Right Atrial Enlargement on Electrocardiogram in Previously Healthy Young Patients.

OBJECTIVES: To assess whether right atrial enlargement (RAE) on electrocardiogram (ECG) correlates with true RAE on echocardiogram in previously healthy young patients and to understand which patients with RAE on ECG may warrant additional testing. STUDY DESIGN: A single-center, retrospective review of previously healthy young patients with (1) ECGs that were read as RAE by a pediatric cardiologist and (2) echocardiograms obtained within 90 days of the ECG. ECGs were reviewed to confirm RAE and determine which leads met criteria. The echocardiograms were then reviewed and RA measurements with z scores obtained. A z score >2 was considered positive for RAE on echocardiogram. RESULTS: In total, 162 patients with median age 10.8 years were included in the study. A total of 23 patients had true RAE on echocardiogram, giving a positive predictive value (PPV) of 14%. In patients <1 year of age, the PPV increased to 35%. In patients older than 1 year, the PPV was low at 7%. Patients with true RAE were more likely to meet criteria for RAE in the anterior precordial leads (V1-V3) (48% vs 5%, P < .001) and meet criteria for right ventricular hypertrophy (22% vs 6%, P = .023). CONCLUSION: Our findings show that RAE on ECG has a low PPV for RAE on echocardiogram in previously healthy young patients. The highest yield for RAE on echocardiogram was observed in patients who were <1 year of age, had RAE in the anterior precordial leads, or displayed right ventricular hypertrophy on ECG.

Occlusion of the os of the left coronary artery by dysplastic aortic valve tissue presenting as progressive mitral insufficiency and cardiac arrest.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction. Such coronary artery abnormalities include anomalous left coronary artery from pulmonary artery, left main coronary artery atresia, or a thromboembolic phenomenon. In this study, we describe a newborn with a dysplastic aortic valve causing obstruction of the os of the left coronary artery leading to progressive mitral insufficiency.

Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease.

OBJECTIVE: To test for associations between abnormal respiratory ciliary motion (CM) and brain abnormalities in infants with congenital heart disease (CHD) STUDY DESIGN: We recruited 35 infants with CHD preoperatively and performed nasal tissue biopsy to assess respiratory CM by videomicroscopy. Cranial ultrasound scan and brain magnetic resonance imaging were obtained pre- and/or postoperatively and systematically reviewed for brain abnormalities. Segmentation was used to quantitate cerebrospinal fluid and regional brain volumes. Perinatal and perioperative clinical variables were collected. RESULTS: A total of 10 (28.5%) patients with CHD had abnormal CM. Abnormal CM was not associated with brain injury but was correlated with increased extraaxial cerebrospinal fluid volume (P < .001), delayed brain maturation (P < .05), and a spectrum of subtle dysplasia including the hippocampus (P < .0078) and olfactory bulb (P < .034). Abnormal CM was associated with higher composite dysplasia score (P < .001), and both were correlated with elevated preoperative serum lactate (P < .001). CONCLUSIONS: Abnormal respiratory CM in infants with CHD is associated with a spectrum of brain dysplasia. These findings suggest that ciliary defects may play a role in brain dysplasia in patients with CHD and have the potential to prognosticate neurodevelopmental risks.

Transcatheter management of mid-aortic syndrome secondary to myofibroma presenting in infancy with severe left ventricular dysfunction: a case report.

In this study, we summarise a case of a myofibroma causing mid-aortic syndrome due to obstruction of the distal thoracic and abdominal aorta leading to severe left ventricular dysfunction. The patient was managed with percutaneous intervention via balloon dilation and stent placement. On follow-up, the patient has normalisation of ventricular function, is off anti-hypertensives, and is being monitored for re-stenosis.

Left ventricular myocardial performance index change for detection of acute cellular rejection in pediatric heart transplantation.

EMB, the gold standard for diagnosis of ACR, poses unique risks in children. Limited cross-sectional data have associated LV MPI with ACR. We hypothesize that a relative change in MPI from baseline without ACR to the time of ACR will better detect ACR than an absolute threshold LV MPI value. We identified 40 children with ACR ≥60 days post-transplant matching them by age and time from transplantation to 40 children without ACR. There was a significant increase in LV MPI at time of ACR vs. baseline (0.59 ± 0.17 vs. 0.41 ± 0.11; p < 0.001). There was no difference in LV MPI between baseline and follow-up (0.41 ± 0.11 vs. 0.42 ± 0.11; p = 0.65). An absolute increase in LV MPI of ≥0.47 had 82.5% sensitivity and 85% specificity for ACR, whereas an increase in LV MPI from baseline of ≥20.4% was 90% sensitive and 100% specific. Serial measurement of LV MPI appears to be a sensitive and specific marker of ACR. LV MPI shows good interobserver agreement and increases at the time of EMB-proven ACR with subsequent resolution to baseline measurements upon EMB-proven resolution of ACR. Future studies in larger, prospective cohorts should be undertaken to validate these findings.

