John L. Gibbs

Transcatheter closure of atrial septal defect and interatrial communications with a new self expanding nitinol double disc device (Amplatzer septal occluder): multicentre UK experience

OBJECTIVE To review the safety and efficacy of the Amplatzer septal occluder for transcatheter closure of interatrial communications (atrial septal defects (ASD), fenestrated Fontan (FF), patent foramen ovale (PFO)). DESIGN Prospective study following a common protocol for patient selection and technique of deployment in all participating centres. SETTING Multicentre study representing total United Kingdom experience. PATIENTS First 100 consecutive patients in whom an Amplatzer septal occluder was used to close a clinically significant ASD or interatrial communication. INTERVENTIONS All procedures performed under general anaesthesia with transoesophageal echocardiographic guidance. Interatrial communications were assessed by transoesophageal echocardiography with reference to size, position in the interatrial septum, proximity to surrounding structures, and adequacy of septal rim. Stretched diameter of the interatrial communications was determined by balloon sizing. Device selection was based on and matched to the stretched diameter of the communication. MAIN OUTCOME MEASURES Success defined as deployment of device in a stable position to occlude the interatrial communication without inducing functional abnormality or anatomical obstruction. Occlusion status determined by transoesophageal echocardiography during procedure and by transthoracic echocardiography on follow up. Clinical status and occlusion rates assessed at 24 hours, one month, and three months. RESULTS 101 procedures were performed in 100 patients (86 ASD, 7 FF, 7 PFO), age 1.7 to 64.3 years (mean (SD), 13.3 (13.9)), weight 9.2 to 100.0 kg (mean 32.5 (23.5)). Procedure time ranged from 30 to 180 minutes (mean 92.4 (29.0)) and fluoroscopy time from 6.0 to 49.0 minutes (mean 16.1 (8.0)). There were seven failures, all occurring in patients with ASD, and one embolisation requiring surgical removal. Immediate total occlusion rate was 20.4%, rising to 84.9% after 24 hours. Total occlusion rates at the one and three month follow up were 92.5% and 98.9%, respectively. Complications were: transient ST elevation (1), transient atrioventricular block (1), presumed deep vein thrombosis (1), presumed transient ischaemic attack (1). CONCLUSIONS It appears feasible to close interatrial communications and atrial septal defects up to 26 mm stretched diameter safely with the Amplatzer septal occluder. Short term results confirm an early high occlusion rate with no major complications. Careful selection of cases based on the echocardiographic morphology of the ASD and accurate assessment of their stretched diameter is of utmost importance. Further experience with the larger devices and longer term results are required before a firm conclusion regarding its use can be made.

Mutations in two nonhomologous genes in a head-to-head configuration cause Ellis-van Creveld syndrome.

Ellis-van Creveld syndrome (EvC) is an autosomal recessive skeletal dysplasia. Elsewhere, we described mutations in EVC in patients with this condition (Ruiz-Perez et al. 2000). We now report that mutations in EVC2 also cause EvC. These two genes lie in a head-to-head configuration that is conserved from fish to man. Affected individuals with mutations in EVC and EVC2 have the typical spectrum of features and are phenotypically indistinguishable.

Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome.

OBJECTIVE--To assess the feasibility of pulmonary artery banding, atrial septectomy or septostomy, and percutaneous stenting of the arterial duct in babies with the hypoplastic left heart syndrome. PATIENTS--Four infants with hypoplastic left heart syndrome. SETTING--Two supraregional paediatric cardiac centres. METHODS--Ductal patency was maintained initially with prostaglandin E. Banding of the proximal branch pulmonary arteries was performed through a median sternotomy and open atrial septectomy was performed if balloon septostomy was not. Stainless steel stents (Johnson & Johnson) mounted in a balloon catheter were implanted into the arterial duct under radiographic control and expanded to a diameter of approximately 8 mm, prostaglandin treatment was then stopped. RESULTS--All the patients survived the immediate postoperative period and maintenance of wide ductal patency was achieved in three of the four patients by stent implantation. Two weeks after the procedure two babies died of right ventricular failure and respiratory infection: some distal ductal constriction had occurred in one where the stent was not quite sufficiently distally placed. One child was discharged home 15 days after treatment and was well at follow up at age 16 weeks and one was stable but required diuretic therapy five weeks after the procedure. CONCLUSIONS--This new approach is technically feasible. At least in the short term it seems to offer hope of effective palliation for the hypoplastic left heart syndrome and it warrants further study.

Surgical and transcatheter (Amplatzer) closure of atrial septal defects: a prospective comparison of results and cost

Objective: To compare effectiveness, complications, and cost of Amplatzer with surgical atrial septal defect (ASD) closure. Design: Prospective study. Setting: Tertiary cardiac referral centre. Patients: 43 consecutive patients (excluding non-UK residents) aged between 2.1 and 56.8 years (median 7) undergoing ASD closure. Main outcome measures: Procedural success, complications, regression of right ventricular dilatation (up to one year postprocedure), cost, inpatient stay, and home convalescent time. Results: Amplatzer ASD closure was successful in 24 of 27 (89%) patients. Surgical closure was successful in all 19 cases. Cardiac complications affecting management occurred in three (11%) of the Amplatzer group (two procedural failures, one device embolisation) and 4 of 19 (21%) surgical patients (one pericardial pain, one global pericardial effusion requiring drainage, and one patient with anaemia requiring haematinics in addition to an incidental pericardial effusion and one further incidental pericardial effusion) (p = NS). There were complications that did not affect management in a further 5 of 19 surgical patients. There was no significant difference in regression of right ventricular dilatation by six months postprocedure (median right ventricular end diastolic diameter decrease: Amplatzer group 17.5%, surgical group 15.1%; median cardiothoracic ratio decrease: Amplatzer 7.9%, surgical 7.5%). Both hospital stay and home convalescent times were significantly shorter after Amplatzer closure (median hospital stay: Amplatzer one day, surgery six days; median convalescent time: Amplatzer two weeks, surgery 5.5 weeks). Median cost was similar for both groups (Amplatzer £5375, surgical £5412). Conclusions: Amplatzer ASD closure has a lower chance of success with a single procedure than surgery. Overall, there were more complications in the surgical group but the majority of these were minor and did not require any change in management. Resolution of right ventricular dilatation over the study period was similar for both techniques. Time spent in hospital and away from work or school was shorter for the Amplatzer group. The cost of both techniques was similar.

