CASE

Highlights • Isolated RSCA from the PA is a rare finding in d-TGA. • Atypical differential cyanosis in d-TGA may indicate abnormal aortic branching. • Fetal imaging can provide evidence of an isolated RSCA.

“Twelve significant photographs in any one year is a good crop.”- Ansel Adams This year, CASE will release an issue each month, expanding from six annual issues since its inaugural issue in 2017, to 10 issues this past year, to an even dozen issues starting in 2023. I thought this would be an ideal time to provide you an annual State-of-the-Journal update.

Introduction Rhabdomyomas are the most common cardiac tumors in the pediatric population, making up 50% to 80% of all primary pediatric tumors. They can be solitary or multiple. Multiple cardiac rhabdomyomas are usually found in association with the tuberous sclerosis complex (TSC), and more than 90% of fetuses and neonates with multiple rhabdomyomas have signs of TSC. Tuberous sclerosis complex is a rare autosomal dominant heritable neurocutaneous disorder with multisystem involvement.

Congenital aneurysms of the muscular interventricular septum are rare, which makes management during fetal life and postnatal life challenging since the natural history is unknown. 1 Carr M. Kearney D.L. Eidem B.W. Congenital aneurysm of the muscular interventricular septum. Ventricular septation is a complex process characterized by fusion of atrioventricular cushion extensions that form the membranous septum along with the process of compaction of trabeculations to form the muscular septum.

The patient was discussed with the pediatric interventional and cardiovascular surgical team, and a decision was made to attempt to close the defect with a percutaneous device if suitable. With this goal in mind, the patient was brought to the pediatric cardiac catheterization suite. A TEE was ordered to further delineate the anatomy prior to closure.

Highlights • QAV is a rare congenital malformation often missed on TTE. • Intervention is typically required in the fifth to sixth decade of life. • Transillumination on 3D TEE offers photorealistic 3D rendering. Introduction Quadricuspid aortic valve (QAV) is a rare congenital malformation that is diagnosed later in life following the development of significant valvular disease, predominantly aortic regurgitation.

Highlights • Cardiac tumors are rare but not uncommon. • There is no definitive treatment available for spindle cell sarcoma. • TTE (or TEE) is usually the initial imaging modality for these tumors. Introduction Cardiac tumors are an entity with low incidence. Classically they can be divided into nonneoplastic and neoplastic, with the latter further classified as primary and secondary. 1 Tyebally S. Chen D. Bhattacharyya S. Mughrabi A. Hussain Z. Manisty C. et al.

A 46-year-old woman presented to our institution with dyspnea and palpitations. Pertinent medical history included pregnancy at advanced maternal age, palpitations, decreased stamina, and anxiety. Physical examination findings were normal, with no evidence of volume overload. On auscultation, a holosystolic 2/6 murmur was detected.

A 39-year-old woman with no cardiovascular risk factors presented to the clinic for atraumatic wrist pain. Review of systems included palpitations. The patient denied chest pain, shortness of breath, dizziness, and syncope. On the follow-up visit, the patient mentioned experiencing palpitations lasting for 30 minutes that were associated with mild intermittent substernal chest pain radiating to the back and neck, which improved at rest.

Highlights • Bioprosthetic valve IE and systemic organ involvement suggest left-sided vegetation. • High bacteremia with bioprosthetic valve IE may be the cause for left-sided vegetation. • The crest of the ventricular septum may serve as a site for IE after patch repair of the VSD. Introduction Transcatheter pulmonary valve implantation (TPVI) has become an alternative to surgical valve replacement in patients with dysfunctional right ventricular outflow tract (RVOT).