Anomalous left pulmonary artery: successful management.
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
F J Fricker
Publications and source records attributed to F J Fricker.
Pneumonectomy for intractable left bronchial compression in d-transposition of the great arteries.
In infants with transposition of the great arteries (TGA) tracheobronchial obstruction may occur as a result of compression by a dilated posterior pulmonary artery, especially if there is a large interventricular septal defect with its attendant large pulmonary blood flow and high pressure. Banding of the pulmonary artery may give temporary relief. A patient with TGA had collapse of the left lung at 2 months of age, following atrial septectomy. Bronchoscopy and bronchography revealed extrinsic compression of the left main bronchus. Banding of the pulmonary artery at 6 months of age relieved the respiratory distress. At 8 years of age atelectasis of the left lung recurred following a Mustard procedure. Two additional procedures were attempted to decrease the pressure of the pulmonary artery on the bronchus but these were unsuccessful. At age 9 years, pneumonectomy, her sixth thoracotomy, was performed as a final attempt to relieve the respiratory problem caused by persistent atelectasis. She is now 17 years of age and has remained asymptomatic during the last 8 years.
Adolescent↗
Echocardiographic and hemodynamic correlation in transposition of the great arteries.
Echocardiography was performed in 36 patients with transposition of the great arteries (TGA). Twenty patients were studied before a Mustard operation, 14 patients after operation and two patients both before and after operation. Right ventricular end-diastolic dimension (RVED) was larger than normal in each patient and tended to increase postoperatively. In contrast, the left ventricular end-diastolic dimension (LVED) was significantly reduced postoperatively in all patients. A linear relationship was demonstrated between the ratio of LVED/RVED and the ratio of peak systolic pressures in the left and right ventricles in studies both before and after Mustard operation. Systolic anterior motion of the mitral valve was observed in 18% of peroperative patients and increased to 44% postoperatively. The incidence of fluttering of the mitral valve increased from 50% to 94% after the operation. Abnormal septal motion was found in 39% of cases. Abnormal movement of the mitral valve and of the interventricular septum seems to be related to a reversed pressure relationship in the ventricles. Shifting of the ventricular septum toward the left ventricle and consequent distortion of the left ventricular cavity and mitral valve apparatus may be responsible for the abnormal echocardiographic findings. Serial echocardiographic studies may be useful as a noninvasive tool in the assessment of left ventricular pressure or the status of the pulmonary vascular bed in TGA.
Adolescent↗
Clinical use of blade atrial septostomy.
A cardiac catheter enclosing an extensible blade was used to enlarge the interatrial opening in seven patients. Two patients with transposition of the great arteries who had balloon atrial septostomy as newborns subsequently presented with clinical evidence of a restrictive interatrial opening at 1 and 4 months of age. Cardiac catheterization confirmed restenosis of the interatrial opening and inadequate intracardiac mixing. After blade atrial septostomy the systemic arterial oxygen saturation increased by 20% and 30%, respectively. Five patients with mitral atresia complex, ages 2 months-9 1/2 years, had a restrictive interatrial communication and severe pulmonary venous hypertension (mean left atrial pressures ranged from 20-38 mm Hg). Following blade atrial septostomy, the pressure gradient between the atria was almost completely abolished and prompt clinical improvement was observed in each patient. All patients tolerated the procedure without complications. Blade atrial septostomy was a safe, effective procedure for enlarging the interatrial communication in this limited series of patients with an interatrial septum too thick to permit adequate rupture by conventional balloon atrial septostomy.
Age Factors↗
Bilateral ductus arteriosus in d-transposition of the great arteries with right aortic arch.
The rare anomaly of a right aortic arch, distal origin of the left subclavian artery, and posterior left ductus arteriosus (forming a vascular ring) plus a right ductus arteriosus is described in an infant with d-transposition of the great arteries with an intact septum. The presence of a right aortic arch and distal left subclavian artery with bilateral ductus arteriosus has not been described previously.
Aorta, Thoracic↗
Clinical implications of postoperative unilateral phrenic nerve paralysis.
Unilateral phrenic nerve paralysis (PNP) folowed 32 (1.7 percent) of 1,891 consecutive cardiac surgical procedures during an 8 year peroid. Diagnosis was based on radiographic criteria with comparison of preoperative and postoperative chest radiographs and was confirmed in all 21 evaluated by fluoroscopy. Six had persistent radiographic abnormality more than 12 months postoperatively. PNP occurred most frequently in association with Blalock-Taussig shunts. These operations represented 22 percent of this series, and PNP complicated 7 percent of all Blalock-Taussig shunts. PNP was less well tolerated in the 14 infants than in the 18 older children. Eleven infants had serious difficulties during weaning from mechanical ventilatory support. Five infants required tracheostomy, one underwent diaphragmatic plication, and three died. Infants had a mean duration of mechanical ventilation of 24 days and required prolonged intensive care and long-term hospitalization. In comparison, older children had a more benign postoperative course. Diaphragmatic plication should be considered in infants with paradoxical motion of the hemidiaphragm who remain dependent on mechanical ventilatory support for more than 2 weeks postoperatively.
Adolescent↗
Valve replacement in children and adolescents.
