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Biomedical subjects

F J Macartney

Publications and source records attributed to F J Macartney.

At least 19 recordsLinked to original sources

Usefulness of biplane transesophageal echocardiography in neonates, infants and children with congenital heart disease.

A study was performed to assess the feasibility, additional diagnostic value and potential applications of biplane transesophageal echocardiography in neonates, infants and children. One hundred thirty-two consecutive studies were attempted in 111 anesthetized children with congenital heart disease. Longitudinal and transverse planes were compared using 3 methods: (1) separate 7 mm longitudinal and transverse pediatric transducers used sequentially; (2) an experimental 9 x 8 mm biplane pediatric transducer; and (3) a standard adult biplane transducer (12 x 9 or 13 x 9 mm). In all but 1 patient, a probe could be inserted. The longitudinal plane provided superior visualization of both the right and left ventricular outflow tracts, the interatrial septum, the main pulmonary artery, the ascending aorta and the right coronary artery. In 18 patients (16%), the longitudinal plane provided completely new diagnostic information that was not obtained with combined transthoracic and transverse plane transesophageal echocardiography. However, the transverse plane was mandatory for demonstration of the 4-chamber view, short-axis cross sections through the great arteries, the distal right pulmonary artery and bifurcation of the left coronary artery. The longitudinal plane is complementary to the transverse plane, but cannot substitute for it.

Adolescent↗

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.

Algorithms↗

Double-inlet ventricle presenting in infancy. II. Results of palliative operations.

The influence of palliation on survival was studied in 191 consecutive infants, presenting at under 1 year of age, with double-inlet ventricle (1973 to 1988, median follow-up 8.5 years). Palliative operations were performed on 154 occasions in 121 patients (63%). Survival after a systemic-pulmonary arterial shunt (n = 57) and banding of the pulmonary trunk (n = 35) was comparable (84% and 77% at 1 year, 62% and 45% at 5 years), but those who underwent repair of aortic arch obstruction fared worse (n = 18, 44% and 22% at 1 and 5 years, p less than 0.001). The remainder did not undergo an operation because of balanced physiology (n = 17, 9% of entire group), complex anatomy (n = 32, 15%), or irreversible low output (n = 19, 12%). Palliative surgery, overall, had a deleterious effect on immediate survival (greater than 1 month relative risk 6.6, p less than 0.001), but, in the survivors, medium-term outcome was improved (greater than 6 months, 0.68, p less than 0.05). This effect was most marked for those undergoing a systemic-pulmonary artery shunt (less than 1 month, 2.52; greater than 6 months, 0.43); by contrast, after banding of the pulmonary trunk, with or without additional repair of the aortic arch repair, medium-term risk was not altered (greater than 6 months, 1.13 and 0.91, respectively). These data will assist the clinician in making decisions concerning the management of infants with double-inlet ventricle and in the judicious use of palliative surgery.

Anastomosis, Surgical↗

Double-inlet ventricle presenting in infancy. I. Survival without definitive repair.

Survival before definitive operations was studied in 191 infants with double-inlet ventricle presenting before 1 year of age (1973 to 1988, median follow-up 8.5 years). The morphologic spectrum was broad, with a great prevalence of associated lesions. The actuarial survival rate before definitive repair was 57% at 1 year, 43% at 5 years, and 42% at 10 years, worse than prior reports because of the younger age at entry into our series. Analysis of univariate risk factors established that right atrial isomerism (18% of the group, relative risk 2.9), common atrioventricular orifice (42%, 2.0), pulmonary atresia (20%, 3.4), obstruction of the systemic outflow tract (18%, 2.5), and extracardiac anomalous pulmonary venous connection (13%, 3.1) were strongly associated with poorer survival. Pulmonary stenosis (40%, 0.35), balanced pulmonary blood flow (9%, 0.40), and presentation at an older age (3%, 0.42 to 0.18) were beneficial (p less than 0.05 to 0.0001). Multivariate analysis allowed the creation of patient-specific curves for prediction of survival for different anatomic and physiologic variants of double-inlet ventricle. A simple additive index was then derived from the multivariate Cox coefficients to enable stratification of risk for these morphologic subgroups of patients and so assist in the making of clinical decisions in infancy.

