Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study.

BACKGROUND: Infants with hypoplastic left heart syndrome (HLHS) commonly undergo cardiac transplantation as primary management. METHODS: We examined outcomes of primary transplantation for unpalliated HLHS. We analyzed data from the 20 institutions of the Pediatric Heart Transplant Study Group, from January 1, 1993, through December 31, 1998, using actuarial and parametric survival analysis and competing outcomes analysis. RESULTS: During the 6 years studied, 1,234 patients were listed for cardiac transplantation; 262 patients (21.2%) had unpalliated HLHS. The number (and percentage) of patients with HLHS decreased from 58 (27% of patients listed) in 1993 to 30 (14%) in 1998. Overall, 25% of infants with HLHS died while waiting; primary cause of death was cardiac failure (50%). Of the remaining patients awaiting transplantation, 23 (9%) underwent Norwood/Fontan-type surgeries as interim palliation: 52% died. Ultimately, 175 patients underwent cardiac transplantation (67%); 50% received organs by 2 months after listing. Post-transplant actuarial survival was 72% at 5 years, with 76% of deaths (35/46) occurring within 3 months; early mortality was caused primarily by graft failure within the first 30 days after transplantation (in 54%). Among 1-month survivors, survival at 1 and at 5 years was 92% and 85%, respectively. Of the 262 patients listed with unpalliated HLHS, overall survival, taking into account mortality after listing and after transplantation, was 68% at 3 months and 54% at 5 years. CONCLUSIONS: Cardiac transplantation offers good intermediate survival for infants with unpalliated HLHS.

Immediate post-operative balloon dilatation of neonatal long segment stenosis of the descending thoracic aorta.

During surgical repair of a neonatal coarctation it was recognized that this was a rare, previously undescribed form of stenosis of the entire descending thoracic aorta. A few hours after the end-to-end surgical repair, the child underwent successful balloon angioplasty, involving the entire descending thoracic aorta, and in which we intentionally avoided the surgical site.

Modified Norwood procedure with a high-flow cardiopulmonary bypass strategy results in low mortality without late arch obstruction.

OBJECTIVE: The results of our modification of the stage I Norwood procedure, in which we use only autologous tissue to reconstruct the aortic arch, were reviewed. A high-flow, low-pressure cardiopulmonary bypass protocol (with phenoxybenzamine), before and after a period of deep hypothermic circulatory arrest, was used. METHODS: Between 1993 and 1999, 59 patients, aged 1 to 353 days (median 4 days) and weighing 1.7 to 6.8 kg (median 3.2 kg), underwent a modified Norwood procedure. The ascending aortic diameter ranged from 1.5 to 8 mm (median 3 mm). The modified Blalock-Taussig shunt was 3 mm in 21 patients (36%) and 3.5 mm or larger in 38 patients (64%). RESULTS: Deep hypothermic circulatory arrest and cardiopulmonary bypass times ranged from 15 to 64 minutes (median 37 minutes) and 44 to 144 minutes (median 88 minutes), respectively. Early postoperative survival was 83%. By univariate analysis, early mortality was associated with an ascending aortic diameter of 2.5 mm or less (P =.01). Weight, circulatory arrest and bypass times, diagnosis (hypoplastic left heart syndrome vs variant), shunt size, and date of the procedure did not affect survival. For a median follow-up period of 37 months (range 4-63 months), 42 (61%) patients underwent bidirectional cavopulmonary shunts, 10 (17%) had Fontan operations, and 1 patient underwent transplantation after a bidirectional cavopulmonary shunt. Eight patients subsequently died, for a 1-year actuarial survival of 72% (95% confidence interval: 60%-84%). Neoaortic arch obstruction was corrected in 3 patients (5%). CONCLUSIONS: At intermediate-term follow-up, our modification of the Norwood procedure together with our perioperative strategies has resulted in acceptable outcomes with a low incidence of neoaortic arch obstruction. Patients with a small ascending aortic diameter have emerged as a high-risk group, but a recent technical modification may improve the outlook for these patients.

Do peritoneal catheters remove pro-inflammatory cytokines after cardiopulmonary bypass in neonates?

