Three-dimensional echocardiography: historical development and current applications.

Three-dimensional (3D) echocardiography facilitates spatial recognition of intracardiac structures, potentially enhancing diagnostic confidence of conventional echocardiography. The accuracy of 3D images has been validated in vitro and in vivo. In vitro, a detail 1.0 mm in dimension and 2 details separated by 1.0 mm can be identified from a volume-rendered 3D image. In vitro 3D volume measurements are underestimated by approximately 4.0 mL. In vivo, left ventricular volume measurements correlate highly with both cineventriculography (limits of agreement +/-18 mL for end diastole and +/-10 mL for end systole) and magnetic resonance imaging, including measurements for patients with functionally single ventricles. Studies on congenital heart lesions have shown good accuracy and good reproducibility of dynamic "surgical" reconstructions of septal defects, aortoseptal continuity, atrioventricular junction, and both left and right ventricular outflow tract morphology. Transthoracic 3D echocardiography was shown feasible in 81% to 96% of patients with congenital heart defects and provided additional information to that available from conventional echocardiography in 36% of patients, mainly in more detailed description of mitral valve morphology, aortoseptal continuity, and atrial septum. In patients with mitral valve insufficiency, 3D echocardiography was shown to be accurate in the quantification of the dynamic mechanism of mitral regurgitation and in the assessment of mitral commissures in patients with mitral stenosis. This includes not only valve tissue reconstruction but also color flow intracardiac jets. Three-dimensional reconstructions of the aortic valve were achieved in 77% of patients, with an accuracy of 90%. In conclusion, the role of 3D echocardiography, which continues to evolve, shows promise in the assessment of congenital and acquired heart disease.

Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology.

A prospective study of 3-dimensional (3-D) transthoracic echocardiographic definition of atrioventricular septal defect (AVSD) morphology and its dynamic changes during the cardiac cycle was performed. The information obtained from 2-D and 3-D transthoracic echocardiography (TTE) was compared with intraoperative findings in an unselected group of 15 patients with AVSD (median age 22 months). In all study patients, 3-D reconstructions provided anatomic views of the atrioventricular valve(s) en face from either atrial or ventricular perspectives that allowed comprehensive assessment of dynamic valve morphology and the mechanism of valve reflux. Left-sided valve function was correctly assessed by 2-D TTE in 11 of 15 patients (73%) and in 14 of 15 (93%) by 3-D TTE. In 6 of 15 patients (40%), the severity of right-sided valve reflux was described precisely by 2-D TTE and in 12 of 15 patients (80%) by 3-D TTE. Additionally, 3-D TTE supplemented the diagnostic information to that available from 2-D TTE on atrial and ventricular septal defects. Although primum atrial septal defects were depicted by 2-D and 3-D TTE in all 15 patients, the description of defect size was more precise by the 3-D TTE (80% vs. 100%, respectively). The presence of secundum atrial septal defect was correctly diagnosed by both TTE techniques in 10 of 15 patients. Disagreement regarding the size of the defect was present only in 2 of 10 patients by 2-D TTE. In another 2 patients, 3-D TTE described multiple defect fenestrations that were missed by 2-D TTE. Thus, the agreement score was 73% for 2-D and 100% for 3-D echo. The agreement for the presence and sizing of ventricular septal defects was 67% for 2-D and 93% for 3-D echo. We conclude that 3-D TTE provided accurate anatomic reconstructions of the common atrioventricular junction and that the use of dynamic 3-D TTE enhanced the anatomic diagnostic capability of standard 2-D TTE. Medica, Inc.

Left ventricular function in adolescents and adults with restrictive ventricular septal defect and moderate left-to-right shunting.

BACKGROUND: The long-term haemodynamic effects of a restrictive ventricular septal defect permitting moderate left-to-right shunting are not known. PATIENTS AND METHODS: Echocardiographic measurements of left heart dimensions and function were compared between a group of 9 older children and adults (median age 21 years, range 12-24.5 years) having restrictive ventricular septal defects (median Qp/Qs 1.7, range 1.4-2.1) and a group of 10 age matched controls. RESULTS: Left ventricular mass indexed to body surface area was significantly greater in subjects than in controls (102+/-29 vs. 75+/-13 g/m2, p=0.02), although there was no significant difference in the ratio of mass to volume. There were no significant differences between indexes of left ventricular systolic function. Ratios of peak early to late diastolic ventricular filling were lower in those with septal defects (1.5+/-0.3 vs. 1.8+/-0.3, p=0.03), but there were no differences in other indexes of diastolic function. CONCLUSIONS: Resting left ventricular function does not appear to have deteriorated by early adult life in patients with restrictive ventricular septal defects and moderate volume loading. This would support a continued conservative approach to management in these patients.

