Biomedical subjects

D Zurakowski

Publications and source records attributed to D Zurakowski.

At least 55 records · Page 3Linked to original sources

PubMed2001

Children with macrocrania: clinical and imaging predictors of disorders requiring surgery.

BACKGROUND AND PURPOSE: Macrocrania is a common pediatric clinical condition affecting up to 5% of the population. The purpose of this study was to determine clinical and imaging predictors that are useful in the differentiation of disorders requiring surgical treatment from those that can be treated medically in children with macrocrania. METHODS: In a 3-year 7-month retrospective study, 88 patients (median age, 8 months; interquartile range, 5--13 months) with macrocrania and no known underlying neurologic disorder underwent imaging of the brain (sonography, n = 36; CT, n = 31; MR imaging = 21). The study was conducted in a pediatric tertiary care referral center. Clinical and imaging data were correlated to final diagnosis by means of logistic regression and receiver operating characteristic curves. RESULTS: Sixteen (18%) of the patients had disorders requiring surgery: communicating hydrocephalus, n = 7; noncommunicating hydrocephalus, n = 3; hemorrhagic subdural collections, n = 3; neoplasm, n = 1; encysted cavum septi pellucidi, n = 1; and vein of Galen malformation, n = 1. Clinical predictors of disorders requiring surgery included vomiting (P =.007), labor instrumentation (P =.026), developmental delay (P =.008), and abnormal neurologic findings (P =.028). Imaging predictors of disorders requiring surgery included a focal space-occupying lesion (P <.0001) and moderate-to-severe ventriculomegaly (P <.0001). The diagnostic sensitivity of the combination of independent clinical and imaging predictors was higher than that of independent clinical predictors alone, being 100% (95% confidence interval = 96.9%, 100%) and 93.8% (95% confidence interval = 88.7%, 98.8%), respectively. A trend indicated that the area under the receiver operating characteristic curve for clinical plus imaging findings (0.95) was greater than that for clinical findings alone (0.85) (P =.09). An increase in the number of clinical and imaging predictors was highly correlated with an increased risk of a disorder requiring surgery (P <.0001). CONCLUSION: Baseline neuroimaging is indicated for children with macrocrania because the combination of clinical and imaging predictors has the best diagnostic performance in determining the need for surgical versus nonsurgical management.

Brain Diseases↗

PubMed2001

Natural history of vesicoureteral reflux in girls after age 5 years.

PURPOSE: Expectations concerning the likelihood that vesicoureteral reflux will resolve during a given interval are predominantly based on experience with children younger than 5 years. We assess the natural course of vesicoureteral reflux in girls older than 5 years. MATERIALS AND METHODS: We reviewed the diagnostic and followup cystograms, medical records and renal imaging studies of 200 girls with vesicoureteral reflux, of whom 97 were diagnosed before age 60 months and 103 were diagnosed at or after age 60 months. Vesicoureteral reflux was considered to have resolved when a followup radionuclide cystogram demonstrated no reflux. RESULTS: Vesicoureteral reflux demonstrated at or after age 60 months by a radionuclide or radiographic examination (index study) resolved in 43% of cases during a mean followup interval of 41 months. The yearly percent chance of resolution approached or exceeded 20% through age 11 years. For girls with moderate vesicoureteral reflux on the index study unilateral moderate vesicoureteral reflux was associated with a higher overall percent chance of resolution and a shorter time from index study to resolution. Evidence of new or progressive parenchymal injury was not indicated in any of 92 girls who underwent serial renal ultrasonograms. CONCLUSIONS: Vesicoureteral reflux resolution continues after age 5 years at a rate similar to that in younger children. Continued medical management in the anticipation of spontaneous resolution is safe and appropriate for most patients.

Child, Preschool↗

PubMed2000

Increased nuclear factor-kappaB and angiotensinogen gene expression in posttransplant recurrent focal segmental glomerulosclerosis.

