Background: Transcatheter device closure has become the established standard of care for suitable atrial septal defects. Device erosion has been a recent focus and has prompted changes in the Instructions for Users documentation released by device companies. We reviewed our entire local experience with atrial septal defect device closure, focussing on the evolution of this procedure in our centre and particularly on complications. Methods: We carried out a retrospective review of 581 consecutive patients undergoing attempted transcatheter device closure of an atrial septal defect in Auckland from December 1997 to June 2014. We reviewed all complications recorded and compared our outcomes with the current literature. We sought to understand the impact of the evolution in recommendations and clinical practice on patient outcomes in our programme. Results: There were a total of 24 complications (4.1%), including 10 device embolisations (1.7%), nine arrhythmias (1.5%), two significant vascular access-related complications (0.3%), one device erosion (0.2%), one malposed device (0.2%), and one probable wire perforation of the left atrial appendage (0.2%). There was one mortality related to device embolisation. All device embolisations occurred following the change in Instructions for Users after publication of the first device erosion report in 2004. This increase in embolisation rate was statistically significant (p-value 0.015). Conclusions: In our series, the incidence of device embolisation was higher than that anticipated, with a significant increase following changes to the Instructions for Users. This highlights the need for ongoing data collection on complication incidence and for ongoing review of the impact of changes in clinical practice on complication rates.

Noncompaction cardiomyopathy (NCCM) is a recently recognized form of cardiomyopathy with a variable clinical presentation. This report describes NCCM of a newborn presenting with hydrops fetalis and pulmonary hypertension. To date, this has not been described in the literature. As knowledge of this condition grows, the need to recognize NCCM becomes more important.

Objective : To analyze the safety and clinical impact of interventional cardiac catheter procedures in the management of early postoperative problems after completion of an extracardiac Fontan procedure. Background : The mortality after Fontan procedure has consistently decreased over the last decade. The role of interventional catheterization to address early postoperative problems in this setting has not been studied systematically. Methods : Over a 9.7‐year period, 289 patients underwent an extracardiac fenestrated Fontan procedure with two early deaths (0.7%) and takedown in four (1.4%). Twenty‐seven patients (9.3%) underwent 32 interventional cardiac catheter procedures at a median interval of 12.2 (1–30) days. The median weight was 14.5 (13.5–25) kg. The case notes and procedure records were reviewed retrospectively. Results : Fontan pathway obstructions were treated in 11 patients with stent implantation with good results and no complications. Stent fenestration of the Fontan circulation was performed in 16 patients with one episode of transient hemiparesis and one episode of pericardial effusion. Three patients underwent initial balloon dilatation of branch pulmonary arteries or fenestration with little effect and underwent stent treatment 6 (5–9) days later. One patient had device closure of a large atrial fenestration. In one patient, residual anterograde pulmonary blood flow was occluded using a device. There were no deaths and in‐hospital course was improved in all. Conclusion : Interventional cardiac catheter procedures can be performed safely and effectively in the early postoperative period after Fontan completion to address hemodynamic problems. These techniques contribute significantly to achieve a very low mortality and address morbidity after Fontan completion. © 2010 Wiley‐Liss, Inc.

Background. Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically Aim: Present our experience with sildenafil treatment in patients with a failing Fontan circulation Patients and methods: Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008 Results: Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1-2 milligrams per kilogram 3-4 times per day Protein-losing enteropathy and alpha-l-antitrypsin levels improved in all three patients on sildenafil treatment One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years Conclusions: Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.

INTRODUCTION The Norwood stage 1 procedure was modified by Sano with right ventricle-pulmonary artery (RV-PA) conduit replacing BT shunt. In our institution, this has been further modified by placing the conduit from the RV outflow tract to the right side of the neo-aorta. PATIENTS AND METHODS Between April 2002 and October 2008, 227 modified Norwood procedures were performed. Eighteen had the Sano modification with the conduit to the left of the neo-aorta whereas 209 had the right-sided modification, which is the study population. A total of 18 (8.6%) patients presented with cyanosis due to conduit stenosis with median age 4 months and median weight 6.3 kg. RESULTS Twelve patients underwent transcatheter stent placement in stenosed RV-PA conduit. A total of 16 coronary stents were implanted in 12 patients with 4 patients each receiving 2 stents. The mean saturations increased from 60% to 74%. There was one late mortality which was non-procedure related. Five patients treated with surgical take down of the RV-PA conduit and creation of a cavo-pulmonary shunt, whilst one patient had replacement of RV-PA conduit. There were no early postoperative deaths. The mean saturations improved from 54% to 75%. CONCLUSIONS The RV-PA conduit stenosis is a life-threatening complication after the modified Norwood Stage I procedure. This may require urgent surgery to replace the conduit or to perform a cavo-pulmonary shunt but as an alternative, transcatheter stent placement can be used with equal effectiveness and with a low risk of complications. The catheter approach is less invasive and the results show that it is an excellent option to relieve the stenosis even in the right-sided RV-PA conduit.

Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is underdeveloped. Surgical management of hypoplastic left heart syndrome has changed the prognosis of the condition that was previously regarded as fatal. We discuss surgical strategies based on staged procedures, with the right ventricle supporting both systemic and pulmonary circulation. We also discuss other management options, such as neonatal transplantation and the recent innovation of hybrid techniques. Surgical techniques and the understanding of the pathophysiology of this condition have been at the forefront of neonatal cardiac surgery and intensive care. The management of the syndrome remains a challenge because affected children grow into adolescence and adulthood posing various new problems and demands.

Objective: Most devices devices available for percutaneous closure of Fontan fenestrations tend to be bulky. The aim of this study was to evaluate a low profile custom made device and assess its efficacy and safety. Patients and Methods: A 15 mm PFO star was used as the basis. The following modifications were made: removal of the left disc to reduce thrombogenicity in the left atrium, increase the length of the LA legs from 2 by 15 mm to 3 by 20 mm to prevent dislodgement and later adding a pivot between the left and right umbrella. A partial occluder was made by removing two opposite quadrants from the proximal disk. Results: Device deployment was possible in 93% (63 of 68) patients. In five patients, the device could not be deployed and an alternative device was used. In 45 patients complete closure of the fenestration was obtained and saturations increased from 84% +/- 4% to 95% +/- 2% (P < 001). In 18 high risk patients with suboptimal Fontan circulation, a modified device was used to effect partial occlusion: saturations increased from 79% +/- 7% to 90% +/- 4% (P < 0.001); a residual shunt persisted in most patients for several months. No thrombotic events were recorded during follow-up. Conclusions: The modified PFO star device can safely be deployed in Fontan patients to occlude or restrict flow through a fenestration. It has a low profile with minimal foreign material, is non-obstructive and minimally thrombogenic. (C) 2009 Wiley-Liss, Inc.

Currently, a three-stage surgical palliation remains the treatment of choice at Birmingham Children's Hospital. After initial introduction of the classical Norwood with pulmonary blood flow provided by a modified Blalock-Taussig shunt, a right ventricular to right pulmonary artery conduit at stage 1 Norwood palliation is now used in most cases, a bi-directional 'Glenn' shunt at second stage and an extra-cardiac Fontan completion at third stage. Mortality and morbidity has improved after modification of the technique. Thirty-day mortality was 32.4% (79/244) for the 'classical' Norwood procedure, 25.0% (7/28) for the left-sided RV-PA conduit and 12.7% (22/173) for the right-sided RV-PA conduit. Interstage mortality was 8.6% (21/244) for the 'classical' Norwood procedure, 14.3% (4/28) for the left and 10.1% (15/148) for right-sided RV-PA conduit. After stage II, 30-day mortality was 3.0% (10/335) for all groups. Stage III 30-day mortality was 0.9% (1/115) for all groups.

OBJECTIVE:The aim of this study was to compare the outcome of the double-switch procedure for congenitally corrected transposition of the great arteries for patients completing morphologic left ventricle training by means of pulmonary artery banding with the outcome of patients whose morphologic left ventricle did not require training.METHODS:A retrospective study of all patients undergoing the double-switch procedure from 1991 through 2004 was performed. Patients were divided into 2 groups: those not requiring morphologic left ventricle training (n = 33) and those completing morphologic left ventricle training by means of pulmonary artery banding (n = 11).RESULTS:The time spent with the morphologic left ventricle conditioned at systemic pressures was longer for the group not requiring morphologic left ventricle training (median, 730 days; interquartile range, 399-1234 vs median, 436 days; interquartile range, 411-646; P = .19). The overall mortality (not requiring morphologic left ventricle training, 12.1%; requiring morphologic left ventricle training, 9.1%; P = 1) and rate of death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both (not requiring morphologic left ventricle training, 21.2%; requiring morphologic left ventricle training, 45.5%; P = .14) were similar between groups. Actuarial freedom from death/transplantation with good morphologic left ventricular function was superior for patients whose morphologic left ventricle did not require training (P = .04). The follow-up was not different between groups (not requiring training: median, 1435 days [interquartile range, 285-2570 days]; requiring morphologic left ventricle training: median, 568 days [interquartile range, 399-1465 days]; P = .14). On multivariate analysis, the completion of morphologic left ventricle training predicted death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both (P = .02).CONCLUSIONS:The early results of the double-switch procedure in patients whose morphologic left ventricle required training compare favorably with those of patients whose morphologic left ventricle required no training. There is an increased risk of deterioration of morphologic left ventricle function over time in patients whose morphologic left ventricle requires training, and these patients need to be followed up regularly to detect this.

