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A. Verner1, A. Sands1, J. S. Craig1, J. Jenkins2. 1Royal Group of Hospitals Trust, Belfast, UK; 2Queen's University, Belfast, UK
BackgroundThe association between long QT interval and sudden death was first reported by Jervill and Lange‐Nielson in 1957. There has been extensive research in the adult population further examining this relation, both in disease state and in health. There are few studies in healthy children, the largest study to date has been that by Schwartz et al. The normal range of QT interval in preterm infants has never been studied. This is part of a study examining ventricular repolarisation in preterm infants.
MethodsInfants were recruited if they were under 36 weeks' gestation, with parental consent, with no family history of long QT syndrome or a major congenital cardiac defect. All had a screening 12 lead ECG prior to starting 24‐h ECG recording.
ResultsEighty two infants, gestational age 25–35 weeks were recruited. 32% of these infants had either complete flattening or very low amplitude T waves (<0.1 mV) on 12 lead ECG in the first 3 days of life. The end of the T wave could be determined in the majority, allowing the QTc to be calculated in 88%. The mean QTc was 0.41 ms (SD 0.039 ms). The QTc and T wave amplitude were the maximum values recorded in leads II, V5 or V6. Other ECG parameters were similar to previously published values for preterm infants, which differ from those of term infants. For those infants who had repeat ECG or 24‐h ECG recording performed at 2 weeks of age, T waves were monophasic and normal amplitude. The presence of low amplitude T wave is not related to gestation or level of intensive care required but is positively correlated to heart rate.
ConclusionT wave amplitude reflects the sympathetic effects on the heart, with lowering of amplitude with increased sympathetic tone. Although this phenomenon is reported in the adult literature, there are few reports in newborns, and none in preterm infants who are not requiring significant intensive care. Animal studies have demonstrated dominance of sympathetic tone in fetal life, with parasympathetic tone exerting little effect until term gestation. The ECG findings in this group of infants may be a reflection of similar altered autonomic tone dynamics which changes with postnatal maturity.
A. Gupta, C. Wren. Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, UK
IntroductionRecognition of cardiovascular malformations (CVM) in asymptomatic neonates is difficult. Many potentially dangerous malformations will have no murmur but babies with murmurs require assessment. The aim of this study was to assess the contribution of a rapid access murmur clinic to the diagnosis of life threatening CVM in neonates and to evaluate findings in babies referred for assessment of murmurs.
MethodsThis was a prospective study of patients referred to a rapid access murmur clinic from 4 neighbouring hospitals to the regional paediatric cardiology centre in January 2002–December 2005. We also identified babies born in same 4 hospitals during the same period referred through other channels (eg antenatal diagnosis, emergency admission, urgent referrals, Down's syndrome). CVM were classified as either life threatening, significant (not life threatening but requiring surgery) or minor.
ResultsIn 4 years 40615 babies were born in the 4 hospitals, accounting for 33% of our referral population. 388 had CVM (9.6 per 1000 live births). 587 neonates were referred to the murmur clinic and were seen by a paediatric cardiologist at a mean age of 25 days (range 1–90). 411 (70%) infants were normal. None had a life threatening CVM, 11 (2%) had a significant CVM (2 tetralogy of Fallot, 1 complete AVSD, 1 partial AVSD, 3 VSD, 1 AS, 1 PS and 2 ASD) and 127 (22%) babies had a minor CVM (small VSD, mild PS, mild AS). 20 had PDA and 18 had ASD (all closed spontaneously). In the same birth cohort 248 babies with CVM were referred through other channels—64 with life threatening, 78 with significant and 106 with minor CVM. Therefore, the murmur clinic led to diagnosis of 0% of life threatening CVM, 12% of significant CVM and 56% of minor CVM. The referral rate varied between the 4 hospitals from 2–45 per 1000 live births. Despite this the percentage of babies with CVM was similar, ranging from 28–43%.
ConclusionThe rapid access murmur clinic does not contribute to the identification of life threatening CVM. The referral rate varied considerably but a higher referral rate did not lead to the recognition of more CVM. Better patient selection in local hospitals could reduce the workload of the regional paediatric cardiology centre.
