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Biomedical subjects

G Wernovsky

Publications and source records attributed to G Wernovsky.

At least 37 records · Page 2Linked to original sources
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↗
PubMed2000

Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome.

OBJECTIVES: The purposes of this study are to describe the quality of life and cognitive function in school-aged children who have undergone staged palliation for hypoplastic left heart syndrome (HLHS), and to identify factors that are predictive of neurodevelopmental outcome in this population. METHODS: School-aged survivors with HLHS who had undergone palliative surgery at our institution were identified and mailed a questionnaire to assess subjectively quality of life, school performance, and incidence of medical complications. A subgroup of local patients underwent standardized testing of cognitive function and neurologic examination. These patients were compared with the larger (remote) group of questionnaire respondents to determine whether results may be generalizable to the entire HLHS population. Potential predictors of neurologic and cognitive outcome were tested for their association with test scores using multivariate regression analysis. RESULTS: Questionnaire results were obtained from 115 of 138 eligible children (83%; mean age: 9.0 +/- 2.0 years). Standardized testing was performed in 28 of 34 (82%) eligible local patients (mean age: 8.6 +/- 2.1 years). The majority of parents or guardians described their child's health as good (34%) or excellent (45%) and their academic performance as average (42%) or above average (42%). One third of the children, however, were receiving some form of special education. Chronic medication usage was common (64%); the incidence of medical complications was comparable to that previously reported in children with Fontan physiology. Cognitive testing of the local group demonstrated a median full scale IQ of 86 (range: 50-116). Mental retardation (IQ: <70) was noted in 18% of patients. In multivariate analysis, only the occurrence of preoperative seizures predicted lower full scale IQ. CONCLUSIONS: Although the majority of school-aged children with HLHS had IQ scores within the normal range, mean performance for this historical cohort of survivors was lower than that in the general population.

Adolescent↗
PubMed2000

Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes.

OBJECTIVE: Necrotizing enterocolitis (NEC) is primarily a disease of the premature infant. Among children born at term, however, congenital heart disease may be an important predisposing factor for this condition. To determine risk factors for NEC in patients with congenital heart disease, we conducted a case-control study of neonates with cardiac disease admitted to the cardiac intensive care unit at our center during the 4-year period from January 1995 to December 1998. METHODS: Cardiac diagnosis and age at admission were analyzed for association with NEC among the 643-patient inception cohort. Demographic, preoperative, and operative variables were recorded retrospectively in 21 neonates with congenital heart disease who developed NEC and 70 control neonates matched by diagnosis and age at admission. Using parametric and nonparametric analysis, cases and controls were compared with respect to previously identified risk factors for NEC. RESULTS: Among the entire cohort of 643 neonates with heart disease admitted to the cardiac intensive care unit, diagnoses of hypoplastic left heart syndrome (odds ratio [OR] = 3.8 [1.6-9.1]) and truncus arteriosus or aortopulmonary window (OR = 6.3 [1.7-23.6]) were independently associated with development of NEC by multivariable analysis. In the case-control analysis, earlier gestational age at birth (36.7 +/- 2. 7 weeks vs 38.1 +/- 2.3 weeks), prematurity (OR = 3.9 [1.2-12.5]), highest dose of prostaglandin >0.05 microg/kg/minute (OR = 3.9 [1. 2-12.5]), and episodes of low cardiac output (meeting specific laboratory criteria) or clinical shock (OR = 6.5 [1.8-23.5]) correlated with the development of NEC. Earlier gestational age and episodes of low output were the only factors that remained significantly associated with NEC by multivariable analysis. Although there was no difference in hospital mortality between patients with and without NEC, mean hospital stay was significantly longer in those who developed NEC (36 +/- 22 days vs 19 +/- 14 days). CONCLUSIONS: The risk of NEC in neonates with congenital heart disease is substantial. Factors associated with an elevated risk of NEC in infants with heart disease include premature birth, hypoplastic left heart syndrome, truncus arteriosus, and episodes of poor systemic perfusion or shock. Heightened suspicion is warranted in newborns with these risk factors.

Case-Control Studies↗
PubMed1999

Early results of the Ross procedure in simple and complex left heart disease.

