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Biomedical subjects

V L Vetter

Publications and source records attributed to V L Vetter.

At least 19 recordsLinked to original sources
PubMed2001

Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children.

OBJECTIVES: We sought to identify the risk factors predicting the development of dilated cardiomyopathy (DCM) in patients with isolated congenital complete atrioventricular block (CCAVB). BACKGROUND: Recently evidence has emerged that a subset of patients with CCAVB develop DCM. METHODS: This was a retrospective study of 149 patients with CCAVB who had heart size and left ventricular (LV) function assessed by echocardiography and chest radiography over a follow-up period of 10 +/- 7 years. RESULTS: Nine patients developed DCM at the age of 6.5 +/- 5 years. No definite cause could be identified. In these nine patients, CCAVB was diagnosed in eight at 23 +/- 2.3 weeks gestation and in one at birth. Maternal SSA/SSB antibodies were confirmed in seven of the nine patients. Pacemakers were implanted in eight patients in the first month and in one patient at five years of age. The initial left ventricular end-diastolic dimension (LVEDD) was in the 96th +/- 2.6 percentile and the cardiothoracic (CT) ratio was 64 +/- 3.8% in the nine patients who developed DCM, and differed significantly in patients with CCAVB (p < 0.005) who did not develop DCM. The LVEDD and CT ratio did not decrease in the patients with CCAVB and DCM, but decreased significantly in the patients with CCAVB without DCM (p < 0.001) once pacing was initiated. Two patients with DCM died within two months of diagnosis; one patient is neurologically compromised; two patients received a heart transplant; and four patients are listed for heart transplantation. CONCLUSIONS: Isolated CCAVB is associated with a long-term risk for the development of DCM. Risk factors may be SSA/SSB antibodies, increased heart size at initial evaluation and the absence of pacemaker-associated improvement.

Adolescent↗
PubMed2001

Epicardial pacemaker implantation and follow-up in patients with a single ventricle after the Fontan operation.

OBJECTIVES: There is an increasing incidence of sinus node dysfunction after the Fontan procedure. Inability to maintain atrioventricular synchrony after the Fontan operation has been associated with an adverse late outcome. Although pacing may be helpful as a primary or adjunct modality after the Fontan procedure, the effects of performing a late thoracotomy or sternotomy for epicardial pacemaker implantation are unknown. In addition, little is known about the long-term effectiveness of epicardial leads in patients with single ventricles. The purpose of this study was to compare the hospital course and follow-up of epicardial pacing lead implantation in patients with Fontan physiology and patients with 2-ventricle physiology. METHODS: We retrospectively reviewed all isolated epicardial pacemaker implantations and outpatient evaluations performed between January 1983 and June 2000. RESULTS: There was no difference in the perioperative course for the 31 Fontan patients (27 atrial and 41 ventricular leads [68 total]) compared with the 56 non-Fontan subjects (9 atrial and 61 ventricular leads [70 total]). The median length of stay in Fontan and non-Fontan patients was 3 and 4 days, respectively. There was no early mortality in either group. Pleural drainage for 5 days or longer was reported in 4% of the Fontan cohort and 3% of the non-Fontan group. Late pleural effusions were identified in only 2 patients in the Fontan group and 2 patients in the non-Fontan group. There was no significant difference in epicardial lead survival between the Fontan group and the non-Fontan group (1 year, 96%; 2 years, 90%; 5 years, 70%). The overall incidence of lead failure was 17% (24/138). CONCLUSIONS: Epicardial leads can be safely placed in Fontan patients at no additional risk compared to patients with biventricular physiology. Sensing and pacing qualities were relatively constant in both the Fontan and non-Fontan groups over the first 2 years after implantation.

Adolescent↗
PubMed2000

Somatic growth failure after the Fontan operation.

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.

Age Factors↗
PubMed2000

Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction.

