Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research. The continued leveraging of technology, commitment to data transparency, focus on team-based practice, and recognition of cultural norms and preferences ensure the success of sustainable models of global collaboration.

As pediatric cardiac critical care becomes more sub-specialized it is reasonable to assume that dedicated units may provide a better infrastructure for improved multidisciplinary care, cardiac-specific patient safety initiatives, and dedicated training of fellows and residents. The knowledge base required to optimally manage pediatric patients with critical cardiac disease has evolved sufficiently to consider a standardized training curriculum and board certification for pediatric cardiac critical care. This strategy would potentially provide consistency of training and healthcare and improve quality of care and patient safety.

Despite many advances in recent years for patients with critical paediatric and congenital cardiac disease, significant variation in outcomes remains across hospitals. Collaborative quality improvement has enhanced the quality and value of health care across specialties, partly by determining the reasons for variation and targeting strategies to reduce it. Developing an infrastructure for collaborative quality improvement in paediatric cardiac critical care holds promise for developing benchmarks of quality, to reduce preventable mortality and morbidity, optimise the long-term health of patients with critical congenital cardiovascular disease, and reduce unnecessary resource utilisation in the cardiac intensive care unit environment. The Pediatric Cardiac Critical Care Consortium (PC4) has been modelled after successful collaborative quality improvement initiatives, and is positioned to provide the data platform necessary to realise these objectives. We describe the development of PC4 including the philosophical, organisational, and infrastructural components that will facilitate collaborative quality improvement in paediatric cardiac critical care.

The management of newborns with hypoplastic left heart syndrome has changed significantly over the past three decades, with an associated dramatic improvement in outcomes. The aim of this paper is to discuss current peri-operative and palliative surgical strategies. Owing to the fact that comparative outcomes for these strategies have been addressed in a limited number of prospective trials and extractions from multi-centred databases, the primary focus of this review is descriptive.

Variability in practice can be considered to foster clinical innovation, and allow for individualized therapeutic plans and independence of practitioners. The Institute of Medicine, however, has issued a report suggesting that variability in patterns of practice are “illogical”, and should be avoided whenever possible. Perhaps nowhere in the field of congenital cardiac disease is variability in practice more apparent than in the management of hypoplastic left heart syndrome. This review assesses the variability in practice at a large number of centres that manage neonates with hypoplastic left heart syndrome, with an emphasis on practice before, during, and after the first stage of the Norwood sequence of operations. We also suggest changes in future strategies for research.

In March, 2007, colleagues were contacted to respond to an internet-based survey using commercially available software (www.surveymonkey.com) to collect responses about the management practices for neonates with “straight-forward” hypoplastic left heart syndrome. No attempt was made to correlate management practices with any measures of outcome, as neither the practices themselves, nor the outcomes of interest, could be externally validated. Data is reported from 52 centers thought to manage over 1000 neonates with hypoplastic left heart syndrome on an annual basis. The first stage of the Norwood sequence was “recommended” to families by approximately five-sixths (86.5%) of the centres. No centre recommended primary cardiac transplantation, a “hybrid” approach, or non-intervention. In 7 centres (14.5%), it was reported that there was discussion of some or all of the above options, but ultimately the families decided upon the appropriate strategy.

Most centres preferentially used antegrade cerebral perfusion (54%) in contrast to deep hypothermia with circulatory arrest (24%), albeit that 11% of centres used a combination of these techniques and in 9% the support strategy was based on surgeon preference. The source of flow of blood for the lungs following the first stage of reconstruction was also highly variable. Of the 51 centres that responded to the question, 13 (25.5%) were participating in a multi-centric randomized clinical trial comparing the modified Blalock-Taussig shunt to the conduit placed from the right ventricle to the pulmonary arteries, the so-called “Sano” modification. Of the remaining 38 centres, 18 “usually” placed a conduit from the right ventricle to the pulmonary artery, 14 “usually” placed a modified Blalock-Taussig shunt, and at six centres, the decision was made “based upon the preference of the surgeon and/or the cardiologist”. Similarly, significant variability in practice was evident in preoperative management, other surgical strategies, postoperative medical support, monitoring and discharge planning. Other than the randomized clinical trial of shunt type, no other medical or surgical management strategy was currently under investigation in a multi-centric or randomized trial in the centres who responded to the survey.

The survey emphasises the extreme variability in our current practices for treatment of children with hypoplastic left heart syndrome. While there are some areas for which there is consensus in management, the majority of our practices are variable between and within centres. These results emphasize that large multicentric trials and registries are necessary to improve care, and to answer important clinical questions, emphasizing the need to shift from analysis of experiences of single centres to multi-centric and multi-disciplinary collaboration.

Pulseless cardiac arrest, defined as the cessation of cardiac mechanical activity, determined by unresponsiveness, apneoa, and the absence of a palpable central pulse, accounts for around one-twentieth of admissions to paediatric intensive care units, be they medical or exclusively cardiac. Such cardiac arrest is higher in children admitted to a cardiac as opposed to a paediatric intensive care unit, but the outcome of these patients is better, with just over two-fifths surviving when treated in the cardiac intensive care unit, versus between one-sixth and one-quarter of those admitted to paediatric intensive care units. Children who receive chest compressions for bradycardia with pulses have a significantly higher rate of survival to discharge, at 60%, than do those presenting with pulseless cardiac arrest, with only 27% surviving to discharge. This suggests that early resuscitation before the patient becomes pulseless, along with early recognition and intervention, are likely to improve outcomes. Recently published reports of in-hospital cardiac arrests in children can be derived from the multi-centric National Registry of Cardiopulmonary Resuscitation provided by the American Heart Association. The population is heterogeneous, but most arrests occurred in children with progressive respiratory insufficiency, and/or progressive circulatory shock. During the past 4 years at the Children’s Hospital of Philadelphia, 3.1% of the average 1000 annual admissions to the cardiac intensive care unit have received cardiopulmonary resuscitation. Overall survival of those receiving cardiopulmonary resuscitation was 46%. Survival was better for those receiving cardiopulmonary resuscitation after cardiac surgery, at 53%, compared with survival of 33% for pre-operative or non-surgical patients undergoing resuscitation. Clearly there is room for improvement in outcomes from cardiac resuscitation in children with cardiac disease. In this review, therefore, we summarize the newest developments in paediatric resuscitation, with an expanded focus upon the unique challenges and importance of anticipatory care in infants and children with cardiac disease.

