Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

A Risk Management Strategy for Managing Critical Human Resource Changes in a Pediatric Heart Program.

BACKGROUND: Relocation, recruitment, or retirement of critical team members may lead to changes in the expertise pool that could threaten patient outcomes in a pediatric heart program. We developed a quality initiative aimed at risk management that uses risk-stratified case complexity and outcomes to guide a program during critical fluxes in the expert staff. The Ramp Down/Up protocol is a systematic, voluntary reduction in the complexity of cases performed, followed by a transparent and intentional escalation of case complexity. METHODS: Institutional Ethics Review Board approval for this quality initiative was obtained. Patient/caregiver consent for quality data collection is obtained at the time of hospital admission. Every surgical patient having their index cardiac surgical procedure at the Izaak Walton Killam (IWK) from January 1, 2003, to December 2015 is included. The Ramp Down/Up protocol evolved to have to 4 critical elements: (1) a trigger and a reduction in case complexity; (2) an external/objective expert observer; (3) an escalation in case complexity; and (4) data (qualitative and quantitative) collection and analysis. RESULTS: The Ramp Down/Up protocol was used 3 times over a 12-year period to address critical expert human resource challenges. The protocol was used for variable duration (3.5-9 months). Patient operative mortality was benchmarked to the Congenital Cardiac Surgery database, and outcomes were stable during and after protocol employment. CONCLUSIONS: A quality initiative aimed at risk management has allowed 1 pediatric heart team to ensure that patient outcomes were maintained during critical human resource changes.

CONTEXTE: La relocalisation, le recrutement ou le départ à la retraite de membres critiques d'une équipe peuvent amener des changements dans le bassin d'expertises pouvant compromettre les résultats obtenus par les patients au sein d'un programme de pédiatrie cardiaque. Nous avons conçu une initiative relative à la qualité de la gestion des risques, qui évalue la complexité des cas et les résultats, stratifiés selon le risque, afin d'orienter le programme durant les flux critiques de personnel spécialisé. Le protocole de réduction/d'augmentation réalise une diminution méthodique et délibérée de la complexité des cas suivie d'une intensification transparente et intentionnelle de celle-ci. MÉTHODOLOGIE: Cette initiative sur la qualité a obtenu l'approbation du comité d'examen de l'éthique des établissements. Le consentement du patient/de l'aidant requis pour la collecte de données sur la qualité est obtenu au moment de l'admission à l'hôpital. L'initiative incluait tous les patients de chirurgie pour lesquels on disposait de l'indice de chirurgie cardiaque établi au centre Izaak Walton Killiam (IWK) entre le 1er janvier 2003 et décembre 2015. Le protocole de réduction/d'augmentation a évolué pour intégrer quatre éléments critiques : 1) un facteur déclencheur et une réduction de la complexité des cas; 2) un observateur externe/expert objectif; 3) une intensification de la complexité des cas; 4) la collecte et l'analyse des données (qualitatives et quantitatives). RÉSULTATS: Le protocole de réduction/d'augmentation a été utilisé à trois reprises sur une période de 12 ans et sur des durées variables (de 3,5 à 9 mois) dans le but de relever des défis majeurs liés aux ressources humaines spécialisées. Les taux de mortalité opératoire des patients ont été comparés à ceux de la base de données sur les chirurgies cardiaques congénitales, et les résultats se sont révélés stables durant et après l'emploi du protocole. CONCLUSIONS: Une initiative sur la qualité ciblant la gestion des risques a permis à une équipe de pédiatrie cardiaque de s'assurer que les patients continuaient d'obtenir des résultats similaires lors de changements critiques des ressources humaines.

Early repair of congenital heart disease associated with increased rate of attention deficit hyperactivity disorder symptoms.