A 12.5-year-old boy with tricuspid atresia and quadriplegic cerebral palsy presented with increasing cyanosis after previous palliation with a cavopulmonary shunt and ligation of the main pulmonary artery (MPA). Because of severe physical disabilities he was not considered suitable for Fontan completion. He underwent successful transcatheter stent recanalization of the ligated MPA. This re-established anterograde flow to the pulmonary arteries resulting in marked improvement in saturations. (C) 2007 Wiley-Liss, Inc.

BACKGROUND:Ventricle-pulmonary artery connections in patients after the Fontan procedure lead to ineffective volume loading and can cause long term problems. In patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but can cause significant volume loading of the heart or complicate the subsequent Fontan procedure.OBJECTIVE:To evaluate the use of transcatheter closure of a ventricle-pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure.PATIENTS AND METHODS:Retrospective study at a tertiary referral centre. Eight patients (age 1.5-18 years, mean 7.8 years).INDICATIONS:cardiac failure or persistent pleural effusions after cavopulmonary shunt (n = 2) or after Fontan (n = 3) and abolishing the volume load of the single ventricle prior to Fontan completion (n = 3).RESULTS:Devices used: Rashkind Umbrella (n = 1), Amplatzer PDA (n = 7) and Amplatzer ASD (n = 1). One patient required two devices. There were no procedural complications. All 3 patients with prolonged pleural effusions (1 post CP shunt and 2 post Fontan) showed complete resolution between 4 and 10 days after catheter closure. Two patients underwent transcatheter occlusion for progressive ventricular dilatation and cardiac failure. The first patient was post Fontan and showed gradual improvement in ventricular function. The second patient (post CP shunt) was in end stage cardiac failure due to severe AV valve regurgitation. The patient died 48 hours after an uncomplicated procedure due to ventricular failure and electromechanical dissociation (non-procedure-related cardiac death). Three patients underwent catheter closure to off-load the systemic ventricle prior to the Fontan procedure. The device had to be removed prior to release in one patient, due to unsatisfactory position.CONCLUSIONS:Transcatheter closure of ventricle-pulmonary artery communication is a safe and effective technique in the treatment of selected patients after cavopulmonary shunt or Fontan procedure with early or late complications due to inappropriate pulmonary blood flow. This intervention should also be considered in the preparation for the Fontan procedure in selected patients with ventricular overload.

Objective: Intraoperative ultrasound was introduced to evaluate the adequacy of repair after surgical repair of congenital cardiac malformations. Our purpose was to review the evolution of this technique at our centre. Methods: We evaluated all intraoperative ultrasound studies undertaken between 1997 and 2002, reviewing the data from 1997 through 2001 retrospectively, but undertaking a prospective audit of studies undertaken from 2002 onwards. In all, we carried out a total number of 639 intraoperative ultrasound studies, from a possible 2737 cardiac operations (23.3%), using the epicardal approach in 580 (90.7%), and transoesophageal ultrasound in the other 59 patients (9.3%). Median age was 0.6 years, with an interquartile range from 0.06 to 3.6 years. Results: The findings obtained using intraoperative ultrasound influenced the surgical management in 63 of the 639 patients (9.9%), suggesting the need for additional surgery in 26, adjustment of the band placed round the pulmonary trunk in 16, preoperative assessment of the cardiac malformation in 5, and confirming the need for prolonged support with cardiopulmonary bypass for impaired ventricular function in 16. There were 18 early reoperations, 5 of which may have been predicted by intraoperative ultrasound. Of the 183 studies reviewed prospectively in 2002, it was not possible to obtain the complete range of views in 8 (4.4%), while in 27 patients (14.7%), the postoperative findings using transthoracic interrogation differed from the findings obtained immediately following bypass. Conclusion: Intraoperative ultrasound is an important technique for monitoring the results of complex congenital cardiac surgery. The immediate recognition of significant lesions, together with multidisciplinary discussion, allows for improved management and prevention of early surgical reintervention.