In an attempt to identify potential markers of steroid-resistance in focal segmental glomerulosclerosis (FSGS) we evaluated intra-graft gene expression of IkappaBalpha, nuclear factor-kappaB (NF-kappaB), and angiotensinogen in 60 biopsies from 27 pediatric renal transplant recipients. Intra-graft NF-kappaB expression was significantly elevated in recurrent FSGS (R-FSGS) (218.3 + 55.6 ag/fg versus NON-FSGS 121.1 + 19.9, P=0.04) but not in acute rejection. NF-kappaB:IkappaBalpha ratios were higher in cadaveric donor versus living related donor recipients (15.7 + 2.8 vs. 8.8 + 1.3, respectively, P=0.015), and in African-American versus Caucasian recipients (15.6 + 2.9 vs. 9.1 + 1.3, respectively, P=0.03). Intra-graft angiotensinogen gene expression was significantly elevated in R-FSGS (30.5 + 8.8 ag/fg R-FSGS vs. 16.0 + 4.7 NON-FSGS, P=0.009). We conclude that increased NF-kappaB and angiotensinogen gene expression are associated with R-FSGS. Increased NF-kappaB:IkappaBalpha ratios are associated with cadaveric donor recipients and African-American race.

Adolescent↗

PubMed2000

Transplanted neuroblasts differentiate appropriately into projection neurons with correct neurotransmitter and receptor phenotype in neocortex undergoing targeted projection neuron degeneration.

Reconstruction of complex neocortical and other CNS circuitry may be possible via transplantation of appropriate neural precursors, guided by cellular and molecular controls. Although cellular repopulation and complex circuitry repair may make possible new avenues of treatment for degenerative, developmental, or acquired CNS diseases, functional integration may depend critically on specificity of neuronal synaptic integration and appropriate neurotransmitter/receptor phenotype. The current study investigated neurotransmitter and receptor phenotypes of newly incorporated neurons after transplantation in regions of targeted neuronal degeneration of cortical callosal projection neurons (CPNs). Donor neuroblasts were compared to the population of normal endogenous CPNs in their expression of appropriate neurotransmitters (glutamate, aspartate, and GABA) and receptors (kainate-R, AMPA-R, NMDA-R. and GABA-R), and the time course over which this phenotype developed after transplantation. Transplanted immature neuroblasts from embryonic day 17 (E17) primary somatosensory (S1) cortex migrated to cortical layers undergoing degeneration, differentiated to a mature CPN phenotype, and received synaptic input from other neurons. In addition, 23.1 +/- 13.6% of the donor-derived neurons extended appropriate long-distance callosal projections to the contralateral S1 cortex. The percentage of donor-derived neurons expressing appropriate neurotransmitters and receptors showed a steady increase with time, reaching numbers equivalent to adult endogenous CPNs by 4-16 weeks after transplantation. These results suggest that previously demonstrated changes in gene expression induced by synchronous apoptotic degeneration of adult CPNs create a cellular and molecular environment that is both permissive and instructive for the specific and appropriate maturation of transplanted neuroblasts. These experiments demonstrate, for the first time, that newly repopulating neurons can undergo directed differentiation with high fidelity of their neurotransmitter and receptor phenotype, toward reconstruction of complex CNS circuitry.

Animals↗

PubMed2000

Caspase inhibitors block zinc-chelator induced death of retinal ganglion cells.

Zinc-chelating agents, including ethambutol and its metabolite 2,2'(ethylenediamino)-dibutyric acid (EDBA) are toxic to retinal ganglion cells through a glutamate dependent mechanism. We explored whether such cell death was mediated through the caspase family of cysteine proteases. Retinal cultures were treated with EDBA alone, or EDBA plus a variety of known caspase inhibitors, and ganglion cell viability was assayed. EDBA killed 20-30% of ganglion cells. A general caspase inhibitor, BAF, prevented EDBA induced ganglion cell death. Specific inhibitors of caspase-3 and caspase-6 showed a similar ability to BAF in preventing EDBA mediated ganglion cell loss, whereas inhibitors of caspase-8 and caspase-9 were not able to rescue EDBA treated ganglion cells. A caspase-1,4 inhibitor was less effective than BAF. These studies show that a caspase mediated mechanism of apoptosis accents for a portion of EDBA mediated retinal ganglion cell death. This toxicity was mediated by downstream effector caspases, 3 and 6. Caspase inhibitors may prevent ganglion cell death secondary to ethambutol treatment.

Animals↗

PubMed2000

Blood glutathione synthesis rates in healthy adults receiving a sulfur amino acid-free diet.

