Anatomic considerations in the management of complete atrioventricular canal.

OBJECTIVE: Patients with complete atrioventricular canal have a variable clinical course prior to repair. Many patients balance their circulations well prior to elective repair. Others manifest clinically significant pulmonary over circulation early in life and require either palliative pulmonary artery banding or complete repair. The objective of this study was to assess anatomic features that impact the clinical course of patients. METHODS: In total, 222 patients underwent complete atrioventricular canal repair between 2012 and 2022 at a single institution. Twenty-seven (12%) patients underwent either pulmonary artery banding (n = 15) or complete repair (n = 12) at less than 3 months of age (Group 1). The remaining 195 (88%) underwent repair after 3 months of age (Group 2). Patient records and imaging were reviewed. RESULTS: The median post-operative length of stay following complete repair was 25 [7,46] days for those patients in Group 1 and 7 [5,12] days for those in Group 2 (p < 0.0001). There was relative hypoplasia of left-sided structures in Group 1 versus Group 2. Mean z-score for the ascending aorta was -1.2 (±0.8) versus -0.3 (±0.9) (p < 0.0001), the aortic isthmus was -2.1 (±0.8) versus -1.4 (±0.8) (p = 0.005). The pulmonary valve to aortic valve diameter ratio was median 1.47 [1.38,1.71] versus 1.38 [1.17,1.53] (p 0.008). CONCLUSIONS: Echocardiographic evaluation of the systemic and pulmonary outflow of patients with complete atrioventricular canal may assist in predicting the clinical course and need for early repair vs pulmonary artery banding.

Immunisation deferral practices surrounding congenital heart surgery.

BACKGROUND: Perioperative immunisation administration surrounding congenital heart surgery is controversial. Delayed immunisation administration results in children being at risk of vaccine-preventable illnesses and is associated with failure to complete immunisation schedules. Among children with CHD, many of whom are medically fragile, vaccine-preventable illnesses can be devastating. Limited research shows perioperative immunisation may be safe and effective. METHODS: We surveyed Pediatric Acute Care Cardiology Collaborative member centres and explored perioperative immunisation practices. We analysed responses using descriptive statistics. RESULTS: Complete responses were submitted by 35/46 (76%) centres. Immunisations were deferred for any period prior to surgery by 23 (66%) centres and after surgery by 31 (89%) centres. Among those who deferred post-operative immunisation, 20 (65%) required deferral only for patients whose operations required cardiopulmonary bypass. Duration of deferral in the pre- and post-operative periods was variable. Many centres included exceptions to their policy for specific vaccine-preventable illnesses. Almost all (34, 97%) centres administer routine childhood immunisation to patients who remain admitted for prolonged periods. CONCLUSIONS: Most centres defer routine childhood immunisation for some period before and after congenital heart surgery. Centre specific practices vary. Immunisation deferral confers risk to patients and may not be warranted in this population. Further research would be necessary to understand the immunologic impact of these practices.

Bacterial Sexually Transmitted Infection Incidence Among Southern Men Who Have Sex With Men With Human Immunodeficiency Virus in the Treatment as Prevention Era, 2014-2019.

In this retrospective analysis of men who have sex with men with human immunodeficiency virus (HIV) in the South from 2014 through 2019, incident bacterial sexually transmitted infections (STIs) increased regardless of virologic control. Clinicians should prioritize STI screening and management in primary HIV care.

Prevalence and impact of comorbid chronic pain and cigarette smoking among people living with HIV.

Rates of chronic pain and cigarette smoking are each substantially higher among people living with HIV (PLWH) than in the general population. The goal of these analyses was to examine the prevalence and impact of comorbid chronic pain and cigarette smoking among PLWH. Participants included 3289 PLWH (83% male) who were recruited from five HIV clinics. As expected, the prevalence of smoking was higher among PLWH with chronic pain (41.9%), than PLWH without chronic pain (26.6%, p < .0001), and the prevalence of chronic pain was higher among current smokers (32.9%), than among former (23.6%) or never (17%) smokers (ps < .0001). PLWH who endorsed comorbid chronic pain and smoking (vs. nonsmokers without chronic pain) were more likely to report cocaine/crack and cannabis use, be prescribed long-term opioid therapy, and have virologic failure, even after controlling for relevant sociodemographic and substance-related variables (ps < .05). These results contribute to a growing empirical literature indicating that chronic pain and cigarette smoking frequently co-occur, and extend this work to a large sample of PLWH. Indeed, PLWH may benefit from interventions that are tailored to address bidirectional pain-smoking effects in the context of HIV.

