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Background Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients’ first year of life. Methods The Pediatric Health Information System database was used to identify by ICD-10 code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n = 2227) and for whom one-year DAOH could be calculated. DAOH quartiles were used to group patients for analysis. Results Median one-year DAOH was 304 (interquartile range [IQR] 250-327), including a median index admission length of stay of 43 days (IQR 28-77). Patients required a median 2 (IQR 1-3) readmissions, each spanning 9 days (IQR 4-20). One-year readmission mortality or hospice discharge occurred in 6% of patients. Patients with lower-quartile DAOH had a median DAOH of 187 (IQR 124-226), whereas upper-quartile DAOH patients had a median DAOH of 335 (IQR 331-340) ( P 7 days at surgery (1.50 [1.14-1.99]), and non-white race/ethnicity (1.33 [1.01-1.75]). Conclusions In the current era, surgically palliated HLHS infants spend approximately 10 months alive and outside of the hospital, although outcomes are highly variable. Knowledge of the factors associated with lower DAOH can inform expectations and guide management decisions.

We report the case of a young female with juvenile idiopathic arthritis presenting with cardiac tamponade secondary to an unusual pericardial mass. Pericardial masses are typically incidental findings. In rare circumstances they can cause compressive physiology warranting urgent intervention. She required surgical excision which revealed a pericardial cyst encapsulating a chronic solidified hematoma. Though certain inflammatory disorders are associated with myopericarditis, to our knowledge this is the first reported case of a pericardial mass in a well-controlled young patient. We theorize her immunosuppressant therapy resulted in hemorrhage into a pre-existing pericardial cyst, suggesting the need for further follow-up in those on adalimumab therapy.

FERREIRA, David L S. Sistemas ERP: uma contribuição ao estudo do ciclo de vida em uma Instituição Federal de Ensino Superior, in Gestão Universitária / Breno Augusto Diniz Pereira, Fernando Carvalho Silva (Org.). – São Luís: Edufma, 2016. p.195-237., sistemas ERP, ciclo de vida de sistemas, universidade federal do maranhão

Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke remains notable. The purpose of this study was to analyze how strokes on VAD support impact post-transplant (post-Tx) outcomes in children.About 520 pediatric (18 years) heart transplant candidates listed from January 2011 to April 2018 with a VAD implant date were matched between the United Network of Organ Sharing and Pediatric Health Information System databases. Patients were divided into pre-Tx Stroke and No Stroke cohorts.About 81% of the 520 patients were transplanted; 28% (n = 146) had a pre-Tx Stroke; and 59% (n = 89) of the Stroke patients were transplanted at a median of 57 (IQR 17-102) days from stroke. Significantly more No Stroke cohort (90%) were transplanted (p 0.001). There was no difference in post-Tx survival between the Stroke and No Stroke cohorts (p = 0.440). Time between stroke and transplant for patients who died within 1 year of transplant was 32.0 days (median) compared to 60.5 days for those alive1 year (p = 0.18). Regarding patients in whom time from stroke to transplant was more than 60 days, one-year survival of Stroke vs. No Stroke patients was 96% vs. 95% (p = 0.811), respectively.Patients with stroke during VAD support, once transplanted, enjoy similar survival compared to No Stroke patients. We hypothesize that allowing Stroke patients more time to recover could improve post-Tx outcomes. Unfortunately, the ideal duration of time between stroke and safe transplantation could not be determined and will require more detailed and larger studies in the future.

The SynCardia temporary total artificial heart (TAH-t) is a pneumatically driven pulsatile device that replaces a patient's ventricles and all four valves. It is now available in two sizes and can be used in patients with sufficient chest size as a bridge to transplant or destination therapy. We describe our experience at a children's hospital with the TAH-t. Seven patients implanted with a TAH-t at Cincinnati Children's Hospital Medical Center from November 2012 to March 2021 were included. Duration of support ranged from 10 to 414 days with a median of 27 days. There was a trend toward decreased time to extubation and intensive care unit (ICU) length of stay, with the three most recent patients being extubated on postoperative day 1. Seventy-one percent of the patients were successfully transplanted following their TAH-t placement. Children and young adults with transplant graft failure requiring durable, biventricular support with a TAH-t had superior outcomes (75% survival to retransplant) than previously described.

Introduction: After Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation. Methods: In this prospective single-arm trial, 15 individuals with Fontan circulation were evaluated at baseline and after four weeks of therapeutic treatment with ISDN. Primary aims were to assess the safety of ISDN and the effect on maximal exercise (peak oxygen consumption, peak VO2). We also aimed to assess the effect of ISDN on ultrasound-assessed liver stiffness, markers of submaximal exercise, and PVP at rest and peak exercise. Repeated measures t-tests were used to assess change in variables of interest in response to ISDN. Results: Mean age was 23.5 ± 9.2 years (range 11.2-39.0 years), and 10/15 (67%) were male. There was no statistically significant change in peak VO2 (1401±428 to 1428±436 mL/min, p=0.128), but VO2 at the anaerobic threshold increased (1087 + 313 to 1115 + 302 mL/min, p= 0.03). ISDN was also associated with a lower peak exercise PVP (22.5 + 4.5 to 20.6 + 3.0 mmHg, p=0.015). Liver stiffness was lower with ISDN, though the difference was not statistically significant (2.3 + 0.4 to 2.1 + 0.5 m/s, p=0.079). Of the patients completing the trial, mild headache was common (67%), but there were no major adverse events. Conclusions: Treatment with ISDN for 4 weeks is well-tolerated in patients with a Fontan circulation. ISDN is associated with an increase in VO2 at anaerobic threshold, lower peak PVP, and a trend towards lower liver stiffness. Larger, longer studies will be necessary to define the impact of ISDN on clinical outcomes in the Fontan circulation. Clinical Trial Registration: URL: https//clinicaltrials.gov. Unique identifier: NCT04297241

Supplemental material, sj-docx-1-pch-10.1177_21501351221122971 for Defining Expectations for Infants With Hypoplastic Left Heart Syndrome Who Survive Initial Surgical Palliation by Jason W. Greenberg, Farhan Zafar, David S. Winlaw, James S. Tweddell, David G. Lehenbauer, James F. Cnota, Haleh C. Heydarian and David L. S. Morales in World Journal for Pediatric and Congenital Heart Surgery

Congenital heart disease (CHD) is common in conjoined twins but reports of surgical repair in this population are rare. We present the case of a conjoined twin diagnosed prenatally with a complete common atrioventricular canal defect (CAVC). Due to wound complications from twin separation, definitive repair was deferred and pulmonary arterial banding was performed initially. At 6 months, a complete CAVC repair was performed. The present case adds to the literature on successful cardiac surgical repairs in conjoined twins.