The availability of cysteine is thought to be the rate limiting factor for synthesis of the tripeptide glutathione (GSH), based on studies in rodents. GSH status is compromised in various disease states and by certain medications leading to increased morbidity and poor survival. To determine the possible importance of dietary cyst(e)ine availability for whole blood glutathione synthesis in humans, we developed a convenient mass spectrometric method for measurement of the isotopic enrichment of intact GSH and then applied it in a controlled metabolic study. Seven healthy male subjects received during two separate 10-day periods an L-amino acid based diet supplying an adequate amino acid intake or a sulfur amino acid (SAA) (methionine and cysteine) free mixture (SAA-free). On day 10, L-[1-(13)C]cysteine was given as a primed, constant i.v. infusion (3 micromol x kg(-1) x h(-1)) for 6 h, and incorporation of label into whole blood GSH determined by GC/MS selected ion monitoring. The fractional synthesis rate (mean +/- SD; day(-1)) of whole blood GSH was 0.65 +/- 0.13 for the adequate diet and 0.49 +/- 0.13 for the SAA-free diet (P < 0.01). Whole blood GSH was 1,142 +/- 243 and 1,216 +/- 162 microM for the adequate and SAA-free periods (P > 0.05), and the absolute rate of GSH synthesis was 747 +/- 216 and 579 +/- 135 micromol x liter(-1) x day(-1), respectively (P < 0.05). Thus, a restricted dietary supply of SAA slows the rate of whole blood GSH synthesis and diminishes turnover, with maintenance of the GSH concentration in healthy subjects.

Adult↗

PubMed2000

The MHC influences NK and NKT cell functions associated with immune abnormalities and lifespan.

The lifespans of H-2 congenic mice differ significantly. The B10.AKM (H-2m) strain has a median survival time (MST) of 15 months, whereas the B10.BR (H-2k) strain has an MST of 24 months. It was previously shown that B10.AKM mice at 13-15 months of age have immunological function comparable to those of B10.BR mice at 22-26 months of age. These functions include: a low proliferative response, reduced levels of intracellular calcium release [Ca2+]i, and an increase in the frequency of memory helper T-cells (CD4+ CD44hiCD45RBlo). In this report similar deficiencies were demonstrated in B10.AKM mice at 2-4 months of age and show that activated spleen NK1.1+CD4+ T (NKT) cells from young B10.AKM mice produce a significantly higher level of IL-4 but a lower level of IFN-gamma as compared to NKT cells from B10.BR mice of the same age. Also, the cytotoxic activity of natural killer (NK) cells from spleens of young (2-4 months) as well as adult (12-16 months) B10.AKM mice is significantly lower (P < 0.01) than that of NK cells from B10.BR mice. These findings suggest that the NKT activity in young B10.AKM mice is a factor for the early onset of immune dysfunction leading to a shorter lifespan.

Aging↗

PubMed2000

The use of oral pentobarbital sodium (Nembutal) versus oral chloral hydrate in infants undergoing CT and MR imaging--a pilot study.

BACKGROUND: Chloral hydrate, a commonly used oral sedative for infants undergoing imaging examinations, has a bitter taste and requires relatively large volume, provoking unpleasant reactions from the infants. Experience with an alternative sedative, oral pentobarbital (Nembutal), has not been reported for infants. OBJECTIVE: To compare patient acceptance of oral Nembutal and oral chloral hydrate for sedation of infants up to 12 months of age. METHODS AND MATERIALS: Fifty-four infants (mean age: 7 months) were prospectively enrolled. Parents chose Nembutal, chloral hydrate, or no preference. Thirty-eight infants received Nembutal (4-6 mg/kg) mixed with cherry syrup and 16 received chloral hydrate (50-100 mg/ kg). We recorded infant's acceptance of sedative, parental impression of infant's acceptance, time to sedation, time to discharge, adverse effects, parental preference of future sedative. RESULTS: Infant acceptance and parental impression were better for Nembutal (P < 0.0001). Fewer parents in the Nembutal group preferred another sedative (P = 0.05). There was a trend toward shorter time to discharge with Nembutal (P = 0.03). There were no adverse effects in either group. One infant failed to sedate with Nembutal. CONCLUSIONS: Compared with chloral hydrate, oral Nembutal has significantly better acceptance by infants and parents, equal effectiveness, and may result in a shorter time to discharge.

Administration, Oral↗

PubMed2000

Karate injuries in children and adolescents.

