These posters have been presented at meetings in Children's Mercy and around the world. They represent research that was done at the time they were created, and may not represent medical knowledge or practice as it exists at the time viewers access these posters.
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Optic Disc Pit Maculopathy Leading to Vision Loss in a Pediatric Patient
Allyson Hall
This case will review optic disc pit maculopathy in children and treatments available. An emphasis in vision rehabilitation will be discussed as any visual insult during this development period can lead to long-term visual consequences.
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Optimizing oral glucose tolerance test completion at a pediatric cystic fibrosis care center: A 10-year continuing quality improvement effort
Christopher M. Oermann, Paula Capel, Jessica Haynes, Michelle Fischer, and Jill Kohmetscher
Background: Cystic fibrosis–related diabetes (CFRD) is a common comorbidity among people with CF (PwCF). It is associated with weight loss, protein catabolism, lung function decline, and increased mortality. Nutritional status and pulmonary function begin to decline in PwCF several years before the diagnosis of CFRD. Early CFRD detection and aggressive insulin therapy have been shown to reduce the mortality gap between PwCF who have CFRD and those who do not. The Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes recommend annual screening for people with CF starting at age 10 [1]. Methods: In 2011, team members at Children’s Mercy Kansas City (CMKC) embarked on a quality improvement (QI) project focused on improving oral glucose tolerance test (OGTT) completion rates in PwCF. During the initial phase of this project, QI methodology including fishbone diagrams and process flowcharts were employed to identify barriers to obtaining OGTTs. Patient education materials (English and Spanish) detailing the importance of and process for completing OGTTs were developed and distributed annually. A database for tracking PwCF who are greater than 10 years old and require OGTT was created. Weekly monitoring of upcoming appointments helped ensure that testing opportunities were not missed. Efforts were made to schedule OGTTs with annual laboratory testing to reduce phlebotomy. PwCF who wished to schedule with a local laboratory or provider were encouraged to do so and were provided with outside orders as needed. When PwCF in this group were admitted to the hospital, every attemptwas made to complete OGTTs near the end of their hospitalization. Results: Due to the lack of a standardized process and education, previous OGTT screening rates were poor: 9% in 2008, 13% in 2009, and 25% in 2010. During the first year of standard interventions (2011), the rate rose to 77%. By identifying barriers and standardizing our process, OGTT completion rates have continued to rise. In 2019 our OGTT completion rate was 92%, and in 2020—despite the COVID-19 pandemic which eliminated 3 months of testing opportunities—it was 81%. In recent years, endocrinology has partnered with the CF team in monthly CF/endocrinology “combo clinics,” which allow PwCF who have impaired glucose tolerance or CFRD to be evaluated by an endocrinology provider during their routine CF clinic visit. Conclusion: This QI project was initiated in 2011 and quality improvement work has continued to the present day. Continued education of PwCF and their families, tracking of testing, and commitment to sustained quality have allowed CMKC to attain high rates of OGTT completion. Earlier identification of impaired glucose tolerance and CFRD has allowed for earlier interventions, including dietary modifications, exercise recommendations, and endocrinology involvement in the plan of care.
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Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution
Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier, and Claire Elson
Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution S. Duehlmeyer1, C. Oermann1, E. Meier1, E. Elson1. 1Pulmonology, Children’s Mercy Kansas City, Kansas City, USA Background: Antibiotic therapy is essential for the treatment of cystic fibrosis (CF) lung infections. Methicillin-resistant Staphylococcus aureus (MRSA) infects 20% to 25% of people with CF (PwCF) and is associated with increased morbidity. Treatment of pulmonary exacerbations (PEs) often requires hospitalization including respiratory treatments and intravenous (IV) antimicrobials. IV vancomycin, which is commonly used for MRSA infections, requires serum concentration monitoring to ensure efficacy and minimize toxicity. Previous guidelines recommended trough concentrations to monitor efficacy and toxicity. Updated guidelines now recommend area under the curve (AUC) modeling as the optimal parameter for monitoring IV vancomycin. Methods: Children’s Mercy Kansas City (CMKC) changed IV vancomycin monitoring from trough to AUC/minimum inhibitory concentration (MIC) modeling on 01 May 2020 for PwCF. Two serum concentrations, a postdistributive and a trough, are obtained to estimate the AUC/MIC. A retrospective chart review collected trough monitoring data for all PwCF that received IV vancomycin at CMKC from01 January 2019 to 31 December 2019. Data for all PwCF treated with IV vancomycin after the AUC monitoring change were collected through 19 March 2021. Information on patient demographics, details of IV vancomycin therapy (dose, frequency, total exposure, nephrotoxicity), and monitoring data (serum concentrations, AUC modeling) were collected. Descriptive statistics were used to assess pre- and post-implementation data. Results: Before AUC monitoring, 25 patients received 42 courses of IV vancomycin; 14 were female (56%), and the median age was 14.02 years (4.25–20.25). Median treatment duration was 9.62 days (1.79–26.54), and median daily vancomycin exposure was 71.43 mg/kg/day (49.58–99.29). Target vancomycin trough concentration (≥15 μg/mL) was reached during 18 courses (43%). The median time to therapeutic trough was 83.58 hours (11.55–273.55) and required a median of 3 phlebotomies (1–9). Post-AUC there have been 15 courses of IV vancomycin in 8 PwCF; 5 were female (63%), and the median agewas 17.96 years (7.60–20.10). Median treatment duration was 9.52 days (5.68–14.63), and median daily vancomycin exposure was 75 mg/kg/day (48.63–92.80). All treatment courses reached target vancomycin AUC/MIC (400–600 μg/mL*h); median time to therapeutic AUC/MIC was 20.13 hours (11.6–106.12) and required a median of 3 phlebotomies (2–8). A median trough of 10 μg/mL (7–15 μg/mL) correlated with an AUC within target range. Conclusion: Changing to AUC monitoring for IV vancomycin in PwCF was not associated with a significant change in vancomycin daily exposure or duration. Fifty-seven percent more individuals achieved therapeutic targets with AUC monitoring (n = 15, 100%) than with trough monitoring (n = 18, 43%). AUC monitoring decreased time to therapeutic target by 63.45 hours. Trough concentrations of 15 μg/mL or less correlated with target AUC/MIC. A difference in nephrotoxicity was not seen. Study limitations include short postimplementation period (10 months) and small sample size. Ongoing data collection is planned.