OBJECTIVE: To determine whether operations that theoretically jeopardize the sinus node (hemi-Fontan and/or lateral tunnel Fontan procedures) are associated with a greater risk of sinus node dysfunction than those that theoretically spare the sinus node (bidirectional Glenn and/or extracardiac conduit). METHODS: Between January 1, 1996, and December 31, 1999, a prospective cohort study was conducted evaluating the incidence of sinus node dysfunction in patients undergoing a bidirectional Glenn or hemi-Fontan procedure and those in whom the Fontan repair was completed with either an extracardiac conduit or a lateral tunnel. Sinus node dysfunction was defined (1) as a heart rate more than 2 SD below age-adjusted norms or (2) as a predominant junctional rhythm and/or a sinus pause of more than 3 seconds as determined by the resting electrocardiogram and/or ambulatory monitoring at hospital discharge. RESULTS: Fifty-one patients had a bidirectional Glenn shunt (mean age 7.8 +/- 5.1 months) and 79 a hemi-Fontan procedure (mean age 6.9 +/- 2.8 months). The incidence of sinus node dysfunction on postoperative day 1 was significantly higher after the hemi-Fontan (36%) than after the bidirectional Glenn shunt (9.8%); however, by hospital discharge this difference was no longer apparent (hemi-Fontan [8%]; bidirectional Glenn [6%]; P = not significant). No difference in early sinus node dysfunction was discernible after the extracardiac conduit (4/30 [13%]) compared with the lateral tunnel Fontan procedure (6/46 [13%]) (P = not significant). No diagnostic or perioperative variables were predictive of sinus node dysfunction. CONCLUSIONS: Avoidance of surgery near the sinus node has no discernible effect on the development of early sinus node dysfunction. Thus, concerns about early sinus node dysfunction should not override patient anatomy or surgeon preference as determinants of which cavopulmonary anastomosis to perform.

Anastomosis, Surgical↗
PubMed1999

Comparison of left and right atrial epicardial pacing in patients with congenital heart disease.

BACKGROUND: Complex congenital heart disease (CHD) often necessitates the use of epicardial pacing. Adequate right atrial (RA) sensing and pacing thresholds are often difficult to obtain due to suture line scarring and RA dilatation. The purpose of this study was to evaluate the placement of left atrial (LA) epicardial leads in children. METHODS: Patient demographics, pacing, and sensing data of atrial pacing systems implanted between January 1994 and January 1997 were collected. RESULTS: Forty-nine pacing systems were implanted: 14 LA epicardial, 19 RA epicardial, and 16 transvenous in the right atrium. Lead impedance, current, and energy were similar in the two epicardial groups throughout the study. Energy thresholds (ET) were lower in the LA than RA at 6 months, and 1 and 2 years (p < 0.05). Analysis of post-Fontan patients performed alone revealed a lower ET in the LA as compared with the RA. Pacing and sensing parameters from transvenous leads are presented for relative comparison. CONCLUSIONS: Transvenous leads are most efficient but often contraindicated in complex CHD. LA leads offer lower energy thresholds than RA leads with similar sensing parameters.

Adolescent↗
PubMed1999

Low temperature and low energy radiofrequency modification of atrioventricular nodal slow pathways in pediatric patients.

OBJECTIVES: To report our experience using low temperature and energy in the modification of the slow pathway in pediatric patients with atrioventricular nodal reentrant tachycardia. BACKGROUND: A concern in performing a slow pathway modification is the possible damage of the normal AV conduction system. Lesion size has been shown to have a linear relationship with temperature. Previous reports have used energy of 25-50 W that generate temperatures of 60 degrees C -70 degrees C for successful procedures. METHODS: Report of results of attempted AV nodal slow pathway modification in 17 consecutive pediatric patients < 15 years of age at The Children's Hospital of Philadelphia from April 1995 to November 1997 using low temperature and energy. RESULTS: There were 18 successful slow pathway modifications with 1 recurrence in 17 patients. The maximum energy used during successful lesions was 32.7 +/- 13.8 W (range 15-50 W) with a mean energy of 26.4 +/- 13.3 W (range 12-48 W). The peak temperature during these lesions was 55.1 degrees C +/- 4.1 degrees C (range 48 degrees C-64 degrees C) with a mean temperature of 47.9 degrees C +/- 2.7 degrees C (range 44 C-540C). The mean number of radiofrequency lesions required for a successful modification was 5.8 +/- 6.7 (median 4.0, range 1-26). Patients have been followed for 2.08 +/- 0.79 years. CONCLUSIONS: Slow pathway modification can be performed successfully with a low incidence of recurrence in the pediatric patient using low energy and temperature. It is possible that this may lead to smaller lesions.

Adolescent↗
PubMed1998

Sinus node function after a systematically staged Fontan procedure.