Follow-Up Studies↗

Double-inlet ventricle presenting in infancy. III. Outcome and potential for definitive repair.

The fate of 191 infants with double-inlet ventricle was studied to determine the influence of morphologic characteristics at presentation and subsequent management on the potential for, and timing of, definitive repair by the Fontan operation or ventricular septation. At presentation, 136 patients (71%) were potential candidates for a Fontan procedure. Actuarial survival was better than for those deemed unsuitable for either definitive option (n = 55; 68% versus 28% at 1 year; p less than 0.001), but still, only 78 patients (57%) were known to be alive and suitable candidates at 2 years of age. This was largely due to death after presentation with low cardiac output (n = 19) and at palliative operation (20 of 98 surgically treated patients). The adverse events of late sudden death (n = 14) and the development of new features precluding a Fontan operation (n = 18) mostly occurred before 4 years of age (n = 22). Patients requiring no operation and those who underwent a systemic-pulmonary arterial shunt fared better than those who underwent isolated banding of the pulmonary trunk (9/13 and 25/42 alive and suitable versus 14/33; p less than 0.05), and than those who required aortic arch repair together with banding (1/12; p less than 0.01), because of the development of subaortic stenosis in the latter group. In contrast, only 43 patients (23% of all 191 patients) had morphologic features that were additionally compatible with future ventricular septation. Actuarial survival free of adverse events for these 43 patients was similar to that of the 136 patients considered suitable for a Fontan operation. Thus management in infancy must be aimed at maintaining potential for a future Fontan operation, which itself should not be delayed, for most patients, beyond 3 years of age, because of the prevalence of adverse events with increasing age.

Follow-Up Studies↗

Combining clinical judgement and statistical data in expert systems: over-the-telephone management decisions for critical congenital heart disease in the first month of life.

When a new-born baby with congenital heart disease is referred to a regional specialist centre, the transportation management is crucial but must be decided on the basis of clinical information obtained over the telephone. We consider algorithmic and naive statistical approaches to helping in this decision, and on the basis of preliminary results the relative strengths and weaknesses are discussed. A synthesised logical and probabilistic approach appears to have the best potential and could be implemented on hand-held computers.

Algorithms↗

Doubly committed subarterial ventricular septal defect: new morphological criteria with echocardiographic and angiocardiographic correlation.

To gain a better understanding of the anatomy of doubly committed subarterial defects and its relation to findings at cross sectional echocardiography and angiocardiography, eight necropsy specimens from patients with this condition were examined, and preoperative echocardiograms and angiocardiograms from 313 patients with surgically or necropsy confirmed outlet defect were reviewed. Of these, 48 had doubly committed subarterial defects. Morphological review showed that doubly committed defects are roofed by the arterial valves in fibrous continuity because of lack of both the outlet septum and the "septal" aspect of the subpulmonary infundibulum. Angiocardiography had a lower sensitivity (50%) than echocardiography (95%) for diagnosis of doubly committed defects, but each was highly specific. In five (14%) of 35 available echocardiograms the arterial valves were normally offset, but in the remainder they were at the same level. The ventriculoarterial connection was concordant in 37/48 (77%), discordant in five (10%) of 48, and double outlet right ventricle in six (13%) of 48. Displacement of the fibrous raphe between the arterial valves in relation to the ventricular septum below was associated with outflow obstruction in 14 patients (pulmonary in nine and aortic in five). These features were readily identified by echocardiography. Thus echocardiography is not only a more accurate method than angiocardiography of recognising these defects, but also shows that the arterial pole of the heart is architecturally abnormal.

Angiocardiography↗

Surgical unifocalization in pulmonary atresia and ventricular septal defect. A realistic goal?