BACKGROUND: Cardiopulmonary bypass (CPB) in neonates induces a cytokine-mediated capillary leak syndrome that can cause organ dysfunction. Removing harmful cytokines after CPB may attenuate this response. This study measured the concentrations of serum and peritoneal fluid (PF) cytokines after CPB to determine if harmful cytokines can be removed with peritoneal catheters. METHODS: Neonates (n = 18) had cardiac surgery using CPB with circulatory arrest. Peritoneal catheters were placed at the end of surgery to drain excess fluid. Serum samples were obtained before and after CPB, and PF after CPB. Cytokines were measured by enzyme-linked immunosorbent assay. RESULTS: Tumor necrosis factor-alpha and interleukin-1beta (IL-1beta) were not detected in any serum or PF sample. Serum concentrations of IL-6, IL-8, and IL-10 increased significantly after CPB. PF concentrations of IL-6 and IL-8 exceeded serum concentrations, whereas IL-10 concentrations were higher in the serum. There was a significant negative correlation between serum and PF concentrations of IL-6 after CPB (r = -0.63; p<0.05). CONCLUSIONS: PF has very high concentrations of the proinflammatory cytokines, IL-6 and IL-8, after CPB but not the antiinflammatory cytokine IL-10. The PF may be a depot for the harmful inflammatory cytokines after CPB, and removing the PF could lower serum concentrations.

Results of the double switch operation in the current era.

BACKGROUND: In patients with atrioventricular and arterioventricular discordance congenitally corrected transposition, the morphologically right ventricle may progressively deteriorate while functioning in the systemic circuit. The double switch operation has been proposed to limit this functional deterioration. METHODS: From October 1993 to August 1998, the records of 27 patients with congenitally corrected transposition were reviewed. Age at operation ranged from 3 months to 55 years. Associated defects included ventricular septal defects in 18, pulmonary atresia in 7, and pulmonary stenosis in 11 patients. Twenty-two patients had double switch operations (10 arterial switch plus Senning procedures and 12 Rastelli plus Senning procedures). Five patients were not candidates for the double switch. Before the double switch, 6 patients required pulmonary artery banding and 10 had functioning systemic to pulmonary artery or cavopulmonary shunts. RESULTS: There was no early or late mortality. Two patients required pacemaker implantation, both later regained normal sinus rhythm. Tricuspid valve function improved in all patients except one. Moderate left ventricular dysfunction developed 5 months postoperatively in 1 patient. CONCLUSIONS: The double switch operation can be performed in selected patients with minimal early morbidity and mortality. Longer follow-up is necessary to determine whether this complex approach is indeed warranted.

Neuroprotective, anesthetic, and cardiovascular effects of the NMDA antagonist, CNS 5161A, in isoflurane-anesthetized lambs.

BACKGROUND: N-methyl-d-aspartate (NMDA) receptor antagonists are neuroprotective in animal models of cerebral ischemia, but adverse cardiovascular and neurobehavioral effects have precluded their clinical use. The authors present the neuroprotective, anesthetic, and cardiovascular effects of a novel NMDA antagonist, CNS 5161A. METHODS: Lambs, 4.0-6.5 kg, were anesthetized with isoflurane, intubated, and ventilated and had thermodilution catheters placed in the pulmonary artery and 20-g catheters placed in the femoral artery. The minimum alveolar concentration (MAC) of isoflurane was determined using the "bracketing technique." CNS 5161A was given as a bolus and then as an infusion at three doses. Cardiovascular measurements were determined every 15 min. Other lambs (n = 25) were subjected to cardiopulmonary bypass (CPB) with hypothermic circulatory arrest (HCA) for 120 min. Eighteen received CNS 5161A, and seven received saline vehicle. One hour after CPB, brains were perfusion-fixed and removed for in situ hybridization and immunohistochemistry analysis in half of the animals. The other half survived 48 h before their brains were examined for neuronal degeneration. RESULTS: Isoflurane at MAC significantly decreased blood pressure, heart rate, cardiac output, and systemic vascular resistance by 30-48% (n = 16; P < 0.05). CNS 5161A (n = 12) had no significant cardiovascular effects. All concentrations of CNS 5161A caused a significant reduction (21-29%) of the MAC of isoflurane (n = 12; P < 0.05). CNS 5161A, at serum concentrations greater than 25 ng/ml, completely inhibited c-fosmRNA and c-FOS protein expression in hippocampal neurons after 120 min of HCA, attenuated neuronal degeneration, and improved functional outcome by 47% (P < 0.05). CONCLUSIONS: CNS 5161A at neuroprotective concentrations before CPB-HCA significantly reduces the MAC of isoflurane without cardiovascular effects.

Banding of a patent arterial duct to palliate complete transposition in a compromised neonate.