BACKGROUND: Sinus node dysfunction has been previously reported to occur in 13% to 16% of patients after the Fontan operation. Although there is concern that an intermediate cavopulmonary connection may increase the risk of sinus node dysfunction, previous studies have not reported on patients routinely staged to a Fontan operation. This study sought to determine the early and late incidences of sinus node dysfunction in patients systematically and uniformly staged to a Fontan operation after a prior hemi-Fontan. METHODS AND RESULTS: To determine the early incidence of sinus node dysfunction, hospital records and perioperative ECGs were reviewed in all 287 patients having had a staged Fontan operation between January 1990 and December 1995. A cross-sectional analysis was performed on 220 of 239 surviving patients (92%) to determine the late incidence of sinus node dysfunction. Sinus node dysfunction was present in 7% of the patients before and in 15% after the hemi-Fontan. Although most patients (81%) regained normal sinus node function between the 2 stages, 23% had sinus node dysfunction in the early postoperative period after the Fontan. Of the 95 patients followed for > 4 years after the Fontan operation, 44% had sinus node dysfunction. However, at a mean follow-up of 3.5 +/- 1.7 years, only 16 patients (6.7%) had received a pacemaker and only 10 (4.1%) had documented atrial flutter. CONCLUSIONS: Perioperative sinus node dysfunction is common after both the hemi-Fontan and the Fontan procedures. Although many patients regain sinus node function between the 2 stages, late sinus node dysfunction is common and more likely to occur in patients with early sinus node dysfunction and those with longer follow-up.

Adolescent↗
PubMed1998

Perioperative conduction and rhythm disturbances after the Ross procedure in young patients.

BACKGROUND: The Ross procedure is performed for a variety of left ventricular outflow tract diseases in children. The preoperative hemodynamic burden of pressure or volume overload and associated ventricular hypertrophy can predispose to ventricular arrhythmias. Additional procedures performed with the Ross procedure (eg, Konno) may damage the conduction system. METHODS: Between January 1995 and February 1997, the Ross procedure was performed in 42 patients, 31 (74%) of whom had 71 prior interventions. Concomitant procedures (n = 42 in 23 patients) included 17 annular-enlarging procedures. Screening was performed for perioperative conduction and rhythm abnormalities. RESULTS: There was one postoperative death. Perioperative ventricular tachycardia occurred in 12 patients (29%), with 2 receiving antiarrhythmic medication for ventricular tachycardia at discharge. Transient complete heart block occurred in 3 patients, all of whom had concomitant procedures performed in the subaortic area; all patients were discharged in sinus rhythm and no patient received a permanent pacemaker. CONCLUSIONS: The Ross procedure can be performed successfully in children with complex cardiac disease with low mortality and perioperative morbidity. The incidence of perioperative ventricular tachycardia is high (29%), suggesting the need for vigilant perioperative monitoring and long-term surveillance.

Arrhythmias, Cardiac↗
PubMed1997

Lack of cystic fibrosis transmembrane regulator-type chloride current in pediatric human atrial myocytes.

Mature cardiomyocytes have been shown to possess a cyclic AMP-mediated chloride channel (I(Cl)) which is the product of the cystic fibrosis transmembrane regulator (CFTR) gene. Species variability has been demonstrated for other ion channels. This study was designed to evaluate human I(Cl) regulation using the whole-cell patch-clamp bioassay. Atrial tissue obtained from children undergoing congenital heart surgery was enzymatically dispersed into isolated myocytes. The patients ranged in age from 1 day to 11 years (mean 2 years). Isoproterenol was used to activate the cAMP second-messenger system in a potassium-free environment. Membrane calcium and sodium channels were pharmacologically blocked. Of 20 human atrial myocytes obtained from 13 pediatric patients, 80% had a small basal chloride current. The current could be inhibited by the anion transport blocker, 9-anthracene carboxylic acid. In 4 of 20 otherwise viable myocytes, no I(Cl) could be elicited, either at baseline or with beta-adrenergic stimulation. Of the 16 myocytes with a basal I(Cl), the current was unaffected by cAMP stimulation in 15 (94%) cells. There were no significant differences in age, gender or clinical status of patients whose cells conducted Cl- current compared with patients whose myocytes had no measurable I(Cl). Ten mature guinea pig ventricular myocytes were evaluated using the same whole-cell patch-clamp technique. Seven of 10 cells showed a reversible increase in I(Cl) with isoproterenol exposure. Despite presence of the CFTR gene in human cardiomyocytes, functional expression of the cAMP-activated I(Cl) does not appear evident in isolated pediatric atrial myocytes. Whether the pathophysiology of congenital heart disease may influence chloride current modulation via alterations in adrenergic tone, intracellular Ca2+ regulation, and cellular osmotic conditions remains to be established.

