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Late cardiovascular morbidity and mortality remain significant despite apparently successful surgical repair of aortic coarctation. Alterations in cardiac function have been reported in normotensive patients who have had successful repair, the reasons for which remain unclear. This study addresses the relation between ambulatory blood pressure measurements and alterations in left ventricular performance in 20 patients with normotension at rest after successful repair of aortic coarctation. Exercise testing, ambulatory blood pressure monitoring and two-dimensional echocardiographic studies in 13 boys and 7 girls (mean age 14.2 ± 2.31 and 14.7 ± 3 years, respectively) who had no evidence of recoarctation were compared with the findings in 20 matched control subjects.No difference was found in systolic blood pressure at rest or peak exercise between patients and control subjects. Male patients developed a significant arm/leg gradient at peak exercise. Systolic ambulatory blood pressure was higher throughout the day in the male group. In the female group, systolic blood pressure was higher only during sleep. No difference was found in diastolic blood pressure or heart rate. The transverse aortic arch was smaller and the left ventricular mass greater in all patients. The relation of wall stress to rate-corrected velocity of shortening was 2 SD above normal in 8 of the 20 patients, suggesting that some have enhanced contractility. The E/A ratio on the atrial echocardiogram was significantly reduced in the patient group.Successfully treated patients who are normotensive at rest after operation are still at risk for developing end organ damage, which is probably explained by incipient mild hypertension documented by ambulatory blood pressure monitoring.

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 geneticsts. 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. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.

Cross-sectional echocardiography has made a major contribution to the preoperative evaluation of the patient with an atrioventricular septal defect,1−5such that in many instances this modality has been the sole imaging technique used to formulate a medical or surgical plan6. With the advent of Doppler echocardiography additional hemodynamic information is now available, which enables the cardiologist and surgeon to evaluate the site and magnitude of associated atrioventricular valvar regurgitation, and obtain an accurate measurement of pulmonary arterial pressure along with an assessment of outflow tract obstruction, if present.

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 arteriosi 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.

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.

Background: Noncompaction of the left ventricular myocardium is an important cause of cardiomyopathy. There is no clear consensus about its diagnostic criteria or the diagnostic test of choice. MRI is increasingly used in the pediatric cardiac field because of its superior and objective image quality. Objective: To compare the echocardiographic and MRI findings in four patients with recently diagnosed ventricular noncompaction. Materials and methods: We compared the extent of myocardial involvement shown at MRI and echocardiography in four individuals, two patients with echocardiographic diagnosis of left ventricular noncompaction, and two family members of one of the patients. Results: In all patients, MRI showed wider area of involvement than echocardiography. A definite diagnosis was entertained in only two patients by echocardiography but in all by MRI. Cine imaging was diagnostic of the disease in all patients. Black-blood pool imaging with double-inversion recovery sequence also helped to visualize the abnormal areas by showing slow flow artifacts in the four- and two-chamber images. Conclusion: MRI provided better delineation of the extent of the abnormal trabeculation in patients with noncompaction of the left ventricular myocardium. It was particularly useful when the myocardial involvement was subtle, as in the asymptomatic family members.

Isolation of a branch pulmonary artery from ductal closure is an unusual finding in patients with tetralogy of Fallot. A case report of a newborn is presented where the closing arterial duct was balloon-dilated and stented to reestablish blood supply to the affected lung as a strategy of improving systemic saturations and promoting the growth of the hiliar branch pulmonary artery. Five months after the initial palliation, complete surgical repair with stent removal was successfully achieved.

The application of ultrasound for the evaluation of the newborn possesses the distinct advantage of being non-invasive at a time of life when the patient is fragile because of the combined effects of illness, small body size and physiological immaturity. It is therefore not surprising that echocardiography has found a distinct and unique niche in the evaluation of the newborn suspected of having heart disease. Thus as technological advances such as high resolution high frequency transducers and color flow mapping have been developed, so have the application of these technologies widened the diagnostic capabilities of echocardiography in the neonate. This chapter will outline the contribution of echocardiography and Doppler to the imaging and hemodynamic evaluation of the newborn with structural heart disease or cardiac dysfunction of other etiologies, and indicate those areas which pose particular difficulties for the echocardiographer and require the use of other imaging modalities prior to palliative or corrective newborn cardiac surgery.

BACKGROUND Percutaneous closure of the persistently patent ductus arteriosus with the Rashkind prosthesis is an established effective therapeutic modality, although some patients are left with residual shunting. To evaluate this, a retrospective study of the prevalence of persistent shunting over a 40-month period in the first 190 patients was undertaken. METHODS AND RESULTS All patients (male 45, female 145; mean age, 3.9 +/- 3.6 years; range, 5 months to 20 years) had serial clinical and color-flow echocardiographic follow-up at 6-12-month intervals (range, 6-40 months). Four patients required surgical removal of an embolized device, leaving a cohort of 186 patients in whom 196 procedures were performed, resulting in successful placement of 195 devices (43 17-mm [22%] and 152 12-mm [78%]). Complications occurred in seven of 195 procedures (3.6%). Nine of 10 attempted reocclusions (all with 12-mm devices) were successful. The prevalence of residual shunting was 38% at 1 year, 18% at 2 years, and 8% at 40 months. Patients with ductus measuring less than 4 mm had a higher success of initial occlusion. Thirty-four patients were left with residual shunting determined by color-flow Doppler study, but no anatomic or echocardiographic features were found predictive for residual shunting. All remain asymptomatic with 26 (76%) having no detectable murmur, two (6%) a continuous murmur, and six (18%) a systolic murmur. CONCLUSIONS Catheter occlusion will obviate the need for surgery in the majority of patients presenting with persistently patent ductus arteriosus. Reocclusion has been found feasible in those with continuous murmurs (nine of nine) and should be offered early because it is unlikely for spontaneous closure to occur in this group. It appears prudent to follow those with small residual shunting because further spontaneous closure can occur.