S. Riggs1, M. Berger1, S. Gosling1, M. Puckey2, J. S. Carvalho2. 1Royal Holloway, University of London, London, UK; 2Royal Brompton Hospital, London, UK
AimTo determine whether prenatal diagnosis impacts on family adjustment to children with congenital heart disease (CHD) and whether diagnosis of CHD impacts on family psychological functioning on medium‐term follow‐up.
MethodsProspective, four group comparative study. Interviews with parents were carried out at family home or tertiary hospital. 131 families with 2–4‐year‐old children were invited to participate. Four groups of families were studied, two in whom children were diagnosed either pre‐ or postnatally with CHD and two control groups without CHD, one who underwent fetal echocardiography (FE) due to high‐risk of CHD and one who did not (low‐risk). Groups with CHD were matched for severity of condition. 43 families (24 mother‐father dyads, 18 mothers and 1 father) completed standard measures of parental coping (Parenting Daily Hassles Scale, PDH), parental psychological distress (General Health Questionnaire‐28, GHQ) and child adjustment (Child Behaviour Checklist, CBCL), and a specifically designed child‐development screening tool.
ResultsNo significant differences were found between pre‐ and postnatally diagnosed groups in child‐development, PDH and GHQ scores or CBCL scores reported by mothers. No significant differences were found between groups with CHD and those without in child development, PDH and GHQ scores or CBCL scores reported by mothers.
ConclusionsPrenatal diagnosis of CHD does not appear to have either detrimental or beneficial effects on the coping and adjustment of 2–4‐year‐old children and their families. Additionally, diagnosis of CHD does not appear to adversely affect the coping and adjustment of 2–4‐year‐old children and their families when compared with healthy controls. It is likely that problems with coping and adjustment would be apparent for families of younger children, in particular perinatally. However our findings perhaps reflect that the care and support provided to families contributes to a degree of adaptation.
D. Kenny, S. Chakrabarti, A. Ranasinghe, D. Grant, R. Martin. Bristol Royal Hospital for Children, Bristol, UK
BackgroundDespite improving screening programmes for congenital heart disease, haemodynamic collapse from congenital and acquired heart disease in infancy remains a major clinical problem. Defining this subgroup of patients may identify possible means of earlier detection and treatment, and improve outcomes.
AimsTo outline the aetiology, clinical course, and outcome of infants with new cardiac diagnoses presenting to PICU with acute cardiovascular collapse.
MethodsRetrospective search of a computerised database and medical case notes for all acute cardiac admissions to PICU from June 2001 to June 2006. Pre‐existing hospital‐based patients were excluded.
ResultsSixty two patients, 34 (55%) of whom were male were identified. There were six main subgroups: obstructive left heart lesions (n=20), transposition of the great arteries (n=9), total anomalous pulmonary venous drainage (n=7), dilated cardiomyopathy (n=9), arrhythmia (n=10), and others (n=7). The median age at presentation was 12.5 days (0–270) with a median duration of symptoms of 1 day (0–61). Eight patients underwent medical review and were discharged prior to subsequent presentation. The median base excess at presentation was −10.1 mEq/l (−43 to +4.2). 47 patients (76%) required intubation and ventilation. The median PICU stay was 6 days (1–49) with a median total hospital stay of 16 days (1–63). Six (10%) patients died before discharge. Of the survivors 3 (5%) had clinical seizures with 2 more having significant EEG changes. Out of 5 patients born at home, 2 patients died, however this was not an independent risk factor for death (p=0.11).
ConclusionsCardiovascular collapse from previously unrecognised congenital or acquired heart disease is associated with significant mortality and morbidity. Improved recognition of congenital heart lesions on routine screening and greater awareness of arrhythmia and cardiomyopathy as potential problems in this age group are potential strategies to improve earlier detection and prompt referral. Longer‐term follow‐up is required to evaluate the initial effect of poor cardiac output on long‐term neurological development.