Adrenergic beta-Agonists↗
PubMed1997

Electrophysiologic properties of the atrioventricular node in pediatric patients.

OBJECTIVES: The purpose of this study was to characterize anterograde and retrograde properties of the atrioventricular (AV) node in children and to determine the presence of ventriculoatrial (VA) conduction and dual AV node pathways. BACKGROUND: Although AV node reentry is common in adults, it accounts for 13% of pediatric supraventricular tachycardia (SVT). The age-related changes in the AV node with development are poorly understood. The incidence of dual AV node pathways and VA conduction in the pediatric population is unknown. METHODS: Electrophysiologic studies were performed in 79 patients with normal hearts and no evidence of AV node arrhythmias. Patients were classified into two groups by age: group I = 49 patients (0.39 to 12.8 years old, mean [+/- SD] age 8.5 +/- 3.6); group II = 30 patients (13.4 to 20.0 years old, mean age 15.6 +/- 1.8). RESULTS: There was a significant difference (p < 0.05) in the cycle length (CL) at which anterograde AV block occurred between group I (305 +/- 63 ms) and group II (350 +/- 91 ms). Sixty-one percent of children had VA conduction with no age-related differences. There was no significant difference in the mean CL of retrograde VA block (360 ms). The incidence of dual AV node pathways in group I was 15% and 44% in group II (p < 0.05). CONCLUSIONS: These findings suggest that AV node electrophysiology undergoes maturational changes. The increase in AV node reentrant tachycardia in adults may relate to changes in the relative refractoriness and conduction of the AV node or to differences in autonomic input into the AV node that allow dual pathway physiology to progress to SVT.

Adolescent↗
PubMed1997

QT and JT dispersion in children with long QT syndrome.

INTRODUCTION: Abnormalities of ventricular repolarization leading to ventricular arrhythmias place children with long QT syndrome at high risk for sudden death. Dispersion of the QT (QTd) and JT (JTd) intervals, as markers of cardiac electrical heterogeneity, may be helpful in evaluating children with long QT syndrome and identifying a subset of patients at high risk for development of critical ventricular arrhythmias (ventricular tachycardia, torsades de pointes, and/or cardiac arrest). METHODS AND RESULTS: The QTd and JTd intervals in 39 children with long QT syndrome were compared to those of 50 normal age-matched children. In the long QT syndrome group, QTd measured 81 +/- 70 msec compared to 28 +/- 14 msec in the control group (P < 0.05), and JTd in the long QT syndrome group was 80 +/- 69 msec compared to 25 +/- 15 msec in the control group (P < 0.05). CONCLUSION: Children with long QT syndrome have an increased QTd and JTd when compared to normal controls. A QTd or JTd > or = 55 msec correlates with the presence of critical ventricular arrhythmias. These ECG measures of dispersion can be useful in stratifying children with the long QT syndrome who are at higher risk for developing critical ventricular arrhythmias.

Adolescent↗
PubMed1996

Slipping rib syndrome as a cause of chest pain in children.

Four patients with the slipping rib syndrome presenting as chest pain are described, and the appropriate literature is reviewed. In two of the patients the physicians caring for the children were initially concerned that a cardiac condition was the cause of the chest pain, and a cardiac evaluation was done. In one patient an emotional cause for the pain was first considered and then a cardiac cause was pursued. In the last patient esophagitis was thought to be the cause and the child was referred to a gastroenterologist. It is suggested that slipping rib syndrome should be considered by physicians when evaluating children with a complaint of chest pain. The condition can be easily diagnosed on physical examination and therefore may save some patients from an unnecessary cardiac or gastroentestinal evaluation.

Adolescent↗
PubMed1994

Use of the rate-corrected JT interval for prediction of repolarization abnormalities in children.

