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Biomedical subjects

Gil Wernovsky

Publications and source records attributed to Gil Wernovsky.

At least 19 recordsLinked to original sources
PubMed2006

Early results of single-stage biventricular repair of severe aortic hypoplasia or atresia with ventricular septal defect and normal left ventricle.

OBJECTIVE: Biventricular repair of aortic atresia (or severe aortic hypoplasia) is possible in the presence of a ventricular septal defect and normal left ventricle. We considered whether primary biventricular repair was a safe alternative in all cases, even in the presence of interrupted aortic arch. METHODS: This was a retrospective analysis of patients who underwent primary biventricular repair consisting of a combination Norwood-type reconstruction of the aortic arch, baffle of the left ventricle to both semilunar roots, and conduit placement from the right ventricle to pulmonary arteries. RESULTS: Between January 1995 and January 2005, a total of 21 patients underwent primary biventricular repair at a median age of 5 days and a median weight of 3.0 kg. Aortic atresia was present in 7 and aortic stenosis in 14; 6 had interrupted aortic arch. All patients with aortic stenosis had annular diameters 3 mm or smaller. Median circulatory arrest time was 55 minutes, aortic crossclamp time was 56 minutes, and total support time was 99 minutes. In-hospital survival was 100%. Postoperative echocardiography in 19 patients demonstrated no significant outflow tract obstruction. Total stay was a median of 17 days. At midterm follow-up, there has been 1 late death, and reoperation has been necessary in 10 cases. CONCLUSION: Primary biventricular repair is a safe alternative to staged repair in all cases of aortic hypoplasia with ventricular septal defect and normal left ventricle.

Aorta, Thoracic↗
PubMed2006

Neurodevelopmental outcome after early repair of a ventricular septal defect with or without aortic arch obstruction.

OBJECTIVES: Cross-sectional studies of intermediate-term survivors of infant cardiac surgery have revealed a high frequency of neurodevelopmental disabilities. Few data exist regarding neurodevelopmental outcome of infants undergoing surgical intervention for a ventricular septal defect. The purpose of this study was to evaluate the neurodevelopmental outcome at 1 year of age of children who had surgical repair in infancy of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction. METHODS: Children who underwent repair of a ventricular septal defect or single-stage repair of a ventricular septal defect with aortic arch obstruction at less than 6 months of age were assessed at 1 year of age by using the Bayley Scales of Infant Development II, which yields the Mental Development Index and the Psychomotor Development Index, both with an expected mean of 100 +/- 15. RESULTS: At 1 year, 55 patients (ventricular septal defect alone = 36; ventricular septal defect with aortic arch obstruction = 19) returned for evaluation. The mean Mental Development Index was 92.6 +/- 11.7, with 3 (5%) patients scoring 70 or less. The mean Psychomotor Development Index was 86.1 +/- 16.4, with 10 (18%) patients scoring 70 or less. Patients with a suspected or confirmed genetic syndrome had both a lower Mental Development Index score (P = .011) and a lower Psychomotor Development Index score (P = .001). Mental Development Index and Psychomotor Development Index were independent of anatomic (specifically aortic arch obstruction) and intraoperative factors (specifically deep hypothermic circulatory arrest). CONCLUSIONS: Neurodevelopmental outcome at 1 year of age was within the normal limits for most patients who underwent repair of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction during infancy.

Aortic Arch Syndromes↗
PubMed2006

Exercise performance in children and adolescents after the Ross procedure.