Medicaid Payer Status, Higher Comorbidity, and Low Income Are Associated With Poorer Outcomes After Total Elbow Arthroplasty.

OBJECTIVE: To assess whether insurance payer, comorbidity, and income are associated with total elbow arthroplasty (TEA) outcomes. METHODS: We used the 1998-2014 US National Inpatient Sample. Multivariable logistic regression adjusted for demographics and underlying diagnosis to estimate odds ratio (OR) and 95% confidence intervals (CI) of insurance payer, comorbidity, and income with TEA outcomes. RESULTS: The mean age was 60 (SE, 0.29) years, 68% were female, and 62% were white among the 7992 TEA procedures. Compared with private insurance, Medicaid was associated with significantly higher ORs (95% CI) of (1) hospital charges above the median, 1.25 (95% CI, 1.01-1.53); (2) discharge to a rehabilitation facility, 1.64 (95% CI, 1.16-2.31); (3) hospital stay >2 days, 1.63 (95% CI, 1.32-2.00); (4) fracture, 1.71 (95% CI, 1.14-2.56). Medicare payer was associated with higher ORs (95% CI) of (1) discharge to a rehabilitation facility, 1.80 (95% CI, 1.42-2.28); and (2) hospital stay >2 days, 1.29 (95% CI, 1.12-1.50). Compared with Deyo-Charlson score of zero, odds of health care utilization outcomes were higher by 14% to 20% for score of 1 and by 62% to 146% for score of 2 or higher, and by 36% to 257% for transfusion. The lowest income quartile had significantly higher OR of 1.51 (95% CI, 1.31-1.73) of hospital charges above the median versus the highest quartile. CONCLUSIONS: Payer type, comorbidity, and income were associated with higher health care utilization and complications post-TEA. Further investigation into potentially modifiable mediators is needed.

Current opinion in pediatric heart transplantation.

PURPOSE OF REVIEW: Cardiac transplant remains the most effective therapy for children with end-stage heart disease. Outcomes remain better than any alternative therapy for this condition, but its use is limited by donor organ availability. As a result, waitlist times and mortality on the waiting list remain unacceptably high. Novel approaches are necessary to address this problem. RECENT FINDINGS: Organ Procurement and Transplant Network/United Network for Organ Sharing readjusted the pediatric heart allocation system in 2016 to prioritize children at highest risk of mortality, encourage judicious listing, and improve appropriate donor organ utilization. Subsequent studies have aligned with these priorities to help risk-stratify patients at the time of listing and identify the importance that should be assigned to donor-specific factors. In addition, many authors are advocating for increased utilization of hearts donated after cardiac death. Pediatric Ventricular Assist Device (VAD) application has also been increasing to help decrease waitlist mortality. Although results have significantly improved, there remain important limitations to widespread use of VADs in the pediatric population. This has prompted novel techniques such as pulmonary artery banding to improve cardiac function and, in some cases, promote recovery. The demand for cardiac replacement continues to increase with an ageing population of patients with congenital heart disease, presenting new challenges and stressors to the system. SUMMARY: Pediatric cardiac transplant outcomes are excellent but remain plagued by the limited supply of donor organs. Recent strategies to combat this problem have focused on judicious listing, maximal utilization of available donor organs, and safely extending the lives of patients on the waitlist. New demands on the organ supply chain will continue to stress the system, making these efforts of the highest importance.Clinical Trial Registry Number not applicable.

Hypersensitivity reactions with allopurinol and febuxostat: a study using the Medicare claims data.

