How to use chest radiographs and ECGs in children with pacemakers.

A child with pacemaker is an uncommon presentation to the general paediatric ward, and most clinicians without previous experience may not feel confident in assessing these patients. This article provides an overview of paediatric pacemakers and commonly found radiological and electrophysiological correlates along with clinical consideration.

Sore knee - why and when to call a cardiologist.

A young girl received a diagnosis of septic arthritis of the knee unresponsive to standard medical and surgical treatment. We report the patient's clinical journey with clinical commentary throughout, underlying the importance of differential diagnosis that may open several scenarios and a different final diagnosis accordingly. Finally, we will discuss the treatment and management of the patient's final diagnosis.

Exercise prescription improves exercise tolerance in young children with CHD: a randomised clinical trial.

OBJECTIVE: The main objective of this study was to ascertain if a structured intervention programme can improve the biophysical health of young children with congenital heart disease (CHD). The primary end point was an increase in measureable physical activity levels following the intervention. METHODS: Patients aged 5-10 years with CHD were identified and invited to participate. Participants completed a baseline biophysical assessment, including a formal exercise stress test and daily activity monitoring using an accelerometer. Following randomisation, the intervention group attended a 1 day education session and received an individual written exercise plan to be continued over the 4-month intervention period. The control group continued with their usual level of care. After 4 months, all participants were reassessed in the same manner as at baseline. RESULTS: One hundred and sixty-three participants (mean age 8.4 years) were recruited, 100 of whom were male (61.3%). At baseline, the majority of the children were active with good exercise tolerance. The cyanotic palliated subgroup participants, however, were found to have lower levels of daily activity and significantly limited peak exercise performance compared with the other subgroups. One hundred and fifty-two participants (93.2%) attended for reassessment. Following the intervention, there was a significant improvement in peak exercise capacity in the intervention group. There was also a trend towards increased daily activity levels. CONCLUSION: Overall physical activity levels are well preserved in the majority of young children with CHD. A structured intervention programme significantly increased peak exercise capacity and improved attitudes towards positive lifestyle changes.

Cardiac strangulation after epicardial pacing: the importance of non-specific symptoms and a low index of suspicion.

Cardiac strangulation is a rare and potentially deadly complication of epicardial pacemaker implantation. A young boy presenting with chest pain and tiredness almost 7 years after pacemaker implantation was found to have cardiac strangulation. Literature review revealed 22 cases reported to date with a worrying rise in the number of reports over the past 3 years. Strangulation is associated with implantation of leads at a young age and appears to be related to somatic growth. Serial assessment with chest X-ray and echocardiogram is recommended, at least until full adult growth is attained with further coronary artery imaging reserved for symptoms or suspicious echocardiographic findings. If cardiac strangulation is diagnosed prompt replacement of the offending system is needed.

32 year follow up of patients following atrial redirection surgery for transposition of the great arteries.

OBJECTIVE: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center. DESIGN: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic information and data regarding the clinical state, NHYA class, cardiopulmonary exercise testing, NT-proBNP measurement, and recent cardiac MRI findings. RESULTS: Forty-six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty-two patients (69.6%) had undergone a Senning procedure. The median length of the follow-up was 32 years. Thirty-two patients (69.6%) were NHYA class 1. The mean VO2max achieved was 24.2 ± 5.8 mL/min/kg. The mean NT-proBNP was 266.4 pg/mL (± 259.9 pg/mL). The mean right ventricular end-diastolic volume (RVEDV) was 212.4 mL ± 73.1 mL (indexed 114.2 mL/m2  ± 34.4 mL/m2 ). The mean right ventricular ejection fraction (RVEF) was 53.7% ± 7.9%. The mean left ventricular end-diastolic volume (LVEDV) was 161.5 mL ± 73.7 mL (indexed 87.8 mL/m2  ± 41.1 mL/m2 ). The mean left ventricular ejection fraction (LVEF) was 59.8% ± 5.7%. There was a significant correlation between right ventricular (RV) size on MRI and NT-proBNP level. CONCLUSIONS: We present a relatively well cohort of patients with overall favorable long-term outcome. The majority of patients are NHYA class 1 and the systemic right ventricular function appears to be well preserved as assessed by MRI. The exercise tolerance is reduced, with the majority of patients achieving around 60% of the estimated VO2max . Regular specialist follow-up and assessment with advanced imaging at regular intervals remain important for this group.

Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair.

