Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically.

The epidemiology of 98 children who underwent surgical intervention for pulmonary atresia and intact ventricular septum is presented. Sixty-one (62.2%) of the children died. Survival time from the date of the first operation ranged from 0 to 17.76 years. Analysis of the survivors revealed that an estimate of the probability of surviving for two years is 47% (95% confidence limits of 37%, 57%). An estimate of the median survival time is 1.43 years. In a Cox proportional hazards model, using survival time in days as the outcome variable (n = 73, using complete data), operative weight at first operation (P = 0.0019), right/left ventricular pressure ratio (P = 0.0185), and absence of ventriculo-coronary arterial connections (P = 0.0362) were identified as significant predictors of survival.

Long-term results in neonates with pulmonary atresia and intact ventricular septum.

Our entire institutional experience with pulmonary atresia and intact ventricular septum (1965 through 1987) included 115 patients, 16 of whom died before surgical intervention. Fifty-six percent of surgical patients (n = 99) had angiographic evidence of right ventricle-coronary arterial connections. The early mortality in the surgical group was 27.2%, and the actuarial survival was 24.7% +/- 6% at 13 years postoperatively. Multivariate analysis indicated that the presence of ventriculocoronary connections (p = 0.037), a decreasing ratio between right ventricular and left ventricular pressure at the initial cardiac catheterization (p = 0.007), and lower weight at operation (p = 0.001) were incremental risk factors for postoperative death; the presence of Ebstein's anomaly was an additional risk factor in the overall experience (including patients not surgically treated) (p = 0.01). Nearly all long-term survivors underwent at least one reoperation, including right ventricular outflow tract reconstruction (n = 39) and thromboexclusion of the right ventricle (n = 9). The presence of severe stenosis or interruption of the proximal left anterior descending coronary artery system was a uniformly lethal risk factor for patients undergoing these procedures (p = 0.0003). We conclude that surgical procedures that successfully decompress the right ventricle will usually result in biventricular circulation in and long-term survival of patients with pulmonary atresia with intact ventricular septum not complicated by Ebstein's anomaly or extensive ventriculocoronary connections. Decompression or thromboexclusion of the right ventricle is contraindicated in patients with ventriculocoronary connections and a right ventricle-dependent coronary circulation.

Surgical experience with left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and essentially intact ventricular septum undergoing the Mustard operation.

During a 24-year period (1963-1987), 46 infants and children with complete transposition of the great arteries (CTGA) and an essentially intact ventricular septum (IVS) have undergone surgical relief of left ventricular outflow tract obstruction (LVOTO) concurrently with the Mustard operation (MO). The obstruction was valvar in 2 patients, subvalvar in 43 and combined valvar and subvalvar in 1; the ventricular septum was intact in 42, and 4 patients had a small VSD at the time of repair. Neonatal and pre-Mustard cardiac catheterization (CC) data were analyzed to obtain LV to right ventricular peak systolic pressure ratio (LV/RVPSP) and LVOT peak systolic gradient (PSG). The mean neonatal LV/RVPSP (n = 31) was 0.79 +/- 0.04; pre-Mustard LV/RVPSP (n = 44) was 0.75 +/- 0.05; pre-Mustard LVOT PSG (n = 34) was 46.5 +/- 3.5 mmHg. Neonatal (n = 29) and pre-Mustard (n = 30) cardiac angiograms were retrospectively reviewed to analyse morphologic substrates of LVOTO. In the neonatal period, LVOTO was present in 7 patients (anatomic, n = 4; dynamic, n = 2; combined, n = 1). Immediately prior to the MO, LVOTO was present in 29 (anatomic, n = 20; dynamic n = 2; combined, n = 7). Techniques to correct LVOTO at the initial MO included pulmonary valvotomy (n = 3), ventriculomyectomy (n = 41), resection of an windsock aneurysm (n = 1), LV to pulmonary artery valved conduit (VC) (n = 1). Two patients underwent VC early postoperatively (PO) for severe residual LVOTO (both died).(ABSTRACT TRUNCATED AT 250 WORDS)

Bronchopleurobiliary fistula. A complication of intrahepatic biliary stent migration.