OBJECTIVE: To assess the risk of hypersensitivity reactions (HSRs) with allopurinol and febuxostat in a population-based study. METHODS: We used the 5% Medicare beneficiary sample (≥65 years) from 2006 to 2012 to identify people with a newly filled prescription for allopurinol, febuxostat or colchicine. We used multivariable-adjusted Cox regression analyses to compare the hazard ratio (HR) of incident HSRs with allopurinol or febuxostat use versus colchicine use; separate analyses were done in people exposed to allopurinol. Propensity-matched analyses (5:1) compared hazards with allopurinol versus febuxostat. RESULTS: Crude incidence rates of HSRs were as follows: allopurinol, 23.7; febuxostat, 30.7; and colchicine, 25.6 per 1000 person-years. Compared with colchicine, allopurinol, febuxostat and febuxostat+colchicine were associated with significantly higher HRs of HSRs, 1.32 (95% CI: 1.10 to 1.60) and 1.54 (95% CI: 1.12 to 2.12) and 2.17 (95% CI: 1.18 to 3.99), respectively. In propensity-matched analyses, febuxostat did not significantly differ from allopurinol; HR for HSRs was 1.25 (95% CI: 0.93 to 1.67). Compared with allopurinol start dose <200 mg/day, allopurinol start dose ≥300 mg/day, diabetes and female sex were associated with significantly higher hazard of HSRs, 1.27 (95% CI: 1.12 to 1.44), 1.21 (95% CI: 1.00 to 1.45) and 1.32 (95% CI: 1.17 to 1.48), respectively. The majority (69%) of HSRs occurred in the outpatient setting. CONCLUSIONS: Compared with colchicine, allopurinol and febuxostat similarly increased the risk of HSRs. Allopurinol and febuxostat did not differ from each other. In allopurinol users, starting dose, female sex and diabetes increased this risk, findings that need further study.

Serious infections in Sjögren's syndrome patients: a national U.S. study.

OBJECTIVES: To study the incidence, time-trends and outcomes of serious infections in Sjögren's syndrome (SS). METHODS: We examined the epidemiology, time-trends and outcomes of five serious infections (opportunistic infections (OI), skin and soft tissue infections (SSTI), urinary tract infection (UTI), pneumonia, and sepsis/bacteremia) in hospitalised patients with SS, using the 1998-2016 U.S. National Inpatient Sample. Multivariable-adjusted logistic regression analyses analysed the association of patient, comorbidity and hospital characteristics with healthcare utilisation (hospital charges, length of hospital stay, discharge to non-home setting), and in-hospital mortality. RESULTS: We found 49,897,331 hospitalisations with serious infections in general population and 69,239 in patients with SS. Compared to serious infections hospitalisations in people without SS, SS patients were older, and more likely to be female, white or have Deyo-Charlson index score ≥2. Serious infections during the study period 1998-2016 were: OI, 3%; SSTI, 19%; UTI, 6%; pneumonia, 37%; and sepsis, 34%. Serious infection rates/100,000 NIS hospitalisations increased from 1998-2000 to 2015-2016: OI, 0.16 to 0.46; SSTI, 0.55 to 2.90; UTI, 0.25 to 1.96; pneumonia, 2.78 to 5.43; sepsis, 0.63 to 10.71. In multivariable-adjusted analyses, older age, Deyo-Charlson index score ≥2 and medium or large hospital bed size were associated with higher healthcare utilisation and in-hospital mortality. Medicare insurance, Northeast region, non-rural hospital were associated with higher healthcare utilisation outcomes only. CONCLUSIONS: We quantified the increasing disease burden of serious infections in people with SS, and described its epidemiology. Association of factors with serious infection hospitalisation outcomes identifies potential targets for future interventions.

Sjogren's syndrome is associated with higher rate of non-home discharge after primary hip arthroplasty and higher transfusion rates after primary hip or knee arthroplasty: a U.S. cohort study.

