Evaluating variation in pre-operative evaluation and planning for children undergoing atrial or ventricular septal defect repair.

BACKGROUND: CHD care is resource-intensive. Unwarranted variation in care may increase cost and result in poorer health outcomes. We hypothesise that process variation exists within the pre-operative evaluation and planning process for children undergoing repair of atrial septal defect or ventricular septal defect and that substantial variation occurs in a small number of care points. METHODS: From interviews with staff of an integrated congenital heart centre, an initial process map was constructed. A retrospective chart review of patients with isolated surgical atrial septal defect and ventricular septal defect repair from 7/1/2018 through 11/1/2020 informed revisions of the process map. The map was assessed for points of consistency and variability. RESULTS: Thirty-two surgical atrial septal defect/ventricular septal defect repair patients were identified. Ten (31%) were reviewed by interventional cardiology before surgical review. Of these, 6(60%) had a failed catheter-based closure and 4 (40%) were deemed inappropriate for catheter-based closure. Thirty (94%) were reviewed in case conference, all attended surgical clinic, and none were admitted prior to surgery. The process map from interviews alone identified surgery rescheduling as a point of major variability; however, chart review revealed this was not as prominent a source of variability as pre-operative interventional cardiology review. CONCLUSIONS: Significant variation in the pre-operative evaluation and planning process for surgical atrial septal defect/ventricular septal defect patients was identified. If such process variation is widespread through CHD care, it may contribute to variations in outcome and cost previously documented within CHD surgery. Future research will focus on determining whether the variation is warranted or unwarranted, associated health outcomes and cost variation attributed to these variations in care processes.

Mental Health Among People Presenting for Care of Physical Symptoms: The Factors Associated with Suicidality and Symptoms of Depression and Anxiety are Similar Across Specialties.

Background: To identify differences in thoughts of suicide and symptoms of depression and anxiety by specialty among people presenting for care of physical symptoms, we analyzed data from routine mental health measurement in a small multispecialty practice and asked: 1. Are there any differences in suicidality (analyzed as an answer of 1 or greater or 2 or greater on the Patient Health Questionnaire [PHQ] question 9) in non-specialty and various types of specialty care? 2. Are there any factors-including specialty-associated with symptoms of depression (mean PHQ score), PHQ thresholds (greater than 0, 3 or greater, 10 or greater), Generalized Anxiety Disorder instrument [GAD] score of 3 or greater, and either GAD score 3 or greater or PHQ score 3 or greater? and 3. What factors are associated with referral to a social worker? Methods: As part of routine specialty and non-specialty care, 13,211 adult patients completed a measure of symptoms of depression (PHQ) that included a question about suicidality and a measure of symptoms of anxiety (GAD). Factors associated with suicidality and symptoms of depression and anxiety at various thresholds, and visit with a social worker, were sought in multivariable models. Results: Accounting for potential confounding in multivariable analyses, a score higher than 0 on the suicidality question (present in 18% of people) was associated with men, younger age, English-speakers, and neurodegenerative specialty care. Symptoms of depression on their continuum and using various thresholds (28% of people had a PHQ score greater than 2) were associated with non-Spanish-speakers, younger age, women, and county insurance or Medicaid insurance. Care from the social worker was associated with PHQ score of 3 or greater and having any suicidal thoughts (score of 1 or greater on question 9) but was less common with Medicare or Commercial Insurance and less common in the unit treating cognitive decline. Conclusion: The notable prevalence of symptoms of depression and suicidality among people presenting for care of physical symptoms across specialties and the relatively similar factors associated with suicidality, symptoms of depression, and symptoms of anxiety at various thresholds suggests that both non-specialty and specialty clinicians can be vigilant for opportunities for improved mental health. Increased recognition that people seeking care for physical symptoms often have mental health priorities has the potential to improve comprehensive care strategies, alleviate distress, and reduce suicide.

Examining the Real-Life Journey of Individuals and Families Affected by Single-Ventricle Congenital Heart Disease.

