Usefulness of doppler echocardiography to determine the timing of surgery for supravalvar aortic stenosis.
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Adolescent↗
Biomedical subjects
L Y Tani
Publications and source records attributed to L Y Tani.
Doppler evidence of subclavian steal in neonates with interrupted aortic arch using transthoracic echocardiography.
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Abnormalities, Multiple↗
Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum.
Although tricuspid valve z-scores have been used to predict outcome in pulmonary atresia with intact ventricular septum, they are statistically generated from local populations, and widespread generalization may not be appropriate. To determine if there are echocardiographic predictors of outcome that can be universally used, the records of all infants with this diagnosis since 1988 were reviewed for age, weight, type of surgery, and outcome. Preoperative and follow-up echocardiograms were reviewed for valve diameter and z-scores, and valve ratios were calculated. Thirty-six patients were divided into 2 groups: group 1 included 23 infants who had a successful biventricular repair; group 2 included the remaining 13 infants who did not have a successful repair. Preoperatively, both groups had similar ages, pulmonary, aortic, and mitral z-scores, and pulmonary/aortic ratios, but the patients in group 2 had significantly lower weight (3.5 +/- 0.6 vs 2.9 +/- 0.5 kg), tricuspid z-scores (-0.7 +/- 1.5 vs -2.3 +/- 1.2), and tricuspid/mitral ratios (0.8 +/- 0.2 vs 0.5 +/- 0.1). At similar follow-up, both groups of patients had similar weight, aortic and mitral z-scores, and pulmonary/aortic ratios, but group 2 infants had significantly lower pulmonary and tricuspid z-scores and tricuspid/mitral ratios. Compared with the preoperative echocardiograms, group 1 had significant increases only in pulmonary z-scores, and pulmonary/aortic and tricuspid/mitral ratios. Group 2 had no significant change in any echocardiographic variable. The tricuspid/mitral ratio was >0.5 in all group 1 infants, and in 6 of 13 group 2 infants (2 sepsis deaths, 4 palliations). Compared with a tricuspid valve z-score >-3, a tricuspid/mitral ratio >0.5 was a better predictor of biventricular repair. Thus, infants who have a successful biventricular repair have significantly greater preoperative weight, tricuspid valve z-scores, and tricuspid/mitral valve ratios. A tricuspid/mitral ratio >0.5 was the best predictor of a biventricular repair.
Echocardiography↗
Coronary interventional procedures in pediatric heart transplant recipients with cardiac allograft vasculopathy.
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Adolescent↗
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.
Aortic Coarctation↗
Aortic input impedance in infants and children.
Flow and pressure measurements were performed in the ascending aortas of six pediatric patients ranging in age from 1 to 4 yr and in weight from 7.2 to 16.4 kg. From these measurements, input impedance was calculated. It was found that total vascular resistance decreased with increasing patient weight and was approximately one to three times higher than those of adults. Conductance per unit weight was relatively constant but was approximately three times higher than for adults. Strong inertial character was observed in the impedance of four of the six patients. Among a three-element and two four-element lumped-parameter models, the model with characteristic aortic resistor (R(c)) and inertance in series followed by parallel peripheral resistor (R(p)) and compliance fitted the data best. R(p) decreased with increasing patient weight and was one to three times higher than in adults, and R(c) decreased with increasing patient weight and was 2 to 15 times higher. The R(p)-to-R(c) ratio differed significantly between infants and children vs. adults. The results suggested that R(p) developed more rapidly with patient weight than did R(c). Compliance values increased with increasing patient weight and were 3 to 16 times lower than adult values.
Aorta↗
What is the yield of screening echocardiography in pediatric syncope?
