Biomedical subjects

Beth F Printz

Publications and source records attributed to Beth F Printz.

11 recordsLinked to original sources

PubMed2007

Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome.

OBJECTIVE: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients. STUDY DESIGN: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29). RESULTS: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end. CONCLUSIONS: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.

Adolescent↗

PubMed2006

Ventricular diastolic stiffness predicts perioperative morbidity and duration of pleural effusions after the Fontan operation.

BACKGROUND: We validated the clinical relevance of ventricular stiffness by examining surgical morbidity in children with univentricular hearts undergoing Fontan operation. We hypothesized that ventricular stiffness affects Fontan morbidity, particularly duration of pleural effusions. METHODS AND RESULTS: Sixteen children with right ventricular (RV) (n =11) or left ventricular (LV) (n =5) dominance were studied intraoperatively at a median age of 3.3 years (1.8 to 5.1). Transesophageal long-axis echocardiograms and ventricular pressure by micromanometer provided end-diastolic pressure (P) area (A) relations during initiation and conclusion of cardiopulmonary bypass. Curve fitting to the equation P=alphae(betaA) defined the ventricular stiffness constant, beta. Changes in beta and clinical correlations were examined. Ventricular stiffness increased after bypass in patients with complete pre-bypass and post-bypass data (n =11, P=0.023, mixed models methodology). Pre-bypass beta correlated well with duration of chest tube (CT) drainage (r=0.90, n =16), net perioperative fluid balance (r=0.71, n=14), and length of stay (LOS) (r=0.81, n =16). CT duration and LOS also correlated significantly with post-bypass beta (r=0.77 for both, n=11), but insignificantly with preoperative catheterization pressures. CONCLUSIONS: Intraoperative beta predicts duration of CT drainage, net perioperative fluid balance, and LOS after the Fontan operation. These observations could improve risk stratification and clinical management of children at high-risk undergoing the Fontan operation.

Chest Tubes↗

PubMed2006

Physiology of isolated anomalous pulmonary venous connection of a single pulmonary vein as determined by cardiac magnetic resonance imaging.

The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular (RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55 (range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort (108 +/- 16 vs 78 +/- 18 cm(3)/m(2), p = 0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting (r = 0.3, p = 0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area (r = 0.96, p = 0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.

Adolescent↗

PubMed2006

Prenatal diagnosis of conotruncal malformations: diagnostic accuracy, outcome, chromosomal abnormalities, and extracardiac anomalies.

The purpose of this study was to determine whether continuing experience in prenatal diagnosis of conotruncal malformations (CTMs) has resulted in improved diagnostic accuracy and outcome. Previous reports have demonstrated particular difficulty with ascertainment of the spatial relationship of the great arteries in patients with CTM. The prognosis for fetuses with CTM was poor. Medical records of 113 consecutive fetuses in whom a CTM (tetralogy of Fallot [TOF], double-outlet right ventricle [DORV], type B aortic arch interruption, transposition of the great arteries [TGA], and persistent truncus arteriosus [TA]) was diagnosed antenatally between 1994 and 2003 were reviewed. The diagnosis of the 91 fetuses with CTM included TOF (n = 32), TGA (n = 29), DORV (n = 22), and TA (n = 8). The great arterial spatial relationship was diagnosed accurately in 84 of the 91 (92%) live-born infants. In the other seven infants with DORV, the great arterial spatial relationship was identified inaccurately. The overall survival to 30 days was 85 of 91 (93%). Twenty-three of 91 (25%) patients had extracardiac anomalies. Genetic diagnosis (amniocentesis) was obtained in 63 of 94 patients; 11 (17%) had chromosomal abnormalities. Maternal glucose tolerance results were obtained in 65 of the 91 patients and were abnormal in 25 of 65 (38%). Prenatal diagnostic accuracy of conotruncal malformations is excellent; the arterial spatial relationship of DORV remains problematic. The populations of fetuses with CTMs who continue to develop to term have an excellent prognosis.

Blood Glucose↗

PubMed2005

Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy.

