Cognitive and behavioral functioning of children listed for heart and/or lung transplantation.

The objective of the study was to assess cognitive function and behavior in a group of children listed for heart and/or lung transplantation. Ninety-nine children (3.7-16.8 years) listed for heart and/or lung transplant for congenital heart disease (CHD), cardiomyopathy (CM), cystic fibrosis (CF) or primary pulmonary hypertension (PPH) were evaluated with standardized measures of cognitive function, academic ability and behavior. Results were compared with a historical cohort of healthy children and comparisons were made between the four diagnostic subgroups. Mean subtest scores, overall IQ and achievement scores were within the normal range for the total group but were significantly lower than those of the healthy group. Although there were few significant differences between the diagnostic subgroups children with CHD or CF tended to perform at a lower level than those with CM or PPH. There were some significant issues with behavior for all diagnostic subgroups, particularly in the domain of social competence. Patients with end-stage heart or lung disease are at increased risk for cognitive and/or behavioral problems, particularly those with CHD. Follow-up of these patients after transplant is important to determine the role that psychological functioning prior to surgery has in the longer term after surgery.

Late donor cardiectomy after paediatric heterotopic cardiac transplantation.

BACKGROUND: Cardiac transplantation is a life-saving procedure in infants and children with advanced cardiomyopathy. However, it is greatly limited by shortage of paediatric donors and the complications of long-term immunosuppression, including post-transplant lymphoproliferative disorder (PTLD). We report the management of an infant who had heterotopic cardiac transplantation for advanced cardiomyopathy with secondary pulmonary hypertension who developed seemingly incurable PTLD. METHODS: An 8-month-old girl presented in 1994 with signs of severe heart failure, secondary to dilated cardiomyopathy. At age 11 months, the patient underwent a heterotopic cardiac transplantation. FINDINGS: The patient developed many episodes of PTLD associated with Epstein-Barr virus infection that were resistant to several therapies, including reduction of immunosuppression. Native heart recovery enabled removal of the donor heart 10.5 years after the original operation to allow complete cessation of immunosuppression. Her postoperative course was uncomplicated and the outcome was excellent. 3.5 years after surgery, the patient remains well, in complete remission from her PTLD, and has normal cardiac function. INTERPRETATION: This case shows several issues relating to the use of heterotopic cardiac transplantation in infants and the capacity of the heart to recover. It also provides new insights into the interaction between the immune system with several aspects of modern management of post-transplantation PTLD. FUNDING: None.

Lung biopsy findings in transposition of the great arteries with ventricular septal defect: potentially reversible pulmonary vascular disease is not always synonymous with operability.

Pulmonary vascular structure was analyzed in lung biopsy specimens taken from 28 children, aged 2 months to 15 years, with transposition of the great arteries and ventricular septal defect. Cellular intimal proliferation occurred in infants as young as 2 months, but it increased markedly between ages 7 to 9 and 10 to 12 months, and the increased obstruction was associated with a lower mean percent arterial medial thickness in patients older than 10 months than was found in younger patients. Early generalized arterial dilation appeared without the intimal fibrosis and dilation lesions characteristic of classical grade III and IV pulmonary vascular disease. Intimal abnormalities increased with age and pulmonary artery pressure, but mean percent arterial medial thickness was inversely related to mean pulmonary artery pressure (r = -0.5; p less than 0.0001). Nine patients survived intracardiac repair and six did not. Five of the patients who died were of similar age (12 months or less), had similar preoperative hemodynamic and pulmonary vascular abnormalities compared with the survivors and died after a clinical course compatible with pulmonary vascular disease. The findings emphasize that potential structural reversibility is not synonymous with "operability." Further studies are indicated on the function of the excessively muscularized pulmonary vascular bed of such infants.

Arrhythmias before and after anatomic correction of transposition of the great arteries.