OBJECTIVES: To identify risk factors for injury and to establish safety guidelines for children in Uechi-Ryu karate. DESIGN: A 1-year retrospective survey of injuries. SETTING: A private karate school (Uechi-Ryu style) in Plymouth, MA. PATIENTS: A total of 68 athletes (age 6-16 years; mean age 10 years) who participated in karate during the 1995-1996 season. INTERVENTIONS: None. MAIN OUTCOME MEASURES: The presence or absence of injury, with grading of injuries as major, moderate or minor. The types of injuries and body region involved were also analyzed. RESULTS: Twenty eight percent of athletes sustained at least one injury. All injuries were minor, with no time off from training required. The injuries consisted primarily of bruises (11 of 19). Other injuries included mild sprains or strains (5 of 19) and having their 'wind knocked out' (3 of 19). Most injuries were localized to the extremities. Logistic regression analysis identified risk factors for injury. Risk of injury increased with number of years of training (odds ratio 2.95; 95% confidence interval 1.81-4.82; P<0.0001), number of hours per week (odds ratio 2.12; CI 1.15-4.21; P = 0.016) and rank, specifically brown belt versus lower belts (odds ratio 6.56; CI 2.02-21.26; P = 0.006). CONCLUSIONS: Karate is a relatively safe sport for children and adolescents when properly taught. Risk of injury increases with experience; therefore, greater supervision is required of higher ranks. Injury increases with weekly training; however, 3 h a week or less appears to be associated with a low risk of significant injury in this age group.

Adolescent↗

PubMed2000

Postoperative recovery in children after minimum versus full-length sternotomy.

BACKGROUND: Minimal access incisions for pediatric cardiac surgery have been reported to hasten postoperative recovery. This prospective study compared recovery after a minimum versus full-length sternotomy for repair of atrial septal defects in children. METHODS: We studied 35 children undergoing atrial septal defect repair using a full-length sternotomy (n = 18) or ministernotomy (n = 17) according to the surgeon's preference. All children were managed according to an established clinical practice guideline. Intraoperative comparisons included patient demographics, bypass and cross-clamp times, and, as a measure of stress response, epinephrine, norepinephrine, and lactate levels at six time intervals throughout the surgical procedure. Postoperative comparisons included pain scores at 6, 12, and 24 hours, frequency of emesis, analgesic requirements, respiratory rate and gas exchange, and length of intensive care unit and total hospital stay. Nurse and parent assessment scores of overall recovery were constructed using visual analog and Likert scales. RESULTS: No significant differences between mini- versus full-length sternotomy were detected for the measured outcome variables. No adverse outcomes were detected. CONCLUSIONS: In this prospective study, a ministernotomy did not enhance postoperative recovery, and the primary advantage appears to be an improved cosmetic result.

Adolescent↗

PubMed2000

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus.

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.

Abnormalities, Multiple↗

PubMed2000

Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations.

OBJECTIVE: We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation. METHODS: A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle. Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia. RESULTS: Operative (>30 days) and 1-year survivals were lower for patients with hypoplastic left heart syndrome than for those with other lesions (63.4% vs 81%, P =.008, and 51.2% vs 71.4%, P =.02, respectively). The presence of a nonhypoplastic left ventricle (n = 27) was associated with higher operative and 1-year survivals (96.3% vs 64.7%, P =.002; 88.9% vs 52. 7%, P <.001). A restrictive atrial septal defect and prematurity tended to increase mortality across both groups. Cox proportional hazards regression indicated that a single right ventricle was the most important independent predictor of death (P <.001). Operative mortality for all patients undergoing the stage I procedure decreased from 38.5% (1990-1994) to 21.4% after 1994 (P =.02). CONCLUSIONS: The survival of patients with malformations other than hypoplastic left heart syndrome after the Norwood procedure is greater than for those with hypoplastic left heart syndrome. Staged palliation is valid surgical therapy in these patients, with good results in intermediate follow-up.

Abnormalities, Multiple↗

PubMed2000

Toronto Facial Grading System: interobserver reliability.

