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Objective: To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children., Methods: We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II)., Results: In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P <.05)., Conclusions: Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.

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.

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.

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.

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.

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%.

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.

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.

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.