BACKGROUND: To assess whether Sjogren's Syndrome (SS) is associated with outcomes after total knee or hip arthroplasty (TKA/THA). METHODS: We used the 1998-2014 U.S. National Inpatient Sample data. We performed multivariable-adjusted logistic regression analyses to assess the association of SS with healthcare utilization (hospital charges, length of hospital stay, discharge to non-home setting), and in-hospital complications (implant infection, revision, transfusion, mortality), controlling for important covariates and confounders. In sensitivity analyses, we additionally adjusted the main models for hospital location/teaching status, bed size, and region. RESULTS: We examined 4,116,485 primary THAs and 8,127,282 primary TKAs performed from 1998 to 2014; 12,772 (0.2%) primary TKAs and 6222 (0.2%) primary THAs were done in people with SS. In multivariable-adjusted models, SS was associated with a statistically significant higher odds ratio (OR; 95% confidence interval (CI)) of discharge to a rehabilitation/inpatient facility post-THA, 1.13 (1.00, 1.28), but not post-TKA, 0.93 (0.86, 1.02). We noted no differences in the length of hospital stay or hospital charges. SS was associated with significantly higher adjusted odds of in-hospital transfusion post-THA, 1.37 (1.22, 1.55) and post-TKA, 1.21 (1.10, 1.34). No significant differences by SS diagnosis were seen in hospital stay, hospital charges implant infection, implant revision or mortality rates. CONCLUSIONS: People with SS had higher transfusion rate post-TKA/THA, and higher rate of discharge to non-home setting post-THA. The lack of association of SS with post-arthroplasty complications should reassure patients, surgeons and policy-makers about the utility of TKA/THA in people with SS undergoing these procedures.

Comparative effectiveness of allopurinol and febuxostat for the risk of atrial fibrillation in the elderly: a propensity-matched analysis of Medicare claims data.

AIMS: Gout is associated with a higher risk of atrial fibrillation (AF). Comparative effectiveness of allopurinol or febuxostat for reducing the AF risk is unknown, which was our study's main objective. METHODS AND RESULTS: We used the 5% Medicare Beneficiary cohort (≥65 years) from 2006 to 2012 to identify people with a new filled prescription for allopurinol or febuxostat, with a baseline period of 365 days without respective medication and without AF. We used 5:1 propensity-matched Cox regression analyses to assess whether allopurinol use differed from febuxostat use regarding the hazard ratio (HR) of incident AF. We found 25 732 eligible episodes in 23 135 beneficiaries. Of these, 2311 incident allopurinol or febuxostat use episodes (9%) ended in incident AF with crude incidence rates of 8.0 and 10.5 per 100 person-years, respectively. In propensity-matched analyses, compared with allopurinol, febuxostat was associated with higher HR of AF, 1.25 [95% confidence interval (CI) 1.05-1.48]. Compared with allopurinol <200 mg/day, febuxostat 80 mg/day was associated with significantly higher HR of AF, 1.62 (95% CI 1.16-2.27), but not febuxostat 40 mg/day or higher allopurinol doses. Compared with 1-180 days of allopurinol use, febuxostat use for 1-180 days was associated with significantly higher HR of AF, 1.36 (95% CI 1.10-1.67), but longer durations were not. CONCLUSION: Febuxostat was associated with a higher risk of AF compared with allopurinol in older adults. Increased AF risk was noted with febuxostat 80 mg/day dose and was most evident in the first 6 months of use. These findings need replication.

Total knee arthroplasty outcomes in lupus: a study using the US National Inpatient Sample.

OBJECTIVE: To assess whether patients with lupus have more complications and/or health care utilization than patients without lupus after primary total knee arthroplasty (TKA). METHODS: We used diagnostic codes to identify lupus (710.0) in patients who underwent primary TKA (procedure code, 81.54) in the US National Inpatient Sample from 1998-2014. We performed separate logistic regression analyses to assess whether lupus was associated with each in-hospital post-primary TKA complication (implant infection, implant revision, transfusion, mortality) or health care utilization for the index hospitalization. RESULTS: Our study cohort consisted of 8 127 282 primary TKAs with a mean age of 66.4 years, 63% were female, 68% White, 97% with OA, and the Deyo-Charlson comorbidity score was zero in 51%; 30 912 TKAs were performed in people with lupus. In multivariable-adjusted analyses, lupus was independently associated with a significantly higher risk of transfusion, but no significant difference in implant infection, implant revision or mortality, with respective odds ratios [ORs] (95% confidence interval [CI]) of 1.34 (1.25, 1.42), 1.38 (0.73, 2.60), 1.35 (0.83, 2.19) and 0.83 (0.34, 2.03). Lupus was independently associated with a significantly higher risk of total hospital charges above the median, the length of hospital stay >3 days (median) and discharge to an inpatient facility, respective ORs (95% CI) were 1.42 (1.22, 1.66), 1.09 (1.04, 1.15) and 1.34 (1.27, 1.41). CONCLUSION: Our finding of higher transfusion rate and health care utilization in lupus compared with non-lupus patients undergoing primary TKA informs surgeons, patients and policy makers regarding TKA outcomes in lupus, and can also guide appropriate resource allocation.