This study evaluated the feasibility and accuracy of the magnetic resonance angiography (MRA) evaluation of scimitar syndrome in early infancy in 4 subjects. MRA imaging was successful, with excellent imaging quality to evaluate the main and lobar pulmonary artery branches, lobar pulmonary veins, scimitar vein, and systemic-pulmonary collateral arteries in all subjects. There was good agreement between findings with MRA, x-ray angiography (n = 3), surgical inspection (n = 3), and autopsy (n = 1).

Collateral Circulation↗

PubMed2005

Methods for noninvasive measurement of tissue iron in Cooley's anemia.

To examine the relationship between myocardial storage iron and body iron burden, as assessed by hepatic storage iron measurements, we studied 22 patients with transfusion-dependent thalassemia syndromes, all being treated with subcutaneous deferoxamine, and 6 healthy subjects. Study participants were examined with a Philips 1.5-T Intera scanner using three multiecho spin echo sequences with electrocardiographic triggering and respiratory navigator gating. Myocardial and hepatic storage iron concentrations were determined using a new magnetic resonance method that estimates total tissue iron stores by separately measuring the two principal forms of storage iron, ferritin and hemosiderin. In a subset of 10 patients with beta-thalassemia major, the hepatic storage iron concentration had been monitored repeatedly for 12-14 years by chemical analysis of tissue obtained by liver biopsy and by magnetic susceptometry. In this subset, we examine the relationship between hepatic iron concentration over time and our current magnetic resonance estimates of myocardial iron stores. No significant relationship was found between simultaneous estimates of myocardial and hepatic storage iron concentrations. By contrast, in the subset of 10 patients with beta-thalassemia major, the correlation between the 5-year average of hepatic iron concentration and the current myocardial storage iron was significant (R = .67, P = .03). In these patients, myocardial storage iron concentrations seem to reflect the control of body iron over a period of years. Magnetic resonance methods promise to provide more effective monitoring of iron deposition in vulnerable tissues, including the liver, heart, and endocrine organs, and could contribute to the development of iron-chelating regimens that more effectively prevent iron toxicity.

Adult↗

PubMed2004

Myocardial performance index in pediatric patients after cardiac transplantation.

BACKGROUND: Myocardial performance index (MPI) has been shown to be a reliable indicator of ventricular performance. This study determined MPI values in pediatric patients after cardiac transplantation without endomyocardial rejection. METHODS: MPI was determined in 41 pediatric patients after cardiac transplantation, without evidence of microscopic rejection, and in 31 pediatric control subjects. RESULTS: MPI in the transplantation group (0.41 +/- 0.12) was higher than in the control group (0.31 +/- 0.09; P =.0003). Isovolumic relaxation time and isovolumic relaxation time/ejection time were higher in the transplant group (55 +/- 20 milliseconds and 0.22 +/- 0.07, respectively) compared with the control group (41 +/- 10 milliseconds and 0.16 +/- 0.06, respectively; P =.0002). Isovolumic contraction time and isovolumic contraction time/ejection time were similar in the transplant group (48 +/- 23 milliseconds and 0.19 +/- 0.09, respectively) and control group (43 +/- 21 milliseconds and 0.16 +/- 0.08, respectively; P = not significant). CONCLUSIONS: Pediatric patients after cardiac transplantation without endomyocardial rejection have a higher MPI compared with a normative pediatric control population. The difference appears to be related to abnormal diastolic function.

Adolescent↗

PubMed2004

Regional functional depression immediately after ventricular septal defect closure.

Left ventricular ejection is depressed immediately after repair of ventricular septal defect (VSD). Postrepair functional depression seen after VSD closure could result from a reduction in preload. However, other mechanisms could be at work. Functional depression could also be caused by closure of a low-impedance path for left ventricular ejection, the introduction of a stiff akinetic patch, or the operation itself. We reasoned that functional depression mediated by changes in preload or afterload should symmetrically affect end-diastole and end-systole, whereas depression resulting from changes in septal mechanics should be localized. We, therefore, performed segmental wall-motion analysis on intraoperative echocardiograms from patients undergoing VSD and atrial septal defect repair. After VSD closure, there was an asymmetric change in left ventricular end-systolic segment length and a decrease in fractional segment shortening localized to the septal and lateral walls, whereas patients with atrial septal defect had a symmetric increase in fractional shortening. These results suggest that acute functional depression after VSD repair is a result of localized impairment of septal function.

