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Objective: This study reviews a single-center experience with the Ross procedure in infants and young children., Methods: From November 1993 to March 2012, 55 children aged less than 17 years underwent a Ross procedure. The patients ranged in age from 2 days to 17 years (median, 5.9 years). Thirteen patients were infants, and 18 patients were preschool children. The predominant indication for the Ross procedure was aortic stenosis. Twenty-seven patients (49%) with left ventricular outflow tract obstruction underwent a modified Ross-Konno procedure. Twenty-five patients (45%) had undergone 40 previous cardiac procedures. Preoperatively, 3 patients showed severe left ventricular dysfunction, with 2 of the patients requiring intubation and inotropic support. Concomitant procedures were performed in 11 patients (20%). Nine patients underwent mitral valve surgery, and 2 patients underwent subaortic membrane resection., Results: Patients were followed up for a median of 66 months (range, 3 months to 17 years). Overall survival at 1, 2, 5, and 10 years was 84.9%. Hospital mortality rate was 13% (7/55 patients). All deaths occurred in neonates or infants, except 1 who was aged less than 4 years. Freedom from reoperation for autograft failure was 100% at 1 year, 96.7% at 5 years, and 73.7% at 10 years. During follow-up, 7 patients required a reoperation on the autograft for dilatation and severe aortic insufficiency. Freedom from reoperation for the right ventricular outflow tract replacement was 56.1% at 10 years., Conclusions: The low rate of autograft failure demonstrates that the Ross procedure is an attractive option for the management of aortic valve disease and complex left ventricular outflow tract obstruction in the pediatric population. However, alternative options must be considered in adolescents and young adults., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Total extracardiac cavopulmonary connection is an established procedure, but the best spatial arrangement remains controversial. On the basis of our clinical experience with total extracardiac cavopulmonary connection, we performed quantitative and qualitative flow analysis on total extracardiac cavopulmonary connection models simulating the two most frequent arrangements applied to our patients to determine the most favorable hydrodynamic pattern., Methods: We selected two main groups among 110 patients who underwent total extracardiac cavopulmonary connection, those with left-sided inferior vena cava anastomosis (type 1) and those with facing superior and inferior vena cava anastomoses (type 2). Blown-glass total extracardiac cavopulmonary connection phantom models were constructed on the basis of nuclear magnetic resonance and angiographic images. Flow measurements were performed with a Nd:YAG Q-switched laser and a particle imaging velocimetry system. A power dissipation study and a finite-element numeric simulation were also carried out., Results: When applying superior and inferior vena caval flow proportions of total systemic venous return of 40% and 60%, respectively, a vortex was visualized in the type 1 phantom that rotated counterclockwise at the junction of the caval streams. This apparent vortex was not a true vortex; rather, it represented a weakly dissipative recirculating zone modulating the flow distribution into the pulmonary arteries. The power dissipation and finite-element numeric stimulation confirmed the beneficial nature of the apparent vortex and a more energy-saving pattern in the type 1 phantom than in the type 2 phantom., Conclusion: Total extracardiac cavopulmonary connection with left-sided diversion of the inferior vena caval conduit anastomosis is characterized by a central vortex that regulates the caval flow partitioning and provides a more favorable energy-saving pattern than is seen with the total extracardiac cavopulmonary connection with directly opposed cavopulmonary anastomoses.

Methods: Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22)., Results: Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years., Conclusion: These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

Between December 1986 and December 1990, a bidirectional cavopulmonary anastomosis was performed in 27 patients younger than 2 years of age, including 12 with heterotaxia syndrome. Age and weight of patients averaged 14.2 +/- 6.6 months and 8.1 +/- 2.2 kg, respectively. Eleven had pulmonary atresia and 16 had pulmonary stenosis. The main pulmonary artery was ligated in seven patients in the latter group (subsequently reopened in one) and left open in nine (subsequently ligated in two). There were four hospital deaths (15%). All patients were discharged with anticoagulant/antithrombotic therapy to be continued for 6 months. There were two late deaths before further operations (8.7%). Two patients, one with acquired massive pulmonary arteriovenous fistulas and one with progressive common atrioventricular valve regurgitation, subsequently underwent definitive repair (biventricular in one), and both died. Heterotaxia syndrome (p = 0.087) and preoperative mean pulmonary artery pressure higher than 15 mm Hg (p = 0.09) were the only risk factors for overall mortality.

From November 1988 to May 1989, four patients underwent total right heart bypass by means of bidirectional cavopulmonary anastomosis and interposition of an extracardiac conduit from the inferior vena cava to the pulmonary artery. All of them had an uneventful postoperative course, and there have been no early or late deaths. We propose this technique as an alternative surgical option in candidates for a Fontan procedure with (1) hypoplasia or atresia of the left atrioventricular valve, (2) common atrioventricular valve, (3) anomalies of systemic and pulmonary venous return, or (4) auricular juxtaposition.

Significant airway obstruction may complicate the natural and surgical history of infants with congenital heart diseases. Airway obstruction occurred in 12 infants who had operations for congenital heart disease. In all cases tracheography demonstrated the cause of airway obstruction. Significant mortality (5/12, 41.7%) and morbidity in this group of infants were the result of airway obstruction. To reduce the complications caused by airway obstruction in infants with congenital heart disease, we recommend (1) preoperative identification of patients with potential airway obstruction, (2) preoperative tracheography in high-risk infants, (3) appropriate choice of the surgical procedure, especially when insertion of a prosthetic conduit is required, (4) early recognition of the problem during the postoperative period, (5) prompt diagnosis by postoperative tracheography, with or without angiography, and (6) therapy directed at the cause of airway obstruction.

Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.

Limited experimental and clinical experience with extracardiac bypass of the mitral valve has been reported. We describe the case history of a 3-year-old child in whom a left atrial-left ventricular valved conduit was successfully used to bypass a severely hypoplastic parachute mitral valve. The potential applications of this unconventional surgical option are reviewed.