In a hemodynamically compromised neonate with complete transposition and intact ventricular septum (concordant atrioventricular and discordant ventriculo-arterial connections), it became necessary to control severe congestive heart failure. The arterial switch operation had been delayed because of intracranial bleeding and subsequent coagulopathy. The cardiac failure was reversed by surgical banding of the patent arterial duct. The arterial switch was performed successfully one week later.

Aortic valve repair after arterial switch operation.

A patient with transposition of the great arteries and a ventricular septal defect underwent an arterial switch operation 15 months after pulmonary artery banding. At 12 years of age, severe neoaortic valve regurgitation, due to dilated aortic sinuses and poor leaflet coaptation, developed. Aortic valve repair involved placement of subcommissural sutures, elliptical excision and tailored reduction of two anterior aortic sinuses, with triangular patch expansion of the proximal ascending aorta. A good result was obtained.

Transcatheter coil occlusion of perivalvular mitral leaks associated with severe hemolysis.

A 6-year-old malnourished child had persisting hemolysis after attempted valve repair and two surgeries for mitral valve replacement due to partial dehiscence of the valve ring. A modified catheter delivery system was utilized to deploy a total of six Gianturco coils. The hemolysis resolved and the patient was doing well 17 months later. The technique may be helpful in other patients with perivalvular mitral leaks. Cathet. Cardiovasc. Intervent. 49:64-67, 2000.

Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair.

BACKGROUND: Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. METHODS: Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. RESULTS: There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. CONCLUSIONS: Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.

Double-outlet right ventricle with complete atrioventricular canal.

A case of double-outlet right ventricle, unbalanced complete atrioventricular canal, hypoplastic aortic arch, coarctation of the aorta, and atrioventricular valve regurgitation was treated with a staged approach. At 4 days of life the patient underwent aortic arch repair, atrial septectomy, common atrioventricular valve regurgitation repair, and pulmonary artery banding. When she was 14 months of age a biventricular repair was accomplished by two-patch complete atrioventricular canal repair and arterial switch procedure.

Ventriculo-arterial discordance: switching the morphologically left ventricle into the systemic circulation after 3 months of age.

OBJECTIVE: To retrospectively examine a 4 year policy of restoring the morphologically left ventricle to the systemic circuit in patients presenting after 3 months of age with ventriculo-arterial discordance with or without associated atrio-ventricular discordance. This policy was stimulated by the known tendency of the morphologically right ventricle to develop dysfunction sooner or later when left in the systemic circuit. Such a policy dictates a more complex surgical approach and, at this point, it remains controversial whether or not the increased surgical complexity is warranted. METHODS: From July 1, 1993 to March 31, 1997, a total of 29 patients were entered into a protocol for placement of the morphologically left ventricle into the systemic circuit. Three groups of patients were identified. Group I; congenitally corrected transposition in 14 patients -- were treated with either a Senning plus arterial switch operation or Senning plus Rastelli procedure. Group II; failed atrial switch procedure in 12 patients of which nine proceeded to arterial switch operation with Senning or Mustard takedown and atrial reseptation. Group III; D-transposition of the great vessels presenting more than 1 year after birth in three patients who underwent arterial switch operation alone. A deconditioned morphologically left ventricle required reconditioning by means of preparatory pulmonary artery banding in 17 of 29 patients. In the patients requiring pulmonary artery banding, an average of 2.1 pulmonary artery bandings was required to prepare the morphologically left ventricle for a systemic pressure workload. RESULTS: In those patients with a deconditioned morphologically left ventricle requiring preparatory pulmonary artery banding, the mean ratio between the left ventricular and right ventricular systolic pressure increased from 0.48 to 0.95. The left ventricular mass increased from 46.6 to 81.8 g/m2 in five patients subjected to serial MRI measurement. Three patients failed the preparatory pulmonary artery banding and did not proceed to anatomical correction. Two subsequently died at a later time. In the patients proceeding to complete anatomical correction: group I -- there were no early or late deaths. Two patients required pacemaker implantation post-operatively. Group II -- there were two in-hospital deaths, one early due to intrapulmonary hemorrhage and one late, secondary to postoperative left ventricular failure with a stormy post-operative course requiring successful ECMO placement and weaning. These patients were 18 and 25 years old, respectively. One patient proceeded to cardiac transplantation 3 months after surgery due to ongoing morphologically left and right ventricular dysfunction. Group III -- all patients continue to do well. CONCLUSIONS: Late anatomic correction of ventriculo-arterial discordance with or without atrio-ventricular discordance can be performed at a relatively low risk. Reconditioning of the morphologically left ventricle can be achieved by sequential pulmonary banding but is not without risk. Failure to achieve adequate reconditioning of the morphologically left ventricle by pulmonary artery banding in the older patient probably increases the risk of non-survival and may be offset by timely transplantation. Longer follow-up and an assessment of the functional status of these patients is required to assess whether or not this complex surgical approach is indeed warranted.