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A Proposal for Developing Academic Partnerships between American Clinical Institutions and NGOs
Anik Patel, Nahreen Ahmed, Alfredo Mena Lora, and Riley Jones
"Humanitarian and health-focused non-governmental organizations, such as MedGlobal, and American clinical institutions can mutually benefit from the formation of academic partnerships. MedGlobal has a longitudinal presence in ongoing humanitarian disasters that can provide rich clinical experiences for academic institutions looking to provide more diverse and equity-driven training for its residents and fellows. American institutions can provide resources, personnel with special skills and knowledge, as well as research assistance to MedGlobal and its partner sites as it continues to promote evidence-based clinical care for refugees. We propose an innovative model that addresses 3 areas of opportunities: education, research/QI, and clinical care. By regularly distributing a "menu" of opportunities dictated by needs on the ground, US academic programs can participate up to their capabilities and skill sets. These opportunities could include virtual lectures and modules, tele-health consultations, creating trainee rotations, participating in rigorous research and QI projects, holding in-person train-the-trainer sessions such as with neonatal resuscitation or ultrasound, and creating a vetted roster of American providers from partnered programs that could be deployed in the setting of acute disaster response. This model could be replicated by other NGOs working in the humanitarian space to improve education, knowledge, and clinical care for some of the most vulnerable patients in the world."
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Assessing Early Use and Complications of Gastrostomy Blended Feeds.
James Fraser, Kristen L. Sayers, Amy L. Pierce, Beth A. Orrick, Wendy Jo Svetanoff, Tolulope A. Oyetunji MD MPH, and Shawn D. St Peter
Providers are hesitant to recommend using blended tube feeds (BF) after gastrostomy tube (GT) placement due to increased risk of bacterial contamination, nutrition inadequacy, tube blockages, and lack of data addressing clinical outcomes. Caregivers often feel that BF are more natural, better tolerated, and more cost-effective. We studied early use of BF, potential complications, and satisfaction among caregivers.
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Descriptive Study of the Safety Behaviors and Attitudes of Portable Pool Owners
Kristyn Jeffries, Kathy W. Monroe, Alicia Webb, Kristin L. Chancellor, Justina C. Goldman, and David C. Schwebel
Background Drowning is the leading cause of injury death for children 1-4 years old and the second leading cause for children 5-9 years old. Most prior epidemiology work has focused on submersions in below-ground swimming pools and natural bodies of water. Portable pools pose a new and emerging risk for drowning due to their affordability, convenience, and easy assembly. Successful drowning prevention consumer products, such as 4-sided fencing, may prove more difficult to implement with portable pools, and currently are not marketed for such use. Furthermore, parental perceptions and knowledge of drowning risks associated with portable pools has not yet been well studied.
Methods We performed a prospective study of caregivers to children aged 9 months to 6 years in an urban pediatric emergency department during summer 2021. Enrolled caregivers were given a QR code that directed them to complete a self-administered questionnaire on their mobile device. Survey questions assessed the caregivers’ access to portable pools and their safety behaviors and attitudes related to portable pools. Frequencies of portable pool ownership, caregivers’ safety practices while using them, and caregivers’ behavioral perceptions were calculated.
Results Of the 85 caregivers enrolled in the study, 54% reported either owning or having access to a portable pool. Of the subset who owned portable pools, a majority (n=23/28) bought their portable pool in 2021, but only 28% (n=8/28) used any safety products with their pool and only 10% had previously enrolled their child in formal swim lessons. The primary reasons portable pool owners did not use safety products included perceived lack of necessity of such products for portable pools and confidence in close supervision while their child is swimming. While all caregivers (n=85/85) responded they would always watch their child in the shallow end of a below-ground pool, 14% (n=12/85) of caregivers responded they would only watch their child intermittently while in a portable pool. Over 48% (n=41/85) of caregivers thought they would hear their child if he/she was drowning in a portable pool.
Conclusions Parents may underestimate the risk associated with portable pools, which could contribute to young children’s risk of drowning in these pools. These results provide insights that could be used in the development of drowning prevention messaging and the development of prevention strategies specifically targeting portable pool users.
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Pharmacogenetic Testing In Patients with Autism Spectrum Disorder Evaluated in a Pediatric Precision Medicine Clinic
Rachel Goodson, Cy Nadler, Jennifer A. Wagner, Sarah Soden, Sarah Nyp, and Tracy L. Sandritter
Pharmacogenetic Testing In Patients with Autism Spectrum Disorder Evaluated in a Pediatric Precision Medicine Clinic Purpose The purpose of this study is to investigate the demographic and presentation profiles of children with autism spectrum disorder (ASD) who present for evaluation in a pediatric precision medicine clinic. Methods: This retrospective, observational cohort study utilized data extracted from a pediatric precision medicine clinic database between 2010 and 2021 with recorded ICD9/10 codes of Autism Spectrum Disorder, Autistic Disorder, Pervasive Developmental Disorder, or Asperger’s Syndrome. Extracted variables included demographic data, presenting medication regimens and concerns to be addressed by precision medicine. Results: A sample of 202 patients was identified (see Table 1). Patients referred for precision medicine services were primarily due to poor medication response (64.8%) and/or adverse drug reactions (48.5%). Referrals were made by subspecialists (78.2%), primary care providers (16.3%), and via self-referral (4.95%). At presentation to the clinic, patients were already prescribed between 1-10 medications (Mean = 6.15, Median 5; see Figure 1). Medications with indication commonly used for sleep, gastrointestinal disorders, and psychiatric/behavioral disorders were among the most common medications taken at the time of evaluation (see Figure 2). At time of presentation to the clinic, males and females did not differ in terms of age (t= 1.22, p = 0.22) or number of medications taken (t = 0.994, p = 0.323). Age was also not significantly associated with number of medications (F = 0.277, p = 0.527). Conclusions: Youth with ASD presenting for precision medicine consultation experienced notable degrees of polypharmacy, with no clear differences associated with sex or age. Trends may emerge with the addition of a typically developing control group, and the robustness of associations must be evaluated in samples drawn from a wider variety of practice settings. While these findings are primarily descriptive, the data fill a critical gap regarding the characteristics of youth with ASD referred for precision medicine. Few dedicated precision medicine clinics exist, yet this service is increasingly recommended for youth with autism due to higher rates of adverse drug reactions and clinical nonresponse. More research is needed to establish how precision medicine fits within existing evidence-based guidelines and can most effectively serve this population.