The medical records of all patients born between 1 September, 2000, and 31 August, 2002, and undergoing the first stage of Norwood reconstruction, were retrospectively reviewed for details of the perioperative course. We found 99 consecutive patients who met the criterions for inclusion. Hospital mortality for the entire cohort was 15.2%, but was 7.3%, with 4 of 55 dying, in the setting of a “standard” risk profile, as opposed to 25.0% for those with a “high” risk profile, 11 of 44 patients dying in this group. Extracorporeal membrane oxygenation was utilized in 7 patients, with 6 deaths. Median postoperative length of stay in the hospital was 14 days, with a range from 2 to 85 days, and stay in the cardiac intensive care unit was 11 days, with a range from 2 to 85 days. Delayed sternal closure was performed in 18.2%, with a median of 1 day until closure, with a range from zero to 5 days. Excluding isolated delayed sternal closure, and cannulation and decannulation for extracorporeal support, 24 patients underwent 33 cardiothoracic reoperations, including exploration for bleeding in 12, diaphragmatic plication in 4; shunt revision in 4, and other procedures in 13. The median duration of total mechanical ventilation was 4.0 days, with a range from 0.7 to 80.5 days. Excluding those who died, the median total duration of mechanical ventilation was 3.8 days, with a range from 0.9 to 46.3 days. Reintubation for cardiorespiratory failure or upper airway obstruction was performed in 31 patients. Postoperative electroencephalographic and/or clinical seizures occurred in 13 patients, with 7 discharged on anti-convulsant medications. Postoperative renal failure, defined as a level of creatinine greater than 1.5 mg/dl, was present in 13 patients. Eleven had significant thrombocytopenia, with fewer than 20,000 platelets per μl, and injury to the vocal cords was identified in eight patients. Risk factors for longer length of stay included lower Apgar scores, preoperative intubation, early reoperations, reintubation and sepsis, but not weight at birth, genetic syndromes, the specific surgeon, or the duration of surgery.

Although mortality rates after the first stage of reconstruction continue to fall, the course in the intensive care unit is remarkable for significant morbidity, especially involving the cardiac, pulmonary and central nervous systems. These patients utilize significant resources during the first hospitalization. Further studies are necessary to stratify the risks faced by patients with hypoplasia of the left heart in whom the first stage of Norwood reconstruction is planned, to determine methods to reduce perioperative morbidity, and to determine the long-term implications of short-term complications, such as diaphragmatic paresis, injury to the vocal cords, prolonged mechanical ventilation, and postoperative seizures.

In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.

Background: Modified ultrafiltration has been shown to reverse haemodilution and improve ventricular function following cardiopulmonary bypass. There has been concern, however, about the safety and efficacy of modified ultrafiltration after the first stage of Norwood reconstruction for palliation of neonates with hypoplasia of the left heart and its variants. Methods: We reviewed the intraoperative course of all patients undergoing the first stage of Norwood reconstruction between September 1, 2000, and August 31, 2002. Results: The first stage of reconstruction was performed in 99 neonates, 78 with classical hypoplasia of the left heart, and 21 with variants. Mean weight at surgery was 3.1 plus or minus 0.7 kilograms. Genetic syndromes, weight less than or equal to 2.5 kilograms, and/or major additional cardiac or non-cardiac anomalies, were present in 44 patients. We deemed these patients to constitute the group at high risk. A modified Blalock–Taussig shunt was utilized in 95 patients, and a conduit from the right ventricle to the pulmonary arteries in 4. Deep hypothermic circulatory arrest was used in all patients for a mean period of 45 minutes, plus or minus 15 minutes. Total support time on cardiopulmonary bypass plus deep hypothermic circulatory arrest was 100 minutes plus or minus 26 minutes. Modified ultrafiltration was performed in all patients. The mean duration of modified ultrafiltration was 10 plus or minus 2 minutes, and the total volume of filtrate removed was 104 plus or minus 29 millilitres per kilogram. There were no complications from modified ultrafiltration, and no patient required discontinuation of modified ultrafiltration for haemodynamic instability. During modified ultrafiltration, the haematocrit increased from 31 percent plus or minus 4 to 46 percent plus or minus 6. Heart rate decreased from 170 plus or minus 17 beats per minute to 158 plus or minus 16 beats per minute. Systolic blood pressure increased from 57 plus or minus 12 to 63 plus or minus 13 millimetres of mercury, and diastolic blood pressure from 30 plus or minus 8 to 35 plus or minus 7 millimetres of mercury. All these values are significant at a p value of less than 0.0001. Hospital morality in the patients at low risk was 3 of 55 (5.5 percent), but was 12 of 44 (27.3 percent) in the patients deemed to be at high-risk. Conclusions: Modified ultrafiltration is safe procedure following the first stage of Norwood reconstruction, with improvement in all haemodynamic parameters measured. Modified ultrafiltration is an additional incremental strategy, which may contribute to the overall improvement in outcome following surgical palliation of patients with hypoplasia of the left heart or its variants.