequately captured by Cardiac Pediatric Early Warning Scores who are at high risk for sudden decompensation. Inclusion criteria were developed and evaluated during pilot and implemented phases (April 2020-April 2021) and then monitored in a sustained phase through June 2022. Our primary outcome was the reduction in the out-of-ICU CA rate. Results: During the 13 months, we enrolled 169 patients, and the CA rate decreased from 0.7 to 0.33 per 1,000 patient days, a 53% reduction. The CA rate further decreased to 0.28 events per 1,000 patient days, a 60% reduction, by June 2022. The most common indications for HeartWatch inclusion were high-risk single-ventricle patients (31%) and patients with diminished ventricular function (20%). Conclusions: Implementation of HeartWatch was associated with a meaningful reduction in CA in the ACCU. Creating shared mental models for high-risk patients is essential for patient safety. Future work will optimize local processes that focus on the sustainability of our gains. We will also evaluate opportunities to adapt and implement a similar framework in other institutions to assess reproducibility.

Velocity encoded mitral valve inflow cine: A novel and more reproducible method to determine cardiac rest periods during coronary magnetic resonance angiography.

A high temporal resolution, 4-chamber (4CH) cine is the standard method for determining cardiac rest periods during whole heart coronary magnetic resonance angiography (CMRA). We evaluated the image quality and reproducibility between the 4CH cine method and a novel approach using a velocity encoded mitral valve inflow cine (MVI). The goal of this study was to compare the quality of CMRAs utilizing MVI versus 4CH methods. Sharpness and vessel length for the LCA and RCA using each method were determined using Soap Bubble and two blinded observers independently assessed coronary image quality. Offline analysis on a separate, retrospective cohort (n = 25) was used to compare MVI and 4CH reproducibility. In the prospectively evaluated cohort there was no difference in overall vessel sharpness (4CH vs MVI mean ± SD) (31.0 ± 5.5% vs 30.5 ± 5.7%, p = .63), LCA vessel sharpness (30.0 ± 5.4% vs 31.1 ± 8.2%, p = .44), LCA length (4.7 ± 1.4 cm vs 4.6 ± 1.6 cm, p = .66), RCA vessel sharpness (32.1 ± 6.9% vs 31.1 ± 7.7%, p = .55), RCA length (5.51 ± 2.6 cm vs 5.95 ± 2.4 cm, p = .38), or image quality rating (2.66 vs 2.62, p = .80) between methods. In the retrospective cohort, the MVI method had 5.4% lower inter-observer variability (95% CI 3.7,7.2%, p < .0001) and 3.9% lower intra-observer variability (95% CI 2.4,5.4%, p < .0001) than the 4CH method. MVI is a technically feasible and more reproducible method to determine cardiac rest periods compared to 4CH while preserving vessel sharpness, vessel length & image quality.

Growth Differentiation Factor 15: A Novel Growth Biomarker for Children With Congenital Heart Disease.

BACKGROUND: Failure to thrive (FTT), defined as weight or height less than the lowest 2.5 percentile for age, is prevalent in up to 66% of children with congenital heart disease (CHD). Risk stratification methods to identify those who would benefit from early intervention are currently lacking. We aimed to identify a novel growth biomarker to aid clinical decision-making in children with CHD. METHODS: This is a cross-sectional study of patients 2 months to 10 years of age with any CHD undergoing cardiac surgery. Preoperative weight-for-age Z scores (WAZ) and height-for-age Z scores (HAZ) were calculated and assessed for association with preoperative plasma biomarkers: growth differentiation factor 15 (GDF-15), fibroblast growth factor 21, leptin, prealbumin, and C-reactive protein (CRP). RESULTS: Of the 238 patients included, approximately 70% of patients had WAZ/HAZ < 0 and 34% had FTT. There was a moderate correlation between GDF-15 and WAZ/HAZ. When stratified by age, the correlation of GDF-15 to WAZ and HAZ was strongest in children under 2 years of age and persisted in the setting of inflammation (CRP > 0.5 mg/dL). Diagnoses commonly associated with congestive heart failure had high proportions of FTT and median GDF-15 levels. Prealbumin was not correlated with WAZ or HAZ. CONCLUSIONS: GDF-15 represents an important growth biomarker in children with CHD, especially those under 2 years of age who have diagnoses commonly associated with CHF. Our data do not support prealbumin as a long-term growth biomarker.

What's That in the Aorta? A Case of Asymptomatic Dislodged Ostial Right Coronary Artery Stent That Was Noted as an Echodense Material in the Aortic Valve on Transesophageal Echocardiogram.

A 58-year-old female with a history of coronary artery disease (CAD) with remote percutaneous intervention (PCI) to ostial right coronary artery (RCA) with a bare-metal stent represented with unstable angina. Left heart catheterization (LHC) showed 90% stenosis of the previously stented ostial RCA with a moderate disease in the circumflex and left anterior descending arteries (LAD). LHC had also demonstrated that the previously placed ostial RCA stent, 19 years ago, was dislodged with only 3-4 mm within RCA and the remainder 10-12 mm in the ascending aorta. The patient miraculously had remained largely asymptomatic of this dislodged RCA stent for many years. Subsequent transthoracic echo (TTE) showed moderate-severe mitral regurgitation (MR). Therefore, she was worked up for a possible single-vessel coronary artery bypass graft surgery (CABG) with mitral valve replacement/repair. However, on transesophageal echo (TEE), MR was noted to be moderate in severity. Also, an echodense material was noted on the right coronary cusp (RCC) of the aortic valve, which was deemed to be the dislodged RCA stent. As the MR was moderate, the patient underwent successful complex PCI of ostial RCA.

Successful minimal approach transcatheter aortic valve replacement in an allograft heart recipient 19 years post transplantation for severe aortic stenosis: A case report.

BACKGROUND: Aortic stenosis is one of the rare valvular complications in a transplanted heart. Over the past 8 years, transcatheter approach for aortic valve replacement (TAVR) has been slowly evolving to be the preferred approach in these patient population when compared to the surgical approach. We report a second case in the United States with successful transfemoral minimal approach with minimal sedation for TAVR in a heart transplant recipient 19 years post transplantation for severe symptomatic calcified aortic stenosis. CASE SUMMARY: We present a case of 73-year-old male who has undergone successful minimal approach transcatheter aortic valve replacement in an allograft heart. Patient had received orthotopic heart transplantation 19 years ago for non-ischemic cardiomyopathy. Follow up transthoracic echocardiograms as per routine protocol did not show any aortic valve disease until 15 years post transplantation. Aortic valve was noted to be mildly sclerotic at that time and gradually progressed to severe symptomatic aortic stenosis over the next 4 years. Patient had complaints of worsening shortness of breath that limited his functional capacity. Overall his post heart transplantation period has been mostly uneventful except for allograft non occlusive vasculopathy and aortic stenosis. His Society of Thoracic Surgery risk score was 12.205% and he was considered to be a high-risk surgical candidate by surgeon. Decision was made to undergo transcatheter aortic valve replacement. CONCLUSION: With the improved survival of these patients, we think it is time to look into pathophysiology of valvular disease in transplant heart recipients. Some other unanswered questions include, underlying donor and recipient risk factors for valvular diseases in heart transplant recipients.