Biomedical subjects
Publications and source records attributed to M L Jacobs.
We report the case of a child with hypoplastic left heart syndrome who developed pulmonary arteriovenous (AV) malformations after superior cavopulmonary anastomoses. Resolution of the pulmonary AV malformations occurred following a completion Fontan procedure. This phenomenon has been reported previously, but only in patients with heterotaxy and polysplenia.
The extant nomenclature for tetralogy of Fallot (TOF) 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. The general categories of TOF are: classic TOF with varying degrees of pulmonary stenosis, TOF with common atrioventricular canal defect, and TOF with absent pulmonary valve. Although centers may choose to code a fourth general category, TOF with pulmonary atresia, this lesion will be grouped with pulmonary atresia-ventricular septal defect for multi-institutional analysis. 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.
The extant nomenclature for single ventricle (SV) hearts 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. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Although many issues regarding single ventricle or univentricular hearts remain unresolved among anatomists and pathologists, a classification is proposed that is relevant to surgical therapy. 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 data 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.
Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.
Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and mass. The severe end of the spectrum consists of aortic atresia and mitral atresia with a nonexistent left ventricle, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, and milder degrees of left ventricular hypoplasia, recently described as hypoplastic left heart complex (HLHC). Although the overwhelming majority of the patients can only have a univentricular repair, a small minority of patients with HLHS, particularly those that are described as having HLHC, may be candidates for biventricular repair. In this paper, the extant nomenclature for HLHS 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 Nomenclature and Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented, which 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 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.
The extant nomenclature for truncus arteriosus (TA) 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 modified Van Praagh (VP) classification is proposed involving three main categories of TA: TA with confluent or near confluent pulmonary arteries (large aorta type, VP A1, A2), TA with absence of one pulmonary artery (VP A3), and TA with interrupted aortic arch or coarctation (large pulmonary artery type, VP A4). 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.
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.
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A new technique to repair tetralogy of Fallot with an anomalous coronary artery crossing the right ventricular outflow tract is described, together with intermediate term follow-up. Using a pedicled flap of the anterior pulmonary artery wall as the floor, and a vascular or prosthetic patch as the roof, a composite conduit with the potential for growth is constructed. Together with the native outflow tract, this provides unobstructed egress from the right ventricle to the branch pulmonary arteries. Since 1990, 4 infants aged 2-weeks to 6-months have undergone primary repair using this technique. Intermediate term follow-up shows adequate durability of the repair.
We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.
Abnormal ventricular systolic torsion is present during histological rejection in adult cardiac transplant patients. Because biomechanical properties of transplanted hearts in the baseline state have not been studied in children, pediatric patients were evaluated to quantify ventricular wall motion and strain. Eight transplant studies and eight normal controls were evaluated. Magnetic resonance tagging was performed to determine radial shortening, twist, and strain in four ventricular anatomic areas at two short-axis levels. Controls had counterclockwise twist. Six transplant studies had clockwise twist, six had akinetic regions, and all had regions of no twist. One demonstrated paradoxical motion of the septum. A comparison between transplant patients and controls revealed strain to be similar in all regions except one (superior wall at the atrioventricular valve level) and strain distribution to be different only in two of eight regions. Pediatric transplant patients demonstrate regional wall motion abnormalities in the absence of rejection. Compared with normal controls, the transplanted left ventricle maintains normal strain in the presence of abnormal twist. This may be a compensatory mechanism and have clinical implications.
AIM: To investigate the late sequellae of necrotizing pancreatitis on the endocrine function of the pancreas. PATIENTS AND METHODS: Twenty patients, 15 men (mean +/- SEM age 52.2+/-2.6 years and BMI 26.8+/-0.8 kg/m2) and 5 women (age 51.0+/-7.6 years and BMI 26.7+/-0.8 kg/m2) were submitted to a glucagon stimulation test 63 (range 8-136) months after an attack of pancreatitis. All nondiabetic patients (n = 15) were also submitted to an oral glucose tolerance test. For comparison, 16 healthy volunteers, 8 men (age 56.0+/-0.9 years and BMI 26.3+/-0.4 kg/m2) and 8 women (age 50.5+/-1.0 years and BMI 28.2+/-0.6 kg/m2), were also studied. RESULTS: Five patients (25%) had diabetes mellitus and needed insulin treatment, 6 patients (30%) had an impaired glucose tolerance (IGT). Nondiabetic patients (IGT included) had a significantly higher basal insulin level (15.8+/-1.9 vs. 10.9 +/-2.2 mU/l, p < 0.05) and a lower glucose/insulin ratio (p < 0.05) compared with controls. The serum concentrations of insulin and C peptide, after stimulation with glucagon, calculated as peak value, maximal increment and as area under the curve were not significantly different in the nondiabetic patients compared to controls. The subgroup of IGT patients had a significantly higher basal C peptide (p < 0.05) and a reduced maximal increment (p < 0.05). CONCLUSIONS: After nonresectional therapy for necrotizing pancreatitis, there is a high prevalence of disturbances in glucose metabolism. Patients with IGT have signs of both loss of beta-cell function and insulin resistance.
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.
