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Biomedical subjects

C W Lillehei

Publications and source records attributed to C W Lillehei.

At least 19 recordsLinked to original sources

The high-risk liver allograft recipient. Should allocation policy consider outcome?

The Boston Center for Liver Transplantation has accumulated one of the larger series of liver allograft recipients. This review has provided an opportunity to examine recent pronouncements by Medicare regarding patient selection and survival and to question whether the current allocation scheme best utilizes a scarce supply of donor liver allografts. Patients with primary biliary cirrhosis, sclerosing cholangitis, and metabolic derangements have enjoyed excellent survival: in aggregate, 78.9% at 1 year. In contrast, patients suffering from acute hepatic failure, patients requiring life support, or patients with primary graft failure who need a second liver transplant did poorly compared with other recipient groups: 45% 1-year survival. This center's experience reflects a more realistic expectation of patient survival because it considers the high-risk recipient by diagnosis and urgency status. This study also suggests that assessment of outcome should be a component of allocation planning in the future.

Adult

The prenatal diagnosis of imperforate anus with rectourinary fistula: dilated fetal colon with enterolithiasis.

The finding of extraluminal calcifications is commonly reported and usually indicates intrauterine intestinal perforation with intraperitoneal extravasation of meconium, most often associated with intestinal obstruction and/or atresias. Intraluminal calcification of meconium is more rare and appears to result from the mixing of stagnant urine and meconium in utero. The presence of the intraluminal calcifications in a dilated loop of intestine, particularly with an associated urinary tract abnormality, should suggest a rectourinary fistula. Two cases of prenatally diagnosed imperforate anus with rectourinary fistulae are reported.

Adult

Echocardiography in the preoperative evaluation of vascular rings.

Vascular rings may produce tracheal and/or esophageal compression in infants and children. Traditionally recognized fluoroscopically, the exact anatomy of the ring and appropriate surgical correction are determined intraoperatively. The role of preoperative echocardiography was examined. Twenty patients with symptomatic vascular rings were evaluated preoperatively with echocardiography at this institution. Their ages ranged from 10 days to 11 years (mean, 17 months). There were 9 boys and 11 girls. Most (17/20) presented with respiratory symptoms in the first year of life, although in 3 patients dysphagia was the primary complaint (at birth, 4 months, 9 years). All underwent initial evaluation with a barium esophagogram prior to the echocardiogram. Surgical correction was subsequently performed and the exact anatomy identified. All barium esophagograms were interpreted prospectively as demonstrating a "vascular ring." Although often suspected fluoroscopically, the actual type of ring was correctly identified by echocardiogram in all cases including determination of the dominant arch and associated anomalies. The types of vascular rings included double aortic arch (10), right aortic arch with left ligamentum arteriosum and/or aberrant left subclavian artery (6); aberrant right subclavian artery (2), and pulmonary artery sling (2). Barium esophagogram remains the best screening test for children in whom a vascular ring is suspected. However, echocardiography is a useful noninvasive complementary examination to confirm the diagnosis, clarify anatomy, and exclude other major intracardiac pathology prior to surgical correction.

Aorta, Thoracic

Reduced-size lung transplantation from adult to neonatal sheep.

Lung transplantation continues to be limited by donor availability. This shortage is particularly acute in the pediatric population. A model was developed in sheep to simulate human pulmonary transplantation from adults into newborns. Pulmonary allografts were performed in 8 neonatal lambs (less than 10 days old) weighing between 2.5 and 5 kg. Unrelated adult sheep donors were used (weighing 55 to 82 kg). The recipient body weight was approximately 6% that of the donor, but ranged from 4% to 8%. Ipsilateral or contralateral upper lobe allografts were used, although the latter required inversion prior to implantation. All venous anastomoses utilized left atrial cuffs. The pulmonary arterial and bronchial anastomoses were constructed end-to-end. Following implantation the lobes were further contoured to fit within the recipient hemithorax using a linear surgical stapler. Animals were immunosuppressed with cyclosporine. Two animals died 1 week postoperatively but the remaining six survived 1 to 5 months. Postoperative lung scans at 10 to 14 days confirmed satisfactory ventilation and perfusion of the allografts. All vascular anastomoses were intact at postmortem examination. Primary bronchial healing accomplished without an omental wrap. We conclude that successful lung transplantation can be performed from adult donors into neonatal recipients. Preservation of the maximal arterial and venous length during native pneumonectomy is essential to allow matching of the vascular cuffs. Short donor lobar bronchi may be responsible for the reliable primary healing. Improved immunosuppression will be required to assess long-term function and growth of these reduced-size allografts.

