Luciana T. Pagotto

Quantitative Assessment of Ventricular Function in Children With Single Ventricles Using the Doppler Myocardial Performance Index

Assessment of ventricular function in patients with functionally single ventricles and unusual ventricular geometry is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to compare the MPI in normal children and in children with a functionally single ventricle, and to compare the MPI in patients with single ventricles before and after bidirectional cavopulmonary anastamosis (BCPA). Echocardiograms of all patients who underwent BCPA between January 1994 and December 1998 were reviewed before and after surgical palliation. Age-matched normal subjects (n = 30) served as controls. The MPI was calculated from Doppler tracings of ventricular inflow and outflow. Of the 60 patients with single ventricles who underwent BCPA, 41 had adequate preoperative Doppler studies to allow calculation of the MPI, and 21 of 41 had adequate studies before and after BCPA. The MPI was higher in patients with single ventricles than in normal controls (0.67 +/- 0.14 vs 0.32 +/- 0.10, p < 0.001). There was no significant difference in the MPI in patients with single ventricles before and after surgery (0.70 +/- 0.16 vs 0.66 +/- 0.15). However, patients who had a BCPA at < 1 year of age had a significant decrease in the MPI after surgery (0.71 +/- 0.10 vs 0.61 +/- 0.11, p = 0.01). Compared with controls, the MPI was significantly higher in patients with single ventricles suggesting an altered hemodynamic state consistent with decreased ventricular function. The MPI decreased in patients who underwent BCPA at < 1 year of age, suggesting an improvement in ventricular function. The MPI provides an objective method of assessing and following ventricular function in patients with single ventricles that is independent of ventricular geometry.

Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable?

OBJECTIVE Although the influence of small left heart structures on outcome of a biventricular repair in neonatal critical aortic stenosis is well documented, little is known about its effect in neonates with aortic arch obstruction and coarctation. The purpose of this study was to evaluate the influence of small left heart structures on early and late results of repair and the ability to achieve a biventricular repair in neonates with coarctation and aortic arch obstruction. PATIENTS Neonates included in this study had a left ventricular adequacy score (as proposed by Rhodes and associates for critical aortic stenosis) that would have predicted a need for a univentricular (Norwood) repair. All were ductus dependent but had antegrade ascending aortic flow and a small but nonstenotic aortic valve (<30 mm Hg gradient). Twenty neonates aged 10 +/- 9 days were identified for the study with weights averaging 3. 1 +/- 0.6 kg. Selected left heart measurements obtained by preoperative echocardiography included the following: aortic anulus 5.3 +/- 0.3 mm, mitral anulus 8.4 +/- 1.0 mm, transverse aortic arch 3.4 +/- 0.6 mm, and left ventricular volume 25 +/- 4 mL/m2. All patients underwent coarctation repair by resection and extended end-to-end anastomosis to enlarge the transverse arch as needed. Three patients underwent simultaneous pulmonary artery banding because of a hemodynamically significant ventricular septal defect. These 3 patients have subsequently had their defects successfully closed without mortality. RESULTS There were no early or late deaths at a follow-up of 38 +/- 16 months after the operation. Three patients (3/20, 15%) have had to undergo reintervention with balloon aortoplasty because of recurrent coarctation (gradient > 20 mm Hg) in 2 and resection of subaortic stenosis in 1. Late follow-up in the remaining patients reveals 1 with moderate subaortic stenosis (gradient = 43 mm Hg), 2 with mild aortic stenosis (gradient < 30 mm Hg), and 2 with mild to moderate mitral stenosis. At late follow-up, 16 patients (16/20, 80%) are completely free of symptoms and 4 (4/20, 20%) have mild residual symptoms. CONCLUSIONS Biventricular physiology can be successfully achieved in neonates with small left heart structures and aortic arch obstruction with minimal mortality and excellent late functional results. Standard echocardiographic measurements used to predict the need for a univentricular repair in critical aortic stenosis are not valid for the neonate with aortic arch obstruction.

Spectrum and influence of hypoplasia of the left heart in Neonatal aortic coarctation

Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocardiogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.

