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The Sano variant of the stage 1 Norwood procedure is commonly performed as initial palliation for hypoplastic left heart syndrome. We present a case of a 2-year-old who developed a mycotic pseudoaneurysm of the Sano graft and discuss the importance of imaging and operative management of this rare presentation. Localization and full description of the lesion require compulsive, often multimodality imaging.

Objective We previously described the hybrid comprehensive stage II operation as an alternate surgical procedure for a subset of patients with single ventricle congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the 4 patients who have undergone this procedure. Methods After undergoing a hybrid approach to the stage I Norwood palliation, the hybrid comprehensive stage II procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection. With this approach, dissection of the distal arch and creation of a Damus-Kaye-Stansel anastomosis was avoided. A standard Fontan procedure was planned after the usual period of growth. Results The first patient, who had trisomy 21 and elevated PA pressures, died postoperatively due to left PA thrombosis. The subsequent 3 patients survived the procedure and remain clinically well. All have required catheterizations for reintervention on their stented intrapulmonary baffles and ductal arches, and all have undergone successful completion of their Fontan procedures. Conclusions The hybrid comprehensive stage II is a feasible, less complex alternative to the conventional comprehensive stage II operation in a subset of patients with single ventricle physiology. Early postoperative anticoagulation therapy to avoid PA thrombosis is recommended, and restenting of the ductal arch is anticipated. Although the long-term consequences of separate outflow tracts supplying the upper and lower body is unknown, the 3 surviving patients with this circulation are doing well with their Fontan circulation at midterm follow-up.

A 13-month-old male sustained cardiac arrest under general anesthesia and was found to have left main coronary artery atresia with collateralization from the right coronary artery. After protracted recovery, the patient underwent coronary ostioplasty. Postoperative imaging confirmed patency of the newly established artery without stenosis. At 12 months, no changes in the vessel were noted. Ventricular function remains mildly decreased and stable.

Around 8% of all newborns with a Congenital Heart Defect (CHD) have only a single functioning ventricle. The Fontan operation has served as palliation for this anomaly for decades, but the surgery entails multiple complications, and the survival rate is less than 50% by adulthood. A rapidly testable novel alternative is proposed by creating a bifurcating graft, or Injection Jet Shunt (IJS), used to “entrain” the pulmonary flow and thus provide assistance while reducing the caval pressure. A dynamically scaled Mock Flow Loop (MFL) has been configured to validate this hypothesis. Three IJS nozzles of varying diameters 2, 3, and 4 mm with three aortic anastomosis angles and pulmonary vascular resistance (PVR) reduction have been tested to validate the hypothesis and optimize the caval pressure reduction. The MFL is based on a Lumped-Parameter Model (LPM) of a non-fenestrated Fontan circulation. The best outcome was achieved with the experimental testing of a 3 mm IJS by producing an average caval pressure reduction of more than 5 mmHg while maintaining the clinically acceptable pulmonary flow rate (Qp) to systemic flow rate (Qs) ratio of ~1.5. Furthermore, alteration of the PVR helped in achieving higher caval pressure reduction with the 3 mm IJS at the expense of an increase in Qp/Qs ratio.

Approximately $$1/2500$$ 1 / 2500 babies are born with only one functioning ventricle and the Fontan is the third and, ideally final staged palliative operation for these patients. This altered circulation is prone to failure with survival rates below $$50\%$$ 50 % into adulthood. Chronically elevated inferior vena cava (IVC) pressure is implicated as one cause of the mortality and morbidity in this population. An injection jet shunt (IJS) drawing blood-flow directly from the aortic arch to significantly lower IVC pressure is proposed. A computer-generated 3D model of a 2–4 year old patient with a fenestrated Fontan and a cardiac output of 2.3 L/min was generated. The detailed 3D pulsatile hemodynamics are resolved in a zero-dimensional lumped parameter network tightly-coupled to a 3D computational fluid dynamics model accounting for non-Newtonian blood rheology and resolving turbulence using large eddy simulation. IVC pressure and systemic oxygen saturation were tracked for various IJS-assisted Fontan configurations, altering design parameters such as shunt and fenestration diameters and locations. A baseline “failing” Fontan with a 4 mm fenestration was tuned to have an elevated IVC pressure (+ 17.8 mmHg). Enlargement of the fenestration to 8 mm resulted in a 3 mmHg IVC pressure drop but an unacceptable reduction in systemic oxygen saturation below 80%. Addition of an IJS with a 2 mm nozzle and minor volume load to the ventricle improved the IVC pressure drop to 3.2 mmHg while increasing systemic oxygen saturation above 80%. The salutary effects of the IJS to effectively lower IVC pressure while retaining acceptable levels of oxygen saturation are successfully demonstrated.