BACKGROUND: Superior cavopulmonary connection reduces the volume work of the single ventricle. METHODS: To determine the effects of superior cavopulmonary connection on preload, wall stress (or afterload), and systolic ventricular function, we studied 9 patients before and after operation, and at hospital discharge. Using echocardiography, preload was estimated by the ventricular end-diastolic area, and wall stress was calculated at end-systole and peak-systole. Ventricular function was represented by rate-corrected velocity of circumferential fiber shortening and fractional area change divided by rate-corrected ejection time. RESULTS: End-diastolic area and wall stress decreased postoperatively. Ventricular wall thickness increased with a concomitant decrease in cavity area. There was no change in mean blood pressure or heart rate or in rate-corrected velocity of circumferential fiber shortening or fractional area change divided by rate-corrected ejection time. These findings persisted at hospital discharge. CONCLUSIONS: In single ventricles, superior cavopulmonary correction results in an immediate decrease in preload and afterload. The decrease in afterload results primarily from alterations in ventricular geometry. Although no improvement in systolic function was noted, diminished work related to the reduction in loading conditions may have beneficial long-term effects on preserving myocardial performance.
BACKGROUND: This study examined the results of a Fontan operation for patients with acquired atresia of one main branch pulmonary artery. METHODS: The data for 7 patients identified as having a hypoplastic left pulmonary artery discontinuous from the right pulmonary artery were compared with those for 65 patients with continuous pulmonary arteries who consecutively underwent a completion Fontan procedure. RESULTS: No significant differences were found preoperatively with respect to right atrial pressure, aortic saturation, ventricular end-diastolic pressure, pulmonary artery pressure, pulmonary blood flow, or pulmonary vascular resistance. In the first 24 postoperative hours, there were no significant differences in heart rate, urine output, systemic venous pressure, or pulmonary venous pressure. Also, data regarding hospitalization length, effusions, and mortality were similar between the two groups. Postoperative systemic arterial saturation was lower in the one-lung group. There were no early postoperative deaths in the one-lung group, and 5 of the 7 patients are long-term survivors. CONCLUSIONS: A completion Fontan procedure can be successfully performed in patients with a hypoplastic and discontinuous left pulmonary artery, although postoperative systemic arterial saturation is not as high as in patients with continuous pulmonary arteries.
A rare case of truncus arteriosus associated with mitral valve atresia, hypoplastic left ventricle, and intact ventricular septum is reported. Successful medical management and surgical palliation of this defect is described. The possible embryology, pertinent hemodynamics, and clinical concerns with this unusual case are discussed.
OBJECTIVE: Controversy persists with regard to the treatment of patients with aortic atresia. Staged reconstructive operations and primary transplantation have been advocated as treatment strategies, but in many instances no treatment is undertaken. A multi-institutional study was undertaken for the purpose of characterizing this challenging patient group, comparing the prevalence and outcomes of the various treatment strategies, and identifying potential predictors of success or failure with each. METHODS AND RESULTS: A total of 323 neonates with aortic atresia were entered into a 21-institution prospective, nonrandomized study between January 1, 1994, and January 1, 1997. Three protocols were used, nonexclusively in many institutions: (1) staged reconstructive surgery with initial palliation by a Norwood procedure and eventual Fontan operation, (2) heart transplantation as initial definitive therapy, and (3) nonsurgical management. Analysis was based on initial protocol assignment: staged reconstructive surgery in 253 patients, heart transplantation in 49 patients, and nonsurgical management in 21 patients. For all patients initially entered into the 2 surgical treatment protocols, survival at 1, 3, 12, 24, and 36 months after entry was 67%, 59%, 52%, 51%, and 50%, respectively. A multivariable analysis found incremental risk factors for death at any time after entry to be lower birth weight (P=.04), associated noncardiac anomaly (P=.007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgery protocol (P=.03). Four institutions had higher survival statistics; 2 used a heart transplantation protocol and 2 used a staged reconstructive surgery protocol. For the 113 patients treated at these 4 institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%, 70%, 64%, 62%, and 61%, respectively. Survival among the 4 institutions was similar (P=0.1). CONCLUSIONS: Among patients with aortic atresia, other features of cardiac structure including aortic size, degree of left ventricular hypoplasia, and degree of mitral hypoplasia or atresia are not predictive of survival from 2 surgical protocols. The highest survival was achieved with either treatment strategy at institutions strongly committed to the use of one or the other surgical management protocol.
In this study we investigated the patterns of pulmonary venous flow in children with functional single ventricles to obtain a better understanding of the determinants of transpulmonary blood flow. Sixty-eight patients with functional single ventricles and aortopulmonary shunt (n = 34, group I), or superior cavopulmonary connection (n = 34, group II) underwent transesophageal Doppler echocardiographic assessment of flow in the left upper pulmonary vein before undergoing the next stage of surgery. Twelve patients from group II also underwent simultaneous evaluation of superior vena caval flow. Biphasic forward pulmonary venous flow was noted in 62 patients in sinus rhythm (S wave in systole, D wave in diastole); in 6 patients with junctional rhythm, significant early systolic reversal of flow was present. Both the S- and D-wave velocity-time integrals (VTI) were greater in group I than in group II (S(VTI) 9.9 +/- 4.2 vs 8.0 +/- 2.6, p = 0.02; D(VTI) 8.0 +/- 3.5 vs 4.2 +/- 2.6, p <0.001). In both groups, pulmonary venous flow was predominantly systolic; however, the proportion of flow during ventricular systole was significantly greater in group II than in group I (S(VTI)/D(VTI) group II: 2.4 +/- 1.5; group I 1.4 +/- 0.5, p = 0.001; percent systolic fraction of pulmonary venous flow group II = 67%, group I = 56%, p <0.001). Analysis of superior vena caval flow in group II revealed a single predominant wave with onset at early systole and peak in late systole at a mean of 150 ms after the pulmonary venous S-wave peak. Our data suggest that ventricular systole (i.e., atrial relaxation, atrioventricular valve descent) asserts great influence on transpulmonary blood flow in the functional single ventricle.