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Biomedical subjects

D W Benson

Publications and source records attributed to D W Benson.

At least 37 records · Page 2Linked to original sources

Fetal tachycardia: mechanisms and predictors of hydrops fetalis.

OBJECTIVES: This study had three objectives: 1) to determine the electrophysiologic mechanisms of fetal supraventricular tachycardia at presentation and postnatally; 2) to identify the clinical and electrophysiologic predictors of hydrops fetalis; and 3) to describe the medium-term follow-up (1 to 7 years) of patients with fetal supraventricular tachycardia. BACKGROUND: Fetal supraventricular tachycardia causes significant fetal and neonatal morbidity and mortality. Prenatal analysis and postnatal confirmation of fetal supraventricular tachycardia mechanisms have been limited. METHODS: Supraventricular tachycardia mechanisms were evaluated by prenatal Doppler/M-mode echocardiography, immediate neonatal surface electrocardiography and postnatal transesophageal electrophysiologic procedures in 30 consecutive patients presenting with fetal supraventricular tachycardia (17 managed prenatally, 13 first managed postnatally). RESULTS: The fetal supraventricular tachycardia mechanism was 1:1 atrioventricular conduction in 22 patients and supraventricular tachycardia with atrioventricular block (atrial flutter) in 8. At the postnatal transesophageal electrophysiologic procedure, tachycardia was induced in 27 of 30 patients; atrioventricular reentrant tachycardia in 25 (93%) of 27 and intraatrial reentrant tachycardia in only 2 (7%) of 27. Hydrops was present in 12 of 30 fetuses. Sustained supraventricular tachycardia (> 12 h) and lower gestation at presentation correlated with hydrops (p < 0.02, p < 0.05), but mechanism of tachycardia and heart rate did not. Gestational age at delivery was significantly greater in those who received intrauterine management (39 +/- 1.3 vs. 37 +/- 2.9 weeks, p = 0.04) despite earlier presentation (32.6 vs. 37.1 weeks). Cesarean section deliveries were reduced in the same group (3 of 17 vs. 11 of 13, p = 0.0006). CONCLUSIONS: Atrioventricular reentrant tachycardia was the predominant mechanism of supraventricular tachycardia in the fetus. There was a high association of supraventricular tachycardia with atrioventricular block in utero and accessory atrioventricular connections. Outcome at 1 to 7 years was excellent regardless of severity of illness at clinical presentation.

Echocardiography, Doppler↗

Transmission of full-length echocardiographic images over ISDN for diagnosing congenital heart disease.

OBJECTIVES: To study the feasibility of transmitting full-length diagnostic-quality echocardiograms with video conferencing systems linked by ISDN. METHODS: Forty-three previously video-recorded full-length echocardiograms (22 complete two-dimensional Doppler studies, 21 separate M-mode studies) were transmitted from a site 30 miles distant using desktop video conferencing systems linked with one ISDN line (128 kbps). Blinded interpretations of the original recorded and transmitted studies were made by two pediatric cardiologists (two-dimensional Doppler) and a pediatric sonographer (M-mode). RESULTS: Diagnoses of normal (N = 12) and abnormal (N = 10) conditions were made from the original tapes; the same interpretations were made from the transmitted studies. Twenty-one specific abnormalities in the 10 abnormal studies were seen on both original and transmitted videotapes. These abnormalities ranged from simple congenital heart defects, including ventricular septal defect, atrial septal defect, and patent ductus arteriosus, to complex ones, such as coronary artery fistula, double-outlet right ventricle, and complete atrioventricular canal. Qualitatively, there was mild degradation in gray scale, brightness, and contrast of the images. Six M-mode variables from the transmitted images had excellent concordance with the original tracings (P = 0.506 to 0.838; r = 0.86 to 0.97). CONCLUSIONS: Although our sample size was small, this preliminary experience indicates that video conferencing equipment utilizing ISDN technology is a reliable method for transmitting full-length diagnostic-quality echocardiographic studies from remote sites. This technology may have a significant impact on the management of pediatric patients with suspected heart disease. Further evaluation is warranted.

Child↗

New understandings in the genetics of congenital heart disease.

In spite of the amazing success during the past half century in diagnosis and treatment of congenital heart disease, very little is known of its cause. However, a genetic cause has been clearly established for many forms of cardiovascular disease, and new understandings in the molecular genetics of congenital heart disease provide further insight. Progress has been quite impressive for some cardiovascular abnormalities, whereas in other areas the findings are more preliminary. For example, the molecular genetic cause of supravalvular aortic stenosis and the heart disease associated with Marfan syndrome has been clearly established. Impressive progress has been made in conotruncal defects, Holt-Oram syndrome, Alagille syndrome, and total anomalous pulmonary venous connection. In other areas, such as patent ductus arteriosus and atrioventricular septal defect, the findings are more preliminary. Taken as a whole, the prospect of understanding the genetic basis of congenital heart disease has never been better. Understanding the cause of congenital heart disease will provide new insights into both normal cardiac development and the pathophysiologic basis of congenital heart defects.