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Racial Disparities in Testicular Torsion
Kayla B. Briggs, Obiyo O. Osuchukwu, Chris E. Roberts, James Fraser, Wendy Jo Svetanoff, Tolulope A. Oyetunji, and Hanna Alemayehu
Racial Disparities in Testicular Torsion Introduction: Testicular torsion (TT) is a surgical emergency requiring prompt intervention to preserve testicular function. Race and insurance status are thought to be associated with higher rates of gonadal loss; however, reports in the literature are conflicting. We sought to determine the effect of race on the treatment and outcomes of testicular torsion. Methods: Following IRB approval, a retrospective review was conducted of patients <18 years of age who presented to 2 institutions>(hereby referred to as H1 and H2) with acute scrotal pain between December 2017 and September 2019. Demographic data, clinical history, imaging results, diagnosis, and surgical outcomes were recorded. Social vulnerability index (SVI), as determined by zip code, was obtained from the Centers for Disease Control and dichotomized using the 75th percentile. Higher SVI denotes increased social vulnerability. Primary outcome was diagnosis of TT. Secondary outcomes included orchiectomy rates. A p-value <0.05 was considered significant. Results: A total of 515 patients (H1 85%, H2 15%) were included in the final analysis. There were no difference in median age, BMI, TT diagnosis and orchiectomy rate between the two institutions. H2 treated more black patients (43% vs. 14%, p=<0.01) and patients with public insurance (57% vs. 41%, p=0.03) compared to H1. In multivariate analysis, older age was associated with TT (median age TT 14.12 years [12.13,15.69]) vs. no TT 9.86 years [5,13.05], OR 1.35, 95% CI 1.18-1.55, p=<0.01). Black patients were ~4 times more likely (OR 4.05, 95% CI 2.13-7.69, p=<0.01) than white patients to be diagnosed with TT when controlled for dichotomized SVI, insurance, and age. In the same model, Hispanic patients were ~2 times more likely (OR 2.20, 95% CI 1.10-4.40, p=0.03) to be diagnosed with TT when compared to white patients. Despite more black and Hispanic patients having dichotomized SVI >75th percentile than white patients (70% and 82%, respectively vs. 16%, p=<0.01), dichotomized SVI was not found to be associated with higher rates of TT or orchiectomy, respectively. Conclusion: At the two centers, more black and Hispanic children had SVI >75th percentile, however, when controlling for this in multivariate analysis, they were significant more likely to be diagnosed with testicular torsion. Conversely, there was no difference in orchiectomy rate between races.
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Severe diastolic dysfunction following prolonged extracorporeal membrane oxygenation in a pediatric burn patient.
Cara Holton, Johanna I. Orrick, Igor Areinamo, and Jenna Miller
A previously healthy 3-year-old female was admitted following a 22% TBSA scald burn. She was initially hemodynamically stable on room air. However, one week into hospitalization she developed ARDS with catecholamine-refractory shock requiring VA-ECMO. She was enterovirus positive, grew MRSA in respiratory cultures and was treated with prolonged antibiotics. The patient spent 26 days on VA-ECMO followed by 61 days on VV- ECMO after failing decannulation due to refractory hypoxia. She developed anuric renal failure requiring CRRT during her second ECMO course. Following VV-ECMO decannulation, she required mechanical ventilation with high settings and had severe recurrent pulmonary hemorrhage requiring FFP, platelets and inhaled TXA. She had coronary dilation on CT imaging but was COVID-19 antibody negative. Serial echocardiograms demonstrated moderate LV dilation with normal systolic function. It was difficult to assess her diastolic function by echo, but with clinical concern for dysfunction she was started on milrinone. She underwent cardiac catheterization due to pulmonary bleeding and concerns for pulmonary hypertension. She had severely elevated biventricular filling pressures (RVEDp of 38 and LVEDp of 55) indicative of diastolic dysfunction. She had a high baseline cardiac index and normal PVR on milrinone and sildenafil. She also had significant AP collateral burden requiring coiling. Following this procedure her pulmonary hemorrhage resolved but her pulmonary mechanics did not improve. She continued to have multi-system organ failure with cardiac dysfunction, respiratory and renal failure. Her family did not wish to pursue organ transplantation and chose to redirect her care. This case is remarkable for several reasons. While large burns are known to produce an inflammatory response with cardiac dysfunction (1, 2), typically the degree of inflammation is proportional to the size of the burn and occurs early (3). This patient had a smaller TBSA burn that did not require surgery, yet she developed profound respiratory failure and shock requiring ECMO. During her hospitalization and 87 days on ECMO, the patient developed severe diastolic heart failure, AP collateral burden and coronary ectasia. This case demonstrates the utility of a diagnostic cardiac catheterization to evaluate for hemodynamic abnormalities after prolonged ECMO support, especially when echocardiogram is unrevealing. It is impossible to say whether these findings were a result of her prolonged ECMO runs, her burn physiologic state, or potentially a synergistic combination of the two. Additionally, while “long run” ECMO is becoming more common, little is known about the long-term cardiovascular effects of ECMO physiology. We report this case to encourage others to share cardiovascular complications after ECMO runs greater than 21 days.
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Short and Long Term Outcomes of Using Cryoablation for Post-operative Pain Control in Patients After Pectus Excavatum Repair
James Fraser, Kayla B. Briggs, Wendy Jo Svetanoff, David Juang, Pablo Aguayo, Jason D. Fraser, Charles L. Snyder, Tolulope A. Oyetunji, and Shawn D. St. Peter MD
Background: Previous prospective trials have demonstrated that intercostal cryoablation reduces length of stay and postoperative opioid consumption compared with thoracic epidural and patient controlled analgesia (PCA) modalities. We report the findings of a 3-year prospective, observational study to elucidate long-term pain control, symptoms, and complications with attention to postoperative short and long-term pain control associated with the cryoablation technique.
Methods: Following IRB approval, we prospectively collected data on patients who underwent bar placement for pectus excavatum with thoracoscopic intercostal cryoablation by six surgeons at our institution from 2017 to 2021. Patients and their parents completed surveys regarding pain scores, narcotic and non-narcotic pain medication usage, and limitations to activity beginning on the day of surgery for up to 10 days, with a supplementary survey on the day of discharge to evaluate pain and associated symptoms. Surveys were administered at two-week and three-month follow-up appointments addressing pain control, symptoms, and limitations to activity. Retrospective review of chart data was performed to identify the number of emergency department visits, phone calls to the outpatient surgery office, and requirement for additional pain medication.