Cardiac rhythm has been evaluated using 24 hour ambulatory electrocardiographic (ECG) recordings in 92 survivors of anatomic correction of transposition of the great arteries. A low incidence of arrhythmias was found in preoperative recordings made in 41 patients. Thirty-four (83%) patients showed sinus rhythm exclusively; one (2.4%) patient had supraventricular premature beats and one (2.4%) paroxysmal atrial fibrillation. Six patients (14.6%) had ventricular arrhythmias, five (12.2%) with modified Lown grade 1 ventricular premature beats and one (2.4%) with a modified Lown grade 5 arrhythmia. The incidence of arrhythmias was unchanged after a first stage operation consisting of banding of the pulmonary artery with or without insertion of a systemic to pulmonary shunt. During a mean 3.1 year follow-up, there have been no cases of arrhythmic death or symptomatic arrhythmias requiring treatment. Postoperative recordings in 40 patients have shown a low incidence of important arrhythmias with 62% having recordings free from arrhythmias. There was an increase in the incidence of infrequent supraventricular premature beats from 4.9% preoperatively to 23% postoperatively (p less than 0.05), with no significant change in the incidence of ventricular arrhythmias. One patient has acquired complete heart block but all others had sinus rhythm postoperatively. Preoperative arrhythmias are uncommon in patients with transposition of the great arteries and are similar to those described in normal children. The incidence of important arrhythmias after anatomic correction is low during the medium-term period of follow-up. Further longitudinal studies will be required to determine whether this promising result is maintained.

Influence of anatomic correction for transposition of the great arteries on myocardial perfusion: radionuclide imaging with technetium-99m 2-methoxy isobutyl isonitrile.

OBJECTIVES: We sought to determine the incidence of late perfusion defects attributable to coronary artery mobilization in patients undergoing anatomic correction for complete transposition of the great arteries. BACKGROUND: Anatomic correction (arterial switch procedure) is currently the surgical treatment of choice for complete transposition. From its conception, there has been concern about the impact on myocardial perfusion of the coronary artery mobilization and reimplantation involved in the correction. Previous studies have demonstrated myocardial perfusion defects in patients after correction, although a causal relation between coronary mobilization, and perfusion abnormality has not been established. METHODS: In a case-comparison study designed to test this hypothesis, 29 children underwent imaging with technetium-99m 2-methoxy isobutyl isonitrile (technetium-99m mibi). Ten had undergone anatomic correction (arterial switch group; interval from operation 6.9 +/- 1.42 years [range 4.9 to 9.1]); 9 had required noncoronary open heart surgery for other cardiac lesions (post-bypass group; interval from operation 5.6 +/- 3.6 years [range 1.0 to 13.25]); and 10 had had no surgical procedure (control group). The latter group comprised children with atrial or ventricular septal defects who required a radionuclide study for shunt calculation. Planar studies were performed in all 29 children, and additional tomographic acquisition was achieved in 25. To assess reversibility of perfusion defects both an exercise and a rest planar study were performed in the arterial switch group. RESULTS: Perfusion abnormalities were observed in seven of the nine children in the postbypass group and in all 10 children in the arterial switch group. The frequency of perfusion defects in these two groups was similar, with at least 25% of the tomographic segments reported being abnormal. The control group had significantly fewer defects than the other two groups (p = 0.02), with only 8% of the tomographic segments judged to be abnormal. In all except one patient in the arterial switch group, the segments reported as abnormal on the planar exercise study were either abnormal or equivocal on the rest study, indicating a fixed abnormality. CONCLUSIONS: Although the precise etiology of these perfusion abnormalities cannot be defined from this study, these data suggest that their origin is related more to the insult of open heart surgery itself than to the coronary manipulation involved in the arterial switch procedure. The functional importance requires further study.

A quantitative evaluation of aortic regurgitation after anatomic correction of transposition of the great arteries.