Background The lifetime journey of patients with single-ventricle congenital heart disease is characterized by long-term challenges that are incompletely understood and still unfolding. Health care redesign requires a thorough understanding of this journey to create and implement solutions that improve outcomes. This study maps the lifetime journey of individuals with single-ventricle congenital heart disease and their families, identifies the most meaningful outcomes to them, and defines significant challenges in the journey. Methods and Results This qualitative research study involved experience group sessions and 1:1 interviews of patients, parents, siblings, partners, and stakeholders. Journey maps were created. The most meaningful outcomes to patients and parents and significant gaps in care were identified across the life journey. A total of 142 participants from 79 families and 28 stakeholders were included. Lifelong and life-stage specific journey maps were created. The most meaningful outcomes to patients and parents were identified and categorized using a "capability (doing the things in life you want to), comfort (experience of physical/emotional pain/distress), and calm (experiencing health care with the least impact on daily life)" framework. Gaps in care were identified and classified into areas of ineffective communication, lack of seamless transitions, lack of comprehensive support, structural deficiencies, and insufficient education. Conclusions There are significant gaps in care during the lifelong journey of individuals with single-ventricle congenital heart disease and their families. A thorough understanding of this journey is a critical first step in developing initiatives to redesign care around their needs and priorities. This approach can be used for people with other forms of congenital heart disease and other chronic conditions. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT04613934.

Designing an interprofessional dementia specialty clinic: Conceptualization and evaluation of a patient-centered model.

The need for blueprints to design specialty care interprofessional collaboration (IPC) models is urgent, given the expanding aging population and current challenges in dementia diagnosis and treatment. We describe key steps creating an interprofessional outpatient dementia specialty clinic, efforts to sustain the model, and evaluation of interprofessional effectiveness and clinician satisfaction. The conception for the Comprehensive Memory Center was informed by qualitative research methodologies including focus groups, interviews, and literature reviews. Quantitative evaluation included satisfaction surveys and team effectiveness measures. The IPC model diverges from typical dementia practices through its interprofessional team, visit structure, approach to decision-making, in-house services, and community collaborations. Team retreats and workshops helped build clinician knowledge of interprofessional values and practices to sustain the IPC model. In the first 3.5 years, we served nearly 750 patients and their caregivers. Team evaluation results revealed that increased access to consultation and sharing the workload and emotional burden were beneficial. The majority of team members preferred the IPC model to traditional models of clinical care.

Experiences and insights from partners of individuals with single-ventricle CHD: a pilot qualitative research study.

INTRODUCTION: With advances in care, an increasing number of individuals with single-ventricle CHD are surviving into adulthood. Partners of individuals with chronic illness have unique experiences and challenges. The goal of this pilot qualitative research study was to explore the lived experiences of partners of individuals with single-ventricle CHD. METHODS: Partners of patients ≥18 years with single-ventricle CHD were recruited and participated in Experience Group sessions and 1:1 interviews. Experience Group sessions are lightly moderated groups that bring together individuals with similar circumstances to discuss their lived experiences, centreing them as the experts. Formal inductive qualitative coding was performed to identify salient themes. RESULTS: Six partners of patients participated. Of these, four were males and four were married; all were partners of someone of the opposite sex. Themes identified included uncertainty about their partners' future health and mortality, becoming a lay CHD specialist, balancing multiple roles, and providing positivity and optimism. Over time, they took on a role as advocates for their partners and as repositories of medical history to help navigate the health system. Despite the uncertainties, participants described championing positivity and optimism for the future. CONCLUSIONS: In this first-of-its-kind pilot study, partners of individuals with single-ventricle CHD expressed unique challenges and experiences in their lives. There is a tacit need to design strategies to help partners cope with those challenges. Further larger-scale research is required to better understand the experiences of this unique population.

Lessons learned from value-based pediatric appendectomy care: A shared savings pilot model.

PURPOSE: We aim to assess the healthcare value achieved from a shared savings program for pediatric appendectomy. METHODS: All appendectomy patients covered by our health plan were included. Quality targets were 15% reduction in time to surgery, length of stay, readmission rate, and patient satisfaction. Quality targets and costs for an appendectomy episode in two 6-month performance periods (PP1, PP2) were compared to baseline. RESULTS: 640 patients were included (baseline:317, PP1:167, PP2:156). No quality targets were met in PP1. Two quality targets were met during PP2: readmission rate (-57%) and patient satisfaction. No savings were realized because the cost reduction threshold (-9%) was not met during PP1 (+1.7%) or PP2 (-0.4%). CONCLUSIONS: Payer-provider partnerships can be a platform for testing value-based reimbursement models. Setting achievable targets, identifying affectable quality metrics, considering case mix index, and allowing sufficient time for interventions to generate cost savings should be considered in future programs.

