Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Infective endocarditis following Melody valve implantation: comparison with a surgical cohort.

BACKGROUND: Infective endocarditis has been reported post Melody percutaneous pulmonary valve implant; the incidence and risk factors, however, remain poorly defined. We identified four cases of endocarditis from our first 25 Melody implants. Our aim was to examine these cases in the context of postulated risk factors and directly compare endocarditis rates with local surgical valves. METHODS: We conducted a retrospective review of patients post Melody percutaneous pulmonary valve implant in New Zealand (October, 2009-May, 2015) and also reviewed the incidence of endocarditis in New Zealand among patients who have undergone surgical pulmonary valve implants. RESULTS: In total, 25 patients underwent Melody implantation at a median age of 18 years. At a median follow-up of 2.9 years, most were well with low valve gradient (median 27 mmHg) and only mild regurgitation. Two patients presented with life-threatening endocarditis and obstructive vegetations at 14 and 26 months post implant, respectively. Two additional patients presented with subacute endocarditis at 5.5 years post implant. From 2009 to May, 2015, 178 surgical pulmonic bioprostheses, largely Hancock valves and homografts, were used at our institution. At a median follow-up of 2.9 years, four patients (2%) had developed endocarditis in this group compared with 4/25 (16%) in the Melody group (p=0.0089). Three surgical valves have been replaced. CONCLUSIONS: The Melody valve offers a good alternative to surgical conduit replacement in selected patients. Many patients have excellent outcomes in the medium term. Endocarditis, however, can occur and if associated with obstruction can be life threatening. The risk for endocarditis in the Melody group was higher in comparison with that in a contemporaneous surgical pulmonary implant cohort.

The New Zealand Norwood Procedure Experience: 22-Year Cumulative Review.

BACKGROUND: Babies born with a functional single ventricle heart and systemic outflow tract obstruction may require a Norwood (NW) procedure as the first of three staged procedures to obtain a Fontan circulation. This procedure and the following treatment pathway are associated with significant mortality. Risk factors for the Norwood procedure and the subsequent pathway are not necessarily the same. To identify these factors within New Zealand, the collective experience with the Norwood procedure was examined. METHODS: Charts from 133 New Zealand children undergoing Norwood procedure from 1992 to 2014 were examined. Deaths were assigned as "early" (30-day or in-hospital) or "late" (based on timeframe from the Norwood procedure). Cardiac anatomic, patient demographic and surgery related variables were examined. Important risk factors were identified using univariate and multivariate analysis. Kaplan-Meier survival curves were calculated. RESULTS: Survival following the Norwood procedure was 83%. Overall survival was 67% at six months, 65% at one year, and 63% at five years. Size of the ascending aorta appeared as an important risk factor with "early" death while New Zealand deprivation index and low birth weight appeared more important for "late" death. These factors each impacted overall survival. CONCLUSIONS: The Norwood procedure and subsequent medical treatment carries significant mortality in New Zealand. Cardiac anatomical factors affect survival of the immediate Norwood procedure whereas social factors weigh more significantly on longer term survival. Nevertheless, both anatomical and social factors carry real survival risk.

Mitral valve geometry in paediatric rheumatic mitral regurgitation.

Rheumatic mitral valve disease is associated with valvulitis and valvular regurgitation; however, the effect of the rheumatic process on the mitral valve geometry and function is not well understood. To assess mitral valve annulus remodelling in rheumatic mitral valve disease, 16 subjects aged 6-15 years with rheumatic mitral valve regurgitation [MR] [6 mild or moderate (Group 1), 10 severe (Group 2)] and 7 age- and body size-matched normal controls with adequate trans-thoracic echocardiograms were recruited. None of the patients had undergone surgical intervention and none had more than mild aortic regurgitation. None of the patients had mitral stenosis. 3D mitral valve geometry was assessed using a Tomtec system. The non-planar angle was increased in all subjects during early (control 147° ± 10, Group 1 168° ± 9, Group 2 166° ± 10; p < 0.05) and late systole (control 149° ± 12, Group 1 162° ± 10, Group 2 164° ± 6; p < 0.05), indicating loss of saddle shape. 2D annular area increased in Group 2 (control 397 ± 48 mm(2)/m(2) vs Group 2 739 ± 207, p < 0.05) with no significant change in annular perimeter indicating leaflet effacement. Mitral valve area correlated with left ventricular size (p < 0.001, r (2) = 0.74). There was no significant change in valve area and perimeter between early and late systole. Remodelling of the mitral valve apparatus in childhood rheumatic heart disease results in abnormal annular geometry. The mitral valve loses its saddle shape regardless of the severity of MR. This may be a consequence of inflammation on the fibrous trigones and surrounding annulus. In contrast, annular area enlargement occurs as a result of leaflet effacement and relates to MR severity and LV size.

