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Biomedical subjects

J W Gaynor

Publications and source records attributed to J W Gaynor.

At least 19 recordsLinked to original sources
PubMed2000

Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution.

BACKGROUND: There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. METHODS AND RESULTS: Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival (P:<0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality (P:=0.004 and P:=0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. CONCLUSIONS: Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.

Body Weight↗
PubMed2000

Bilateral sequential lung transplant for ectodermal dysplasia.

A case of bilateral sequential lung transplantation for anhidrotic ectodermal dysplasia is presented. The patient was a 16-year-old male with end-stage lung disease secondary to chronic severe respiratory infection. Although a relatively rare disease, the common association of fatal pulmonary compromise in those affected with this disorder warrants consideration of lung transplantation as a viable therapeutic option.

Adolescent↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window.

The extant nomenclature for aortopulmonary window (AP window) and pulmonary artery origin from ascending aorta (hemitruncus) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Aortopulmonary Septal Defect↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: systemic venous anomalies.

The extant nomenclature for systemic venous anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. This nomenclature system classifies systemic venous anomalies into two primary groups by venous segment: (1) systemic venous anomalies, superior vena cava; and (2) systemic venous anomalies, inferior vena cava. Subsets are clearly defined and categorized. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Coronary Vessel Anomalies↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: end-stage lung disease.

The extant nomenclature for end-stage lung disease is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories, using synonyms where appropriate. Indications for lung transplantation are coded under a broad category called pulmonary failure. The proposed hierarchical scheme also allows classification of complications of lung transplantation under a category called status post lung transplant. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Databases, Factual↗
PubMed2000

Prolonged extracorporeal membrane oxygenation as a bridge to cardiac transplantation.

Cardiac transplantation provides the best option for neonates with congenital heart disease that is not amenable to surgical repair or palliation. The scarcity of suitable organs for this group has resulted in prolonged waiting times; many infants die awaiting transplantation. We present the case of a newborn with severe Ebstein's anomaly and low cardiac output who was supported with extracorporeal membrane oxygenation for 1,126 hours, until an appropriate organ became available.

Cardiac Output, Low↗
PubMed2000

Preoperative risk-of-death prediction model in heart surgery with deep hypothermic circulatory arrest in the neonate.

OBJECTIVE: Our goal was to generate a preoperative risk-of-death prediction model in selected neonates with congenital heart disease undergoing surgery with deep hypothermic circulatory arrest. METHODS: We completed a single-center, prospective, randomized, double-blind, placebo- controlled neuroprotection trial in selected neonates with congenital heart disease requiring operations for which deep hypothermic circulatory arrest was used. An extensive database was generated that included preoperative, intraoperative, and postoperative variables. Variables (delivery, maternal, and infant related) were evaluated to produce a preoperative risk-of-death prediction model by means of logistic regression. An operative risk-of-death prediction model including duration of deep hypothermic circulatory arrest was also generated. RESULTS: Between July 1992 and September 1997, 350 (74%) of 473 eligible infants were enrolled with 318 undergoing deep hypothermic circulatory arrest. The mortality was 52 of 318 (16.4%), unaffected by investigational drug. The resulting preoperative risk model contained 4 variables: (1) cardiac anatomy (two-ventricle vs single ventricle surgery, with/without arch obstruction), (2) 1-minute Apgar score (</=5 vs >5), (3) presence of genetic syndrome, and (4) age at hospital admission for surgery (</=5 or >5 days). Mortality for two-ventricle repair was 3.2% (4/130). Mortality for single ventricle palliation was 25.5% (48/188) and was significantly influenced by Apgar score, genetic diagnosis, and admission age. The preoperative model had a prediction accuracy of 80%. The operative risk model included duration of deep hypothermic circulatory arrest, which significantly (P =.03) increased risk of death, with a prediction accuracy of 82%. CONCLUSIONS: In this selected population, postoperative mortality risk is significantly affected by preoperative conditions. Identification of infants with varying mortality risks may affect family counseling, therapeutic intervention, and risk stratification for future study designs.