A prospective same day discharge protocol for pediatric appendicitis: Adding value to a common surgical condition.

PURPOSE: Standardized clinical pathways for simple appendicitis decrease length of stay and result in cost savings. We performed a prospective cohort study to assess a same day discharge (SDD) protocol for children with simple appendicitis. METHODS: All children undergoing laparoscopic appendectomy for simple appendicitis after protocol implementation (February 2016 to January 2017) were assessed. Length of stay (LOS), 30-day resource utilization (ED visits and hospital readmissions), patient satisfaction, and hospital accounting costs for SDD were compared to non-SDD patients. RESULTS: Of 602 children treated at our institution, 185 (31%) were successfully discharged per protocol. SDD patients had longer median PACU duration (3.0 vs. 1.0h, p<0.001), but postoperative LOS (4.4 vs. 17.4h, p<0.001) and overall LOS (17.1 vs. 31.2h, p<0.001) were significantly shorter. Complication rates (1.6% vs. 3.1%), ED visits (4.3% vs. 6.0%), and readmissions (0.5% vs. 2.4%) were not significantly different for SDD compared to non-SDD patients. However, SDD decreases total cost of an appendectomy episode ($8073 vs $8424, p=0.002), and patients report high satisfaction with their hospital experience (mean 9.4 out of 10). CONCLUSIONS: Safe and satisfactory outpatient management of pediatric simple appendicitis is achievable with appropriate patient selection. An SDD protocol can lead to significant generation of value to the healthcare system. LEVEL OF EVIDENCE: Prognosis study, Level II.

Family perception of unmet support needs following a diagnosis of congenital coronary anomaly in children: Results of a survey.

BACKGROUND: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families. METHODS: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed. RESULTS: A total of 31 (47%) families responded. Of these, 27 expressed the need to interact with other patients/families with AAOCA. The majority were interested in either face-to-face meetings (77%) or online support groups (71%). Regarding content of the meeting, 74% were interested in brief talks by medical personnel/families, 58% suggested informal interactions with families, 55% proposed a structured discussion with a moderator and 39% mentioned fun activities/games. Regarding participants in these meetings, 90% would like to include healthcare providers, 61% suggested including family friends, 58% wished to include psychologists and 16% mentioned including social workers. The families currently use various social media including Facebook (87%), YouTube (39%), Google+ (36%), and LinkedIn (32%). For future online resources, 77% of families would like a Facebook site, an informative website (58%), a blog (52%), or an open forum (29%). The majority of the families (77%) were interested in attending a dedicated AAOCA meeting. CONCLUSION: There appears to be an unmet need for family support in those affected by AAOCA, a substantial life changing diagnosis for patients and families. Further research is needed to assess quality of life in this population.

Time-driven activity-based costing: A dynamic value assessment model in pediatric appendicitis.

OBJECTIVES: Healthcare reform policies are emphasizing value-based healthcare delivery. We hypothesize that time-driven activity-based costing (TDABC) can be used to appraise healthcare interventions in pediatric appendicitis. METHODS: Triage-based standing delegation orders, surgical advanced practice providers, and a same-day discharge protocol were implemented to target deficiencies identified in our initial TDABC model. Post-intervention process maps for a hospital episode were created using electronic time stamp data for simple appendicitis cases during February to March 2016. Total personnel and consumable costs were determined using TDABC methodology. RESULTS: The post-intervention TDABC model featured 6 phases of care, 33 processes, and 19 personnel types. Our interventions reduced duration and costs in the emergency department (-41min, -$23) and pre-operative floor (-57min, -$18). While post-anesthesia care unit duration and costs increased (+224min, +$41), the same-day discharge protocol eliminated post-operative floor costs (-$306). Our model incorporating all three interventions reduced total direct costs by 11% ($2753.39 to $2447.68) and duration of hospitalization by 51% (1984min to 966min). CONCLUSION: Time-driven activity-based costing can dynamically model changes in our healthcare delivery as a result of process improvement interventions. It is an effective tool to continuously assess the impact of these interventions on the value of appendicitis care. LEVEL OF EVIDENCE: II, Type of study: Economic Analysis.

