Echocardiographic follow-up of atrial septal defect after catheter closure by double-umbrella device.

BACKGROUND: Transcatheter device occlusion of atrial septal defects is an attractive approach, but its efficacy and place in patient management remain to be determined. METHODS AND RESULTS: To evaluate the medium-term results of atrial septal defect device occlusion and factors influencing residual shunting, transesophageal and transthoracic echocardiograms of 49 patients were reviewed. Transesophageal echocardiograms on 48 patients immediately following surgical closure revealed residual shunting in 2% compared with 91% after device occlusion; this proportion decreased to 53% after a mean follow-up of 10 months. The actuarial analysis suggests a progressive resolution of shunting with time. One patient had residual shunting by transesophageal echocardiography immediately after surgical closure compared with 29 after a mean follow-up of 10 months after device occlusion. Residual shunting was not influenced by (1) dimension, location, or position with relation to the device as assessed by transesophageal echocardiography; (2) location of the defect; or (3) device size relative to the stretched dimension of the defect. In 15 patients, a poor correlation existed between transesophageal and transthoracic echocardiographic findings. Variability in serial transthoracic echocardiographic findings was observed in 14. Right ventricular dimension, heart size, and presence of a murmur at follow-up did not correlate with the presence or size of residual shunting after device occlusion. CONCLUSIONS: These results suggest that ongoing spontaneous resolution of residual shunting occurs after device insertion. Factors related to the defect or device could not predict eventual resolution of residual shunting. Transthoracic echocardiography in the follow-up of these patients may not be reliable in determining presence of residual shunting.

Tetralogy of Fallot in three siblings: a familial study and review of the literature.

We report a family in which three out of four siblings had tetralogy of Fallot (TOF). The family history showed TOF in the daughter of a maternal cousin, while no other congenital heart diseases were discovered. Although no teratogenic environmental agent was discovered, the absence of parental consanguinity and the presence of another affected relative suggest multifactorial inheritance. Autosomal recessive inheritance cannot be ruled out.

Spectrum of cardiovascular anomalies in Williams-Beuren syndrome.

This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2:1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticists. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10-40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic regurgitation during systole: color flow mapping and Doppler interrogation following the Damus-Kaye-Stansel procedure.

Echocardiographic evidence of systolic aortic regurgitation following a Damus-Kaye-Stansel procedure for palliation of complex double-outlet right ventricle is presented. This procedure directs left ventricular output to the aorta through a proximal main pulmonary artery-aortic anastomosis and utilizes a valved conduit between the right ventricle and distal pulmonary artery. Postoperative Doppler and color flow echocardiographic findings revealed systolic and diastolic regurgitation from the native aorta to the right ventricle. Aortic valve closure at the time of the original Damus-Kaye-Stansel procedure would eliminate regurgitant flow and circumvent subsequent closure of this valve due to increased systolic aortic regurgitation.

Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18.

To investigate the role that cardiac anomalies play in the early death frequently seen in the trisomy 13 and the trisomy 18 syndromes, two-dimensional and Doppler echocardiograms from 31 newborn infants with cytogenetic confirmation of these syndromes seen at one institution over a 4.5 year period (1983 to 1988) were reviewed. The mean age at echocardiography was 1.5 days, and the median age at death was 14 days. Significant cyanosis was present in 58%. Cardiac anomalies that would be considered lethal within the neonatal period were present in only 19% of patients. The most common lesions were atrial septal defect (81%), ventricular septal defect (61%) and patent ductus arteriosus (85%). Most ventricular septal defects and patent ductus arteriosus were large. Valvular dysplasia of one or more valves, graded as mild in most cases, was found in 68%, but was not associated with Doppler evidence of significant regurgitation or stenosis in any subject. Of the four valves, the pulmonary valve, followed by the tricuspid valve, was the most commonly dysplastic. Doppler evidence suggestive of elevated pulmonary artery pressure (low velocity bidirectional flow across the ventricular septal defect and patent ductus arteriosus), although expected, was accompanied by greater than normal mean right ventricular cavity and free wall dimensions in these patients. Thus, although the cardiac anomalies most frequently encountered in trisomy 13 and trisomy 18 are nonlethal, the combined findings of frequent cyanosis and increased right ventricular dimensions suggest that other factors such as pulmonary hypertension, perhaps related to maldevelopment of the pulmonary vasculature, may contribute to early death in some of these infants.

Doppler echocardiographic measurement of pulmonary artery pressure from ductal Doppler velocities in the newborn.