Gout and the risk of Parkinson's disease in older adults: a study of U.S. Medicare data.

BACKGROUND: In the presence of limited available data, our objective was to assess the association of gout with the risk of incident Parkinson's disease (PD) in adults 65 years or older. METHODS: We used the 5% random sample of Medicare claims data from 2006 to 2012 to examine the association of gout with incident PD. The multivariable Cox regression model adjusted for demographics, comorbidity, and common cardiovascular disease and gout medications. We calculated hazard ratios (HR) and 95% confidence interval (CI). Sensitivity analyses adjusted for comorbidity categorically, or individually and for additional cardiovascular comorbidities. RESULTS: In a cohort study, 1.72 million Medicare beneficiaries were eligible. The mean age was 75 years (standard deviation [SD], 7.6), 58% were female, 86% were White and 37% had Charlson-Romano comorbidity index score of ≥2. We found that 22,636 people developed incident PD, 1129 with gout and 21,507 without gout. The respective crude incidence rates of incident PD were 3.7 vs. 2.2 per 1000 person-years. We found that gout was associated with 1.14-times higher hazard ratio (95% CI, 1.07, 1.21) of PD in the main analysis; findings were confirmed in sensitivity analyses. We noted that the risk differed slightly by age; ages 65-75, 75-85 and > 85 had hazard ratios of incident PD with gout of 1.27 (95% CI, 1.16, 1.39), 1.07 (95% CI, 0.97, 1.16) and 0.97 (95% CI, 0.79, 1.20), respectively, but no gender or race differences were noted. CONCLUSIONS: Gout was associated with a higher risk of incident PD in older adults, with the risk being significant in the age group 65-75 years. Future studies need to assess the mechanisms of this increased risk.

Gout is associated with a higher risk of chronic renal disease in older adults: a retrospective cohort study of U.S. Medicare population.

BACKGROUND: Hyperuricemia and gout have been linked to chronic kidney disease (CKD). Whether the increased risk of CKD in gout is due to shared risk factors such as hypertension, diabetes or heart disease, or due to gout itself is not known. Studies in older adults, who tend to have a high incidence of CKD, are limited. Our objective was to assess whether gout was associated with incident CKD in older adults. METHODS: Using the 5% random sample of Medicare claims, we assessed whether gout is associated with higher risk of incident (new) CKD in adults 65 years or older, using multivariable-adjusted Cox regression analyses, adjusting for demographics (age, gender, race), medical comorbidity and common medications. We calculated hazard ratios (HR) and 95% confidence interval (CI). Sensitivity analyses varied comorbidity variable (models 2, 3), or limited CKD to the most specific codes. RESULTS: Of the 1,699,613 eligible people, 168,065 developed incident CKD; 150,162 people without gout and 17,903 people with gout. Respective crude incidence rates were 15.6 vs. 78.1 per 1000 person-years. We found that gout was associated with a higher risk of incident CKD in multivariable-adjusted analyses, HR was 3.05 (95% CI, 2.99, 3.10), with minimal attenuation in sensitivity analyses, with HR 2.96 (95% CI, 2.91, 3.01) (model 2, categorical Charlson-Romano) and 2.59 (95% CI, 2.54, 2.63) (model 3, individual Charlson-Romano comorbidities plus hypertension, heart disease, obesity, coronary artery disease). Sensitivity analyses that limited the CKD diagnostic codes to more specific codes, confirmed findings from the main models with respective HRs of 3.10 (95% CI, 3.05, 3.15; Model 1), 3.03 (95% CI, 2.97, 3.08; Model 2) and 2.60 (95% CI, 2.56, 2.65; Model 3). CONCLUSION: Gout was associated with a 3-fold higher risk of CKD, confirmed in multiple sensitivity analyses. Future studies should provide insights into underlying mechanisms that are responsible for an increased CKD risk in gout.

