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Background: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity., Objective: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease., Materials and Methods: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes., Results: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein., Conclusion: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Modern CT scanners with lower radiation doses have resulted in large numbers of cardiac CTs being performed in children. As seen in adults, pediatric cardiac CT has the potential to demonstrate extracardiac variants and pathology that can occur in conjunction with congenital heart disease (CHD). Prior publications demonstrated a high incidence of extracardiac findings in various locations but the prevalence of urgent unexpected extracardiac findings in children is unknown., Objective: The purpose of this study was to describe the incidence, distribution and clinical significance of the extracardiac findings on pediatric cardiac CT at a tertiary referral center., Materials and Methods: We reviewed all reports (n = 648) for 554 children through young adults who received a cardiac CT study between Jan. 2, 2018, and March 10, 2020, at our tertiary referral pediatric hospital. We interrogated CT reports for extracardiac findings and categorized them by system (airway, pulmonary, abdomen, malpositioned lines and musculoskeletal). We then subclassified each of these findings by level of clinical importance based upon the need for intervention or treatment into low, medium or high importance. High-importance findings were confirmed with a focused chart review. If a patient had more than one CT with a persistent extracardiac finding, the finding was only counted once., Results: We identified 562 individual extracardiac findings, with one or more extracardiac findings present in 91% of the study population. Extracardiac findings with high clinical importance, requiring urgent attention or intervention, were present in 10% (57/554) of cases. The most common location of extracardiac findings was pulmonary (50%; 280/562), followed by airway (22%; 125/562) and abdomen (9%; 52/562)., Conclusion: Unexpected highly important extracardiac findings were found in 10% of patients. Therefore, extracardiac structures should be scrutinized for the timely identification of potentially highly important findings., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: As CT technology has advanced, techniques for pediatric cardiac CT in congenital heart disease have evolved from retrospective electrocardiography (ECG)-gating with relatively high radiation doses to lower-dose prospective ECG-gating and even single-beat gated scans. Despite these advances, coronary artery imaging in children remains challenging because of their small vessel size and high heart rates, often necessitating retrospective gating., Objective: Evaluate coronary artery visualization in pediatric patients (<20 years) who underwent low-dose high-pitch ECG-triggered scans and stratify the probability of coronary artery visualization based upon heart rate and body surface area (BSA)., Materials and Methods: Two hundred eleven high-pitch ECG-triggered studies from April 2014 to November 2017 were reviewed by two pediatric cardiac imagers in this retrospective study. Patient age, gender, BSA, average heart rate, heart rate variability and use of general anesthesia were recorded as well as dose-length product (DLP) and volumetric CT dose index (CTDI vol ). We assessed the coronary artery score using a 5-point scale, with score of ≥3 considered of diagnostic quality. We performed multivariate statistical analysis including logistic regression to analyze effects of heart rate and BSA., Results: Patient age range was 1 day to 19 years (median age 3 years). Heart rate range was 49-188 beats per minute (bpm; median 122 bpm) and BSA range was 0.15-2.07 m 2 (median 0.53 m 2 ). The origin and proximal coronary arteries were confidently seen (score ≥3) in 61% of studies in this cohort. Coronary artery visualization scores further increased with increased BSA (P<0.002) and with decreased heart rate (P<0.001). At heart rates <100 bpm or in patients with BSA>0.58, adequate coronary artery visualization was present 72% of the time., Conclusion: While in many patients the coronary artery origins are visualized using high-pitch ECG-triggered technique, the importance of coronary artery visualization needs to be weighed with the radiation dose penalty in individual patients to achieve optimal imaging.