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Background: Recent reports suggest worse outcomes in pediatric orthotopic heart transplantation (OHT) for congenital heart disease (CHD) and restrictive cardiomyopathy (RCM). We examined early outcomes in these diverse groups of patients in comparison with patients with dilatated cardiomyopathy (DCM). Methods: From 2000 to 2011, 209 patients were included: 50 with CHD, 23 with RCM, and 136 with DCM. Early survival was studied, as was the occurrence of acute rejection, donor-specific antibodies (DSAs) and nondonor-specific antibodies (NSDAs), incidence of pulmonary hypertension (PHT), right ventricular failure (RVF), and the need for mechanical circulatory support (MCS). Results: The incidence of preoperative PHT was greatest in the RCM group (χ2 p = 0.0006); the requirement for mechanical support before OHT was greatest in patients with DCM. Thirty-day survival was 92.0%, 97.1%, and 100% for patients with CHD, DCM, and RCM respectively. The incidence of RVF was highest for patients with RCM (43.5%; versus CHD, 26.0%; versus DCM, 14.7%). One-year survival estimates for patients with CHD, DCM, and RCM were 92.0%, 97.8%, and 82.6%, respectively (log-rank p = 0.165). Multivariable analysis revealed 4 significant risk factors for mortality: age, incidence of acute rejection, preoperative PHT, and the presence of NDSAs. The occurrence of DSAs was similar, although there was a significantly higher incidence of NDSAs in the CHD and RCM groups (36.0% and 30.4%, respectively, versus 14.0% in the DCM group; χ2 p = 0.0024). Conclusions: Equivalent outcomes are achievable in pediatric OHT despite marked heterogeneity in anatomic and physiologic complexity in recipients. Physiologic factors such as PHT are likely to be more important than anatomic complexities in determining survival. The potential relevance of NDSAs warrants further investigation. [ABSTRACT FROM AUTHOR]

Care pathways are used increasingly worldwide to organize patient care. However, different views exist about their effectiveness. One of the reasons for this is that pathways are complex interventions. A recent Cochrane review was published which reported positive results, but although the Cochrane team performed excellent work with an enormous commitment, the conclusions may be inappropriate. To fully understand the potential and problems of care pathways, it is important to define (a) exactly what we are talking about (b) whether the study methods are appropriate, and (c) whether we can properly define the outcomes. [ABSTRACT FROM AUTHOR]

The question posed in the title of this article is: “Congenital Heart Surgery Databases Around the World: Do We Need a Global Database?” The answer to this question is “Yes and No”! Yes - we need to create a global database to track the outcomes of patients with pediatric and congenital heart disease. No–we do not need to create a new “global database.” Instead, we need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This review article will achieve the following objectives: (A) Consider the current state of analysis of outcomes of treatments for patients with congenitally malformed hearts. (B) Present some principles that might make it possible to achieve life-long longitudinal monitoring and follow-up. (C) Describe the rationale for the creation of a Global Federated Multispecialty Congenital Heart Disease Database. (D) Propose a methodology for the creation of a Global Federated Multispecialty Congenital Heart Disease Database that is based on linking together currently existing databases without creating a new database. To perform meaningful multi-institutional analyses, any database must incorporate the following six essential elements: (1) Use of a common language and nomenclature. (2) Use of a database with an established uniform core dataset for collection of information. (3) Incorporation of a mechanism to evaluate the complexity of cases. (4) Implementation of a mechanism to assure and verify the completeness and accuracy of the data collected. (5) Collaboration between medical and surgical subspecialties. (6) Standardization of protocols for life-long longitudinal follow-up. Analysis of outcomes must move beyond recording 30-day or hospital mortality, and encompass longer-term follow-up, including cardiac and non-cardiac morbidities, and importantly, those morbidities impacting health-related quality of life. Methodologies must be implemented in our databases to allow uniform, protocol-driven, and meaningful long-term follow-up. We need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This “Global Federated Multispecialty Congenital Heart Disease Database” will not be a new database, but will be a platform that effortlessly links multiple databases and maintains the integrity of these extant databases. Description of outcomes requires true multi-disciplinary involvement, and should include surgeons, cardiologists, anesthesiologists, intensivists, perfusionists, neurologists, educators, primary care physicians, nurses, and physical therapists. Outcomes should determine primary therapy, and as such must be monitored life-long. The relatively small numbers of patients with congenitally malformed hearts requires multi-institutional cooperation to accomplish these goals. The creation of a Global Federated Multispecialty Congenital Heart Disease Database that links extant databases from pediatric cardiology, pediatric cardiac surgery, pediatric cardiac anesthesia, and pediatric critical care will create a platform for improving patient care, research, and teaching related to patients with congenital and pediatric cardiac disease. [Copyright &y& Elsevier]