BACKGROUND: The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. Benefits include the lack of anticoagulation after surgery and the potential growth and durability of the autograft. The purpose of this study was to review our institutional experience with the Ross procedure and to compare early outcome in simple aortic valve disease and complex left heart disease. METHODS AND RESULTS: Between January 1995 and October 1998, 66 patients (median age, 10.8 years; range, 6 days to 34.8 years) underwent the Ross procedure. The primary indication for surgery was isolated valvular disease in 41 patients: aortic stenosis (AS; n=3), aortic insufficiency (AI; n=11), and AS/AI (n=27). The remaining 25 patients had multiple levels of left ventricular outflow tract obstruction, 12 of whom had at least moderate AI. Additional left heart disease in the complex group included subaortic stenosis (n=20), arch obstruction (n=7), mitral valve disease (n=5), apical aortic conduit stenosis or insufficiency (n=3), and supravalvar AS (n=2). There were 123 prior interventions performed in 51 patients, including aortic valvotomy/valvuloplasty (n=56), coarctation repair (n=21), subaortic stenosis resection/Konno procedure (n=10), ventricular septal defect closure (n=8), apical aortic conduit placement (n=3), aortic valve replacement (n=3), and other (n=22). An isolated Ross procedure was performed in 41 patients, 10 of whom required concurrent aortic annulus enlargement procedure to accommodate the larger pulmonary autograft. In the remaining 25 patients, 49 concurrent procedures were performed, including the Konno procedure (n=17), aortic annulus enlargement (n=2), subaortic membrane resection (n=9), arch augmentation (n=5), mitral valvuloplasty (n=5), ventricular septal defect closure (n=4), apicoaortic conduit division (n=3), and other (n=4). One patient (1.5%) died 3 days after a Ross-Konno procedure, which included arch reconstruction, from presumed arrhythmia. There were no other early deaths. One patient required ECMO (extracorporeal membrane oxygenation) for 3 days after a ventricular tachycardia (VT)-related cardiac arrest. Transient complete heart block was seen in 4 patients; the duration was <5 days. No patient had left ventricular outflow tract obstruction on discharge echocardiography. Neo-AI was graded as none (n=5), trivial-mild (n=57), or moderate (n=3). All 3 patients with moderate neo-AI at discharge had abnormal pulmonary valves before surgery. Perioperative VT was noted in 18 patients (27.2%), 2 of whom were discharged on antiarrhythmic medication. CONCLUSIONS: The Ross procedure can be performed in isolation or in combination with other complex procedures with low mortality (1.5%) and acceptable short-term results, even in patients with complex left heart disease and multiple prior interventions. Postoperative VT is common. Anatomic abnormalities of the pulmonary valve preclude its use as an autograft.

Adolescent↗
PubMed1999

Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome.

BACKGROUND: Results of staged palliation for hypoplastic left heart syndrome (HLHS) have improved in recent years; however, certain risk factors have been associated with decreased survival rates. METHODS AND RESULTS: We retrospectively reviewed the medical records of 67 patients weighing </=2.5 kg undergoing the first stage of reconstructive surgery at our institution between January 1, 1990, and December 31, 1997. HLHS was present in 45 patients, complex double-outlet right ventricle in 10, unbalanced AV canal in 5, tricuspid atresia with transposition of the great vessels in 4, and other diagnoses in 3. Mean age at surgery was 10.1+/-10.7 days (median, 8 days), and mean weight was 2.2+/-0.3 kg (median, 2.2 kg). Fourteen patients weighed </=2.0 kg, and 2 patients weighed </=1.5 kg. Early mortality (death within 30 days or before hospital discharge) was 51% (34 of 67). No patient, procedural, or time-related variables correlated with increased mortality. However, there was a trend toward increased mortality with increased cardiopulmonary bypass time (P=0.076) and decreased preoperative ventricular performance (P=0.139). CONCLUSIONS: These findings suggest that low weight alone in a patient with HLHS or an anatomic variant should not be considered a contraindication to staged reconstructive surgery.

Body Weight↗
PubMed1999

Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology.

