Medical missions for the provision of paediatric cardiac surgery in low- and middle-income countries.

This review will outline the role of visiting cardiac surgical teams in low- and middle-income countries drawing on the collective experience of the authors in a wide range of locations. Requests for assistance can emerge from local programmes at a beginner or advanced stage. However, in all circumstances, careful pre-trip planning is necessary in conjunction with clinical and non-clinical local partners. The clinical evaluation, surgical procedures, and postoperative care all serve as a template for collaboration and education between the visiting and local teams in every aspect of care. Education focusses on both common and patient-specific issues. Case selection must appropriately balance the clinical priorities, safety, and educational objectives within the time constraints of trip duration. Considerable communication and practical challenges will present, and clinicians may need to make significant adjustments to their usual practice in order to function effectively in a resource-limited, unfamiliar, and multilingual environment. The effectiveness of visiting trips should be measured and constantly evaluated. Local and visiting teams should use data-driven evaluations of measurable outcomes and critical qualitative evaluation to repeatedly re-assess their interim goals. Progress invariably takes several years to achieve the final goal: an autonomous self-governing, self-financed, cardiac programme capable of providing care for children with complex CHD. This outcome is consistent with redundancy for the visiting trips model at the site, although fraternal, professional, and academic links will invariably remain for many years.

Experience with an Acuity Adaptable Care Model for Pediatric Cardiac Surgery.

BACKGROUND: We describe the implementation of and outcomes associated with an acuity adaptable care model for pediatric patients undergoing cardiac surgery. METHODS: Consecutive patients undergoing an index cardiac operation between July 2007 and June 2015 were included. From July 2007 through June 2010, a conventional model existed in which patients moved among units and care teams based on age, severity of illness, and operative status (conventional group). A transitional period existed between July 2010 and June 8, 2012 (transitional group). From June 9, 2012, through June 2015, an acuity adaptable model was used in which patients remained in the cardiac care unit and received care from the same clinical team throughout their hospitalization (acuity adaptable group). RESULTS: Included were 2,363 patients: 925 in the conventional group, 520 in the transitional group, and 918 in the acuity adaptable group. In relation to the conventional group, the adjusted odds of operative mortality in the acuity adaptable group was 0.55 (95% confidence interval: 0.26-1.18; P = .12). The failure to rescue rate (ie, number of deaths in patients with any complication divided by the number of total patients with any complication) decreased (conventional group, 8.7%; acuity adaptable group, 4.2%; P = .04). In relation to the conventional group, postoperative hospital length of stay tended to be shorter in the acuity adaptable group ( P = .07). CONCLUSIONS: The implementation of an acuity adaptable care model was feasible in our pediatric cardiac program. The favorable associations identified between the new model and outcomes are promising but warrant confirmation in a larger, multicenter study.

Fluid Management: Pharmacologic and Renal Replacement Therapies.

OBJECTIVES: Focusing on critically ill children with cardiac disease, we will review common causes of fluid perturbations, clinical recognition, and strategies to minimize and treat fluid-related complications. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: Meticulous fluid management is vital in critically ill children with cardiac disease. Fluid therapy is important to maintain adequate blood volume and perfusion pressure in order to support cardiac output, tissue perfusion, and oxygen delivery. However, fluid overload and acute kidney injury are common and are associated with increased morbidity and mortality. Understanding the etiologies for disturbances in volume status and the pathophysiology surrounding those conditions is crucial for providing optimal care.

Pharmacological Manipulation of Peripheral Vascular Resistance in Special Clinical Situations after Pediatric Cardiac Surgery.

Pediatric cardiac surgery patients commonly suffer from alterations in vascular tone in the early post-operative period. Pharmacologic manipulation of systemic vascular resistance (SVR) can be complex in a variety of special patient situations including extremes of age, presence of left sided valvar lesions and the use of mechanical circulatory support. Familiarity with how these special circumstances alter SVR and the response to pharmacologic intervention will allow for tailored therapy and hopefully, optimized outcomes. This article addresses the eighth topic of the special issue entitled "Pharmacologic strategies with afterload reduction in low cardiac output syndrome after pediatric cardiac surgery".

