Elizabeth Profita, MD

Abstract

Pediatric heart transplantation (HT) continues to be limited by the shortage of donor organs, distance constraints, and the number of potential donor offers that are declined due to the presence of multiple risk factors.We report a case of successful pediatric HT in which multiple risk factors were mitigated through a combination of innovative donor utilization improvement strategies.An 11-year-old, 25-kilogram child with cardiomyopathy and pulmonary hypertension, on chronic milrinone therapy and anticoagulated with apixaban, was transplanted with a heart from a Hepatitis C virus positive donor and an increased donor-to-recipient weight ratio. Due to extended geographic distance, an extracorporeal heart preservation system (TransMedics OCS Heart) was used for procurement. No significant bleeding was observed post-operatively, and she was discharged by post-operative day 15 with normal biventricular systolic function. Post-transplant Hepatitis C virus seroconversion was successfully treated.Heart transplantation in donors with multiple risk factor can be achieved with an integrative team approach and should be taken into consideration when evaluating marginal donors in order to expand the current limited donor pool in pediatric patients.

View details for DOI 10.1111/petr.14584

View details for PubMedID 37470130

Abstract

OBJECTIVE: This document is designed to outline the definition, pathogenesis, diagnostic modalities and therapeutic measures to treat antibody-mediated rejection in children postheart transplant METHODS: Literature review was conducted by a Pediatric Heart Transplant Society (PHTS) working group to identify existing pediatric and adult studies on antibody-mediated rejection (AMR). In addition, the centers participating in PHTS were asked to submit their approach to diagnosis and management of pediatric AMR. This document synthesizes information gathered from both these sources to highlight a practical approach to diagnosing and managing a child with AMR postheart transplant. This document may not represent the practice at all centers in the PHTS and serves as a starting point to understand an approach to this clinical scenario.

View details for DOI 10.1111/petr.14398

View details for PubMedID 36377325

Abstract

BACKGROUND: Early detection of cardiac allograft rejection is crucial for post-transplant graft survival. Despite the progress made in immunosuppression strategies, acute cellular rejection remains a serious complication during and after the first post-transplant year, and there is a continued lack of consensus regarding its treatment, especially in pediatric transplant patients.METHODS: An open request was placed via the listserv to the membership of the Pediatric Heart Transplant Society (PHTS). Along with a broad literature search, numerous institutional protocols were pooled, analyzed and consolidated. A clinical approach document was generated highlighting areas of consensus and practice variation.RESULTS: The clinical approach document divides cellular rejection by International Society for Heart and Lung Transplantation grades and provides management strategies for each, including persistent cellular rejection.CONCLUSIONS: Cellular rejection treatment can be tailored to the clinical status, graft function, and the grade of cellular rejection. A case of mild and asymptomatic rejection may not require treatment, whereas a higher-grade rejection or rejection with graft dysfunction or hemodynamic compromise may require aggressive intravenous therapies, changes to maintenance immunosuppression therapy and augmented surveillance.

View details for DOI 10.1111/petr.14393

View details for PubMedID 36377327

View details for DOI 10.1111/petr.14360

View details for PubMedID 35854405

Abstract

Access to pediatric sub-specialty training is a critical unmet need in many resource-limited settings. In Rwanda, only two pediatric cardiologists are responsible for the country's clinical care of a population of 12 million, along with the medical education of all pediatric trainees. To strengthen physician training opportunities, we developed an e-learning curriculum in pediatric cardiology. This curriculum aimed to "flip the classroom", allowing residents to learn key pediatric cardiology concepts digitally before an in-person session with the specialist, thus efficiently utilizing the specialist for additional case based and bedside teaching.We surveyed Rwandan and US faculty and residents using a modified Delphi approach to identify key topics in pediatric cardiology. Lead authors from Rwanda and the USA collaborated with OPENPediatrics, a free digital knowledge-sharing platform, to produce ten core topics presented in structured videos spanning 4.5h. A mixed methods evaluation was completed with Rwandan pediatric residents, including surveys assessing knowledge, utilization, and satisfaction. Qualitative analysis of structured interviews was conducted using NVivo.Among the 43 residents who participated in the OPENPediatrics cardiology curriculum, 33 (77%) completed the curriculum assessment. Residents reported using the curriculum for a median of 8h. Thirty-eight (88%) reported viewing the curriculum on their personal or hospital computer via pre-downloaded materials on a USB flash drive, with another seven (16%) reporting viewing it online. Twenty-seven residents viewed the course during core lecture time (63%). Commonly reported barriers to utilization included lack of time (70%), access to internet (40%) and language (24%). Scores on knowledge assessment improved from 66.2% to 76.7% upon completion of the curriculum (p<0.001) across all levels of training, with most significant improvement in scores for PGY-1 and PGY-2 residents. Residents reported high satisfaction with the visuals, engaging presentation, and organization of the curriculum. Residents opined the need for expanded training material in cardiac electrocardiogram and echocardiogram and requested for slower narration by foreign presenters.Video-based e-learning via OPENPediatrics in a resource-limited setting was effective in improving resident's knowledge in pediatric cardiology with high levels of utilization and satisfaction. Expanding access to digital curriculums for other pediatric sub-specialties may be both an effective and efficient strategy for improving training in settings with limited access to subspecialist faculty.

View details for DOI 10.1186/s12909-022-03222-z

View details for PubMedID 35291997

Abstract

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA COVID-19 vaccine-associated myopericarditis (VAM) was noted. Only one adolescent with life-threatening complications was reported with no deaths at any of the participating institutions.

