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Quick Fire Cases

Quick Fire 1- Miscellaneous I

Bolt From the Blue: Cardiac Mass Disguised as Congenital Pulmonary Airway Malformation (CPAM) in a Neonate (QF_TH_016)

Thursday, January 30, 2025
8:30 AM – 8:40 AM East Coast USA Time
Location: Blue Room PreFunction
Description of Clinical Presentation: 1-day old infant, suspected of having fetal CPAM on OB ultrasound, was noted to have severe cardiomegaly on postnatal chest XR. Transthoracic echo was performed which showed a large cardiac mass without any evidence of cardiac tamponade. Based on echo, it was not clear if it was a pericardial or epicardial mass. ECG showed ventricular pre-excitation with non-specific ST segment changes. A cardiac MRI (CMR) was performed to help identify mass characteristics to determine clinical management.

Diagnostic Techniques and Their Most Important Findings: CMR with contrast was performed on a 1.5T Siemens Aera MRI scanner. Due to potential cardiovascular compromise from the large cardiac mass under anesthesia, decision was made to perform a non-sedate scan with feed and swaddle protocol. Cardiac mass protocol included T1W and T2W imaging with and without fat suppression, first pass perfusion imaging and late gadolinium imaging. Sequence parameters were adjusted to accommodate for patient size (2.8 kg birth weight) and heart rate (90-130 BPM during scan). 

Learning Points from this Case: CMR showed a large homogenous epicardial cardiac mass (25mm x 36 mm, Figure 1) involving the lateral wall of the left ventricle. Based on tissue characterization (see Figure 2), a large epicardial rhabdomyoma was suspected. Genetics was consulted to initiate work up for tuberous sclerosis. Given the natural history of rhabdomyomas for spontaneous regression, the patient was observed for any signs of clinical distress. Once deemed stable, they were discharged home with close outpatient surveillance. This case report highlights the role of CMR in identifying cardiac tumors. CMR data was invaluable in guiding patient management. It importantly highlights how CMR can provide clinically pertinent data in potentially high-risk neonates in a safe, non-invasive fashion without the use of sedation.