Between 1979 and 1986, 26 patients with pulmonary atresia, ventricular septal defect, and multifocal pulmonary blood supply underwent surgery (median age, 2 years) to reconnect major aortopulmonary collateral arteries to other collateral arteries or to the central pulmonary arteries in an attempt to prepare the pulmonary circulation for complete repair. In four patients, central pulmonary arteries were found to be absent at exploratory thoracotomy, and therefore, the surgery aimed for optimal palliation. Four (15%) patients died perioperatively. The survivors had symptomatic improvement. At restudy 1 week-110 months (median time, 25 months) postoperatively, modest growth of central pulmonary arteries had occurred [right plus left pulmonary artery diameters/descending aortic diameter from 0.64 +/- 0.61 (mean +/- SD) preoperatively to 0.95 +/- 0.60 postoperatively (p less than 0.01)], with a greater number of bronchopulmonary segments connected to central pulmonary arteries [from 6.3 +/- 4.8 preoperatively to 10.0 +/- 4.5 postoperatively (p less than 0.001)]. However, only seven (27%) patients met the established criteria for repair, of whom three (12%) have undergone definitive surgery. Thirteen (50%) patients are unsuitable for repair, and two (8%) await restudy. Failure of anastomoses involving major aortopulmonary collateral arteries (13 of 33) was higher than for grafts from a systemic artery to the central pulmonary artery (1 of 22, p = 0.003). Failure was not related to the number of anastomoses per operation, suggesting that poor distal runoff may have been responsible. This surgical approach provided reasonable palliation but was rarely effective as a prelude to complete repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Diagnostic logic.

Explore the source record for details and available documents.

Algorithms↗

Cardiac rhythm in atrial isomerism.

Standard electrocardiograms from 126 consecutive patients with atrial isomerism were reviewed. Of 67 patients with left isomerism, 49 had sinus rhythm, 8 nodal rhythm and 10 atrioventricular (AV) block. Fifty-eight of 59 patients with right isomerism had sinus rhythm. Complete AV block was significantly more frequent in association with AV septal defect in left isomerism (5 of 45 patients) than in right isomerism (0 of 47 patients, p = 0.049). The P-wave axis was superior in 49% of patients with left isomerism but did not correlate with abnormalities of systemic or pulmonary venous connection. A significant shift of P-wave axis (more than 90 degrees) was seen on a subsequent electrocardiogram in 14 of 44 patients (32%) with left isomerism and 2 of 16 (13%) with right isomerism. Ambulatory electrocardiographic monitoring in 17 patients (14 with left isomerism) showed that only 4 had sinus rhythm throughout 24 hours. Only 1 patient with complete AV block received a permanent pacemaker. Despite the high incidence of electrocardiographic abnormalities, significant arrhythmias appear to be rare. The arrhythmias apparently do not influence the natural history of this condition or affect the outcome of palliative or corrective surgery. The prognosis is determined mainly or solely by the associated anatomic abnormalities.

Arrhythmias, Cardiac↗

Assessment of residual right ventricular outflow tract obstruction following surgery using the response to intravenous propranolol.

The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.

Blood Pressure↗

Reversed offsetting of the septal attachments of the atrioventricular valves and Ebstein's malformation of the morphologically mitral valve.

Ebstein's anomaly of the tricuspid valve is characterised by downstream displacement of the tricuspid leaflets. In the case of the septal leaflet, the result is exaggerated offsetting of the atrioventricular valves. Thus it might be supposed that in Ebstein's anomaly of the mitral valve, downstream displacement of the aortic (septal) leaflet of the mitral valve could move its attachment beyond that of the tricuspid valve, resulting in reversed offsetting. To examine this hypothesis three patients each with a concordant atrioventricular connection were studied. The first, with Marfan's syndrome and without an abnormal mitral valve, had reversed offsetting shown by echocardiography. The second, without Marfan's syndrome and with Ebstein's anomaly of the mitral valve, showed echocardiographically normal offsetting but a displaced mural leaflet. The third, in whom Ebstein's anomaly of the mitral valve was confirmed at necropsy, showed all the features of the second case, with normal offsetting of the atrioventricular valves. Thus reversed offsetting is not a feature of Ebstein's anomaly of the mitral valve and in the first case this feature was caused by upward displacement of the tricuspid valve secondary to dilation of the aortic root.

Abnormalities, Multiple↗

A rare variant of common arterial trunk.

