Biomedical subjects

E N Mendeloff

Publications and source records attributed to E N Mendeloff.

At least 19 recordsLinked to original sources

PubMed2001

Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve.

BACKGROUND: Infants presenting with anomalous left coronary artery off the pulmonary artery (ALCAPA) are generally in heart failure and often have significant mitral valve regurgitation (MR). Although establishing a dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled. METHODS: We reviewed our experience with this lesion at St. Louis Children's Hospital. Over the past 15 years, 17 infants under 18 months of age have undergone repair, with all but one being treated with reimplantation of the left coronary artery into the aorta; the other underwent the Takeuchi procedure (intrapulmonary artery baffle) and was excluded from this evaluation. The average age and weight at operation were 0.5 +/- 0.3 years and 6.1 +/- 1.9 kg, respectively. All presented with varying degrees of heart failure and 9 patients also had either moderate or severe MR. RESULTS: There was one early and no late deaths after reimplantation of the left coronary artery. The left ventricular function postrepair improved from a preoperative shortening fraction of 0.19 +/- 0.09 to 0.34 +/- 0.08 (p < 0.01). Moderate or severe MR was present in 2 patients postoperatively, and both developed significant obstruction in the left coronary artery postoperatively as well. Both underwent mitral valve repair and revascularization of the left coronary artery. CONCLUSIONS: Excellent results can be obtained in the treatment of this very high-risk group of patients. Mitral valve repair is not generally necessary at the time of the initial operation. However, should MR recur or persist late, it may herald the presence of a coexistent, significant coronary stenosis. Cardiac catheterization should be performed to assess the patency of the left coronary artery before performing mitral valve surgery.

Child, Preschool↗

PubMed2001

Lung transplantation in infants.

Lung transplantation is an accepted mode of therapy for patients with end-stage pulmonary vascular or parenchymal diseases. To date, application to the pediatric age population in general, and the infantile group in particular, has been limited both in terms of number of transplants and follow-up thereafter. We performed our first transplant in an infant in 1993 and have performed 30 lung transplants in children under 1 year of age subsequent to that. This chapter will review the experience to date including indications for transplantation, post-transplant management, complications, and long-term follow-up.

Bronchiolitis Obliterans↗

PubMed2000

Quality of life after aortic valve replacement at the age of >80 years.

BACKGROUND: The optimal management of aortic valve disease in patients >80 years old depends on functional outcome as well as operative risks and late survival. METHODS AND RESULTS: We retrospectively identified 133 patients (62 men, 71 women) aged 80 to 91 years (mean 84+/-3 years) who underwent aortic valve replacement alone or in combination with another procedure between January 1, 1993, and April 31, 1998. Demographics included hypertension 68%, diabetes mellitus 17%, and history of stroke 11%. Operative (30 day) mortality rate was 11%. Urgent or emergent surgery, aortic insufficiency, and perioperative stroke or renal dysfunction were risk factors for operative death by multivariable analysis. Intensive care unit and total hospital length of stay were prolonged at 6.2 and 14.7 days, respectively. Late follow-up between July 1, 1998, and November 1, 1999, was 98% complete. Actuarial survival at 1 and 5 years was 80% and 55%, respectively. Predictors of late mortality were preoperative or perioperative stroke, chronic obstructive pulmonary disease, aortic stenosis, and postoperative renal dysfunction. The mean New York Heart Association functional class for 65 long-term survivors improved from 3.1 to 1.7. Quality of life assessed with the Medical Outcomes Study Short Form-36 was comparable to that predicted for the general population >75 years old. CONCLUSIONS: Functional outcome after aortic valve replacement in patients >80 years old is excellent, the operative risk is acceptable, and the late survival rate is good. Surgery should not be withheld from the elderly on the basis of age alone.

Age Factors↗

PubMed1999

Scimitar syndrome presenting in infancy.