Late aortic homograft valve endocarditis caused by Cardiobacterium hominis: a case report and review of the literature.

An unusual case of Cardiobacterium hominis endocarditis involving an aortic homograft valve is presented. Although the patient was young (a 17 year old man) and showed few of the characteristic features of the disease, the report does illustrate a number of the problems associated with this illness and highlights the need for the careful assessment of apparent culture negative endocarditis. The organism itself is susceptible to most antibiotics but further treatment, including surgery, may be necessary. Patients must therefore be examined repeatedly and assessed for haemodynamic deterioration, valve destruction or embolic phenomena. Homograft valve replacement may offer some benefits in the setting of aortic valve endocarditis and is therefore an attractive option in this situation.

Transcatheter closure of atrial septal defect and interatrial communications with a new self expanding nitinol double disc device (Amplatzer septal occluder): multicentre UK experience.

OBJECTIVE: To review the safety and efficacy of the Amplatzer septal occluder for transcatheter closure of interatrial communications (atrial septal defects (ASD), fenestrated Fontan (FF), patent foramen ovale (PFO)). DESIGN: Prospective study following a common protocol for patient selection and technique of deployment in all participating centres. SETTING: Multicentre study representing total United Kingdom experience. PATIENTS: First 100 consecutive patients in whom an Amplatzer septal occluder was used to close a clinically significant ASD or interatrial communication. INTERVENTIONS: All procedures performed under general anaesthesia with transoesophageal echocardiographic guidance. Interatrial communications were assessed by transoesophageal echocardiography with reference to size, position in the interatrial septum, proximity to surrounding structures, and adequacy of septal rim. Stretched diameter of the interatrial communications was determined by balloon sizing. Device selection was based on and matched to the stretched diameter of the communication. MAIN OUTCOME MEASURES: Success defined as deployment of device in a stable position to occlude the interatrial communication without inducing functional abnormality or anatomical obstruction. Occlusion status determined by transoesophageal echocardiography during procedure and by transthoracic echocardiography on follow up. Clinical status and occlusion rates assessed at 24 hours, one month, and three months. RESULTS: 101 procedures were performed in 100 patients (86 ASD, 7 FF, 7 PFO), age 1.7 to 64.3 years (mean (SD), 13.3 (13.9)), weight 9.2 to 100.0 kg (mean 32.5 (23.5)). Procedure time ranged from 30 to 180 minutes (mean 92.4 (29.0)) and fluoroscopy time from 6.0 to 49.0 minutes (mean 16.1 (8.0)). There were seven failures, all occurring in patients with ASD, and one embolisation requiring surgical removal. Immediate total occlusion rate was 20.4%, rising to 84.9% after 24 hours. Total occlusion rates at the one and three month follow up were 92.5% and 98.9%, respectively. Complications were: transient ST elevation (1), transient atrioventricular block (1), presumed deep vein thrombosis (1), presumed transient ischaemic attack (1). CONCLUSIONS: It appears feasible to close interatrial communications and atrial septal defects up to 26 mm stretched diameter safely with the Amplatzer septal occluder. Short term results confirm an early high occlusion rate with no major complications. Careful selection of cases based on the echocardiographic morphology of the ASD and accurate assessment of their stretched diameter is of utmost importance. Further experience with the larger devices and longer term results are required before a firm conclusion regarding its use can be made.

Simultaneous transcatheter valvuloplasty and Amplatzer septal occlusion for pulmonary valvar stenosis and secundum atrial septal defect.

Transcatheter balloon valvuloplasty had been established as the treatment of choice in patients with pulmonary valvar stenosis. Non surgical closure of secundum atrial defects by various occlusive devices is currently being evaluated. We report the first successful simultaneous transvenous Mansfield balloon dilation and Amplatzer septal occlusion in a 7-year-old girl with moderately severe pulmonary valvar stenosis and secundum atrial defect in April 1997. Satisfactory reduction of pressure gradient across pulmonary valve and complete obliteration of left to right atrial shunt were achieved.