OBJECTIVES: The Ross procedure is increasingly utilized in the treatment of aortic valvar disease in children and adolescents. Our purpose was to compare pre- and post-operative exercise state in this population. METHODS: We included patients who underwent the Ross procedure at our institution between January, 1995, and December, 2003, and in whom we had performed pre- and post-operative exercise stress tests. We used a ramp bicycle protocol to measure consumption of oxygen and production of carbon dioxide. Cardiac output was estimated from effective pulmonary blood flow by the helium acetylene re-breathing technique. RESULTS: We studied 26 patients, having a median age at surgery of 15.7 years, with a range from 7.5 to 24.1 years. The primary indication for surgery in two-thirds was combined aortic stenosis and insufficiency. Median time from the operation to the post-operative exercise stress test was 17.4 months, with a range from 6.7 to 30.2 months. There was a trend toward lower maximal consumption of oxygen after the procedure, at 36.3 plus or minus 7.6 millilitres per kilogram per minute (83.9% predicted) as opposed to 38.6 plus or minus 8.4 millilitres per kilogram per minute (88.5% predicted, p equal to 0.06). Patients after the procedure, however, had significantly increased adiposity, so that there was no difference in maximal consumption of oxygen indexed to ideal body weight before and after the operation. In 20 of the patients, aerobic capacity improved or was stable after the operation. There was no post-operative chronotropic impairment. CONCLUSIONS: In the majority of patients following the Ross procedure, exercise performance is stable and within the normal range of a healthy age and sex matched population, despite sedentary lifestyles and increased adiposity.

Adolescent↗
PubMed2006

Neo-aortic valvar function after the arterial switch.

OBJECTIVES: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. METHODS: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. RESULTS: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. CONCLUSIONS: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.

Aortic Valve Insufficiency↗
PubMed2006

Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome.

OBJECTIVE: Although extracorporeal membrane oxygenation (ECMO) is an acceptable strategy for children with refractory cardiac dysfunction after cardiac surgery, its role after stage I reconstruction for hypoplastic left heart syndrome and its variants is controversial. Our objective is to describe the outcome of "nonelective" ECMO after stage I reconstruction. DESIGN: Retrospective case series. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Infants placed on ECMO after stage I reconstruction from January 1998 to May 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 382 infants who underwent stage I reconstruction during the study period, 36 (9.4%) required ECMO in the postoperative period. There were 22 infants with hypoplastic left heart syndrome. Indications for ECMO included inability to separate from cardiopulmonary bypass in 14 and cardiac arrest in 22. Fourteen infants (38.8%) survived to hospital discharge. Nonsurvivors had longer cardiopulmonary bypass time (150.1 +/- 70.0 mins vs. 103.9 +/- 30.0 mins, p =. 01). 9/14 infants (64%) supported with ECMO> than 24 hrs after stage I reconstruction survived while only 5/22 infants (22%) requiring ECMO< 24 hrs of stage I reconstruction survived (p =. 02). Of note, all five infants diagnosed with an acute shunt thrombosis were early survivors. Mean duration of ECMO was 50.1 +/- 12.5 hrs for survivors and 125.2 +/- 25.0 for nonsurvivors (p =. 01). 7/14 early survivors are alive at a median follow-up of 20 months (2-78 months). CONCLUSIONS: In our experience, ECMO after stage I reconstruction can be life saving in about a third of infants with otherwise fatal conditions. It is particularly useful in potentially reversible conditions such as acute shunt thrombosis and transient depression of ventricular function.

Extracorporeal Membrane Oxygenation↗
PubMed2006

Population pharmacokinetics of milrinone in neonates with hypoplastic left heart syndrome undergoing stage I reconstruction.

We performed a blinded, randomized pharmacokinetic study of milrinone in 16 neonates with hypoplastic left heart undergoing stage I reconstruction to determine the impact of cardiopulmonary bypass and modified ultrafiltration on drug disposition and to define the drug exposure during a continuous IV infusion of drug postoperatively. Neonates received an initial dose of either a 100 or 250 microg/kg of milrinone into the cardiopulmonary bypass circuit at the start of rewarming. Postoperatively, milrinone was infused to clinical needs. A mixed-effect modeling approach was used to characterize milrinone pharmacokinetics during cardiopulmonary bypass, modified ultrafiltration, and postoperatively using the NONMEM algorithm. All patients in this study demonstrated a modified ultrafiltration concentrating effect that occurred despite a modified ultrafiltration drug clearance of 3.3 mL x kg(-1) x min(-1). The infants in this study demonstrated an impaired renal clearance during the immediate postoperative period. A constant infusion of 0.5 microg x kg(-1) x min(-1) resulted in drug accumulation during the initial 12 h of drug administration. Postoperatively, milrinone clearance was significantly impaired (0.4 mL x kg(-1) x min(-1)), improved by the 12th postoperative hour, and approached steady-state clearance (2.6 mL x kg(-1) x min(-1)) by postoperative day 4. In the postoperative setting of markedly impaired renal function, an infusion rate of 0.2 microg x kg(-1) x min(-1) should be considered.

