Paediatric Cardiac Services Research Articles

Offline: Lessons in whistleblowing

On Friday, March 29, 2013, Bruce Keogh, Medical Director of the NHS, said this: “There had been rumblings in the cardiac surgical community for some time that all was not well at Leeds and on Tuesday I had two phone calls which I found disturbing, both from highly respected, temperate surgeons who commenced the conversations by saying that they had to speak out...then I had a call from an extremely agitated senior cardiologist who had a preliminary cut of some mortality data...which showed that...there was clear blue water between the Leeds mortality rate and other units, so as medical director I couldn't do nothing.” The whistleblowing from paediatric cardiac surgeons in Newcastle and Birmingham contributed substantially last year to the suspension of surgery for children with congenital heart disease at Leeds. Families faced the prospect of their children being re-routed to other hospitals in England. These events took place on a background of efforts to “reconfigure” paediatric congenital cardiac services—to reduce the number of centres nationally from 11 to 7. In the North of England, 3 centres (Liverpool, Newcastle, and Leeds) had to be cut to 2. In 2012, a decision was taken to shut down Leeds. A campaign ensued to reverse this decision on the grounds that the consultation was unfair. The Royal Courts of Justice agreed the process was flawed. But with Keogh's intervention, the Leeds unit was forced, finally, to close.

PS-291 Heterotaxia And Intestinal Rotational Status: Is It Safe To Watch And Wait?

Background The association of heterotaxia with intestinal rotation anomalies is well documented. Controversy still persists though in regards to the optimal diagnostic strategy, as well as to the benefit of surgical correction of identified rotational abnormalities in patients with no gastrointestinal symptoms, especially given the frequent associated cardiac anomalies. The aim of this study was to test the validity of the watchful wait approach in a series of patients with heterotaxia. Method A retrospective analysis of the case notes and electronic records of patients diagnosed with heterotaxia syndromes was performed at a single tertiary paediatric referral centre that offers paediatric cardiac surgery services. All patients with situs anomalies, born within the 1st January 1998 to 31st December 2013, were included. Results 59 patients were identified, of which 13 underwent contrast studies to identify rotational anomalies. Only 2 studies were performed in symptomatic patients (vomiting, failure to thrive). 10 of the studies identified abnormal anatomy, but only 4 patients proceeded to surgical correction. No complications were reported following the Ladd’s procedures. There was a single death within 30 days postoperatively, attributed to cardiac failure. Regarding the patients that had no surgery, during the follow-up period (median 9.2 years, range 11- 187 months), no patients presented clinically with malrotation or volvulus. Conclusions This study supports the ‘watch and wait’ approach for children with heterotaxia, provided that close surveillance can be achieved. Diagnosis and treatment should be individualised and tailored to each patient’s clinical characteristics in order to avoid unnecessary morbidity and mortality.

PO-0033 Referrals For Echocardiograms In A District General Hospital – Are We On The Pulse?

<h3>Background and aims</h3> Echocardiograms are a major resource for diagnosing congenital and acquired heart disease. Paediatricians with special expertise in Paediatric Cardiology are now at the forefront of the paediatric cardiac care service. The aim of this audit was to report on the quality of referrals – investigating the indications for referral, documentation of symptoms and signs. <h3>Methods</h3> The medical notes of 46 children who attended outpatient echocardiogram clinic were retrospectively reviewed (April to June 2012). We looked at indications for referral, documentation of symptoms and signs in the initial referral letters. <h3>Results</h3> 41 out of 46 children attended the clinics, of which 39 sets of notes were available to be reviewed. 22 were new and 17 were follow-ups. The main indications for referral were presence of murmur, family history, congenital heart disease, syndromes and Kawasaki Disease. 16 children were referred because of murmur, 12 of whom were asymptomatic. Half did not comment on grade, position or radiation of the murmur. One-third did not have documentation of femoral pulses, signs of heart failure (crackles on auscultation, hepatomegaly, oedema). <h3>Conclusions</h3> Indications for referral to echocardiogram clinic were appropriate. However, the quality of documentation of basic examination findings was poor. This audit starts a discussion regarding setting the standard for quality of referrals to echocardiogram clinic, and highlights deficiencies in documentation. Recommendations include a standardised pro forma and an emphasis during general practice study days.

Successful training of self-sufficient interventional paediatric cardiology team in a sub-Saharan setting: a multicentre collaborative model.

