Objectives: Persistent truncus arteriosus represents less than 3% of all congenital heart defects. We aim to analyze mid-term outcomes after primary Truncus arteriosus repair at different ages and to identify the risk factors contributing to mortality and the need for intervention after surgical repair. Methods: This retrospective cohort study included 36 children, underwent repair of Truncus arteriosus in the period from January 2011 to December 2018 in two institutions. We recorded the clinical and echocardiographic data for the patients preoperatively, early postoperative, 6 months postoperative, then every year until their last documented follow-up appointment. Results: Thirty-six patients had truncus arteriosus repair during the study period. Thirty-one patients had open sternum post-repair, and two patients required extracorporeal membrane oxygenation. Bleeding occurred in 15 patients (41.67%), and operative mortality occurred in 5 patients (14.7%). Patients with truncus arteriosus type 2 (p = 0.008) and 3 (p = 0.001) and who were ventilated preoperatively (p < 0.001) had a longer hospital stay. Surgical re-intervention was required in 8 patients (22.86%), and 11 patients (30.56%) had catheter-based reintervention. Freedom from reintervention was 86% at 1 year, 75% at 2 years and 65% at 3 years. Survival at 1 year was 81% and at 3 years was 76%. High postoperative inotropic score predicted mortality (p = 0.013). Conclusion: Repair of the truncus arteriosus can be performed safely with low morbidity and mortality, both in neonates, infants, and older children. Re-intervention is common, preferably through a transcatheter approach.

Background/Objectives: Deep hypothermic circulatory arrest (DHCA) is safe, but subtle neurodevelopmental deficits may persist far beyond the perioperative period. We aimed to investigate the relationship between DHCA duration and neurodevelopmental outcomes in young children undergoing cardiac surgery with DHCA. Methods: Children aged < 42 months, including neonates who underwent cardiac surgery using DHCA without regional perfusion techniques, were included as the DHCA group. Children in the same age range who underwent cardiac surgery without DHCA were included as the control group. All enrolled patients underwent neurodevelopmental assessment using the Bayley Scales of Infant and Toddler Development (BSTID) by a trained pediatrician, and 17 DHCA patients and 6 control patients completed the BSTID assessment. Results: Both groups showed no significant preoperative, operative, or postoperative differences. Adjusted multivariable analysis revealed that prematurity and age at assessment were significant changing predictors of each of the BSTID components (p < 0.001), except for the gross motor component, where only age at assessment was a significant adjusting predictor. Longer DHCA was associated with lower fine and gross motor BSTID components; however, the association was not statistically significant (p = 0.06). Conclusions: Long-duration DHCA without regional perfusion techniques may be associated with less optimal neurodevelopmental outcomes.

The natural history of cardiac tumors is dismal, with death ensuing rapidly. This is especially true with incomplete resection (called R-2 resection). We present a patient with metastatic sarcoma, also involving the heart, too unwell to undergo chemotherapy, who underwent a debulking operation, resulting in early rapid recurrence but response from chemotherapy. Debulking cardiac tumors may be selectively offered to enable chemotherapy initiation.

Deep hypothermic circulatory arrest (DHCA) is safe, but subtle neurodevelopmental deficits may persist far beyond the perioperative period. We investigated the relationship between DHCA duration and neurodevelopmental outcomes in young children undergoing cardiac surgery with DHCA. Children aged < 42 months, including neonates who underwent cardiac surgery using DHCA without regional perfusion techniques, were included as the DHCA group. Children in the same age range undergoing cardiac surgery without DHCA were included as the control group. All patients underwent neurodevelopmental assessment using the Bayley Scales of Infant and Toddler Development (BSTID) by a trained pediatrician. Seventeen of 25 DHCA and six of 10 control patients completed the BSTID assessment. The groups showed no preoperative, operative, or postoperative significant differences. In adjusted multivariable analysis, prematurity and age at assessment were significant changing predictors of each of the BSTID components (p < 0.001), other than the gross motor component, where only age at assessment was a significant adjusting predictor. Longer DHCA was associated with lower fine and gross motor BSTID components; however, the association was not statistically significant (p = 0.06). Use of DHCA without regional perfusion techniques may be associated with less optimal neurodevelopmental outcomes if DHCA has a long duration.

Metastatic disease is a relative contraindication for resection of malignant cardiac tumors. However, certain situations may present themselves when primary cardiac resection may be warranted. We present a 21-year-old male diagnosed with metastatic epithelioid hemangioendothelioma with right ventricular outflow tract involvement for whom surgical resection was successfully performed and discuss strategies.

Left atrial masses are most commonly diagnosed as myxomas. When clinicians doubt the diagnosis, a biopsy is warranted. However, this can be very difficult with left-sided tumors. We present a 71-year-old woman with a presumed left atrial myxoma with features concerning for a malignant lesion. She underwent a computed tomography–fluoroscopy-guided percutaneous biopsy, revealing a diagnosis of malignant spindle cell neoplasm, thus avoiding surgery and allowing for early treatment.