New white matter brain injury after infant heart surgery is associated with diagnostic group and the use of circulatory arrest.

BACKGROUND: Abnormalities on magnetic resonance imaging scans are common both before and after surgery for congenital heart disease in early infancy. The aim of this study was to prospectively investigate the nature, timing, and consequences of brain injury on magnetic resonance imaging in a cohort of young infants undergoing surgery for congenital heart disease both with and without cardiopulmonary bypass. METHODS AND RESULTS: A total of 153 infants undergoing surgery for congenital heart disease at <8 weeks of age underwent serial magnetic resonance imaging scans before and after surgery and at 3 months of age, as well as neurodevelopmental assessment at 2 years of age. White matter injury (WMI) was the commonest type of injury both before and after surgery. It occurred in 20% of infants before surgery and was associated with a less mature brain. New WMI after surgery was present in 44% of infants and at similar rates after surgery with or without cardiopulmonary bypass. The most important association was diagnostic group (P<0.001). In infants having arch reconstruction, the use and duration of circulatory arrest were significantly associated with new WMI. New WMI was also associated with the duration of cardiopulmonary bypass, postoperative lactate level, brain maturity, and WMI before surgery. Brain immaturity but not brain injury was associated with impaired neurodevelopment at 2 years of age. CONCLUSIONS: New WMI is common after surgery for congenital heart disease and occurs at the same rate in infants undergoing surgery with and without cardiopulmonary bypass. New WMI is associated with diagnostic group and, in infants undergoing arch surgery, the use of circulatory arrest.

Emergency arterial switch: Rescue therapy for life-threatening hypoxemia in infants with transposition of great arteries with intact intraventricular septum.

OBJECTIVE: A small percentage of infants with d-loop transposition of the great arteries with intact intraventricular septum have life-threatening refractory hypoxemia often due to coexistent persistent pulmonary hypertension of the newborn. In this case series we describe the outcomes of a "rescue" emergency arterial switch operation (ASO). METHODS: We undertook a retrospective medical record analysis of infants with d-loop transposition of the great arteries with intact intraventricular septum who underwent an ASO in New Zealand from January 1, 1996, to April 30, 2017. Data were compared for those who received an emergency ASO and those with a nonemergency ASO for descriptive purposes. An emergency ASO was defined as one that was undertaken for life-threatening refractory hypoxemia when the only alternative stabilization strategy was preoperative extracorporeal life support. Primary outcome measures were 30-day postoperative mortality and abnormal neurodevelopmental outcome in the survivors. Secondary outcomes were low cardiac output, arrhythmia, renal dysfunction, postoperative seizures, and length of stay. Other known risk factors for morbidity and mortality were also assessed. RESULTS: Two hundred seventy-two infants underwent an ASO with 25 (9%) who received an emergency ASO. No infants received preoperative extracorporeal life support. The emergency group had greater 30-day postoperative mortality (8.0% vs 0.4%; P = .01) with no difference in abnormal neurodevelopmental outcome among the survivors (17.4% vs 13.8%; P = .35). The emergency group had more therapies for low cardiac output syndrome, more postoperative seizures, and a longer length of stay. CONCLUSIONS: An emergency ASO is a definitive rescue therapy that can be undertaken with acceptable mortality and neurodevelopmental outcome with consideration of the preoperative clinical state.

Factors influencing the choice-of-care pathway and survival in the fetus with hypoplastic left heart syndrome in New Zealand: a population-based cohort study.

