Anomalous aortic origin of a coronary artery: a universally applicable surgical strategy.

The most commonly reported coronary arterial malformation, in accounts of sudden deaths, is anomalous aortic origin of a coronary artery. Anomalous coronary arteries may arise from the left, right, or non-coronary sinuses of Valsalva. Importantly, although the left coronary artery from the right sinus has the worst prognosis, sudden death has been reported in all variants of origin from the various sinuses of Valsalva. This paper describes a technique that addresses all of the problems relating to anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. This technique includes the transection of the ascending aorta and pulmonary trunk, coronary arterial enlargement with a pericardial patch, and lateral translocation of the pulmonary trunk to the left pulmonary artery. Anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva is a potentially lethal cardiac anomaly that can be corrected in all cases using this simplified surgical technique that addresses the major anatomic and physiological problems.

The current surgical perspective to repair of atrioventricular septal defect with common atrioventricular junction.

Atrioventricular septal defect with common atrioventricular junction is a relatively common congenital cardiac malformation. It sometimes presents challenging surgical problems, especially when seen in combination with tetralogy of Fallot. We describe herein our current concepts regarding surgical repair of atrioventricular septal defect with and without tetralogy, discussing indications for surgery, timing, technical aspects, and outcomes.

Digital video recording of congenital heart operations with "surgical eye".

We describe our experience with routine digital video recording of congenital heart operations. We currently use the "surgical eye," in which a small camera is mounted between the standard surgical loupe lenses. This technique has evolved during the years of experience with various other techniques. We believe this is a good method for accurate definition of intracardiac anatomy that closely resembles the surgeon's view. This arrangement is economical and invaluable in training. This method of recording has been used in more than 1,000 operations.

The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society 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 World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

Durability of hand-sewn valves in the right ventricular outlet.

OBJECTIVE: The objective was to compare the medium- and long-term outcomes for pericardial monocusp valves, polytetrafluoroethylene (Gore-Tex, WL Gore and Associates Inc, Flagstaff, Ariz) 0.1-mm monocusp valves, and bileaflet 0.l-mm polytetrafluoroethylene valves and their efficiency in the right ventricular outlet. METHODS: We reviewed all hand-sewn right ventricular outlet valves created by the author (Graham R. Nunn) in the setting of repaired tetralogy of Fallot or equivalent right ventricular outlet pathology when the native pulmonary valve could not be preserved. The valves were assessed by serial transthoracic echocardiography and more recently by magnetic resonance imaging angiography for late valve function. The bileaflet polytetrafluoroethylene valves were constructed in a standardized fashion from a semicircle of 0.1-mm polytetrafluoroethylene (the radius of which equaled the length of the outflow tract incision) that gave a lengthened free edge to the leaflets, central fixation of the free edge posteriorly just proximal to the branch pulmonary arteries, and generous augmentation of the outflow tract with polytetrafluoroethylene patch-plasty. The bileaflet configuration shortens the closing time against the posterior wall, and the leaflets are forced to maintain their configuration without prolapse into the right ventricular outlet. The valve can be generously oversized in young children to try to avoid the need for replacement. RESULTS: A total of 54 patients met the selection criteria--22 patients received fresh autologous pericardial monocusps, 7 patients received polytetrafluoroethylene (0.1-mm) monocusps, and 25 patients received bileaflet polytetrafluoroethylene (0.1-mm) outlet valves. The pericardial valves have the longest follow-up, and all valves developed free pulmonary incompetence. Polytetrafluoroethylene monocusps had reliable competence early after surgery but progressed to pulmonary incompetence. The bileaflet polytetrafluoroethylene (0.1-mm) valves have remained competent with regurgitant fractions of only 5% to 30% (magnetic resonance imaging angiography), and this has remained stable with time. The maximum follow-up for these valves is 5 years. No stenosis or peripheral emboli have been recognized, and no valves have been replaced to date. CONCLUSION: Hand-sewn bileaflet polytetrafluoroethylene valves in the right ventricular outlet can reliably provide competence and maintain function in the medium term. Their shape and size allow placement in young children with a reasonable expectation that they will remain competent with growth of the native annulus and not require replacement. Their durability is superior to the pericardial and polytetrafluoroethylene monocusp valves in this series.