Results: 110 patients were included in the analysis; 90% were male with a median age of 15.8 years [14.6, 16.7]. The median length of stay was 25.6 hours [22.3, 31.7]. Forty-eight patients (44%) completed the discharge survey; sharp pain and pressure on the first night of surgery were the most described pain characteristics, with sharp pain being the worst symptom reported most frequently in the middle of the chest. (Table 1) On postoperative follow-up, 55% of patients reported tolerable residual pain at two weeks and 41% at three months, with a median pain score of 3 at each follow-up interval and 25% of survey respondents requiring intermittent non-narcotic pain medication at three months. There were 18 (16%) ED visits by two-week follow-up, resulting in 3 readmissions for inadequate pain control: two on postoperative day two and one on postoperative day five. By three-month follow-up, there were 65 calls to the surgery clinic, most commonly for persistent pain and frequent popping sensation with movement, and no additional patients required readmission for pain control within this longer interval. (Table 2)
Conclusion: Although cryoablation is an excellent pain control modality to attenuate the acute insult of bar placement, these data suggest that patients underreport functional symptoms and experience more frequent discomfort and alteration of activities of daily living than perceived by providers. Prospective validation is necessary to examine the nature of specific symptoms, duration, and impact on the patient’s quality of life. -
Improving Skin and Soft Tissue Antibiotic Duration Concordance with National Guidelines in Pediatric Urgent Care Clinics
Megan Hamner, Amanda Nedved, Holly Austin, Donna Wyly, Alaina N. Burns, Diana King, Brian Lee, and Rana El Feghaly
Introduction: Skin and soft tissue infections (SSTIs) are the second most common diagnosis leading to pediatric antibiotic prescriptions in the outpatient setting after respiratory diagnoses. Children with SSTIs often receive >7 days of antibiotics, although current guidelines recommend 5-7 days for most diagnoses. At CMH urgent care clinics (UCCs), only 58% patients received the recommended 5-7 days of antibiotics. We aimed to increase the percentage of patients receiving 5-7 days of oral antibiotics for SSTIs from 58% to 75% by December 31st, 2021. Methods: We formed a multidisciplinary team in April 2020. A provider survey assessed factors influencing prescribing habits. We completed cause-and-effect analyses and developed a driver diagram (Figure 1). Interventions were chosen based on the potential for highest impact and lowest effort. Our first Plan-Do-Study-Act (PDSA) cycle provided an update on current guidelines for UCC providers. The second PDSA cycle updated prescription sentences in the electronic health record (EHR) and organized them from shortest to longest duration. The third PDSA cycle provided a project update via email to UCC providers. Our outcome measure is the percentage of patients receiving 5-7 days of antibiotics for SSTIs. Process measure is the number of updated prescriptions used. Balancing measure is the number of patients returning for SSTI within 14 days of their visit. Results are displayed using a run chart. Results: After initiation of the project in April 2020, the percentage of patients receiving 5-7 days of antibiotics increased to 68% (Figure 2). This percentage increased to our goal of 75% after the 1st PDSA cycle (October-December 2020), 80% following the second PDSA cycle in February 2021, and 90% following the third PDSA cycle in April 2021. There was no change in balancing measure numbers. Conclusion: Prior to our project, only 58% of children seen in CMH UCCs for SSTIs received the recommended antibiotic duration. By addressing the primary drivers uncovered through QI methodology, we surpassed our goal of 75%. Additional PDSA cycles are planned along with expansion to other departments. This work will allow us to expand antibiotic stewardship efforts to other infectious diagnoses as well.
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Reconsidering Perioperative Antibiotic Use in Elective Laparoscopic Cholecystectomy
Kayla B. Briggs, James Fraser, Wendy Jo Svetanoff, Charles L. Snyder, Pablo Aguayo, David Juang, Rebecca M. Rentea, Jason D. Fraser, Shawn D. St. Peter MD, and Tolulope A. Oyetunji
Introduction: Prophylactic preoperative antibiotics (PPA) are questionable in cases with a low rate of surgical site infection (SSI). We report institutional PPA usage and SSI rates after elective laparoscopic cholecystectomy in a children’s hospital. Methods: Children <18 years old who underwent outpatient laparoscopic cholecystectomy between 7>/2010 and 8/2020 were included. SSI was defined as clinical signs of infection, requiring antibiotics, within 30 days of surgery. Results: 502 patients met inclusion criteria; 50% were pre-operatively diagnosed with symptomatic cholelithiasis, 47% biliary dyskinesia, 2% hyperkinetic gallbladder, and 1% gallbladder polyp(s). The majority were female (78%) and Caucasian (80%). 60% (n=301) of patients received PPA while 40% (n=201) did not; 1.3% (n=4) of those who received PPA developed SSI compared to 5.5% (n=11) of those who did not (p=0.01). Though PPA use was associated with an 84% reduction in risk of SSI on multivariate analysis (p=0.01), all SSIs were superficial. One child required readmission for intravenous antibiotics while the remainder were treated with outpatient antibiotics. Gender, age, BMI, ethnicity, and preoperative diagnosis did not influence the likelihood of receiving PPA (Table 1). Conclusion: Given the relatively low morbidity of the superficial SSI, conservative use of PPA should be carefully considered in outpatient laparoscopic cholecystectomy to avoid contributing to antibiotic-related complications.
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Challenges in Echocardiographic Diagnosis of Corrected Malposition of Great Arteries: The Segmental Approach coming Handy
Amulya Buddhavarapu, Anmol Goyal, Sanket Shah, Nitin Madan, Hayley S. Hancock, and Maria Kiaffas
Clinical Presentation
Two cases of {S,D,L} anatomically corrected malposition of great arteries (ACMGA) are presented with differences in conal anatomy. Case 1: A fetal echocardiogram (echo) performed at 28 weeks gestation due to multiple anomalies revealed atrial situs solitus, D-loop ventricles, a large conoventricular septal defect (VSD) and an overriding, anterior and leftward aorta. The diagnosis of double outlet right ventricle (DORV) vs ACGMA was entertained. Postnatal echo revealed {S,D,L} segmental anatomy with ventriculoarterial concordance consistent with ACGMA and a muscular VSD. Case 2: A 3-week-old boy presented to clinic for a murmur evaluation with no associated cardiac symptoms. Transthoracic echocardiogram revealed {S,D,L} ACGMA and a small membranous VSD.