BACKGROUND: As more children survive with congenital heart disease, their neurodevelopmental outcomes (including attention deficit hyperactivity disorder [ADHD]) are becoming increasingly important. The objective of our study was to determine if school-aged children who underwent early cardiac surgery for congenital heart disease are more likely than healthy control subjects to have screening scores on the Swanson, Nolan, and Pelham IV (SNAP-IV) questionnaire suggestive of ADHD. METHODS: Children aged 7-15 years who underwent open-heart surgery before 1 year of age were identified from the Izaak Walton Killam (IWK) Children's Heart Centre Database. Control subjects were recruited from healthy volunteers. The SNAP-IV questionnaire was administered to all participants and a chart review was performed on all eligible children in the cardiac surgery group. Case and control subjects were compared using Fisher's exact test, linear, and logistic regression analyses. Potential predictors of a positive screening score were sought. RESULTS: A positive screening score was found in 29% (16/56) of the surgical group compared with 3% (2/60) of the control group (P < 0.001). Surgical and control subjects differed in average overall scores (0.93 vs 0.30; P < 0.001) and in scores for hyperactivity (0.83 vs 0.24; P < 0.001) and inattention (1.04 vs 0.37; P < 0.001). No other significant predictors of a positive screening score were identified. The early open-heart surgery participants who responded to the questionnaire did not differ in baseline characteristics compared with nonresponders. CONCLUSIONS: Children who have open-heart surgery at younger than 1 year of age are more likely than healthy control subjects to have a SNAP-IV score suggestive of ADHD when they reach school age.

Neutrophils are responsible for impaired medial smooth muscle cell recovery and exaggerated allograft vasculopathy in aortic allografts exposed to prolonged cold ischemia.

BACKGROUND: Ischemia and reperfusion injury is critical in allograft vasculopathy (AV) development. We have shown that neutrophil-mediated medial smooth muscle cell (SMC) loss precedes AV and that prolonged cold ischemia (CI) impairs medial SMC recovery and accelerates AV development. We hypothesize that neutrophils (NØs) are responsible for failed medial SMC recovery that precedes AV. METHODS: Aortic transplants were performed between fully disparate C3H/HeJ murine donors and wild-type C57BL/6 (WT B6), B6.129S7-Rag1 (Rag1(-/-); intact innate but no adaptive immunity), and B6.129S-Cybb (NOX2(-/-); NØ loss-of-function) recipients under cyclosporine A immunosuppression. Grafts were exposed to 20 or 60 minutes CI before transplant and harvested at 1 day, 2 weeks, and 8 weeks after transplant. Some WT B6 recipients were treated with remote ischemic pre-conditioning (rIPC). Grafts were assessed for medial SMCs, NØs, and lesion area. RESULTS: The 60-minute vs 20-minute CI grafts exhibited reduced SMC recovery at 2 weeks in WT B6 and Rag1(-/-) recipients (WT B6: p = 0.0009; Rag1(-/-): p = 0.0006). NØ influx was greater in Rag1(-/-) recipients of 60-minute vs 20-minute CI grafts at 1 day (p = 0.0002). The difference in 2-week medial SMC recovery between ischemia groups was abrogated in NOX2(-/-) recipients. At 8 weeks, NOX2(-/-) and rIPC recipients of 60-minute CI grafts exhibited smaller neointimal lesions than B6 recipients (NOX2(-/-): p = 0.0009; rIPC: p = 0.0005). CONCLUSIONS: Impaired medial SMC recovery in murine aortic allografts at 2 weeks occurs in the absence of adaptive immunity. Enhanced medial SMC recovery and reduced neointimal lesion formation in NOX2(-/-) and rIPC recipients of 60-minute CI grafts suggest a causal role for NØs in impaired medial SMC repopulation and the development of AV.

Complete revascularization is compromised in off-pump coronary artery bypass grafting.

OBJECTIVE: Patients who undergo off-pump coronary artery bypass grafting (OPCAB) commonly receive fewer bypass grafts and are more often incompletely revascularized compared with those receiving conventional coronary artery bypass (CCAB) recipients. Because this can compromise survival, we sought to determine whether patients undergoing OPCAB are incompletely revascularized and whether this affects long-term survival and freedom from cardiac events. METHODS: OPCAB cases (n = 411) performed from January 1, 1997 to June 30, 2003 were considered for inclusion and matching with 874 randomly selected, contemporary CCAB cases. After propensity matching, 308 OPCAB cases and 308 CCAB cases were included in the final analysis. We compared the number of bypass grafts and the completeness of revascularization by coronary territory. Survival and readmission for cardiac causes were monitored for up to 10 years postoperatively, with a median follow-up period of 5.9 years. RESULTS: On average, the patients undergoing OPCAB received significantly fewer distal anastomoses than did those undergoing CCAB (mean ± standard deviation, 2.6 ± 0.9 vs 3.0 ± 1.0, P < .0001). The circumflex territory was the most likely territory to be ungrafted during OPCAB in patients with angiographically significant obstruction (P = .0006). The frequency of complete revascularization was significantly different between the 2 groups (OPCAB, 79.2% vs CCAB, 88.3%; P = .0.002). The OPCAB group had a significantly greater rate of total arterial grafting (OPCAB, 66.6% vs CCAB, 49.7%; P = .0001). No difference was seen in 8-year survival or freedom from cardiac cause hospital readmission between the 2 groups. CONCLUSIONS: Despite receiving fewer distal anastomoses and the decreased frequency of complete revascularization, OPCAB and CCAB techniques produced comparable results.