Atrioventricular canal: modified single patch technique.

A single surgeon experience using a modified single patch technique for the repair of 128 patients with complete atrioventricular canal is presented. Thirty-day mortality was 1.6%. Follow-up of these patients has shown no incidence of significant residual ventricular septal defect, a 2.3% incidence of reoperation on the mitral valve, and no instances of left ventricular outlet obstruction requiring resection in the follow-up period. Comparisons are drawn between these results and the author's own experience with repair of complete atrioventricular canal using a two-patch technique (46 cases) and repair of partial atrioventricular canal (126 cases) to shed light on late valve function and left ventricular outlet obstruction in all groups.

Clinical assessment of cardiac performance in infants and children following cardiac surgery.

OBJECTIVE: To compare clinical assessment of cardiac performance with an invasive method of haemodynamic monitoring. DESIGN AND SETTING: Prospective observational study in a 16-bed tertiary paediatric intensive care unit. PATIENTS AND PARTICIPANTS: Infants and children undergoing cardiopulmonary bypass and surgical repair of congenital heart lesions. INTERVENTIONS: Based on physical examination and routinely available haemodynamic monitoring in the paediatric intensive care unit, medical and nursing staff assessed cardiac index, systemic vascular resistance index and volume status. Clinical assessment was compared with cardiac index, systemic vascular resistance index and global end diastolic volume index, obtained by femoral artery thermodilution. MEASUREMENTS AND RESULTS: A total of 76 clinical estimations of the three parameters were made in 16 infants and children undergoing biventricular repair of congenital heart lesions. Agreement was poor between clinical and invasive methods of determining all three studied parameters of cardiac performance. Cardiac index was significantly underestimated clinically; mean difference was 0.71 l min(-1) m(-2) (95% range of agreement +/-2.7). Clinical estimates of systemic vascular resistance (weighted kappa=0.15) and volume status (weighted kappa=0.04) showed poor levels of agreement with measured values and were overestimated clinically. There was one complication related to a femoral arterial catheter and one device failure. CONCLUSIONS: Routine clinical assessment of parameters of cardiac performance agreed poorly with invasive determinations of these indices. Management decisions based on inaccurate clinical assessments may be detrimental to patients. Invasive haemodynamic monitoring using femoral artery thermodilution warrants cautious further evaluation as there is little agreement with clinical assessment which is presently standard accepted care in this patient population.

Mid-term results for double inlet left ventricle and similar morphologies: timing of Damus-Kaye-Stansel.

BACKGROUND: Patients with double inlet left ventricle/l-transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. A Norwood procedure may be performed as the initial palliation. We prefer aortic arch repair and pulmonary artery banding, delaying Damus-Kaye-Stansel (DKS) or BVF resection until the second palliation. The aims of this study were to compare our results with those reported for Norwood strategy and examine the development of systemic outflow obstruction. METHODS: Retrospective study of patients with double inlet left ventricle, L-TGA or similar morphology presenting between 1990 and 2000. Follow-up with clinical assessment, echocardiography and catheter studies. RESULTS: Twenty-five patients had initial palliation with pulmonary artery banding with repair of any associated arch obstruction. Twelve patients had DKS performed as part of their second stage procedure, and 3 had DKS performed later for recurrent stenosis after prior enlargement of BVF. Six patients had BVF resection without later restenosis and 4 patients did not develop BVF stenosis. There was one early death (4%) and two late (8%). Fontan completion was achieved in 20 of the 22 survivors. There were no cases of DKS obstruction, no pulmonary valve had more than mild regurgitation. CONCLUSIONS: Our approach achieves low operative mortality and morbidity and compares favorably with reported results for Norwood palliation. The significant rate of systemic outflow obstruction in those who did not undergo DKS at the second stage confirms the utility of early DKS in children with this morphology.

Successful early surgical recruitment of the congenitally disconnected pulmonary artery.