Most children in the Third World do not have access to treatment for heart diseases, as the priorities of health care are different from the developed countries. Since 2009, teams supported by the Chain of Hope and Spanish medical volunteers have travelled twice a year to help develop paediatric cardiac services in the Cardiac Center in Ethiopia, undertaking four missions each year. As of December 2012, 296 procedures were performed on 287 patients. The procedures included 128 duct occlusions, 55 pulmonary valve dilations, 25 atrial septal defect closures, 14 mitral valve dilations, and others. The local staff were trained to perform a majority of these cases. Procedural success was achieved in 264 (89.2%). There were three deaths, five device embolisations, and three complications in mitral valve dilation. During the visits, the local staff were trained including one cardiologist, six nurses, and two technicians. The local team performed percutaneous interventions on its own after a couple of years. The goal is also to enable the local team to perform interventions independently. Training of an interventional cardiology team in a sub-Saharan setting is challenging but achievable. It may be difficult for a single centre to commit to sending frequent missions to a developing country to make a meaningful contribution to the training of local teams. In our case, coordination between the teams from the two countries helped to achieve our goals.

Fetal Echocardiography Assists in Determining Optimal Delivery Site

ObjectiveCongenital heart disease is one of the most common types of structural fetal abnormalities and a major cause of perinatal morbidity and mortality. Fetal echocardiography aids in the diagnosis of congenital heart disease, which allows management planning for parents and physicians, including continuation or termination of the pregnancy and triaging for location of delivery. This is a key component of planning, as transport of neonates entails risks, costs, and parental stress. In this study, we examined the outcomes of pregnancies with fetal cardiac anomalies diagnosed at a single tertiary care centre. We aimed to assess whether the system of directing affected pregnancies to either a tertiary and quaternary care centre is effective. MethodsWe identified pregnancies with fetal cardiac anomalies diagnosed on fetal echocardiography between 2005 and 2009. Information about diagnosis, pregnancy outcome, delivery location, and surgical management was collected. This information was analyzed retrospectively. ResultsAnomalies were demonstrated in 120 fetal echocardiography studies. Four of the babies (3.3%) were stillborn, and 27 (22.5%) pregnancies were terminated. There were 89 live born babies, and 74 of these (61.7%) survived the neonatal period. Fifteen babies (12.5%) died as neonates. Thirty-two pregnant women were triaged to deliver at the quaternary centre with pediatric cardiac surgery services, and 20 of these babies underwent surgery. Two of the 89 live born babies (2.2%) required emergency transfer. ConclusionFetal echocardiography is an important contributor to efficient use of pediatric cardiac services and minimizes need for neonatal transfer. Contemporary use of fetal echocardiography is associated with optimized delivery location.

Risk of sudden cardiac death following acute coronary syndromes: Interplay of single nucleotide polymorphisms and the presence of heart failure

N. Earle , K. Poppe , V. Cameron , A. Pilbrow , R. Troughton , J. Skinner , D. Love , A. Shelling , M. Richards , R. Doughty 1 Department of Medicine, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand Christchurch Heart Institute, Department of Medicine, University of Otago, Christchurch, New Zealand Department of Obstetrics and Gynaecology, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand Greenlane Paediatric and Congenital Cardiac Services, Starship Childrens Hospital, Auckland, New Zealand Diagnostic Genetics, LabPlus, Auckland City Hospital, Auckland, New Zealand Corresponding author.

The left and right atria finally express themselves

Jonathan R. Skinner, MBChB, MD, FRACP, Donald R. Love, PhD From the Cardiac Inherited Disease Group, Auckland City Hospital, Auckland, New Zealand, Green Lane Paediatric and Congenital Cardiac Services, Starship Children’s Hospital, Auckland, New Zealand, Diagnostic Genetics, LabPlus, Auckland City Hospital, Auckland, New Zealand, and School of Biological Sciences, University of Auckland, Auckland, New Zealand.