We describe the management of bronchopleurobiliary fistula in a 56-year-old woman who underwent a (L) mastectomy with postoperative radio- and chemotherapy for advanced breast carcinoma and required insertion of inhabiliary Silastic stents for the relief of severe obstructive jaundice. During restaging of her carcinoma for further chemotherapy, she complained of dyspnea, right chest pain and productive cough with yellow sputum. Her chest x-ray film and thoraco-abdominal CT scan demonstrated right pleural effusion with a stent protruding through the right hemidiaphragm. The objective evidence of bile in the pleural aspirate with history of bile-stained sputum established the diagnosis of bronchopleurobiliary fistula resulting from biliary stent migration.

Telemetric detection of cardiac allograft rejection. Correlation of electrophysiological, histological, and biochemical changes during unmodified rejection.

Intra-abdominal mismatched heterotopic cardiac allograft transplantation without immunosuppression was performed in eight pigs. Postoperatively, daily electrophysiological studies were carried out either with exteriorized temporary epicardial pacing wires (n = 4) or by a telemetrically controlled implanted pacemaker connected to permanent epicardial pacing leads (n = 4). Electrophysiological studies data were correlated with histopathologic and biochemical findings from daily myocardial biopsies. Electrophysiological studies revealed no significant alteration of sinus or atrioventricular node function, refractoriness, or ventricular pacing threshold. However, ventricular voltage amplitude, measured through the electrodes, decreased steadily with time in all donor hearts and was significantly correlated with histopathologic rejection grade (p less than 0.001) and with adenosine 5'-triphosphate (ATP) depletion (p less than 0.001). Ventricular voltage amplitude less than 75% of baseline occurred 4.5 +/- 1.5 days after transplantation, and this decreased voltage amplitude coincided with a moderate to severe (Grade 2 or 3) histological rejection pattern with a sensitivity of 89% (17 of 19) and a specificity of 77% (17 of 22). Similar changes in voltage amplitude were not found in control hearts. Myocardial tissue ATP values fell significantly from control values with early (Grade 1) rejection (p less than 0.05). Evidence for oxygen free radical injury was indicated by a rise in conjugated dienes of free fatty acids; this increase in diene level occurred 4.3 +/- 1.2 days postoperatively and then regressed during the terminal stages.(ABSTRACT TRUNCATED AT 250 WORDS)

Relationship between cerebrospinal fluid dynamics and reversible spinal cord ischemia during experimental thoracic aortic occlusion.

This study was designed to assess the effects of hemodynamic changes and cerebrospinal fluid dynamics on spinal cord function during experimental thoracic aortic occlusion. We investigated the effects of dopamine, sodium nitroprusside, and sodium thiopental in this model. Proximal and distal aortic pressures and cerebrospinal fluid pressure were measured during occlusion in 12 adult mongrel dogs under control conditions and during drug interventions. Spinal cord function was assessed by spinal somatosensory evoked potentials recorded during 3-minute intervals of reversible spinal cord ischemia. By multiple regression analysis, the degree of spinal cord ischemia was positively related to the cerebrospinal fluid pressure (p = 0.0092) and negatively related to the percent change in cerebrospinal fluid pressure (p = 0.028); there were no significant drug effects on cerebrospinal fluid pressure or on the degree of spinal cord ischemia. This study indicates that cerebrospinal fluid pressure is an important factor in determining the degree of spinal cord ischemia during aortic occlusion and suggests that measures to reduce cerebrospinal fluid pressure will mitigate the degree of spinal cord ischemia.

A new technique of experimental heterotopic cardiac transplantation.

Heterotopic cardiac transplantation is a useful method for studying allograft rejection. In this study a new technique of cardiac transplantation was carried out, which involved retroperitoneal anastomoses of the donor ascending aorta and main pulmonary artery with the recipient abdominal aorta and inferior vena cava respectively. The procedure was simple and effective and was accomplished with minimal operative mortality and postoperative morbidity. The method allows better access to the allograft for repeated open myocardial biopsies, obviating the limitations of transvenous fluoroscopically directed endomyocardial biopsy. This technique of retroperitoneal heterotopic cardiac transplantation has important advantages compared with similar procedures performed in the neck, abdomen, or thorax.

