Destructive gouty arthritis of the hip

We present a case of a 19-year old female with systemic pulmonary artery (PA) pressure due to a congenital ventricular septal defect (VSD) and atrial septal defect (ASD). She was pink at rest and cyanotic on exercise. Lung biopsy revealed grade IV pulmonary vascular changes. As a preliminary step PA was banded to increase right-to-left shunt and decrease aortic (Ao) saturation with consequent decrease in PA saturation. After one year, when she was no longer cyanotic, even on exercise, lung biopsy revealed total regression of pulmonary vascular changes. As a definitive procedure VSD and ASD were closed and PA was debanded. Cardiac catheterization one week postoperatively showed PA pressure to be 50% of systemic pressure. We postulate that reversal of pulmonary vascular changes were due to lowered PA saturation. We further believe that lower PA pressure could have contributed to this regression of pulmonary vascular changes. We performed the same procedure in six more patients with similar positive clinical response. This new concept brings renewed hope to many children who otherwise are candidates for heart lung transplantation.     

Assessment of the role and reliability of sonographic post-prandial flow response in grading Crohn''s disease activity

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.     

Acute, life-threatening complications of lung transplantation

Acute, life-threatening complications of lung transplantation are common in all reported series. The clinical courses and images of 70 patients who underwent heart-lung (n = 5), bilateral sequential lung (n = 31), or single-lung (n = 38) transplantation were retrospectively reviewed. Sixty-five acute, life-threatening complications occurred in 26 patients (37%) within 3 months after transplantation. Nine deaths occurred as a result of these complications for a mortality rate due to acute complications of 13%. The deaths were a result of bleeding (n = 4), sepsis (n = 2), severe acute rejection and adult respiratory distress syndrome (n = 1), multiorgan failure (n = 1), and diffuse alveolar damage and respiratory failure (n = 1), a distribution of causes similar to those in other reported series. Specific diagnoses that can be made with imaging include hemothorax, lung torsion, pneumomediastinum, pulmonary embolism, pneumothorax, bronchial anastomotic dehiscence, lung collapse, paralysis of the diaphragm, and sternal dehiscence.     

1990-1996: the experience of the La Fe Lung Transplant Group (Valencia)

Objective to review the experience of the lung transplantation unit at Hospital La Fe (Valencia). Between February 1990 and March 1996 we performed 40 lung transplants. The following causes were most common: cystic fibrosis (9 cases), emphysema (8), pulmonary fibrosis (8) and bronchiectasis (7). Types of intervention were 27 double lung transplants (25 sequential and 9 blocked), 9 single lung transplants, and 4 heart-lung transplants. We then reviewed the 36 single and double lung transplants. The main exclusion criteria were age over 65 years, malignant disease, kidney or liver disease, severe or non reversible central nervous system disease, and drug addiction. Prior surgery, mechanical ventilation and the presence of Aspergillus were considered lower-order contraindications. Mean patient age was 37.7 years (14-59). Six patients were colonized by Aspergillus before transplantation. Five had undergone earlier surgery and two were mechanically ventilated before the transplant. The most common complication was respiratory infection, which was present in 6 of the 7 patients who died. Other complications in order of frequency were dehiscence and/or bronchial stenosis, corticoid myopathy and postoperative bleeding. The actuarial survival rate of single and double lung transplants was 67.85 after 3 years, and 87.5% in patients with cystic fibrosis. Lung transplantation is a well-established procedure that is gradually being extended to treat more conditions. The main obstacle is the scarcity of donors. The main challenge at present is bronchiolitis obliterans.     

Pulmonary interstitial fibrosis and haemosiderin-laden macrophages: late following heart transplantation