Real-time complication monitoring in pediatric cardiac surgery.

BACKGROUND: As overall mortality rates have fallen in pediatric cardiac surgical procedures, complication monitoring is becoming an increasingly important metric of patient outcome. Currently there is no standardized method available to monitor severity-adjusted complications in congenital cardiac surgical procedures. METHODS: Complications associated with pediatric cardiac surgical procedures were prospectively collected from consecutive cases in a single pediatric cardiac surgical unit from October 1, 2009 to September 31, 2011. Complications were accounted for by frequency and severity and then stratified by surgical complexity, using the Risk Adjustment for Congenital Heart Surgery (RACHS) method, giving an average morbidity burden per RACHS category. "Expected" morbidity burden for each RACHS category was derived from year 1 (2009-2010) data. Observed minus expected (O:E) plots were then generated for the entire series of complications from year 2 (2010-2011) data. Separate O:E plots were also created for 5 complication classes and monitored for increases. RESULTS: There were 181 index surgical procedures performed in 178 patients. Two hundred and seventeen complications occurred in 80 procedures. The frequency and severity of complications increased with surgical complexity. The overall O:E plot was flagged twice for unanticipated increases in severity-adjusted complications. When the class-specific O:E plots were monitored for increases, the overall flags were found to originate from increased rates of infections and cardiac/operative complications. CONCLUSIONS: The O:E plot provides a simple and effective system to monitor complication rates over time based on severity-adjusted complication data. Grouping complications into classes allows us to identify specific subsets of complications that can be focused on to improve patient outcomes.

Improving and standardizing capture of pediatric cardiac surgical complications.

OBJECTIVE: Our objective was to establish baseline data and develop a tool to allow for systematic evaluation of pediatric cardiac surgical complications. As a first step, we examined the incidence and distribution of complications, risk stratified by case complexity in a single institution. With improving mortality rates for congenital heart surgery, the next frontier for improving patient outcomes is characterizing and reducing complications. Currently, no standardized approach is available to monitor the incidence and severity of all complications associated with a congenital cardiac surgery program. METHODS: Complications occurring in pediatric cardiac surgical patients (January 2006 to March 2009) were collected by database review applying standardized definitions. The surgical procedures were stratified by complexity to analyze the distribution of complications over the risk spectrum. Each complication was assigned a severity coefficient (1-3) used to calculate the combined effect of frequency and severity. The cumulative sum method was used to determined the trend of the adverse outcomes. RESULTS: Of 292 procedures, 84 (28.8%) were associated with a total of 150 complications. Of the 150 complications, 37 occurred in patients who died. The most common complications were arrhythmias (14.5%), cardiac (12.6%), and operative (12.6%). There was a linear relationship between the frequency and severity of complications and surgical complexity, as stratified using the Risk Adjustment for Congenital Heart Surgery category or Aristotle basic complexity levels (Spearman's coefficient = 1). CONCLUSIONS: When examined in a systematic fashion, the risk of complications in pediatric cardiac surgical patients is considerable. Our data illustrate that it is possible to track complications over time in a consistent manner. The effect of complication monitoring on patient outcomes remains to be proved.

Early innate immune events induced by prolonged cold ischemia exacerbate allograft vasculopathy.

BACKGROUND: Ischemia/reperfusion induced innate immune injury is inescapable in solid organ transplantation. Prolonged cold ischemia exacerbates the primary manifestation of late graft rejection, allograft vasculopathy (AV). The relationship between prolonged cold ischemia and late graft events is unclear and the subject of this study. METHODS: Aortic interposition transplants were performed between fully disparate mice treated with CyclosporineA. Allografts were exposed to 20 min or 60 min of cold ischemia and harvested between 1 d-6 wk. Lesion size, smooth muscle cells (SMC), neutrophils (NØ), and CD8+ T cells were quantified. RESULTS: Early SMC loss was identical in both groups. When compared to 20 min cold ischemia, grafts exposed to 60 min exhibited greater early NØ influx, greater SMC proliferation but fewer medial SMC at 1 wk and 2 wk. Subsequently, earlier and greater CD8+ T cell infiltration were seen in the 60 min group with larger lesions at every time point. CONCLUSIONS: These data suggest that the larger neointimal lesions in grafts exposed to 60 min cold ischemia result from enhanced early innate immune events resulting in impaired SMC recovery and subsequent increased adaptive immune response.