BACKGROUND: Scimitar syndrome has a variable presentation based on the age at which the diagnosis is made. In general, infants presenting in heart failure have a greater number of associated anomalies and their prognosis is much worse. METHODS: We reviewed the records of all patients under our care at St. Louis Children's Hospital who presented with symptoms attributable to scimitar syndrome during their infancy. Twelve patients were identified. The average age at presentation was 6 weeks. RESULTS: The most common symptom at presentation was tachypnea. The chest roentgenogram demonstrated dextroposition of the heart and hypoplastic right lung. Only 1 patient had the classic "scimitar sign." Cardiac catheterization demonstrated pulmonary hypertension (pulmonary artery systolic pressure, 73.9+/-21.8 mm Hg). The Qp:Qs was 3.1+/-1.5:1. Two patients with severe associated anomalies were treated medically and both died. Two patients underwent occlusion of the systemic collaterals; one died and the other ultimately underwent complete repair due to persistence of the symptoms of heart failure. Two patients had primary right pneumonectomy and both are alive and well. Seven patients underwent complete repair (one after coil occlusion of the systemic arterial collaterals) and one died; three subsequently developed occlusion of the baffle from the orifice of the anomalous pulmonary vein and required pneumonectomy. Two patients required lung transplantation due to persistent pulmonary hypertension in one and recurrent bilateral pulmonary venous stenosis in the other. CONCLUSIONS: Infants presenting with scimitar syndrome generally have symptoms of tachypnea and chest roentgenograms showing dextrocardia and hypoplastic right lung. Although repair of the anomalous venous return and ligation of collaterals is generally recommended, right pneumonectomy (either as primary therapy or if repair failed) had similar early and late results.

Collateral Circulation↗

PubMed1999

Rejection with heart failure after pediatric cardiac transplantation.

BACKGROUND: Rejection associated with heart failure or death occurs after pediatric cardiac transplantation but has had limited analysis. METHODS: We analyzed the records of 96 consecutive pediatric cardiac transplant recipients who survived to hospital discharge. RESULTS: Eighteen patients (19%) experienced 23 episodes of heart failure or death associated with rejection. Univariate analysis demonstrated black race (p = 0.041), transplantation after 12 months of age (p = 0.032), later time after transplantation (p = 0.037), rejection episode in the first year after transplantation (p = 0.001), and history of two or more rejection episodes (p < 0.001) were significantly associated with rejection seen with heart failure. A multivariate regression analysis identified two or more rejection episodes to be the only independent risk factor for the development of rejection with heart failure (odds ratio 20; 95% confidence limits, 4-104; p < 0.0001). CONCLUSIONS: This study identified pediatric heart transplant recipients with a history of previous rejection episodes to be at a higher risk for symptomatic or fatal rejection. Further studies are needed to determine if intensification of maintenance immunosuppression, long-term rejection surveillance, or both in patients with multiple rejection episodes could reduce morbidity and mortality from rejection.

Child↗

PubMed1999

Reappraisal of coronary endarterectomy for the treatment of diffuse coronary artery disease.

BACKGROUND: The development of new technologies such as transmyocardial laser revascularization and, more recently, local delivery of angiogenic growth factors has refocused attention on the surgical management of diffuse coronary artery disease. In some cases, coronary endarterectomy is also technically feasible. To facilitate decision-making among these options, we reviewed our experience with coronary endarterectomy to determine the results to be expected with this more traditional approach. METHODS: A search of our computerized database identified 7,096 patients undergoing myocardial revascularization between January 1, 1986 and March 30, 1997, of whom 177 (2.3%) underwent endarterectomy of at least one coronary artery. Perioperative events were derived from the database. Follow-up information was obtained from patients at 3 months to 11.5 years (mean = 55.7 +/- 38.8 months) after surgery. RESULTS: Endarterectomy was performed on the right coronary artery (RCA) system in 100 patients, the left anterior descending (LAD) system in 52, the circumflex system in 18, and in multiple distributions in 7. The 30-day mortality was 7% for RCA, 0% for LAD, 17% for circumflex, and 14% for multi-system endarterectomy (p = 0.20). There were no statistically significant differences in perioperative myocardial infarction or ventricular dysrhythmia between these groups. Actuarial survival at 5 years was 76% for patients undergoing RCA endarterectomy and 75% for left sided (LAD or circumflex) endarterectomy (p = 0.91). At late follow-up, 74% (86/117) of survivors were angina-free, 6% (7/117) had undergone subsequent angioplasty, and 3% (4/117) had undergone subsequent surgery. CONCLUSIONS: Coronary endarterectomy can be accomplished with acceptable operative risk and good long-term results, even when applied in a highly selective manner. The results of novel therapies for diffuse coronary artery disease should be considered in the context of those achievable with more traditional approaches.