Cardiopulmonary Bypass↗
PubMed2005

The relationship of postoperative electrographic seizures to neurodevelopmental outcome at 1 year of age after neonatal and infant cardiac surgery.

OBJECTIVE: The occurrence of a seizure after the arterial switch operation is associated with a worse long-term neurodevelopmental outcome. The significance of seizures after neonatal and infant repair of other congenital heart defects is not known. METHODS: A recent study at our institution demonstrated seizures documented by 48-hour electroencephalographic monitoring in 20 (11%) of 178 neonates and infants after surgery for complex congenital heart defects, including hypoplastic left heart syndrome or variants. The developmental outcomes of this cohort were evaluated at 1 year of age by using the Bayley Scales of Infant Development II, which yields 2 scores: the Mental Developmental Index and the Psychomotor Developmental Index. RESULTS: Developmental evaluations were performed in 114 (70%) of 164 survivors, including 36 with hypoplastic left heart syndrome. Postoperative electroencephalographic seizures had occurred in 15 (13%) of 114 of the entire group and in 8 (22%) of 36 of those with hypoplastic left heart syndrome. For the entire cohort, the Mental Developmental Index was 92.3 +/- 13.5, and the Psychomotor Developmental Index was 79.9 +/- 18.8 for patients without seizures, compared with 90.3 +/- 10.7 and 74.4 +/- 19.3 for those with seizures (both P > .5). For the hypoplastic left heart syndrome subgroup, the Mental Developmental Index was 92.3 +/- 14.9, and the Psychomotor Developmental Index was 74.8 +/- 19.3 for patients with seizures, compared with 91.9 +/- 12.4 and 73.9 +/- 18.3 for those without seizures (both P > .5). A frontal onset of seizures was predictive of a lower score on the Psychomotor Developmental Index, but not on the Mental Developmental Index. CONCLUSIONS: The occurrence of a seizure after cardiac operation is a marker of central nervous system injury. However, in this cohort of neonates and infants with complex congenital heart defects, the occurrence of a seizure was not predictive of a worse developmental outcome at 1 year of age as assessed by the Bayley Scales of Infant Development II.

Cardiac Surgical Procedures↗
PubMed2005

Intermediate outcomes after the Fontan procedure in the current era.

OBJECTIVE: This study was undertaken to investigate the intermediate outcomes after the Fontan operation in the current era. METHODS: A cross-sectional analysis was performed by using data gathered between October 13, 2003, and February 22, 2005, on all 310 survivors of the Fontan procedure between January 1, 1992, and December 31, 1999. Medical records were reviewed, and a customized questionnaire was administered to caregivers. Outcome variables included failure of the Fontan operation (ie, death, takedown of Fontan, or transplantation), number of cardiac-related rehospitalizations, parental assessment of health, school performance, and cardiac functional status. RESULTS: A total of 332 patients underwent the Fontan procedure during the study period. A lateral tunnel was constructed in 281 (85%), and an extracardiac Fontan procedure was performed in 51 (15%). A total of 210 (63%) children had undergone previous stage I reconstruction, and 162 (49%) had received an initial diagnosis of hypoplastic left heart syndrome. There were 310 hospital survivors of the Fontan procedure (93.4%), with an additional 16 deaths (5.2%) during follow-up and 1 living heart transplant patient before the cross-sectional period. For initial hospital survivors, Kaplan-Meier estimates of freedom from death or transplantation were 98.0% (95% confidence interval, 95.6%-99.1%) at 1 year after the Fontan procedure, 94.9% (91.6%-97.0%) at 5 years, and 93.9% (90.1%-96.2%) at 8 years. Questionnaires were completed for 240 (81.9%) of the 293 available patients. By parental recollection, a total of 130 (54.2%) children required cardiac-related rehospitalization at some point during the follow-up period. At a median follow-up of 8.6 years (range, 4.1-12.8 years), 94.6% of guardians described their child's overall health as excellent or good, and 5.4%, as fair or poor. School performance was described as above average in 30.2%, average in 39.9%, and below average in 29.8%. With regard to cardiac functional status, 34.2% responded that their child had no limitations to physical activity, 52.5% reported a slight limitation, 12.1% reported a significant limitation, and 1.2% reported a severe limitation. CONCLUSIONS: Acceptable survival outcomes have been observed at intermediate follow-up of the Fontan operation in this cohort; a significant proportion of patients had hypoplastic left heart syndrome. Although cardiac-related rehospitalization is common, the parents and guardians believed that overall health, school performance, and cardiac functional status were good to excellent for most patients.