R. Adiga1, K. Brown2, D. Ridout3, W. Kelsall1. 1Cambridge University Hospital NHS Trust, Cambridge, UK; 2Great Ormond Street Hospital, London, UK; 3Institute of Child Health, London, UK
BackgroundCongenital heart disease (CHD) may be first diagnosed prenatally, postnatally or at postmortem. Six subtypes of CHD have been described. The aim of this study was to review the initial clinical findings in a group of infants diagnosed with CHD before birth (“known”) and compare their spectrum of disease with those who presented after discharge from the maternity unit (“missed”).
MethodsA retrospective case note review was conducted for neonates born with “known” CHD between 1994 and 2004 in centre A and “missed” CHD requiring cardiac intensive care in centre B between 1998 and 2002.
ResultsSeventy neonates with “known” CHD had a median birth weight of 3065 (983–4249) grams and gestational age of 39 (30–42) weeks. The initial clinical examination after birth was abnormal in 61 (88%) neonates: heart murmur in 42 (61%), cyanosis in 20 (30%), tachypnoea in 18 (26%), weak femoral pulses in 2 (3%), hepatomegaly in 1 (1%). 13 (19%) had additional extra‐cardiac anomalies. Of the 9 neonates with a normal first examination, 4 had systemic ventricular outflow obstruction (SVOO), 2 had low pulmonary blood flow and 3 had left to right shunts. When pulse oximetry was also performed only 4 (6%) of the “known” neonates had a normal clinical examination and normal saturations (>95%). Two of these infants had SVOO. The 65 neonates with “missed” CHD presented at a mean of 14 (SD 8) days, median weight 3175 (2000–4700) grams. The commonest type of defect in “missed” patients was SVOO in 38 (58%), compared to 19% of the antenatal diagnosis group (p<0.01).
ConclusionSVOO was the most common abnormality in the missed group and 31% of neonates known to have this abnormality had a normal newborn examination. Pulse oximetry does add to the clinical examination but may not allow the identification of all cases of CHD before hospital discharge.
Health Technology Report on Neonatal CHD published in 2005
S. Chakrabarti, R. Tulloh, R. Martin. Bristol Congenital Heart Centre, Bristol, UK
BackgroundPulmonary vein stenosis (PVS) of normally connected pulmonary veins is a rare condition in paediatric population, associated with extremely poor prognosis. The presenting symptoms and signs are non‐specific and similar to those of chronic lung disease.
AimTo identify possible aetiology, progress, treatment options and outcome children with acquired PVS of normally connected pulmonary veins.
Patients and MethodsRetrospective observational study in single tertiary cardiac referral centre.
ResultsSeven infants were identified with acquired PVS from August 2000 to August 2006. Four of them were born at 28 weeks of gestation. All of them were males. Prolonged ventilation was needed in all patients. Indications for ventilation were respiratory distress syndrome followed by chronic lung disease (n=4), bronchiolitis (n=1), pulmonary mesenchymal dysplasia (n=1) and post cardiac surgery (n=1). All infants (n=7) had cardiac lesions with no evidence of pulmonary vein stenosis on initial assessment by the paediatric cardiology team. Cardiac lesions were patent arterial duct (n=5), ventricular septal defect (n=2) and atrial septal defect (n=3). Multiple cardiac lesions were present in 3 infants. Five patients had interventions (surgical/catheter based) for the cardiac lesions. All these patients had been discharged from hospital and had developed/persisted to have respiratory symptoms. PVS was acquired and diagnosed in all the patients over a period of median 7 months (range 4–12 months) after birth. Four patients had interventions for PVS (surgery in 2 patients, balloon angioplasty in 2 patients (1 patient had cutting balloon angioplasty twice), stent angioplasty in 1 patient who also had surgery). The infants who did not receive any intervention (n=3) died before their first birthday. The four other children are alive (age range 14–62 months) with significant morbidity associated with residual pulmonary hypertension.
ConclusionsAcquired PVS is a rare but increasingly recognised, progressively worsening condition with significant morbidity and mortality. The aetiology is multifactorial including male sex, prematurity, lung inflammation and increased pulmonary blood flow from intracardiac shunt. The progression of the disease may be modified by surgical or catheter based interventions, but overall prognosis remains poor.