OBJECTIVES: To better understand the relative influence of fetal and maternal factors in determining the choice-of-care pathway (CCP) and outcome in the fetus with hypoplastic left heart syndrome (HLHS). DESIGN: A retrospective, population-based study of fetuses with HLHS from a national dataset with near-complete case ascertainment from 20 weeks' gestation. Fetal cardiac and non-cardiac factors were recorded from the patient record and maternal factors from the national maternity dataset. The primary endpoint was a prenatal decision for active treatment after birth (intention-to-treat). Factors associated with a delayed diagnosis (≥24 weeks' gestation) were also reviewed. Secondary endpoints included proceeding to surgical treatment, and 30-day postoperative mortality in liveborns with an intention-to-treat. SETTING: New Zealand population-wide. PARTICIPANTS: Fetuses with a prenatal diagnosis of HLHS between 2006 and 2015. RESULTS: Of 105 fetuses, the CCP was intention-to-treat in 43 (41%), and pregnancy termination or comfort care in 62 (59%). Factors associated with intention-to-treat by multivariable analysis included a delay in diagnosis (OR: 7.8, 95% CI: 3.0 to 20.6, p<0.001) and domicile in the maternal fetal medicine (MFM) region with the most widely dispersed population (OR: 5.3, 95% CI: 1.4 to 20.3, p=0.02). Delay in diagnosis was associated with Maori maternal ethnicity compared with European (OR: 12.9, 95% CI: 3.1 to 54, p<0.001) and greater distance from the MFM centre (OR: 3.1, 95% CI: 1.2 to 8.2, p=0.02). In those with a prenatal intention-to-treat, a decision not to proceed to surgery was associated with maternal ethnicity other than European (p=0.005) and the presence of major non-cardiac anomalies (p=0.01). Thirty-day postoperative mortality occurred in 5/32 (16%) and was more frequent when there were major non-cardiac anomalies (p=0.02). CONCLUSIONS: Factors associated with the prenatal CCP relate to healthcare access. Anatomic characteristics impact treatment decisions after birth and early postoperative mortality. The association of ethnicity with delayed prenatal diagnosis and postnatal decision-making suggests systemic inequity and requires further investigation.

Congenital heart disease affects cerebral size but not brain growth.

Infants with congenital heart disease (CHD) have delayed brain maturation and alterations in brain volume. Brain metrics is a simple measurement technique that can be used to evaluate brain growth. This study used brain metrics to test the hypothesis that alterations in brain size persist at 3 months of age and that infants with CHD have slower rates of brain growth than control infants. Fifty-seven infants with CHD underwent serial brain magnetic resonance imaging (MRI). To evaluate brain growth across the first 3 months of life, brain metrics were undertaken using 19 tissue and fluid spaces shown on MRIs performed before surgery and again at 3 months of age. Before surgery, infants with CHD have smaller frontal, parietal, cerebellar, and brain stem measures (p < 0.001). At 3 months of age, alterations persisted in all measures except the cerebellum. There was no difference between control and CHD infants in brain growth. However, the cerebellum trended toward greater growth in infants with CHD. Somatic growth was the primary factor that related to brain growth. Presence of focal white matter lesions before and after surgery did not relate to alterations in brain size or growth. Although infants with CHD have persistent alterations in brain size at 3 months of age, rates of brain growth are similar to that of healthy term infants. Somatic growth was the primary predictor of brain growth, emphasizing the importance of optimal weight gain in this population.

Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal.

BACKGROUND: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. METHODS: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. RESULTS: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. CONCLUSION: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Isolated congenital mitral valve regurgitation presenting in the first year of life.

AIM: Isolated congenital mitral regurgitation is rare and, when presenting in infancy, reflects severity of the malformation. The natural history is often fatal, and management during the first year of life remains a therapeutic challenge. These infants are poorly understood largely because of an absence of reporting in the medical literature and limited experience in each institution. We reviewed our own experience in order to add to the understanding of this condition. METHODS: A retrospective review was performed on seven infants with significant isolated congenital mitral regurgitation. An associated patent ductus arteriosus was present in two. Mean age at referral was 17.7 weeks (1 day to 47 weeks) and mean weight was 6.8 kg (3.7-12.5 kg). RESULTS: Two infants were managed conservatively, and one underwent surgical ligation of a patent ductus arteriosus. Following spontaneous and surgical duct closure, no further intervention was required in two infants. The remaining four infants underwent three valve repairs and three valve replacements. The in-hospital mortality was 29%, occurring in those under 1 year of age undergoing emergency valve surgery. Two reoperations followed mitral valve repair in the first year of life. No significant complications or late deaths occurred. CONCLUSIONS: Our experience suggests closure of a patent ductus arteriosus should be undertaken prior to mitral valve surgery. There may be a poorer prognosis in those under one year of age requiring emergency mitral valve surgery. Those who can be managed conservatively or undergo mitral valve surgery as an elective procedure tend to have a better outcome.

