James P. Gnanapragasam

Outcome after repair of tetralogy of Fallot in the first year of life

BACKGROUND The purpose of this study was to evaluate the early and late outcome after repair of tetralogy of Fallot in the first year of life. METHODS Between 1974 and 2000, 89 consecutive infants with a mean age of 6.3 +/- 2.6 months (range, 15 days to 12 months) underwent repair of tetralogy of Fallot (ventricular septal defect and pulmonary stenosis) by one surgeon (J.L.M.). Three infants had previous palliative operations. Sixty-seven procedures were urgent or emergency. A transannular patch was inserted in 69 patients (77.5%). Follow-up was complete, averaging 13.4 +/- 5.6 years (range, 0 to 25.4 years). RESULTS There was one operative death (1.1%). Mean right ventricular to left ventricular pressure ratio postoperatively was 0.4 +/- 1.1 (in 79 patients, < 0.5). Fourteen patients underwent reoperations or reinterventions. There were no reoperations for residual or recurrent ventricular septal defect. Kaplan-Meier freedom from reoperation or reintervention for any cause at 20 years was 85% +/- 4.4%, for relief of right ventricular outflow tract obstruction it was 94% +/- 3.1%, and for pulmonary valve replacement this was 95.4% +/- 2.6%. Use of a transannular patch did not significantly affect the need for reoperation or reintervention. There was one late death (leukemia). Kaplan-Meier 20-year survival was 97.8% +/- 1.9%. On latest echocardiography, 42 patients had moderate pulmonary regurgitation, 4 had a right ventricular outflow tract gradient more than 40 mm Hg, and 86 had good biventricular function. Twelve-lead electrocardiography was performed in all and 24-hour electrocardiography in 61 patients. One patient (1.1%) exhibited late recurrent ventricular tachycardia requiring implantation of a defibrillator. The remaining 86 patients are in New York Heart Association class I with none of them receiving antiarrhythmic medications. CONCLUSIONS These data strongly support the concept of early repair of tetralogy of Fallot. It is associated with an acceptable operative risk and a low incidence of significant arrhythmias, and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and long-term follow-up for their early recognition is essential.

Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach

OBJECTIVE The optimal time and approach of repair of tetralogy of Fallot (TOF) remain controversial. The purpose of this study was to evaluate the outcome following repair of TOF in infants with particular regard to the surgical approach used. PATIENTS One hundred and sixty infants (mean age 195+/-89 days, range 11-364 days) undergoing repair of a simple TOF were studied. Between 1974 and 2000, a transventricular approach (RV) was used in 91 and between 1988 and 2000, a transatrial (RA) approach in 69 infants. Ten of these infants (6.2%) had a previous palliative shunt (four in the RV versus six in the RA group). A transannular patch (TAP) was inserted in 96 (60%) infants (76 versus 20). Follow-up was complete (mean 14.5+/-5.2 versus 6+/-1 years). RESULTS There were three operative deaths (1.9%), (two in RV versus one in RA group). A re-operation for right ventricular outflow tract obstruction (RVOTO) was performed in 19 patients (3 versus 16). Ten-year freedom from re-operation for RVOTO (+/-standard error of the mean) was 88+/-4% (98+/-2 versus 72+/-6%, P<0.0001). Within the RA group, 5-year freedom from re-operation for RVOTO for those who had a TAP was 79+/-9% and it was 75+/-4% for those having a simple repair. Six patients in the RV group required pulmonary valve replacement (PVR). Ten-year freedom from PVR was 98+/-1% (97+/-2 versus 100%, P=0.3). There were two late deaths, one in each group. Ten-year survival was 97+/-1%. One patient in the RV group developed late recurrent ventricular tachycardia requiring the implantation of a defibrillator. At most recent echocardiography, all but the patient who had the defibrillator had good right and left ventricular function. CONCLUSIONS Transventricular and transatrial repair of TOF in infancy, are associated with an acceptable operative risk, low incidence of late arrhythmia, good bi-ventricular function and excellent survival. In our experience, however, transatrial repair has a disturbing incidence of early and mid-term residual or recurrent RVOTO, even when a TAP has been used.

Implantable cardioverter defibrillator therapy in paediatric practice: a single-centre UK experience with focus on subcutaneous defibrillation

AIMS Sudden cardiac death (SCD) risk can be managed by implantable cardioverter defibrillators (ICD). Defibrillation shocks can be delivered via ICD generator and/or intracardiac or subcutaneous coil configurations. We present our single-centre use of childhood ICDs. METHODS AND RESULTS Twenty-three patients had ICD implantation, with median age and weight of 12.96 years and 41.35 kg. Indications included eight long QT; four hypertrophic cardiomyopathy; three Brugada syndrome; two idiopathic ventricular fibrillation; two post-congenital heart repair; two family history of SCD with abnormal repolarization; one catecholaminergic polymorphic ventricular tachycardia; and one left ventricle non-compaction. Twelve had out of hospital cardiac arrests prior to implantation. Techniques included 13 conventional ICD implants (pre-pectoral device with endocardial leads), 7 with subcutaneous defibrillation coils (sensing via epicardial or endocardial leads tunnelled to the ICD), and 3 with exclusive subcutaneous ICD (sensing and defibrillation via the same subcutaneous lead). Satisfactory defibrillation efficacy and ventricular arrhythmia sensing was confirmed at implantation. Follow-up ranged from 0.17 to 11.08 years. One child died with the ICD in situ. Ten children received appropriate shocks; five on more than one occasion. Five received inappropriate shocks (for inappropriate recognition of sinus tachycardia or supraventricular tachycardia). Five children underwent six further interventions; all had intracardiac leads. CONCLUSION Innovative shock delivery systems can be used in children requiring an ICD. The insertion technique and device used need to accommodate the age and weight of the child, and concomitant need for pacing therapy. We have demonstrated effective defibrillation with shocks delivered via configurations employing subcutaneous coils in children.

