Background Evidence suggests that the anatomical characteristics of the Fontan circulation, in particular the size of the pulmonary arteries, play a key role in the hemodynamic efficiency of the circulation and consequently, patient outcome. Understanding and optimizing early growth of the pulmonary arteries is crucial to avoid later increase in pulmonary vascular resistance, a major risk facto...

Decades ago, the surgeons were asking themselves if it was possible to improve the natural history of patients with “functionally” univentricular heart (1). In order to facilitate the understanding and the subsequent decision making, for the first time, the patients with “functionally” univentricular heart were divided into two groups: (1) restricted pulmonary blood flow and (2) unrestricted pu...

In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber su...

BACKGROUND Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies. METHODS Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed...

OBJECTIVE Despite that surgical outcomes of patients with hypoplastic left heart syndrome have improved, one of the problems remaining is the high interstage mortality after a stage I Norwood procedure. The purpose of this study was to determine the hemodynamic characteristics of hypoplastic left heart syndrome after a Norwood procedure. We examined the perioperative hemodynamic differences of ...

In recent decades, the Fontan procedure and its variations have resulted in markedly improved outcomes of patients with single-ventricle physiology. These improvements are expected to increase greatly the number of surviving patients, particularly those surviving long into adulthood. However, there is still a progressive risk of attrition and failure of the Fontan circulation over time, the und...

Two cases are presented. In the first patient (8-year-old boy) after Glenn operation without closure of pulmonary outflow from the common ventricle (SV-PA) despite transcatheter SV-PA closure increased mean pulmonary artery pressure (mPAP) (22 mm Hg) remained. After 6 months of sildenafil therapy he was catheterized again. His mPAP dropped to 10 mm Hg and a Fontan operation was subsequently per...

Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy consist of 2 components. One is a smooth adjus...

patients with valvular heart disease suffer from atrial fibrillation for more than 12 months after valve surgery and have a low probability of remaining in sinus rhythm. we performed an intra-operative procedure similar to surgical maze ііі procedure for conversion of this arrhythmia to sinus rhythm. we did this study to evaluate the efficacy of this procedure to restore the sinus rhythm in pat...

The Fontan procedure has become a generic term to define a surgical procedure that orients the systemic venous return directly to the pulmonary arteries, and has been used as a palliative operation for pulmonary atresia, tricuspid atresia, double inlet ventricle and complex single ventricle. The earliest type of Fontan procedure was a simple atriopulmonary anastomosis between the right atrium a...