Animals

Delayed repair and preoperative ECMO does not improve survival in high-risk congenital diaphragmatic hernia.

It has been suggested that delayed repair with preoperative stabilization might improve survival in high-risk (symptomatic within 6 hours of birth) congenital diaphragmatic hernia (CDH). This study compares the results of immediate operation versus delayed repair using extracorporeal membrane oxygenation (ECMO) when necessary. Since we first used ECMO in 1984, 101 high-risk CDH infants have been treated. Prior to 1987, we used immediate repair and postoperative ECMO if necessary. Between 1987 and 1990 we combined delayed operation (24 to 36 hours) with preoperative ECMO as necessary. No infant in this series was excluded from ECMO therapy unless absolute contraindications existed (prematurity, intracranial hemorrhage, or other major anomalies). Fifty-five patients received immediate operation and 46 had delayed repair. The two groups were comparable populations based on gestational age, birth weight, age at onset of symptoms, Apgar scores, best postductal PO2 (BPDPO2), and frequency of antenatal diagnosis. There was no statistically significant difference in overall survival between the two groups. Differences in survival among subpopulations (BPDPO2 greater than 100 or less than 100, antenatal diagnosis, inborn v outborn) also are not significant. The requirement for ECMO was similar in both groups. Survivors in the delayed repair group were ventilated longer and on ECMO longer, but had fewer late deaths (greater than 21 days) and fewer pulmonary sequelae (O2 dependency at discharge) than infants in the immediate repair group (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal Membrane Oxygenation

Congenital diaphragmatic hernia. Stabilization and repair on ECMO.

Availability of extracorporeal membrane oxygenation (ECMO) support and the potential advantages of delayed repair of congenital diaphragmatic hernia (CDH) have led several centers to delay CDH repair, using ECMO support if necessary. This study reviews the combined experience of five ECMO centers with infants who underwent stabilization with ECMO and repair of CDH while still on ECMO. All infants were symptomatic at birth, with a mean arterial oxygen pressure (PaO2) of 34 mmHg on institution of bypass despite maximal ventilatory support. A total of 42 infants were repaired on ECMO, with 18 (43%) surviving. Seven infants had total absence of the diaphragm, and 28 required a prosthetic patch to close the defect. Only five infants ever achieved a best postductal PaO2 over 100 mmHg before institution of ECMO. Prematurity was a significant risk factor, with no infants younger than 37 weeks of age surviving. Significant hemorrhage on bypass was also a hallmark of a poor outcome, with 10 of the 24 nonsurvivors requiring five thoracotomies and six laparotomies to control bleeding, whereas only one survivor required a thoracotomy to control bleeding. In follow-up, nine of the 18 survivors (50%) have developed recurrent herniation and seven (43%) have significant gastroesophageal reflux. Importantly, five of the 18 survivors were in the extremely high-risk group who never achieved a PaO2 over 100 mmHg or an arterial carbon dioxide pressure (PaCO2) less than 40 mmHg before the institution of ECMO. In conclusion, preoperative stabilization with ECMO and repair on bypass may allow some high-risk infants to survive. Surviving infants will require long-term follow-up because many will require secondary operations.

Extracorporeal Membrane Oxygenation

Late results (30 to 35 years) after operative closure of isolated ventricular septal defect from 1954 to 1960.