Background The hybrid comprehensive stage 2 (HCS2) procedure is a novel palliative operation applicable to a select subset of single ventricle patients with adequate native antegrade aortic flow to the upper body. Flow to the descending aorta, through the pulmonary outlet and ductal arch, is influenced by a stented intrapulmonary baffle connecting the branch pulmonary arteries. We used computational fluid dynamics (CFD) to elucidate the hemodynamic characteristics of this reconstruction. Methods We used multiscale CFD analysis of a synthetic, patient-derived HCS2 anatomic configuration with unsteady laminar flow conditions and a non-Newtonian blood model to quantify the resultant hemodynamics. The 3-dimensional CFD model was coupled to a 0-dimensional lumped parameter model of the peripheral circulation to determine the required boundary conditions. Results For the specific anatomy studied, the intrapulmonary baffle did not obstruct flow from the pulmonary trunk to ductal arch as long as the distance between the anterior pulmonary artery wall and baffle wall exceeded ∼7 mm. Vortex shedding off of the baffle wall did not develop, because of the short distance to the ductal arch. The stented baffle experienced significantly uneven “inward” loading from the systemic side. Pulmonary outlet flow separation distal to the baffle produced a low-speed recirculation region. Conclusions Hemodynamic patterns in this complex anatomy are generally favorable. Low flow recirculation could be mitigated by preoperative shape optimization. Calculated inward stresses on the pulmonary baffle can be used in the future to study baffle stent deformation, which is expected to be small.

IntroductionNew paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children’s Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice.MethodsThe PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities.ResultsA total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly.ConclusionsWe describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

Given the rising costs of health care in today's economic environment, the need for effective, value-driven care has never been more pressing. While the U.S. health care system strives continually to improve patient outcomes, it struggles with the inadequacies due to variation in care and the inefficiencies of unnecessary resource utilization. The tools traditionally used to study care, from retrospective studies to randomized controlled trials, may be inadequate to address the complicated, interdependent questions related to defining effective care. To overcome the deficiencies of these traditional tools and better optimize our health care system, a new kind of methodology is required--one that integrates the functionality of previously existing tools in a novel way. Standardized Clinical Assessment and Management Plans (SCAMPs) were designed to accomplish this goal. A SCAMP is a care pathway, designed by clinicians, to guide medical decision making around a particular disorder. SCAMPs are unique in that they invite knowledge-based diversions from their recommendations and are accompanied by data collection and continuous improvement processes. Through these mechanisms, SCAMPs successfully reduce practice variation, optimize resource use, and create an integrated medical learning system which overcomes many of the inadequacies of traditional research tools. As such, the SCAMP paradigm may represent an important breakthrough in the effort to define and implement effective health care.

Variability in medical practice in the United States leads to higher costs without achieving better patient outcomes. Clinical practice guidelines, which are intended to reduce variation and improve care, have several drawbacks that limit the extent of buy-in by clinicians. In contrast, standardized clinical assessment and management plans (SCAMPs) offer a clinician-designed approach to promoting care standardization that accommodates patients’ individual differences, respects providers’ clinical acumen, and keeps pace with the rapid growth of medical knowledge. Since early 2009 more than 12,000 patients have been enrolled in forty-nine SCAMPs in nine states and Washington, D.C. In one example, a SCAMP was credited with increasing clinicians’ rate of compliance with a recommended specialist referral for children from 19.6 percent to 75 percent. In another example, SCAMPs were associated with an 11–51 percent decrease in total medical expenses for six conditions when compared with a historical cohort. Innovative tools such as SCAMPs should be carefully examined by policy makers searching for methods to promote the delivery of high-quality, cost-effective care.