Pediatric cardiac services in Iraq: current status and future plans

Methods The database of the International Children’s Heart Foundation was searched for all patients receiving surgery in Iraq. The sites of assistance were Sulimaniyah, Nasiriyah, Najaf and Basra in the order in which assistance was started. Two week mission trips were performed in all sites, but a program of near continuous presence was instituted in Nasiriyah in July 2012. Total and specific institutional analysis was performed. The RACHS-1 model of risk classification was employed for analysis. Results Total mission trips were 14 (Nasiriyah 6, Najaf 5, Sulimaniyah 2, Basra 1) and beginning in August 2012 until May 31, 2012 we have spent 33 weeks in-country. The total number of patients undergoing primary operations was 425. The distribution of cases and mortality by RACHS-1 was; 1- 51/425 (14%) and 1.9% (1/51); 2- 179/425 (48%) and 3.4% (6/179); 3- 122/425 (33%) and 16% (20/122); 4- 17/425 (6%) and 59% (10/17); there was only one RACHS 6 operation. There were 49/425 adults and 6/425 non-classifiable pediatric cases. Mortality and total case volume improved over time p < 0.05 mortality and p < 0.01 for case volume. Neonates comprised 4% of the total cases with mortality of 18.7%. Conclusions Pediatric cardiac surgery in Iraq has increased over time with our assistance. Neonatal cardiac surgery is now offered for the first time in Iraq.

Call for multinational studies of the epidemiology of congenital heart disease in the Arab world

Congenital Heart Disease (CHD) is the commonest congenital anomaly worldwide representing nearly one third of all congenital malformations presenting in the neonatal period. Arabs have a high fertility rates and they are liable to have more children with congenital defects including CHD. High rates of consanguineous marriages may increase rates of congenital and genetic anomalies. No reliable statistics exist and assumption that the incidence of congenital defects in the Arab world being greater than the rest of the world is only anecdotal. It is therefore crucial and timely to have a multinational and multicenter study of the incidence of CHD in the Arab world. Creation of cross the Arab world multinational congenital cardiac defects registry program and stratification is essential prerequisite to develop good pediatric cardiac services. Over the last two decades, some Arab countries have already started their pediatric cardiac programs. The author call upon other countries to follow suit and learn from their experiences.

Neonatal Ultrasound in Transport

Current practices for monitoring the haemodynamics of critically ill newborns whilst in transport to tertiary care are poorly validated. These include arterial blood pressure monitoring, capillary return and urine output. Clinician performed ultrasound (CPU) has been validated in the NICU and is currently being trialled in transport. This case based discussion describes the retrieval of four newborn infants utilising CPU assessment of the newborn systemic blood flow and investigation of intracranial blood flow and pathology. Case one describes a baby with subgaleal haemorrhage who was pre arrest where the CPU (cardiac) allowed the treating team to effectively change the inotropes and alter the clinical course. Case two is a baby with the classical diagnostic dilemma of persistent pulmonary hypertension of the newborn (PPHN) versus cyanotic congenital heart disease (CCHD) – the screening CPU (cardiac) was able to define CCHD and the transport was re-directed to tertiary paediatric cardiac services. The CPU (cardiac) in case three, a baby born at 25 weeks, also assisted in the choice of therapeutic options for the treating team. Both case three and four were born at 25 week gestation and the CPU (cranial) provided information prior to transport that aided in the counselling of the parents.

Challenges and opportunities in paediatric cardiac services: Time for action

The 6th World Congress of Paediatric Cardiology and Cardiac Surgery recently held in Cape Town attracted over 3 000 registered delegates, the vast majority of which were scientific attendees. This congress, hosted by the South African Heart Association and organised by the Paediatric Cardiac Society of South Africa, highlighted the needs of children and adults with highly complex congenital cardiac lesions as well as those with acquired preventable disease such as rheumatic heart disease and noncommunicable cardiovascular diseases with their origins in childhood. The scientific programme addressed the most recent advances in paediatric cardiac disease and reflected new developments in treatment, diagnosis and management while constantly reminding delegates of inadequacies, inequalities and health system concerns affecting patient care.

Children's congenital cardiac services

Journal of Paramedic PracticeVol. 4, No. 3 EditorialChildren's congenital cardiac servicesKerry Gaskin (Cook)Kerry Gaskin (Cook)Search for more papers by this authorKerry Gaskin (Cook)Published Online:16 Aug 2013https://doi.org/10.12968/jpar.2012.4.3.129AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Department of Health (2003) Report of the Paediatric and Congenital Cardiac Services Review. DH, London Google ScholarKennedy I (2001) The Bristol Royal Infirmary Inquiry. DH, London Google ScholarNSCG (2009) Safe and Sustainable Paediatric Cardiac Surgery Services. NSCG, London. Google ScholarNSCG (2010) Safe and Sustainable Children's Heart Surgery. The Need for Change. NSCG, London. Google ScholarNSCG (2011) Safe and Sustainable: Review of Children's Congenital Heart Surgery Services in England. Health Impact Assessment: Scoping Report. NSCG, London Google Scholar FiguresReferencesRelatedDetails 2 March 2012Volume 4Issue 3ISSN (print): 1759-1376ISSN (online): 2041-9457 Metrics History Published online 16 August 2013 Published in print 2 March 2012 Information© MA Healthcare LimitedPDF download