Imaging Findings
Echocardiographic subcostal, parasternal and suprasternal sweeps help diagnose ACGMA by delineating ventriculoarterial alignment, ventricular and outflow relations and conal anatomy. Case 1, a rare ACMGA type, posed the most diagnostic challenges. A more anterior rotation of the left ventricle, horizontal orientation of the ventricular septum due to absence of a sub-pulmonary conus, in combination with an elongated subaortic conus led to the misconception of an overriding aorta and DORV prenatally. Pulmonary to tricuspid valve fibrous continuity was present. The unusual position of the aortic valve resulted in an elongated curvature of a left aortic arch, coursing from anterior and right towards the left of the trachea. Case 2, the most typical type of {S,D,L} ACGM, had the usual anatomic characteristics of bilateral sub-arterial conus and parallel outflow tracts. In both cases cardiac magnetic resonance imaging was performed for evaluation of the Qp/Qs caused by the VSD and confirmed the diagnosis of ACGMA.
Roles of Imaging in Patient Care
Accurate prenatal diagnosis of ACMGA is essential for appropriate counseling and postnatal management. Postnatally, standard echocardiographic views and multimodality imaging will elucidate ventriculoarterial connections, conal anatomy, and severity of associated anomalies.
Discussion
ACMGA is a rare congenital heart disease occurring from failure of involution and rightward rotation of the subaortic conus resulting to a parallel spatial relationship of the great arteries while maintaining ventriculo-arterial concordance. Subtypes include {S,D,L} (most common, usually with bilateral conus) and {I,L,D} with normal, and {S,L,D} and {I,D,L} with transposition physiology. Identifying the segmental anatomy and relations will result in accurate diagnosis of this rare entity.
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Correlation between strain and weight status in infants with a univentricular hear
Amulya Buddhavarapu, Mathew Warren, Lori Erickson, Christopher Mathis, Whitney Haas, and Daniel Forsha
Background
Infants with univentricular physiology are at risk of poor weight gain and reduced ventricular function, both of which have been independently associated with worse outcomes. Since nutritional status has been correlated to ventricular function in other populations, we evaluated the relationship between nutritional status and ventricular function including speckle-tracking strain during this period.
Methods
Thirty term infants (median age 55 days, 13 females) with univentricular physiology prior to stage II palliation were included with data obtained at the time of their initial hospital discharge. Ventricular function was quantified using 2D global longitudinal strain (GLS) and strain rate, analyzed from an apical “4-chamber” view (TomtecCPA 2.31). Ventricular myocardium was tracked along the dominant walls producing systolic ejection-- the lateral and septal walls of the dominant ventricle in cases of a single RV or LV and both the lateral LV and RV walls in the presence of an unrestrictive ventricular septal defect and biventricular mass, such as an unbalanced AV septal defect. Statistical analysis using SPSS reported the median with interquartile range (IQR) and utilized Spearman or Pearson correlation testing as appropriate (P < 0.05 significant).
Results
Stage I palliation was performed in 29/30 (14 Norwood, 8 BT shunt, 5 PA bands, and 2 hybrids). GLS correlated with weight for age z-score (WAZ) scores (r= -0.45, p= 0.01) and trended toward correlation with weight for length z-score (r=-0.31, p =0.09) in this small study. Multivariate regression model using GLS as the dependent variable against WAZ score and atrioventricular regurgitation revealed an independent association between GLS and WAZ score (p=0.03) while the association of GLS with atrioventricular valve regurgitation lost significance (p=0.06). On ANOVA analysis of GLS based on ventricular morphology, GLS was diminished in the single RV subgroup [median -13.1% IQR (-15, -10.7)] compared to GLS in single LV [-17.2% (-21, -12.5)] and biventricular [-17.2% (-18.1, -14)] subgroups (p=0.043).
Conclusion
Lower WAZ-scores at the time of neonatal discharge are associated with lower GLS. Systemic right ventricles have lower GLS.
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Longitudinal analysis of myocardial function using strain in patients receiving cardiotoxic chemotherapy
Anmol Goyal, Amulya Buddhavarapu, Kayla Simpson, Nataliya Kibiryeva, Wendy Hein, Joy M. Fulbright, and Sanket Shah
Background:
Chemotherapy-related cardiotoxicity (CTRC) is associated with significant morbidity and mortality in long-term cancer survivors. Left ventricular [LV] Global longitudinal strain (GLS) is a sensitive parameter that can be used for earlier detection of contractile function changes in these patients. Recent literature suggested certain genotypes may influence CTRC. Our goal was to assess longitudinal myocardial function and identify any correlation between certain genetic variations and LV function regarding CTRC in our pediatric population.
Methods:
50 subjects ≥10 years of age who have survived >2 years after completion of cancer treatment were enrolled out of which 29 subjects agreed for genetic analysis and follow-up evaluation of echocardiograms. Echocardiographic data from these 29 subjects was reviewed. Apical 4 chamber and short axis at the level of papillary muscles were used for area-length 2D Ejection fraction (EF) [bullet method], and apical 4 chamber image was used for GLS. GLS was performed by using speckle tracing retrospectively with 2D STE offline analysis software (4D LV-Analysis 3) developed by TomTec Imaging Systems. Seventeen of these 29 patients had longitudinal echocardiographic data available for serial measurement of their EF and GLS. GLS ≤-18% was considered normal. Paired t-test analysis was performed for these patients with follow-up data available using SPSS statistics 27.0. All 29 patients underwent whole exome sequencing for genetic polymorphism, the results of which are pending at the time of submitting this abstract.
Results:
Baseline GLS was normal in 28/29 patients (96.5%). Mean GLS was -21.71% ± 2.36 %. Mean EF was 60.1% ± 5.4%. Mean follow-up duration was 4.1 years [range 2.2-6.5 years]. At follow-up evaluation, the mean reduction of strain was 1.4 % [standard deviation (SD) of 2.1, p = 0.015] and reduction of EF was 1% [SD of 5.6, p=0.45]. Longitudinal data in 17 patients showed a decrease in GLS by >2% in 47 % of patients, however, only 11.7% of the patients had a concomitant significant decrease in EF of ≥5%.
Conclusions:
In this cohort of pediatric cancer patients, clinically all were asymptomatic; in New York Heart Association Heart failure class 1. With a mean follow-up period of 4 years, there was statistically significant reduction in GLS while EF essentially remained stable. GLS can be a more sensitive marker to assess longitudinal ventricular function than EF and can be a valuable tool in risk-stratifying patient receiving cardiotoxic chemotherapy. Additional multi-center longitudinal studies evaluating the long-term prognostic implications of reduced LV GLS in this population are essential.
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Transient Tricuspid Valvulitis: Another Brief Casualty of COVID-19 in Children
Amulya Buddhavarapu, Girish S. Shirali, Stephen Kaine, and Doaa Aly
Clinical Presentation:
We discuss 3 patients, 8-10 years old, with significant tricuspid valve dysfunction during initial days of admission for multi-system inflammatory syndrome in children (MIS-C) associated with coronavirus 2019 (COVID-19) infection.
