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.

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)

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.

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.

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.

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.

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.

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.

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.

Functional and anatomical correlates in atrial septal defect. An echocardiographic analysis.

The results of cross sectional echocardiography, intracardiac contrast echocardiography, and balloon sizing techniques and conventional haemodynamic assessment were correlated in 40 consecutive patients evaluated for an isolated left to right shunt at atrial level. Echo free areas along the septum were identified in 23 of 25 patients with a secundum defect, but not in two with a fenestrated defect, and in the upper atrial septum in three of four patients with a sinus venosus defect. No false positive results occurred in 11 patients with a probe patent foramen ovale. Saline contrast injection into the left atrium showed significant left to right shunting in all patients with atrial septal defect; inferior vena caval injection produced right to left shunting in 15 of 29 patients and a negative contrast effect in eight of 29 patients with an atrial septal defect, although neither correlated quantitatively with defect diameter or magnitude of the left to right shunt. Echocardiographic assessment of defect size as small, moderate, or large showed a highly significant correlation with balloon measurement of defect diameter, although some overlap between the groups was evident. In contrast, the correlation between defect diameter and pulmonary to systemic blood flow ratio was poor, mainly because of highly variable shunting in patients with an anatomically large defect. Cross sectional echocardiography has high sensitivity and specificity in the diagnosis of the non-fenestrated atrial septal defect and provides quantitative information about defect diameter. Contrast studies do not add to the diagnostic value of imaging from the subcostal position. The poor correlation between defect size and the measured shunt suggests that the latter may not be the best criterion for surgical management and that size could be an important factor likely to influence both the long term prognosis and the decision for closure.

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.

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.

Atrial flutter in the newborn resulting from maternal lithium ingestion.

We report a case of isolated atrial flutter in a neonate, attributable to maternal lithium treatment, and suggest that the assessment of all infants born to mothers on lithium treatment during pregnancy should include an electrocardiogram.

The silent ductus arteriosus in idiopathic respiratory distress syndrome.

Among 425 pre-term low birthweight babies who survived more than 48 hours there were 130 with idiopathic respiratory distress syndrome, 73 of whom received ventilation for their disease. The ductus arteriosus was considered patent by clinical criteria in 41 of these babies, all but 1 of whom had a murmur. Of the remaining 32 infants there were 2 babies only who died and both were found to have a patent ductus arteriosus which had not been detected clinically. Another baby died whilst being ventilated for idiopathic respiratory distress syndrome and although autopsy information is not available it seems likely that factors other than a patent ductus arteriosus caused death. The mean age of murmur detection in those ventilated infants considered to have a patent ductus arteriosus was 6 days (range 2-19). Infants without a murmur or other features of a ductus arteriosus did not require to be ventilated beyond day 7 with the exception of the 2 fatalities already mentioned. Thus, relying on clinical criteria and particularly on the presence of a murmur, if sought often, rarely results in missing or seriously delaying the diagnosis of patent ductus arteriosus in babies with severe idiopathic respiratory distress syndrome.

Truncus arteriosus: successful surgical correction without the use of a valved conduit.

A new operation for the restoration of continuity between the right ventricle and pulmonary circulation in truncus arteriosus in infancy without the use of a tube graft is described. This was achieved by anastomosing the inferior margin of the detached confluent branch pulmonary arteries to the apex of a vertical infundibular ventriculotomy to form the posterior wall of the reconstructed right ventricular outflow tract. A patch of pericardium was used to form the anterior wall and complete the primary cardiac repair.

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.

Superior QRS axis in ventricular septal defect.

The relation between the superior orientation of the QRS axis and the anatomical site of the defect in the ventricular septum in patients with a ventricular septal defect was studied. Of 1031 patients with a ventricular septal defect, 64 (6.2%) had a superior axis on their electrocardiogram. In 59% of these patients the defect was in the inlet portion of the ventricular septum or affected this area. None of the cases was classified as an isolated ventricular septal defect of the persistent atrioventricular canal type. While there is an association between a superior axis and perimembranous inlet ventricular septal defect, a superior QRS axis does not characterise a ventricular septal defect as being of the atrioventricular canal type.