Time-driven activity-based costing to identify opportunities for cost reduction in pediatric appendectomy.

PURPOSE: As reimbursement programs shift to value-based payment models emphasizing quality and efficient healthcare delivery, there exists a need to better understand process management to unearth true costs of patient care. We sought to identify cost-reduction opportunities in simple appendicitis management by applying a time-driven activity-based costing (TDABC) methodology to this high-volume surgical condition. METHODS: Process maps were created using medical record time stamps. Labor capacity cost rates were calculated using national median physician salaries, weighted nurse-patient ratios, and hospital cost data. Consumable costs for supplies, pharmacy, laboratory, and food were derived from the hospital general ledger. RESULTS: Time-driven activity-based costing resulted in precise per-minute calculation of personnel costs. Highest costs were in the operating room ($747.07), hospital floor ($388.20), and emergency department ($296.21). Major contributors to length of stay were emergency department evaluation (270min), operating room availability (395min), and post-operative monitoring (1128min). The TDABC model led to $1712.16 in personnel costs and $1041.23 in consumable costs for a total appendicitis cost of $2753.39. CONCLUSION: Inefficiencies in healthcare delivery can be identified through TDABC. Triage-based standing delegation orders, advanced practice providers, and same day discharge protocols are proposed cost-reducing interventions to optimize value-based care for simple appendicitis. LEVEL OF EVIDENCE: II.

Incorporating longitudinal pediatric patient-centered outcome measurement into the clinical workflow using a commercial electronic health record: a step toward increasing value for the patient.

Patient-centered outcomes measurement provides healthcare organizations with crucial information for increasing value for patients; however, organizations have struggled to obtain outcomes data from electronic health record (EHR) systems. This study describes how Texas Children's Hospital customized a commercial EHR system and assembled a cross-functional team to capture outcomes data using existing functionality. Prior to its installation and customization, no surgical subspecialties besides the congenital heart and transplant surgery groups conducted prospective, patient outcomes measurement, but by 2015, the outcomes of over 1300 unique patients with supracondylar fractures, cleft lip and/or palate, or voiding dysfunction had been tracked. Key factors for integrating outcomes measurement into the clinical workflow include ongoing communication between cross-functional teams composed of clinicians and technical professionals, an iterative design process, organizational commitment, and prioritizing measurement as early as possible during EHR optimization.

Anomalous aortic origin of a coronary artery: toward a standardized approach.

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients.

Aortic arch advancement for aortic coarctation and hypoplastic aortic arch in neonates and infants.

BACKGROUND: The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch. METHODS: All infants who underwent AAA at our institution from 1995 to 2012 were included. AAA consisted of coarctectomy and end-to-side anastomosis of the descending aorta to the distal ascending aorta/proximal arch through a median sternotomy. The cohort was divided into four groups: (1) isolated AAA (n=29, 11%), (2) AAA with closure of ventricular septal defect (n=56, 20%), (3) AAA with other biventricular repairs (n=115, 42%), and (4) AAA as part of single-ventricle palliation (n=75, 27%). RESULTS: The cohort included 275 patients: 125 (45%) were female, and the median age was 14 days (interquartile range, 7-34 days). Genetic abnormalities were present in 48 patients (17%). Neurologic adverse events occurred in 3 patients (1%), all in group 4. Left bronchial compression was seen in 2 patients (0.7%); only one required intervention. Vocal cord dysfunction was noted in 36 of 95 patients (38%) on routine laryngoscopy. Only 1 patient had clinical residual dysfunction at the last follow-up visit. Perioperative mortality was 3% (n=8). At a median follow-up time of 6 years, 8 patients (3%) had reinterventions at a median time of 5 months (3-17 months) after repair. CONCLUSIONS: AAA is a safe, effective, and durable operation with low rates of adverse events and mid-term reintervention. The advantages include native tissue-to-tissue reconstruction and preserved potential for growth. As such, it is the ideal technique for the management of hypoplastic aortic arch in neonates and infants.

Outcomes after the palliative arterial switch operation in neonates with single-ventricle anatomy.