Replacement of diseased heart valves has become the treatment of choice in many adults. Valve replacement in older children and adolescents has been done in our institution only when other procedures are ineffective. Over the past 10 years, 24 patients have received 28 artificial valves. Thirteen (54 per cent) had rheumatic heart disease; the remainder had congenital or acquired valvular abnormalities. The Björk-Shiley valve was most commonly used in the aortic position and the Beall prosthesis in the mitral position. Sixteen patients (67 per cent) survived operation and have been followed from 3 to 79 months, averaging 27 months. All are free of cardiac symptoms. Hemarthrosis occurred once in 2 survivors (13 per cent). Eight patients died (33 per cent), 5 immediately postoperatively and 3 within a year after the operation. The mortality rate has declined to 21 percent during the past 3 years. Our experience in the pediatric age group suggests that valve replacement is a serious undertaking with higher mortality rate than in adults. The operation should be reserved for those patients in whom valvuloplasty or valvulotomy is expected to be ineffective.
Adolescent↗
Unilateral pulmonary vein atresia: clinical and radiographic spectrum.
Three cases of unilateral pulmonary vein atresia are presented to illustrate part of the clinical and radiographic spectrum of this anomaly. One patient had major associated cardiovascular abnormalities and pulmonary hypertension, one had recurrent hemoptysis, and the other patient was asymptomatic with normal pulmonary artery pressure and no associated abnormalities. In one case, serial catheterizations indicated that the pulmonary vein atresia was acquired. This report stresses the radiographic, hemodynamic, and angiographic findings in patients with unilateral severe pulmonary venous obstruction. The embryology, pathology, and surgical approach are also discussed. The diagnosis should be strongly suspected before cardiac catheterization when typical features are present on the chest radiograph and isotopic ventilation perfusion scan.
Abnormalities, Multiple↗
Pilot study of cardiac function after treatment of childhood Wilms' tumor with doxorubicin.
To assess the cardiotoxicity of moderate doses of anthracyclines and to control for possible contributions of other drugs, cardiac function in 12 patients 1 year 11 months to 9 years 4 months off treatment for Wilms' tumor whose therapy had included vincristine, dactinomycin, and the anthracycline doxorubicin (270 to 300 mg/m2 cumulative dose) was compared with that of 9 patients (controls) whose therapy had included vincristine and dactinomycin but not doxorubicin. The small numbers of patients studied suggest that a a medium of 6 years off therapy only small numbers of patients will have cardiac function values below age-related normal values. To confirm these results and to determine whether abnormalities are progressive or are clinically important, large cohorts of patients such as those available to the National Wilms' Tumor Study need to be studied.
Child↗
Posttransplant lymphoproliferative disease in thoracic organ transplant patients: ten years of cyclosporine-based immunosuppression.
Lymphoproliferative disease developed in 15 heart and five lung transplant recipients during a decade of heart and lung transplantation from 1980 through 1989. The overall incidence of posttransplant lymphoproliferative disease in patients who survived more than 30 days is 4%. The incidence after heart transplantation is 3.4% and after lung transplantation is 7.9% (p = 0.08). The peak occurrence of posttransplant lymphoproliferative disease is 3 to 4 months after transplantation. However, posttransplant lymphoproliferative disease occurring early versus late (defined as before or after 1 year after transplantation) appears to have different clinical outcomes. The mortality of early onset of posttransplant lymphoproliferative disease as a result of lymphoma is 36%; response to reduction in immunotherapy occurs in 89% and presentation with disseminated disease occurs in 23%. The mortality of late onset of posttransplant lymphoproliferative disease as a result of lymphoma is 70%; no patient responded to reduction in immunotherapy and presentation with disseminated disease occurs in 86% of patients. Epstein-Barr virus primary infection was present in 14 and secondary Epstein-Barr virus infection was present in three of the 20 patients with posttransplant lymphoproliferative disease. The other three patients were positive for Epstein-Barr virus also but had no pretransplant sera for comparison. There is no correlation with immunoprophylaxis or maintenance immunosuppression and the development of posttransplant lymphoproliferative disease in our series.
Cyclosporine↗
Session VII: Physiologic and psychological growth and development in pediatric heart transplant recipients.
Explore the source record for details and available documents.
Adolescent↗
Heart transplantation in patients with malignant disease.
We have traditionally pushed the limits of conservative candidate criteria for heart transplantation. We have been gratified by our results in the aged, the diabetic, and the mortally ill. Our inclusion of patients with malignant disease underscores our philosophy to include patients as candidates for transplantation for whom the procedure has reasonable expectation of success. We report here our early results of heart transplantation in 11 patients with malignant disease. Our survival rate in this group is 100%, and all patients are leading active lives with no evidence of recurrent or metastatic tumor. Immunosuppression protocols were adjusted on an individual basis determined by the chemotherapy dosage, duration, and relation to transplantation. Whenever possible a 1-year disease-free interval after completion of adequate cancer therapy is desired before transplantation.
Adult↗
Pediatric heart transplantation: quality of life.