OBJECTIVE: To determine the yield of screening echocardiography in the evaluation of pediatric syncope. DESIGN: All patients diagnosed with syncope from January 1993 to January 1999 were identified and their records were reviewed for age, weight, sex, year of presentation, personal and family history, physical examination, and cardiac diagnostic testing. Cardiac defects were identified by reviewing echocardiograms and reports. RESULTS: The 480 patients (268 females) ranged in age from 1.5 to 18.0 years old and ranged in weight from 10.3 to 113.6 kg. Final diagnoses included noncardiac causes in 458, long QT syndrome in 14, arrhythmias in 6, and cardiomyopathy in 2. An abnormal history, physical examination, or electrocardiogram identified 21 of the 22 patients with a cardiac cause of syncope. Of the 322 (67%) echocardiograms performed, abnormalities were detected in 37. These abnormalities included 26 minor valve anomalies, 7 hemodynamically insignificant shunt lesions, 2 mildly decreased left ventricular shortening fractions, and 2 cardiomyopathies. Only the 2 cardiomyopathies were considered to be potential causes of syncope, and in both cases, the electrocardiogram was markedly abnormal. A similar percentage of echocardiograms were ordered during the first and last 3 years of the study (61% vs 71%). CONCLUSION: History, physical examination, and electrocardiography provide a screening protocol that allows the identification of a cardiac cause of syncope in the overwhelming majority of pediatric patients. In the absence of a positive screen result, the echocardiogram does not contribute to the evaluation of syncope in children. We speculate that primary care providers and pediatric cardiologists continue to use echocardiography because of the paucity of data regarding its value in pediatric syncope. However, this study shows little benefit of screening echocardiography and should discourage its routine use.
Adolescent↗
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.
Aortic Coarctation↗
Are screening echocardiograms warranted for neonates with meningomyelocele?
OBJECTIVE: To evaluate the incidence and types of congenital heart defects associated with meningomyelocele. DESIGN: All neonates who underwent meningomyelocele repair and had a perioperative echocardiogram from July 1990 to October 1998 were studied. Medical records were reviewed for age, weight, clinical cardiac examination results, meningomyelocele location, and associated noncardiac defects. Heart defects were identified from reviewing echocardiographic reports and videotapes. RESULTS: At meningomyelocele surgery, the 105 patients (53 female; 52 male) ranged in age from 1 to 20 days and in weight from 0.6 to 4.1 kg. Congenital heart disease was detected in 39 patients (37%). A secundum atrial septal defect was the most common defect (24%). A ventricular septal defect was found in 10 patients, 2 patients had anomalous pulmonary venous return, and 1 each had tetralogy of Fallot, bicuspid aortic valve, coarctation, and hypoplastic left heart syndrome. A patent ductus arteriosus and patent foramen ovale were not considered abnormal in these neonates. The cardiac examination was abnormal in only 5 of the 39 patients with heart defects (sensitivity = 13%). The presence of associated noncardiac defects (in addition to meningomyelocele) and location of the meningomyelocele (cervicothoracic vs lumbar) did not affect the incidence of heart disease. Of the patients with heart defects, girls were more frequently affected (25 of 39 vs 14 of 39, P<.05). CONCLUSIONS: Congenital heart defects are common in neonates, especially girls, with meningomyelocele and are unrelated to meningomyelocele location or associated noncardiac defects. Because the clinical examination is insensitive for detecting heart defects in this group, screening echocardiograms are warranted. This information has important implications for ventriculoatrial shunting, urinary tract instrumentation (antibiotic prophylaxis), and neurosurgical procedures (venous air embolism).
Abnormalities, Multiple↗
Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome.
BACKGROUND: Pulmonary vein (PV) stenosis with anatomically normal connection is considered rare, unresponsive to treatment, progressive, and usually fatal. METHODS: We reviewed the records of 13 children with this diagnosis at our center since 1990. RESULTS: The number of stenosed PVs ranged from all PVs (n = 5); three PVs (n = 1); two PVs (n = 5); and one PV (n = 2). All patients had associated congenital cardiac abnormalities. Operation on PV stenosis was attempted in 7 patients (54%), 2 of whom have done well and 5 of whom have not. Two patients underwent heart transplantation for inoperable associated cardiac lesions. Significantly more patients with three or four stenosed PVs died (83%) compared with patients with one or two stenosed PVs (0%). CONCLUSIONS: (1) Pulmonary vein stenosis with anatomically normal connection is associated with other congenital cardiac abnormalities, (2) presentation and outcome are contingent on the number of stenosed PVs, (3) surgical palliation may be helpful in some patients, and (4) heart transplantation for inoperable associated cardiac abnormalities may be an option in patients with only one or two stenosed PVs.