A 13 day old baby was admitted to hospital with multiple abnormalities, increasing cyanosis, and cardiac failure. Cardiac catheterisation was performed on the day of admission and he died shortly after the procedure. A rare variant of common arterial trunk, in which the pulmonary arteries arose directly from the underside of the aortic arch, was found at necropsy.

Humans↗

Absent right superior caval vein (vena cava) with normal atrial arrangement.

Eight cases of absent right superior caval vein (vena cava) with normal atrial arrangement from the Great Ormond Street database were reviewed. In each case the right subclavian vein and jugular vein drained into a persistent left superior caval vein through a bridging vein. In six cases the left superior caval vein drained into the right atrium by way of the coronary sinus, and in two cases it drained into the left atrium directly because of the complete unroofing of the coronary sinus. The frontal plane P wave axis was displaced leftwards and upwards in four out of seven cases in which an electrocardiogram was available. No case showed arrhythmia on a standard electrocardiogram preoperatively. Echocardiograms were available in four cases and in each case diagnosis of the anomalous connections of the systemic vein was possible. Only one third of the cases showed a crescentic shadow of the persistent left superior caval vein on chest x ray. A chest x ray and electrocardiogram do not provide the means of reliable diagnosis of this condition, though they may suggest the possibility of its existence. Definitive diagnosis requires cross sectional echocardiography or angiocardiography or both.

Child↗

Evaluation of long-term results of homograft and heterograft valves in extracardiac conduits.

Between 1971 and 1982, 249 patients received right heart extracardiac conduits. The 173 patients who survived 30 days were reviewed retrospectively; 72 had antibiotic-sterilized aortic homografts, 97 had xenografts of various types, and four had valveless tubes. Reoperation for conduit obstruction was required as early as 13 months after the original operation, but actuarial analysis showed no differences in overall performance of homograft and heterograft conduit groups with respect to late death or survival free of conduit obstruction. More important risk factors for late death or the need for reoperation were the severity of the underlying cardiac lesions and the influence of early postoperative complications. When analyzed statistically, the performance of conduits bearing homografts was disappointing, influenced sometimes by complications in the Dacron extension tubes. Repairs dispensing with conduits entirely should be evaluated.

Actuarial Analysis↗

Continuous wave Doppler in the evaluation of simple and complex congenital heart disease in infants and children.

Continuous wave Doppler assessment of systolic pressure gradients was performed using a digital maximal frequency estimator in 118 consecutive infants and children with suspected ventricular outflow obstruction who were undergoing cardiac catheterisation. There was satisfactory correlation with measured systolic pressure gradients in most patients with isolated pulmonary valve stenosis, aortic stenosis or aortic coarctation. Important under-estimation of gradients, however, occurred frequently in patients with more complex lesions. In many of these, the stenotic jet was posteriorly located or obstruction to flow occurred at more than one level. Continuous wave Doppler assessment of outflow tract gradients should be interpreted with caution in complex congenital heart lesions. A low predicted gradient should not be relied upon in isolation for clinical decision making. On the other hand, demonstration of a large gradient by continuous wave Doppler ultrasound provides additional information which may obviate the need for invasive investigation.

Adolescent↗

Long-term results of the "palliative" Mustard operation.

A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 +/- 6.7 Um2. A ventricular septal defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 +/- 22.8 months). Before operation 4 children were in New York Heart Association functional class IV and 33 were in class III. Mean hemoglobin concentration was 19.43 +/- 3.14 g/dl and arterial oxygen saturation was 63.44 +/- 11.29%. After operation 18 patients were in functional class I, 17 in class II and only 1 in class III. Hemoglobin level decreased to 14.19 +/- 2.3 g/dl and arterial oxygen saturation improved to 89.12 +/- 7.25%. Cardiac catheterization in 21 survivors confirmed no change in pulmonary artery pressure and resistance. Effective pulmonary blood flow improved from 1.39 +/- 0.39 before to 2.6 +/- 0.78 liters/min per m2 after operation. The palliative Mustard operation carries a low risk and provides excellent symptomatic improvement up to 10 years in patients with transposition of the great arteries and pulmonary vascular disease.

Adolescent↗