Impairment of pulmonary diffusion is recognized following heart transplantation. This study was undertaken to determine the histopathological basis for the defect in pulmonary physiology. Heart transplant recipients (HTR) entered into a prospective study of post-transplant pulmonary physiology were asked to undergo bronchoscopy, bronchoalveolar lavage (BAL) and transbronchial biopsy (n = 18) in the presence of impaired gas transfer. Transbronchial biopsies were examined under light microscopy and demonstrated focal interstitial fibrosis in 12 patients, cytomegalovirus disease in four patients and Pneumocystis carinii pneumonia in three patients. Bronchoalveolar lavage differential counts were normal in HTR but BAL macrophages contained haemosiderin. The histological features of interstitial fibrosis may underlie the fall in gas transfer seen following heart transplantation. The presence of haemosiderin-laden macrophages late following heart transplantation suggests a capillary leak syndrome.     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure) in infants and children with congenitally corrected transposition of the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitally corrected transposition underwent surgery at UCSF. Anatomical repair was performed in 11 of these patients, at ages ranging from 4.8 months to 7.8 years (median 3.2 years). The remaining six patients did not undergo anatomical repair due to unfavourable anatomy (n = 2), prior conduit repair (n = 2), biventricular dysfunction (n = 1), and isolated complete atrioventricular block (n = 1). The 11 patients who underwent anatomical repair make up the study group for the present report. All 11 patients had a malalignment ventricular septal defect, while pulmonary outflow tract obstruction was present in nine patients and significant tricuspid valve pathology or dysfunction was present in five. Anatomical repair was achieved with a Senning (n = 7) or a Mustard (n = 4) procedure combined with an arterial switch operation plus ventricular septal defect closure (n = 4), or a Rastelli procedure with left ventricle to aortic baffle and right ventricle to pulmonary artery conduit (n = 7). There was one early death and no patients developed surgical complete atrioventricular block. At a median follow-up of 22 months, there were no late deaths. Two patients required a total of three late reoperations, and all patients were asymptomatic on no cardiac medication. Follow-up echocardiography revealed normal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achieved with low rates of early mortality and surgical heart block, and favourable mid-term results. Long-term follow-up will be necessary to determine if the double switch approach improves the natural history of corrected transposition when compared to less aggressive surgical approaches that leave the right ventricle in the systemic circulation.     

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.     

Biodegradation of phthalic acid esters in river water and activated sludge

Infants born in New England with congenital heart defects were examined for association with time of year or population density. Moderate seasonal peaks in births were noted for complex ventricular septal defect, malposition defects, and transposition of the great arteries. Positive associations with population density were found for pulmonary atresia with ventricular septal defect, ventricular septal defect with secondary anomalies, and tricuspid atresia.     

Bronchiolitis obliterans syndrome: thin-section CT diagnosis of obstructive changes in infants and young children after lung transplantation

PURPOSE: To characterize the thin-section computed tomographic (CT) appearance of bronchiolitis fibrosa obliterans syndrome in infants and young children after lung transplantation. MATERIALS AND METHODS: Thin-section CT studies in six patients with bronchiolitis obliterans syndrome (age range, 2 months to 5 1/2 years) and in 15 control patients without obstructive airway disease (age range, 2 months to 7 years) who underwent bilateral lung transplantation were retrospectively reviewed. The thin-section CT scans were obtained during quiet sleep at a median of 24 months (range, 6-36 months) after transplantation. The CT studies were evaluated for mosaic perfusion, bronchial dilatation, bronchial wall thickening, and mucous plugging Final diagnoses in all patients were based pulmonary function test results. RESULTS: Thin-section CT findings in the six patients with clinically proved bronchiolitis obliterans syndrome were mosaic perfusion in five (83%) bronchial dilation in three (50%), and bronchial wall thickening in one (17%). Of the 15 control patients with normal pulmonary function test results, six (40%) had mosaic perfusion; none had bronchial dilatation or bronchial wall thickening. Mucous plugging was not seen in either group. Only the association of bronchial dilatation with bronchiolitis obliterans syndrome was significant (P = .02). CONCLUSION: Infants and young children with bronchiolitis obliterans syndrome after lung transplantation are more likely to have CT abnormalities than those with normal pulmonary function test results.     

Hypertrophic obstructive cardiomyopathy in pediatric patients: results of surgical treatment