Actuarial Analysis↗

PubMed1999

Intestinal obstruction after lung transplantation in children with cystic fibrosis.

BACKGROUND/PURPOSE: Distal intestinal obstruction syndrome (DIOS) occurs in 15% of patients with cystic fibrosis (CF). The authors reviewed their experience to determine the incidence, risk factors, and natural history of adhesive intestinal obstruction and DIOS after lung transplantation. METHODS: Eighty-three bilateral transplants were performed in 70 CF patients between January 1990 and September 1998. All were on pancreatic enzymes preoperatively, and none had preoperative bowel preparation. Fifty-six patients (80%) had prior gastrostomy (n = 54) or jejunostomy (n = 2). Eighteen patients (25.7%) had a previous laparotomy for meconium ileus (n = 8), fundoplication (n = 4), liver transplant (n = 1), jejunal atresia (n = 1), Janeway gastrostomy takedown (n = 1), pyloromyotomy (n = 1), free air (n = 1), or appendectomy (n = 1). RESULTS: After lung transplantation, 7 patients (10%) required laparotomy for bowel obstruction (6 during the same hospitalization, and 1 during a subsequent hospitalization). The causes of obstruction were adhesions only (n = 1), DIOS only (n = 2), and a combination of DIOS and adhesions (n = 4). Adhesiolysis was performed in the 5 patients with adhesions, and a small bowel resection was also performed in 1 patient. DIOS was treated by milking secretions distally without an enterotomy (n = 3) with an enterotomy and primary closure (n = 1) or with an end ileostomy and mucus fistula (n = 2). Five had recurrent DIOS early postoperatively. One resolved with intestinal lavage, 2 were treated successfully with hypaque disimpaction, and 2 underwent reoperation; 1 required an ileostomy. The most important risk factor for posttransplant obstruction was a previous major abdominal operation. Obstruction occurred in 7 of 18 (39%) who had undergone a prior laparotomy versus 0 of 52 who had not (P < .001, chi2). CONCLUSIONS: (1) The incidence of intestinal obstruction is high after lung transplantation in children with CF. (2) Previous laparotomy is a significant risk factor. (3) Recurrent obstruction after surgery for this condition is common. (4) Preventive measures such as pretransplant bowel preparation and early postoperative bowel lavage may be beneficial in these patients.

Adolescent↗

PubMed1999

Lung transplantation in very young infants.

INTRODUCTION: Established successes with adult lung transplantation have laid the foundation for extension of this therapeutic modality to infants and children dying of end-stage pulmonary disease. The purpose of this report is to convey our experience with 19 infants undergoing lung transplantation before the age of 6 months. METHODS: Six patients with predominantly pulmonary vascular disease and 13 patients with primarily pulmonary parenchymal disease have undergone bilateral sequential lung transplantation at our institution since 1990. Mean age at transplant was 104 +/- 44 days, and mean weight was 4.9 +/- 1.6 kg. RESULTS: Although early mortality (32%, 6/19) was higher than that previously reported for older pediatric age groups, long-term survival was similar (44% at a maximum follow-up of 6 years). Although anastomotic complications and infections occurred at a rate approximating that seen in older pediatric age groups, episodes of acute rejection appear to occur with decreased frequency. Similarly, at a mean follow-up of 3 years, only 2 (15%) of 13 long-term survivors have evidence of bronchiolitis obliterans. The functional residual capacity, as measured on infant pulmonary function tests, has gradually increased as the children have grown, suggesting that lung growth is occurring. CONCLUSIONS: Bilateral lung transplantation is a viable alternative in infants dying of end-stage pulmonary disease. Efforts directed toward avoiding the complications that lead to early posttransplant mortality combined with the seemingly lower incidence of early and late rejection may provide long-term results better than those in other age groups.