Influence of Procedural Risk on Cardiac Catheterisation Complications

Introduction: The Congenital Heart Disease Adjustment for Risk Method (CHARM) was developed to adjust risk of cardiac catheterisation with case mix complexity. We have investigated this methodology in a pilot study to assess outcomes at Queensland Paediatric Cardiac Service (QPCS). Methods: From January 2001 to December 2011 demographic, procedural and complication data were prospectively collected on 2367 patients undergoing cardiac catheterisation. Procedural risk and associated complication severity was assigned a classification, consistent with CHARM, retrospectively from this dataset. Results: Patient median age was 2.9 years (range 1 day–20.6 years) and weight 13.6 kg (range 1.5–103 kg). The interventional catheterisation rate was 34.9% for the 10-year study period, although this rate has been 41.4% for 2008–2011. Lower risk procedures predominated with 87.6% category 1–2 and 12.4% category 3–4 procedures. The overall complication rate was 6.5% and these patients had a median weight of 6.2 kg (range 1.9–71 kg). Higher complication rates were associated with high-risk procedures – complication rate of 5.1% for category 1–2 and 16.6% for category 3–4 procedures. 76.1% of complications were minor category 1–3 incidents (mainly minor peripheral vascular). Increased procedural risk was associated with a higher incidence of categories 3 and 4 complications. Category 5 complications occurred in 0.1% of patients all of whom were in a lower procedural risk group. Conclusions: Cardiac catheter complication rates at QPCS are consistent with published international literature. This study demonstrates that risk adjustment for case complexity may be a valuable tool for assessing catheter laboratory performance in the Australasian environment.

The antenatal detection rate of major congenital heart disease in Scotland

Ultrasonography has been used to detect fetal congenital heart disease since the 1980s, although there has been little uniformity in policy. In the UK, data published for 1993-1995 found an average detection rate of 23.4% with a Scottish rate of 9.6%. At the time of this study, 10 out of the 14 health boards in Scotland were implementing the 16-22-week fetal anomaly scan (FAS). The aims of this study were to determine the rate of antenatal diagnosis of major congenital heart disease (MCHD) throughout Scotland during 1 January to 31 December 2006, to document the regional differences in detection and to determine if implementation of a FAS significantly improved rates. Retrospective data were collected from the centralized Paediatric Cardiac Services in Edinburgh and Glasgow. The database contains information on all infants with cardiac disease in Scotland. All infants requiring cardiac intervention or dying in the first year of life were included. The results showed that the antenatal diagnosis rate for Scotland was 28% (95% CI 21.2%, 34.9%). The Scottish health boards that had implemented the FAS had a significantly higher antenatal detection rate (χ(2) test P = 0.037). In conclusion, antenatal detection rate of MCHD varies widely throughout Scotland. Implementation of the FAS has made a significant difference to antenatal diagnosis.

Sustainable Knowledge Transfer in Pediatric Cardiac Surgery

The study assesses the impact of a program using a model of knowledge transfer on the long-term development of a pediatric cardiac service in Lithuania. A team from the United Kingdom evaluated Lithuanian pediatric cardiac services and provided support in areas targeted for improvement. The total number of infant operations performed in Lithuania from 1990 to 2008, together with in-hospital mortality rates, was broken down into 3 time periods and analyzed to estimate the efficacy of the program: (1) 1990-1998, before the program; (2) 1999-2002, during the program; (3) 2003-2008, following the end of the program. Lithuanian results in children older than 1 year were comparable with other European centers. However, only 315 infant and neonatal cardiac procedures were performed between 1990 and 1998, and there was an in-hospital mortality of 34.2%. Between 1999 and 2002, the UK team performed 23 highly complex demonstration procedures (in-hospital mortality, 13%). During the same period the Lithuanian team performed 305 additional operations in neonates and infants, and in-hospital mortality decreased to 18.7%. From 2003 to 2008 results continued to improve-559 infant operations were performed, with in-hospital mortality of 11.3% (P < .0001). Knowledge transfer has led to substantial and sustainable long-term improvement in the results of infant cardiac surgery in Lithuania. Demonstrating techniques and care on a limited number of more complex cases is an efficient way of transferring knowledge and skills to the developing pediatric cardiac centers.