The ductal flow velocities in 37 newborns (group 1: persistent pulmonary hypertension [n = 16], transient tachypnea [n = 3], other [n = 2]; group 2: respiratory distress syndrome [n = 16]) were prospectively evaluated by Doppler ultrasound for the purpose of deriving systolic pulmonary artery pressures. Maximal tricuspid regurgitant Doppler velocity in 21 of these patients was used to validate the pulmonary artery pressures derived from ductal flow velocities. There was a significant linear correlation between tricuspid regurgitant Doppler velocity and pulmonary artery systolic pressure derived from ductal Doppler velocities in patients with unidirectional (pure left to right or pure right to left) ductal shunting (p less than 0.001, r = 0.95, SEE 8) and in those with bidirectional shunting (p less than 0.001, r = 0.95, SEE 4.5). Systolic pulmonary artery pressure in group 1 (67 +/- 13 mm Hg) was significantly higher than that in group 2 (39 +/- 10 mm Hg) (p less than 0.001). In those with bidirectional shunting, duration of right to left shunting less than 60% of systole was found when pulmonary artery pressure was systemic or less, whereas duration greater than or equal to 60% was associated with suprasystemic pulmonary artery pressures. Serial changes in pulmonary artery systolic pressure, reflected by changes in ductal Doppler velocities, correlated with clinical status in persistent pulmonary hypertension of the newborn. Persistently suprasystemic pulmonary artery pressure was associated with death in five group 1 patients. It is concluded that ductal Doppler velocities can be reliably utilized to monitor the course of pulmonary artery systolic pressures in newborns.

Two-dimensional echocardiographic and color flow Doppler evaluation of ductal occlusion with the Rashkind prosthesis.

To evaluate the results of ductal occlusion with the Rashkind prosthesis, 78 children (group 1, 19 boys and 59 girls; mean age at occlusion, 4.5 +/- 4.0 years) with isolated patent ductus arteriosus (n = 73) or in association with other lesions (n = 5) were evaluated by pulsed and color flow Doppler 9 +/- 7 months (range, 2-26 months) after occlusion. Thirty children who had undergone patent ductus arteriosus ligation (group 2, 9 boys and 21 girls; mean age at study, 5.7 +/- 4.9 years; mean follow-up after ligation, 44 +/- 58 months) were evaluated in the same way. The prevalence of residual ductal shunting and the main pulmonary arterial flow patterns were recorded. Residual ductal shunting in group 1 was 38% on day 1, decreasing slowly to 31% at 3 months, 27% at 6 months, and 19.7% at 1 year or more due to further spontaneous shunt resolution. The residual shunting rate in group 2 (6%) was significantly lower than that at 1 year or more in group 1 (p less than 0.001). Successful reocclusion in 5 of 6 in a subset of patients in group 1 followed for 1 year or less reduced further the prevalence of residual shunting. Residual shunting after patent ductus arteriosus occlusion is more common than after ligation, but continues to decrease during follow-up.

Distinguishing between anomalous origin of the left coronary artery from the pulmonary trunk and dilated cardiomyopathy: role of echocardiographic measurement of the right coronary artery diameter.

Patients with anomalous origin of the left coronary artery from the pulmonary trunk usually have a large right coronary artery. This study examines the diagnostic value of measuring the diameter of the right coronary artery by echocardiography in distinguishing between this lesion and other causes of dilated cardiomyopathy. The diameter of the right coronary artery and the right coronary artery/aorta ratio were measured in the parasternal short axis view in 40 controls, 11 patients with dilated cardiomyopathy, and 10 with anomalous origin of the left coronary artery from the pulmonary trunk. In the controls, the diameter of the right coronary artery increased with age, but the right coronary artery/aorta ratio remained constant. In the control group the 95% upper limits of prediction for right coronary artery diameter were 1.6 mm for one month of age, 1.8 mm for three months, 2.0 mm for one year, 2.2 mm for two years, 2.4 mm for three years, 2.6 mm for four years, 2.7 mm for six years, 3.0 mm for eight years, and 3.2 mm for 10 years; and for right coronary/aorta ratios the limits were 0.17 for one month to one year, 0.18 for one to six years, 0.19 for six to 10 years, and 0.20 for more than 10 years. All patients with dilated cardiomyopathy had normal right coronary artery diameters and right coronary artery/aorta ratios (0.10-0.13). Those patients with anomalous origin of the left coronary artery from the pulmonary trunk had larger than normal right coronary artery diameter and a significant increase in the right coronary artery/aorta ratio (0.21-0.29). The presence of an anomalous left coronary artery was likely if the diameter of the right coronary artery or the right coronary artery/aorta ratio was larger than the normal 95% limits of prediction.

Echocardiographic and Doppler evaluation of the aortic arch and brachiocephalic vessels in cerebral and systemic arteriovenous fistulas.

