Stephen G. Cooper

Simplified single patch technique for the repair of atrioventricular septal defect

OBJECTIVE Because of the complexity of traditional 1- and 2-patch techniques for the repair of complete atrioventricular septal defect, we modified our repair technique to avoid the use of any ventricular septal patch material. We report our prospective experience with this simplified 1-patch technique. METHOD Forty-seven consecutive patients between May 1995 and August 1998 underwent repair with the use of this technique without modification. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum. No division of valve leaflets was necessary. A single pericardial patch was used to close the defect in the atrial septal component. Follow-up included electrocardiography and echocardiographic assessment of ventricular function, atrioventricular valve function, and adequacy of the left ventricular outflow tract. RESULTS There were 2 deaths (4%), only 1 cardiac related, in the series. There were 17 male patients and 30 female patients. Mean age at repair was 5.6 months (median, 3.4 months). Associated lesions were repaired in 19 patients (40%). Mean follow-up was 1.85 years (median, 1.9 years). There was no heart block. There were no significant residual ventricular septal defects detected and no left ventricular outflow tract obstruction seen on echocardiography in any patient to date. Mitral valve status after operation was assessed as no incompetence in 13 patients (28%), minimal in 19 patients (40%), mild in 12 patients (26%), and moderate in 3 patients (6%). CONCLUSION The repair of complete atrioventricular septal defect by direct suturing of the atrioventricular valve leaflets to the crest of the ventricular septum with a single-patch technique greatly simplifies the repair and does not lead to left ventricular outflow tract obstruction nor interfere with valve function.

Three-dimensional quantitative echocardiographic assessment of ventricular volume in healthy human fetuses and in fetuses with congenital heart disease.

The purpose of this study was to evaluate the feasibility of three‐dimensional freehand echocardiographic assessment of ventricular volumetry in healthy fetuses and in fetuses with congenital heart disease. The study was approved by the hospital institutional review board. After echocardiographic examination by conventional ultrasonographic equipment interfaced with a magnetic tracking system, three‐dimensional cardiac data were collected prospectively in 57 fetuses. Ventricular volumes were determined from three‐dimensional data sets, and 22 fetuses with congenital heart disease were compared with 29 healthy fetuses. A multiple regression analysis of covariance was performed to assess between‐group differences. Gated three‐dimensional volume data sets enabled assessment of ventricular volumes in 51 of the 57 fetuses. Both fetuses with and without congenital heart disease had exponential increases in cardiac volumes during gestation. In fetuses with congenital heart disease and a marked inequality of ventricular size but no heart failure, the combined end‐diastolic and stroke volumes of both ventricles were found to be significantly reduced compared with controls with no disease and fetuses with other types of congenital heart disease. Three‐dimensional imaging can provide estimates of ventricular volume changes in fetal hearts with abnormal ventricular morphology that cannot easily be performed by two‐dimensional echocardiography, and it may provide insight into evolving congenital heart disease.

Outcomes following surgery for congenital heart disease in low-birthweight infants

Aim:  To describe cardiac surgery, survival and outcomes for low‐birthweight (≤2500 g) infants undergoing surgery for congenital heart disease.

Balloon dilatation of aortic stenosis in infants younger than 6 months of age: Intermediate outcome

The objectives of this study were to review the outcome of balloon dilatation of critical/severe aortic valve stenosis in patients younger than 6 months of age, with particular emphasis on subdivisions of age at intervention, and to identify factors that might influence outcome. From 1988 to 1998, 42 patients underwent dilatation. Patients were divided into three groups (group 1: 1–7 days, n = 16; group 2: 8–30 days; n = 10; group 3: 1–6 months, n = 16). Medical records and echocardiograms were reviewed retrospectively for presentation, clinical course, and left ventricular, aortic valve, and Doppler flow parameters. Median follow-up was 53 months (range, 6 months to 10 years). Of 16 group 1 patients, 11 (70%) had, respiratory distress requiring ventilator support, 12 (80%) received prostaglandin, and 5 (30%) received inotropic support. Nine (56.2%) patients died and 7 (44%) required reintervention. Of 10 group 2 patients, 4 (40%) were ventilated, 2 (20%) received prostaglandin, and 3 (30%) received inotropic support. Three (30%) patients died and 5 (50%) required reintervention. Of 16 group 3 patients, only 1 had symptoms (respiratory distress) at presentation. One (6%) patient died and 4 (15%) required reintervention. The overall actuarial survival rate at 10 years was 72% (88% at 10 years for indexed aortic annulus > 25 mm/m2. Freedom from reintervention was 70% and 21% at 5 and 10 years, respectively (80 and 33% at 5 and 10 years, respectively, for indexed aortic annulus > 25 mm/m2). The actuarial survival rates at 10 years for groups 1, 2, and 3 were 42%, 65%, and 93%, respectively. Predictors of death included young age at presentation, and multivariate analysis of left heart measures yielded an 83% positive prediction of outcome. An improved chance of survival was associated with indexed aortic valve annulus > 25 mm/m2. Patients with critical aortic stenosis who require balloon dilatation within the first month of life, but especially within the first week, have a poorer outcome than those requiring the procedure later, and this can be accounted for by a tendency toward less favorable anatomical features. Many will require repeat intervention.