Child↗
PubMed2005

Increasing duration of deep hypothermic circulatory arrest is associated with an increased incidence of postoperative electroencephalographic seizures.

OBJECTIVE: Electroencephalographic seizures have been shown to occur in 5% to 20% of neonates and infants after biventricular repair of a variety of cardiac defects. Occurrence of a seizure is a predictor of adverse long-term neurodevelopmental sequelae. The contemporary incidence of postoperative seizures after repair of cardiac defects such as hypoplastic left heart syndrome and other forms of single ventricle is not known. METHODS: A prospective study of 178 patients less than 6 months of age undergoing cardiopulmonary bypass with or without deep hypothermic circulatory arrest (DHCA) was conducted at a single institution from September 2001 through March 2003 to identify postoperative seizures assessed by 48-hour continuous video electroencephalographic monitoring. RESULTS: Cardiac defects included transposition of the great arteries with or without a ventricular septal defect (n = 12), ventricular septal defect with or without coarctation (n = 28), tetralogy of Fallot (n = 24), hypoplastic left heart syndrome or variant (n = 60), other functional single ventricle (n = 14), and other defects suitable for biventricular repair (n = 40). Median age at the time of the operation was 7 days (range, 1-188 days) and was 30 days or less in 110 (62%) patients. DHCA was used in 117 (66%) patients, with multiple episodes in 9 patients. Median total duration of DHCA was 40 minutes (range, 1-90 minutes). Electroencephalographic seizures were identified in 20 (11.2%) patients. Seizures occurred in 15 (14%) of 110 neonates and 5 (7%) of 68 older infants. Seizures occurred in 1 (4%) of 24 patients with tetralogy of Fallot, 1 (8%) of 12 with transposition of the great arteries, and 11 (18%) of 60 with hypoplastic left heart syndrome or variant. By stepwise logistic regression analysis, once increasing duration of total DHCA (P = .001) was considered, no other variable improved prediction of occurrence of a seizure. Patients with DHCA duration of more than 40 minutes had an increased incidence of seizures (14/58 [24.1%]) compared with those with a DHCA duration of 40 minutes or less (4/59 [6.8%], P = .04). The incidence of seizures for patients with a DHCA duration of 40 minutes or less was not significantly different from those in whom DHCA was not used (2/61 [3.3%], P = .38). CONCLUSIONS: In the current era, continuous electroencephalographic monitoring demonstrates early postoperative seizures in 11.2% of a heterogeneous cohort of neonates and infants with complex congenital heart defects. Increasing duration of DHCA was identified as a predictor of seizures. However, the incidence of seizures in children with limited duration of DHCA was similar to that in infants undergoing continuous cardiopulmonary bypass alone.

Adolescent↗
PubMed2005

Outcomes after the stage I reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock Taussig shunt.