BACKGROUND: Newborns with single-ventricle anatomy, transposition of the great arteries, and systemic outflow obstruction are challenging patients most often managed with a Norwood or Damus-Kaye-Stansel (DKS) procedure. The palliative arterial switch operation (pASO) offers the theoretical advantage of avoiding a systemic-to-pulmonary artery shunt physiology and posterior entrapment of the left pulmonary artery while aligning the single left ventricle with the posterior semilunar valve. Limited outcome data exist for the pASO. The purpose of this study was to examine the clinical course of patients after this operation. METHODS: We conducted a retrospective review of all neonates undergoing a pASO at our institution from July 1995 to June 2011. RESULTS: Fourteen patients underwent pASO at a median age of 7 days (2-16 days). Primary diagnoses included double-inlet left ventricle (6 patients [43%]), tricuspid atresia (TA) (4 patients [29%]), and other (4 patients [29%]). Concomitant procedures at initial operation included aortic arch reconstruction (13 patients [93%]), pulmonary artery banding (6 patients [43%]), and placement of a systemic-to-pulmonary artery shunt (1 patient [7%]). Median intensive care unit stay and hospital length of stay were 11 days (4-60 days) and 21 days (9-84), respectively. There were no deaths. All patients had advanced to a bidirectional cavopulmonary shunt (BCPS). Four patients required intervention between the pASO and BCPS procedures: systemic-to-pulmonary artery shunt (3 patients [21%]) and pulmonary artery banding revision (1 patient [7%]). Eleven patients had Fontan completion; the remaining 3 patients are candidates for this procedure. At the time of the Fontan operation, 7 (64%) patients required pulmonary artery augmentation. All patients were alive at last follow-up (median, 5 years [3 months-14 years]). At last echocardiographic follow-up (median, 5 years [3 months-10 years]), all patients had normal ventricular function, trivial to mild neoaortic insufficiency, and no left ventricular outflow obstruction. CONCLUSIONS: The pASO should be considered in neonates with single ventricle, transposition, and systemic outflow obstruction because it affords a favorable anatomic arrangement for long-term palliation, with excellent survival and preserved ventricular function.

Prospective trial of a pediatric ventricular assist device.

BACKGROUND: Options for mechanical circulatory support as a bridge to heart transplantation in children with severe heart failure are limited. METHODS: We conducted a prospective, single-group trial of a ventricular assist device designed specifically for children as a bridge to heart transplantation. Patients 16 years of age or younger were divided into two cohorts according to body-surface area (cohort 1, <0.7 m(2); cohort 2, 0.7 to <1.5 m(2)), with 24 patients in each group. Survival in the two cohorts receiving mechanical support (with data censored at the time of transplantation or weaning from the device owing to recovery) was compared with survival in two propensity-score-matched historical control groups (one for each cohort) undergoing extracorporeal membrane oxygenation (ECMO). RESULTS: For participants in cohort 1, the median survival time had not been reached at 174 days, whereas in the matched ECMO group, the median survival was 13 days (P<0.001 by the log-rank test). For participants in cohort 2 and the matched ECMO group, the median survival was 144 days and 10 days, respectively (P<0.001 by the log-rank test). Serious adverse events in cohort 1 and cohort 2 included major bleeding (in 42% and 50% of patients, respectively), infection (in 63% and 50%), and stroke (in 29% and 29%). CONCLUSIONS: Our trial showed that survival rates were significantly higher with the ventricular assist device than with ECMO. Serious adverse events, including infection, stroke, and bleeding, occurred in a majority of study participants. (Funded by Berlin Heart and the Food and Drug Administration Office of Orphan Product Development; ClinicalTrials.gov number, NCT00583661.).

Interstage attrition between bidirectional Glenn and Fontan palliation in children with hypoplastic left heart syndrome.