Cardiac Surgical Procedures↗

PubMed2004

Early and medium-term results for repair of Ebstein anomaly.

OBJECTIVES: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. METHODS: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. RESULTS: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. DISCUSSION: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.

Adolescent↗

PubMed2004

Echocardiographic analysis of ventricular geometry and function during repair of congenital septal defects.

BACKGROUND: This study investigated changes in left ventricular (LV) geometry and systolic function after corrective surgery for atrial (ASD) and ventricular septal defects (VSD). METHODS: Transesophageal LV short-axis echocardiograms were recorded before and after operative repair of ASD (n = 11) and VSD (n = 7). Preload was measured using LV end-diastolic area indexed for body surface area. Measurements of septal-freewall (D1) and anterior-posterior (D2) endocardial diameters were used to assess LV symmetry from D1/D2. Systolic indices included stroke area, area ejection fraction, and fractional shortening. RESULTS: Preload, stroke area, area ejection fraction, and fractional shortening of D1 increased after ASD repair but decreased after VSD repair (p < 0.05). End-diastolic symmetry increased after ASD closure and decreased after VSD closure (p < 0.05). Increases in stroke area and ejection fraction after ASD correction primarily reflected increased shortening of D1. A positive correlation was found overall between percent change in end-diastolic area (EDA) and percent change in area ejection fraction (r(2) = 0.80, p < 0.0001, n = 18). CONCLUSIONS: Preload was the primary determinant of changes in LV function in this series of ASD and VSD repairs. Intraoperative changes in position of the interventricular septum affected systolic and diastolic LV symmetry and septal free wall shortening. Additional studies are needed to define changes in afterload and contractility as well as diastolic compliance and systolic mechanics.

Child↗

PubMed2003

Correlation of the Tei index with invasive measurements of ventricular function in a porcine model.

BACKGROUND: The Doppler myocardial performance (Tei) index has been reported to be clinically useful in assessing left ventricular systolic and diastolic function in both adults and children. However, there are limited data to compare the Tei index with invasive measurements of ventricular function. We used a porcine model to directly correlate the Tei index with invasive indices of systolic and diastolic function. METHODS: Pressure volume loops were obtained from 10 pigs (32-45 kg). A micromanometer and a conductance catheter were placed in the left ventricle to record pressure and volume, respectively. A flow probe was placed around the ascending aorta to record cardiac output. Baseline pressure volume loops were generated during preload reduction through caval occlusion. Epicardial echocardiograms were performed just before the caval occlusion. Invasive indices including preload recruitable stroke work, ventricular stiffness constant, and cardiac output were assessed, as were noninvasive echocardiographic indices including Tei index and ejection fraction. An ischemic insult, ventricular fibrillation, was induced to alter ventricular function. After cardioversion and 40 minutes of reperfusion, echocardiographic and invasive measurements were repeated. RESULTS: There was a statistically significant inverse relationship between the percent change in Tei and the percent change in preload recruitable stroke work after ventricular fibrillation (r = -0.70, P =.02), although the correlation between the actual values of Tei and preload recruitable stroke work were not statistically significant. There was a statistically significant inverse relationship between the percent change in Tei and the percent change in cardiac output (r = -0.65, P =.03). There was a direct correlation between the value of Tei and the ventricular stiffness constant at baseline (r = 0.63, P <.05). As anticipated, the value of Tei was inversely related to ejection fraction by epicardial echocardiogram at baseline (r = -0.85, P <.001). The percent change in Tei was inversely related to the percent change in ejection fraction as well (r = -0.69, P <.05). CONCLUSIONS: This animal model is one of the first studies to demonstrate a direct correlation between the Tei index and systolic and diastolic invasive measurements of ventricular function. This supports the clinical use of this index as a measure of global ventricular function.

Animals↗