All 3 patients had COVID antibodies and presented in uncompensated shock needing aggressive fluid resuscitation and vasoactive support. Elevated acute inflammatory markers, acute kidney injury (2/3 patients), troponin leak and repolarization abnormality on ECG were present on admission and resolved by discharge. All were treated with intravenous immunoglobulin (IVIG) infusion, high-dose steroids and prophylactic low molecular weight heparin. They were discharged home on aspirin and steroid taper.
All patients had depressed left ventricular systolic function with LVEF of 38-52% and trivial to mild mitral regurgitation. Following fluid resuscitation and administration of IVIG, all patients developed moderate to severe, new-onset tricuspid valve regurgitation (TR) with failure of leaflet coaptation and normal tricuspid regurgitation velocities, with a TR vena contracta of 6-9 mm. Right ventricular (RV) systolic function as measured by fraction area change and TAPSE ranged from low normal to moderately decreased. RV size and tricuspid annular diameter Z score was normal for all patients.
All 3 patients responded to diuresis and fluid restriction and had mild TR at discharge.
Imaging Findings:
Our patients demonstrated an interesting pattern of early, acute onset, moderate to severe tricuspid valve regurgitation, with a wide gap of non-coaptation between the septal and anterior tricuspid valve leaflets leading to a broad central jet of TR. The degree of TR was disproportionate to the degree of RV systolic dysfunction or annular dilatation.
Role of Imaging in Patient Care:
We postulate that the etiology of tricuspid valve dysfunction is a combination of valvulitis / papillary muscle dysfunction, RV diastolic dysfunction and volume overload due to fluid resuscitation and IVIG. The frequency of these findings points to the need for judicious volume resuscitation and slower IVIG infusions in MIS-C.
Summary/Discussion Points:
TR associated with MIS-C in the pediatric population appears to be transient, and responsive to alterations in the patient’s volume status and inflammatory state. This is in contrast to adults, in whom TR with RV dysfunction has been identified as a predictor of mortality.
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A Deleterious EPHB4 Mutation Suppresses PROX1 Expression and Disrupts Lymphatic Development in Neonatal Non-immune Hydrops
Gangaram Akangire, Heather Menden, Sheng Xia, Atif Ahmed, and Venkatesh Sampath
Background: Non-immune hydrops fetalis is often a fatal condition and in 20% of cases related to lymphatic anomalies. Maldevelopment of lymphatic valves, atretic or absence of central lymphatic vessels has been described in hydrops fetalis, but the genetic basis of these anomalies remains undefined. Recent reports suggest that Ephrin type B receptor 4 (EPHB4) gene has a significant role in embryonic development of lymphatic system. To test the hypothesis that EPHB4 mutations can cause hydrops fetalis, we combined whole exome sequencing (WES) with functional and pathological analysis of an infant who succumbed to neonatal hydrops.
Objective: To discover the possible mechanism of non-immune hydrops in EPHB4 variant by WES, histopathologic analysis and in vitro functional analysis of genetic mutation in human lung endothelial cells (HLEC).
Design/Methods: 35-week, 4.11kg male infant developed hydrops prenatally requiring multiple thoracentesis postnatally due to bilateral chylous pleural effusions and ascites, and eventually succumbed to infection and immunodeficiency from T-lymphocyte depletion. WES was performed to identify genetic causes of non-immune hydrops. Immunohistochemistry (IHC) was performed on autopsy specimens of lung and intestinal tissue. In vitro functional analysis was done using HLEC, and immunoblotting used of investigating signaling events.
Results: Magnetic resonance imaging (MRI) lymphangiogram showed complete absence of central lymphatic ducts (Figure 1). WES showed a rare heterozygous missense mutation in EPHB4 (p.Ala700Thr) that was maternally inherited. Autopsy revealed pulmonary lymphangiectasia, depletion of lymphoid tissue, and staphylococcus aureus in spleen. IHC showed loss of PROX1 expression (prospero homeobox protein 1) in large lymphatic channels in lung and small intestinal villi (Figure 2). In vitrofunctional studies showed the EPHB4 mutation resulted in loss of phosphorylation, decreased ERK phosphorylation and suppressed PROX1 expression, necessary for lymphatic valve development (Figure 3).
Conclusion(s): We report a novel case of fatal non-immune hydrops with lymphatic anomalies associated with an EPHB4functional mutation that suppresses its phosphorylation and PROX1 expression. This report highlights the importance of screening for EPHB4 variant in infants with the diagnosis of non-immune hydrops fetalis and lymphatic anomalies. This discovery will trigger further studies to find genetic basis of non-immune hydrops and novel therapies for this fatal disease.Presented at the 2021 PAS Virtual Conference
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A novel way to describe variation in antibiotic use
Adrienne G. DePorre, Troy Richardson, Jason Newland, and Mark Neuman
Background: A broader understanding of antibiotic variation can inform stewardship efforts. Diversity indices are frequently calculated in ecologic studies to measure the variety of species in a given habitat, however they have not yet been used to study variation in antibiotic use in the healthcare setting.
Objective: To develop a novel assessment of antibiotic variation, based on the breadth of an antibiotic’s associated conditions as well as its use, measured by days of therapy (DOT).
Design/Methods: We assessed antibiotic use in children aged 60 days-18 years hospitalized at a US Children’s hospital included in the Pediatric Health Information System (PHIS) database. We identified common antibiotics, defined as those that account for >1% of total antibiotic DOT at >80% of hospitals. An antibiotic diversity index (ADI) was calculated for each antibiotic at each hospital using the number of conditions and DOT to mimic the Shannon-Weiner entropy index, an index used in ecology to measure the complexity of an ecosystem. Hospital-level variation in antibiotic use was measured by the spread of ADI scores across hospitals relative to the mean ADI (i.e. coefficient of variation [CV]).
Results: Table 1 shows commonly used antibiotics at 49 hospitals. Cefazolin has the highest mean ADI indicating it is used in the highest number of conditions. However, it also has the smallest CV indicating consistent use across hospitals. . Conversely, meropenem has a relatively small mean ADI but has the highest CV. This indicates that meropenem is used in a relatively small number of conditions but is used more variably across hospitals. Figure 1 shows the utility of the ADI to identify variation in distribution of both an antibiotic’s DOT and associated conditions. In most hospitals, use of cefazolin is focused consistently in 7-9 conditions while meropenem is used consistently across hospitals in 2-3 conditions.