The Toronto Facial Grading System (TFGS) is an observer scale for rating facial nerve dysfunction. The TFGS scores aspects of resting symmetry, symmetry of voluntary movement, and synkinesis for each division of the face (subscores) and then provides calculated total scores and an overall composite score of facial function. The developers of the scale have validated its sensitivity for identifying small changes in facial dysfunction and the independence of the different components measured. Herein we report our results in a study of interobserver reliability using the TFGS. Twenty-five patients from the Massachusetts Eye and Ear Infirmary Facial Nerve Center with varying degrees of facial paresis, paralysis, and synkinesis were videotaped, and the video recordings were scored by 5 independent observers using the TFGS. Intraclass correlation coefficients (kappa) and 95% confidence intervals were calculated for subscores and for each total and composite score. Intraclass correlation coefficients ranged from 0.59 to 0.85, all considered substantial to near-perfect agreement between observers. We believe the TFGS is superior to other scales by virtue of its sensitivity, comprehensiveness, ease of use, and interobserver reliability. The TFGS presently appears to be the best option in those situations in which accurate and precise documentation of facial function is required.

Adult↗

PubMed2000

Accuracy of capnography with a 30 foot nasal cannula for monitoring respiratory rate and end-tidal CO2 in children.

We tested the accuracy of a low flow (50 cc/min) sidestream capnography system equipped with an experimental 30-foot nasal cannula to monitor ventilatory status in children. End-tidal CO2 and respiratory rate, both at room air and in the presence of supplemental oxygen, were recorded simultaneously from the experimental 30-foot nasal cannula and the standard, FDA approved, 10-foot nasal cannula. The 30-foot nasal cannula was as accurate as the 10-foot nasal cannula in measuring respiratory rate and end-tidal CO2 in children. When supplemental oxygen was delivered by face-mask, there was no dilutional effect on the respiratory rate or end-tidal CO2 recorded with either the 10-foot or 30-foot nasal cannulas in place.

Adolescent↗

PubMed2000

Parameters associated with perioperative baffle fenestration closure in the Fontan operation.

OBJECTIVE: To identify clinical parameters indicating perioperative fenestration closure in children who underwent the fenestrated Fontan operation. DESIGN: Retrospective. SETTING: Single children's hospital. PARTICIPANTS: Patients who underwent a fenestrated Fontan operation in 1996 through 1997 (n = 101). INTERVENTION: A fenestrated Fontan operation was performed in children with single-ventricle physiology. MEASUREMENTS AND MAIN RESULTS: Early perioperative closure of the fenestration occurred in 14 patients (group 1), whereas the fenestration remained patent in 87 patients (group 2). The groups were compared by the following parameters: demographics, cardiac catheterization and ultrasound data, and use of aspirin or warfarin preoperatively and intraoperatively by assessing the composition of the cardiopulmonary bypass solution, use of ultrafiltration and antifibrinolytics, protamine dose, last hematocrit on cardiopulmonary bypass, and requirement of blood products. Immediately postoperatively in the intensive care unit (ICU), cardiac filling pressures (central venous and left atrial pressure), coagulation profile, cardiac rhythm, chest tube drainage, length of stay in the ICU, and use of atrial pacing were reviewed. Significant indicators of early fenestration closure in this study as determined by multivariate stepwise logistic regression were a high transpulmonary pressure gradient (p = 0.015) and a higher oxygen saturation (p = 0.001) 1 hour after arrival in the ICU, a low fibrinogen level (p < 0.0001), and the need for temporary atrial pacing (p = 0.029). The fenestration was reopened in 13 patients in group 1. In 101 patients, there was no early mortality, and all patients survived to discharge. CONCLUSION: Factors that correlated with postoperative fenestration closure in the fenestrated Fontan operation in this study were a high transpulmonary pressure gradient and a high oxygen saturation 1 hour after arrival in the ICU, a low fibrinogen level, and the need for temporary atrial pacing.

Child↗

PubMed2000

Oral tacrolimus treatment of severe colitis in children.