Actuarial Analysis↗

PubMed1999

Scimitar syndrome.

Explore the source record for details and available documents.

Cardiac Catheterization↗

PubMed1998

Patterns and potential value of cardiac troponin I elevations after pediatric cardiac operations.

BACKGROUND: Perioperative myocardial injury is a major determinant of postoperative cardiac dysfunction for congenital heart disease, but its assessment during this period is difficult. The objective of this study was to determine the suitability of using postoperative serum concentrations of cardiac troponin I (cTnI) for this purpose. METHODS: Cardiac troponin I levels were measured serially in the serum of patients undergoing uncomplicated repairs of atrial septal defect (n = 23), ventricular septal defect (n = 16) or tetralogy of Fallot (n = 16). The concentrations were correlated with intraoperative parameters (cardiopulmonary bypass time, aortic cross-clamp time, and cardiac bypass temperature), and postoperative parameters (magnitude of inotropic support, duration of intubation, and postoperative intensive care and hospital stay). RESULTS: Postoperative absolute cTnI levels were lesion specific, with a pattern of increase and decrease similar for each lesion. For the total cohort, significant correlations between postoperative cTnI levels at all times (r = 0.43 to 0.83, p < 0.05) until 72 hours were noted for all parameters, except for cardiac bypass temperature. When evaluated as individual procedure groups, no significant relationships were noted in the atrial septal defect group, whereas postoperative cTnI levels were more strongly correlated with all intraoperative and postoperative parameters in the ventricular septal defect group than in the tetralogy of Fallot group. CONCLUSIONS: This study suggests that cTnI values immediately after operation reflect the extent of myocardial damage from both incisional injury and intraoperative factors. Cardiac tropinin I levels in the first hours after operation for congenital heart disease are a potentially useful prognostic indicator for difficulty of recovery.

Body Temperature↗

PubMed1998

Lung retransplantation in children.

BACKGROUND: Early primary graft failure due to reperfusion injury may occur in up to 10% of all patients undergoing lung transplantation. Late graft failure in the form of bronchiolitis obliterans progressively increases in frequency as posttransplantation follow-up increases. In both situations, the degree of pulmonary dysfunction may worsen and result in the death of the recipient. The only treatment in many instances is retransplantation. The results in adults are reasonably well established. METHODS: We reviewed our experience in children. Of the 136 transplant procedures performed to date in children, 14 have been retransplantations. Six patients required retransplantation for early primary graft failure and 8 underwent retransplantation for bronchiolitis obliterans. RESULTS: There were three early and three late deaths. The actuarial survival at 2 years is 58%. The retransplant procedures were more complex than the primary transplant operations as evidenced by the longer time on cardiopulmonary bypass (199 +/- 71 versus 150 +/- 41 minutes; p < 0.01) and the greater volume of blood transfused (1,303 +/- 936 versus 570 +/- 300 mL; p < 0.01). Two of the long-term survivors who received transplants for bronchiolitis obliterans have subsequently had development of this same condition and 1 died secondary to this. In four instances living related donors were used for the retransplant procedure. The most striking difference in these procedures compared with those transplantations performed with cadaveric donors was the shorter donor lung ischemic times (99.5 and 123.3 minutes for the two lungs for living related donors and 251 and 293 minutes for the first and second lung for the cadaveric donors; p < 0.01). CONCLUSIONS: We believe that lung retransplantation in children is a reasonable therapy to offer in the circumstance of severe graft dysfunction. In the older child, the option of living donor transplantation offers advantages that might offset of the overall higher risk of this procedure.