Abnormalities, Multiple↗

Ventricular relaxation in the stage 24 chick embryo following changes in volume and blockade of Na+ and Ca2+ channels.

OBJECTIVE: Early in cardiac development, regulation of cytosolic Ca2+ has been thought to depend primarily on sarcolemmal Ca2+ transport. We hypothesized that perturbation of cytosolic Ca2+ in the embryonic ventricle would result in a change in ventricular relaxation which could be quantified by a monoexponential model. We reasoned that since it has been difficult to selectively block the Na(+)-Ca2+ exchanger in vivo, that blockade of Na+ (lidocaine) or Ca2+ (verapamil) channels in the embryonic heart may perturb cytosolic Ca2+ and thereby alter ventricular relaxation. METHODS: All studies were performed in ovo in Hamilton-Hamburger stage 24 chick embryos. Isovolumic relaxation time (mean +/- standard deviation, 58 +/- 19 ms) was derived from dorsal aortic flow and atrioventricular inflow during 61 cardiac cycles in 4 embryos. Ventricular pressure was digitally recorded from 13 embryos during 188 cycles following intravenous injection of chick Ringer's solution (5 embryos), verapamil (4 embryos) or lidocaine (4 embryos). Ventricular relaxation was characterized by a monoexponential model: P(t) = P infinity + Poe-1/tau where P(t) = pressure at time (t), P infinity = pressure asymptote, Po = pressure at the onset of relaxation and tau = the isovolumic relaxation constant. Non-linear least-squares regression was used to estimate tau and P infinity during isovolumic relaxation at baseline and at 30 s and 60 s post-injection. RESULTS: Ventricular end-diastolic pressure was increased by all three interventions. Both lidocaine and verapamil prolonged cycle length. Lidocaine prolonged tau while chick Ringer's solution and verapamil did not. No significant change in P chi was observed. CONCLUSIONS: This study demonstrates that blockade of Na+ channels with lidocaine slows ventricular relaxation presumably by perturbing cytosolic Ca2+ via the Na(+)-Ca2+ exchange system. Changes following Ca2+ channel blockade with verapamil are less evident in the stage 24 chick embryo. Evaluation of ventricular relaxation may provide a useful way to study developmental aspects of Ca2+ transport.

Animals↗

Comparison of transesophageal and intracardiac electrophysiologic studies in characterization of supraventricular tachycardia in pediatric patients.

OBJECTIVES: This study sought to determine the accuracy of transesophageal electrophysiologic studies in diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients. BACKGROUND: Transesophageal electrophysiologic studies are a relatively noninvasive means of characterizing supraventricular tachycardia. Although widely used, to our knowledge no data exist that directly compare information obtained from transesophageal electrophysiologic studies with that from intracardiac electrophysiologic studies. METHODS: We reviewed the records of 57 pediatric patients undergoing both transesophageal and intracardiac electrophysiologic studies at our institution. The results of these studies were compared with respect to mechanism of tachycardia, localization of accessory atrioventricular (AV) connections (if present) and characterization of anterograde accessory connection conduction properties. RESULTS: Tachycardia mechanisms were concordant in 56 of 57 patients: orthodromic reciprocating tachycardia in 43, antidromic reciprocating tachycardia in 1, both orthodromic and antidromic tachycardia in 2, AV node reentrant tachycardia in 5, atrial reentrant tachycardia in 4 and ectopic atrial tachycardia in 2. Of 29 patients with orthodromic reciprocating tachycardia using a concealed accessory connection, transesophageal study predicted the accessory connection site through changes induced by transient bundle branch block in 12. By the Bland-Altman method in 14 patients with pre-excitation, the anterograde accessory connection effective refractory period determined by transesophageal study compared favorably with that determined by intracardiac study (mean difference 5.0 ms, limits of agreement -55 and 65 ms). CONCLUSIONS: Transesophageal electrophysiologic studies are a highly accurate means of diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients.

Adolescent↗

Heart rate-dependent characteristics of diastolic ventricular filling in the developing chick embryo.