Influence of left ventricular cavity size on interventricular shunt timing and outcome in neonates with coarctation of the aorta and ventricular septal defect

In this study, we reviewed the records and echocardiograms of 39 consecutive patients with coarctation of the aorta and ventricular septal defect who underwent neonatal coarctation repair to examine the influence of left heart size on ventricular shunting and outcome. We found smaller left heart structures (initial mitral and aortic annular diameters) to be associated with diastolic interventricular shunting and to be predictive of the development of mitral or aortic and/or subaortic stenosis.

Size of Ventricular Structures Influences Surgical Outcome in Down Syndrome Infants With Atrioventricular Septal Defect

To evaluate the relation between ventricular structure size and surgical outcome in Down versus non-Down syndrome infants with an atrioventricular septal defect, we reviewed the charts and echocardiograms of 44 consecutive infants (34 with Down syndrome) who underwent atrioventricular septal defect repair. Children with Down syndrome had significantly greater aortic valve diameters, left ventricular valve areas, and left/right atrioventricular valve area ratios as well as fewer adverse outcomes than non-Down syndrome children.

Usefulness of doppler echocardiography to determine the timing of surgery for supravalvar aortic stenosis

S aortic stenosis (SVAS) may occur as part of Williams’ syndrome, with autosomal dominant inheritance or sporadically.1–3 Previous studies have shown that SVAS tends to progress with time, so serial evaluation is important.4–6 Operative intervention for SVAS has been recommended for symptoms or for a catheter-measured gradient of .50 mm Hg.2,3,7,8 Although previous studies have shown the value of 2-dimensional and Doppler imaging in the diagnosis of SVAS,9–14 the value of echocardiography for predicting the catheter-measured gradient and assessing the need for surgery has not been evaluated. This study assesses the value of echocardiography and Doppler imaging in predicting catheter-measured pressure gradients and determining the need for surgery for SVAS. • • • The Primary Children’s Medical Center pediatric cardiology database was searched to identify patients with the diagnosis of SVAS who underwent echocardiography within 2.5 months of catheterization. Demographic data collected included patient weight, body surface area, gender, age at catheterization, and surgical history. Complete 2-dimensional, M-mode, and Doppler echocardiograms were retrospectively reviewed. Measurements were made off-line from digitized images selected from videotape. Each measurement was made in triplicate and averaged. Two-dimensional measurements were made from inner edge to inner edge. The narrowest diameter of the ascending aorta and the aortic annulus diameter were measured, allowing calculation of a ratio (supravalvar aorta/annulus) that was always ,1. In each case, color Doppler was used to align the continuous-wave Doppler beam as close to parallel as possible with the jet in the ascending aorta, but no correction was made for angle of incidence. The peak instantaneous and mean gradients were obtained from Doppler tracings (modified Bernoulli equation). The presence of aortic regurgitation and the presence of head and neck vessel or branch pulmonary artery stenoses were noted. Left ventricular shortening fraction, wall thickness, and left ventricular mass (American Society of Echocardiography convention) were measured from the M-mode tracing.16 A cardiologist blinded to the results of the echocardiograms reviewed catheterization data and angiograms. In each case, the peak-to-peak pressure gradient, the type of SVAS (discrete, diffuse, or membranous), the ratio of the ascending aortic narrowing to the aortic annulus, the presence of stenoses of other vessels (head and neck vessels, coronaries, branch pulmonary arteries, renal arteries), and the presence of aortic regurgitation were noted. Linear regression analysis was used to assess the correlation between Doppler and catheter gradients, and between other variables and Doppler or catheter gradients. The influence of variables on the difference between Doppler and catheter gradients was assessed using either linear regression or unpaired t tests. Agreement between Doppler and catheter gradients was assessed using the method of Bland and Altman, which involves plotting the difference between the Doppler and catheter gradients against the average of the same 2 gradients for each case.16 The mean difference between the 2 measurements gives an indication of the bias, and the limits of agreement are defined by the mean difference 6 2 SDs (95% confidence intervals). When there is good agreement between 2 measurements, there is an expected small mean difference and narrow limits of agreement. Because of the poor agreement between Doppler and catheter gradients, we then assessed whether Doppler might be clinically useful in predicting a catheter-measured peak gradient of .50 mm Hg, the currently published criterion for surgery for SVAS.2,3,7,8 The relation between a Doppler peak instantaneous gradient of .85 mm Hg and a catheter peak-to-peak gradient of .50 mm Hg was assessed using Fisher’s exact test. Sensitivity, specificity, positive and negative predictive values, and accuracy of a Doppler peak instantaneous gradient of .85 mm Hg for predicting a catheter-measured peak-to-peak gradient of .50 mm Hg were also calculated. A p value of ,0.05 was considered statistically significant. A total of 26 paired echocardiograms and catheterizations from 18 patients (12 males, 6 females) with SVAS were identified. When .1 echocardiographic catherterization pair was used from the same patient, the time interval ranged from 6.3 to 108 months (median 22.5). Age at catheterization ranged from 1 month to 18 years (median 2.5 years). From the aortic angiograms, the SVAS was discrete or localized in 20 cases, and diffuse in the other 6. Membranous obstruction was not seen in this series. Sedation was required for 10 echocardiograms. The other patients were cooperative, and studied in a quiet, resting state. The Doppler tracing used for determination of the peak instantaneous and mean gradients was obtained using blind continuous-wave Doppler in 11 cases and by 2-dimensional directed continuous-wave Doppler in From the Department of Pediatrics, Primary Children’s Medical Center and the University of Utah, Salt Lake City, Utah. Dr. Tani’s address is: Primary Children’s Medical Center, 100 North Medical Drive, Salt Lake City, Utah 84113. Manuscript received and accepted December 2, 1999; revised manuscript received and accepted January 18, 2000.