Background: Mitral valve replacement (MVR) is occasionally indicated in infants and young children, necessitating the use of small prostheses. The performance of these small valves during somatic growth of the patient can lead to patient-prosthesis mismatch. This study examines performance of these valves over time to establish predicted performance and timing of replacement. Methods: Records were reviewed of all patients under 5 years of who underwent small mechanical MVR between 1988 and 2004 (n = 24). Valve sizes were between 17 and 23 mm (Bileaflet 91.6%, Tilting Disc 8.3%) with a median size of 19 mm. Mean age of patients was 1.4 +/- 1.3 years with a mean weight of 7.8 +/- 3.4 kg. Results: Early deaths (n = 5, 20.8%) were excluded. There were two late deaths and five patients required redo-MVR: four for outgrowth and one for acute thrombosis at 3 months. Age at redo for outgrowth was 8.6 +/- 6.6 years with mean body weight of 22.5 +/- 17.5 kg. Mean time between original operation and redo was 8.6 +/- 6.1 years in these four patients. Follow-up was a median of 7.5 years (range 0.1-15.7 years). Overall freedom from death or valve replacement was 82.6 +/- 9.1% at 5 years and 75.7 +/- 10.6% at 10 years. The performance of the original prostheses showed a peak blood flow velocity across the valves of 1.5 +/- 0.6 m/s at 5 years and 2.2 +/- 0.5 m/s at 10 years. Seventy-five percent of the survivors still have their original valve at a mean of 8.1 +/- 4.4 years postoperative with New York Heart Asociation status of I or II. Actuarial curves suggest that gradients across the valves reach a peak of > 10 mmHg at a mean between 6.5 and 7 years postoperative. Conclusion: MVR in children under 5 years carries a high mortality. Nevertheless, small mechanical MVR perform remarkably well. in young children with durable haemodynamics despite growth of the patients welt beyond more than double the initial bodyweight. Valves can be expected to last over 8 years before requiring re-replacement. (c) 2006 Elsevier B.V. All. rights reserved.

Objective: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. Methods: Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock-Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). Results: Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60 +/- 3%, 52 +/- 4% and 50 +/- 4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR 8.3, 5.3-13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98 +/- 1%, 72 +/- 4% and 58 +/- 6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6-9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. Conclusions: CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage I I procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP. (c) 2006 Elsevier B.V. All rights reserved.

BJOG: An International Journal of Obstetrics & GynaecologyVolume 110, Issue 7 p. 710-710 Prenatal diagnosis of the Wolf–Parkinson–White syndrome by fetal magnetocardiography Alexander McKenzie Pirie, Alexander McKenzie Pirie City Hospital, Birmingham, UKSearch for more papers by this authorJohn Wright, John Wright Birmingham Children's Hospital, Birmingham, UKSearch for more papers by this author Alexander McKenzie Pirie, Alexander McKenzie Pirie City Hospital, Birmingham, UKSearch for more papers by this authorJohn Wright, John Wright Birmingham Children's Hospital, Birmingham, UKSearch for more papers by this author First published: 22 December 2003 https://doi.org/10.1046/j.1471-0528.2003.02036.xCitations: 5Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume110, Issue7July 2003Pages 710-710 RelatedInformation

Objective: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology. Methods: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n = 44, 42%) or right ventricular morphology (n = 59, 58%). The TCPC was performed using an extracardiac conduit (n = 84, 82%) or a lateral atrial tunnel (n = 19.18%). and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation: early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years). Results: Early mortality was 1.9% (n = 2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/- 1 SEM) was 95.6 +/- 2.5%. Forty-two patients (41%) had prolonged pleural drainage (+/- 14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/- 1 SEM) were 92.2 +/- 5.0 and 73.4 +/- 6.0%, respectively. The Fontan procedure was associated with an improved functional class (P < 0.005) and all current survivors (n = 99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P < 0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P < 0.05), increased postoperative pulmonary artery pressures (P < 0.005) and an unfenestrated Fontan procedure (P < 0.01). Conclusions: In this contemporary series, the modified Fontan procedure was characterised by low early mortality. excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival. (C) 2003 Elsevier Science B.V. All rights reserved.