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).

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.

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.

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%).

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.

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.

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.

Arrhythmia and late mortality after Mustard and Senning operation for transposition of the great arteries. An eight-year prospective study.

Arrhythmia and late sudden death are recognized complications of intraatrial repair of transposition of the great arteries. We performed a prospective study over 8 years in 100 consecutive hospital survivors who underwent Mustard (46 patients) or Senning (54 patients) operations between 1978 and 1982. Arrhythmia was analyzed by preoperative, postoperative, and serial follow-up Holter monitoring and standard electrocardiograms and was related to clinical outcome.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Percutaneous balloon valvuloplasty for pulmonary valve stenosis in infants and children.

Pulmonary valve stenosis was relieved by balloon dilatation during cardiac catheterisation on 27 occasions in 23 infants and children aged 7 days to 12 years, median 31 months (three aged less than 2 weeks). Pulmonary valve diameter was estimated by cross sectional echocardiography to assist in the choice of balloon size. Before dilatation the right ventricular systolic pressure ranged from 41 to 190 (median 92) mm Hg and was suprasystemic in 10 patients.

Decision making in the definitive repair of the heart with a small right ventricle.

Data from 51 patients with small right ventricle who underwent a definitive repair were analyzed retrospectively. The repair was considered complete (29 patients) when it resulted in relief of the right ventricular outflow tract obstruction by closure of intracardiac (atrial septal defect) and extracardiac (when present) shunts. The repair was considered a definitive palliation when the intracardiac and the extracardiac shunts were left open (11 patients).

Left ventricular outflow tract obstruction in complete transposition of the great arteries with intact ventricular septum. A cross sectional echocardiography study.

The roles of posterior bulging of the interventricular septum (septal bulge) and of systolic septal mitral apposition in patients with simple transposition of the great arteries are not known. Cross sectional echocardiograms of 40 such patients were reviewed (after exclusion of those with fixed left ventricular outflow tract obstruction) and haemodynamic findings were compared with long and short axis measurements within the left ventricle. There was no significant correlation between the degree of septal bulge and systolic gradient across the left ventricular outflow tract, but septal bulge correlated weakly with systolic right ventricular pressure and inversely with pulmonary arteriolar resistance index.

Cross sectional echocardiographic and angiocardiographic correlation in criss cross hearts.

Cross sectional echocardiography can provide accurate anatomical diagnosis in congenital heart diseases and therefore should be able reliably to identify criss cross hearts and enable the analysis of their sequential arrangement non-invasively. The cross sectional echocardiographic diagnoses in eight consecutive patients with this condition were compared with those made at cardiac catheterisation and cineangiocardiography (five retrospectively, three prospectively). The mean number of invasive studies required to reach the diagnosis was 1.

Localisation of ventricular septal defects by simultaneous display of superimposed colour Doppler and cross sectional echocardiographic images.

Precise non-invasive localisation of the site of a small ventricular septal defect was attempted using a new technique that simultaneously combines conventional cross sectional echocardiography with a Doppler system by superimposing the colour coded direction and velocity of blood flow directly on to real time ultrasound images. Twenty three patients with unoperated ventricular septal defects and a further eight after surgical closure were studied; 12 children with normal hearts served as controls. A colour coded blood flow jet entering the right ventricle during systole was identified in all 23 unoperated patients, in 11 of whom the defect was too small to be visualised by conventional cross sectional echocardiography.