The contributions of the early (passive) and late (active) components of ventricular filling have been reported to decrease and increase, respectively, during chick embryo cardiac development. We hypothesized that the observed changes in ventricular filling during early cardiac development results from a decrease in cycle length. We studied the effect of development and cycle length on atrioventricular inflow in 28 chick embryos, Hamilton-Hamburger stages 17, 24, and 26. Cycle length was perturbed (range 240-1040 ms) in ovo by transiently heating or cooling the sinus venosus. Atrioventricular inflow and dorsal aortic velocities were obtained by 20-MHz pulsed Doppler flow-meter and digitally recorded at 500 samples per second. Stroke volume was calculated from dorsal aortic velocity and cross-sectional area. The atrioventricular inflow wave form was integrated and partitioned by area and percentage of total into early (passive) and late (active) components using three methods. Regardless of method, the proportion of filling volume due to the early and late components was cycle length and stage dependent (p < 0.05). The early and late filling volumes were large in the older embryos, and during cycle length decrease (heart rate increase) the early filling volume decrease was greater than the late filling volume decrease. When compared with the percentage of intrinsic heart rate, the percentage of stroke volume due to early filling decreased as heart rate increased and was greater in younger embryos at all heart rates. That due to late filling increased as the percentage of intrinsic heart rate increased. Ventricular filling characteristics are both developmentally determined and cycle length dependent.

Animals↗

Role of endomyocardial biopsy in rejection surveillance after heart transplantation in neonates and children.

OBJECTIVES: The aim of this study was to retrospectively evaluate the sensitivity of noninvasive surveillance (physical examination, echocardiography) of rejection in accurately predicting histologically documented rejection episodes. Additionally, the usefulness of routine scheduled biopsy and its safety in pediatric patients was explored. BACKGROUND: Endomyocardial biopsy has been utilized as the standard for rejection surveillance after heart transplantation in adults, but its role in documenting clinically suspected rejection and in routine surveillance of pediatric patients has not been agreed upon. METHODS: Heart transplantation was performed in 14 neonates and 21 children. The immunosuppressive regimen consisted of cyclosporine, azathioprine and prednisone. All patients underwent routine noninvasive rejection surveillance that included clinical examination and echocardiography. In the neonates, biopsy was performed quarterly beginning 6 months after transplantation, after cessation of prednisone therapy. In the children, biopsy was performed 15 times in the 1st year. A minimum of five biopsy samples were interpreted using the Working Formulation for Heart Transplant Rejection. RESULTS: In the neonates, 37 biopsies were performed. Evidence of rejection was present in only three biopsy samples obtained during eight episodes (38%) of clinically suspected rejection. In 29 biopsies performed when rejection was not clinically suspected, each biopsy was free of cellular infiltrate. In the children, 291 biopsies were performed. Evidence of rejection was present in only seven biopsies (41%) from 17 episodes of clinically suspected rejection. Cellular rejection was discovered during routine rejection surveillance biopsies in asymptomatic patients in 23 (8.4%) of 274 biopsies. CONCLUSIONS: In neonates with clinically suspected rejection, endomyocardial biopsy identified which patients did not require rejection therapy. Endomyocardial biopsy surveillance did not detect any unsuspected cases of rejection. In children, noninvasive rejection surveillance was less reliable even in asymptomatic patients, suggesting that periodic endomyocardial biopsy should be utilized.

Biopsy↗

Assessment of ventricular relaxation in the developing chick embryo using a monoexponential model.

In this study, the ventricular pressure decline during isovolumic relaxation in early diastole was modeled by regressing pressure and pressure change vs. time to a monoexponential formula: P(t) = P infinity+P0e-t/tau, where P(t) is ventricular pressure at time t, P infinity is the pressure in the fully relaxed ventricle, P0 is the pressure at the end of ventricular ejection, and tau is the ventricular relaxation rate constant. Analysis was performed on 330 ventricular pressure waveforms from 22 chick embryos, stages 17, 23, and 26, during baseline and following cardiac cycle length perturbation. Three computational models were evaluated. Based on analysis of the confidence intervals of estimates of P infinity and analysis of residuals, a least-squares nonlinear regression of pressure vs. time, which allowed estimates of tau and P infinity, best approximated the pressure decline. Isovolumic pressure decline in the embryonic heart is well approximated by a monoexponential model if both P infinity and tau are estimated. Negative values of P infinity during early stages of cardiac morphogenesis support the idea that diastolic suction plays a role in ventricular filling in the developing heart.

Animals↗

Normal left ventricular muscle mass and mass/volume ratio after pediatric cardiac transplantation.