Paediatric cardiac surgery in low-income and middle-income countries: a continuing challenge.

Despite advances in surgical and catheter-based treatment for congenital heart disease (CHD), there remain wide disparities across the globe. Ongoing international humanitarian and in-country programmes are working to address these issues with the ultimate goal to increase the quality and quantity of paediatric cardiac care, particularly in under-served regions of the world. This review aims to illustrate the reasons for these inequalities and suggests novel ways of improving access and sustainability of CHD programmes in low-income and middle-income countries.

Pediatric cardiac surgery in low- and middle-income countries or emerging economies: a continuing challenge.

A number of recent publications, addresses, seminars, and conferences have addressed the global backlog and increasing incidence of both congenital and acquired cardiac diseases in children, with reference to early and delayed recognition, late referral, availability of and access to services, costs, risks, databases, and early and long-term results and follow-up. A variety of proposals, recommendations, and projects have been outlined and documented. The ultimate goal of these endeavors is to increase the quality and quantity of pediatric cardiac care and surgery worldwide and particularly in underserved areas. A contemporary review of past and present initiatives is presented with a subsequent focus on the more challenging areas.

Current Perioperative Anticoagulation Practices in Children with Prosthetic Mechanical Heart Valves.

OBJECTIVE: This study investigated the clinician practices on perioperative anticoagulation in children with prosthetic mechanical heart valves who undergo elective surgeries. DESIGN: An online survey was administered to members of PediHeartNet. The survey consisted of multiple choice questions and clinical scenarios. OUTCOME MEASURES: The study described clinical practice patterns and variables that influence the clinicians' bridging anticoagulation decisions. RESULTS: Ninety-one respondents completed the survey; 68% were affiliated with university settings; 91% were pediatric cardiologists, and 49% had ≥10 years of experience in pediatric cardiology. Approximately one-half of the respondents (54%) independently provided perioperative anticoagulation management to their patients, while 46% utilized cardiac or hematology anticoagulation services. Resources that influenced bridging decisions included hematology experts (20%), American College of Chest Physicians guidelines (34%), and the clinicians' personal experience (56%). In planning for major surgeries, 47% of the respondents hospitalized patients for unfractionated heparin (UFH) and 46% prescribed outpatient low molecular weight heparin (LMWH). For minor surgeries, 58% hospitalized patients for UFH, 22% prescribed outpatient LMWH, and 17% opted out of bridging anticoagulation. Immediately after mitral valve replacement, 23% used bridging anticoagulation with UFH. When LMWH was used, there were no reports of thromboembolic complications. Major bleeding complications were rare and reported by 2% of the respondents. CONCLUSIONS: This was the first documentation that clinical practice of bridging perioperative anticoagulation in children with mechanical heart valves varies widely among pediatric cardiac specialists. There is poor adoption of published guidelines and a tendency toward more conservative strategies. Further studies comparing the safety and efficacy of LMWH vs. UFH as perioperative anticoagulation agents in children with mechanical heart valves are needed to further clarify our findings. Quality assurance initiatives and education are also needed to improve guidelines adherence and standardize practice management.

Survey of nongovernmental organizations providing pediatric cardiovascular care in low- and middle-income countries.