BACKGROUND: The population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK. METHODS: A retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK. RESULTS: 50 women had 124 pregnancies, resulting in 68 (54.8%) miscarriages, 2 terminations of pregnancy, 1 intrauterine death (at 30 weeks), 53 (42.7%) live births and 4 neonatal deaths. Cardiac complications in pregnancies with a live birth included heart failure (n=7, 13.5%), arrhythmia (n=6, 11.3%) and pulmonary embolism (n=1, 1.9%). Very low baseline maternal oxygen saturations at first obstetric review were associated with miscarriage. All eight women with saturations of less than 85% miscarried, compared with 60 of 116 (51.7%) who had baseline saturations of ≥85% (p=0.008). Obstetric and neonatal complications were common: preterm delivery (n=39, 72.2%), small for gestational age (<10th percentile, n=30, 55.6%; <5th centile, n=19, 35.2%) and postpartum haemorrhage (n=23, 42.6%). There were no maternal deaths in the study period. CONCLUSION: Women with a Fontan circulation have a high rate of miscarriage and, even if pregnancy progresses to a viable gestational age, a high rate of obstetric and neonatal complications.

Removal of scatter radiation in paediatric cardiac catheterisation: a randomised controlled clinical trial.

OBJECTIVE: This study sought to determine if DNA integrity was compromised by ionising radiation from paediatric cardiac catheterisations and if dose optimisation techniques allowed DNA integrity to be maintained. MATERIALS AND METHODS: Children were imaged using either: (i) an anti-scatter grid (current departmental protocol), (ii) no anti-scatter grid or, (iii) no anti-scatter grid and a 15 cm air-gap between the child and the x-ray detector. Dose area product and image quality were assessed, lifetime attributable cancer risk estimates were calculated and DNA double-strand breakages quantified using the γH2AX assay. RESULTS: Consent was obtained from 70 parents/guardians/children. Image quality was sufficient for each procedure performed. Removal of the anti-scatter grid resulted in dose reductions of 20% (no anti-scatter grid) and 30% (15 cm air-gap), DNA double-strand break reductions of 30% (no anti-scatter grid) and 20% (15 cm air-gap) and a reduction of radiation-induced cancer mortality risk of up to 45%. CONCLUSION: Radiation doses received during paediatric cardiac catheterisation procedures resulted in a significant increase in DNA damage while maintaining acceptable image quality and diagnostic efficacy. It is feasible to remove the anti-scatter grid resulting in a reduction in DNA damage to the patient. The γH2AX assay may be used for assessment of dose optimisation strategies in children.

Perinatal management of major congenital heart disease.

Congenital heart disease (CHD) is the most common form of congenital anomaly. Prenatal diagnosis of CHD has been associated with decreased morbidity and mortality for some forms of major CHD. As most cases of major CHD are not identified prenatally, clinical examination of the newborn and pulse oximetry are also important means of identifying more cases. Clinicians must suspect CHD as a diagnosis in a cyanosed or shocked neonate and be familiar with appropriate management, namely the commencement of prostaglandin if a duct dependent cardiac lesion is suspected. Telemedicine can aid prompt diagnosis of CHD and therefore direct appropriate management.

Survey of paediatricians' opinions on a regional paediatric telecardiology service.

AIM: The aim of this study is to evaluate consultant general paediatricians' opinions of a UK paediatric telecardiology service. METHODS: A structured questionnaire was developed and sent to all consultant paediatricians working in a district general hospital in Northern Ireland. RESULTS: Paediatricians (n = 35) regarded the regional paediatric telecardiology service as very useful and of good value for money. Paediatricans in hospitals without access to telecardiology expressed a desire to join the network (86%, 12/14). More frequent use of the paediatric telecardiology service was associated with increased confidence in performing echocardiography and using the telemedicine equipment and a special interest in neonatology. The vast majority of paediatricians (32/35, 91%) believed that there should be a shared clinical responsibility for the patient following a teleconsultation. A total of 33/35 (94%) stated that the telephone costs of the consultation should be paid by the paediatrician but that the professional time of the cardiologist should be paid by the tertiary centre (29/35, 83%). CONCLUSIONS: Paediatricians have consistently positive experiences of a regional paediatric telecardiology service. They believe that clinical responsibility is shared, and there should not be any professional fee for telemedicine activities.

A randomised trial of a remote home support programme for infants with major congenital heart disease.

OBJECTIVES: To assess the sustainability, clinical utility and acceptability to clinicians and parents of a tele-homecare programme for infants with major congenital heart disease (CHD), and to evaluate the impact on healthcare resource use. DESIGN: Randomised control trial. SETTING: UK tertiary congenital cardiac centre. PARTICIPANTS: 83 infants with major CHD. INTERVENTION: Participants were randomised to one of three groups: video-conferencing support (n=35), telephone support (n=24) and a control group (n=24). Patients in the two intervention groups received regular, standardised remote consultations. Video-conferences (VCs) were facilitated by Integrated Systems Digital Network lines and replaced by home broadband connections later in the study. MAIN OUTCOME MEASURES: Healthcare resource use, utilisation including hospitalisation, clinicians' opinions on utility and quality of interventions, parental opinions on quality of interventions. RESULTS: Clinicians were more confident making medical decisions following VCs compared with telephone consultations (p=0.01). Both VC and telephone support were very well received, but parents expressed significantly higher levels of satisfaction with VC support (p=0.001). Healthcare resource use was 37% lower in the video-conferencing group compared with both telephone support and control groups (p<0.001), as was the risk of hospitalisation (p=0.006). Direct health service costs were significantly lower in the video-conferencing group (p<0.05). CONCLUSIONS: A tele-medicine home support programme for families of infants with major CHD is feasible, sustainable and effective. Home support with video-conferencing is superior to telephone consultations. Parents are highly satisfied with tele-homecare. Tele-homecare significantly reduces health service utilisation and may reduce health service costs.