A prolonged rate-corrected QT interval (QTc) may be associated with an increased risk of developing ventricular arrhythmias and sudden death, particularly in patients with the hereditary long QT syndrome (LQTS), myocardial ischemia, or antiarrhythmic medication toxicity. It is known that there are some patients with LQTS who sometimes have a borderline or normal QTc (< or = 0.45 second). Although the QTc has been the standard measurement of ventricular repolarization, it includes both depolarization and repolarization and may not always be a sensitive indicator of the type of repolarization abnormalities seen in LQTS. Intraventricular conduction abnormalities complicate evaluation of the QTc interval. The rate-corrected JT interval (JTc) is a more accurate measurement of ventricular repolarization, and therefore may be a more sensitive means of assessing abnormalities. The QTc on a resting electrocardiogram was determined in 40 patients with LQTS and in 31 patients with right bundle branch block after tetralogy of Fallot repair. These were compared with 1,000 age-matched control subjects. The right bundle branch block group had normal JT and JTc measurements, despite having prolonged QT and QTc intervals compared with controls. The JTc identified 85% of patients affected with LQTS compared with only 58% identified using only the QTc as a marker for the syndrome. The JTc is a more specific measurement of ventricular repolarization than the QTc by eliminating QRS duration variability. It appears to be a more sensitive predictor of repolarization abnormalities, and may be helpful in identifying patients with LQTS who have borderline or normal QTc measurements on resting electrocardiograms.

Adolescent↗
PubMed1992

Electrocardiographic changes and arrhythmias after cancer therapy in children and young adults.

Transient electrocardiographic changes and arrhythmias are known to be acute manifestations of cardiotoxicity secondary to cancer therapy with anthracyclines or cardiac irradiation. However, despite the known risk of late cardiac dysfunction in survivors of childhood cancer therapy, the risk of clinically important electrocardiographic abnormalities and arrhythmias after treatment is unknown. Standard 12-lead and 24-hour ambulatory electrocardiograms were recorded in 73 patients who received anthracyclines alone, 24 who received cardiac irradiation alone, and 27 who received both anthracyclines and cardiac irradiation. The mean age of the patients was 15 years. Mean cumulative anthracycline dose was 282 mg/m2 in patients who received anthracyclines alone and 244 mg/m2 in patients who received both anthracyclines and cardiac irradiation. Analysis of the 12-lead and 24-hour electrocardiograms demonstrated increased frequency of QTc prolongation, supraventricular premature complexes, supraventricular tachycardia, ventricular premature complexes, couplets and ventricular tachycardia (all p less than 0.001) when compared with an age-matched healthy population. Most patients had abnormalities limited to single supraventricular or ventricular premature complexes; however, potentially serious ventricular ectopy, including ventricular pairs and ventricular tachycardia, were noted in patients with cumulative doses greater than 200 mg/m2. Electrocardiographic abnormalities and arrhythmias are not limited to the acute phase of treatment with anthracyclines and cardiac irradiation. Survivors of childhood malignancy who received anthracyclines or cardiac irradiation, or both, probably should undergo ambulatory electrocardiographic monitoring as part of their follow-up to detect potentially life-threatening arrhythmias.

Adolescent↗
PubMed1991

Electrophysiologic findings after Fontan repair of functional single ventricle.

Cardiac arrhythmias are well recognized sequelae of the Fontan operation for complex congenital anomalies. In this study the electrophysiologic effects of the Fontan procedure were evaluated in 30 patients who underwent cardiac catheterization with electrophysiologic study 1.9 +/- 1.3 years (mean +/- SD) after modified Fontan repair for functional single ventricle. Abnormalities of sinus node or ectopic pacemaker automaticity were detected in 50% (15 patients) by determination of a prolonged corrected sinus node or pacemaker recovery time. Total sinoatrial conduction time was prolonged in 50% of the patients with normal sinus rhythm. Sinus node or ectopic atrial pacemaker function was entirely normal in only 43% of patients. The predominant atrial rhythm was normal sinus in 70% and ectopic atrial or junctional in 30%. Abnormalities of atrial effective and functional refractory periods were noted in 43% of patients and were most pronounced at faster paced cycle lengths. Atrial endocardial catheter mapping revealed intraatrial conduction delays between adjacent sites in 76% of the patients tested and in eight of nine patients with inducible intraatrial reentry. Programmed atrial stimulation induced nonsustained supraventricular arrhythmias in 10% of the 30 patients and sustained arrhythmias in 27%. Intraatrial reentry was the most common inducible arrhythmia and was present in seven of the eight patients with sustained and two of the three patients with nonsustained atrial arrhythmias. Atrioventricular conduction abnormalities were noted in 10% (three patients). No patient had inducible ventricular arrhythmias with programmed ventricular stimulation. The electrophysiologic findings after Fontan repair include abnormal sinus node function, prolonged atrial refractoriness, delayed intraatrial conduction and inducible atrial arrhythmias.(ABSTRACT TRUNCATED AT 250 WORDS)

Arrhythmias, Cardiac↗