Transcatheter occlusion of atrial septal defects: an initial experience with the Amplatzer septal occluder.

OBJECTIVE: To discuss the initial experience with the use of a new double disc occluding device, the Amplatzer septal occluder in transcatheter occlusion of secundum atrial septal defects (ASD). METHODS: Transvenous sizing of secundum ASD was performed in five children under general anaesthesia using transoesophageal echocardiographic and fluoroscopic guidance. An Amplatzer septal occluder equal to or minimally larger than the stretched diameter of the ASD was used for transcatheter occlusion of the defect in three suitable patients. Pulmonary balloon valvuloplasty with a 18 mm x 3 cm Mansfield balloon catheter was carried out in one patient with associated pulmonary valvar stenosis in the same procedure setting. RESULTS: Stretched diameters of the defects in the three patients ranged from 14 to 17 mm. Devices of sizes 14, 17 and 17 were deployed through 7F and 8F sheaths, respectively. The upper and lower rims of interatrial septum were more than 8 mm in all patients. All patients had successful occlusion with complete obliteration of the atrial left to right shunting. Simultaneous pulmonary balloon valvuloplasty for the valvar stenosis reduced pressure gradient from 53 mmHg to 22 mmHg across the valve prior to septal occlusion in one patient. No intraprocedural or short-term complication was encountered. CONCLUSIONS: The design of the Amplatzer septal occluder permits ease in loading, delivery, deployment and stable seating of the device. This initial experience shows that Amplatzer device occlusion is feasible, relatively safe and effective and appears to be a viable alternative to surgical closure of secundum atrial septal defects in properly selected patients.

Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study.

BACKGROUND: Congenital heart defects are generally assumed to have a multifactorial aetiology. We have tested this hypothesis by studying adults with heart defects and their families. METHODS: We identified 1094 patients who survived surgery for major cardiac defects before 1970. We chose individuals with disturbance of situs or segmental connection, with atrioventricular septal defect or with tetralogy of Fallot. After exclusion and non-participation, 727 individuals were traced. Each was visited by an investigator and completed a detailed questionnaire. If possible, all "normal" offspring were examined by a paediatric cardiologist. FINDINGS: The 727 individuals had 393 live offspring. There were 71 miscarriages and five terminated pregnancies. Overall, we found recurrent heart defects in 16 liveborn offspring--a recurrence risk of 4.1%. This result differed significantly from sibling risk (2.1%; p=0.021). More congenital heart defects occurred in the offspring of affected women than in those of affected men (p=0.047); when all malformations (cardiac and non-cardiac) in the offspring were taken into account the excess was more significant (p=0.032). We found an excess of miscarriages in the offspring of affected women (p=0.001). In tetralogy of Fallot, heart defects occurred in seven (3.1%) of 223 offspring, 12 (2.2%) of 539 siblings, five (0.3%) of 1575 second-degree relatives, and eight (0.3%) of 2728 third-degree relatives. INTERPRETATION: Our findings do not support a polygenic basis for all heart defects. Atrioventricular septal defect seems to be a single-gene defect and tetralogy of Fallot a polygenic disorder with a small number of interacting genes. Our data suggest that isolated transposition of the great arteries is a sporadic defect.

Assessment of atrial septal defect morphology by transthoracic three dimensional echocardiography using standard grey scale and Doppler myocardial imaging techniques: comparison with magnetic resonance imaging and intraoperative findings.

