The primary extracardiac inferior cavopulmonary connection is an unusual novel palliation for single-ventricle physiology, which we first performed in the setting of unfavourable upper-body systemic venous anatomy for a standard bi-directional Glenn, and in lieu of leaving our patient with shunt-dependent physiology. After an initial 16-month satisfactory follow-up, increasing cyanosis led to the discovery of a veno-venous collateral that was coiled, but, more importantly, to impressive growth of a previously diminutive superior caval vein, which allowed us to perform completion Fontan with a good outcome. Performing the single-ventricle staging in a reverse manner, first from below with a primary inferior cavopulmonary connection, followed by Fontan completion from above with a standard superior caval vein bi-directional Glenn, is also possible when deemed necessary.

The superior cavopulmonary anastomosis – bi-directional Glenn – is the standard palliation for single ventricle physiology. When upper body systemic venous anatomic concerns such as superior caval vein stenosis, hypoplasia, or inadequate collateral tributaries are present, a Glenn may be precluded or have a high risk of poor outcome. A primary inferior cavopulmonary connection with an extracardiac conduit is an alternative palliation that provides a generous pathway for pulmonary blood flow, with the additional benefit of including hepatic venous return. We report a case of primary extracardiac inferior cavopulmonary connection in a patient unsuitable for Glenn, with successful post-operative outcome and early follow-up.

The tricuspid valve is being increasingly recognised as an important safeguard to the heart with congenital heart disease. Both structural anomalies of the valve and functional burdens from other malformations of the right heart can lead to major haemodynamic consequences both upstream and downstream. The indications to surgically intervene on the tricuspid valve are evolving and vary depending on the malformation. The extant surgical techniques and their applications to corresponding frequent congenital anomalies of the tricuspid valve are reviewed.

The arterial switch operation is the extant surgical correction after a long series of palliations attempted and/or successfully achieved for the treatment of discordant ventriculoarterial connections. As early as 1954, pioneers such as Mustard, Bailey, Kay, and Idriss led the way with at first disheartening failures, temporarily leading to abandoning the procedure. The first successful atrial baffle procedure in 1958 established itself as the procedure of choice for treating discordant ventriculoarterial connections, but tenacity, courage, and vision to pursue anatomic correction finally led to the first successful arterial switch in 1975 by Jatene. After a decade to perfect surgical technique and timing indications for the various anatomic subtypes, the new era of the neonatal arterial switch since the late 1980s set the very high standards that we all know and expect today. Despite excellent early and long-term survival, important residual lesions are increasingly being recognised. Expected anatomic residuals include supravalvar pulmonary stenosis, neoaortic valve insufficiency, and coronary ostial stenosis. Reinterventions and rare, but challenging surgical reoperations address these residual findings with satisfactory outcomes. Quality of life into young adulthood is satisfactory, but functional problems include reduced exercise capacity, diffuse coronary insufficiency, and neurodevelopmental shortcomings, of which the true incidence and potential clinical implications are still unknown. The arterial switch is a spectacular anatomic correction for a once lethal condition and currently the best surgical solution for patients with discordant ventriculoarterial connections. It is, however, far from a true cure; closer and ongoing follow-up for future care will continue to be required.

Paclitaxel-eluting balloons are a new and innovative method in the treatment of in-stent stenosis and small vessel disease in adult cardiac pathology. The treatment of congenital pulmonary vein stenosis is difficult to manage, and results in a high mortality rate due to residual or recurrent stenosis. We report the first case of treatment for neonatal pulmonary vein restenosis with a paclitaxel-eluting balloon.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.

The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the haematological system and to infectious complications. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.

The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of haematological and infectious complications. Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.

Right ventricular function is of great importance in patients with both acute and chronic ventricular overload. The early detection of right ventricular dysfunction may have an impact on therapeutic decision making, helping to prevent or further delay functional deterioration of the right ventricle.

In patients with right ventricular overload due to congenital cardiac diseases, dobutamine stress testing combined with magnetic resonance imaging, electrocardiographic changes, and monitoring of concentrations of plasma brain natriuretic peptide are very suitable parameters for the early detection of ventricular dysfunction, and should therefore be used in the follow-up of these patients.

It is apparent that no single measurement of anatomy or function can ever adequately describe the form or performance of the right ventricle. Rather, we should be looking more towards an integrated approach of different parameters for right ventricular function. The quantitative parameters described in this study can serve this purpose. The strong correlation found between these non-invasive and independent parameters encourages their clinical implementation.

Patients with congenitally corrected transposition are at risk of right ventricular dysfunction and failure. With this in mind, we examined 13 patients with congenitally corrected transposition, 7 not having undergone surgery, and 6 after physiological repair, comparing them with 6 healthy subjects matched for age and sex, using cardiac magnetic resonance imaging, at rest and during dobutamine stress, in order to determine regional and global right ventricular response to stress.

At rest, the patients had significantly decreased overall wall motion compared to their healthy peers (7.2 ± 0.5, versus 9.8 ± 0.4 mm). During infusion of dobutamine, overall wall motion increased to 12.8 ± 0.4 mm in the healthy subjects, versus 8.8 ± 1.0 mm in patients. At the regional level, significant differences in mural motion were found between patients and controls in the anterior (9.5 ± 1.1, versus 13.2 ± 0.6 mm), posterior (10.2 ± 1.6, versus 13.2 ± 0.8 mm), and septal segments (5.0 ± 0.8, versus 11.2 ± 0.6 mm).

At rest, overall mural thickening in patients was similar to that of controls, but significantly less in patients during stress. During dobutamine stress, patients showed significantly less regional wall thickening than controls, particularly in the septal (2.7 ± 0.6, versus 6.0 ± 0.4 mm, respectively) and in the anterior segments (4.2 ± 0.6, versus 7.8 ± 0.6 mm, respectively). Right ventricular ejection fraction strongly correlated with mural motion and thickening, both at rest and during stress.

Abnormal regional function in the systemic morphologically right ventricle may occur in patients with congenitally corrected transposition, which strongly correlates with right ventricular ejection fraction. Our findings support the hypothesis that, in patients with congenitally corrected transposition, ischemia of the right ventricular myocardium contributes to the development of right ventricular dysfunction.