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.

High-flow cerebral arteriovenous fistulae are well known to present in the neonatal period with macrocephaly, cranial bruits and high-output cardiac failure. This report describes a newborn infant with such a clinical presentation, who had unilateral megalencephaly without macroscopic arteriovenous shunts. Ultrasound Doppler examination of the carotid and cerebral vessels showed diastolic flow, or a decreased pulsatility index, consistent with decreased intracranial vascular resistance. The ipsilateral cerebral arteries and veins were markedly enlarged at angiography and at post-mortem examination, but there was no arteriovenous malformation. The infant expired from high-output cardiac failure and hypoglycemia. It is postulated that the high-output cardiac failure was due to increased blood flow through the enlarged, dysplastic cerebral hemisphere.

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)

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.

Staphy lococcus aureus has become a more impor tan t pa thogen in both hospitaland communi ty-acqui red infections over the last decade. ~ The exact incidence of endocarditis in S. aureus septicemia remains controversial, with est imates ranging from as high as 60% in early reports to less than 10% in recent reviews. 2 S. aureus sept icemia in chi ldhood and adolescence is par t icular ly devastat ing when it occurs in previously heal thy children, ~,4 in whom it usually begins as a "flulike" illness. In association with this entity, endocardit is almost always involves the left side of the hear t , and occurs in the absence of the classic signs usually associated With subacute bacterial endocardit is , 1-5 making diagnosis difficult. S. aureus endocardit is involving the tr icuspid valve has been described only once before in a child with a normal heart . 6 We describe two chi ldren (aged 5 and 8 years), with previously normal hear ts and no evidence of immunodeficiency, with tricuspid valve endocarditis.

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 < 0.001), right and left subclavian arteries (p < 0.001) and left common carotid artery (p < 0.05). The thoracic descending aorta was, however, significantly smaller in patients (p < 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

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.

Despite the trend toward earlier total surgical correction of tetralogy of Fallot, a number of complicating factors, most often related to unfavorable pulmonary arterial anatomy, 1 and, rarely, reduced left ventricular size, 2 may make palliation with a systemic to pulmonary arterial anastomosis necessary before corrective surgery. A restrictive ventricular septal defect (VSD) complicating tetralogy of Fallot is one of the less well-recognized features associated with a high mortality when surgical repair is delayed beyond infancy. 3–11 The echocardiographic recognition of this anomaly should be of importance because of the trend toward use of noninvasive diagnostic modalities in infants with various forms of congenital heart disease.12 This study reviews echocardiographic features of tetralogy of Fallot in patients with a documented restrictive VSD, and examines the contribution of clinical findings to the diagnosis of this entity. The progression over time of VSD restriction and its impact on surgical management are also assessed.

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.

Direct visualization of an anomalous left coronary artery from the pulmonary trunk (ALCA) is not always possible by cross-sectional echocardiography (CSE). By contrast, a large right coronary artery (RCA) in ALCA can usually be seen. We reviewed the diagnostic value of the RCA diameter measurement by CSE in the differentiation between ALCA and dilated cardiomyopathy (DCM) of other etiology. In 28 controls, RCA increased with age; RCA(mm) = 0.781 log(months) + 0.639 (r=0.829, SEE=0.306), but RCA/aortic root ratio (RCA/AO) showed no variation with age (11.9±2.0%). Morphometric criteria for ALCA was made as RCA larger than the 95% confidence limit of normal or RCA/AO larger than 17%. Diagnostic criteria of (1) direct visualization of ALCA, (2) diastolic flow in pulmonary artery by Doppler echocardiography and (3) RCA morphometry were assessed in 10 patients with ALCA (3-105 mos; median 8.5 mos) and 11 with DCM (2-113 mos; median 7 mos) by blind observers. RESULT: There were no false positive diagnoses of ALCA. Sensitivity was 40% by (1); 40% by (2); 70% by (3); 60% by (1+2); and 80% by (1+3). Interobserver agreement was assessed in 18 patients and was significantly concordant: r=0.934 for RCA and r=0.905 for RCA/AO. CONCLUSION: Careful visualization and measurement of RCA can accurately confirm a diagnosis of ALCA.

Measurement of pulmonary artery pressure(PAP) in preterm infants would be valuable in monitoring their clinical course. The presence of a patent ductus arteriosus (PDA) in this setting provides access to the pulmonary vascular bed by non-invasive means. This study addresses the reliability of Doppler derived (DD) mean, systolic and diastolic pressure measurements in 2 groups of children (Group 1: n=15, age 3.5±4.0yrs, mean PAP 33±16; Group 2 n=5, age 0.6±0.1yrs, mean PAP 77±15) undergoing simultaneous cardiac catheterization. The influence of ductal size on pressure measurement was also assessed. Ductal size (1-9 mm) did not affect results. Pure left to right shunting was noted in group 1, while bidirectional shunting occurred in group 2. Three patients with near systemic PAP showed no significant DD change with 100% oxygen or vasodilator infusion, consistent with measured PAP. PAP can be predicted under varying hemodynamic states by Doppler measurement of PDA shunt velocities.