Twenty patients who had undergone anatomic correction of transposition of the great arteries were assessed by Doppler echocardiography or cardiac catheterization, or both, to identify the presence of aortic regurgitation. The severity of aortic regurgitation was evaluated by radionuclide angiographic measurement of the stroke volume index a mean of 47.1 months postoperatively. The stroke volume index was defined as the ratio of the stroke counts between the left and right ventricles. A value greater than 1.8 was considered to indicate significant left ventricular volume overload. Eight patients (40%) were shown to have various degrees of aortic regurgitation by Doppler echocardiography or cardiac catheterization, or both. The mean (+/- SD) stroke volume index was 1.03 +/- 0.15 in these patients and 1.01 +/- 0.21 in the 12 patients without aortic regurgitation (p = NS). The stroke volume index was not above the normal range in any patient, indicating that the degree of aortic regurgitation present was trivial. This medium-term study indicates that trivial or mild aortic regurgitation is a frequent finding after anatomic correction of transposition of the great arteries. However, it rarely results in an audible cardiac murmur or significant left ventricular volume overload. Long-term evaluation is required to determine its importance.

Returning to school after heart or heart-lung transplantation: how well do children adjust?

BACKGROUND: Up to 40% of children and adolescents with chronic illness experience school-related problems, including learning difficulties and problems in social adjustment and peer relationships. Despite the life-threatening nature of heart and heart-lung transplantation and the severity of illness, which results in the necessity for surgery, there is little information on the school performance of children after transplantation. METHODS: Eighty-one children and adolescents were assessed with regard to their academic attainments and behavior at school at regular intervals after heart (n=47) or heart-lung (n=34) transplantation and comparisons made with a group of healthy children. RESULTS: Cognitive ability and performance on academic attainments were within the normal range and did not change significantly as a function of time since transplant. However, performance was at a significantly lower level than that of the healthy children. Although the prevalence of behavior problems was only 8% at 6 months posttransplant, at 3 years, it had increased to 29% and, at 5 years, it was still 27%. Children with an initial diagnosis of congenital heart disease had more academic and behavioral difficulties than those with either cardiomyopathy or cystic fibrosis. CONCLUSIONS: A significant number of children who had undergone successful transplantation experienced difficulties at school. Contrary to expectations, educational problems were more prevalent in the medium term, rather than short term, after transplant. Initial diagnosis was a salient factor in posttransplant psychological functioning at school. Early intervention and close liaison with schools is indicated to reduce psychological morbidity and enhance adaptation within the school environment.

Cardiac rhythm and conduction before and after anatomic correction of transposition of the great arteries.

To assess pre- and postoperative arrhythmias associated with anatomic correction of transposition of the great arteries, 34 patients had 145 standard electrocardiograms (ECGs) and 24 of these patients had 46 24-hour ECGs from 11 months before to 54 months after anatomic repair. Twenty-two patients underwent balloon atrial septostomy and 7 surgical atrial septectomy before the initial 24-hour ECG. Anatomic correction included repair of a large ventricular septal defect in 16 patients and an aortopulmonary window in 1 patient. The preoperative standard ECG showed sinus rhythm in every patient. Preoperative 24-hour tapes revealed sinus arrhythmias in 2, sinus bradycardia at a rate of less than or equal to 50 beats/min in 1, junctional rhythm in 1 and rare premature ventricular contractions (PVCs) in 1. The postoperative 24-hour ECG showed atrioventricular dissociation in 1 patient, rare premature atrial contractions in 7, rare PVCs in 8 and sinus bradycardia with junctional escape in 1. Eight patients had PVCs on the postoperative ECG that were not noted on preoperative tapes. No patient had prolonged bradycardia (less than or equal to 50 beats/min), life-threatening arrhythmias or sudden death. Except for 1 patient with atrioventricular dissociation believed to be secondary to a preventable cause, 34 patients followed for 890 patient-months after anatomic correction for transposition of the great arteries had no significant arrhythmias.

Influence of the two-stage anatomic correction of simple transposition of the great arteries on left ventricular function.

