Rodney Franklin

Morphologic and Functional Predictors of Eventual Circulation in the Fetus With Pulmonary Atresia or Critical Pulmonary Stenosis With Intact Septum

OBJECTIVES The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling. BACKGROUND Pulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation. METHODS Predictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004. We also assessed the predictive value of coronary fistulae and right atrial pressure (RAP) score (comprising the tricuspid valve, foramen ovale, and ductus venosus Doppler). RESULTS One-half of the cohort was first assessed before 23 gestational weeks (range 15.7 to 33.7 weeks). The TV z-score was a good predictor at all gestations, but the best predictive scores for specific gestations were pulmonary valve (PV) z-score (<23 weeks), median TV z-score (<26 weeks), the combination of median PV z-score and the median TV/MV ratio (26 to 31 weeks), and the combination of median TV z-score and median TV/MV ratio (>31 weeks). The RAP score and coronary fistulae were good independent predictors: RAP score >3 predicted BV with area under the curve of 0.833, and detection of fistulae usually predicted a UV route. CONCLUSIONS The best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.

Octreotide to treat postoperative chylothorax after cardiac operations in children

Chylothorax after pediatric cardiac operations is associated with significant morbidity and increased hospitalization. An octreotide (a synthetic somatostatin analogue) infusion (1 to 4 microg/kg per hour) with medium-chain triglyceride diet or parenteral nutrition was used in 4 pediatric cardiac surgical patients after chylothorax was diagnosed. Resolution followed within 5 days in all without recurrence, while on a normal diet.

Assessment, Criticism and Improvement of Imprecise Subjective Probabilities for a Medical Expert System

Abstract Three paediatric cardiologists assessed nearly 1000 imprecise subjective conditional probabilities for a simple belief network representing congenital heart disease, and the quality of the assessments has been measured using prospective data on 200 babies. Quality has been assessed by a Brier scoring rule, which decomposes into terms measuring lack of discrimination and reliability. The results are displayed for each of 27 diseases and 24 questions, and generally the assessments are reliable although there was a tendency for the probabilities to be too extreme. The imprecision allows the judgements to be converted to implicit samples, and by combining with the observed data the probabilities naturally adapt with experience. This appears to be a practical procedure even for reasonably large expert systems.

Prenatal screening for major congenital heart disease: Assessing performance by combining national cardiac audit with maternity data

Objective Determine maternity hospital and lesion-specific prenatal detection rates of major congenital heart disease (mCHD) for hospitals referring prenatally and postnatally to one Congenital Cardiac Centre, and assess interhospital relative performance (relative risk, RR). Methods We manually linked maternity data (3 hospitals prospectively and another 16 retrospectively) with admissions, fetal diagnostic and surgical cardiac data from one Congenital Cardiac Centre. This Centre submits verified information to National Institute for Cardiovascular Outcomes Research (NICOR-Congenital), which publishes aggregate antenatal diagnosis data from infant surgical procedures. We included 120 198 unselected women screened prospectively over 11 years in 3 maternity hospitals (A, B, C). Hospital A: colocated with fetal medicine, proactive superintendent, on-site training, case-review and audit, hospital B: on-site training, proactive superintendent, monthly telemedicine clinics, and hospital C: sonographers supported by local obstetrician. We then studied 321 infants undergoing surgery for complete transposition (transposition of the great arteries (TGA), n=157) and isolated aortic coarctation (CoA, n=164) screened in hospitals A, B, C prospectively, and 16 hospitals retrospectively. Results 385 mCHD recorded prospectively from 120 198 (3.2/1000) screened women in 3 hospitals. Interhospital relative performance (RR) in Hospital A:1.68 (1.4 to 2.0), B:0.70 (0.54 to 0.91), C:0.65 (0.5 to 0.8). Standardised prenatal detection rates (funnel plots) demonstrating inter-hospital variation across 19 hospitals for TGA (37%, 0.00 to 0.81) and CoA (34%, 0.00 to 1.06). Conclusions Manually linking data sources produced hospital-specific and lesion-specific prenatal mCHD detection rates. More granular, rather than aggregate, data provides meaningful feedback to improve screening performance. Automatic maternal and infant record linkage on a national scale, requires verified, prospective maternity audit and integration of health information systems.