Actuarial Analysis↗

PubMed1998

Tracheal mucoepidermoid carcinoma in a 7-year-old child.

Mucoepidermoid cancers are rare tumors that arise from the serous and mucous glands of the upper airway and salivary glands. Patients, especially children, with tumors that arise in the trachea and upper airways are often misdiagnosed as asthmatic and are treated with bronchodilators without resolution. A 7-year-old girl who had been diagnosed as asthmatic was subsequently found to have a mucoepidermoid tumor of the trachea. She underwent a successful tracheal resection and remains tumor free on follow-up bronchoscopy at 16 months.

Carcinoma, Mucoepidermoid↗

PubMed1998

Pediatric and adult lung transplantation for cystic fibrosis.

OBJECTIVE: This paper was undertaken to review the experience at our institution with bilateral sequential lung transplantation for cystic fibrosis. METHODS: Since 1989, 103 bilateral sequential lung transplants for cystic fibrosis have been performed (46 pediatric, 48 adult, 9 redo); the mean age was 21 +/- 10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplant, and in 15% of adults. RESULTS: Hospital mortality was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1 +/- 1.6 years). Mean forced expiratory volume in 1 second increased from 25% +/- 9% before transplantation to 79% +/- 35% 1 year after transplantation. Acute rejection occurred 1.7 times per patient-year, with most episodes taking place within the first 6 months after transplantation. The need for treatment of lower respiratory tract infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population and was associated with an early mortality of 33%. Eight living donor transplants (four primary transplants, four redo transplants) were performed with an early survival of 87.5%. CONCLUSION: Patients with end-stage cystic fibrosis can undergo bilateral lung transplantation with morbidity and mortality comparable to that seen in pulmonary transplantation for other disease entities.

Actuarial Analysis↗

PubMed1998

Lung transplantation and repair of complex congenital heart lesions in patients with pulmonary hypertension.

Pulmonary vascular disease in conjunction with either a previously repaired or an unrepaired congenital heart defect is the third most common indication for lung transplantation in the pediatric age range. Because scarcity of donor organs remains a critical issue and heart-lung donor blocks are becoming diminishingly available, efforts must be directed towards other options such as combining lung transplantation with correction of the underlying congenital heart defect. Certain defects like congenital pulmonary vein stenosis are eradicated by removal of the diseased lungs, whereas others such as complete atrioventricular canal and pulmonary atresia with ventricular septal defect require cardioplegic arrest of the heart and intracardiac repair in conjunction with the lung transplantation. A breakdown of this patient population into subgroups may be helpful both in thinking about the pathophysiology and in determining appropriate indications and timing of transplantation. Earlier studies from our center showed the high-risk nature and formidable undertaking of caring for this complex group of patients. Through continued experience, there has been gradual improvement in early outcomes. As with all other groups of lung transplantation patients, obliterative bronchiolitis remains the major deterrent to long-term survival.

Adolescent↗

PubMed1998

Lung transplantation for cystic fibrosis.

Cystic fibrosis (CF) is an inherited disease in which the fundamental physiological defect is failure of cAMP regulation of chloride transport. More than 90% of patients with CF will die of chronic, suppurative, obstructive lung disease, with the median survival in the United States currently being 29 years of age. Currently, although other therapies are being aggressively investigated, bilateral lung transplantation offers the only hope for short-term and mid-term survival in patients with CF and end-stage pulmonary disease. Since 1989, 103 bilateral sequential lung transplants (BLT) for CF have been performed at our institution (46 pediatric, 48 adult, 9 redo) at a mean age of 21+/-10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplantation, and in 15% of adults. The hospital mortality rate was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival rates are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1+/-1.6 years). Mean forced expiratory volume in 1 second increased from 25%+/-9% pretransplantation to 79%+/-35% 1 year posttransplantation. Acute rejection occurred 1.7 times per patient-year, with the majority of these episodes taking place the first 6 months posttransplantation. Need for treatment of lower respiratory infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population, and was associated with an early mortality of 33%. Eight living donor transplants (4 primary transplants, 4 redo transplants) were performed with an early survival of 87.5%. Patients with end-stage CF can undergo BLT with morbidity and mortality comparable with that observed in pulmonary transplantation for other disease entities.

