In seven patients who underwent Fontan procedures but in whom no valves were inserted, dynamics of right heart flow were evaluated with the use of a catheter-tipped velocity transducer, pulsed Doppler echocardiography, and angiocardiography. Right atrial (RA) contraction caused a forward flow to the pulmonary artery (PA) and a backward flow to the inferior vena cava (IVC). Backward flow to the ...

BACKGROUND After the Fontan procedure, patients are at risk for suboptimal health status related to their complex healthcare experience, physiological limitations, medical complications, and guarded long-term prognosis. METHODS AND RESULTS In the Pediatric Heart Network cross-sectional study of Fontan survivors 6 to 18 years of age, parents completed the Child Health Questionnaire, and scores...

Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left ...

BACKGROUND Before the era of the Fontan procedure, the typical course of patients with single left ventricle (LV) consisted of heart failure and death during the second or third decade of life. Despite the advent of effective palliative therapy, ventricular dysfunction remains a significant clinical problem for these patients. METHODS AND RESULTS To investigate the causes of ventricular dysfu...

BACKGROUND The Fontan circulation is a palliation for patients with a functionally univentricular heart. It is characterized by gradual attrition over time. An increase in pulmonary vascular resistance could be a key factor in the long-term failure of the Fontan circulation. In this study we aimed to identify pulmonary vascular remodeling in patients with a Fontan circulation. METHODS Pulmona...

OBJECTIVES This study aims to compare the outcome of the two co-existing modifications of staged total cavopulmonary connection (TCPC) - the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). METHODS We included 209 patients after staged TCPC (102 ILT and 107 ECC), operated on between 1988 and 2008. Medical and surgical records were reviewed for (1) patient demographics and...

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeab...

OBJECTIVES Novel protocols were used to focus on dynamic cardiorespiratory function during submaximal exercise and on the recovery from 1-min pulses of exercise in children who had undergone Fontan corrections for single-ventricle lesions. BACKGROUND Particularly in children, maximal oxygen uptake (VO2max), which is commonly used to assess the functional capability of patients after the Fonta...

The Fontan operation is the definitive step in creating a compatible circulation in SV patients. This type of procedure may fail due to the known decrease survival rate, and the inability of the systemic venous blood to pass through the lungs, which leads to further complications in the patient. To improve the Fontan circulation an injection jet shunt (IJS) from the single ventricle to the Font...

BACKGROUND Elevated neurohumoral activity and an abnormal cardiopulmonary response to exercise are well-established characteristics in patients after the Fontan operation. However, there have been few studies addressing cardiac autonomic nervous activity (CANA) in these patients. METHODS AND RESULTS We evaluated CANA in 63 post-Fontan patients and 44 controls. Cardiac parasympathetic nervous ...