Telemedicine in pediatric and perinatal cardiology: Economic evaluation of a service in English hospitals

OBJECTIVES Pediatric cardiology has an expanding role in fetal and pediatric screening. The aims of this study were to observe how district hospitals use a pediatric telecardiology service, and to compare the costs and outcomes of patients referred to specialists by means of this service or conventionally. METHODS A telemedicine service was set up between a pediatric cardiac center in London and four district hospitals for referrals of second trimester women, newborn babies, and older children. Clinicians in each hospital decided on the role for their service. Clinical events were audited prospectively and costed, and patient surveys were conducted. RESULTS The hospitals differed in their selection of patient groups for the service. In all, 117 telemedicine patients were compared with 387 patients seen in London or in outreach clinics. Patients selected for telemedicine were generally healthier. For all patients, the mean cost for the initial consultation was 411 UK pounds for tele-referrals and 277 UK pounds for conventional referrals, a nonsignificant difference. Teleconsultations for women and children were significantly more expensive because of technology costs, whereas for babies, ambulance transfers were much more costly. After 6-months follow-up, the difference between referral methods for all patients was nonsignificant (telemedicine, 3,350 UK pounds; conventional referrals, 2,172 UK pounds), and nonsignificant within the patient groups. CONCLUSIONS Telemedicine was perceived by cardiologists, district clinicians, and families as reliable and efficient. The equivocal 6-month cost results indicate that investment in the technology is warranted to enhance pediatric and perinatal cardiology services.

Cost implications of introducing a telecardiology service to support fetal ultrasound screening

A district hospital in south-east England used a telecardiology service for fetal cardiac diagnosis alongside an existing arrangement for referring pregnant women directly to perinatal cardiologists in London for detailed fetal echocardiography. Women were identified for referral according to local protocols when having a second trimester anomaly scan. For the telemedicine referrals, the sonographers video-recorded images from the anomaly scans for transmission during monthly videoconferences. The cost of the womens antenatal care was calculated from the specialist assessment until delivery, while family costs were collected in a postal survey. Over 15 months, telemedicine was used in 52 cases, while 24 women were seen in London. The London women were more likely to have had an ultrasound abnormality (29% v 10%, P = 0.047). A telemedicine assessment of 5 min duration was more costly than an examination in London (mean cost per referral of £206 v £74, P < 0.001). However, the telecardiology service was cost neutral after 14 days and for the extended period until delivery. Travel costs for London women averaged £37 compared with £5.50 for the telemedicine referrals. Telemedicine may be useful to support perinatal cardiologists in the UK whose workloads are expanding in response to improved standards in antenatal ultrasound screening.

Empirical Evaluation of Prior Beliefs about Frequencies: Methodology and a Case Study in Congenital Heart Disease

Abstract We consider the problem of critiquing prior beliefs concerning the distribution of a discrete random variable in the light of a sequentially obtained sample. A topical application concerns a probabilistic expert system for the diagnosis of congenital heart disease, which requires specification of a large number of conditional probabilities that are initially imprecisely estimated by a suitable “expert.” These prior beliefs may be formally updated as data become available, but it would seem essential to contrast the original expert assessments with the data obtained to quickly identify inappropriate subjective inputs. We consider both Bayes factor and significance testing techniques for such a prior/data comparison, both in nonsequential and sequential forms. The common basis as alternative standardizations of the logarithm of the predictive ordinate of the observed data is emphasised, and a Bayesian discrepancy statistic with a variety of interpretations provides a formal means of discounting the...

Evaluation of a diagnostic algorithm for heart disease in neonates.