To evaluate the influence of the 2-stage anatomic correction of simple transposition of the great arteries on left ventricular (LV) function, pressure and angiocardiographic volume data were analyzed during resting conditions shortly before banding of the pulmonary trunk (n = 12) and before (n = 17) and after anatomic correction (n = 11), and compared with data from controls (n = 12). Age at banding and anatomic correction was between 1 and 44 months (mean 16 +/- 10) and between 13 and 47 months (mean 24 +/- 10), respectively. The interval between anatomic correction and the investigation ranged from 10 to 29 months (mean 20 +/- 7). After banding, LV ejection fraction decreased (p less than 0.01) and LV peak systolic pressure (p less than 0.01) as well as LV end-diastolic pressure (p less than 0.05) increased. After anatomic correction, these variables and LV end-systolic wall stress were not significantly different from control values. The LV end-systolic wall stress-ejection fraction relation in 7 of 11 patients after anatomic correction was within control range. The highest values were found in the youngest patients at banding and at anatomic correction. In contrast to measures of global myocardial function, such as LV ejection fraction and LV end-diastolic pressure data, the LV end-systolic stress-ejection fraction relation suggest that LV function may not be normal in some patients 20 months after anatomic correction. Young age at operation, however, appears to be advantageous in preserving LV function. Hemodynamic alterations after banding probably reflect LV adaptation to systemic pressures in a hypoxemic circulation.

Catheter evaluation of left ventricular shape and function 1 or more years after anatomic correction of transposition of the great arteries.

Twenty-eight children were reinvestigated by cardiac catheterization and angiography greater than 1 year after anatomic correction of transposition of the great arteries (TGA). Seventeen patients with simple TGA underwent banding of the pulmonary trunk plus or minus systemic to pulmonary artery shunt to prepare the left ventricle for anatomic correction. In addition to TGA, 10 of the remaining 11 patients had a large ventricular septal defect and 1 had an aorticopulmonary window. They required no preparation of the left ventricle. Age at repair ranged from 2 to 120 months (mean 26). Catheterization 12 to 48 months after anatomic repair revealed a left ventricular end-diastolic pressure of 4 to 14 mm Hg (mean 9.5 +/- 2.5 [+/- standard deviation]). Ejection fraction ranged from 52 to 75% (mean 66 +/- 8). Frame-by-frame computer-assisted analysis of left ventricular (LV) contraction and relaxation was performed in 14 patients and compared with normal left ventriculograms. Shape index, derived as 4 pi X cavity area/perimeter2 X 100, was measured in 24 patients and showed a mean index of 89 +/- 3% at end-diastole and 79 +/- 8% at end-systole. A control group had a mean diastolic index of 86 +/- 6% and mean systolic index of 73 +/- 8%. It is concluded that LV shape after anatomic correction tends to be more globular than normal and changes little during systole. LV ejection fraction and end-diastolic pressure are normal.

Cardiac transplantation in the seventh decade of life.

Twenty-five patients older than 60 years of age underwent cardiac transplantation using an immunosuppression protocol with cyclosporin and azathioprine, but without routine use of oral steroids. There were 24 men and 1 woman (age range 60 to 69 years, mean 63). The etiology of heart disease was coronary artery disease in 21 and idiopathic dilated cardiomyopathy in 4. Six patients had previous coronary artery bypass operations, 1 had undergone repair of an abdominal aneurysm and 1 had pulmonary embolism. Sixteen patients were in New York Heart Association class IV and 9 in class III. Donor mean age was 30 (14 to 46) years. Hospital stay after transplantation was 10 to 90 days (median 11). Four died within 30 days and none from 5 to 59 months (mean 22). The 1-year actuarial survival was 84%. The incidence of rejection was 2.16 episodes per patient. Only 1 patient (4%) had serious infection. Six patients received antihypertensive treatment, 3 had reversible impairment of renal function, 2 had gout and 1 had drop foot. No patient had convulsions, transient ischemic attack or cerebrovascular accident. None had significant psychological problems. The 21 patients currently alive are in New York Heart Association class I. Quality of life, assessed by the Nottingham Health Profile, showed marked improvement. It is concluded that the initial results of cardiac transplantation in the seventh decade of life are encouraging.

Anatomic correction of the Taussig-Bing anomaly.