Staff members at the University of Pittsburgh have performed pediatric heart transplantations since 1982. As heart transplantation becomes an accepted therapeutic treatment, more children undergo surgery and return home. Assessment of the quality of life after transplantation of these children is essential. This article describes the life-styles of seven surviving transplant patients. The children lived for periods ranging from 3 months to 3 years. Patients were evaluated psychosocially by the Personality Inventory for Children, Offer Self-Image Questionnaire, Eyberg Child Behavior Inventory, and projective drawings. Daily routines and school achievement were assessed by interviews with the parents. Cardiovascular function was measured by exercise testing. When possible, comparisons were made with the patient's preoperative status. Heart transplantation assaults the body, both physically and emotionally. This study concludes that the surviving children can adapt to the experience. Heart transplantation enables them to return to a level of functioning that is appropriate for age. However, speculation is made whether the benefits gained for a child who undergoes transplantation can be sustained long enough to warrant such extensive treatment.
Adolescent↗
Pediatric lung transplantation: expanding indications, 1985 to 1993.
The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplant program, which began in 1982. Thirty-two pediatric patients (age range 1 to 18 years) have undergone heart-lung (n = 16), double-lung (n = 14), and single-lung (n = 2) transplantation procedures. The cause of end-stage lung disease was primary pulmonary hypertension (n = 7), congenital heart disease (n = 7), cystic fibrosis (n = 9), pulmonary arteriovenous malformation (n = 2), desquamative interstitial pneumonitis (n = 2), graft-versus-host disease (n = 1), emphysema (n = 1), rheumatoid lung (n = 1), cardiomyopathy (n = 1), and Proteus syndrome (n = 1). Six patients (19%) underwent pretransplantation thoracic surgical procedures. The survival rate was 78% at a mean follow-up of 1.8 years. The survival rate in the 23 recipients without cystic fibrosis was 87% (95% since 1985). The actuarial 1-year survival rate in the nine recipients with cystic fibrosis was 55%. Immunosuppression was cyclosporine (n = 9) or FK 506 (n = 23)-based therapy with azathioprine and steroids. Children were followed up by spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection per patient in the groups treated with cyclosporine and FK 506, respectively, was 1.0 and 1.2 at 30 days, 0.67 and 0.38 at 30 to 90 days, and 2.33 and 0.46 at greater than 90 days (p < 0.001, Fisher exact test).(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Pediatric retransplantation.
Previous studies have attempted to outline the efficacy of heart retransplantation in adults. A limited number of these retransplantation procedures have been performed in children; however, no study to date has evaluated the risk of heart retransplantation in this specific patient population. We conducted a retrospective review of 17 pediatric (non-neonatal) heart transplant recipients who subsequently underwent heart retransplantation. Thirteen male and four female patients underwent retransplantation at four different institutions between 1974 and 1992. Patient age at the time of primary transplantation ranged from 2 to 19 years (mean, 12.5 years) and from 3 to 30 years (mean, 16 years) at retransplantation. The time interval between transplantation procedures ranged from 2 days to 15.6 years and was not predictive of patient outcome. Follow-up after retransplantation ranged from 1 day to 11 years (mean, 2.8 years). All patients were New York Heart Association class 3 or class 4 at the time of retransplantation. After retransplantation all survivors were New York Heart Association class 1. No patient had mechanical device implantation or extracorporeal membrane oxygenation bridge-to-transplantation at primary transplantation or retransplantation. The indication for retransplantation was transplant coronary artery disease (TxCAD) in seven patients (41.2%), acute rejection in four (23.5%), chronic rejection in one (5.8%), chronic rejection associated with TxCAD in four (23.5%), and intraoperative donor organ failure in one (5.8%). No significant difference occurred in linearized rates of rejection or infection or in actuarial freedom from rejection or infection when secondary grafts were compared with primary grafts in patients undergoing retransplantation.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Occult restrictive hemodynamics after pediatric heart transplantation.
BACKGROUND: Although resting hemodynamics after pediatric heart transplantation are generally within normal limits, we hypothesized that occult restrictive hemodynamics suggesting diastolic dysfunction may be unmasked by acute volume loading (fluid challenge) during cardiac catheterization. We wished to determine the incidence of diastolic dysfunction and to assess whether it progressed over time. METHODS: From 1988 through 1993, a total of 100 fluid challenges were performed at the time of surveillance endomyocardial biopsy in 31 survivors of orthotopic heart transplantation. Cyclosporine-based immunosuppression was used in 16 patients, and FK506 was used in 15 patients. Right heart hemodynamics and cardiac output (thermodilution) were obtained at baseline and after a fluid challenge with 10 ml/kg of normal saline solution. The data were analyzed to determine whether type of immunosuppression or time elapsed since transplantation predicted the response to fluid challenge. RESULTS: Baseline hemodynamics were normal; however, a marked increase in atrial filling pressures occurred after fluid challenge (p < 0.001). Findings were similar in cyclosporine- and FK506-treated patients. Hemodynamic response to fluid challenge was not related to duration of time since transplantation, including studies on patients surviving more than 4 years. CONCLUSIONS: Diastolic dysfunction after heart transplantation is common; however, the abnormalities do not progress in severity, suggesting stable long-term graft function.
Adolescent↗