This study was designed to determine the clinical status, cause of death, and effects of pulmonary vascular disease and conduction abnormalities 30 to 35 years after surgery in 296 consecutive surviving patients of closure of ventricular septal defect. Of the 296 patients, current status was determined by contact with patient and physician in 290 cases, with 6 (2%) lost to follow-up (7,912 patient years are included). Cardiac catheterization after surgery in 168 patients showed complete closure of the defect in 80%. Death occurred in 59 patients (20%), with higher mortality rates in those operated on after the age of 5 years, those with pulmonary vascular resistance greater than 7 units (51%), and those with complete heart block (78%). Of 37 patients with transient heart block after surgery, 8 (22%) have died (3 pulmonary vascular disease, 2 sudden death, 2 unknown causes and 1 complete heart block). Twenty other patients had a dysarrhythmia after surgery, and none of these died. Nine episodes of endocarditis occurred (11.4/10,000 patient years). Nine of 296 (3%) offspring had cardiac malformation. Most patients are in New York Heart Association class I, 57% attended college and 15% received an advanced degree. The data show good results for this group of patients operated on during an early era (1954 to 1960) of open cardiac surgery. They support the current trend toward operation in patients with ventricular septal defects at an early age and with low pulmonary vascular resistance.

Adult

Congenital diaphragmatic hernia: predictors of severity in the ECMO era.

Infants with congenital diaphragmatic hernia (CDH) demonstrate a wide range of anatomic and physiologic abnormalities, making it difficult to compare the efficacy of new forms of therapy such as extracorporeal membrane oxygenation (ECMO) among institutions. This study was undertaken to determine whether any predictors of severity could be identified in the ECMO era. The charts of all patients with CDH treated at this institution since 1984, when ECMO became available. (n = 110), were reviewed. Infants were considered high risk and included in this study if they presented with respiratory distress within the first 6 hours of life (n = 94). In order to focus on predictors of pulmonary insufficiency, patients who died of nonpulmonary causes or had other significant congenital anomalies were excluded from this review, leaving 59 patients for analysis. All the infants during this period had intensive pharmacological and ventilatory support. When needed, ECMO was offered postoperatively from 1984 to 1987, and preoperatively from 1987 to the present. Forty-five of 59 had a best postductal PO2 (BPDPO2) greater than 100 mm Hg, and 41 of these responders survived (91%). Fourteen patients had a BPDPO2 less than 100 mm Hg and only one survived (7%) (P = .0001). Mean BPDPO2 between survivors with or without ECMO, and nonsurvivors were also significantly different (P = .001). To incorporate ventilatory information, an oxygenation/ventilation index was devised: [OVI = PO2/(mean airway pressure x respiratory rate) x 100]. Differences in OVI between these three groups were also significant. When analyzing the data by the method proposed by Bohn (PCO2 v VI), no correlation between ventilatory parameters and outcome was found.(ABSTRACT TRUNCATED AT 250 WORDS)

Carbon Dioxide

The effect of extracorporeal membrane oxygenation on the survival of neonates with high-risk congenital diaphragmatic hernia: 45 cases from a single institution.

At The Children's Hospital, Boston (TCH), in the 3 years before extracorporeal membrane oxygenation (ECMO) was available, infants with high-risk congenital diaphragmatic hernia (CDH) had a 47% survival rate. In February 1984, ECMO was introduced and offered to all high-risk CDH infants with a 100% predicted mortality. Since February 1984, 45 infants with high-risk CDH presented to TCH. Twenty-six (58%) were supported with ECMO; 19 (42%) never met the criteria for 100% predicted mortality and were supported with conventional mechanical ventilation (CMV). Overall survival was 49%. Nine (35%) of the 26 ECMO patients survived. Thirteen (68%) of the 19 CMV patients survived. Although there was no change in survival, there was a change in the cause of death. Deaths in the ECMO group were either early (n = 8, secondary to a complication of ECMO or lack of pulmonary improvement) or late (n = 9). The late deaths were infants who were successfully weaned from ECMO, never weaned from CMV, and who died secondary to complications of chronic lung disease.

Extracorporeal Membrane Oxygenation

The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience.