Anatomic correction of the Taussig-Bing anomaly depends on thorough understanding of the variable interrelations between the atrioventricular valves, ventricular septal defect, infundibular muscle bands, and aortic and pulmonary outflows and the resulting haemodynamic changes. Depending on the relative position of the great arteries, two clinicopathological types have been identified. In the two patients with side-by-side relationship of the great arteries, the conal septum was well developed, so that there were various degrees of subaortic stenosis. Associated coarctation of the aorta was present in both patients. Coronary anatomy was identical (Type E) to that present in complete transposition with side-by-side great arteries. In these patients, anatomic correction was achieved by wide excision of the conal septum and intraventricular repair. In contrast, two patients with anteroposterior relationship of the great arteries had a poorly developed conal septum and no subaortic stenosis or coarctation of the aorta. In these patients, repair was achieved by diverting blood from the ventricular septal defect to the adjoining pulmonary artery combined with switching of the great arteries and coronary transfer. There were no early or late deaths associated with either method of repair, and there was evidence of good correction of the hemodynamic abnormalities. It is concluded that anatomic correction is possible in all patients with the Taussig-Bing anomaly and that the method of repair depends on the relative position of the great arteries.

Mobilization of the left and right fibrous trigones for relief of severe left ventricular outflow obstruction.

BACKGROUND: There is still no agreement about the optimal method of surgical relief of fixed subaortic stenosis, particularly the severe forms. OBJECTIVES: The purpose of this study was to describe a new technique for the relief of subaortic stenosis based on analysis of the functional anatomy of the left ventricular outflow tract and pathophysiologic features of subaortic stenosis. METHODS AND PATIENTS: We propose that one of the basic abnormalities in subaortic stenosis is interference with the hinge mechanism provided by the 2 fibrous trigones with progressive deposition of fibrous tissue in these angles. The technique described in this paper consists of excision of all components of the fibrous "ring," with mobilization of the left and right fibrous trigones. This results in the restoration of the normal dynamic behavior of the left ventricular outflow tract with maximal widening of the outflow tract as the result of backward displacement of the subaortic curtain and anterior leaflet of the mitral valve. This technique has been used in 57 consecutive patients who ranged in age between 5 months and 56 years (mean, 15.5 +/- 10.6 years). Gradients across the left ventricular outflow tract were between 45 and 200 mm Hg (mean, 86.7 mm Hg). Additional lesions were present in 10 patients, and 7 patients had had 8 previous operations on the left ventricular outflow tract. At operation, in addition to resection of subaortic stenosis, 3 patients had aortic valvotomy, 2 patients had homograft replacement of the aortic valve, 7 patients had patch closure of a ventricular septal defect, and 1 patient had open mitral valvotomy. RESULTS: There were 2 early deaths and 1 late sudden death during the follow-up period that ranged from 1 month to 25 years (mean, 15. 2 years). One patient experienced the development of endocarditis on the aortic valve 7 years after operation, which was successfully treated by homograft replacement. Postoperative gradients across the left ventricular outflow tract varied from no gradient to 30 mm Hg (mean, 8 mm Hg). There were no instances of recurrence of a gradient across the left ventricular outflow tract. CONCLUSION: It is concluded that mobilization of the left and right fibrous trigones results in durable relief of subaortic stenosis.

One-stage correction of interrupted aortic arch combined with heart-lung transplantation.

A technique for correction of interrupted aortic arch during heart-lung transplantation is described. This operation was performed in a 14-year-old patient with situs solitus, absent right atrioventricular connection, large atrial septal defect, ventricular septal defect, transposition of the great arteries, type B interrupted aortic arch, and severe pulmonary hypertension. The postoperative course was uneventful. Clinical, radiologic, and nuclear magnetic resonance investigation have shown evidence of good correction of the aortic arch anomaly. We concluded that donor tissue available during heart-lung transplantation is useful in correcting extracardiac anomalies.

Heterotopic cardiac transplantation in infants and children.

