Ectopia cordis is a rare congenital condition where the heart is located outside the chest cavity, often associated with midline defects like omphalocele and Pentalogy of Cantrell. This condition presents significant challenges in surgical management and has a high mortality rate due to complications related to cardiac and associated anomalies. A newborn delivered at 37 weeks gestation was admitted for surgical management of ectopia cordis and omphalocele. Prenatal imaging suggested Pentalogy of Cantrell. An echocardiogram was performed, but visualization was limited due to the heart’s external position, hindering comprehensive assessment. A follow-up CT angiogram confirmed ectopia cordis with complex cardiac and vascular findings. The left-sided superior vena cava drained into the coronary sinus, and the inferior vena cava was dilated, draining into the right atrium. The right atrium was dilated, while the left atrium was elongated with normal pulmonary venous drainage. A 4 mm atrial septal defect and a possible 2 mm perimembranous ventricular septal defect were noted. The right ventricle appeared normal, but the left ventricle was hypoplastic. The aortic root and ascending aorta were hypoplastic, with Z-scores ranging from -3.20 to -4.90, while the aortic isthmus and descending aorta were within normal limits. The pulmonary arteries were normal in size, and a large PDA was observed. 3D modeling was used to assess the feasibility of surgical repair and chest closure. The heart outside the chest measured 30.65 ml, and the total intrathoracic volume was 107.31 ml. The combined volume of the lungs and pleural effusions totaled 77.69 ml, leaving limited space for the heart within the thoracic cavity. This made the prospect of repositioning the heart into the chest highly challenging, with significant risk of compressing the lungs or other critical structures. The baby passed away during the hospital stay. This case highlights the role of CTA and 3D modeling in congenital heart disease, illustrating how these tools can assist in evaluating spatial constraints and informing surgical decisions in complex conditions like ectopia cordis. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Priya Sarv, Nagpal Prashant

Keywords: 3D Model, Pentalogy Of Cantrell, Cardiovascular

Pediatric polyarteritis nodosa (PAN) is a rare systemic vasculitis affecting medium-sized arteries, leading to inflammation and organ damage. It often involves the skin, kidneys, and gastrointestinal tract, with symptoms such as fever, abdominal pain, and hypertension. Early diagnosis and treatment are essential to managing this condition. This report discusses renal imaging findings in two pediatric patients with PAN. A 10-year-old male presented with abdominal pain, nausea, and acute renal failure. A left renal biopsy revealed medium vessel arteritis, raising suspicion for polyarteritis nodosa. Renal Doppler ultrasound showed normal renal arteries but increased echogenicity of the kidneys. CTA demonstrated patent renal arteries without beading, but bilateral cortical hypoenhancement was observed, indicating cortical necrosis consistent with vasculitis. MRA further confirmed the suspicion of PAN by revealing minimal contrast blushing and nodularity at the corticomedullary junction, suggestive of microaneurysms. Imaging played a key role in confirming the diagnosis of PAN in this patient. A 16-year-old male presented with a six-month history of abdominal pain, fatigue, weight loss, chills, and hypertension. Laboratory results showed elevated inflammatory markers. MRA of the chest, abdomen, and pelvis revealed numerous bilateral arterial enhancing foci within the renal parenchyma, measuring 2 to 5 mm, suggestive of microaneurysms. A renal biopsy confirmed secondary focal segmental glomerulosclerosis. CT and MR imaging are essential for diagnosing pediatric polyarteritis nodosa by identifying key vascular abnormalities such as microaneurysms and ischemic damage. These imaging techniques not only confirm the diagnosis but also guide timely intervention, improving the management of this rare condition in pediatric patients. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Priya Sarv, Nagpal Prashant

Keywords: Renovascular Hypertension, Vasculitis, Abdominal Imaging

This exhibit explores the microbolus injection technique in pediatric cardiac CT angiography, providing a comparison to traditional bolus and test bolus methods. The microbolus technique involves delivering multiple small, precisely timed boluses of contrast media, each followed by saline flushes, using a dual-head power injector. Unlike the standard bolus injection, which administers a single large volume of contrast, the microbolus technique dispenses several smaller doses of contrast at regular intervals, interspersed with saline. This approach synchronizes the contrast circulation with the patient’s cardiac output and vascular dynamics, ensuring enhanced opacification of key heart structures, particularly in pediatric patients. The saline flushes help maintain a steady flow and promote even distribution of the contrast, preventing pooling. The primary aim is to achieve simultaneous, uniform opacification of both right and left heart structures, including coronary arteries, pulmonary arteries, and systemic veins, all within a single scan. This precise timing and contrast distribution have the potential to reduce the need for repeat imaging caused by suboptimal visualization, thus potentially lowering radiation exposure and contrast volume. This educational exhibit will address the following: A comprehensive overview of the microbolus injection technique, highlighting its distinction from traditional bolus and test bolus injections. An explanation of injector requirements and how the precise timing of contrast and saline boluses is achieved. Potential advantages of achieving simultaneous opacification of both right and left heart structures. The possibility of reducing the need for repeat or delayed scans, potentially decreasing radiation exposure. A discussion of the technique's limitations and practical considerations for clinical implementation. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Priya Sarv, Nagpal Prashant

Keywords: Cardiovascular, CT Angiography, Teaching