Between April 1975 and May 1995, 25 pediatric patients on one hospital service underwent extended left ventricular septal myectomy because of hypertrophic obstructive cardiomyopathy. Ages ranged from 2 months to 20 years (mean, 11.2 years). Seventeen patients had moderate to severe mitral valve insufficiency. Medical therapy had failed in all patients and one patient had undergone dual-chamber pacemaker implantation without improvement. Left ventricular outflow tract gradients ranged from 50 to 154 mm Hg (mean, 99.9 +/- 25.2). Concomitant cardiac procedures included mitral valve repair (n = 2), automatic implantable cardioverter defibrillator implantation (n = 1), and closure of atrial septal defect (n = 1). Intraoperative premyectomy left ventricular outflow tract gradients ranged from 20 to 117 mm Hg (mean, 60.4 + 26.2) and postmyectomy gradients ranged from 0 to 20 mm Hg (mean, 6.6 +/- 5.9). Postmyectomy mitral insufficiency was reduced to a regurgitant fraction of 0% to 12%, and no patient required mitral valve replacement. One patient required a pacemaker because of complete heart block; on subsequent follow-up, normal sinus rhythm had returned. There was no early mortality and no instance of aortic or mitral valve injury or ventricular septal defect. Follow-up ranged from 10 months to 20 years (mean, 6.4 years). There were no late deaths. Left ventricular outflow tract gradients by echocardiography were a mean of 14.2 mm Hg with a median of 5.0 mm Hg. All patients had normal sinus rhythm. Reoperation because of recurrent left ventricular outflow tract obstruction was necessary in two patients at 3.2 years and 12.4 years after initial myectomy, respectively. All patients but one have New York Heart Association class I or II function. We conclude that extended septal myectomy is a safe and effective means of relieving cardiac symptoms and left ventricular outflow tract obstruction in pediatric patients with severe hypertrophic obstructive cardiomyopathy unresponsive to medical management, and late survivorship compares favorably with the natural history of the disease.     

Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure

BACKGROUND: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one (n = 5) or two (n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular-aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right (n = 6) or the left (n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. RESULTS: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart. Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. CONCLUSION: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).     

Localization of the somatostatin sst2(a) receptor in human cerebral cortex, hippocampus and cerebellum

AIMS: To quantify birth prevalence and spectrum of congenital heart disease in the Island population of Malta, and compare these rates with previous studies. METHODS: All patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post-mortem by 1 year of age between 1990-1994 were included. There were 231 cases of live born congenital heart disease with a birth prevalence of 8.8/1000 live births. The commonest lesions were ventricular septal defect, pulmonary stenosis and tetralogy of Fallot. The rates of individual lesions were compared with two recent epidemiological studies with similar methodologies. RESULTS: Although the overall birth prevalence of congenital heart disease was similar in three studies, significantly higher rates of pulmonary stenosis, tetralogy of Fallot and double outlet right ventricle were found in Malta, all of which predispose to right ventricular outflow tract obstruction. In contrast, there were lower rates of lesions causing left ventricular outflow tract obstruction. A higher rate of ventricular septal defect was also found. The Maltese gene pool may contain an inherent predisposition towards lesions causing right ventricular outflow tract obstruction.     

Transcatheter closure of atrial septal defect

Isolated secundum atrial septal defect is one of the most common congenital heart defects. Surgical closure is the treatment of choice but is associated with a chest scar, some morbidity and a relatively long recovery and the use of cardiopulmonary bypass. Transcatheter closure of secundum atrial septal defect is therefore an attractive approach. 3 children, aged 5-10 years, underwent successful transcatheter closure of moderate to large central atrial septal defects with the Cardioseal device. The procedures were performed under x-ray and transesophageal echocardiographic guidance. Our initial experience, and that of others, indicates that transcatheter occlusion of secundum atrial septal defects is safe and effective and can be an appropriate alternative in approximately 60% of patients.     

Obliterative bronchiolitis after lung and heart-lung transplantation

Obliterative bronchiolitis (OB) has emerged as the main cause of morbidity and mortality in the long-term follow-up after lung and heart-lung transplantation. The pathogenesis of OB is multifactorial, with acute rejection and cytomegalovirus infection being the main risk factors for the development of OB. The final common pathway of all inciting events seems to be an alloimmune injury, with subsequent release of immunologic mediators and production of growth factors leading to luminal obliteration and fibrous scarring of the small airways. Analyzing the 14 years of experience in 163 patients at Stanford University, we found a current incidence of bronchiolitis obliterans syndrome or histologically proven OB within the first 3 years after lung and heart-lung transplantation of 36.3%, with an overall prevalence of 58.1% after heart-lung and 51.4% after lung transplantation. Both pulmonary function indices (forced expiratory flow between 25% and 75% of forced vital capacity and forced expiratory volume in 1 second) and transbronchial biopsies have proven helpful in diagnosing bronchiolitis obliterans syndrome or OB at an early stage. Early diagnosis of OB and improved management have achieved survival rates in patients with OB after 1, 3, 5, and 10 years of 83%, 66%, 46%, and 22%, compared with 86%, 83%, 67%, and 67% in patients without OB. Recently, different experimental models have been developed to investigate the cellular and molecular events leading to OB and to evaluate new treatment strategies for this complication, which currently limits the long-term success of heart-lung and lung transplantation.     