Adolescent↗
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.
Abnormalities, Multiple↗
Septal cystic mass in the right atrium: echocardiographic evaluation.
Right atrial masses rarely are seen in children and are usually benign. Clinical manifestations mainly depend on the location of the mass and whether it alters blood flow. This report describes the clinical course, echocardiographic findings, pathology, and serial follow-up of an unusual cystic mass with a mixture of tissue common to the atrioventricular septum located in the posteroinferior right atrium.
Cysts↗
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.
Anticoagulants↗
Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children: a multi-institutional experience.
BACKGROUND: Dilated cardiomyopathy is the primary indication for heart transplantation in children beyond infancy. Although beta-blockers improve symptoms, ejection fraction, and survival in adults with congestive heart failure, little is known of their effects in children. METHODS: This study reviews our pediatric experience with the beta-blocker, metoprolol, at 3 institutions. We gave metoprolol to 15 children, age 8.6 +/- 1.3 years (range 2.5 to 15 years), with idiopathic dilated cardiomyopathy (n = 9), anthracycline cardiomyopathy (n = 3), and Duchenne muscular dystrophy cardiomyopathy, postmyocarditis cardiomyopathy, and post-surgical cardiomyopathy (n = 1 each). All had been treated with conventional medications (digoxin, diuretics, and ACE inhibitors) for 22.5 +/- 9 months before starting metoprolol. Metoprolol was started at 0.1 to 0.2 mg/kg/ dose given twice daily and slowly increased over a period of weeks to a dose of 1.1 +/- 0.1 mg/kg/day (range 0.5 to 2.3 mg/kg/day). RESULTS: Between the time point of stabilization on conventional medications and the initiation of metoprolol therapy, there was no significant change in fractional shortening (13.1 +/- 1.2% vs 15.0 +/- 1.2%) or ejection fraction (25.6 +/- 2.1% vs 27.0 +/- 3.4%). However, after metoprolol therapy for 23.2 +/- 7 months, there was a significant increase in fractional shortening(23.3 +/- 2.6%) and ejection fraction (41.1 +/- 4.3%) (p < 0.05). CONCLUSIONS: Metoprolol improves ventricular function in some children with dilated cardiomyopathy and congestive heart failure. Further study is warranted to better define which children may benefit most from beta-blocker therapy and which beta-blockers are most efficacious.
Adolescent↗
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.
Child, Preschool↗
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.
Aortic Valve↗
Platelet glycoprotein IIb/IIIa receptor blockade therapy for large coronary aneurysms and thrombi in Kawasaki disease.
A 4-month-old girl with Kawasaki disease, large coronary artery aneurysms, and coronary thrombi was treated with standard therapy followed by abciximab, a platelet glycoprotein IIb/IIIa antagonist, in addition to standard heparin and warfarin sodium anticoagulation and low-dose aspirin. She did not develop evidence of ischemia, had no complications from the therapy, and showed resolution of the aneurysms and thrombi after 6 wk of therapy.
Abciximab↗
Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants.
BACKGROUND: Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era. METHODS: From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques. RESULTS: Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 +/- 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% +/- 6% at 1 month, 78% +/- 9% at 1 year, and 73.4% +/- 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% +/- 3% at 1 month, 73% +/- 9% at 1 year, and 55% +/- 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 +/- 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 +/- 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients. CONCLUSIONS: Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis.
Actuarial Analysis↗