BACKGROUND: Children with advanced heart failure, particularly those with elevated pulmonary vascular resistance, pose a difficult management problem because the normal donor right ventricle cannot cope with the high pulmonary resistance and because of the relative shortage of donor organs of an appropriate size for this age group. METHODS: In an attempt to address these issues and evaluate the role of heterotopic transplantation in this context, we operated on 12 children, six boys and six girls, in the period between January 1, 1991, and March 31, 1996. Their ages ranged from 11 months to 15.2 years (mean 81.6 +/- 62.8 months) and their mean weight was 23.3 kg (range 7.6 to 56.8 kg). Eight patients (66.6%) had significant elevation of pulmonary artery pressure (pulmonary artery systolic pressure = 66 +/- 9.4 mm Hg, mean transpulmonary gradient = 22.3 +/- 3.4 mm Hg). In all patients the donor pulmonary artery was anastomosed to the recipient right atrium without the use of any prosthetic material. Ischemic times varied between 135 and 255 minutes (mean 182.1 +/- 30.7 minutes). The immunosuppression regimen included cyclosporine and azathioprine. Steroids were not routinely used. RESULTS: One patient died in the hospital of acute rejection on postoperative day 16. Three patients had lobe collapse within 1 week and all were treated successfully. Two late deaths (18.2%) occurred as a result of cardiac rejection 3 months and 2 years after the operations. Nine survivors (75%) are alive, active, and growing normally at a mean follow-up of 2.2 years (range 11 months to 4.75 years). Repeated cardiac catheterization performed in seven patients with preoperative pulmonary hypertension showed a slow progressive drop in mean pulmonary artery pressure. No significant change was observed in the function of the recipient hearts. CONCLUSION: We conclude that heterotopic heart transplantation is feasible for a selected group of children with good medium-term results, notably regression of pulmonary artery pressure, normal growth, and lack of long-term chest complications.

Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root.

OBJECTIVES: There is still no agreement about the best method of dealing with malfunction of the aortic valve caused by aneurysm or dissection of the aortic root. The experience, rationale, and development of a valve-preserving technique introduced and used since 1979 is described. METHODS: During this period 158 patients (78% of all patients undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Their ages ranged from 2 to 72 years (mean 46.6 years). Of the patients 107 were male and 51 were female. A total of 68 patients had skeletal manifestations of Marfan's syndrome. The original disease was chronic aneurysm of the ascending aorta or root in 92 (58.2%), chronic dissection in 17 (10.8%), and acute dissection in 49 (31%) patients. One hundred eleven additional procedures were performed in 84 patients. In all there were five early deaths (4.6% +/- 2%) in the 109 patients with chronic aneurysm and one death in the 103 patients operated on electively (0.97% +/- 0.9%). Actuarial survival for patients operated on for chronic aneurysm was 93.3%, 88.0%, 79.0%, and 57.9% at 1, 5, 10, and 15 years and 96.8%, 91.2%, 82.0%, and 60.0% for those operated on electively. Actuarial survival for patients operated on for acute dissection was 72.8%, 63.4%, and 53.3% at 1, 5, and 10 years. The probability of needing reoperation was 3.0% +/- 2%, 11% +/- 0.5%, and 11% +/- 0.5% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in two patients operated on early in the series who had cusp extension. No anticoagulants were used. Echocardiography showed reduction in left ventricular end-systolic and end-diastolic dimensions, which was maintained. At the end of follow-up trivial or no aortic regurgitation was demonstrated in 63.6%, mild to moderate in 33.3%, and severe in 3%. CONCLUSIONS: Valve-sparing operations are possible in a large proportion of patients with aneurysms of the ascending aorta and the medium and long-term results are encouraging.

Anatomic correction of the syndrome of prolapsing right coronary aortic cusp, dilatation of the sinus of Valsalva, and ventricular septal defect.