OBJECTIVES: We sought to determine if early ventricular volume unloading improves aerobic capacity in patients with single ventricle Fontan physiology. BACKGROUND: Surgical strategies for patients with single ventricle include intermediate staging or early Fontan completion to reduce the adverse affects of prolonged ventricular volume load. The impact of this strategy on exercise performance has not been evaluated. METHODS: Retrospectively, we reviewed the exercise stress test results of all preadolescents with single ventricle Fontan physiology. "Volume unloading" was considered to have occurred at the time of bidirectional cavopulmonary anastomosis or at the time of Fontan surgery in those patients who did not undergo intermediate staging. Potential predictors of aerobic capacity were analyzed using multivariate regression. RESULTS: The patients (n = 46) achieved a mean percentage predicted of maximal oxygen consumption (VO2max) of 76.1% +/- 21.1%. The mean age at the time of volume unloading was 2.7 +/- 2.4 years, and the mean age at testing was 8.7 +/- 2 years. Intermediate staging was performed in 16 of 46 patients (35%). In multivariate analysis, younger age at volume unloading was associated with increased aerobic capacity (p = 0.003). Other variables were not predictive. The subgroup of patients who underwent volume unloading before two years of age achieved a mean percentage predicted VO2max of 88.6% +/- 24.1%. CONCLUSIONS: Preadolescents with single ventricle who undergo volume unloading surgery at an early age demonstrate superior aerobic capacity compared with those whose surgery is delayed until a later age.

Age Factors↗
PubMed1999

Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.

BACKGROUND: It is not known whether developmental and neurological outcomes in the preschool period differ depending on whether the predominant vital organ support strategy used in infant heart surgery was total circulatory arrest (CA) or low-flow cardiopulmonary bypass. METHODS AND RESULTS: Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a support method consisting predominantly of CA or low-flow cardiopulmonary bypass. Developmental and neurological status were evaluated blindly at 4 years of age in 158 of 163 eligible children (97%). Neither IQ scores nor overall neurological status were significantly associated with either treatment group or duration of CA. The CA group scored lower on tests of motor function (gross motor, P=0.01; fine motor, P=0.03) and had more severe speech abnormalities (oromotor apraxia, P=0.007). Seizures in the perioperative period, detected either clinically or by continuous electroencephalographic monitoring, were associated with lower mean IQ scores (12.6 and 7.7 points, respectively) and increased risk of neurological abnormalities (odds ratios, 8.4 and 5.6, respectively). The performance of the full cohort was below expectations in several domains, including IQ, expressive language, visual-motor integration, motor function, and oromotor control. CONCLUSIONS: Use of CA to support vital organs during open heart surgery in infancy is associated, at the age of 4 years, with worse motor coordination and planning but not with lower IQ or with worse overall neurological status.

Cardiac Surgical Procedures↗
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

Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome. Boston Circulatory Arrest Study Group.

BACKGROUND: The outcome of infants who have transient seizures after open heart surgery has not been studied. Using the database of the Boston Circulatory Arrest Study involving 171 children with D-transposition of the great arteries, we explored the relationship between early postoperative clinical and EEG seizures and neurodevelopmental outcomes at ages 1 and 2 1/2 years. METHODS AND RESULTS: At 1 year, children returned for developmental and neurological evaluations and MRI. Parent-completed developmental questionnaires were collected at 2 1/2 years of age. At 1 year, children with early postoperative seizures had lower Psychomotor Development Index (motor function) scores (clinical seizures: 12.9 mean difference [MD]; 95% confidence interval [CI], 2.2 to 23.6; P=.02; EEG seizures: 13.3 MD; 95% CI, 6.8 to 19.7; P<.001). Mental Developmental Index scores of children with clinical or EEG seizures were also lower, but the differences were not statistically significant. Infants with seizures were more likely to have an abnormal neurological examination (clinical seizures: 78% versus 31%; P=.008; EEG seizures: 58% versus 34%; P=.04). Children with EEG seizures were more likely to have MRI abnormalities (43% versus 13%, P=.002). At age 2 1/2, children with EEG seizures had lower scores in several areas of function. CONCLUSIONS: In infants undergoing the arterial switch operation for correction of D-transposition of the great arteries, transient postoperative clinical and EEG seizures were associated with worse neurodevelopmental outcomes at ages 1 and 2 1/2 years as well as neurological and MRI abnormalities at 1 year of age. The occurrence of such seizures may provide an early sign of brain injury with neurological and developmental sequelae.

Age Factors↗
PubMed1998

Surgical management of tricuspid atresia and anomalous left brachiocephalic vein.

An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia.

Anastomosis, Surgical↗
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

Modified ultrafiltration reduces postoperative morbidity after cavopulmonary connection.