Pulmonary haemorrhage and cardiac dysfunction in a neonate with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

We report on a favourable case of MCAD deficiency (homozygous 985A>G) that presented as lethargy, poor feeding, pulmonary haemorrhage and cardiac arrest without hypoglycaemia. The cessation of intralipid and the commencement of carnitine supplementation were associated with a rapid clinical improvement.

Parents' perceptions of genetics services for congenital heart disease: the role of demographic, clinical, and psychological factors in determining service attendance

Purpose:We sought to identify the demographic, clinical, and psychological factors associated with parents’ attendance at clinical genetics services for congenital heart disease.Methods:A survey assessing access to cardiac genetics services and a range of other variables was sent to the families of 213 children diagnosed with congenital heart disease between the years 2000 and 2009 at the Sydney Children’s Hospital, Australia.Results:Of the 114 respondents, 22% had accessed cardiac genetics services. Variables strongly associated with service attendance included presence of a syndrome associated with congenital heart disease (odds ratio = 17.93; P < 0.001) and antenatal diagnosis of congenital heart disease (odds ratio = 4.13; P = 0.02). Most participants (87%) perceived genetic factors as “quite” or “extremely important” in the development of congenital heart disease, and many (73%) believed that receiving information about congenital heart disease and genetics was “quite” or “extremely important”; however, only 36% of participants could recall receiving information of this nature. Forty-two percent of parents reported current concerns about their child’s health, and a substantial subset reported levels of depression (26%), anxiety (27%), and stress (32%) warranting clinical attention.Conclusion:There is a strong desire among parents of children with congenital heart disease for greater information about the role of genetic factors; however, most families do not access cardiac genetics services and report limited recall of information gathered from other sources.Genet Med 16 6, 460–468.

Use of sildenafil and nitric oxide in the management of hypoxemia owing to pulmonary arteriovenous fistulas after total cavopulmonary connection

Use of sildenafil and nitric oxide in the management of hypoxemia owing to pulmonary arteriovenous fistulas after total cavopulmonary connection Sameer Bhate, MCh DNB(CVTS), Michael Rossiter-Thornton, BMedSc(Hons), Stephen G. Cooper, MB, ChB, FRACP, Jonathan Gillis, PhD, FRACP, Andrew D. Cole, BAppSci(Hons), Gary S. Sholler, MBBS, FRACP, Richard B. Chard, BDS, MBBS, FRACS, and David S. Winlaw, MD, FRACS, Sydney, Australia

Hypoplastic left heart syndrome in context

Abstract:  Hypoplastic left heart syndrome is a rare condition requiring major cardiac surgery during the neonatal period to sustain life, with subsequent procedures culminating in completion of the Fontan circulation – the common pathway for all ‘single ventricle’ conditions. Algorithms for care of these children are now well defined with predictable medium‐term outcomes with the majority achieving a Fontan circulation. Hypoplastic left heart syndrome is one of a group of conditions that require complex surgery as a neonate and require a similar perioperative approach. Antenatal diagnosis is common in this patient subgroup, and there is a significant body of work that can be drawn on to inform parental choice.

Successful radiofrequency ablation in an infant with drug-resistant permanent junctional reciprocating tachycardia

Over the past decade, the technique of radiofrequency ablation has evolved substantially. Currently, most forms of cardiac arrhythmias seen in children can be treated with good long-term results and low risk of adverse outcome. Curative arrhythmia treatment with this technique, however, is still uncommon in neonates and infants. Reported here is our experience in the management of an 8-week-old with drug-resistant permanent junctional reciprocating tachycardia.

An Unusual Combination of Truncus Arteriosus, Interrupted Aortic Arch, and Hypoplastic Left Ventricle

Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.