Transcatheter closure of a residual postmyocardial infarction ventricular septal defect with the Amplatzer septal occluder

Acute ventricular septal rupture following myocardial infarction carries a high mortality. Early surgery improves survival but long term outcome depends on residual shunting and left ventricular function. Residual shunting is common despite apparently successful closure and may require reoperation. Transcatheter closure is an established method of treating selected congenital defects but clinical experience of transcatheter closure in postinfarction ventricular septal rupture is minimal. Transcatheter closure of a residual ventricular septal defect was successfully done using a new device, the Amplatzer septal occluder, in a 50 year old Indian man who had previously undergone emergency surgical repair for postinfarction acute ventricular septal rupture. The technique is described and its potential as a treatment in postinfarction ventricular septal rupture, its possible complications, and the important aspects of case selection and device design are discussed.     

Utility of psychophysiological measurement in the diagnosis of posttraumatic stress disorder: results from a Department of Veterans Affairs Cooperative Study

OBJECTIVE: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. METHODS: Since January 1994, 15 consecutive patients (aged 64 +/- 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm(2)/m(2) underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm(2)/m(2) underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. RESULTS: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P=.006) within 22 +/- 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 +/- 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 +/- 12 months), all patients are free of symptoms, requiring no medications. CONCLUSIONS: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results.     

Afterload mismatch-related problems after "domino" heart transplantation

The heart transplants with domino technique, which uses donor hearts from heart-lung recipients, increases the pool of donors, provides the advantage of shortening the ischemic time and makes suitable hearts for patients with pulmonary hypertension. The present study aimed to characterise the pre- and post-transplant clinical and hemodynamic profiles of patients that underwent domino transplant in Pavia. METHODS: Between 1991 and 1992, 9 heart transplants were performed with the domino procedure at I.R.C.C.S. Policlinico S. Matteo of Pavia. Domino donors (6 with primary pulmonary hypertension, 2 with Eisenmenger's syndrome due to atrial septal defect, 1 with cystic fibrosis) underwent electrocardiographic, echocardiographic, chest roentgenogram studies, and right heart catheterization and coronary angiography (for donor older than 40). Domino recipients, 6 males and 3 females with a mean age of 44 years, had dilated cardiomyopathy (4 cases), coronary artery disease (4 cases) and valvular heart disease (1 case) (group 1). Seven of the 9 cases entered the study; 2 were excluded: one because had undergone heterotopic transplantation, the other had received the heart from another country and therefore the graft had suffered from a very long ischemic time. Controls group consisted of 12 patients who had consecutively undergone cardiac transplantation with non-domino donors during the same period (group 2). Immunosuppression was similar in both groups, and consisted of a combination of cyclosporin A, azathioprine and corticosteroids, plus a 7-day-course of antithymocyte globulin. Hemodynamic and echocardiographic controls were performed at 2, 3 and 4 weeks (short-term control) and at 2 and 6 months (mid-term control) after surgery. RESULTS: Domino donors (39 +/- 12.5 years) had significantly higher mean right ventricular end-diastolic diameter and lower left ventricular diameter than normal mean values. Domino recipients had significantly higher mean pulmonary arteriolar resistances than controls; mean ischemic time was also significantly lower in group 1 than in group 2. Short- and mid-term controls after surgery in group 1 showed persistently higher systemic vascular resistances and pulmonary vascular resistances and lower cardiac output than in group 1. Two patients developed an early and unexpected increase in pulmonary wedge pressure accompanied by severe paroxysmal nocturnal dyspnea and mitral regurgitation. In all cases, the left ventricles were relatively inadequate; the combination of low cardiac output and of high systemic vascular resistances favoured the occurrence of an afterload mismatch condition that was worsened by chronic hypoxia. This condition must be known and expected in these patients after transplantation in order to provide timely and effective treatment to potentially life-threatening left ventricular failure episodes. IN CONCLUSION, the subset of transplanted patients that receives domino donors may develop left-side afterload mismatch which, combined with low cardiac output, with high systemic vascular resistances and with the effects of chronic hypoxia originally suffered by the heart, may cause sudden and unexpected left-side heart failure which has to be timely recognised and managed. Although hemodynamic adaptation of this patients is highly problematic, it does not limit the value of the domino procedure.     

