Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Central aortic blood pressure estimation in children and adolescents: results of the KidCoreBP study.

BACKGROUND: Central aortic SBP (cSBP) may have superior prognostic value compared with peripheral SBP (pSBP), but noninvasive cSBP measurement techniques have not been formally validated in children and adolescents. METHOD: This study assessed the accuracy of two automated devices and the radial tonometry/transfer function method (RT-TF) for estimating central pressures and pulse pressure amplification (PPA) in this population, with adherence to validation guidelines for central pressure devices. In 69 children/adolescents aged 3-18 years undergoing clinically indicated aortic catheterization, high fidelity ascending aortic cSBP was measured with a micromanometer-tipped wire and compared with values from SphygmoCor XCEL, Mobil-O-Graph (systolic/diastolic calibration, MoG-C1, or mean/diastolic calibration, MoG-C2) and RT-TF. Reference intra-arterial pSBP was derived from the tonometry pulse calibrated to central mean/diastolic pressures. RESULTS: XCEL, MoG-C1 and MoG-C2 overestimated cSBP by 7.9 ±â€Š6.8 mmHg (mean ±â€ŠSD), 5.7 ±â€Š10.3 mmHg, and 19.1 ±â€Š14.9 mmHg, exceeding the validation cut-off (5 ±â€Š8 mmHg). Brachial pSBP was also overestimated by XCEL (10.9 ±â€Š8.4 mmHg) and Mobil-O-Graph (11.5 ±â€Š12.3 mmHg). By contrast, central and brachial diastolic pressures were underestimated by the automated devices, albeit mostly within acceptable limits; pulse pressures were, therefore, substantially overestimated. Central-brachial PPA (4.5 ±â€Š4.4 mmHg) was overestimated by XCEL (8.7 ±â€Š3.2 mmHg) and MoG-C1 (11.1 ±â€Š6.4 mmHg), but underestimated by MoG-C2 (-3.0 ±â€Š6.6 mmHg). Given accurate pulse calibration, RT-TF achieved acceptable accuracy for cSBP (-0.2 ±â€Š4.6 mmHg) and central-radial PPA (1.9 ±â€Š5.1 mmHg). CONCLUSION: In conclusion, XCEL and Mobil-O-Graph overestimated pSBP and cSBP in children and adolescents. cSBP can be obtained via the same transfer function used in adults, but accurate pressure pulse calibration is critical.Video Abstracts: http://links.lww.com/HJH/B222.

Biventricular repair versus Fontan completion for patients with d- or l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients. METHODS: We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015. RESULTS: Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups. CONCLUSIONS: Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair.

Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. RESULTS: Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). CONCLUSIONS: A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.

Atrioventricular block after ASD closure.

OBJECTIVE: Secundum atrial septal defect (ASD) is a common congenital heart defect. There is limited data on both early and late atrioventricular (AV) block post ASD closure. The aim of this study was to determine the incidence and risk factors of AV block associated with ASD closure. METHODS: A retrospective analysis of all patients who underwent ASD closure either with a device or surgical method at the Royal Children's Hospital Melbourne between 1996 and 2010 was performed. Baseline demographics, procedural details and follow-up data were collected from medical records. RESULTS: A total of 378 patients were identified; 242 in the device group and 136 in the surgical group. Fourteen patients (3.7%) had AV block (1 with second degree and 13 with first degree) at a median follow-up of 28 months; 11/242 (4.5%) in the device group and 3/135 (2.2%) in the surgical group (p=0.39). Six patients had new-onset AV block after ASD closure. In the device subgroup, patients with AV block at follow-up had a larger indexed device size compared with those without (22 (15-31) vs 18(7-38), p=0.02). Multivariate analysis revealed the presence of AV block either pre procedure or post procedure to be the only variables associated with late AV block. CONCLUSIONS: Late AV block in patients with repaired ASD is rare and most likely independent of the technique used. In the device subgroup, the only risk factor identified to be associated with late AV block was the presence of either preprocedural or postprocedural AV block, so long-term follow-up for these patients should be provided.

Percutaneous left atrial decompression in patients supported with extracorporeal membrane oxygenation for cardiac disease.

OBJECTIVES: Left atrial decompression using cardiac catheterization techniques has been described at centers with extracorporeal membrane oxygenation programs. Left atrial decompression can decrease cardiogenic edema, minimize ventricular distension, and allow myocardial recovery. We describe Boston Children's Hospital's experience with percutaneous left atrial decompression techniques, acute outcomes, and clinical impact of left atrial decompression in extracorporeal membrane oxygenation patients. SUBJECTS: Patients supported with extracorporeal membrane oxygenation undergoing percutaneous left atrial decompression were identified and assigned to two groups 1) myocarditis/suspected myocarditis or 2) nonmyocarditis cardiac disease. INTERVENTIONS: Three techniques including vent placement, static balloon dilation, and stent implantation were used. MEASUREMENTS AND MAIN RESULTS: Change in left atrial pressure and severity of pulmonary edema on chest radiography pre and post procedure, impact of timing and technique of left atrial decompression on resolution of left atrial hypertension, and extracorporeal membrane oxygenation survival were evaluated. Furthermore, we evaluated the presence of residual atrial septal defect during follow-up. Percutaneous left atrial decompression was performed in 44 of 419 extracorporeal membrane oxygenation cases (10.5%) and was frequently used for myocarditis (22 of 44 patients; 50%). Techniques included 25 vents, 17 static balloon dilations, and two stents. All techniques were equally successful and significantly reduced left atrial pressure and pulmonary edema. Survival to hospital discharge was not associated with extracorporeal membrane oxygenation duration prior to left atrial decompression, change in left atrial pressure, or technique used. Persistent atrial septal defect was noted in five surviving patients (excluding transplant recipients and deceased), two required closure. CONCLUSIONS: Left atrial decompression can be performed effectively in children on extracorporeal membrane oxygenation using various percutaneous techniques. Reduction in pulmonary venous congestion is usually evident by chest radiography within 48 hours of intervention. Persistent atrial septal defect may require closure at the time of extracorporeal membrane oxygenation decannulation or during long-term follow-up.