Neutrophil mediated smooth muscle cell loss precedes allograft vasculopathy.

BACKGROUND: Cardiac allograft vasculopathy (AV) is a pathological process of vascular remodeling leading to late graft loss following cardiac transplantation. While there is consensus that AV is alloimmune mediated, and evidence that the most important alloimmune target is medial smooth muscle cells (SMC), the role of the innate immune response in the initiation of this disease is still being elucidated. As ischemia reperfusion (IR) injury plays a pivotal role in the initiation of AV, we hypothesize that IR enhances the early innate response to cardiac allografts. METHODS: Aortic transplants were performed between fully disparate mouse strains (C3H/HeJ and C57BL/6), in the presence of therapeutic levels of Cyclosporine A, as a model for cardiac AV. Neutrophils were depleted from some recipients using anti-PMN serum. Grafts were harvested at 1,2,3,5d and 1,2wk post-transplant. Ultrastructural integrity was examined by transmission electron microscopy. SMC and neutrophils were quantified from histological sections in a blinded manner. RESULTS: Grafts exposed to cold ischemia, but not transplanted, showed no medial SMC loss and normal ultrastructural integrity. In comparison, allografts harvested 1d post-transplant exhibited > 90% loss of SMC (p < 0.0001). SMC partially recovered by 5d but a second loss of SMC was observed at 1wk. SMC loss at 1d and 1wk post-transplant correlated with neutrophil influx. SMC loss was significantly reduced in neutrophil depleted recipients (p < 0.01). CONCLUSIONS: These novel data show that there is extensive damage to medial SMC at 1d post-transplant. By depleting neutrophils from recipients it was demonstrated that a portion of the SMC loss was mediated by neutrophils. These results provide evidence that IR activation of early innate events contributes to the etiology of AV.

Inferior vena cava division to access post-repair pulmonary venous obstruction.

Post-repair pulmonary venous obstruction is a complication that may occur after surgical repair of total anomalous pulmonary veins. Obstruction may occur at the site of surgical anastomosis or it may be intrinsic to the pulmonary veins. The optimal approach to post-repair pulmonary venous obstruction remains controversial because of high mortality rates and persistent risk of re-stenosis. We present a previously undescribed, inferior approach to anastomotic post-repair pulmonary venous obstruction. This approach allowed easy access and excellent visualization of the pulmonary confluence. Three years after the reoperation, the patient had a widely patent confluence-to-left atrial anastomosis.

Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution.

BACKGROUND: Repair of total anomalous pulmonary venous connection (TAPVC) continues to be associated with significant mortality. We reviewed patients undergoing consecutive TAPVC repairs over a 10-year period at Children's Hospital Boston. The impact of current surgical and perioperative management strategies on short-term outcomes (postrepair pulmonary venous obstruction and mortality) is evaluated. METHODS: All patients with surgically corrected TAPVC from November 1989 to December 2000 were included. Charts were reviewed for patient demographics, operation variables, and postoperative course. RESULTS: There were 123 patients in the cohort, of which 72 (59%) were male. The median age and weight at operation were 10 days and 3.6 kg, respectively. Sixty-eight (55%) patients presented with pulmonary venous obstruction, and 65 (53%) underwent emergent TAPVC repair. Thirty-nine (32%) had single-ventricle anatomy, and 84 (68%) had two-ventricle anatomy. Thirty patients (24%) died. Kaplan-Meier survival at 1 month was 65% (95% confidence interval [CI], 55% to 75%) for single-ventricle patients versus 90% (95% CI, 90% to 100%) for two-ventricle patients; at 36 months it was 47% (95% CI, 35% to 59%) versus 87% (95% CI, 81% to 93%), respectively. By Cox multivariable regression analysis, a single ventricle (p < 0.001, hazard ratio, 4.8; 95% CI, 2.5 to 9.2) was an independent mortality risk factor. Prerepair pulmonary venous obstruction was a multivariate risk factor for death among single-ventricle patients. Postrepair pulmonary venous obstruction occurred in 11%. If year of operation is used as a predictor, two-ventricle patient survival has significantly improved (p < 0.05). CONCLUSIONS: Despite current interventions, single-ventricle patients continue to have a worse prognosis than two-ventricle patients.