View details for DOI 10.1016/j.jpeds.2021.12.025

View details for PubMedID 34952008

Abstract

Background Previous studies suggest that infant heart transplant (HT) recipients are at higher risk of developing severe primary graft dysfunction (PGD) than older children. We sought to identify risk factors for developing severe PGD in infant HT recipients. Methods and Results We identified all HT recipients aged <1year in the United States during 1996 to 2015 using the Organ Procurement and Transplant Network database. We linked their data to ELSO (Extracorporeal Life Support Organization) registry data to identify those with severe PGD, defined by initiation of extracorporeal membrane oxygenation support for PGD within 2days following HT. We used multivariable logistic regression to assess risk factors for developing severe PGD. Of 1718 infants analyzed, 600 (35%) were <90days old and 1079 (63%) had congenital heart disease. Overall, 134 (7.8%) developed severe PGD; 95 (71%) were initiated on extracorporeal membrane oxygenation support on the day of HT, 34 (25%) the next day, and 5 (4%) the following day. In adjusted analysis, recipient congenital heart disease, extracorporeal membrane oxygenation, or biventricular assist device support at transplant, recipient blood type AB, donor-recipient weight ratio <0.9, and graft ischemic time 4hours were independently associated with developing severe PGD whereas left ventricular assist device support at HT was not. One-year graft survival was 48% in infants with severe PGD versus 87% without severe PGD. Conclusions Infant HT recipients with severe PGD have poor graft survival. Although some recipient-level risk factors are nonmodifiable, avoiding modifiable risk factors may mitigate further risk in infants at high risk of developing severe PGD.

View details for DOI 10.1161/JAHA.121.021082

View details for PubMedID 34184543

View details for DOI 10.1016/j.healun.2021.02.003

View details for PubMedID 33674153

Abstract

BACKGROUND: Indications for a heartliver transplantation (HLT) for Fontan recipients are not well defined. We compared listing characteristics, post-operative complications, and post-transplant outcomes of Fontan recipients who underwent HLT with those of patients who underwent heart-only transplantation (HT). We hypothesized that patients who underwent HLT have increased post-operative complications but superior survival outcomes compared with patients who underwent HT.METHODS: We performed a retrospective review of Fontan recipients who underwent HLT or HT at a single institution. Characteristics at the time of listing, including the extent of liver disease determined by laboratory, imaging, and biopsy data, were compared. Post-operative complications were assessed, and the KaplanMeier survival method was used to compare post-transplant survival. Univariate regression analyses were performed to identify the risk factors for increased mortality and morbidity among patients who underwent HT.RESULTS: A total of 47 patients (9 for HLT, 38 for HT) were included. Patients who underwent HLT were older, were more likely to be on dual inotrope therapy, and had evidence of worse liver disease. Whereas ischemic time was longer for the group who underwent HLT, post-operative complications were similar. Over a median post-transplant follow-up of 17 (interquartile range: 5-52) months, overall mortality for the cohort was 17%; only 1 patient who underwent HLT died (11%) vs 7 patients who underwent HT (18%) (p=0.64). Among patients who underwent HT, cirrhosis on pre-transplant imaging was associated with worse outcomes.CONCLUSIONS: Despite greater inotrope need and more severe liver disease at the time of listing, Fontan recipients undergoing HLT have post-transplant outcomes comparable with those of patients undergoing HT. HLT may offer a survival benefit for Fontan recipients with liver disease.

View details for DOI 10.1016/j.healun.2020.12.008

View details for PubMedID 33485775

View details for DOI 10.1007/s40472-020-00286-y

View details for Web of Science ID 000705322200005

Abstract

BACKGROUND: Currently, there are no simple tools to evaluate the acute heart failure (HF) symptom severity in children hospitalized with acute decompensated HF (ADHF). We sought to develop an inpatient HF score (HFS) that could be used as a clinical tool and for clinical trials.METHODS: Pediatric HF clinicians at Stanford reviewed the limitations of existing HFSs, which include lack of calibration to the inpatient setting, omission of gastrointestinal symptoms, need for multiple age-based tools, and scores that prioritize treatment intensity over patient symptoms. To address these, we developed an acute HFS corresponding to the 3 cardinal symptoms of HF: difficulty with breathing, feeding, and activity. The score was iteratively improved over a 3-year pilot phase until no further changes were made. The inter-rater reliability (IRR) across a range of providers was assessed using the final version. Peak HFSs were analyzed against mortality and length of stay (LOS) for all pediatric HF discharges between July and October 2019.RESULTS: The final HFS was a 4-point ordinal severity score for each of the 3 symptom domains (total score 0-12). Among clinicians who scored 12 inpatients with ADHF simultaneously, the intraclass correlation (ICC) was 0.94 (respiratory ICC=0.89, feeding ICC=0.85, and activity ICC=0.80). Score trajectory reflected our clinical impression of patient response to HF therapies across a range of HF syndromes including 1- and 2-ventricle heart disease and reduced or preserved ejection fraction. Among the 28 patients hospitalized during a 3-months period (N = 28), quartiles of peak score were associated with LOS (p < 0.01) and in-hospital mortality (p < 0.01): HFS 0 to 3 (median LOS of 5 days and mortality of 0%), HFS 4 to 6 (median LOS of 18 days and mortality of 0%), HFS 5 to 9 (median LOS of 29 days and mortality of 23%), and HFS 10 to 12 (median LOS of 121 days and mortality of 50%).CONCLUSION: This simple acute HFS may be a useful tool to quantify and monitor day-to-day HF symptoms in children hospitalized with ADHF regardless of etiology or age group. The score has excellent IRR across provider levels and is associated with major hospital outcomes supporting its clinical validity. Validation in a multicenter cohort is warranted.

View details for DOI 10.1016/j.healun.2020.08.002

View details for PubMedID 33032871

View details for DOI 10.1016/j.healun.2019.01.1310

View details for Web of Science ID 000468597800004

View details for DOI 10.1111/petr.13414

View details for Web of Science ID 000470844700017