Usefulness of echocardiography for detection of coronary artery thrombi in patients with kawasaki disease

To evaluate the role of echocardiography for predicting and accurately detecting thrombi in patients with abnormal coronary arteries after Kawasaki disease, we reviewed the echocardiograms of 40 consecutive patients and compared echocardiographic findings with angiographic findings when available. Patients with Kawasaki disease who had coronary artery aneurysms > or =5 mm had significantly greater multivessel involvement, thrombi, and persistent coronary abnormalities than those with diameters <5 mm.

An Unusual Variant of Total Anomalous Pulmonary Venous Connection with Varices and Multiple Drainage Sites

Abstract. Total anomalous pulmonary venous connection (TAPVC) is an uncommon cardiac anomaly that has also rarely been associated with esophageal atresia. We report an unusual case of esophageal atresia with TAPVC with several varices and multiple drainage sites into the superior vena cava and portal vein.

Can Doppler systemic venous flow indices predict central venous pressure in children

Tricuspid valve, superior vena cava (SVC), and hepatic vein Doppler patterns may be abnormal in right heart anomalies and have been used to predict high central venous pressure (CVP) in adults. The purpose of this study was to evaluate the relationship of these systemic venous flow indices to CVP in children. Children undergoing cardiac catheterization were studied prospectively using simultaneous recordings of mean CVP with pulsed‐Doppler tracings of SVC, hepatic vein, and tricuspid valve flow. Systemic venous Doppler measurements included peak velocities and velocity time integrals for ventricular systole (S), ventricular diastole (D), end ventricular systole (B), and atrial systole (A). Tricuspid inflow Doppler E and A waves were recorded also. Patients with significant tricuspid stenosis or regurgitation, systemic venous obstruction, and nonsinus rhythm were excluded. The 42 patients ranged in age from 0.2 to 21.0 years and in weight from 3.0 to 68.0 kg. Mean CVPs ranged from 1 to 17 mmHg. Catheterization indications included hemodynamic evaluation (25 patients), transplant biopsy, (11 patients), and interventional procedures (6 patients). No SVC or tricuspid valve Doppler measurement correlated with CVP. Hepatic vein peak D, peak B, and peak A significantly correlated with CVP (r= 0.34–0.55; P< 0.05, linear regression). For all correlations, the r values were low with significant overlap among patients. Thus, in children, only hepatic vein peak velocities correlate with CVP. Because of the low r values and significant overlap among patients, the currently used Doppler indices have a low sensitivity for predicting CVP in this age group.

Echocardiographic Diagnosis of Thrombus Originating from the Ductus Arteriosus

Initial functional closure of the ductus arteriosus normally occurs within hours after birth, with permanent closure taking several weeks. The mechanism for ductal closure has been well studied and has not been shown to include thrombus formation. We describe a normal infant found to have a thrombus originating in the ductus arteriosus that occluded the ductus and subsequently extended into the left pulmonary artery, threatening to occlude it as well. This case illustrates the importance of echocardiography in making this rare diagnosis. It also emphasizes the role of echocardiography as an effective means of following the progression or regression of such a thrombus.