BACKGROUND: Modified ultrafiltration reduces the deleterious effects of cardiopulmonary bypass in children. Patients undergoing repair of single-ventricle cardiac anomalies may be particularly sensitive to these adverse effects, and benefit from the use of modified ultrafiltration. METHODS: From January 1995 to June 1996, 120 consecutive cavopulmonary operations were performed at The Children's Hospital of Philadelphia. Procedures included lateral tunnel fenestrated Fontan (n = 50), extracardiac Fontan (n = 5), hemi-Fontan (n = 60), and bidirectional Glenn shunt (n = 5). Modified ultrafiltration was performed after cardiopulmonary bypass in 41 patients, and results were compared by t test with a control group of 79 patients in whom modified ultrafiltration was not used. RESULTS: There was one death for an operative (30-day) mortality of 0.8%. Age, weight, diagnosis, ischemic arrest time, and cardiopulmonary bypass time were similar between the modified ultrafiltration and control groups. Postoperative blood use, chest tube output, the incidence of pleural and pericardial effusions, and hospital stay were all significantly decreased when modified ultrafiltration was used. CONCLUSIONS: By lowering the perioperative morbidity of staged cavopulmonary operations, modified ultrafiltration makes an important contribution to improving outcome after the correction of single-ventricle cardiac anomalies.

Adolescent↗
PubMed1997

Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

OBJECTIVES: The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. METHODS: The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. RESULTS: The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). CONCLUSIONS: These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.

Child↗
PubMed1997

Functional outcome after the Fontan operation: factors influencing late morbidity.

OBJECTIVES: The purpose of this study was to describe the functional outcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might influence late functional state. METHODS: A comprehensive cross-sectional review of the first 500 patients undergoing a Fontan operation at our institution was undertaken. Those surviving with an intact Fontan circulation were reviewed by questionnaire to assess functional status and medication history. Medical records, chest roentgenograms, echocardiograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status. RESULTS: Three hundred sixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up. Median age at operation was 5.0 years (range 0.4 to 31 years), and median follow-up was 5.4 years (range 1.7 to 20 years). Most patients (91.1%) were in New York Heart Association class I or II. In a multivariate model, poor (class III or IV) functional state was associated with longer duration of follow-up (p < 0.001), a prior atrial septectomy (p = 0.03), and a prior main pulmonary artery-ascending aorta anastomosis (p = 0.05). CONCLUSIONS: A poor functional outcome is uncommon after the Fontan operation but becomes more frequent with increasing duration of follow-up.

Adolescent↗
PubMed1997

Factors that influence the development of atrial flutter after the Fontan operation.

OBJECTIVES: Atrial flutter is a frequent, potentially fatal complication of the Fontan operation, but risk factors for its development are ill defined. We evaluated clinical features that might predict the development of atrial flutter in patients who had a Fontan operation. METHODS: We evaluated 334 early survivors of a Fontan operation done between April 1973 and July 1991 (mean follow-up, 5.0 +/- 3.8 years). Evaluation included electrocardiography, Holter monitor recordings, and chart review. Modifications of the Fontan operation included an extracardiac conduit (n = 43), an atriopulmonary anastomosis (n = 117), or a total cavopulmonary anastomosis (n = 174). Patient, time, and procedure-related variables were analyzed with respect to the development of atrial flutter. RESULTS: Atrial flutter was identified in 54 (16%) patients at a mean of 5.3 +/- 4.7 years (range 0 to 19.7 years) after Fontan operation. Atrial flutter developed sooner and was more likely to occur in patients who were older at the time of Fontan operation (12.4 +/- 7.6 vs 6.3 +/- 5.2 years; p < 0.001), had a longer follow-up interval (8.7 +/- 3.9 vs 4.4 +/- 3.4 years; p < 0.001), had a prior atrial septectomy or pulmonary artery reconstruction (p < 0.01), and had worse New York Heart Association class symptoms (p < 0.02). The presence of sinus node dysfunction was associated with a higher incidence of atrial flutter (p < 0.001). Although there was a lower prevalence of atrial flutter in those patients with a total cavopulmonary anastomosis, the follow-up for this group was shorter. Anatomic diagnoses, perioperative hemodynamics, and other previous palliative operations were not associated with an increased incidence of atrial flutter. Multivariate analysis identified age at operation, duration of follow-up, extensive atrial baffling, and type of repair as factors associated with the development of atrial flutter after Fontan operation. CONCLUSION: Atrial flutter continues to develop with time after the Fontan operation. Further follow-up is necessary to determine whether a total cavopulmonary anastomosis reduces the incidence of atrial flutter.

Atrial Flutter↗