OBJECTIVE:A Standardized Clinical Assessment and Management Plan (SCAMP) standardizes the care of patients with a predefined diagnosis while actively inviting and collecting data on clinician deviations (DEVs) from its recommendations. For 3 different pediatric cardiac diagnoses managed by SCAMPs, we determined the frequency of, types of, and reasons for DEVs, which are considered to be a valuable source of information and innovation.METHODS:DEVs were collected as part of SCAMP implementation. DEVs were reviewed by the SCAMP committee chairperson and by a separate protocol deviation committee; they were characterized as either justifiable (J), possibly justifiable (PJ), or not justifiable (NJ).RESULTS:We analyzed 415 patients, 484 clinic encounters, and 216 DEVs. Eighty-six (39.8%) of the DEVs were J, 21 (9.7%) were PJ, and 109 (50.4%) were NJ. The percentage of NJ DEVs relative to the number of opportunities for DEV was 4.1%. J and PJ DEVs were mostly due to management of unrelated conditions (11% overall) or special circumstances (22% overall). NJ DEVs primarily involved follow-up intervals (66%) and deleted tests (24%). The reason for deviating from SCAMP recommendations was not given for 31% of DEVs, even though such information was requested.CONCLUSIONS:The overall low rate of NJ DEV suggests that practitioners generally accept SCAMP recommendations, but improved capture of practitioners' reasons for deviating from those recommendations is needed. This analysis revealed multiple opportunities for improving patient care, suggesting that this process can be useful in both promulgating sound practice and evolving improved approaches to patient management.

Introduction. Despite the growing importance of clinical guidelines, their adoption has encountered significant resistance among clinicians. We developed Standardized Clinical Assessment and Management Plans (SCAMPs) as an innovative, clinician-led approach to building, implementing, and constantly improving flexible guidelines. We hypothesized that SCAMPs would fit well within the culture of medicine and that clinicians would therefore prefer SCAMPs over other guidelines. Methods. We implemented an anonymous, computer-based survey to analyze provider attitudes toward SCAMPs at our institution. Results. Sixty-nine providers completed the questionnaire (73% response rate). Most providers reported a positive opinion about SCAMPs along axes of overall familiarity (83%), trust (91-94%), utility (75-87%), and overall attitude (64%). Fewer providers felt familiar with the SCAMP improvement process (60% neutral to unfamiliar) or knew that they played a role in this process (62% said no or unsure). Sixty-five percent reported experiencing an erosion in their autonomy with SCAMPs; when comparing this to other guidelines, 38% said other guidelines erode more, 26% felt SCAMPs erode more, and 36% were neutral. The plurality of providers chose SCAMPs as their preferred means to incorporate evidence-based medicine into their practice (46% vs 29% for clinical practice guidelines, 25% for other guidelines). Conclusion. Providers look upon SCAMPs favorably and believe that SCAMPs successfully address numerous barriers to guideline adoption. Furthermore, SCAMPs are the preferred means to incorporate evidence-based medicine into practice among providers surveyed. SCAMPs may represent an important step in building guidelines that fit into the culture of medicine, obtain clinician "buy-in," and better influence clinical decision making.

The current tools to adequately inform the process of improving health-care delivery consist primarily of retrospective studies, prospective trials, and clinical practice guidelines. We propose a novel and systematic approach that bridges the gap of our current tools to affect change, provides an infrastructure to improve health-care delivery, and identifies unnecessary resource utilization. The objective of this special article is to introduce the rationale and methods for this endeavor entitled "Standardized Clinical Assessment and Management Plans" (SCAMPs). SCAMPs take a relatively heterogeneous patient population and through a process of iterative analysis and modification of standardized assessment and management algorithms, SCAMPs allow the intrinsic biologic variability in a patient population to emerge and be understood. SCAMPs can be used to complement our currently available tools in order to result in incremental and sustained improvement in health-care delivery.

