Darren Hutchinson

Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization.

OBJECTIVES This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants. BACKGROUND Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis. METHODS Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis. RESULTS From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years. CONCLUSIONS Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer.

Arterial ischemic stroke in children with cardiac disease

Objective: To describe the spectrum of cardiac disorders, timing in relation to interventional procedures, and outcome in children with cardiac disease and arterial ischemic stroke (AIS). Methods: Children younger than 18 years with cardiac disease and radiologically confirmed AIS admitted to the Royal Childrens Hospital Melbourne between 1993 and 2010 were retrospectively identified using ICD-9 and ICD-10 searches. Results: Seventy-six children with cardiac disease and radiologically confirmed AIS were identified with the median age at diagnosis of 5 months (interquartile range 0–58). Cardiac lesions included cyanotic congenital heart disease (CHD) in 42 (55%), acyanotic heart disease in 24 (29%), cardiomyopathies/myocarditis in 6 (8%), infective endocarditis in 3 (4%), and primary arrhythmias in 3 (4%). Stroke occurred following cardiac procedures in 52 patients (68%): 41 post cardiac surgery (4.6 strokes per 1,000 surgical procedures) and 11 post cardiac catheterization (1.7 strokes per 1,000 catheterizations). The median time from procedure to diagnosis of stroke was 3 days (interquartile range 2–7), with 68% (95% confidence interval 58%–79%) of strokes estimated to occur within the periprocedural period. Prevalence of periprocedural stroke varied by diagnostic category, but was most common in patients with cyanotic CHD undergoing palliative surgery (22/2,256, 1%) (p < 0.005). There were 3 AIS-related deaths, and 54 survivors (84%) had persisting neurologic deficits. Conclusions: Infants with cyanotic CHD were most frequently affected by AIS during the periprocedural period. Prospective cohort studies are required to determine effective primary and secondary prevention strategies.

Right‐sided aortic arch in the age of microarray

For fetuses with a diagnosis of right aortic arch and normal cardiac anatomy, we aimed to establish the frequency of chromosomal anomaly diagnosed with single nucleotide polymorphism microarray analysis, particularly focusing on microduplications or microdeletions which would have gone undetected by conventional karyotyping and six‐probe fish (13,18,21, X,Y, TUPLE).

OP10.07: Relevance of detection of isolated aberrant right subclavian artery (ARSA) in a low-risk screened population: Short oral presentation abstracts

G.R. DeVore1,2, G. Satou3, M. Sklansky3, B. Cuneo4 1Fetal Diagnostic Centre, Pasadena, CA, USA; 2Obstetrics and Gynecology, David Geffen School of Medicine at UCLA, Pasadena, CA, USA; 3Division of Pediatric Cardiology, Department of Pediatrics, UCLA Mattel Children’s Hospital, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 4Heart Institute, Children’s Hospital Colorado, Denver, CO, USA

Successful ablation of refractory neonatal atrial flutter.

The patient was a male infant born at 38 weeks’ gestation.Atrialflutterwasdiagnosedinuteroat35weeksofgestation,at which time no hydrops fetalis was present and notreatment was commenced. At 6 hours of life, the infantdeveloped tachycardia. The electrocardiogram (ECG)showed atrial flutter with 2:1 atrioventricular conduction(Figure 1). Initially the rhythm could be reverted to sinus bybiphasic direct current (DC) cardioversion. However, atrialflutter recurred despite intravenous amiodarone infusion.The infant developed heart failure on day 3 of life. Anechocardiogram demonstrated a structurally normal heartwith significant biatrial dilation and moderately impairedbiventricular function. No intracardiac thrombus was evi-dent. Transesophageal overdrive pacing was attempted butfailed. DC cardioversion (at 1 J/kg) could revert the rhythmto sinus, but atrial flutter recurred within 12 hours. In total,DC cardioversion was attempted 6 times with varyingcombinations of intravenous amiodarone (15 μg/kg/min),intravenous esmolol (up to 175 μg/kg/min), oral digoxin(8 μg/kg/day), sotalol (4.5 mg/kg/day), and flecainide (4 mg/kg/day), but none of them could maintain the rhythm insinus, and atrial flutter recurred within 12 hours. In view ofthe persistent ventricular dysfunction with biatrial dilation,electrophysiologic study with potential radiofrequency abla-tion of the atrial flutter was performed on day 14 of life.A 6Fr transesophageal electrode was placed to record leftatrial activation and allow pacing. A 5Fr Therapy