Acute myocardial infarction in the neonatal period.

We describe two neonates presenting with myocardial infarction, due to two different aetiologies of this extremely rare but potentially treatable condition, and discuss the management. One neonate had myocardial infarction complicating enteroviral myocarditis and recovered completely. The second had fatal myocardial infarction due to thrombosis of the left coronary artery. Although rare, the attending paediatrician should have a high index of suspicion when evaluating a neonate with acute onset of collapse. Electrocardiographic changes are diagnostic, but further echocardiographic assessment and prompt management at a tertiary cardiac centre are advised.

Quality of life after repair of tetralogy of Fallot

OBJECTIVE To determine the quality of life in individuals with corrected tetralogy of Fallot. METHODS AND SUBJECTS Questionnaires concerning quality of life were sent to all 87 surviving patients aged between 16 and 40 years who had undergone intracardiac repair of tetralogy of Fallot and follow-up in the Wessex Cardiothoracic Unit, and to 87 age and sex matched controls, with medically treated haemodynamically insignificant ventricular septal defects. RESULTS The only significant difference found between the cases and controls was in requirements for schooling, where those with tetralogy of Fallot were more likely to require additional educational help at school (p = 0.044). For all other aspects of quality of life examined by the questionnaire, including social and genetic history, exercise ability, and health related quality of life, no significant differences were found. Different operative techniques, such as transjunctional patching, right ventriculotomy, and previous palliative shunting, did not affect the quality of life of our population with Tetralogy of Fallot, on average twenty years after their surgery, although the range of operative techniques was limited. Neither age at surgery, nor time since surgery, was correlated with measurements of quality of life. CONCLUSIONS Those who have undergone surgical correction of tetralogy of Fallot have a normal quality of life, with few differences compared to controls.

Acquired stenosis of normally connected pulmonary veins.

Pulmonary venous stenosis has been a challenge to paediatric cardiologists and surgeons. In spite of significant improvements in the management and outcome of various congenital cardiac problems, the prognosis of this condition continues to be dismal. Acquired stenosis of the pulmonary veins has been described in the adult population, especially after radiofrequency ablation for atrial arrhythmias. The outcome of this condition has generally been described to be favourable in the long term. Acquisition of pulmonary venous stenosis in childhood has a very different outcome, and is not a very well recognised entity, with symptoms and signs which are non-specific. We present 7 infants who, when first assessed, had normal pulmonary veins, but who subsequently acquired stenoses of the veins, with very significant resultant morbidity and mortality.

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex‐premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

The Collapsing Teenager

In this chapter we describe a teenager with exertional collapse. He was found to have an aberrant left coronary artery arising from the right coronary sinus and running between the great arteries. Although this lesion is uncommon, there is a strong association with sudden cardiac death, particularly during or immediately after strenuous exercise. Diagnosis requires careful echocardiographic evaluation of the coronary arteries often supported by CT. We discuss the presentation, diagnosis and management of this lesion.

Short-term renal support in postoperative repair of tetralogy of Fallot in the paediatric intensive care unit: can we predict those who need it?

INTRODUCTION Fluid balance and renal function can be difficult to manage in the postoperative infant with tetralogy of Fallot. High fluid volumes are often needed to maintain cardiac output. Aims To stratify patients at risk for advanced renal support following tetralogy of Fallot repair. METHODS Retrospective analysis of all consecutive tetralogy of Fallot cases operated at a single centre in a 3-year period. RESULTS A total of 41 children were identified. All cases had loop diuretics administered. Of the cases, 17% required support with a peritoneal dialysis catheter, with only one complication of peritoneal dialysis catheter blockage. The mean length of paediatric intensive care unit stay in those receiving peritoneal dialysis catheter insertion was prolonged by an additional mean of 6 days (p<0.001). No statistical significance was found between those children requiring peritoneal dialysis and those who did not when considering patient age and weight at time of repair, cardiopulmonary bypass and aortic cross clamp times, the presence of a transannular patch, or junctional ectopic tachycardia. However, volume requirement of more than 35 ml/kg in the first 12 hours following repair did increase the likelihood to need peritoneal dialysis (p<0.0001). Furthermore, the higher the peak creatinine, the longer the stay on intensive care (p<0.01). CONCLUSIONS Peritoneal dialysis is an effective method of dealing with fluid balance in children after tetralogy of Fallot repair, with minimal complications. Early consideration should be given to peritoneal dialysis when it is clear that high fluid volumes are required postoperatively.