Morphology, Clinical Associations, and Pathologic Follow-up of Quadricuspid Aortic Valves in Children.

While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y). Associated congenital heart diseases were present in 54% (most commonly tetralogy of Fallot (TOF) and valvar pulmonary stenosis). Among patients with isolated QAV, 11 had genetic syndrome and 5 had skeletal anomalies. Valve morphology was most commonly type B (41%) and A (35%; Hurvitz and Roberts). Dilated aortic root (Z ≥ 2) was present in 5 and dilated ascending aorta in 9 patients at diagnosis. All patients with type C (n = 3) and G (n = 1) had aortic dilation. At diagnosis, >mild AR was rare (n = 1), mild regurgitation was common (n = 12, 32%), >mild AS was rare (n = 2), and mild AS was uncommon (n = 4). Over a median follow-up of 3.3y (IQR 0.9-11y), progression of AR was seen in 2 patients and progression of aortic root or ascending aorta dilation (increase in Z score by ≥ 2) was seen in 5 patients. In conclusion, QAV is a rare congenital anomaly and about half of the cases are found in hearts that are otherwise structurally normal. A relatively high prevalence is seen in patients with TOF, pulmonary stenosis, skeletal deformities, and genetic syndromes. Meticulous evaluation of aortic valve morphology should be conducted on echocardiograms performed for these indications.

A comparison between the apical and subcostal view for three-dimensional echocardiographic assessment of right ventricular volumes in pediatric patients.

Background: Accurate measurement of ventricular volumes is an important clinical imaging goal. Three-dimensional echocardiography (3DEcho) is used increasingly as it is more available and less costly than cardiac magnetic resonance (CMR). For the right ventricle (RV), the current practice is to acquire 3DEcho volumes from the apical view. However, in some patients the RV may be better seen from the subcostal view. Therefore, this study compared RV volume measurements from the apical vs. the subcostal view, using CMR as a reference standard. Methods: Patients <18 years old undergoing a clinical CMR examination were prospectively enrolled. 3DEcho was performed on the day of the CMR. 3DEcho images were acquired with Philips Epic 7 ultrasound system from apical and subcostal views. Offline analysis was performed with TomTec 4DRV Function for 3DEcho images and cvi42 for CMR ones. RV end-diastolic volume and end-systolic volume were collected. Agreement between 3DEcho and CMR was assessed with Bland-Altman analysis and the intraclass correlation coefficient (ICC). Percentage (%) error was calculated using CMR as the reference standard. Results: Forty-seven patients were included in the analysis (age range 10 months to 16 years). The ICC was moderate to excellent for all volume comparisons to CMR (subcostal vs. CMR: end-diastolic volume 0.93, end-systolic volume 0.81; apical vs. CMR: end-diastolic volume 0.94, end-systolic volume 0.74).The 3DEcho mean % error vs. CMR for end-systolic volume was 25% for subcostal and 31% for apical; for end-diastolic volume it was 15% for subcostal and 16% for apical. The % error was not significantly different between apical vs. subcostal views for end-systolic and end-diastolic volume measurements. Conclusions: For apical and subcostal views, 3DEcho-derived ventricular volumes agree well with CMR. Neither echo view has a consistently smaller error when compared to CMR volumes. Accordingly, the subcostal view can be used as an alternative to the apical view when acquiring 3DEcho volumes in pediatric patients, particularly when the image quality from this window is superior.

Electrocardiogram Before Tricyclic Antidepressant Use: Minimal Impact in Pediatric Functional Gastrointestinal Disorders.

OBJECTIVES: The aim of this study was to determine the effect of electrocardiogram (ECG) findings on the initiation of tricyclic antidepressants (TCAs) for functional gastrointestinal disorders (FGIDs) and to evaluate cardiac outcomes related to low dose TCA use. METHODS: We performed a retrospective chart review of all pediatric outpatients at a tertiary pediatric hospital with an ECG ordered by a pediatric gastroenterologist when considering initiation of a TCA between January 2011 and February 2018. We collected demographics, previous cardiovascular testing results, TCA dosing, and pertinent outcomes, including cardiology referrals, emergency department, and hospital admissions, and death during the study period. All ECGs were reviewed for corrected QT (QTc) interval, heart rate, and other abnormalities. RESULTS: Of 233 patients with screening ECGs, most (84.1%) were prescribed a TCA. Functional abdominal pain or dyspepsia account for 82.0% of diagnoses. Initial TCA dosing of amitriptyline varied widely, 10-50 mg/day, and the dose was not associated with QTc intervals. TCAs were not started in only 1.7% (4/233) due to ECG results. A significant ECG abnormality prompting cardiology referral was found in eight (3.4%) with a prolonged QTc interval in one (0.4%). In 10.7% (25/233) of patients, screening ECG was obtained despite available ECG in the chart. No deaths and no emergency department or hospital visits for arrhythmia or drug overdose occurred. CONCLUSION: Screening ECGs infrequently influence TCA initiation and may lead to increased resource utilization. The overall frequency of cardiology referral due to ECG results is low. Serious adverse cardiac events are unlikely with low dose TCA administration.

Rapid regression of large cardiac rhabdomyomas in neonates after sirolimus therapy.

Cardiac rhabdomyomas are the most common tumours in children and are typically seen in association with the tuberous sclerosis complex. Although benign and often associated with spontaneous regression, in rare circumstances surgical resection is indicated to relieve obstruction or other mass-related effects. Recent clinical trials have demonstrated the benefits of mammalian target of rapamycin inhibitors for the treatment of other tumour sub-types associated with tuberous sclerosis. Here we report rapid regression of several massive cardiac rhadomyomas in two neonates with the use of the mammalian target of rapamycin inhibitor sirolimus.

Accuracy of computed tomographic angiography compared to digital subtraction angiography in the diagnosis of intracranial stenosis and its impact on clinical decision-making.

BACKGROUND: Few studies to date have examined the accuracy of computed tomographic angiography (CTA) compared to digital subtraction angiography (DSA) in diagnosing intracranial stenosis. The purpose of this study was to compare CTA to DSA in diagnosing intracranial stenosis and to explore the impact of the addition of DSA on the management of stroke patients. METHODS: We retrospectively reviewed all ischemic stroke or patients with transient ischemic attack who underwent CTA and DSA within 30 days of each other at our institution between January 2008 and July 2011. For each study, 2 blinded observers rated the degree of stenosis of 11 intracranial vessels. Disagreements were adjudicated by a third blinded observer. Sensitivity, specificity, negative predictive value, and receiver operating characteristic curves were determined using DSA as the criterion standard. All patient charts were reviewed to determine if the addition of DSA to CTA impacted clinical management. RESULTS: Six hundred twenty-seven arterial segments were reviewed. The sensitivity of CTA to diagnose stenosis >50% was 96.6% (95% confidence interval [CI] 88.1-99.6), specificity 99.4% (95% CI 98.1-99.9), and negative predictive value 99.6% (95% CI 98.4-99.9). The intraclass correlation between CTA and DSA measurements was 0.96 (95% CI 0.95-0.97). Five of 57 patients underwent intracranial stenting procedures during the study period. All 5 lesions were correctly characterized as having >70% stenosis on CTA. Of the remaining 52 patients, none had clinical management change based on DSA findings. CONCLUSIONS: CTA has a high sensitivity and specificity compared to DSA to diagnose intracranial stenosis. The addition of DSA to CTA may not affect clinical management in most patients with suspected stenosis.