BACKGROUND: Nearly 90% of the children with heart disease in low- and middle-income countries (LMICs) cannot access cardiovascular (CV) services. Limitations include inadequate financial, human, and infrastructure resources. Nongovernmental organizations (NGOs) have played crucial roles in providing clinical services and infrastructure supports to LMICs CV programs; however, these outreach efforts are dispersed, inadequate, and lack coordination. METHODS: A survey was sent to members of the World Society for Pediatric and Congenital Heart Society and PediHeart. RESULTS: A clearinghouse was created to provide information on NGO structures, geographic reach, and scope of services. The survey identified 80 NGOs supporting CV programs in 92 LMICs. The largest outreach efforts were in South and Central America (42%), followed by Africa (18%), Europe (17%), Asia (17%), and Asia-Western Pacific (6%). Most NGOs (51%) supported two to five outreach missions per year. The majority (87%) of NGOs provided education, diagnostics, and surgical or catheter-based interventions. Working jointly with LMIC partners, 59% of the NGOs performed operations in children and infants; 41% performed nonbypass neonatal operations. Approximately a quarter (26%) reported that partner sites do not perform interventions in between missions. CONCLUSIONS: Disparity and inadequacy in pediatric CV services remain an important problem for LMICs. A global consensus and coordinated efforts are needed to guide strategies on the development of regional centers of excellence, a global outcome database, and a CV program registry. Future efforts should be held accountable for impacts such as growth in the number of independent LMIC programs as well as reduction in mortality and patient waiting lists.

Genetic and clinical determinants influencing warfarin dosing in children with heart disease.

Warfarin is a common anticoagulant with narrow therapeutic window and variable anticoagulation effects. Single gene polymorphisms in cytochrome P450 2C9 (CYP2C9) and vitamin K epoxide reductase (VKORC1) have been shown to impact warfarin dosing in adults. Insufficient data exists on genetic and clinical factors which influence warfarin dosing in children. Pediatric patients with heart disease who received long-term warfarin therapy were tested for VKORC1 and CYP2C9 polymorphisms. Clinical and demographic data were reviewed in those children who achieved stable therapeutic international normalized ratio (INR). Multiple linear regression modeling was used to assess relationships between stable warfarin doses and genetic or clinical variables. Fifty children were tested for VKORC1 and CYP2C9 polymorphisms; 37 patients (M 26: F 11) had complete data, achieved stable therapeutic INR, and were included in dose variability analysis. There were predominance of white race 73% and male sex 70.3%. The mean age was 9.6 years (1.8-18.6 years). The mean weight was 37.8 kg (7.7-95 kg). Fontan physiology and mechanical cardiac valves were two most common indications for chronic warfarin therapy (25/37 or 67.6%). Twelve patients (32.4%) had ≥ 2 indications for warfarin therapy. Three patients had documented venous or arterial clots, and 5 patients had strokes. Congenital heart disease was present in 29 patients (78.4%), including Fontan physiology (20), complex biventricular physiology (4), and congenital mitral valve disease (5). Acquired heart disease was present in 8 patients (21.6%), including Kawasaki disease with coronary aneurysms (3), acquired mitral valve disease (3), and Marfan syndrome (2). Stable warfarin dose (mg/kg/day) was strongly associated with VKORC1 polymorphism (p < 0.0001) and goal therapeutic INR (p = 0.009). Negative correlations were observed between stable warfarin dose and age, weight, height, and BSA (p = 0.04, 0.02, 0.02, and 0.02 respectively). Factors which did not influence warfarin dose included CYP2C9 polymorphism (p = 0.17), concurrent medications (p = 0.85), sex (p = 0.4), race (p = 0.14), congenital heart disease (p = 0.09), and Fontan physiology (p = 0.76). The gene-dose effect was observed in children with homozygous wild type VKORC1 CC, who required higher warfarin dose compared to those carrying heterozygous TC or homozygous TT (p = 0.028 and 0.0004 respectively). The full multiple linear regression model revealed that VKORC1 genotypes accounted for 47% of dosing variability; CYPC29 accounted for 5%. Overall, the combination of VKORC1, CYP2C9, age, and target INR accounted for 82% of dosing variability. In children with heart disease, VKORC1 genotypes, age, and target INR are important determinants influencing warfarin dosing in children with heart disease. Future warfarin dosing algorithm in children should factor both genetic and clinical factors.

Bilateral coronary artery fistulas and left ventricle noncompaction in a neonate: diagnosis and management.

This report describes a neonate with heart failure resulting from bilateral coronary artery fistulas and left ventricle noncompaction. The patient underwent successful surgical ligation of the fistulas.