Loss of the somatosensory evoked response as an indicator of reversible cerebral ischemia during hypothermic, low-flow cardiopulmonary bypass.

We assessed somatosensory evoked response (SSER) as a monitor of cerebral protection during nonpulsatile, hypothermic cardiopulmonary bypass (CPB). In 13 dogs under CPB, extracorporeal flow rate (EFR) thresholds for loss of SSER were determined by stepwise reduction of the EFR from 2.0 to 0.25 L/min/m2 at perfusion temperatures of 35 degrees C, 30 degrees C, 25 degrees C, and 20 degrees C. Testing began at 35 degrees C in Group 1 (N = 6) and at 20 degrees C in Group 2 (N = 7). Immediately on loss of SSER (denoted as a decrease of 80% or more in the amplitude of the somatosensory evoked potentials), EFR was restored to 2.0 L/min/m. Thresholds for loss of SSER ranged between 0.75 and 0.25 L/min/m2. SSER was always restored on return of EFR to 2.0 L/min/m2; thus loss of SSER was a reversible ischemic change. Both groups had similar threshold values at 35 degrees C, but at lower temperatures, Group 1 thresholds were significantly higher than those in Group 2. Since 35 degrees C was the first test temperature for Group 1 but the last for Group 2, EFR reduction at 35 degrees C apparently caused neurophysiological changes (depletion of cortical energy reserves), which diminished subsequent tolerance to ischemia, but EFR reduction at 20 degrees C did not. Our findings show that loss of SSER warns of reversible cerebral ischemia, and support SSER monitoring as a useful measure of cerebral function during low-flow, hypothermic CPB.

Surgical experience with reparative techniques in patients with congenital mitral valvular anomalies.

Our review of an entire institutional experience with primary repair of congenital and acquired mitral valve (MV) anomalies in children with concordant atrial-ventricular-arterial connections but without atrioventricular septal defects included 48 patients (1962 to September 1986). Mitral stenosis (MS) was the predominate lesion in 24 patients, mitral incompetence in 22, and mixed in two. Most patients with MS had so-called typical congenital MS (Van Praagh) with abnormalities of all valvular components; virtual or complete absence of chordal development was a consistent finding. Other anatomic substrates (not mutually exclusive) producing MS included supramitral ring with Shone's complex (n = 5), parachute mitral valve (n = 2), hypoplastic mitral ring (n = 3), and rheumatic lesions (n = 3). The anatomic substrates producing mitral incompetence included annular dilatation (n = 16; isolated in seven), leaflet prolapse (n = 7), cleft leaflet (n = 6), leaflet deficiency (n = 3), and rheumatic lesions (n = 2). Concurrent repair of associated lesions was performed in 29 (60%) patients for relief of left ventricular outflow tract obstruction (n = 16) and closure of ventricular septal defects (n = 9; double-outlet right ventricle in three). Six (12.5%) patients had previous repair of coarctation of the aorta. The operative mortality rate was 18.7% (70% confidence limits [CL]; 12.8% to 24.6%), with one death in 35 patients since 1975 (2.9%; 70% CL; 0% to 5.8%). The actuarial freedom from early or late death and reoperation (+/- SEM) was 44.0% +/- 14.8% at 8 years postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Identification of risk factors for spinal cord ischemia by the use of monitoring of somatosensory evoked potentials during coarctation repair.