A fetal telecardiology service: patient preference and socio-economic factors.

OBJECTIVE: The aims of this study were to evaluate patients' opinions on a fetal cardiology telemedicine service compared with usual outpatient care, the effect of the telemedicine consultation on maternal anxiety and its impact on travel times and time absent from work. METHODS: Prospective study over 20 months. Eligible patients attended for routine anomaly scan followed by fetal echocardiogram transmitted to the regional centre with live guidance by a fetal cardiologist, followed by parental counselling. All patients were offered a fetal cardiology appointment at the regional centre. Structured questionnaires assessing maternal satisfaction, travel times/days off and anxiety scores completed at time of both fetal echocardiograms. RESULTS: Sixty-seven patients were recruited and 66 completed the study. Participants expressed very high satisfaction rates with fetal telecardiology, equivalent to face-to-face consultation. The telecardiology appointments were associated with significantly reduced travel times and days off work (p < 0.01). Expectant mothers expressed a clear inclination for a fetal cardiology appointment at the local hospital facilitated by telemedicine (p < 0.01). CONCLUSIONS: Fetal telecardiology is highly acceptable to patients and is even preferred compared with travelling to a regional centre. There are additional socio-economic benefits that should encourage the development of remote fetal cardiology services.

Fetal diagnosis of congenital heart disease by telemedicine.

OBJECTIVES: This study aimed to evaluate the feasibility, accuracy and user acceptability of performing remote fetal echocardiograms (FEs). SETTING: A regional fetal cardiology unit and a district general hospital (DGH). DESIGN: A prospective study over 20 months. An initial FE was performed by a radiographer in the DGH (D1) followed by a second FE transmitted to the regional centre, in real time, via a telemedicine link with live guidance by a fetal cardiologist (D2). A FE was performed later at the regional centre (D3, reference standard). Structured questionnaires were employed to evaluate the technical quality of each tele-link and the radiographers' confidence at performing FE. RESULTS: 69 remote FEs were performed and showed 58 normal hearts and 11 with congenital heart disease (CHD). D2 was accurate in 97% of cases compared with D3 (κ score=0.89) indicating excellent agreement. All tele-links connected at first attempt with a mean study time = 13.9 min. Overall tele-link quality was rated highly (median=4/5). In 94% of tele-links, at least 11/12 components of the FE were confidently assessed. The mean composite radiographer's questionnaire score increased significantly during the study period (p<0.05). CONCLUSIONS: To date this is the largest study of its kind. CHD can be confidently diagnosed and excluded by remote FE. Radiographers report increased confidence and proficiency following involvement in real-time telemedicine. This application of telemedicine could improve access to fetal cardiology and support radiographers screening for CHD.

Remote diagnosis of congenital heart disease: the impact of telemedicine.

OBJECTIVES: To determine the accuracy of remote diagnosis of congenital heart disease (CHD) by real-time transmission of echocardiographic images via integrated services digital network (ISDN) lines, to assess the impact on patient management and examine cost implications. DESIGN: Prospective comparison of echocardiograms on infants with suspected significant CHD performed as follows: (1) hands-on evaluation and echocardiogram by a paediatrician at a district general hospital (DGH) followed by (2) transmission of the echocardiogram via ISDN 6 with guidance from a paediatric cardiologist and finally (3) hands-on evaluation and echocardiogram by a paediatric cardiologist. The economic analysis compares the cost of patient care associated with the telemedicine service with a hypothetical control group. SETTING: Neonatal units of three DGH and a UK regional paediatric cardiology unit. RESULTS: Echocardiograms were transmitted on 124 infants. In five cases scans were inadequate for diagnosis. Of the remaining 119 tele-echocardiograms, a follow-up echocardiogram was performed on 109/119 (92%). Major CHD was diagnosed in 39/109 infants (36%) and minor CHD in 45 (41%). The tele-echo diagnosis was accurate in 96% of cases (kappa=0.89). Unnecessary transfer to the regional unit was avoided in 93/124 patients (75%). Despite relatively high implementation costs, telemedicine care was substantially cheaper than standard care. Each DGH potentially saved money by utilising the telemedicine service (mean saving: pound728/patient). CONCLUSIONS: CHD is accurately diagnosed by realtime transmission of echocardiograms performed by paediatricians under live guidance and interpretation by a paediatric cardiologist. Remote diagnosis and exclusion of CHD affects patient management and may be cost saving.