Creating a new cardiac service: the Brisbane experience

Objective: This article discusses the creation of a new paediatric cardiac surgical service via the merging of two units. Setting: In 2005, the Queensland Government made a commitment to improve health outcomes for Queensland’s children. This pledge included a review of paediatric cardiology and paediatric cardiac surgery. A recent cluster of deaths following cardiac surgery at The Prince Charles Hospital (TPCH) and concerns expressed by clinicians regarding the adequacy of the service, resulted in the review being commissioned. A panel of well respected, eminent professionals were appointed with the brief to review the adequacy of paediatric cardiac services in Queensland, to determine the optimal configuration of the cardiac service and to also make recommendations to ensure a high quality of service in the future. Conclusion: The process of amalgamating the two units and creating a new cardiac service has increased output in terms of surgical cases. The care of cardiac children in Queensland is now undertaken from a major specialist children’s hospital. However, the costs associated with the amalgamation have been high, particularly in terms of training staff and development of infrastructure. The dynamics of change whilst prioritised by management resulted in a highly stressful working environment and a number of staff resigned. Eighteen months post merge, the morale within the unit has improved and staff are working positively towards a cohesive unit.

Paediatric cardiac services in South Africa

South Africa has failed to treat its children well, as demonstrated by our failure to achieve adequate progress towards the Millennium Development Goal of reducing the under-5 mortality rate, doing far worse than many poorer countries.

Safe and sustainable paediatric cardiac surgical services

Kerry Cook explains how the programme to restructure children’s heart surgery services offers an opportunity for children and young people’s cardiac nursing services to change

The Long Road to Better ACHD Care

The care of adult patients with congenital heart defects in the United States is spotty at best, and needs to improve greatly if the needs of these patients are to be met. The care of American children with congenital heart defects is generally excellent. Pediatric cardiac services are well established and well supported. The care of adults with congenital heart disease (CHD) is well established in only a few American centers. While there are an increasing number of clinics, they are generally poorly resourced with relatively few patients. If located in adult cardiology programs, they are usually minor players. If located in pediatric cardiac programs, they are usually minor players as well. Training programs for adult CHD (ACHD) caregivers are few, informal, and poorly funded. To improve the situation, we need perhaps 25 well-resourced and well-established regional ACHD centers in the United States. We need to stop the loss to care of CHD patients at risk of poor outcomes. We need to educate patients and families about the need for lifelong and skilled surveillance and care. We need to effect an orderly transfer from pediatric to adult care. We need to strengthen the human resource infrastructure of ACHD care through the training and hiring of healthcare professionals of a quality equivalent to those working in the pediatric care environment. We need to demonstrate that adult care is high quality care. We need more high-quality ACHD research. The ACHD community needs to establish its credibility with pediatric cardiac providers, adult cardiology groups, with governments, with professional organizations, and with research funding agencies. Accordingly, there is a need for strong political action on behalf of American ACHD patients. This must be led by patients and families. These efforts should be supported by pediatric cardiologists and children's hospitals, as well as by national professional organizations, governments, and health insurance companies. The goal of this political action should be to see that ACHD patients can receive high-quality lifelong surveillance, that we lose fewer patients to care, and that the staff and other services needed are available nationwide.

Pediatric Cardiac Surgery In Eritrea: A retrospective analysis of 300 cardiac patients operated, 2002-2008.

A review of pediatric cardiac operations performed in the International Operation Center for Children in Asmara (IOCCA) between 2002 and 2008 was made. A total of 300 patients underwent corrective cardiac procedures during the time specified. Out of these, 273(91%) were patients suffering from congenital heart defects while the rest 27(9%) had acquired heart diseases. The female to male ratio was 1.4:1, and 157(52%) patients were under five years of age. 188(63%) of the procedures were open heart operations using the heart lung-machine and 112(37%) were closed heart operations without the cardiopulmonary bypass. The commonest operations performed were for PDA 103(34%), while the commonest open heart surgery was for VSD 74(25%). The mortality rate for patients operated was 7(2.3%) and the re-operation rate was 5(1.7%). The presence of an organized pediatric cardiac service in the country and its role in addressing different cardiac defects surgically and minimizing the cost of the procedures done elsewhere is emphasized.