OBJECTIVE: To determine whether transthoracic three dimensional echocardiography is an accurate non-invasive technique for defining the morphology of atrial septal defects (ASD). METHODS: In 34 patients with secundum ASD, mean (SD) age 20 (17) years (14 male, 20 female), the measurements obtained from three dimensional echocardiography were compared to those obtained from magnetic resonance imaging (MRI) or surgery. Three dimensional images were constructed to simulate the ASD view as seen by a surgeon. Measured variables were: maximum and minimum vertical and horizontal ASD dimension, and distances to inferior and superior vena cava, coronary sinus, and tricuspid valve. In each patient two ultrasound techniques were used to acquire three dimensional data: standard grey scale imaging (GSI) and Doppler myocardial imaging (DMI). RESULTS: Good correlation was found in maximum ASD dimension (both horizontal and vertical) between three dimensional echocardiography and both MRI (GSI r = 0.96, SEE = 0.05 cm; DMI r = 0.97, SEE = 0.04 cm) and surgery (GSI r = 0.92, SEE = 0.06 cm; DMI r = 0.95, SEE = 0.06 cm). The systematic error was similar for both three dimensional techniques when compared to both MRI (GSI = 0.40 cm (27%); DMI = 0.38 cm (25%)) and surgery (GSI = 0.50 cm (29%); DMI = 0.37 cm (22%)). A significant difference was found in both horizontal and vertical ASD dimension changes during the cardiac cycle. This change was inversely correlated with age. These findings were consistent for both DMI and GSI technique. In children (age < or = 17 years), the feasibility of detecting structures and undertaking measurements was similar for both echo techniques. However, in adult ASD patients (age > or = 18 years) this feasibility was higher for DMI than for GSI. CONCLUSIONS: Transthoracic three dimensional imaging using both GSI and DMI accurately displayed the varying morphology, dimensions, and spatial relations of ASD. However, DMI was a more effective technique than GSI in describing ASD morphology in adults.

Stage IV malignant intrapericardial germ cell tumor: a case report.

The mediastinum is a common site for germ cell tumors. We describe an unusual case in an 18-month-old girl who presented with respiratory distress and cardiomegaly. An echocardiogram revealed a large pericardial effusion and a mass adjacent to the heart. She underwent detailed investigation and at open exploration was found to have a wholly intrapericardial malignant yolk sac tumor. Staging investigations revealed a bony metastasis. The serum marker alpha-fetoprotein was elevated. She was treated with chemotherapy and is in remission 1 year after completion of treatment. We review the methods of diagnosis and follow-up of children with these tumors.

Transcatheter occlusion of the patent ductus arteriosus with Cook detachable coils.

OBJECTIVE: To report initial experience with a new occlusion device for native and residual patent ductus arteriosus. DESIGN: Descriptive study of consecutive non-randomised patients undergoing a new method of patent ductus arteriosus closure with detachable coils. SETTING: Tertiary centres for paediatric cardiology. PATIENTS: 71 consecutive patients, aged 1.2-22 years, with a patent ductus arteriosus (PDA) underwent elective transcatheter closure. 45 had native PDAs (group A) with a minimum diameter of 1.0 mm-5.0 mm (median 2.0 mm). A further 26 had undergone one or more previous occlusion attempts (group B). INTERVENTIONS: A total of 133 detachable (Cook) spring coils were successfully implanted in 70 patients. The procedure was performed transvenously in 51 patients, retrograde arterially in 13, and by both routes in a further 6 patients. One 5 mm coil migrated but was successfully retrieved. MAIN OUTCOME MEASURES: In group A colour flow Doppler echocardiography showed that complete occlusion was achieved in 40/45 (89%) at 24 hours, 41/45 (91%) at 1 month, and 44/45 (98%) by 6 months post procedure. Occlusion rates in residual PDAs were 22/25 (88%) occluded at 24 hours, 23/25 (92%) at 1 month, and 24/25 (96%) at 6 months follow up. CONCLUSIONS: Transcatheter occlusion using detachable (Cook) spring coils is a safe and effective alternative to presently available devices. The delivery system allows full retrieval of the coil until a satisfactory position is obtained.

[Value of biplane transesophageal echocardiography in congenital abnormalities of the heart atrium and venous-atrial connection]. / Valore dell'ecocardiografia transesofagea biplanare nelle anomalie congenite dell'atrio e della connessione veno atriale.