Conclusion(s): ADI is a novel tool to measure variability in antibiotic use across hospitals. Comparisons of ADIs highlight antibiotics associated with unequal patterns of use and their associated conditions.Presented at the 2021 PAS Virtual Conference
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Antibiotic Durations for Skin and Soft Tissue Infections in Pediatric Urgent Care Clinics
Megan Hamner, Amanda Nedved, Holly Austin, Donna Wyly, Alaina N. Burns, Diana King, Brian R. Lee, and Rana El Feghaly
Background: Skin and soft tissue infections (SSTIs) are the second most common diagnosis leading to pediatric antibiotic prescriptions in the outpatient setting after respiratory diagnoses. However, most antibiotic stewardship programs have mainly focused on the latter. Children seen in the ambulatory setting for SSTIs often receive >7 days of antibiotics, although current society guidelines recommend 5-7 days for most diagnoses.
Objective: To determine the baseline percentage of patients receiving antibiotic prescriptions for >7 days for SSTIs in urgent care clinics (UCC)s of a pediatric health system and to evaluate factors that influence providers towards longer durations.
Design/Methods: We built a report that extracted patient encounters from the three UCCs based on International Classification of Diseases (ICD)-10 codes for common SSTIs including impetigo, abscesses, cellulitis, erysipelas, folliculitis, paronychia, and animal bites. Data was pulled from June 2019 through June 2020. The report included patient age, concomitant diagnoses, antibiotics prescribed and their duration. We excluded encounters if the patient was transferred to the emergency department or admitted, the patient was younger than 3 months of age, no antibiotics were prescribed, or if there was a concurrent infectious diagnosis affecting antibiotic duration. We sent a 22-question survey to UCC providers to understand prescribing habits particularly focusing on factors prompting administration of longer antibiotic courses.
Results: From June 2019-June 2020, we reviewed 2,575 encounters; we excluded 208 of those (8%). 823 (35%) of patients received >7 days of antibiotics for SSTIs while 1181 (50%) received 5-7 days and 35 (1%) received <5 days of antibiotics. 328>(14%) received topical therapy only. Most common antibiotics prescribed included cephalexin, clindamycin, and trimethoprim-sulfamethoxazole. A mild improvement in the 5-7 days duration was noted through our study period (Figure 1). The survey was sent to 50 providers with 27 responding (54% response rate) (Tables 1 and 2). Barriers for shorter treatment courses included concern for acute rheumatic fever development, parental pressure, fear of complications, and accustomed antibiotic duration.
Conclusion(s): A third of children with SSTIs in our UCCs receive long courses of antibiotics. A mild improvement noted in our study period may be due to existing antibiotic stewardship interventions. Specific provider concerns leading to overprescribing will be targeted by quality improvement efforts.Presented at the 2021 PAS Virtual Conference
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A SMARTer Way to Round
Charles Maloy, Adrienne G. DePorre, Erica Adams, Jessica Olson, Amber Hunley, and Darcy K. Weidemann
Background: Communication is a key driver of health care outcomes. Poor communication practices contribute to sentinel safety events, poor family/patient experiences, and delays in care. Use of checklists in the healthcare setting is important to the development of high reliability and is increasingly common, however, the development and implementation of medical checklists for the standard patient floor are inadequately described.
Objective: To develop a sustainable rounding checklist for multidisciplinary discussion of patient safety measures and clinical plans.
Design/Methods: Key stakeholders in the rounding process (subspecialty and general pediatrics physicians, resident physician, bedside nurses, and nursing leadership) created a rounding checklist tool for use on a medical unit at our tertiary care children’s hospital. This checklist was modified from an existing checklist used in the pediatric intensive care unit. It focused on reducing harm, improving quality of care, and facilitating communication. To foster open communication within the multidisciplinary team and project sustainability, bedside nurses owned the task to prompt daily review of checklist items with the team during rounds. We developed badge buddies as an aid and an audit tool to assess checklist compliance. Compliance with checklist use was assessed by iterative Plan-Do-Study-Act (PDSA) cycles. Acceptability and usefulness of the checklist was measured by a 6 month-post implementation survey of nurses, residents, and staff.
Results: A five-item checklist entitled SMART (Situational Awareness, Medications, Access, Routine, and Transition) was created (Figure 1). Daily audits showed between 75%-88% usage of the checklist during rounds (Figure 2). Our first PDSA cycle showed improved compliance with implementation of a streamlined audit tool. 29% of eligible providers completed the survey (n=51). 77% of respondents perceived communication improvement with SMART card usage with 4% disagreement. 66% reported vital patient care details were discussed that would otherwise have been missed. Only 2% found the checklist led to delays in patient care with a majority responding that checklist completion took 30-60 seconds.
Conclusion(s): This project emphasizes the importance of multidisciplinary teams in development and implementation of a daily rounding checklist for a pediatric floor. We demonstrate the feasibility and acceptability of inserting a rounding checklist into the workflow of a multidisciplinary pediatric care team. Further study is needed to determine long-term effects on this initiative on process of care outcomes.Presented at the 2021 PAS Virtual Conference
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Assessing the Effects of Social Determinants on Serious Safety Events
Lisa L. Schroeder, Jessi Van Roekel, and John Cowden
Background: We have long known that various social determinants of health (SDH) such as race, gender, socioeconomic status and others can affect health outcomes, such as readmission rates and mortality. To our knowledge, the impact of SDH on safety events has not been studied.
Objective: We sought to understand the role that social determinants may play in contributing to safety events at our institution and to incorporate this information into the creation of corrective action items following the serious safety event.
Design/Methods: The Clinical Safety team partnered with the Office of Equity and Diversity to develop a standard question regarding the potential influence of any SDH to be asked of each staff member interviewed after a potential serious safety event. The question was asked at the end of the interview and was read to maintain consistency. The question was modified early in the process based on feedback from the interviewers and the staff interviewed. All identified factors were collected, and the clinical safety team ultimately determined the likelihood that factors contributed to the adverse outcomes.
Results: Over the first two and a half years, 129 interviews were conducted spanning 20 safety events. The question was asked in 101 (78%) of the interviews. It was asked more consistently as the project went on, with only 58% in interviewees being asked the first year and 90% thereafter. At least one social determinant was identified as potentially contributing to the safety event in 21 interviews (11 cases). In these cases, an average of 1.36 factors were identified (range 1-3). Language and socioeconomic status were the most frequently identified factors. Responses to the social determinants question were then considered throughout the Root Cause Analysis process, including the development of action items.