Congenital arteriovenous fistulas presenting in the newborn period pose difficult diagnostic problems and simulate structural heart disease. Angiocardiography, when performed, demonstrates enlarged brachiocephalic vessels and rapid cerebral venous return. The value of echocardiographic imaging and measurement of the aortic arch and brachiocephalic vessels, and evaluation of the Doppler flow profile in these vessels as a means of making a rapid diagnosis of cerebral or thoracic arteriovenous fistula, was therefore assessed in 10 infants with these diagnoses seen over a 4 year period (1983 to 1987). Twenty-nine infants (median age 6 weeks) undergoing two-dimensional echocardiography but with no significant lesions were prospectively selected as controls. Nine of the 10 patients had congestive heart failure at presentation (mean age 2 days). A cranial bruit was heard in three and arteriovenous fistula was suspected in five patients. Aortic arch segments and brachiocephalic vessel dimensions expressed as ratios of the abdominal aorta showed significantly larger values in patients for the ascending aorta (p = 0.01), innominate artery (p less than 0.001), right and left subclavian arteries (p less than 0.001) and left common carotid artery (p less than 0.05). The thoracic descending aorta was, however, significantly smaller in patients (p less than 0.002). Retrograde diastolic Doppler flow in the descending aorta proximal to the ductus arteriosus and anterograde diastolic flow with a mean spectral flow-time integral 27% of systolic were present in patients only, whereas Doppler diastolic flow in brachiocephalic vessels, present in 5 of 29 control infants, was less than 15% of systolic flow and not accompanied by dilation of these vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiopulmonary adaptation at rest and during exercise 10 years after Mustard atrial repair for transposition of the great arteries.

Discordance exists between apparently reduced systemic right ventricular function and the reported asymptomatic state of many patients after atrial repair for transposition of the great arteries. To evaluate this clinical observation, cardiopulmonary response to exercise in 17 asymptomatic patients with no significant postoperative hemodynamic abnormalities was assessed by upright bicycle ergometry according to a modified Jomes protocol 11.5 +/- 1.5 years after Mustard atrial repair. Seventeen age- and sex-matched normal adolescents constituted the control group. Incremental exercise was performed to determine maximum work capacity, heart rate, blood pressure, oxygen saturation, and minute ventilation. Cardiac output was computed at rest and during steady-state exercise by a carbon dioxide rebreathing method at 50% of the maximum workload achieved during incremental exercise. Height and weight were similar in patients and controls (p greater than .05). Resting pulmonary function variables were normal in all subjects. At peak exercise, respiratory quotient was greater than 1 in both patients and controls (1.12 +/- 0.09 and 1.09 +/- 0.08 respectively, p greater than .05). Patients achieved a lower peak heart rate (172 +/- 14 vs 185 +/- 11 beats/min, p less than .01), lower maximum work capacity (2.3 +/- 0.6 vs 3.3 +/- 0.7 W/kg, p less than .01). The ratio of minute ventilation at peak exercise to maximum resting voluntary ventilation was normal (less than or equal to 80%) in both groups. The ventilatory equivalent for oxygen was similar at rest, but significantly higher in patients than in control subjects at peak exercise (42 +/- 7 vs 36 +/- 5, p less than .006).(ABSTRACT TRUNCATED AT 250 WORDS)

Validation of Doppler-derived pulmonary arterial pressure in patients with ductus arteriosus under different hemodynamic states.

Twenty-nine patients with a patent ductus arteriosus (PDA) in isolation (n = 17) or in combination with other lesions (n = 12) underwent simultaneous hemodynamic assessment and evaluation of PDA flow velocity by the Doppler method. The accuracy with which Doppler velocity across the PDA predicted pulmonary arterial pressure and the influence of PDA size and shape on the Doppler velocity-pressure relationship were examined. Seventy percent had a cone-shaped PDA (narrowest at the pulmonary artery end), and the remainder were tubular. Narrowest PDA diameter ranged from 1.5 to 9 mm (mean 3.5 mm). Peak systolic and mean pulmonary arterial pressure ranged from 10 to 116 and 8 to 72 mm Hg, respectively. Twenty-one patients (group 1) had left-to-right shunting only. The following variables showed significant correlation in this group: peak instantaneous systolic aortic-to-main pulmonary arterial (MPA) pressure gradient and maximum Doppler velocity across the PDA (slope = 1.03, SEE = 13 mm Hg, r = .94, p less than .001), mean aortic-to-MPA pressure gradient and mean Doppler velocity (slope = 1.06, SEE = 10 mm Hg, r = .95, p less than .001), and end diastolic aortic-to-MPA pressure gradient and minimum Doppler velocity (slope = 1.12, SEE = 8 mm Hg, r = .96, p less than .001). Eight patients (group 2) had bidirectional shunting. In this group peak instantaneous aortic-to-MPA pressure gradient significantly correlated with maximum Doppler velocity measured from the left-to-right shunt (slope = .70, SEE = 2 mm Hg, r = .92, p less than .002) and mean pressure gradient correlated with mean Doppler velocity (slope = .83, SEE = 3 mm Hg, r = .78, p less than .003). Right-to-left Doppler velocities showed no correlation with pressures. In six patients with pulmonary hypertension Doppler velocity changes accurately predicted the effect of pulmonary vasodilation on pulmonary arterial pressure. Doppler velocity of PDA flow reliably predicts pulmonary arterial pressure over a wide range of pressures and PDA shapes and sizes.

The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.

Adult respiratory distress syndrome in a child with acute epiglottitis.

Intubation of a 23-month-old child suffering epiglottitis was complicated by pulmonary edema progressing to the adult respiratory distress syndrome. Increased pulmonary shunt and pulmonary hypertension required high levels of positive end-expiratory pressure and prolonged mechanical ventilation to correct hypoxemia. A possible role for a cuffed endotracheal tube in epiglottitis is discussed.