OBJECTIVE--To develop, test, and validate an algorithm for diagnosing disease in neonates during an over the telephone referral to a specialist cardiac centre. DESIGN--A draft algorithm requiring only data available to a referring paediatrician was generated. This was modified in the light of a retrospective review of case records. A questionnaire to elicit all the data required by the algorithm was then generated. There followed a prospective three phase evaluation during consecutive over the telephone referrals. This consisted of (a) a conventional phase with unstructured referral consultations, (b) a phase with referrals structured around the questionnaire but independent of the algorithm, and (c) a validation phase with the algorithm (and its previous errors) available during the referral consultation. SETTING--59 paediatric centres in south east England and a central specialist paediatric cardiology unit. PATIENTS--Consecutive neonates (aged less than 31 days) referred with suspected heart disease. The retrospective review was of records of 174 neonates from 1979. In the prospective evaluation (1987-90) the conventional phase comprised 71 neonates (over 5.5 months), the structured phase 203 neonates (over 14 months), and the validation phase 195 neonates (over 12 months). MAIN OUTCOME MEASURES--Diagnostic accuracy (assigning patients to the correct diagnostic category (out of 27)), of the referring paediatrician, the specialist after the referral consultation, and the algorithm as compared with the definitive diagnosis by echocardiography at the specialist centre, and score for the appropriateness of management in transit. RESULTS--Simply structuring the consultation by questionnaire (that is, proceeding from the conventional phase to the structured phase) improved the diagnostic accuracy of both paediatricians (from 34% (24/71 cases) to 48% (97/203) correct) and specialists (from 54% (38/71 cases) to 64% (130/203) correct). The algorithm (structured phase) would have been even more accurate (78% (158/203 cases); p less than 0.01). Management scores in the structured phase were also better than in the conventional phase (80%(162/203 cases)v 58% (41/71) appropriate; p less than 0.01). Management scores would have improved to 91% appropriate (185/203; p less than 0.001) had the algorithmic diagnoses dictated management. The superiority of the algorithm was maintained but not bettered in the validation phase. CONCLUSIONS--Applying the algorithm should reduce the morbidity and mortality of neonates with critical heart disease by aiding clinicians in therapeutic decisions for in transit care.

A paediatric telecardiology service for district hospitals in south-east England: an observational study.

Objectives: To compare caseloads of new patients assessed by paediatric cardiologists face-to-face or during teleconferences, and assess NHS costs for the alternative referral arrangements. Design: Prospective cohort study over 15 months. Setting: Four district hospitals in south-east England and a London paediatric cardiology centre. Patients: Babies and children. Intervention: A telecardiology service introduced alongside outreach clinics. Measurements: Clinical outcomes and mean NHS costs per patient. Results: 266 new patients were studied: 75 had teleconsultations (19 of 42 newborns and 56 of 224 infants and children). Teleconsultation patients generally were younger (49% being under 1 year compared with 32% seen personally (p = 0.025)) and their symptoms were not as severe. A cardiac intervention was undertaken immediately or planned for five telemedicine patients (7%) and 30 conventional patients (16%). However, similar proportions of patients were discharged after being assessed (32% telemedicine and 39% conventional). During scheduled teleconferences the mean duration of time per patient in sessions involving real-time echocardiography was 14.4 min, and 8.5 min in sessions where pre-recorded videos were transmitted. Mean cost comparisons for telemedicine and face-to-face patients over 14-day and 6-month follow-up showed the telecardiology service to be cost neutral for the three hospitals with infrequently-held outreach clinics (£1519 vs £1724 respectively after 14 days). Conclusion: Paediatric cardiology centres with small cadres of specialists are under pressure to cope with ever-expanding caseloads of new patients with suspected anomalies. Innovative use of telecardiology alongside conventional outreach services should suitably, and economically, enhance access to these specialists.

Costs of neonatal care for low-birthweight babies in English hospitals

Aim:  To estimate mean costs of neonatal care for babies with birthweights ≤1800 g in a regional Level 3 unit and three Level 2 units providing short‐term intensive care.