BACKGROUND: Transesophageal echocardiography with transversal planes offers many advantages in the evaluation of patients with congenital anomalies of atrium, allowing visualization of obscure areas, not visualized with traditional echocardiography, as appendages, venous connections, upper interatrial defects. The aim of this paper is to check what advantages the transesophageal imaging in longitudinal plane, recently insert in biplane probes, might confer over transversal plane imaging in the evaluation of patients with congenital heart disease of atrium and venous connections. METHODS: We carried out a prospective study on 70 patients (49 adults and 22 children) with congenital heart diseases of this area. Fifty-six studies out of 71 were preoperative diagnostic, 15 were carried out in the late postoperative period. Twenty-two studies were performed under general anaesthesia during concomitant cardiac catheterisation, 49 were carried out in outpatient clinic. All the transesophageal diagnoses were subsequently confirmed at either catheterization or surgery. Lesions studied included 36 atrial septal defects (ASD) ostium secundum, 6 interatrial septal aneurysms, 1 ASD coronary sinus, 5 ASD venous sinus, 5 partial and 2 complete atrioventricular septal defects, 1 double outlet right ventricle; in operated patients 2 ASD ostium secundum, 2 ASD sinus venosus, 11 post Mustard or Senning in TGA. RESULTS: Anomalies either better defined or that obtained important additional informations by long axis scanning (vs transverse scanning) included: ASD ostium secundum 36/36, ASD multiple 1/3, caval obstruction in Mustard or Senning 4/5, anterior mitral valve cleft 2/5, left superior vena cava to coronary sinus 3/3. Features visualized by longitudinal plane alone were: ASD coronary sinus 1/1, caval obstruction in sinus venosus operated 1/1, multiple ASD 2/3, coronary artery fistula to right atrium 1/1; for associated features anterior bringing leaflets 2/2, left ventricular outflow tract obstruction 2/2, right ventricular outflow tract obstruction 1/1. Anomalies better characterized by transversal plane versus longitudinal plane were: ASD ostium primum 2/7, ASD sinus venosus sinus 3/5; for associated anomalies VSD inlet 1/1. Features visualized by transversal plane alone were: right upper pulmonary vein in superior vena cava 4/4, right upper pulmonary vein to right atrium 1/1, DIA ostium primum 5/7, ASD sinus venosus 2/5, superior vena cava obstruction in Mustard 1/5, left juxtaposition of the atrial appendages 1/1. The transversal plane alone provided the correct morphologic and hemodynamic diagnosis in 74/88 (84%) (in this group longitudinal plane gave 68% additional informations), the longitudinal planes in 69/88 (78%). However in 11% of cases longitudinal plane was the only means for identifying the congenital lesion and transversal plane in 16%. It was the combination of longitudinal and transverse scanning which gave in 97% the correct complete morphologic diagnosis. CONCLUSIONS: Thus we conclude that both planes are required for optimal transesophageal evaluation of congenital disease of atrium and venous connection.

Medium-term follow up of residual shunting and potential complications after transcatheter occlusion of the ductus arteriosus.

OBJECTIVES: To determine the causes and outcome of residual shunting after transcatheter occlusion of persistent ductus arteriosus with the Rashkind double umbrella occluder, and to determine the potential of the device to produce obstruction to flow in the aorta and left pulmonary artery. DESIGN: Angiographic examination of morphology of ductus followed by prospective clinical and ultrasound evaluation (including cross sectional imaging, colour flow mapping, and pulse wave Doppler) of all patients undergoing occlusion of persistent ductus arteriosus between October 1987 and July 1992. PATIENTS: 140 patients with ages between 0.5 and 78 (median 3.8) years and weights between 6.8 and 74 (median 13.8) kg. INTERVENTIONS: Attempted implantation of the Rashkind double umbrella ductus occluder under angiographic control through a transvenous (n = 136) or transarterial (n = 4) approach. MAIN OUTCOME MEASURES: Successful occlusion of ductus; frequency, pattern, and prognosis of residual shunts; Doppler velocities in left pulmonary artery and aorta; volume loading of the left heart. RESULTS: Including reocclusions the overall rate of successful occlusion was 96%. A total of six devices embolised at the time of operation (4.3%) with no sequelae. There were no anatomical factors that predicted a poor outcome, but suboptimal positioning of the device led to a significantly higher incidence of residual shunts (p < 0.001). Colour flow mapping correctly identified shunts that were unlikely to close spontaneously (n = 9) and to date seven have undergone successful closure with a second device. Encroachment of device legs produced statistically (p < 0.001) but not clinically significant increases in left pulmonary artery Doppler velocities that diminished with time. CONCLUSIONS: Transcatheter occlusion provides a safe and effective means of closing a persistent ductus arteriosus. Doppler colour flow mapping is necessary for follow up and shows those ducts requiring reocclusion. The device did not produce significant disturbance to flow in the pulmonary arteries or aorta.

Cardiac dysfunction in paediatric HIV infection.