Survival has improved dramatically for children with extrahepatic biliary atresia (EHBA), but optimal surgical management remains controversial. We have studied 28 infants born between June 1981, and April 1988, who underwent Kasai's portoenterostomy as primary surgical treatment. Those with evidence of subsequent hepatic decompensation were evaluated for liver transplantation (LT). All were cared for by surgeons who perform both the Kasai portoenterostomy and LT. Following the Kasai operation, 16 of 28 patients (57.1%) have achieved total biliary drainage, while 5 of 28 (17.9%) achieved partial drainage, and 7 of 28 (25%) achieved no drainage. Nine of 28 (32.1%) have undergone LT, 4 of whom were transplanted at greater than 2 years of age. To date, 25 of 28 (89.3%) are alive. Twenty three of 25 (92%) living are jaundice-free, 65% of whom have undergone the Kasai operation only. We project that 5 of 28 (17.8%) will come to transplantation, and 12 of 28 (43%) have no current indication that they are likely to come to transplantation. We conclude that combining Kasai's portoenterostomy with LT as needed is an effective therapy for children with EHBA, that the Kasai operation offers significant benefit by delaying LT in many, and that the long-term results of management remain to be determined.

Analysis of Variance

Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 38 cases.

A previously published survey has evaluated the natural history and clinical outcome of fetal diaphragmatic hernia (CDH) in 94 cases. This study showed that the prenatal diagnosis is accurate, the mortality is high (80%), and polyhydramnios is a prenatal predictor of poor clinical outcome. As a follow-up study, 38 consecutive cases of CDH diagnosed in utero were evaluated and treated by the same surgical team. This permitted detailed assessment of prognostic factors and evaluation of the impact of extracorporeal membrane oxygenation (ECMO) on outcome. We found the following. (1) Survival is poor despite optimal postnatal therapy including ECMO. (2) Polyhydramnios is both a common prenatal marker for CDH (present in 69% of fetuses) and a predictor for poor clinical outcome (only 18% survival), but tends to occur after the second trimester. (3) Amniocentesis is indicated to rule out chromosomal abnormalities that were present in 16% of fetuses. (4) All 14 fetuses diagnosed prior to 25 weeks' gestation died. Improved postnatal therapy or surgical intervention before birth will be necessary to salvage the CDH fetus with an early gestational diagnosis or associated polyhydramnios.

Extracorporeal Membrane Oxygenation

Extracorporeal membrane oxygenation and conventional medical therapy in neonates with persistent pulmonary hypertension of the newborn: a prospective randomized study.

Thirty-nine newborn infants with severe persistent pulmonary hypertension and respiratory failure who met criteria for 85% likelihood of dying were enrolled in a randomized trial in which extracorporeal membrane oxygenation (ECMO) therapy was compared with conventional medical therapy (CMT). In phase I, 4 of 10 babies in the CMT group died and 9 of 9 babies in the ECMO group survived. Randomization was halted after the fourth CMT death, as planned before initiating the study, and the next 20 babies were treated with ECMO (phase II). Of the 20, 19 survived. All three treatment groups (CMT and ECMO in phase I and ECMO, phase II) were comparable in severity of illness and mechanical ventilator support. The overall survival of ECMO-treated infants was 97% (28 of 29) compared with 60% (6 of 10) in the CMT group (P less than .05).

Extracorporeal Membrane Oxygenation

Role of extracorporeal membrane oxygenation in selected pediatric respiratory problems.

Between 1984 and 1988, 89 infants and children with severe respiratory failure were supported by extracorporeal membrane oxygenation. Major clinical diagnoses included congenital diaphragmatic hernias (34), meconium aspiration syndrome (26), and sepsis (8). Extracorporeal membrane oxygenation was used for patients with a predicted mortality rate of at least 80% based on an oxygenation index greater than 0.4. Venoarterial bypass was accomplished by way of right cervical cannulation of the common carotid artery and internal jugular vein. Overall survival was 71% but varied widely by diagnosis and progressively improved over time. The average extracorporeal membrane oxygenation run was 5.7 days. Intracranial hemorrhage was the most serious complication occurring in 16% of patients. Mechanical circuit complications were seen in 22% but rarely related to significant morbidity. Extracorporeal membrane oxygenation appears to provide effective cardiopulmonary support for selected pediatric respiratory problems. It affords those with potentially reversible pathophysiology the temporal opportunity for successful medical or surgical therapies.

Cerebral Hemorrhage