Lung transplantation in children

The first lung transplantation in the Netherlands was carried out in 1990. Since, the operation has been performed in over one hundred adults and two children (up to 16 years) with chronic respiratory insufficiency. Most lung transplantations in children are performed because of cystic fibrosis. After referral, the patient is given information about the operation and the pre- and postoperative periods, and the transplantation team advises on the time of screening. Some one-third of the children referred ultimately undergo transplantation; the others refrain from the operation, are rejected or die during the waiting period. Owing to a shortage of donor lungs the waiting period for transplantation may last over one year. A possible but controversial solution is donation of a pulmonary lobe by a close relative. Undergoing lung transplantation places a heavy (psychic) burden on the patient and his parents. Every patient after lung transplantation goes through an average of two or three periods of acute rejection. Chronic rejection occurs in 20-40%. Other complications are connected to medication (infections, renal dysfunction) or to the underlying disease (diabetes mellitus in cystic fibrosis). The percentages of survival for 1, 2 and 4 years are approx. 60-70, 55-60 and 30-50, dependent in part on the type of lung transplantation (unilateral, bilateral or combined heart-lung transplantation). A reasonable condition of the patient owing to timely referral is a positive prognostic factor.     

Lung transplantation for lymphangioleiomyomatosis

BACKGROUND: Lymphangioleiomyomatosis is a rare disease of unknown origin that usually leads to progressive deterioration of lung function and eventual death from respiratory failure. It occurs in women of reproductive age and people with tuberous sclerosis. Lung transplantation is a recent therapeutic approach. METHODS: We conducted a retrospective study by questionnaire of 34 patients, treated at 16 transplantation centers, who underwent lung transplantation for end-stage lymphangioleiomyomatosis between 1983 and 1995. RESULTS: Of the 34 patients, 27 received single-lung transplants; 6, bilateral transplants; and 1, a heart-lung transplant. As of August 31, 1995, the actuarial survival calculated by the Kaplan-Meier method was 69 percent after one year and 58 percent after two years. Eighteen patients were alive 33 +/- 20 months (range, 3 to 74) after transplantation. Forced expiratory volume in one second increased from 24 +/- 12 percent of the predicted value before transplantation to 48 +/- 16 percent six months after transplantation. Five early deaths (within one month) were due to hemorrhage (in one patient), acute lung injury (in three), and dehiscence of the bronchial anastomosis (in one). Eleven late deaths (after one month) were due to infections (in eight patients), bronchiolitis obliterans (in two), and metastatic nephroblastoma (in one). Disease-associated problems were extensive pleural adhesions in 18 patients, leading to moderate-to-severe intraoperative hemorrhage in 4; pneumothorax in the native lung after single-lung transplantation in 6 patients; postoperative chylothorax in 3; and recurrent lymphangioleiomyomatosis in the allograft in 1 patient, who died of disseminated aspergillosis. CONCLUSIONS: Although disease-related complications are frequent, lung transplantation can be a valuable therapy for patients with end-stage lymphangioleiomyomatosis.     

Mechanical circulatory support for post cardiotomy cardiogenic shock in infants

Eleven infants weighing 2.3 to 7.8 kg underwent mechanical circulatory support for post cardiotomy cardiogenic shock. Initiated pre-operatively in two patients, extracorporeal membrane oxygenation was used in a total of eight patients aged 6 days to 3 months in association with repair of cyanotic congenital heart disease with increased pulmonary blood flow or with a right sided obstructive lesion. Ventricular assist devices were used in three other patients: a centrifugal left ventricular assist device in Patient 1 (10 months, 5.7 kg) after repair of the anomalous left coronary artery, and a pneumatic biventricular assist device (stroke volume 12 ml) in Patient 2 (6 months, 7.0 kg) for cardiac arrest after closure of ventricular septal defect and in Patient 3 (10 months, 7.8 kg) for post transplant graft failure. Duration of extracorporeal membrane oxygenation duration ranged from 26 to 192 hr (mean, 88 hr). Three patients were weaned from extracorporeal membrane oxygenation and two survived. Two others were separated from extracorporeal membrane oxygenation because of bleeding, but both subsequently died. Patient 1 was weaned from the left ventricular assist device after 192 hr and discharged from the hospital. Support was discontinued after 45 hr in Patient 2 who exhibited irreversible brain damage. Patient 3 was weaned from a biventricular assist device after 174 hr, but suffered recurrent graft failure. Our results show that an appropriate circulatory support system should be selected according to the cardiac anatomy in infants.