A. M. Bhoyar, A. Chikermane, J. Stickley, J. G. C. W. Wright, O. Stumper, P. Miller, R. Dhillon, J. DeGiovanni. Birmingham Children's Hospital, Birmingham, UK
BackgroundEchocardiographic and angiographic data indicate that 20% to 40% of untreated Kawasaki's disease (KD) patients develop coronary artery abnormalities. The data are obscure in UK in the current era of high suspicion and early treatment.
AimsTo study the trends coronary involvement in patients with coronary involvement who received treatment according to current protocol.
MethodsWe retrospectively reviewed patient records on our database with the diagnostic code of KD from August 2001 to March 2006, selecting those patients who had at least one year of follow‐up from the time of the onset of the disease. These children were considered to have KD by referring paediatricians and treated according to current protocol.
ResultsWe had a total of 170 patients newly diagnosed with KD in this period. There were 102 (60%) were male, and the age of presentation ranged from 2 months to 12 years (median age 4 years). Three children had atypical KD (median age 13 months), 42 children had suspected KD (median age 4 years), and the rest were confirmed based on the classical pattern of the disease with a median age of 4 years. Only 1 child had an abnormal ECG. 17 (10%) children had cardiac involvement—16 children had the classical form while 1 child with atypical form of KD had a cardiac involvement; in our series the median age for those children who developed cardiac involvement was 20 months. No child with suspected KD had coronary artery involvement. 11 had a mild lesion, 4 had moderate lesions with 1 child having severe dilatation. One child did not have a coronary artery lesion, but had a small, transient pericardial effusion. 12/17 patients with cardiac pathology had a complete resolution, 75% of them in a year or less.
ConclusionOur echocardiographic data showed that 10% of the treated patients had coronary involvement. Severe involvement is rare. None of the children with normal echocardiogram during acute illness developed abnormality on subsequent examinations. Electrocardiogram had a low sensitivity in picking up cardiac pathology in KD. This will serve as valuable information for counselling parents during the initial presentation.
A. M. Bhoyar, A. Chikermane, J. Stickley, J. DeGiovanni, J. G. C. W. Wright, O. Stumper, R. Dhillon, P. Miller. Birmingham Children's Hospital, Birmingham, UK
AimsTo look at the patterns of cardiac involvement in patients with Kawasaki's disease (KD).
MethodsWe retrospective reviewed patient records on our database with the diagnostic code of KD between 2001 and 2006, selecting those with cardiac involvement following the disease. We accepted patients with diagnosis of KD made by the referring paediatricians. These children underwent cross sectional cardiac ultrasound during the acute phase, and were subsequently followed up. All children were followed up for at least one year following their presentation.
ResultsWe found 41 patients with KD who had cardiac involvement; there was no mortality in our series. 24 (58.5%) were males, and the age range of 2 months to 8.6 years (median age 3 years) at the time of onset of the disease. Only one patient had an atypical KD. All these patients were diagnosed to have a cardiac abnormality on cardiac ultrasound, and then followed up using other modalities of investigation. Only one child had an abnormal ECG. The majority had coronary artery dilatations (38/41), with pericardial effusion and mitral regurgitation seen in 4. All except two children had a normal cardiac function and no wall motion abnormalities at rest. Left ventricular dysfunction was seen in 1, and 1 child had antero‐lateral infarction. One patient was lost to follow‐up, and complete resolution was seen in 29 (72.5%). Of those who had coronary artery involvement, in 11 the degree of dilatation was not mentioned, 17 had mild dilatations; 6 had a moderate form while 4 were found to have to have severe dilatation. Single coronary was involved in 28, 9 had two coronaries affected while 1 had all three major coronary artery involvement.
ConclusionBased on the current treatment protocols, there is a successful resolution of cardiac abnormalities in majority of these children with a very small risk of significant cardiac dysfunction. Whilst coronary involvement is a significant complication, the overall outlook is good.