Hybrid procedure for acute severe aortic regurgitation in an uncorrected adult tetralogy of Fallot.

Adult survivors of surgically uncorrected complex tetralogy of Fallot [TOF] with multiple aorto-pulmonary coronary arteries [MAPCAs] are unusual and the development of severe aortic regurgitation [AR] due to endocarditis is a rare and difficult problem to manage. We report a successful hybrid approach utilising temporary balloon occlusion of the collaterals during the cross-clamp time as a strategy to allow adequate brain perfusion and safe aortic root replacement.

The impact of morphological characteristics on late outcomes in patients born with hypoplastic left heart syndrome†.

OBJECTIVES: Patients with hypoplastic left heart syndrome are at high risk of late adverse events after Fontan completion, but it is unclear whether their morphological characteristics influence these outcomes. METHODS: Retrospective review of the data from the Australian and New Zealand Fontan Registry identified 185 patients with hypoplastic left heart syndrome who survived to hospital discharge after Fontan completion. Their outcomes were reviewed to identify predictors of adverse events with a particular focus on the impact of morphological characteristics. All available echocardiographic parameters were collected, and the hypoplasia of the left ventricle was subjectively considered to be mild, moderate or severe. RESULTS: The mean follow-up after the Fontan procedure was 6.4 ± 4.7 years. The median age at Fontan procedure was 4.41 years, 95% (176/185) of patients underwent an extracardiac conduit Fontan procedure and 71% (132/185) of those were fenestrated. At 15 years after Fontan, freedom from death and cardiac transplantation was 90% [95% confidence interval (CI) 85-97], freedom from Fontan failure was 78% (95% CI 70-87) and freedom from adverse events was 32% (95% CI 22-46). Morphological parameters did not influence transplant-free survival or Fontan failure. Independent risk factors predicting higher incidence of adverse events included aortic atresia (P = 0.003). CONCLUSIONS: The long-term survival of Fontan survivors with hypoplastic left heart syndrome is excellent and appears comparable to that of the general Fontan population. However, intrinsic morphological characteristics may continue to burden patients with late morbidity.

Proceedings From the 3rd Symposium of the World Database for Pediatric and Congenital Heart Surgery.

The World Database for Pediatric and Congenital Heart Surgery was created to provide a resource for centers to be able to perform complex outcomes analyses of children undergoing repair of a congenital heart defect. In just under two years, the World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has amassed over 13,000 procedures from 55 centers into the database. This Proceedings of the 3rd World Database Symposium held at the 6th Scientific Meeting of the WSPCHS summarizes the presentations of international experts in the fields of outcomes analysis and care of children with congenital heart surgery.

Fulminant mitral regurgitation due to ruptured chordae tendinae in acute rheumatic fever.

AIMS: Description of the presentation and management of cases of fulminant mitral regurgitation in acute rheumatic fever (ARF). METHODS: Retrospective case series of 4 children, aged 6-10 years, presenting in acute pulmonary oedema because of rupture of elongation of the chordae tendinae of the mitral valve leading to flail leaflets and severe mitral regurgitation. RESULTS: Urgent cardiac surgery with mitral valve repair was performed. Resolution of heart failure was achieved in each case. The difficulties in diagnosis and management of this uncommon and often unrecognised presentation of ARF are discussed. CONCLUSIONS: Cardiac surgery can be life saving for fulminant mitral regurgitation due to rupture of chordae tendinae of the mitral valve in ARF.

Cardiac outcome up to 15 years after the arterial switch operation.

AIMS: To assess the cardiac outcome and risk factors for mortality of infants following the arterial switch operation (ASO). METHODS: A single-centre retrospective review was conducted. Preoperative assessment, operative management and outcome was detailed for 244 patients undergoing the ASO at Green Lane Hospital for transposition of the great arteries (TGA) or double outlet right ventricle. RESULTS: The postoperative survival at 1, 5 and 15 years was 85%, 84% and 83%, respectively. The calendar year of ASO and the presence of a ventricular septal defect (VSD) were the primary predictors of early mortality. Late mortality was associated with a side-by-side configuration of the great arteries. Re-intervention following ASO was more common in patients with prolonged cardiopulmonary bypass time. CONCLUSIONS: Low early and late morbidity and mortality can be obtained in infants with TGA or double outlet right ventricle by definitive repair utilising the ASO.

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.