BACKGROUND: The adaptive growth of the transplanted heart within the growing child may contribute to long-term cardiac performance. The ability to achieve increased ventricular volume and appropriate muscle mass in the face of immunosuppression and cardiac denervation has not been studied. We previously reported normal left ventricular (LV) volume growth over a 3-year period after cardiac transplantation. This study was designed to assess changes in LV mass and mass/volume ratio and their relation to LV end-diastolic pressure (LVEDP) 1 to 4 years after cardiac transplantation. METHODS AND RESULTS: Cardiac transplantation was performed in 18 patients, age 7 days to 18 years (median, 3.7 years). The indications for cardiac transplantation were cardiomyopathy (8 patients), hypoplastic left heart syndrome (7 patients), and postoperative structural congenital heart disease with ventricular failure (3 patients). The mean follow-up was 48 months, with a range from 29 to 70 months. Serial annual catheterizations were performed after 1 year (16 patients), 2 years (18 patients), 3 years (15 patients), and 4 years (8 patients). Cardiac index (Fick), LVEDP (baseline and after 10-mL/kg saline infusion delivered over 5 minutes), and systemic vascular resistance (SVR) were measured. LV diastolic volume index (LVDVI), LV mass index, and mass/volume ratio were determined angiographically according to the method of Lange and Rackley. The data were analyzed by repeated-measures ANOVA. Least-squares means and group SEM were calculated. No change in cardiac index, SVR, or baseline LVEDP was noted. The LVEDP doubled after fluid challenge, suggesting a restrictive process. The LVDVI remained near 60 mL/m2. The LV mass/volume ratio remained one. CONCLUSIONS: Appropriate increases in muscle mass occurred after cardiac transplantation, preserving normal mass/volume ratios despite somatic growth deficits associated with immunosuppressive therapy and denervation of the donor heart.

Body Surface Area↗

Effectiveness of radiofrequency catheter ablation for treatment of atrial tachycardia.

Catheter ablation has been used to treat atrioventricular node reentrant and atrioventricular reentrant tachycardias with extremely high success rates. The suitability of catheter ablation for treatment of atrial tachycardia, a much less common type of supraventricular tachycardia, has not been well addressed. Fifteen patients (8 females) ranging from 10 to 83 years (mean 38 +/- 22) were referred for catheter ablation of supraventricular tachycardia. The diagnosis of atrial tachycardia was established by standard electrophysiologic techniques. A combination of activation and pace mapping was used to identify a suitable site for radiofrequency current catheter ablation. Medical therapy was unsuccessful in all but 1 patient. Two patients had surgically corrected congenital heart disease, 2 had coronary artery disease and 1 had dilated cardiomyopathy. Seven patients had depressed left ventricular function. Six patients had incessant tachycardias. Presumed tachycardia mechanism was automatic in 11 patients and reentrant in 4. Mean tachycardia cycle length was 372 +/- 74 ms. Catheter ablation was acutely successful in 12 patients (80%) with application of 11.1 +/- 6.6 lesions at a mean voltage of 60 +/- 9 V. In the other 3 patients, 16 to 38 lesions were applied. At a mean follow-up of 18.5 +/- 6.5 months, 2 patients have had recurrences with different P-wave morphologies and underwent a second successful catheter ablation procedure. An additional 2 patients had recurrences with the same P-wave morphology and 1 underwent a second successful catheter ablation procedure. Thus, radiofrequency ablation can be used in a diverse population of patients with atrial tachycardia with an acute success rate of 80% and a long-term success rate of 73%.

Adult↗

Usefulness of metoprolol for unexplained syncope and positive response to tilt testing in young persons.

The efficacy of intravenous metoprolol in preventing symptoms during a repeat tilt test was compared with the outcome of chronic oral treatment in 21 patients (14 female, 7 male), age 8 to 20 years (mean 13 +/- 3) with unexplained syncope (> or = 1 episode) and a positive response to tilt testing. A positive response was defined as the development of either syncope or presyncope. During the initial tilt test, a positive response occurred during baseline (14 patients) or isoproterenol (0.03 to 0.1 microgram/kg/min) infusion (7 patients) with a cardioinhibitory (1 patient), vasodepressor (5 patients) or mixed (15 patients) pattern. Metoprolol (0.1 to 0.2 mg/kg) was administered intravenously. During the repeat tilt test, response was negative in 18 patients, including 11 of 14 patients with a positive response in the baseline and 7 of 7 patients with a positive response during isoproterenol infusion. Metoprolol (0.8 to 2.8 mg/kg/day) was administered orally to 15 patients for an average of 10 months. Symptoms were absent (7 patients) or improved (2 patients); metoprolol was discontinued because of adverse effects (3 patients) or recurrence of symptoms (3 patients). In 7 of 12 patients with a negative response and 2 of 3 patients with a positive response after intravenous metoprolol, oral administration of metoprolol prevented or improved symptoms without adverse effects. Many young patients (60%) with recurrent syncope obtained symptomatic improvement from chronic oral metoprolol treatment without adverse effects; repeat tilt testing after intravenous metoprolol did not appear to offer any additional information than would have been obtained from a trial of chronic oral treatment.