Midterm survival of infants requiring postoperative extracorporeal membrane oxygenation after Norwood palliation.

This study reports the mid-term survival for neonates undergoing extracorporeal membrane oxygenation (ECMO) after Norwood palliation at a single center. Limited data exist on the mid-term survival of patients undergoing ECMO after Norwood palliation. We reviewed our ECMO experience from July 1994 to October 2008 and compared two groups: patients who required ECMO after Norwood palliation and patients who underwent Norwood palliation without ECMO. We analyzed 30-day survival, survival to hospital discharge, and survival to most recent follow-up. One hundred sixty patients underwent Norwood palliation for hypoplastic left heart syndrome (HLHS) and its variants. A total of 32 patients (20%) required postoperative ECMO. Using Kaplan-Meier analysis, the predicted survival rates for Norwood/non-ECMO patients to 30 days, 1 year, and 3 years after the procedure are 87.6% (CI 79.5-91.5%), 62.5% (CI 54.3-71.0%), and 59.9% (CI 50.8-67.8%), respectively. Survival to 30 days, 1 year, and 3 years after Norwood was significantly decreased in Norwood/ ECMO patients, with predicted survival rates of 50.0% (CI 31.9-65.7%), 24.6% (CI 11.4-40.4), and 13.2% (CI 3.9-28.3%), respectively (p < 0.0001). Risk factors for hospital mortality included nonelective or emergency placement onto ECMO, longer duration of ECMO support, and the development of acute renal failure while on ECMO. Of the original Norwood/ECMO hospital survivors, only half of these patients survived a mean of nearly 4 years. ECMO after Norwood palliation is associated with significant mortality. Our data suggest that neonates who require ECMO after Norwood palliation are prone to continued attrition once discharged from the hospital.

The Use of Automated Atrial CMR Measures and a Novel Atrioventricular Coupling Index for Predicting Risk in Repaired Tetralogy of Fallot.

Atrial size and function have been recognized as markers of diastolic function, and diastolic dysfunction has been identified as a predictor of adverse outcomes in repaired tetralogy of Fallot (rTOF). This was a retrospective single-center study with the objective of investigating the use of atrial measurements obtained via CMR for predicting outcomes in rTOF patients. Automated contours of the left and right atria (LA and RA) were performed. A novel parameter, termed the Right Atrioventricular Coupling Index (RACI), was defined as the ratio of RA end-diastolic volume to right ventricle (RV) end-diastolic volume. Patients were risk-stratified using a previously validated Importance Factor Score for the prediction of life-threatening arrhythmias in rTOF. Patients with a high-risk Importance Factor Score (>2) had a significantly larger minimum RA volume (p = 0.04) and RACI (p = 0.03) compared to those with scores ≤2. ROC analysis demonstrated RACI to be the best overall predictor of a high-risk Importance Factor Score (AUC 0.73, p = 0.03). Older age at the time of repair and a diagnosis of pulmonary atresia were associated with a larger RACI. Automated atrial CMR measurements are easily obtained from standard CMRs and have the potential to serve as noninvasive predictors of adverse outcomes in rTOF.

Identifying Food Insecurity in Cardiology Clinic and Connecting Families to Resources.

BACKGROUND: Food insecurity (FI) increases children's risk for illness and developmental and behavioral problems, which are ongoing concerns for congenital heart disease (CHD) patients. In 2020, 14.8% of households with children suffered from FI. The Hunger Vital Signs (HVS) asks 2 questions to assess FI. The global aim of the project is to implement HVS and connect FI families to resources. METHODS: Stakeholders identified 6 critical drivers in implementing FI screening at an outpatient cardiology clinic and conducted plan-do-study-act (PDSA) cycles to implement HVS. Over the 13-month study period, time series analyses were performed to assess our process measure (FI screening) and outcome measure (connection of FI families to resources). Demographics and severity of CHD were analyzed for FI families. RESULTS: Screening rates increased from 0% to >85%, screening 5064 families. Process evaluations revealed roadblocks including screening discomfort. FI families were more likely to identify as Black or multiple or other ethnicity. Severe CHD patients were at higher risk for FI (n = 106, odds ratio [OR] 1.67 [1.21-2.29], P = .002). Face-to-face meetings with social work and community partnerships reduced loss to follow-up and our ability to offer all FI families individualized FI resources. CONCLUSION: HVS screening can be implemented in a cardiology clinic to improve identification of FI families. A written tool can combat screening discomfort and improve identification of FI families. Children with severe CHD may be at increased risk for FI. A multidisciplinary team and community partnerships can improve individualized resource distribution.

Acquired toxoplasmosis after orthotopic heart transplantation in a sulfonamide-allergic patient.

We report the case of a young adult with a history of an allergic reaction to a sulfonamide antibiotic who developed toxoplasmosis after his second orthotopic heart transplant. As a result of this drug allergy, the patient did not receive prophylaxis with trimethoprim and sulfamethoxazole. He was successfully treated with clindamycin, pyrimethamine, and folic acid.