BACKGROUND: Recent reports advocate that a right ventricular to pulmonary artery (RV-PA) conduit improves outcome after the stage I reconstruction. METHODS: We retrospectively compared the outcomes of all neonates who underwent a stage I reconstruction between January 1, 2002, and October 1, 2004, with use of the RV-PA conduit and modified Blalock-Taussig shunt (mBTS) interspersed over this time period. RESULTS: In all, 149 infants underwent a stage I reconstruction (95 mBTS, 54 RV-PA) for hypoplastic left heart syndrome (HLHS) or variants. There was a preference for the RV-PA conduit in patients with aortic atresia (mBTS 30% versus RV-PA 67%, p < 0.01). There was no difference in surgical mortality (mBTS 14% versus RV-PA 17%, p = 0.67), time to extubation (mBTS 4.5 +/- 4.8 days versus RV-PA 3.9 +/- 3.5 days, p = 0.47), or length of hospital stay (mBTS 25 +/- 29 days versus RV-PA 21 +/- 23 days, p = 0.52). There was an increased incidence of shunt reinterventions in the patients with the RV-PA conduit (mBTS 17% versus RV-PA 32%, p = 0.04). Patients with RV-PA conduit returned earlier for stage II reconstruction (mBTS 6.5 +/- 2.5 months versus RV-PA 5.6 +/- 1.7 months, p = 0.05). There was no difference in overall mortality (mBTS 32% versus RV-PA 30%, p = 0.45) with a median duration of follow-up of 18 +/- 8 months. CONCLUSIONS: Comparing shunt strategies (mBTS versus RV-PA) over the same time period, we found no difference in outcome. These data support the need for a larger prospective, randomized trial.

Arteriovenous Shunt, Surgical↗
PubMed2005

Long-term and developmental outcomes of children with complex congenital heart disease.

As short-term survival of complex congenital heart disease continues to improve dramatically with advances in medical and surgical treatment, further efforts must be made to understand the long-term outcomes of our efforts. As survival continues to improve, cardiovascular morbidity and, equally importantly, neurodevelopmental and social outcomes must be a continual focus in our treatment of these complex patients. Further study of these effects is underway, and more is certainly warranted. Understanding should lead to modification of current techniques and management strategies, all with the ultimate goal of improving our patients' quality of life.

Child↗
PubMed2005

Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection.

Objective We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy. Methods All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing. Results The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1-141 days), and age at testing was 11 years (range, 6-19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17-55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 +/- 18.5) and Verbal IQ (98.6 +/- 20.2) were not different from population norms, but Performance IQ (92.3 +/- 16.9) was significantly lower than population norms ( P = .02). Fine motor skills and visual-motor coordination were significantly impaired ( P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms. Conclusions School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.

Actuarial Analysis↗
PubMed2005

Effect of congenital heart disease on neurodevelopmental outcomes within multiple-gestation births.

OBJECTIVES: We sought to assess the effect of congenital heart disease requiring surgical intervention with cardiopulmonary bypass at 6 months of age or less on developmental outcomes and growth at 1 year of age while controlling for socioeconomic status, prematurity, home environment, and parental intelligence. METHODS: We performed within-family comparison of 11 multiple-gestation births in which one child had congenital heart disease. At 1 year of age, the Bayley Scales of Infant Development II were administered, and growth parameters were assessed. Paired comparisons were made by using fixed effects regression conditioned on family. RESULTS: The multiple-gestation subjects were mildly premature on average (mean gestational age, 35.4 +/- 3.0 weeks). At 1 year of age, children with congenital heart disease scored lower on the Mental Development Index (85.0 +/- 19.3 vs 93.9 +/- 16.0, P = .037) and the Psychomotor Development Index (76.6 +/- 16.9 vs 91.3 +/- 14.9, P = .015) on the Bayley Scales of Infant Development II than did their siblings without congenital heart disease. There were no differences between siblings in weight, height, or head circumference. CONCLUSIONS: The presence of congenital heart disease requiring surgical intervention with cardiopulmonary bypass at 6 months of age or less is associated with a deficit in developmental achievement at 1 year of age, as measured by using the Bayley Scales of Infant Development II.

Female↗