Administration, Oral↗

Restrictive ventricular septal defect: how small is too small to close?

Historically, indications for ventricular septal defect closure have included congestive heart failure, pulmonary hypertension, aortic insufficiency with or without aortic valve prolapse, and prior bacterial endocarditis. However, controversy exists as to how the lifetime risk of an isolated, nonoperated restrictive ventricular septal defect compares with the risk of surgical closure in an asymptomatic child. Between 1980 and 1991, cardiac catheterization and elective ventricular septal defect closure (age > 1 year, pulmonary to systemic flow ratio < 2.0) were performed in 141 patients aged 1 to 23 years (mean age, 6.1 +/- 4.7 years). Mean systolic pulmonary artery pressure was 26.9 +/- 13.0 mm Hg, and mean pulmonary to systemic flow ratio was 1.6 +/- 0.3. Aortic valve prolapse was present in 63 patients (45%), aortic insufficiency was present in 25 (18%), and 5 (3.5%) had prior bacterial endocarditis. There were no early or late deaths or major morbidity. No patient required a ventriculotomy to accomplish ventricular septal defect closure. Mean postoperative intensive care unit stay was 1.3 +/- 0.9 days, and mean hospital stay was 5.5 +/- 1.9 days. There were no instances of permanent complete atrioventricular dissociation, reoperations for bleeding, postoperative wound infections, or reoperations for residual or recurrent ventricular septal defect. These improved results justify a reevaluation of historic indications for ventricular septal defect closure.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Successful radiofrequency energy ablation of automatic junctional tachycardia preserving normal atrioventricular nodal conduction.

Automatic junctional tachycardia is frequently refractory to medical management and difficult to treat with nonpharmacological methods. A 12-year-old female with symptomatic, refractory automatic junctional tachycardia is reported. Earliest atrial activation during supraventricular tachycardia was in the posterior portion of the intraatrial septum. The patient underwent electrophysiological study and successful radiofrequency current ablation of the ectopic automatic focus within the atrioventricular junction. Normal atrioventricular junctional conduction was maintained, and at 7-month follow-up the patient has been free of tachycardia.

Atrioventricular Node↗

Heart rate perturbation in the stage 17-27 chick embryo: effect on stroke volume and aortic flow.

Heart rate (HR), stroke volume (SV), and aortic flow increase linearly between developmental stages 17 and 27, as the embryonic chick heart progresses from a bent tube to a rudimentary four-chambered structure and cardiac mass increases fourfold. We hypothesized that HR perturbation, expressed as percent of intrinsic HR (%HR), would have a developmentally dependent effect on flow and SV. HR was transiently perturbed to 40-250% of intrinsic rate with a 1-mm cooled or heated steel probe applied to the sinus venosus of 81 embryos. Aortic blood velocity, cross-sectional area, and HR were used to calculate flow and SV. At each stage, flow was maximal at intrinsic HR. The %HR vs. SV relationship was linear, inverse, and developmentally dependent. In spite of a tremendous change in ventricular shape, mass, and volume, HR control during development of the preinnervated heart maximizes blood flow to the developing embryo.

Animals↗

Reproducibility of a symptomatic response to upright tilt in young patients with unexplained syncope.

Serial upright tilt testing has been advocated as a possible therapeutic end point in treating patients with recurrent syncope, but the reproducibility of such testing has not been well-established in the absence of therapy. In 21 patients with recurrent syncope and a symptomatic response to upright tilt testing, tilt was repeated following a 25- to 30-minute recovery. Syncope or presyncope was reproduced in 14 of 21 patients on repeat tilt; 4 of 21 patients experienced milder symptoms, and 3 of 21 patients remained entirely asymptomatic on repeat tilt. The pattern of physiologic response was different during initial and repeat tilt in 7 of the 21 patients. Blood pressure and heart rate (before and during symptoms) were similar for the group on initial and repeat tilt. The abnormal physiologic response and associated symptoms elicited during upright tilt testing for unexplained syncope can be reproduced on immediate repeat tilt testing in most patients. However, the frequently attenuated response during repeat testing may limit its use as a therapeutic end point in individual patients.

Adolescent↗