BACKGROUND: Although the syndrome of ventricular septal defect and aortic regurgitation was described a long time ago, there is still no agreement about the anatomic and functional components of the syndrome and the optimal methods of management. OBJECTIVE: Our objective was to describe a new simple technique of anatomic correction of all the components of the syndrome, based on redefining the salient anatomic and functional features of the syndrome. METHODS: Anatomic correction of the syndrome is achieved through a transaortic approach with the placement of a series of pledget-supported mattress sutures using autogenous pericardium. The sutures are used to close the ventricular septal defect, plicate the aortic sinus, and correct the outward and downward displacement of the anulus of the aortic valve. The technique is designed to correct all the anatomic functional components including severe aortic regurgitation when present. RESULTS: Between 1972 and 1996, 46 patients with this syndrome underwent surgical treatment. The current technique was used in most of the patients operated on before 1981 and in all patients since that date. There were no early or late deaths during a follow-up period varying from 3 months to 24 years (mean 8.4 years). Aortic regurgitation was abolished in 16 and improved in the remaining patients, The hemodynamic results have been maintained except in five patients operated on early in the series, in whom additional procedures on the cusps were performed. CONCLUSIONS: Anatomic correction of all the components of the syndrome of prolapsing right coronary cusp, dilatation of the sinus of Valsalva, and ventricular septal defect, can be achieved by a very simple technique. This technique can be applied in young children and prevents progression and secondary changes. Early correction in all patients with this syndrome is warranted.

Fourteen-year experience with homovital homografts for aortic valve replacement.

Two hundred seventy-five unprocessed, viable homograft ("homovital") aortic valves were used for aortic valve replacement in patients aged 1.5 to 79 years (mean 45.8 +/- 19 years) with maximum follow-up of a 14-year period (mean 4.8 years). Ninety-two percent (252 patients) had New York Heart Association class III or IV functional status before operation and 25 underwent emergency operation. Valves were harvested under sterile conditions and kept in nutrient medium 199. Freehand (subcoronary) technique was used in 147 patients and freestanding root replacement was used in 128. Cumulative survival rates for the whole group were 92% +/- 2% at 5 years and 85% +/- 3% at 10 years, as compared with 96% +/- 2% and 94% +/- 4%, respectively, for the 98 patients who underwent isolated root replacement. Multivariate analysis determined that root replacement with associated procedures and operation for prosthetic endocarditis were risk factors for death, whereas previous xenograft valve, operation for endocarditis, and operation for aortic regurgitation were risk factors for reoperation. Actuarial rates for freedom from degenerative valve failure diagnosed at operation, by postmortem examination, or by routine echocardiography were 94% +/- 2% at 5 years and 89% +/- 3% at 10 years. Recipient age younger than 30 years and previous xenograft valve were risk factors for late degeneration. We conclude that homovital valves demonstrate good durability, particularly in patients older than 30 years, who had a 10-year freedom from degeneration rate of 97%.

Assessment of coronary and aortic anastomoses after anatomic correction of transposition of the great arteries.

Anatomic correction of transposition of the great arteries always entails circumferential anastomoses of the aorta and coronary arteries. Long-term success of this procedure is predicted on adequate growth of these anastomotic sites. To assess the size of these arteries, we performed one or two cardiac catheterization on 25 children from 1 to 53 months (mean 18.8 months) following anatomic correction. Early studies (mean 12 months) were performed in 23 patients and late studies (mean 30 months) in 13 patients. Age at repair ranged from 2 to 168 months (mean 25.5 months) and 15 patients were less than a year of age. Fifteen patients had undergone previous pulmonary artery banding in preparation for anatomic repair. Postoperative catheterizations showed no area of narrowing at the aortic or coronary anastomoses and no kinking of the proximal coronary arteries. Almost all normalized diameters of the aortic root were larger than normal. There were no differences between early and late measurements after anatomic correction. No patient had a pressure gradient across the aortic anastomosis. It is, therefore, concluded that the coronary and aortic anastomoses allow for satisfactory growth even when there has been previous pulmonary artery banding.

Pheochromocytoma presenting after cardiac transplantation for dilated cardiomyopathy.

Pheochromocytoma may present with a clinical picture indistinguishable from that of idiopathic dilated cardiomyopathy. We report 2 such patients who underwent cardiac transplantation following which we diagnosed and successfully treated pheochromocytoma.