BACKGROUND: Our purpose was to document our experience with early recruitment of congenitally disconnected pulmonary arteries and to assess subsequent pulmonary artery growth and function. METHODS: Patients born in the 10-year period from 1989 to 1999 with a disconnected pulmonary artery diagnosed in infancy and treated in our unit were studied. To be included patients had nonconfluent pulmonary arteries with one or both completely disconnected from the main pulmonary artery. This series did not include patients with acquired stenosis causing occlusion of a pulmonary artery. Echocardiography, cardiac catheterization, MRI, lung perfusion scans, and intraoperative assessment were used to gauge pulmonary artery growth and function. RESULTS: Seven patients with a disconnected pulmonary artery associated with intracardiac conotruncal congenital cardiac disease underwent successful early surgical recruitment of the affected pulmonary artery at 3 months of age or younger. Median follow-up from date of first operation was 4.2 years (range, 1.6 to 13.4). All 7 patients had postrecruitment lung perfusion scans showing a mean of 44% (range, 27% to 78%) of total pulmonary flow through the affected lung. Significant growth in the diameter of the recruited native pulmonary artery was demonstrated in all patients. There were no deaths reported in our series to date. CONCLUSIONS: The rare possibility of a congenitally disconnected pulmonary artery needs to be considered in all patients with a conotruncal cardiac anomaly. To facilitate surgical correction, ensure subsequent growth of the pulmonary artery, and optimize associated lung development, early diagnosis and surgical recruitment is recommended.

Recombinant activated factor VII in paediatric cardiac surgery.

OBJECTIVE: To review the use of recombinant activated factor VII in paediatric cardiac surgery. DESIGN: Retrospective chart review. SETTING: Paediatric intensive care unit in a stand-alone university-affiliated children's hospital. PATIENTS AND PARTICIPANTS: Cardiac surgical patients who received recombinant activated factor VII (rFVIIa, NovoSeven; NovoNordisk, Copenhagen, Denmark) between June 2002 and June 2003 at The Children's Hospital at Westmead. RESULTS: Six children undergoing cardiac surgery received rFVIIa. Recombinant activated factor VII was administered if bleeding was excessive and persisted despite appropriate investigation and attention to haemostasis by surgical and medical staff. An intravenous dose of 180 microg/kg was given and repeated 2 h later. All of the six patients responded well to rFVIIa with achievement of haemostasis. No adverse events were noted. CONCLUSIONS: Recombinant activated factor VII achieved haemostasis in six paediatric cardiac surgical patients. Good outcomes and no adverse events were noted in these children.

Contrast echocardiography: potential for the in-vivo study of pediatric myocardial preservation.

BACKGROUND: Myocardial contrast echocardiography (MCE) has been used successfully during adult cardiac surgery to image myocardial perfusion. Recently it has been suggested this technique is capable of detecting microvascular injury and inflammation because sonicated albumin microbubbles adhere to activated neutrophils and, in the presence of denuded or inflamed endothelium, they persist within the microvasculature rather than passing unimpeded, which results in profound slowing of their transit rates. The technique has not previously been used during congenital heart surgery; however significant potential is suggested in this setting in which myocardial inflammation may contribute to postoperative myocardial dysfunction, a leading cause of morbidity and mortality. We have performed a preliminary study to assess the safety and feasibility of MCE in the pediatric intraoperative environment and to examine myocardial transit rates. METHODS: Sonicated albumin microbubbles were injected with cardioplegia during bypass in 16 children (aged 3 weeks to 8.5 years). Images were collected using transesophageal echocardiography. Complications, post-bypass electrocardiographic, echocardiographic, and outcome data were recorded. Myocardial transit rates were calculated using videointensity analysis, assessed for reproducibility and correlated with demographic and intraoperative variables and postoperative outcome. RESULTS: The technique was performed safely, with good reproducibility. Myocardial persistence of microbubbles, which occurred in 6 patients, was associated with crystalloid cardioplegia, prolonged preischemic bypass (r = 0.72, p = 0.004), or ischemic time (r = 0.69, p = 0.002). CONCLUSIONS: Intraoperative MCE shows potential as an in vivo technique for the study of pediatric myocardial preservation.