Adolescent↗

PubMed1997

Previous coronary artery bypass grafting is not a risk factor for aortic valve replacement.

BACKGROUND: The risk of aortic valve replacement (AVR) after previous coronary artery bypass grafting (CABG) is controversial. Its magnitude influences the threshold for recommending this procedure and has been cited in arguments regarding the optimal management of mild aortic stenosis at primary CABG. We therefore reviewed our experience with reoperative AVR +/- CABG and the primary combined procedure. METHODS: Between January 1, 1985, and June 30, 1996, 427 patients underwent primary AVR+CABG, and 52 underwent AVR +/- CABG after prior CABG. Demographics, operative characteristics, and operative results were compared between groups. Data for all patients were pooled and analyzed collectively for risk factors influencing mortality. RESULTS: The extent of native coronary artery disease and the incidence of prior myocardial infarction and stroke were greater in the reoperative group. Aortic cross-clamp and cardiopulmonary bypass times were slightly shorter, and fewer distal anastomoses were performed in the reoperative group. Operative mortality (primary group, 6.3% versus reoperative group, 7.4%) and morbidity were similar. Stepwise multivariate logistic regression analysis identified age, perioperative myocardial infarction, intraaortic balloon support, ventricular arrhythmia, perioperative stroke, and development of renal failure or acute respiratory distress syndrome, but not reoperative status, as predictors of mortality. CONCLUSIONS: The risk of AVR after previous CABG is similar to that for primary AVR+CABG. Valve replacement should, therefore, be pursued despite prior CABG when hemodynamically significant aortic stenosis develops. Furthermore, a circumspect approach to "prophylactic" AVR for mild aortic stenosis at primary CABG seems warranted.

Age Factors↗

PubMed1997

Unusual cause of pulmonary hypertension and congestive heart failure in a newborn.

Anomalous systemic arterial supply to a lobe of the lung is a rare cause of pulmonary hypertension and congestive heart failure in the newborn period. We report the presentation and successful treatment of a neonate with this unusual anatomy. Proper diagnosis required both echocardiography and aortography, and surgical resection of the involved lobe was curative.

Aorta↗

PubMed1996

Infant and donor organ growth after heart transplantation in neonates with hypoplastic left heart syndrome.

BACKGROUND: There is little published data regarding somatic growth and changes in allograft size after heart transplantation in infants with hypoplastic left heart syndrome. METHODS: We evaluated the somatic growth of 26 infants with hypoplastic left heart syndrome who underwent heart transplantation over a 5-year period and measured changes in left ventricular dimensions in 22 of those infants. Age at transplantation was 27 +/- 17 days (mean +/- standard deviation), and the follow-up period was 43 +/- 14 months. Growth and echocardiographic data were converted to standard deviation (Z) scores for comparison with normal populations. RESULTS: Height and weight were always within normal limits (two standard deviations), with a trend toward smaller size throughout the follow-up period. The somatic growth of infants on low-dose maintenance steroids was not significantly different from that of infants withdrawn from chronic steroid regimens. Initial left ventricular posterior wall and septal dimensions were greater than two standard deviations (+4.3 and +6, respectively), probably the result of routine use of oversized donors, but the dimensions decreased to within the normal range during the first year. They then remained within normal limits during follow-up. With one exception at 2 years after transplantation, left ventricular diastolic dimensions were always within two standard deviations of the mean. Left ventricular dimensions of patients with hypertension, rejection, or acute graft failure were not significantly different from patients without these complications. CONCLUSIONS: Neonates with hypoplastic left heart syndrome who undergo heart transplantation can be expected to have somatic growth within normal limits. However, the trend toward growth retardation is worrisome. Left ventricular wall dimensions adjust to smaller recipient size during the first year after transplantation and then remain appropriate for the recipient's size over time.

Analysis of Variance↗