Echocardiographic Indicators Associated with Adverse Clinical Course and Cardiac Sequelae in Multisystem Inflammatory Syndrome in Children with Coronavirus Disease 2019.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 causes significant cardiovascular involvement, which can be a determinant of clinical course and outcome. The aim of this study was to investigate whether echocardiographic measures of ventricular function were independently associated with adverse clinical course and cardiac sequelae in patients with MIS-C. METHODS: In a longitudinal observational study of 54 patients with MIS-C (mean age, 6.8 ± 4.4 years; 46% male; 56% African American), measures of ventricular function and morphometry at initial presentation, predischarge, and at a median of 3- and 10-week follow-up were retrospectively analyzed and were compared with those in 108 age- and gender-matched normal control subjects. The magnitude of strain is expressed as an absolute value. Risk stratification for adverse clinical course and outcomes were analyzed among the tertiles of clinical and echocardiographic data using analysis of variance and univariate and multivariate regression. RESULTS: Median left ventricular apical four-chamber peak longitudinal strain (LVA4LS) and left ventricular global longitudinal strain (LVGLS) at initial presentation were significantly decreased in patients with MIS-C compared with the normal cohort (16.2% and 15.1% vs 22.3% and 22.0%, respectively, P < .01). Patients in the lowest LVA4LS tertile (<13%) had significantly higher C-reactive protein and high-sensitivity troponin, need for intensive care, and need for mechanical life support as well as longer hospital length of stay compared with those in the highest tertile (>18.5%; P < .01). Initial LVA4LS and LVGLS were normal in 13 of 54 and 10 of 39 patients, respectively. There was no mortality. In multivariate regression, only LVA4LS was associated with both the need for intensive care and length of stay. At median 10-week follow-up to date, seven of 36 patients (19%) and six of 25 patients (24%) had abnormal LVA4LS and LVGLS, respectively. Initial LVA4LS < 16.2% indicated abnormal LVA4LS at follow-up with 100% sensitivity. CONCLUSION: Impaired LVGLS and LVA4LS at initial presentation independently indicate a higher risk for adverse acute clinical course and persistent subclinical left ventricular dysfunction at 10-week follow-up, suggesting that they could be applied to identify higher risk children with MIS-C.

Percutaneous Closure of Perimembranous Ventricular Septal Defects Using the Second-Generation Amplatzer Vascular Occluders.

Earlier attempts at percutaneous closure of perimembranous ventricular septal defects (Pm VSDs) were abandoned because of incidence of heart block likely as a result of device rigidity and/or oversizing. This is retrospective review and data reporting of patients who underwent percutaneous closure using the softer second-generation Amplatzer vascular occluders; namely the Amplatzer vascular plug, second generation, (AVP II) and the Amplatzer duct occluder, second generation (ADO II) in our institution. A total of 20 patients were identified; AVP II was used in 9 patients and ADO II in 11 patients. Median weight was 13.45 kg (range 6.5 to 76); age 28.5 months (range 11 to 352). After procedure, 4 were noted to have aortic insufficiency; trivial in 3 and mild in 1 (unrelated to the device). Mild tricuspid regurgitation possibly device or procedure related was seen in 4. Residual flow through the device was common after procedure and disappeared in all but 3, graded as trivial in 1, small in 2. Average follow-up period was 7.54 months ± 7.5 (1 day to 25 months). There was no incidence of heart block, bacterial endocarditis, hemolysis, device embolization, or fracture. The aortic insufficiency resolved in 1 patient and was estimated to be trivial in the remaining 3 patients. In conclusion, percutaneous closure of Pm VSDs using the softer new generation devices as the AVP II and the ADO II is feasible and safe. Longer follow-up and larger series are needed.

Porcine xenograft aortic root replacement in a three month old with severe truncal insufficiency.

Outcomes for truncus arteriosus repair are impacted significantly by the severity of truncal valve dysfunction. When satisfactory repair of the regurgitant truncal valve is unattainable, replacement is required. Given our experience in children with stentless porcine xenografts in the aortic position and the incidence of early valve failure for aortic homografts in infants, we replaced a severely regurgitant truncal valve with a full-root porcine xenograft in a 3-month-old infant. The initial and early result are encouraging, suggesting that the stentless porcine xenograft may be considered an option in cases where primary repair of the truncal (or aortic) valve is not possible.

Ventricular structure and function in children with sickle cell disease using conventional and tissue Doppler echocardiography.

Conventional 2-dimensional, M-mode, and spectral Doppler echocardiographic techniques have documented abnormal ventricular function in adults with sickle cell disease (SCD), but assessments in children are conflicting. Tissue Doppler echocardiography (TDE) provides additional information about myocardial function. Two-dimensional, M-mode, tricuspid regurgitation jet velocity (TRJV) data, and tissue Doppler echocardiographically derived myocardial velocity measurements of left ventricular (LV) and right ventricular function were taken from children with SCD compared to those of similar healthy historical controls and correlated with clinical characteristics and hemoglobin levels. Compared to 55 controls, 54 children with SCD (mean age 14.2 years, range 6 to 21) had a larger left ventricle, greater LV mass, and higher LV fractional shortening; 30% had increased pulmonary artery pressure (TRJV ≥2.5 m/s). Conventional echocardiographic measurements of LV systolic function and spectral Doppler measurements of LV and right ventricular diastolic function were essentially normal, but TDE indicated that 31% of SCD children had evidence of LV diastolic dysfunction (peak early diastolic velocity of LV inflow Doppler/peak early diastolic velocity at lateral mitral valve annulus >8), a finding that correlated with lower hemoglobin levels. Although decreasing hemoglobin levels in children with SCD correlated with LV hypertrophy, LV dilation, and LV diastolic dysfunction, long-term transfusion or hydroxyurea therapy did not affect these measurements. In conclusion, 1/3 of children with SCD had tissue Doppler echocardiographic evidence of LV diastolic dysfunction, which was correlated with hemoglobin levels. Adding serial assessments of ventricular function with TDE to conventional echocardiography may detect early cardiac changes, especially in children with severe anemia.