Tetralogy of Fallot in adults.

Background: Following surgical repair in infancy, a high proportion of children born with tetralogy of Fallot are surviving into adulthood, and it is important that these patients are monitored by an adult congenital heart disease specialist for problems and complications that can occur in the adult patient.

Objective: To provide an overview on the management of adult patients with repaired tetralogy of Fallot and emphasise the need for specialist follow-up.

Discussion: Following surgical repair of tetralogy of Fallot, it is important to have lifelong surveillance in adulthood for complications such as pulmonary valve dysfunction.

Case report on the radiographic features of epipericardial fat necrosis in a patient presenting with acute chest pain.

Epipericardial fat necrosis (EFN) is a rare and benign condition. The three cardinal features of EFN are acute pleuritic chest pain, radiological features of an encapsulated fatty lesion within the epipericardial fat and the presence of inflammation within the surrounding pericardium. The exact aetiology is still unknown, and there are no known risk factors.

Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design.

Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe-and for unique reasons, may even be of heightened importance in the setting of Fontan physiology.

Sexual Function in Men Living With a Fontan Circulation.

It is unknown if the Fontan circulation has an impact on sexual health in men. This study assessed self-reported sexual health and fertility in men with a Fontan circulation. In this prospective, cross-sectional study, Australian men ≥18 years enrolled in the Fontan Registry of Australia and New Zealand were invited to complete the International Index of Erectile Function (IIEF), alongside questions assessing fertility.

Fontan-associated nephropathy: Predictors and outcomes.

Background: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown.

Methods: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up.

Propensity-matched comparison of the long-term outcome of the Nunn and two-patch techniques for the repair of complete atrioventricular septal defects.

Objectives: To compare the long-term performance of the Nunn and 2-patch techniques for the repair of complete atrioventricular septal defects.

Methods: Between January 1995 and December 2015, a total of 188 patients (Nunn n = 41; 2-patch n = 147) were identified from hospital databases. Univariable Cox regression was performed to calculate the risk of reintervention in each group.

A Rare Case of a Giant Coronary Sinus with Focal Aneurysm Secondary to Multiple Fistulous Connections Arising from a Dilated, Tortuous Left Circumflex Coronary Artery.

• TTE adds value in the diagnosis of a coronary artery-to-coronary sinus fistula. • AV fistula was observed via two connections between the LCx and coronary sinus. • Multimodality imaging aids in the diagnosis of coronary artery fistula.

Sequential Right and Left Ventricular Assessment in Posttetralogy of Fallot Patients with Significant Pulmonary Regurgitation.

Background: The natural history of right ventricular (RV) and left ventricular (LV) size and function among adults with tetralogy of Fallot (TOF) repair and hemodynamically significant pulmonary regurgitation (PR) is not known. The main aim of this study was to determine changes in RV and LV size and function over time in an adult population with TOF repair and hemodynamically significant pulmonary regurgitation.

Methods: Forty patients with repaired TOF and hemodynamically significant PR were included.

The Feasibility and Clinical Utility of Microsphere Contrast-enhanced Transthoracic Echocardiography in Adult Congenital Heart Disease.

Background: Transthoracic echocardiography (TTE) plays a key role in adult congenital heart disease (ACHD). However, a significant number of studies are nondiagnostic due to poor image quality. Enhancement of the blood pool-tissue interface with contrast-enhanced TTE (CE-TTE) can improve image quality in suboptimal studies.

Routine angiography in survivors of out of hospital cardiac arrest with return of spontaneous circulation: a single site registry.

Background: Coronary revascularization in resuscitated out of hospital cardiac arrest (OOHCA) patients has been associated with improved survival.

Methods: This was a retrospective review of patients with OOHCA between 01/07/2007 and 31/03/2009 surviving to hospital admission. Cardiac risk factors, demographics, treatment times, electrocardiogram (ECG), angiographic findings and in-hospital outcomes were recorded.

Hypertrophic osteoarthropathy in Eisenmenger syndrome.

Hypertrophic osteoarthropathy secondary to Eisenmenger syndrome can produce severe disabling pain. We report two cases treated by intravenous pamidronate infusion with good symptomatic relief. Such therapy can greatly improve the quality of life of end-stage cyanotic congenital heart patients.