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.

Transplantation as a treatment for end-stage pulmonary hypertension in childhood.

Despite advances in drug therapy, severe sustained pulmonary arterial hypertension can be a fatal disease. When medical therapy is exhausted, transplantation may be an option. The most common approaches are heart-lung transplantation or double-lung transplantation, with repair of the cardiac defect if necessary. Single-lung transplantation optimises the use of scarce donors but leads to particular management problems. Heterotopic cardiac transplantation has occasionally been employed in subjects with pulmonary vascular disease secondary to cardiac disease. Timing of transplantation is a difficult decision and depends largely on the aetiology of pulmonary arterial hypertension and the rate of decline. Outcomes following transplantation are steadily improving but remain poorer than for other solid organ transplants, mainly because of the high incidence of post-transplant bronchiolitis obliterans syndrome.

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.

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.

Heterotopic cardiac transplantation and Batista operation.

We report the case of an infant who was first seen with dilated cardiomyopathy at the age of 3 1/2 months and 2 months later, successfully underwent heterotopic cardiac transplantation in combination with partial left ventriculectomy. The benefits of combining both procedures in such a young infant particularly with regard to containment of early graft dysfunction, reduction of respiratory problems, and weight mismatch are discussed.

Early and long-term results of a valve-sparing operation for Marfan syndrome.

BACKGROUND: We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. METHODS AND RESULTS: Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63. 6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. CONCLUSIONS: Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

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.

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.

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.

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.

Reoperative aortic valve operation after homograft root replacement: surgical options and results.

The surgical options available and the associated operative risks for repeat aortic valve replacement after free-standing homograft root replacement with reimplantation of the coronary arteries are as yet undefined. We therefore reviewed our experience with repeat aortic valve replacement between January 1976 and July 1994 and identified 22 such procedures performed on 21 patients after homograft or autograft root replacement. Reoperation was indicated for structural deterioration in 16 and for bacterial endocarditis in 6 patients. Associated procedures were undertaken in 5 patients, and 4 procedures were performed emergently. Significant calcification of the homograft wall was noted in over half of all patients. There were no coronary ostial complications. In 13 patients new valves were inserted within the previous root: 9 mechanical valves and 4 subcoronary homografts. In 9 patients the root was re-replaced. The mean cross-clamp time was 85 (+/- 20) minutes and mean cardiopulmonary bypass time was 123 (+/- 32) minutes. There were no early deaths and 5 late deaths. Two patients required reoperation for bleeding and 2 experienced low cardiac output syndromes postoperatively. Univariate analysis failed to identify any variables predictive of outcome. We conclude that repeat aortic valve replacement after homograft root replacement, even in the presence of significant calcification, can be undertaken with an acceptable operative risk and should not be delayed until irreversible ventricular dysfunction has occurred. The same options available at initial valve replacement may be employed at reoperation, although extensive calcification mandates repeat root replacement.

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%.

Heterotopic heart transplantation for elevated pulmonary vascular resistance in pediatric patients.

BACKGROUND: The presence of significant elevation of pulmonary vascular resistance is a major risk factor for death after orthotopic heart transplantation. The choice of procedure for the pediatric patient remains contentious. METHODS: We report three pediatric patients with pulmonary hypertension and raised pulmonary resistance, including two infants, in whom heterotopic transplantation was performed with smaller donor hearts. The hearts were anastomosed to provide left ventricular support alone because predominant left ventricular failure was present, the pulmonary resistance was expected to fall gradually after surgery, and we wished to avoid the potential problems of a pulmonary conduit associated with growth and possible reoperation. RESULTS: The clinical results have been satisfactory in all three patients, who were well and without symptoms. No pulmonary compromise was observed in the smaller thoracic cavity of the two infants. The transpulmonary gradient fell in all three patients, although this reduction was not immediate. CONCLUSIONS: This technique in carefully selected recipients can allow safer transplantation in pediatric patients with elevated pulmonary resistance, can increase the donor pool by allowing use of smaller hearts and nonideal donors, and may reduce the mortality on the transplant waiting list by providing earlier transplantation.