A child under long term surveillance for vertically acquired HIV-I infection developed respiratory symptoms. Dilated cardiomyopathy was diagnosed. Clinical and echocardiographic assessment of six other children with symptomatic HIV disease showed no evidence of cardiac dysfunction. The exact prevalence of this complication in paediatric HIV infection is unknown, but the potential diagnostic difficulties are highlighted.

Morphological variations of fossa ovalis atrial septal defects (secundum): feasibility for transcutaneous closure with the clam-shell device.

OBJECTIVE--To assess the morphology of fossa ovalis atrial septal defects (FOASD; secundum atrial septal defect) to determine in what proportion percutaneous closure with the clam-shell device might be feasible. DESIGN--Review of the intraoperative description of the morphology and size of FOASD. PATIENTS--106 consecutive patients with FOASD. SETTING--A regional children's cardiac referral centre. MAIN OUTCOME MEASURES--Determination of morphology and size of FOASD. RESULTS--Morphological variations in the FOASD were found: (1) central FOASD, 70 (66%); (2) superior FOASD, 4 (3.7%); (3) inferior FOASD, 8 (7.6%); (4) posterior FOASD, 2 (1.9%); (5) subtotal absence of atrial septum, 3 (2.8%); (6) completely fenestrated FOASD, 10 (9.4%); (7) partially fenestrated FOASD, 9 (8.5%). Depending on its site, the edge of the FOASD may be close to important atrial structures such as the orifices of the superior and inferior caval veins, coronary sinus, and right pulmonary veins. The shape of the FOASD was usually oval with the major diameter ranging from 10 to 50 mm with a mean (SD) of 27.8 (0.93) mm. The minor diameter ranged from 4 to 30 mm with a mean (SD) of 15.3 (5.9) mm. The ratio of the major to minor diameter ranged from 1.0 to 5.0 with a mean of 2.01. Based on intraoperative measurement and description, it is postulated that it should be possible to close about 50% of these defects with the clam-shell device.

Pulmonary venous obstruction following correction for total anomalous pulmonary venous drainage: a challenge.

Pulmonary venous obstruction after surgical correction of total anomalous pulmonary venous drainage (TAPVD) is a serious condition. Pulmonary venous obstruction can be the result of a primary developmental error or is due to post-operative anastomotic stricture and is usually manifest within 6 months of surgery. Prompt restudy is indicated and if a stricture is present, urgent surgical relief is indicated. However, the results are often disappointing with a high early mortality and a significant chance of restenosis.

Transesophageal echocardiographic monitoring of interventional cardiac catheterization in children.

Transesophageal echocardiography was used prospectively in 22 children scheduled for interventional cardiac catheterization (9 with pulmonary valvuloplasty, 5 with aortic valvuloplasty, 1 with pulmonary angioplasty, 2 with aortic angioplasty, 2 with patent ductus arteriosus occlusion and 3 with Mustard baffle dilation) to determine its potential value as a monitoring technique. The patients ranged in age from 0.9 to 14.6 years (mean 5.4) and in weight from 9.5 to 49.2 kg (mean 21.1). Studies were completed in all patients without complications. Preintervention studies provided important new information in two patients, leading to cancellation of the planned procedure. Major contributions of transesophageal monitoring included 1) a real time assessment of catheter placement across either atrioventricular valve and the aortic valve during balloon valvuloplasty; 2) immediate assessment of aortic valve and aortic wall morphology during balloon dilation; and 3) detailed morphologic and hemodynamic information together with enhanced catheter guidance during Mustard baffle dilation. After pulmonary valvuloplasty, partial chordal rupture of the tricuspid valve was documented in one patient. In two patients, balloon catheter position was modified according to the transesophageal findings. The assessment of changes in pulmonary valve morphology and transcatheter occlusion of a patent ductus arteriosus was not enhanced by single-plane transesophageal monitoring. Pulsed wave Doppler studies contributed additional information in the assessment of immediate hemodynamic changes after interventional procedures. Transesophageal echocardiography is a new important guiding and monitoring technique during interventional cardiac catheterization procedures in children. It can provide additional real time imaging information, immediate identification of complications and assessment of hemodynamic changes.

Life threatening 'epilepsy'.

A 5 year old girl presenting with episodes of sudden loss of consciousness was found to have intermittent ventricular tachycardia and, on one occasion, self limiting fibrillation. Corrected QT interval was normal. After several therapeutic measures clinical and electrocardiographic improvement was achieved by administration of sotalol.

Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure.

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)