Dyspraxia or developmental coordination disorder? Unravelling the enigma

Dyspraxia is an enigma to many people, both professional and lay alike—what is it, how does it relate to developmental coordination disorder and associated conditions, how common is it, how is it recognised and diagnosed and how should it be managed? This article attempts to unravel this enigma by: dealing with the terminology of coordination difficulties from the “clumsy child syndrome” through “dyspraxia” to “developmental coordination disorder (DCD)”; briefly examining the debate as to whether dyspraxia or DCD should be regarded as a medical or social disorder; discussing the differential diagnosis of dyspraxia or DCD; considering the assessment of children with dyspraxia or DCD; reviewing the range of current treatment approaches in the UK.

Transcatheter occlusion of the arterial duct with Cook detachable coils: early experience.

OBJECTIVE: To assess the effectiveness of modified, controlled release Gianturco coils for transcatheter occlusion of the arterial duct. DESIGN: Prospective study, approved by local medical ethics committee. SETTING: Tertiary paediatric cardiac centre. PATIENTS: 43 patients with left to right shunting through the arterial duct, two of whom had a residual leak after surgical ligation and three had residual shunting after previous Rashkind umbrella implantation. INTERVENTION: Transcatheter delivery of one or more coils to the arterial duct. MAIN OUTCOME MEASURES: Complete occlusion of the arterial duct, based on intention to treat and judged by Doppler echocardiography. Absence of flow disturbance in the branch pulmonary arteries and the descending aorta following the procedure. Assessment of cost of the disposable items used. RESULTS: At a median follow up period of three months complete duct occlusion was achieved in 37 (86%) of the 43 patients. No flow disturbance in the branch pulmonary arteries or the descending aorta was detected in any patient. The median cost of disposable items used during the procedure was 342 pounds. CONCLUSIONS: The Cook detachable coil is an effective and financially attractive alternative to the Rashkind umbrella for closure of the arterial duct.

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.

Cardiovascular collapse after verapamil in supraventricular tachycardia.

Five babies who presented with supraventricular tachycardia were treated with verapamil intravenously. All developed severe hypotension and two died. Verapamil should not be used in the initial management of supraventricular tachycardia in neonates.

Cardiac monitoring and treatment for children and adolescents with neuromuscular disorders

Dilated cardiomyopathy, hypertrophic cardiomyopathy, and cardiac rhythm disturbances are important features of certain neuromuscular disorders in children, adolescents, and young adults. This article summarizes the cardiac features seen in patients with Duchenne muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy, Friedreichs ataxia, and Emery‐Dreifuss muscular dystrophy. The optimal management of these cardiac features remains contentious, but increasingly these patients are referred for routine cardiological assessment in the absence of symptoms. This article examines the value of routine screening and drug interventions for cardiac complications in asymptomatic and symptomatic individuals with neuromuscular disorders. We recommend a pragmatic approach, actively looking for cardiac conditions which will benefit from early intervention, but avoiding routine screening for asymptomatic conditions in which there is no evidence of benefit from early intervention.

Right ventricular outflow stent implantation: an alternative to palliative surgical relief of infundibular pulmonary stenosis.

OBJECTIVE: Preliminary assessment of the use of stents for palliative relief of right ventricular infundibular stenosis as an alternative to palliative surgical ventricular outflow enlargement. DESIGN: Descriptive clinical study. PATIENTS: Four patients with right ventricular outflow obstruction, aged between 2 and 15 years. One had had previous palliative surgery for pulmonary atresia, one had hypoplastic pulmonary arteries after palliative surgery for tetralogy of Fallot, one had multiple congenital abnormalities, and one had hypertrophic cardiomyopathy. SETTING: Tertiary paediatric cardiac centre. METHODS: After initial echocardiographic diagnosis the extent of right ventricular outflow obstruction was assessed by angiography. Balloon expandable stainless steel stents (Johnson & Johnson) were deployed in the right ventricular infundibulum. MAIN OUTCOME MEASURES: Improvement in right ventricular outflow assessed by ventriculography and change in right ventricular/ left ventricular pressure ratio, change in systemic oxygen saturation, freedom from arrhythmias, and sustained improvement in echocardiographic indices of obstruction. RESULTS: Mean right to left ventricular pressure ratio fell from 0.95 to 0.35 in the three patients with intact ventricular septum. Oxygen saturation increased from 76% to 91% in the patient with tetralogy. No arrhythmias were detected. Improvement was maintained at mean follow up of 9.7 months in three cases, but one patient required stent enlargement 17 months later because of neoendothelial proliferation within the stent. CONCLUSION: Stent implantation provides an effective alternative to palliative surgical enlargement of the right ventricular infundibulum. Neoendothelial proliferation causes reduction in lumen in some cases, but this may respond to redilatation.