Gout and dementia in the elderly: a cohort study of Medicare claims.

BACKGROUND: Conflicting data in the literature raise the question whether gout, independent of its treatment, increases the risk of dementia in the elderly. Our objective was to assess whether gout in older adults is associated with the risk of incident dementia. METHODS: We used the 5% Medicare claims data for this observational cohort study. We used multivariable-adjusted Cox proportional hazard models to assess the association of gout with a new diagnosis of dementia (incident dementia), adjusting for potential confounders/covariates including demographics (age, race, sex), comorbidities (Charlson-Romano comorbidity index), and medications commonly used for cardiac diseases (statins, beta-blockers, diuretics, and angiotensin converting enzyme (ACE)-inhibitors) and gout (allopurinol and febuxostat). RESULTS: In our cohort of 1.71 million Medicare beneficiaries, 111,656 had incident dementia. The crude incidence rates of dementia in people without and with gout were 10.9 and 17.9 per 1000 person-years, respectively. In multivariable-adjusted analyses, gout was independently associated with a significantly higher hazard ratio of incident dementia, with a HR of 1.15 (95% CI, 1.12, 1.18); sensitivity analyses confirmed the main findings. Compared to age 65 to < 75 years, age 75 to < 85 and ≥ 85 years were associated with 3.5 and 7.8-fold higher hazards of dementia; hazards were also higher for females, black race or people with higher medical comorbidity. CONCLUSION: Gout was independently associated with a 15% higher risk of incident dementia in the elderly. Future studies need to understand the pathogenic pathways involved in this increased risk.

Comparative effectiveness of allopurinol versus febuxostat for preventing incident renal disease in older adults: an analysis of Medicare claims data.

OBJECTIVE: To assess the comparative effectiveness of allopurinol versus febuxostat for preventing incident renal disease in elderly. METHODS: In a retrospective cohort study using 2006-2012 Medicare claims data, we included patients newly treated with allopurinol or febuxostat (baseline period of 183 days without either medication). We used 5:1 propensity-matched Cox regression analyses to compare the HR of incident renal disease with allopurinol use (and dose) versus febuxostat (reference). Sensitivity analyses included multivariable-adjusted regression models. RESULTS: There were 31 465 new allopurinol or febuxostat treatment episodes in 26 443 patients; 8570 ended in incident renal disease. Crude rates of incident renal disease per 1000 person-years were 192 with allopurinol versus 338 with febuxostat. Crude rates of incident renal disease per 1000 person-years were lower with higher daily dose: allopurinol <200, 200-299 and ≥300 mg/day with 238, 176 and 155; and febuxostat 40 and 80 mg/day with 341 and 326, respectively. In propensity-matched analyses, compared with febuxostat, allopurinol use was associated with lower HR of incident renal disease, 0.61 (95% CI 0.49 to 0.77). Compared with febuxostat 40 mg/day, allopurinol doses <200, 200-299 and ≥300 mg/day were associated with lower HR of incident renal disease, 0.75 (95% CI 0.65 to 0.86), 0.61 (95% CI 0.52 to 0.73) and 0.48 (95% CI 0.41 to 0.55), respectively. Sensitivity analyses using multivariable-adjusted regression confirmed these findings. CONCLUSIONS: Allopurinol was associated with a lower risk of incident renal disease in elderly patients than febuxostat. Future studies need to examine the mechanism of this potential renal benefit of allopurinol.

Early Wound and Sternal Management Following Pediatric Cardiac Surgery.