Cardiac Surgical Procedures↗
PubMed2000

Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency.

We present a case report of a patient with conal septal hypoplasia (supracristal) ventricular septal defect (VSD) complicated by aortic insufficiency (AI) and subpulmonic stenosis from a fibrous membrane. The development of AI with VSD is a well-recognized problem. However, the association of VSD, AI, and significant right ventricular outflow tract obstruction (RVOTO) is less common. Mechanisms of RVOTO include prolapse of an aortic valve cusp across the VSD, as well as infundibular hypertrophy or muscle bundles. Technical echocardiographic issues can make the diagnosis of VSD, AI, and RVOTO challenging. The presence of a discrete fibrous subpulmonary membrane is uncommon in this setting.

Abnormalities, Multiple↗
PubMed2000

Outcome following single-stage repair of coarctation with ventricular septal defect.

OBJECTIVE: A recent multi-institutional study suggested improved survival (97%) after staged repair of coarctation with ventricular septal defect (VSD) compared with single-stage repair. The current study was undertaken to determine outcome and need for reintervention following single-stage repair of coarctation and VSD at our institution. METHODS: Retrospective review of patients undergoing single-stage repair of coarctation with VSD between October 1, 1994 and August 15, 1999. RESULTS: Single-stage repair of coarctation with VSD was performed in 25 infants (12 males, 13 females) at a median age of 12 days (range 1-87 days) and median weight of 3.3 kg (range 1.3-4.4 kg). The VSD was conoventricular in ten patients, malalignment type with posterior deviation of the infundibular septum in ten, muscular in four and conal septal hypoplasia in one. Arch hypoplasia was present in all patients and bicuspid aortic valve in 13. At least moderate subaortic narrowing was present in six patients (secondary to prolapse of tricuspid valve tissue across the VSD in four). Repairs were performed via a median sternotomy with a mean circulatory arrest time of 38+/-12 min. Overall patient survival was 96% with one operative death and no late deaths at a median follow-up of 16 months (range 1-50 months). Reinterventions included balloon dilatation of recurrent coarctation (five), closure of residual VSD (one) and Ross-Konno procedure (two). Actuarial freedom from reintervention for the hospital survivors was 81% (95% confidence limit (CL) 61%, 92%) at 6 months, 71% (95% CL 47%, 87%) at 1 year and 59% (95% CL 31%, 82%) at 2 years following surgery. CONCLUSION: Single-stage repair of coarctation with VSD can be performed with low operative mortality and good midterm survival equivalent to reported results for staged repair.

Aortic Coarctation↗
PubMed2000

Evaluation of ventricular septal defect repair using intraoperative transesophageal echocardiography: frequency and significance of residual defects in infants and children.

Intraoperative transesophageal echocardiography (IOTEE) is commonly used to assess for residual defect and the need to return to bypass after repair of ventricular septal defect (VSD). The frequency and significance of residual septal defects as noted on IOTEE has not been well defined. We evaluated the frequency of residual VSD via IOTEE and the relationship between size of a residual VSD and rate of reoperation. In addition, we looked at the relationship between the presence of a residual VSD via IOTEE and the presence of residual VSD at follow-up transthoracic echocardiography (TTE). Residual VSD was measured via the largest width of the Doppler color jet diameter originating at the left ventricular septal surface. Of the 294 patients evaluated with IOTEE after VSD repair, one-third had a residual defect by IOTEE Doppler color flow mapping. Two-thirds of these defects closed spontaneously on TTE by the time of hospital discharge. There was no difference in frequency of residual VSD between simple (VSD closure alone, n = 90) and complex (VSD with associated lesions, n = 204) repair. Return to bypass with immediate reoperation was undertaken in nine patients, all of whom had significant shunt via oximetry (Qp/Qs > 1.5:1.0). All had residual VSD color jet diameters > 3 mm. Seven patients had residual color jet equal to 3 mm; however, hemodynamic studies did not reveal a significant shunt and none of these had reoperation. Seven patients with no VSD or < 3 mm residual VSD via had late reoperation to close residual VSD at 4 days to 5 months after initial operation. These were due to patch dehiscence or development of an "intramural" VSD in patients with conotruncal anomaly. A residual defect on IOTEE color Doppler measuring > or = 4 mm predicts the need for immediate reoperation, while a 3 mm defect may be significant and requires additional intraoperative hemodynamic evaluation. The majority of small defects noted on IOTEE are not present at discharge TTE. Patients with conotruncal defect repair should be followed closely for development of late significant "intramural" defects.