Diagnosis of congenital heart disease in neonates by videoconferencing: an eight-year experience.

Over an eight-year period, echocardiograms were transmitted by ISDN at 384 kbit/s for a total of 132 patients suspected of having congenital heart disease (CHD). Five transmitted scans were inadequate. Hands-on echocardiograms were performed subsequently on 116 of the remaining 127 cases (91%). Major CHD was diagnosed in 42 of the 116 infants (36%) and minor CHD in 49 (42%). The telemedicine diagnosis was accurate in 97% of the cases (kappa = 0.90). There were four diagnostic errors. Transfer to the regional unit was avoided in 95 patients (72%). The present study shows that high diagnostic accuracy is possible using a telemedicine link to transmit images obtained with the assistance of real-time guidance by a paediatric cardiologist. The results also demonstrate the importance of an expert interpreting the echocardiographic images, since the accuracy of diagnosis was considerably improved (the kappa coefficient increased from 0.14 to 0.90).

Home support for children with complex congenital heart disease using videoconferencing via broadband: initial results.

We originally developed a home support service for babies with complex congenital heart disease, in which videoconferencing was delivered via three ISDN lines. We have now investigated the feasibility of using broadband (Internet protocol) transmission instead of ISDN lines. Five patients were enrolled (age range 14-58 days) and 78 videoconferences were conducted over a six-month period. In 70 videoconferences (90%), a successful connection was established at the first attempt. In the last 56 videoconferences a connection bandwidth of 256 kbit/s was consistently achieved. The clinician's opinions of the videoconferences were good. Parental opinions on the videoconferences were very high. There was also a significant reduction in parental anxiety following the video consultations: the median reduction in the STAI score was 6 points (P < 0.05) (n = 78). Home support for infants or children with complex congenital heart disease can be provided successfully by video consultations utilizing home broadband links.

Down syndrome, achondroplasia and tetralogy of Fallot.

This paper describes a female infant with achondroplasia, Down syndrome and tetralogy of Fallot. Down syndrome and achondroplasia were confirmed by karyotyping and presence of a common fibroblast growth factor receptor 3 mutation (Gly380Arg), respectively. The clinical course was complicated by pulmonary hypoplasia and subsequent intractable respiratory failure secondary to the combination of congenital conditions, which resulted in the patient's death at 5 months.

Uncorrected tetralogy of Fallot: adult presentation in the 61st year of life.

Tetralogy of Fallot (TOF) is the commonest form of cyanotic congenital heart defect after infancy [Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults-Second of Two Parts. NEJM 2000; 342(5):334-342.]. There are few studies assessing the risk of surgical correction in adult patients and long-term survival into the fourth decade of life is rare. The case history is described of a 61-year old female presenting with probable viral myocarditis. Subsequent investigations revealed an underlying diagnosis of tetralogy of Fallot. The patient remains asymptomatic despite persistent hypoxia. Potential factors contributing to longevity in this case are relatively good pulmonary blood flow via large branch pulmonary arteries, and the possible gradual development of right ventricular outflow tract obstruction over a long time period.

Inaccurate electrocardiographic interpretation of long QT: the majority of physicians cannot recognize a long QT when they see one.

BACKGROUND: Physicians in all fields of medicine may encounter patients with long QT syndrome (LQTS). It is important to define the percentage of physicians capable of distinguishing QT intervals that are long from those that are normal because LQTS can be lethal when left untreated. OBJECTIVES: The purpose of this study was to define the percentage of physicians in the different disciplines of medicine who can recognize a long QT when they see one. METHODS: We presented the ECGs of two patients with LQTS and two healthy females to 902 physicians (25 world-renowned QT experts, 106 arrhythmia specialists, 329 cardiologists, and 442 noncardiologists) from 12 countries. They were asked to measure the QT, calculate the QTc (the QT interval corrected for the heart rate), and determine whether the QT is normal or prolonged. RESULTS: For patients with LQTS, >80% of arrhythmia experts but <50% of cardiologists and <40% of noncardiologists calculated the QTc correctly. Underestimation of the QTc of patients with LQTS and overestimation of the QTc of healthy patients were common. Interobserver agreement was excellent among QT experts, moderate among arrhythmia experts, and low among cardiologists and noncardiologists (kappa coefficient = 0.82, 0.44, and < 0.3, respectively). Correct classification of all QT intervals as either "long" or "normal" was achieved by 96% of QT experts and 62% of arrhythmia experts, but by only <25% of cardiologists and noncardiologists. CONCLUSIONS: Most physicians, including many cardiologists, cannot accurately calculate a QTc and cannot correctly identify a long QT.