Introduction. A Standardized Clinical Assessment and Management Plan (SCAMP) is a novel quality improvement initiative that standardizes the assessment and management of all patients who carry a predefined diagnosis. Based on periodic review of systemically collected data the SCAMP is designed to be modified to improve its own algorithm. One of the objectives of a SCAMP is to identify and reduce resource utilization and patient care costs. Methods. We retrospectively reviewed resource utilization in the first 93 arterial switch operation (ASO) SCAMP patients and 186 age-matched control ASO patients. We compared diagnostic and laboratory testing obtained at the initial SCAMP clinic visit and control patient visits. To evaluate the effect of the SCAMP over time, the number of clinic visits per patient year and echocardiograms per patient year in historical control ASO patients were compared to the projected rates for ASO SCAMP participants. Results. Cardiac magnetic resonance imaging (MRI), stress echocardiogram, and lipid profile utilization were higher in the initial SCAMP clinic visit group than in age-matched control patients. Total echocardiogram and lung scan usage were similar. Chest X-ray and exercise stress testing were obtained less in SCAMP patients. ASO SCAMP patients are projected to have 0.5 clinic visits and 0.5 echocardiograms per year. Historical control patients had more clinic visits (1.2 vs. 0.5 visits/patient year, P < .01) and a higher echocardiogram rate (0.92 vs. 0.5 echocardiograms/ patient year, P < .01) Conclusion. Implementation of a SCAMP may initially lead to increased resource utilization, but over time resource utilization is projected to decrease.

Standardized Clinical Assessment and Management Plans (SCAMPs), a novel decision-making tool, were created to standardize care, optimize resource use, and improve patient outcomes.

OBJECTIVES: Chest pain is a common reason for referral to pediatric cardiologists and often leads to an extensive cardiac evaluation. The objective of this study is to describe current management practices in the assessment of pediatric chest pain and to determine whether a standardized care approach could reduce unnecessary testing. PATIENTS AND METHODS: We reviewed all patients, aged 7 to 21 years, presenting to our outpatient pediatric cardiology division in 2009 for evaluation of chest pain. Demographics, clinical characteristics, patient outcomes, and resource use were analyzed. RESULTS: Testing included electrocardiography (ECG) in all 406 patients, echocardiography in 175 (43%), exercise stress testing in 114 (28%), event monitoring in 40 (10%), and Holter monitoring in 30 (7%). A total of 44 (11%) patients had a clinically significant medical or family history, an abnormal cardiac examination, and/or an abnormal ECG. Exertional chest pain was present in 150 (37%) patients. In the entire cohort, a cardiac etiology for chest pain was found in only 5 of 406 (1.2%) patients. Two patients had pericarditits, and 3 had arrhythmias. We developed an algorithm using pertinent history, physical examination, and ECG findings to suggest when additional testing is indicated. Applying the algorithm to this cohort could lead to an ∼20% reduction in echocardiogram and outpatient rhythm monitor use and elimination of exercise stress testing while still capturing all cardiac diagnoses. CONCLUSIONS: Evaluation of pediatric chest pain is often extensive and rarely yields a cardiac etiology. Practice variation and unnecessary resource use remain concerns. Targeted testing can reduce resource use and lead to more cost-effective care.

A region-specific ENU mutagenesis screen was conducted to elucidate the functional content of proximal mouse Chr 5. We used the visibly marked, recessive, lethal inversion Rump White (Rw) as a balancer in a three-generation breeding scheme to identify recessive mutations within the ∼50 megabases spanned by Rw. A total of 1003 pedigrees were produced, representing the largest inversion screen performed in mice. Test-class animals, homozygous for the ENU-mutagenized proximal Chr 5 and visibly distinguishable from nonhomozygous littermates, were screened for fertility, hearing, vestibular function, DNA repair, behavior, and dysmorphology. Lethals were identifiable by failure to derive test-class animals within a pedigree. Embryonic lethal mutations (total of 34) were overwhelmingly the largest class of mutants recovered. We characterized them with respect to the time of embryonic death, revealing that most act at midgestation (8.5–10.5) or sooner. To position the mutations within the Rw region and to guide allelism tests, we performed complementation analyses with a set of new and existing chromosomal deletions, as well as standard recombinational mapping on a subset of the mutations. By pooling the data from this and other region-specific mutagenesis projects, we calculate that the mouse genome contains ∼3479–4825 embryonic lethal genes, or about 13.7%–19% of all genes.