Conclusion(s): By treating health equity as fundamental to patient safety, we integrated a question on the potential impact of SDH on safety events. Socialization to the process took time, but staff have now expressed increased awareness of the potential effects of social determinants. The consideration of impact of SDH on adverse events has informed the improvement team in their development of action items. The success of this project has led to the incorporation of health equity questions into other areas, including Performance Improvement, Evidence Based Practice, Patient Family Experience, and others. We hope to incorporate a similar question into the overall event reporting system to help inform future initiatives.Presented at the 2021 PAS Virtual Conference
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Association Between ESA Dose and Blood Pressure in Pediatric Patients on Dialysis
Heather A. Morgans, Judith Sebestyen VanSickle, Franz Schaefer, and Bradley A. Warady
Background: Hypertension is a reported side effect of Erythropoiesis Stimulating Agents (ESAs), with a mechanism of action related to elevated hematocrit levels and direct vasopressor effects. Limited information exists on the relationship between ESA dosage and hypertension in children receiving maintenance dialysis.
Objective: The primary aim of this study was to determine whether there is a significant correlation between ESA dose and blood pressure (BP) in pediatric patients on dialysis. The secondary aim was to determine confounding variables in relation to ESA dose and BP.
Design/Methods: Data from the International Pediatric Dialysis Network (IPDN) database was used to retrospectively evaluate the association between ESA dose and BP. Data collected from January 2007 to September 2019 was analyzed. Systolic and diastolic BP measurements obtained at clinic visits were averaged and standardized based on age, height, and sex. ESA dose was measured in units/kg/week with Darbepoetin and continuous erythropoietin receptor activator (CERA) converted to equivalent units of Epogen. The confounding variables analyzed include hemoglobin level, BMI, dialysis modality, total fluid output (daily ultrafiltrate plus 24-hour urine output), number of antihypertensive medications, and use of growth hormone. Linear regression with Pearson correlation was used to analyze continuous variables.
Results: A total of 3790 children were included in the analysis with a mean age of 11 years and 55.7% male. The mean prescribed dose of ESA was 192 units/kg/week. A significant positive correlation was noted between ESA dose and systolic BP (p < 0.001) although there was no significant correlation with diastolic BP (p=0.2). Further evaluation showed a significant negative correlation between ESA dose, total fluid output, and hemoglobin level in univariate and multivariate analysis, p < 0.001.
Conclusion(s): The preliminary results from this study suggest that there may be a correlation between higher doses of ESAs and higher systolic BP in children receiving maintenance dialysis. Ongoing analysis of confounding variables will be helpful in determining the full correlation between ESA dose and BP.Presented at the 2021 PAS Virtual Conference
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Atrial standstill in a pediatric patient with SCN5A mutation following procainamide challenge
Anmol Goyal, Lindsey Malloy-Walton, and Christopher Follansbee
Background: Atrial standstill (AS) is a rare arrhythmia characterized by absence of electrical and mechanical atrial activity associated with SCN5A channelopathy.
Case: An 18 year old male with structurally normal heart, frequent sinus pauses, nonsustained atrial tachycardia and high-grade block was found to have SCN5A mutation c.3823G>A (p.Asp1275Asn). An electrophysiology study (EPS) with high density voltage mapping of the right atrium was done (Fig 1a). Nonsustained multifocal atrial tachycardia was induced without ablative targets (Fib 1b). Procainamide challenge was negative for Brugada, however induced AS (Fig 1c-d). No atrial capture could be achieved at maximal output. Empiric atrial lead positioning in the right atrial appendage was utilized based on prior atrial mapping (Fig 1e). AS resolved in <24 hours with resultant functioning of the atrial>lead.
Decision-Making: SCN5A disease can have a variable phenotype ranging from asymptomatic to progressive AS. A detailed EP study with high density mapping should be considered to assess for viable atrial tissue prior to pacemaker implantation. Progressive disease may result in high thresholds, failure to capture or AS, and patients should be followed closely.
Conclusion: SCN5A channelopathy can result in a unique phenotype that requires careful and serial evaluation by an electrophysiologist. As progressive AS can occur, a detailed EPS with high density atrial mapping should be considered when pacemaker implantation is required.
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CONGENITAL MITRAL VALVE REGURGITATION, THE DILEMMA OF REPAIR VERSUS REPLACEMENT
Bianca Cherestal and Doaa Aly
Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period.
Case: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x m2 and CT was non-specific for lung parenchymal disease. Sildenafil and Flolan were added to reverse PHN prior to proceeding with MV repair. At 4 weeks of age he underwent mitral valvuloplasty which was complicated by severe MR and left heart failure (fig 1 c-d). Successful MV replacement with 17 mm St Jude mechanical valve was performed at 11 weeks (fig 1 e). PHN medications were weaned and patient is now ready for discharge.
Decision Making: Patient presented with severe left heart failure and PHN secondary to severe congenital MR. MV intervention was indicated due to failed medical management. While MV replacement, can be a challenge, it was ultimately necessary given the severe post repair residual regurgitation.
Conclusion: This case highlights the complexity of decision making for congenital MR, and the role of MV replacement in the case of failed repair.
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Constrictive Pericarditis After Repair of a Ruptured Sinus of Valsalva
Sarah Studyvin and Sanket Shah
Background: Constrictive pericarditis (CP) is a rare complication of cardiac surgery. We report a patient who developed CP after ruptured sinus of Valsalva (RSOV) repair.
Case: A 23-year-old male presented with severe exertional dyspnea one year after RSOV repair. Echocardiogram showed thickened pericardium, ventricular septal bounce (Fig 1A), left atrial enlargement, diastolic hepatic flow reversal (Fig 1B), and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI on the same day confirmed pericardial thickening (Fig D) with paradoxic septal motion, dilated pulmonary veins (Fig 1F) and retrograde flow in the SVC.
Decision‐making: Pericardiectomy of thickened and adherent pericardium was performed without the use of cardiopulmonary bypass. The central venous pressure decreased from 23 to 7 mmHg and TEE showed normal systolic function with less septal bounce posteroperatively. Pathology specimens of the pericardium exhibited fibrosis and mild chronic inflammation. He continued to do well off diuretics at one-month follow-up.
Conclusion: Constrictive pericarditis is an uncommon complication of aortic root surgery. MRI is the ideal study to confirm the thickened pericardium and paradoxic septal motion in patients with suspected pericarditis. Cardiac catheterization can be performed to confirm the diagnosis.