OBJECTIVE: To evaluate the efficacy of oral tacrolimus as an induction agent in steroid-refractory severe colitis. STUDY DESIGN: Open-label, multicenter trial of oral tacrolimus in patients with severe colitis. Patients not responding to conventional therapy received tacrolimus, 0.1 mg/kg/dose given twice a day, and the dosage was adjusted to achieve blood levels between 10 and 15 ng/mL. Response was defined as improvement in a number of clinical parameters (including abdominal pain, diarrhea, rectal bleeding, and cessation of transfusions). Patients who responded by 14 days continued to receive tacrolimus, and 6-mercaptopurine or azathioprine was added as a steroid-sparing agent 4 to 6 weeks after the tacrolimus was instituted. RESULTS: Fourteen patients were enrolled in the study. One patient elected to withdraw after 48 hours. Of the 13 remaining, 9 (69%) responded and were discharged. Tacrolimus was continued for 2 to 3 months in the responders, except for 1 patient who was given tacrolimus for 11 months. After 1 year of follow-up, only 5 (38%) patients were receiving maintenance therapy; the other 4 responders had undergone colectomy. CONCLUSION: Although tacrolimus is effective induction therapy for severe ulcerative or Crohn's colitis, fewer than 50% of patients treated will successfully achieve a long-term remission.

Adolescent↗

PubMed2000

Surgery for bilateral outflow tract obstruction in elastin arteriopathy.

OBJECTIVE: A number of patients with Williams syndrome or other forms of elastin arteriopathy have stenoses of pulmonary arteries in addition to supravalvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for supravalvular aortic stenosis to help define the optimal treatment strategy for patients with severe forms of elastin arteriopathy. METHODS: Between 1960 and 1999, 33 patients underwent operations for supravalvular aortic stenosis while having significant stenoses of the pulmonary arteries. We retrospectively reviewed patient charts, obtained current follow-up information, and determined risk factors for survival and reoperation. RESULTS: Fifteen patients with moderate right-sided obstructions (confirmed by pulmonary artery Z-scores and right ventricular/descending aortic pressure ratio) underwent operations for supravalvular aortic stenosis only. Eighteen patients had more severe right-sided obstructions and underwent surgical relief of pulmonary arterial stenoses or right ventricular outflow tract obstruction in addition to operations for supravalvular aortic stenosis. Eight patients had undergone preoperative balloon dilations of stenotic pulmonary arteries. There were 6 early deaths and 1 late death in our series. Survival at 10 and 20 years was 76% (70% confidence interval, 68%-84%) and freedom from reintervention was 59% (70% confidence interval, 46%-71%) at 10 years and 49% (70% confidence interval, 35%-62%) at 20 years. Multivariate analysis revealed that patients with a right ventricular/descending aortic pressure ratio of 1.0 or more were at higher risk for reintervention but not for death. CONCLUSIONS: Surgical treatment of pulmonary artery obstructions in elastin arteriopathy is palliative but, in conjunction with balloon dilation of peripheral pulmonary arteries, offers good long-term survival to patients with the severest form of elastin arteriopathy.

Adolescent↗

PubMed2000

Subclavian flap angioplasty: does the arch look after itself?

OBJECTIVES: We sought to assess the early and long-term results of subclavian flap angioplasty in neonates and infants, with particular attention to growth of the hypoplastic arch. METHODS: A retrospective analysis of 185 consecutive patients who underwent subclavian flap angioplasty between 1974 and 1998 was carried out. The patients included 125 neonates and 60 infants, with a median age of 18 days. Sixty-six (36%) patients had an additional ventricular septal defect, 41 (22%) patients had aortic arch hypoplasia diagnosed preoperatively, 141 (76%) had an associated patent ductus arteriosus, and 41 (22%) had additional complex heart disease. Follow-up was with transthoracic Doppler echocardiography in all patients. RESULTS: The early mortality was 3%. Recoarctation, defined as a Doppler gradient of 25 mm Hg or more, occurred in 11 (6%) patients at a median follow-up of 6.2 years (6.2 +/- 4.6 years). This included 4 of the 41 patients in whom arch hypoplasia was diagnosed preoperatively. There were no complications with the left arm. By multivariate analysis, risk factors for death were determined to be residual arch hypoplasia and low birth weight. The only risk factor for recoarctation was persistent arch hypoplasia after surgical treatment. However, angiographic imaging of the aorta showed that recoarctation was not due to a hypoplastic transverse arch, and it was probably at the site of ductal tissue. Survival at 5 and 10 years was 98% and 96%, respectively. Freedom from reoperation for recoarctation at 2 years was 95%, and at 5, 10, and 15 years, it was 92%. CONCLUSIONS: Subclavian flap repair remains an effective technique for repair of aortic coarctation with excellent results and low mortality. In the majority of patients, arch hypoplasia regresses after this procedure.

Angioplasty↗