Vascular access for pediatric coronary angiography on extracorporeal membrane oxygenation.

Vascular access for catheterization of a pediatric patient on extracorporeal membrane oxygenation (ECMO) was facilitated by adding an accessory limb to the ECMO circuit. This limb was terminated with a hemostatic valve allowing insertion of a catheter. Changes in support parameters compensated for the change in the effective diameter of the ECMO cannula on insertion of the catheter. This method can overcome difficulties in obtaining vascular access for patients on ECMO.

Respiratory syncytial virus immunoprophylaxis in high-risk infants with heart disease.

AIM: Passive immunisation with palivizumab is recommended in many countries for children with haemodynamically significant cardiac disease. We trialled respiratory syncytial virus (RSV) immunoprophylaxis in such infants during 2008­2009. METHODS: We identified all RSV admissions between 2005­2009 and examined all patients with significant cardiac disease who received palivizumab in 2008­2009. RESULTS: Infants with symptomatic cardiac disease had a more complicated course of RSV bronchiolitis with longer hospital stay, more frequent intensive care admission, longer intensive care stay and were more likely to receive respiratory support (all P < 0.05). One hundred seventeen infants with symptomatic cardiac disease received palivizumab. Of these, two (1.7%) required admission for RSV bronchiolitis. Overall, there was a reduction in admission of infants with symptomatic cardiac disease with RSV bronchiolitis in 2008­2009 (2% per year) compared with 2005­2007 (5­9% per year; P < 0.03). The number of patients with symptomatic cardiac disease who required intensive care for RSV bronchiolitis in the same period was unchanged, as a number presented to our service with RSV infection prior to commencing immunoprophylaxis or having had their cardiac diagnosis made in other centres. CONCLUSIONS: Compared with other infants, those with haemodynamically significant cardiac disease have a more complicated course of illness with RSV bronchiolitis. In these infants, palivizumab reduced the number of hospitalisations because of RSV. Cohorting patients for maximal palivizumab use reduced overall cost. To significantly impact on intensive care admissions overall, immunoprophylaxis should be considered at a regional level.

The fellows stitch: large caliber venous hemostasis in pediatric practice.

OBJECTIVE: To analyze safety and efficiency of a subcutaneous figure of eight suture for hemostasis after large caliber venous sheath access in children. BACKGROUND: Vascular complications remain a significant cause of morbidity after pediatric cardiac catheterization. In an attempt to reduce such complications and yet improve lab efficiency and decrease length of stay, various techniques have been applied to improve time to hemostasis. METHODS: Prospectively recorded were vascular complications and hemostasis times in children where hemostasis was attempted using a figure of eight subcutaneous suture following large caliber venous cannulation. These were compared to a matched group achieving hemostasis using standard manual pressure techniques. Vascular ultrasound assessments were performed within 24 hr of hemostasis in both groups. RESULTS: Thirty-two subcutaneous sutures were placed in 26 children, mean weights 31.9 kg [median (range): 29.4 (8.4 to 96) kg], with a mean sheath French size of 9.2 [8; (6 to 22)], 11 >10 French, compared to 33 sheaths in 30 cases using manual compression, mean sheath French size 9.1 (9; (6 to 13), with 10 cases ≥10 French. The mean and median times to hemostasis were shorter in the suture group: 13.6 min (P < 0.05) and 10 min (P < 0.05), respectively. Vascular complication rate was also lower in the suture group (n = 0) compared with the control group (n = 2) but did not achieve statistical significance. CONCLUSIONS: A subcutaneous figure of eight suture hemostasis strategy can provide a safe and efficient method for large caliber venous hemostasis in a pediatric practice with improved hemostasis times and no additional morbidity.

Brugada syndrome caused by a large deletion in SCN5A only detected by multiplex ligation-dependent probe amplification.

A 14-year-old boy presented with atrial flutter. His ECG showed Brugada changes, first-degree AV block and major sinus pauses. Polymorphic VT was inducible at electrophysiology study. A pacemaker defibrillator was placed. Classic sequencing for SCN5A was normal. Multiplex ligation-dependent probe amplification, however, detected a major deletion in SCN5A. It is predicted that this deletion would result in haploinsufficiency. The report is the first description of a large-scale rearrangement of the SCN5A gene and supports the association between the molecular pathology and the phenotypic expression.