The infrequency of spinal cord infarction and paraplegia after occlusion of the descending thoracic aorta has effectively precluded statistical identification of risk factors. Reversible spinal cord ischemia (SCI), however, is more common, can be detected by intraoperative neurophysiologic monitoring, and can lead to irreversible spinal cord damage. Spinal somatosensory evoked potentials (SEPs) were monitored intraoperatively in 38 patients (18 days to 18 years) undergoing coarctation repair (1982-1986). Although no patients sustained perioperative neurologic dysfunction, 10 of 38 (26%) patients developed reversible SCI, as reflected by greater than 75% loss of SEP N1-P1 interpeak amplitude during aortic occlusion (mean clamp time, 29.1 +/- 1.1 min). During occlusion, seven of 38 (18%) sustained complete loss of the SEP; uniform and prompt (1 to 6 min after clamp release) recovery of the signal occurred in these patients with reperfusion following completion of the repair (n = 6), or temporary institution of partial occlusion (n = 1). By multiple regression analysis the degree of SCI was negatively related to the distal aortic pressure (mean 32.4 +/- 2.4 mm Hg, p = .03), and the occlusion PCO2 (mean 33.1 +/- 1.1 mm Hg; p = .013), and positively related to the change in proximal systolic pressure with aortic occlusion (mean 19.8 +/- 3 mm Hg, p = .003). We conclude that: (1) distal hypotension and SCI commonly occur during aortic occlusion for coarctation repair, and (2) intraoperative interventions that can potentially influence distal aortic perfusion and/or PCO2 should be used judiciously.

Combined plasmacytoma and squamous cell carcinoma.

A 63-year-old man was admitted to the Cardiothoracic Department of Glasgow Royal Infirmary for surgical treatment of a left suprahilar opacity. A left pneumonectomy was required to deal with an extensive upper lobe tumor. Histopathological examination, in addition to a moderately well-differentiated squamous cell carcinoma, revealed the presence of a band-like infiltrate of plasma cells and lymphoplasmacytoid cells at the main bronchial resection margin. Immunohistochemical studies confirmed this to be an extramedullary plasmacytoma by showing IgA and kappa positive monotypic cells. No paraprotein was detected in the serum. The value of immunohistochemistry in the differential diagnosis has been emphasized.

Surgical closure of persistent ductus arteriosus (PDA) in infants before 30 weeks gestation.

Between October 1981 and December 1983 21 premature infants of mean gestational age 27.5 weeks (range 26-29 weeks) underwent surgical closure of persistent ductus arteriosus. Mean birth weight was 1080 g. There was no operative mortality. One death in an infant with pseudomonas septicaemia occurred two days after surgery. Twenty infants had features of idiopathic respiratory distress syndrome (IRDS) and required assisted ventilation prior to operation. Six infants had associated bronchopulmonary dysplasia (BPD) and 11 had signs of congestive cardiac failure. All infants presented with clinical features suggesting the diagnosis of PDA and in 18 the left atrial/aortic ratio was increased (mean 1.9:1). In 18 infants a trial of Indomethacin therapy had failed. This experience supports the view that surgical closure of PDA in infants born before 30 weeks gestation can be accomplished safely. We believe that surgical treatment of PDA represents the optimal therapy in this high risk group of infants.

Permanent cardiac pacemaker in infants and children.

Between October 1970 and November 1984, 26 infants and children aged 11 days to 18 years (mean 5.7 years) received 42 permanent cardiac pacemakers (26 primary implants, 16 re-implants) for congenital or surgically acquired heart block, bradycardia and sinus node dysfunction. Twenty-two patients had unipolar pacing and 4 bipolar pacing. Of 26 primary implantations, 2 had fixed rate epicardial pacing, 16 ventricular demand pacing (13 epicardial, 3 endocardial), 3 epicardial VAT (P-synchronous) pacing and 5 DDD (universal) pacing (4 epicardial, one endocardial). Fourteen patients required a further 19 operations for change of generators (16), ventricular lead (1), generator site (1) and generator encasing (1). Thirty-day hospital mortality was 11.5% (3/26), of which one death was possibly related to pacing failure. Four patients died during the follow-up period (3 months to 10 years; mean 3.4 years). Sixteen of the 19 survivors achieved complete symptomatic relief, without any medical therapy. Our results indicate that modern cardiac pacemaker systems are safe and reliable, and are associated with major relief of symptoms in this age group.