A. Dhelaria1, M. Kuruvilla2, W. Kelsall2. 1Luton & Dunstable Hospital, Luton, UK; 2Addenbrooke's Hospital, Cambridge, UK
IntroductionPatent ductus arteriosus (PDA) can be an important problem in premature infants and there remains debate over management. A small number of infants require PDA ligation when medical treatment has failed or is contra‐indicated. A retrospective study in 3 centres reported good 30 day survival post ligation with substantial late mortality and a high incidence of morbidity in the survivors. The study was limited by problems with data collection. The aim of this prospective study was to review the management and outcome of neonates undergoing PDA ligation from a single region of the UK.
MethodsPatients were identified from the Acute Neonatal Transport Service database who moved infants to cardiothoracic centres performing the procedure. The study started in December 2004 and is ongoing.
ResultsOver a 19‐month period up to June 2006, 21 infants from the region have required PDA ligation. Their birthweight median (range) was 732 (462–1572) g, gestational age 24 (23–31) weeks. 20 (95%) infants were treated with prostaglandin synthetase inhibitors (PSI) before surgery, 18 received Indomethacin and 2 Ibuprofen alone. 10 (50%) babies received 2 or more courses of PSI. PDA ligation was carried out in one of 5 centres at a weight of 1195 (757–2770) g and age of 46 (26–91) days. The procedure was performed as a daycase for 2 babies. Following the decision to undertake PDA ligation, paediatricians contacted up to 6 cardiothoracic units to arrange admission, the interval between decision to ligate and surgery was 13 (3–39) days. All babies survived for 30 days and 1 baby died 65 days after surgery. Surgical complications occurred in 24% of cases: pneumothoax (2); recurrent laryngeal nerve palsy (2) and Chylothorax (1). The incidence of chronic lung disease, intraventricular haemorrhage, necrotising enterocolitis and retinopathy of prematurity were 95%, 38%, 24% and 20% respectively.
ConclusionsShort and long term survival after PDA ligation in this cohort has improved. There is significant longer term morbidity. There are long delays in arranging PDA ligation which may influence outcome.
LCLee et al Outcome following patent ductus arteriosus ligation in premature infants: a retrospective cohort analysis. BMC Pediatr200611 pp 15
S. Sivakumar1, L. Lee1, A. Tillett1, F. Wells2, J. Dunning2, A. W. Kelsall1. 1Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 2Papworth Hospital NHS Trust, Cambridge, UK
IntroductionPatent ductus arteriosus (PDA) ligation may be required in a small number of neonates who do not respond to medical therapy or where drug treatment is contra‐indicated. Arranging PDA ligation can be difficult. There are many potential advantages of a surgical team travelling to the baby to perform the procedure rather than the baby travelling to the surgeon. The aim of this study was to review the outcome of PDA ligation performed outside a paediatric cardiothoracic centre by a trained surgical team.
MethodsA retrospective observational study of all neonatal PDA ligations performed in a non‐cardiac centre between January 1988 and December 2002.
ResultsForty three infants underwent PDA ligation over 15‐year period. The median (range) gestational age at birth was 26 (23–35) weeks and birth weight was 722 (500–2100) g. Age at ligation was 25 (10–89) days and weight 963 (568–2221) g. Successful ligation was achieved in 42 babies (98%). 30 day postoperative mortality was 5%. 29 babies (67%) survived to discharge from the hospital. The deaths occurred at a median of 80 (4–194) days after surgery and were due to complications of prematurity rather than the ligation procedure.
ConclusionPDA ligation can be successfully performed by an outreach surgical team. This minimises potential delays in scheduling surgery, avoids the transport of critically ill neonates to distant paediatric cardiothoracic centres and achieves optimal continuity of care. Following the Bristol Inquiry and centralisation of paediatric cardiac surgery, this outreach service despite its excellent results was discontinued. While it may no longer be appropriate for a trained adult cardiothoracic or general paediatric surgeon to perform PDA ligation, it may be in the infants' best interest for an experienced paediatric cardiothoracic surgeon to offer an outreach ligation service in centres with expertise in the perioperative management of neonates. In view of the number of late deaths, long‐term follow‐up of this high risk population is also recommended.