Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

Cyanosis in a patient after Fontan palliation due to unrecognised hepatic vein to coronary sinus communication

A 6-year-old male with heterotaxia, abnormal systemic and pulmonary venous drainage, and a history of Fontan completion presented with desaturations and was found by cardiac catheterisation to have a hepatic vein to coronary sinus connection. This was successfully occluded using an Amplatzer Muscular Ventricular Septal Defect Occluder.

Pregnancy in the FONTAN palliation: physiology, management and new insights from bioengineering

Fontan palliation for the single ventricle results in a challenging and delicate physiological state. At rest, the body adapts to a low cardiac output and high systemic venous pressure. However, when physiological demands increase, such as in the case of exercise or pregnancy, this delicate physiology struggles to adapt due to the inability of the heart to pump blood into the lungs and the consequent lack of augmentation of the cardiac output.Due to the advances in paediatric cardiology, surgery and intensive care, today most patients born with congenital heart disease reach adulthood. Consequently, many women with a Fontan circulation are becoming pregnant and so far data suggest that, although maternal risk is not high, the outcomes are poor for the foetus. Little is known about the reasons for this disparity and how the Fontan circulation adapts to the physiological demands of pregnancy.Here we review current knowledge about pregnancy in Fontan patients and explore the potential role of computational modelling as a means of better understanding this complex physiology in order to potentially improve outcomes, particularly for the foetus.