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Fontan circulation is a well-established palliation in patients with functional single ventricles. Absence of a sub-pulmonary pumping chamber creates a unique physiology in which blood flow is mainly guided by negative intrathoracic and elevated central venous pressures. Various pulmonary anatomic or pathophysiologic changes can jeopardize optimal Fontan circulation. Long-term survival of patients who have undergone the contemporary total cavopulmonary connection is satisfactory. Thorough literature review in conjunction with accumulated clinical experience can lead clinicians to extract conclusions regarding Fontan and lung interactions indicating the purpose of this review.

Background: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions., Methods: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone ( n  = 10), pulmonary artery sling with tracheoplasty ( n  = 41), and pulmonary artery sling with both intracardiac and tracheal surgery ( n  = 28)., Results: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery ( p  = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality., Conclusion: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

Heart transplantation is a relatively infrequent but technically demanding procedure in pediatric cardiac surgery. Orthotopic heart transplantation is often offered to older children or adolescents following multiple previous operations, but with advances in intensive care, diagnostics, and management, more infants with complex congenital heart diseases are being listed for transplantation. Primary cardiomyopathies remain the most common indication. Outcomes following cardiothoracic transplantation have improved steadily in recent times. Donor availability has been helped by increasing public awareness and donation after cardiac death, especially of lungs, increasing the number of patients benefiting from heart and/or lung transplantation. While there are still insufficient donors to meet the current needs, effective devices to bridge patients to transplantation are now widely used. Although the many advances have improved the outlook of transplantation, there are still difficulties that have not changed over time. Nevertheless, heart transplantation in children carries a reasonably good survival rate over the entire spectrum of recipient heart conditions. We discuss the salient features of acceptance of an organ and the technical aspects of both simple and complex heart transplantation in children.

Postpneumonectomy syndrome is a rare complication following pneumonectomy with its related change in mediastinal configuration. Symptoms range from airway obstruction to esophageal symptoms, leading at times to a persistent requirement for respiratory support. Surgery is often beneficial, which is in the form of placement of a prosthesis, with variable results. We report 2 cases of postpneumonectomy syndrome: one with successful relief, and the other in which the saline-filled prosthesis failed to achieve the desired result., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Chest wall and spine deformities are common in Marfan syndrome, and often coexist with cardiac defects. Surgery is often needed to both the aortic root and the anterior chest wall, and early spinal surgery is not uncommon. We describe a case of severe thoracic cage deformity with aortic root dilatation, which was managed by a staged approach, with a very good final result., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Combined tracheobronchial and thoracic vascular injury in children following blunt trauma to the chest is potentially life-threatening and almost certain to be fatal unless managed promptly. We report one such incident where prompt identification and early aggressive surgical management prevented an almost certain fatal outcome in a 5-year-old girl with complete disruption of the right main bronchus just distal to the carina, and a tear in the right pulmonary artery.

Chordopapillary apparatus preservation was compared with valve-excising mitral valve replacement in a retrospective analysis of 360 patients, of whom 98 had total or partial chordal preservation and 262 had the conventional operation. No significant differences were seen in age, sex, pathology, crossclamp or cardiopulmonary bypass times between the 3 groups. Left ventricular fractional shortening decreased significantly in patients whose valves had been excised completely, whereas it remained unchanged in patients with either partial or total chordal conservation. There was a survival benefit for patients undergoing leaflet preservation (92% vs. 80% for conventional excision at 5 years; p=0.001). Chordal preservation during valve replacement for mitral valve disease improves survival, enhances functional status, preserves left ventricular geometry and function, and improves overall cardiac performance. Preservation of the posterior leaflet alone offers excellent results that are comparable to those of patients with total chordal preservation.