Branch retinal artery occlusion from a retained left atrial catheter 21 years after operation.

A case of branch retinal artery occlusion due to an embolus from a retained left atrial catheter is presented. Removal was accomplished by reoperation. Prompt removal of any retained intracardiac catheter is recommended.

Immediate-early gene expression in ovine brain after hypothermic circulatory arrest: effects of aptiganel.

BACKGROUND: Altered gene expression occurs in the brain after global ischemia. We have developed a model to examine the effects of cardiopulmonary bypass and hypothermic circulatory arrest (HCA) on the induction of the immediate-early gene c-fos in the brains of neonatal lambs. We then tested the effects of the noncompetitive N-methyl-D-aspartate antagonist, aptiganel hydrochloride (Cerestat), on c-fos expression and neuronal injury. METHODS: Neonatal lambs (weight, 4 to 6 kg) anesthetized with isoflurane were supported by cardiopulmonary bypass, subjected to 90 or 120 minutes of HCA at 15 degrees C, and rewarmed on bypass to 38 degrees C. One hour after cardiopulmonary bypass was terminated, the brains were perfusion fixed and removed for in situ hybridization and immunohistochemical analysis. Some animals survived 3 days before their brains were removed to examine for neuronal necrosis. One group of lambs (n = 20) received aptiganel (2.5 mg/kg). A second group (n = 25) received saline vehicle only. RESULTS: Increasing duration of HCA induced a corresponding increase in c-fos messenger RNA expression throughout the hippocampal formation and cortex. However, Fos protein synthesis peaked after 90 minutes of HCA and decreased significantly (p < 0.01) after 120 minutes of HCA. Aptiganel administration caused a significant decrease in (p < 0.001) c-fos messenger RNA expression and Fos protein synthesis after 90 minutes of HCA and preserved Fos protein synthesis after 120 minutes of HCA. Neuronal necrosis was observed in the brains of vehicle-treated lambs after 120 minutes of HCA but was significantly decreased (p < 0.05) in the lambs given aptiganel. CONCLUSIONS: These experiments indicate that the transcriptional processes of immediate-early genes remain intact, whereas translational processes are impaired after prolonged HCA. The inability to synthesize Fos proteins after 120 minutes of HCA was associated with neuronal degeneration. Aptiganel preserved translational processes and caused a significant improvement in the neurologic outcome.

Anomalous origin of left coronary from right pulmonary artery in hypoplastic left heart syndrome.

An infant with hypoplastic left heart syndrome presented for surgical repair at 9 months of age, the ductus having remained open in the presence of a restrictive atrial septal defect. In addition, an anomalous left coronary artery originating from the right pulmonary artery was found. After preliminary blade/balloon atrial septostomy, a successful modified Norwood procedure with concomitant reimplantation of the anomalous coronary artery was performed.

'Kissing sequestra'--a forgotten manifestation of osteo-articular tuberculosis. A case report.

Osteo-articular tuberculosis may be difficult to diagnose radiologically. A male patient with pain in the left hip presented with radiological features of the disease--narrowing of the joint space, peri-articular osteopenia, subchondral erosions and cavity formation. In addition 'kissing sequestra' were present in the adjacent femoral head and acetabular joint surfaces. This radiological feature, described as common by Phemeister and Pomerantz in 1933, has been largely overlooked in recent literature. The presence of 'kissing sequestra' is a valuable diagnostic aid and should not be overlooked.

Delayed post-traumatic cauda equina compression syndrome.

Acute lumbosacral fracture-dislocation is an unusual injury. A delayed cauda equina injury accompanying the fracture-dislocation is seldom encountered. We report a cauda equina lesion complicating an acute lumbosacral fracture-dislocation 3 weeks postinjury. The patient had been trapped in a knee-elbow position under an overturned vehicle. Symptoms were relieved following posterior decompression and fusion from L5 to S1.

Acute osteomyelitis complicating a simple fracture. A case report.

Osteomyelitis complicating a simple fracture is unusual, but the reason for its rarity is unknown. We report on a 9-year-old Black boy who developed acute osteomyelitis after sustaining an acute simple fracture of the femur. The causative role of trauma in acute osteomyelitis is discussed and 'the relative resistance of healthy bone' questioned.