Quality-of-care evaluation must take into account variations in “case mix.” This study reviewed the application of two case-mix complexity-adjustment tools in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database: the Aristotle Basic Complexity (ABC) score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The 2006 STS Congenital Heart Surgery Database Report, the first STS report to incorporate both methods, included 45,635 operations from 47 centers. Each operation was assigned an ABC score in a range from 1.5 (lowest complexity) to 15 (highest complexity), an ABC level in a range from 1 (lowest complexity) to 4 (highest complexity), and a RACHS-1 category in a range from 1 (lowest risk) to 6 (highest risk). The overall discharge mortality was 3.9% (1,222/31,719 eligible cardiac index operations). Of the eligible cardiac index operations, 85.8% (27,202/31,719) were eligible for analysis by the RACHS-1 method, and 94.0% (29,813/31,719) were eligible for analysis by the ABC approach. With both RACHS-1 and ABC, as complexity increases, discharge mortality also increases. The ABC approach allows classification of more operations, whereas the RACHS-1 discriminates better at the higher end of complexity. Complexity stratification is a useful method for analyzing the impact of case mix on pediatric cardiac surgical outcomes. Both the RACHS-1 and ABC methods facilitate complexity stratification in the STS database. [ABSTRACT FROM AUTHOR]

Comments on an article about tracheal occlusion for congenital diaphragmatic hernia treatment in fetus. Processes involved in fetoscopic surgery; Outcome assessment following tracheal occlusion; Factors that need to be considered in fetal surgery.

This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from "exercise-prescription" rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Fast-track patient pathways for cardiac surgery are used in adult practice and by necessity is a mainstay in the developing world. We aimed to introduce a fast-track protocol for uncomplicated paediatric open-heart surgery cases and to subsequently review the results of this change in practice. Methods: A fast-track protocol co-ordinated by the Advanced Nurse Practitioners was introduced in January 2006 for children aged over 6 months undergoing uncomplicated open-heart procedures. We conducted a review of prospectively collected data on all included patients. The setting was a tertiary paediatric cardiac surgical centre in the UK. The outcome measures for audit were: patient fitness to leave the intensive care unit (ICU) on the day of surgery and hospital length of stay. Results: Included children had a mean age 6 (standard deviation (SD) 4.9) years and mean weight 22.7 (SD 17.6) kg. Of the 194 patients included, 153 (79%) were fit to leave the ICU on the day of surgery. Patients undergoing surgery for ventricular septal defect: odds ratio (OR) 2.8 (95% CI: 1.2–5.6) P =0.01 and left ventricular outflow tract obstruction: OR 5.5 (95% CI: 1.4–21.2) P =0.01, were more likely to be unfit than atrial septal defect and right ventricular outflow tract obstruction. Patients undergoing surgery in the afternoon were more likely to be unfit than those undergoing surgery in the morning: OR 2.3 (95% CI: 1.2–4.8) P =0.03. No relationship was found between age or weight and fitness to fast track. Median length of hospital stay for the whole cohort was 3 (range: 2–11) days. After adjustment for case mix, there was significant evidence that length of hospital stay reduced as experience with the protocol increased over the series of patients RC −0.02 (95% CI: −0.01 to −0.03) P <0.01. Conclusion: A fast-track programme can be implemented safely and effectively if the appropriate support including a step-down ward area is put in place. Greater experience with this type of protocol leads to reductions in the length of hospital stay for children aged over 6 months undergoing uncomplicated open-heart surgery. Fast-track cases should be performed in the morning. [Copyright &y& Elsevier]

Abstract: Objective: The aim of the study is to assess the surgical outcome of slide tracheoplasty in patients with congenital tracheal stenosis and single lung. Methods: Pre, intra- and postoperative data were collected. Anatomy and associated anomalies were described. Results: Seven patients (median age 5 months; range 39 days–1 year) with single lung underwent slide tracheoplasty. Single right lung was present in four patients. Associated anomalies were present in four patients (56%) including vascular ring, left pulmonary artery (LPA) sling, VACTERL (abnormalities of the vertebrae, anus, cardiovascular tree, trachea, oesophagus, renal system, and limb buds) syndrome, atrial septal defect (ASD) and aberrant left subclavian artery. Six patients (85%) needed preoperative ventilation and two (28%) needed preoperative extracorporeal membrane oxygenation (ECMO). Median postoperative ventilation was 7 days (6–35 days). Two patients needed postoperative ECMO, one of whom required preoperative ECMO. Complications occurred in five patients (71%): reintubation due to pneumothorax, pneumonia and several tracheal dilatations due to recurrent tracheal stenosis, which was eventually stented. There was one hospital death in a patient, who could not be weaned off ECMO due to severe distal malacia. At a median follow-up of 16 months (7 days–7 years), all survivors are in good clinical condition and without additional stenting. Conclusion: Slide tracheoplasty can be performed in patients with single lung and tracheal stenosis with a good surgical outcome. [Copyright &y& Elsevier]