Screening for congenital heart disease in a Singapore neonatal unit

INTRODUCTION@#Congenital heart disease (CHD) is a leading cause of infant mortality. The aim of this study was to evaluate the efficacy of a neonatal screening programme for CHD before the introduction of pulse oximetry.@*METHODS@#This was a retrospective review of live births in the period 2003-2012. Cases of CHD were detected through prenatal ultrasonography and/or postnatal examination, and confirmed using two-dimensional echocardiography. Data was rigorously checked against multiple sources. The antenatal detection rate, sensitivity, specificity, predictive values and likelihood ratios of the screening programme were analysed for all cases of CHD and critical CHD.@*RESULTS@#The incidence of CHD was 9.7 per 1,000 live births. The commonest CHD was ventricular septal defect (54.8%). The antenatal detection rate was three times higher in the critical CHD group (64.0%) compared to the group as a whole (21.1%). The sensitivity and specificity of screening was 64.5% and 99.7% for all CHD, and 92.9% and 99.1% for the critical CHD group, respectively. The positive likelihood ratio was 215 and 103, while the negative likelihood ratio was 0.36 and 0.07 for all CHD and critical CHD, respectively.@*CONCLUSION@#The CHD screening programme had excellent specificity but limited sensitivity. The high positive likelihood ratios indicate that where sufficient risk factors for CHD are present, a positive result effectively confirms the presence of CHD. The low negative likelihood ratio for critical CHD indicates that, where prior suspicion for critical CHD is low, a negative result is reassuring.

Neonatal and Paediatric Extracorporeal Membrane Oxygenation (ECMO) in a Single Asian Tertiary Centre

<p><b>INTRODUCTION</b>Extracorporeal membrane oxygenation (ECMO) is a cardiopulmonary bypass technique (CPB) which provides life-saving support in patients with refractory cardiorespiratory failure until cardiopulmonary recovery or organ replacement.</p><p><b>MATERIALS AND METHODS</b>This is a single centre retrospective study reporting the largest series of paediatric patients in Singapore who received ECMO support over an 11-year period from January 2002 to December 2012. The objective is to describe the characteristics of the patients and to report the survival to hospital discharge, complications during ECMO and other long-term complications.</p><p><b>RESULTS</b>Forty-eight patients received ECMO during the study period. ECMO was initiated for myocarditis in majority of the paediatric patients whereas postoperative low cardiac output state was the most common indication in the neonatal population. The overall survival rate to hospital discharge was 45.8%. Survival was highest in the neonates with respiratory failure (75%). Haematological and cardiac complications were most common during ECMO. Age group, gender, duration of ECMO, need for renal replacement therapy, acute neurological complications were not associated with mortality. Those needing inotropic support during ECMO had poorer survival while those with hypertension requiring vasodilator treatment had a higher survival rate. The survival rates for ECMO patients more than doubled from the initial 6 years of 23% to 54% in the last 5 years of the study period. Long-term complications encountered included neurological, respiratory and cardiac problems.</p><p><b>CONCLUSION</b>ECMO is a life-saving modality for neonatal and paediatric patients with cardiopulmonary failure from diverse causes. Patients with persistent need for inotropes during ECMO had poorer outcome. Centre experience had an impact on ECMO outcome.</p>

Late complications following tetralogy of Fallot repair: the need for long-term follow-up

<p><b>INTRODUCTION</b>We report a series of operated tetralogy of Fallot (TOF) patients focusing on complications and outcomes.</p><p><b>MATERIALS AND METHODS</b>Data from TOF patients seen at our centre's adult congenital heart disease clinic was analysed.</p><p><b>RESULTS</b>There were 21 patients: the mean age was 32.2 +/- 12.4 years; the age at first operation was 9.0 +/- 7.9 years; the mean postoperative follow-up duration was 23.5 +/- 12.1 years; and the current New York Heart Association (NYHA) status: I, 82%; II, 4%; III, 14%. Fourteen patients had complete operative notes. All these patients underwent total TOF correction; 2 had staged aortopulmonary shunt with total correction at a mean of 3.2 years later, pulmonary artery patch augmentation in 8 patients and pulmonary valvotomy in 8 patients. Three patients required pulmonary valve homograft replacement for severe pulmonary regurgitation (PR) at 13, 28 and 36 years after the initial corrective operation.</p><p><b>CURRENT INVESTIGATIONS</b>RBBB on ECG (91%), QRS duration 137 +/- 29 ms. Echocardiography showed dilated right ventricular end-diastolic (RVED) diameters (3.2 +/- 0.8 cm); severe PR (67%), residual right ventricular outflow tract obstruction (RVOTO) (42%) and VSD patch leakage (9%). Cardiac magnetic resonance (CMR) (8 patients) showed dilated RVED volumes 252.6 +/- 93.8 mL, indexed RV volume 165.7 +/- 34.8 mL; RV systolic function was preserved in most patients with a RV ejection fraction of 49.5 +/- 5.7%. One patient had atrial tachycardia and another had frequent non-sustained ventricular tachycardia that required radiofrequency ablation.</p><p><b>CONCLUSION</b>Patients with TOF who had full corrective surgery during childhood are now surviving into adulthood. Many challenges arising from complications in the postoperative period remain. It is imperative that adult TOF patients should have regular followup to monitor development and subsequent management of these complications.</p>