OBJECTIVE: With improving operative mortality for staged palliation of hypoplastic left heart syndrome, interstage death accounts for an increasing proportion of hypoplastic left heart syndrome mortality. We investigated risk factors for death or cardiac transplantation during the interstage period between bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome. METHODS: Patients with hypoplastic left heart syndrome who underwent bidirectional Glenn between August 1995 and June 2007 were screened. Standard risk patients, defined by having been discharged after both Norwood and bidirectional Glenn, were included for analysis. Patient demographic, echocardiographic, cardiac catheterization, and operative data were reviewed. Interstage attrition was defined as death or cardiac transplantation more than 30 days after bidirectional Glenn and before the Fontan procedure. Statistical analysis was carried out using the Student t test, Pearson chi-square correlation, and Cox proportional hazard modeling for multivariable analysis. RESULTS: Ninety-two patients with hypoplastic left heart syndrome were alive at 30 days after bidirectional Glenn. Of these patients, 8 died and 3 underwent cardiac transplantation at a median of 391 days (range, 59-1175 days) after bidirectional Glenn, yielding an interstage attrition rate of 12%. Removing the 7 patients who are still awaiting Fontan (but all of whom are at least 3.5 years after bidirectional Glenn) adjusts the attrition rate to 13%. Interstage attrition did not correlate with hemodynamic data obtained at cardiac catheterization, aortic arch obstruction, or right ventricular dysfunction. Multivariable analysis demonstrated that the presence of moderate or severe tricuspid valve regurgitation (hazard ratio, 6.02; 95% confidence interval, 1.56-23.24; P < .01) and weight z score (hazard ratio, 0.38; 95% confidence interval, 0.16-0.88; P = .02) were independent preoperative risk factors for interstage attrition. CONCLUSIONS: Interstage attrition between bidirectional Glenn and Fontan procedures occurred in 12% of our study population. Moderate or greater tricuspid valve regurgitation and low weight z score at the time of bidirectional Glenn are important risk factors for interstage attrition between the bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome.

Extracellular matrix remodeling and cell phenotypic changes in dysplastic and hemodynamically altered semilunar human cardiac valves.

INTRODUCTION: Congenital cardiac valve disease is common, affecting ∼1% of the population, with substantial morbidity and mortality, but suboptimal treatment options. Characterization of the specific matrix and valve cell phenotypic abnormalities in these valves could lend insight into disease pathogenesis and potentially pave the way for novel therapies. METHODS: Thirty-five human aortic and pulmonic valves were categorized based on gross and microscopic assessment into control valves (n=21); dysplastic valves, all except one also displaying hemodynamic changes (HEMO/DYSP, n=6); and hemodynamically altered valves (HEMO, n=8). Immunohistochemistry was performed on valve sections and flow cytometry on valvular interstitial cells. RESULTS: While both hemodynamically altered aortic and pulmonic valves demonstrated increased collagen turnover and cell activation, prolyl 4-hydroxylase and hyaluronan increased in hemodynamically altered aortic valves but decreased in hemodynamically altered pulmonic valves relative to control valves (P<.001). HEMO/DYSP aortic valves demonstrated decreased collagen and elastic fiber synthesis and turnover compared to both hemodynamically altered aortic valves and control aortic valves (each P<.006). Valvular interstitial cells from both hemodynamically altered and HEMO/DYSP pulmonic valves showed altered cell phenotype compared to control valves (each P<.032), especially increased non-muscle myosin. Furthermore, valvular interstitial cells from hemodynamically altered pulmonic valves and HEMO/DYSP aortic and pulmonic valves each demonstrated greater size and complexity compared to control valves (each P<.05). CONCLUSIONS: Dysplastic semilunar valves displayed alterations in collagen and elastic fiber turnover that were distinct from valves similarly exposed to altered hemodynamics as well as to control valves. These results demonstrate that dysplastic valves are not simply valves with gross changes or loss of leaflet layers, but contain complex matrix and cell phenotype changes that, with future study, could potentially be targets for novel nonsurgical treatments.

Development of factor V and thrombin inhibitors in children following bovine thrombin exposure during cardiac surgery: a report of three cases.

Factor V and thrombin inhibitors may develop following exposure to bovine thrombin preparations. In patient populations where exposure to bovine thrombin is common, such as children undergoing cardiovascular surgery, the development of such inhibitors should be considered in the evaluation of prolonged prothrombin times. We present three cases of children developing factor V and thrombin inhibitors following repeated exposure during cardiac surgical procedures.

Discontinuous pulmonary arteries do not preclude good Fontan outcomes.

OBJECTIVE: Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. DESIGN: The study is a retrospective case series. PATIENTS: From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. OUTCOME MEASURES: The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. RESULTS: The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1-2.1). Median follow-up after Fontan = 4.4 years (range: 1.2-9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428-0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. CONCLUSIONS: This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.