Early postoperative wound management following congenital heart surgery remains an area without equipoise. Precautionary restrictions can impact quality of life, development, and delay access to other needed care. The influence of different practices on wound healing and complications is unknown. We surveyed Pediatric Acute Care Cardiology Collaborative member centers regarding postoperative wound closure, wound vacuum-assisted closure (VAC) use, sternal precautions, and restrictions in the early postoperative period. We analyzed responses using descriptive statistics. Responses were submitted by 35/46 (76%) centers. Most centers perform primary skin closure with subcutaneous sutures. Wound covers are removed after 48 h at 43% (15/35) of centers and after ≥72 h at 34% (12/35) of centers. For delayed sternal closure, 16 centers close skin with interrupted, externalized sutures, 5 utilize wound VAC-assisted closure, and 12 use variable practices. Generally, 33 centers use wound VACs for wound care. Patient selection for VAC use and length of therapy varies. We found great variability in duration of sternal precautions and in activity, bathing, and submersion restrictions. Finally, 29 centers require a waiting period between cardiothoracic surgery and other surgeries such as tracheostomy or gastrostomy tube placement. Postoperative wound and sternal management lack consistency across North American pediatric heart institutes. Some restrictive practices may prolong length of stay and/or negatively impact quality of life and neurodevelopment. Practices may also impact wound infection rates. Research linking practices with clinical outcomes is needed to better define standards of care and reduce potential negative consequences of overly conservative or aggressive practices.

A Novel Model for Xenograft Right Ventricle to Pulmonary Artery Conduit.

The last 40 years have shown dramatic improvement in outcomes for neonatal cardiac surgery for a spectrum of congenital heart disease diagnoses. With more patients surviving into adulthood, the long-term impact of initial management strategies of these patients has come into focus. This is particularly true for patients with pediatric heart valve disease. Many patients born with right ventricular to pulmonary artery (RVPA) discontinuity require placement of a valved conduit in the neonatal period. Valved conduit options are limited in this patient population due to patient size and inability to respond to somatic growth. Genetically engineered porcine (GEP) donors may offer a xenograft conduit alternative that can grow with the patient. We have developed a model utilizing GEP donor RVPA conduits placed in infantile nonhuman primate (NHP) recipients. Our recipient is maintained on single-drug immunosuppression and demonstrates no evidence of pulmonary valve insufficiency or stenosis during short-term follow-up. Further studies and long-term outcomes are necessary to determine the utility of this technology in human application.

Surgical pulmonary arterioplasty at bidirectional cavopulmonary anastomosis leads to favorable pulmonary hemodynamics at final stage palliation.

Objective: Pulmonary arterioplasty (PA plasty) at bidirectional cavopulmonary anastomosis (BDCA) is associated with increased morbidity, but outcomes to final stage palliation are unknown. We sought to determine the influence of PA plasty on pulmonary artery growth and hemodyamics at Fontan. Methods: We retrospectively reviewed clinical data and outcomes for BDCA patients from 2006 to 2018. PA plasty was categorized by extent (type 1-4), as previously described. Outcomes included pulmonary artery reintervention and mortality before final palliation. Results: Five hundred eighty-eight patients underwent BDCA. One hundred seventy-nine patients (30.0%) underwent concomitant PA plasty. Five hundred seventy (97%) patients (169 [94%] PA plasty) survived to BDCA discharge. One hundred forty out of 570 survivors (25%) required PA/Glenn reintervention before final stage palliation (59 out of 169 [35%]) PA plasty; 81 out of 401 (20%) non-PA plasty; P < .001). Twelve-, 24-, and 36-month freedom from reintervention after BDCA was 80% (95% CI, 74-86%), 75% (95% CI, 69-82%), and 64% (95% CI, 57-73%) for PA plasty, and 95% (95% CI, 93-97%), 91% (95% CI, 88-94%), and 81% (95% CI, 76-85%) for non-PA plasty (P < .001). Prefinal stage mortality was 37 (6.3%) (14 out of 169 PA plasty; 23 out of 401 non-PA plasty; P = .4). Five hundred four (144 PA plasty and 360 non-PA plasty) patients reached final stage palliation (471 Fontan, 26 1.5-ventricle, and 7 2-ventricular repair). Pre-Fontan PA pressure and pulmonary vascular resistance were 10 mm Hg (range, 9-12 mm Hg) and 1.6 mm Hg (range, 1.3-1.9 mm Hg) in PA plasty and 10 mm Hg (range, 8-12 mm Hg) and 1.5 mm Hg (range, 1.3-1.9 mm Hg) in non-PA plasty patients, respectively (P = .29, .6). Fontan hospital mortality, length of stay, and morbidity were similar. Conclusions: PA plasty at BDCA does not confer additional mortality risk leading to final palliation. Despite increased pulmonary artery reintervention, there was reliable pulmonary artery growth and favorable pulmonary hemodynamics at final stage palliation.