Age Factors↗
PubMed2000

Assessment of pulmonary/systemic blood flow ratio after first-stage palliation for hypoplastic left heart syndrome: development of a new index with the use of doppler echocardiography.

OBJECTIVE: Circulatory maldistribution is believed to be a major cause of early death after first-stage palliation for hypoplastic left heart syndrome. Flow reversal in the reconstructed aorta may reflect the pulmonary/systemic blood flow ratio. The purpose of our study was to investigate the utility of arterial PO (2), arterial oxygen saturation, and a newly developed Doppler-derived flow index in predicting the pulmonary/systemic flow ratio after first-stage palliation for hypoplastic left heart syndrome. METHODS: Twenty-four infants who underwent first-stage palliation for hypoplastic left heart syndrome or a variant were studied. Superior vena cava blood samples were drawn to estimate the mixed venous saturation and permit calculation of the pulmonary/systemic blood flow ratio. Fifty-four samples were evaluated within the first 24 hours after surgery. Simultaneous blood draw and Doppler echocardiography were performed with interrogation in the distal aspect of the arch reconstruction. The ratio of the Doppler velocity-time integral of retrograde flow to the velocity-time integral of forward flow was calculated and compared with the pulmonary/systemic blood flow ratio RESULTS: The median mixed venous saturation for the 54 samples was low (38.5%; range, 18%-64%). The median calculated pulmonary/systemic blood flow ratio was 1.4:1 (range, 0.3:1 to 4. 2:1). Pulse pressure, mixed venous saturation, and arterial PO (2) were not statistically significant predictors of the measured pulmonary/systemic blood flow ratio. Although both aortic oxygen saturation (R (2) = 0.84, P <.01) and Doppler flow reversal ratio (R (2) = 0.94, P <.001) were significantly associated with the measured pulmonary/systemic blood flow ratio, the model coefficient of determination was greatest for Doppler flow reversal ratio. CONCLUSION: Measures of arterial oxygen saturation and arterial PO (2) may be misleading in assessing the circulatory status of infants after first-stage palliation for hypoplastic left heart syndrome. Doppler echocardiography, through use of the Doppler flow reversal ratio, provides a more useful measure of pulmonary/systemic blood flow ratio. Low mixed venous saturation after surgery may be due to factors other than pulmonary overcirculation, such as ventricular dysfunction and low cardiac output.

Blood Flow Velocity↗
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

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 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

Usefulness of intraoperative transesophageal echocardiography in predicting the degree of mitral regurgitation secondary to atrioventricular defect in children.

The objectives of this study were to determine the validity of the grade of mitral regurgitation (MR) as imaged by intraoperative transesophageal echocardiography (TEE) in predicting the grade of MR at follow-up. Intraoperative TEE and corresponding follow-up transthoracic studies were retrospectively reviewed and the regurgitant jet area to left atrial area ratio was used to quantify the MR. Patient records were reviewed to identify factors contributing to the development of a certain grade of MR. Intraoperative TEE was useful in detecting severe MR that required further repair at the same time. However, discrepancy in the grade of MR at follow-up was noted in 47% of patients (21 of 47) and unchanged grade of MR was found only in 53% of patients (26 of 47). Blood pressures were significantly lower and heart rates higher intraoperatively. Initial preoperative grade of MR and type of atrioventricular canal defect did not predispose for a particular grade of MR at follow-up. The grade of MR by intraoperative TEE does not predict the grade of MR at follow-up as imaged by transthoracic echocardiography.

Adolescent↗
PubMed1999

Outcome after repair of tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression. METHODS: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years). RESULTS: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04). CONCLUSIONS: Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Adolescent↗
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↗