Long Term Consequences of the Fontan Procedure and How to Manage Them

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae

Exercise Testing and Prescription in Patients with Congenital Heart Disease

The present paper provides a review of the literature regarding exercise testing, exercise capacity, and the role of exercise training in patients with congenital heart disease (CHD). Different measures of exercise capacity are discussed, including both simple and more advanced exercise parameters. Different groups of patients, including shunt lesions, pulmonary valvar stenosis, patients after completion of Fontan circulation, and patients with pulmonary arterial hypertension are discussed separately in more detail. It has been underscored that an active lifestyle, taking exercise limitations and potential risks of exercise into account is of utmost importance. Increased exercise capacity in these patients is furthermore correlated with an improvement of objective and subjective quality of life

Hypoplastic left heart syndrome : a review

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of the disease led to death, usually within the first weeks of life. Recently surgical palliative techniques have been developed allowing for a five-year survival in more than half the cases. MATERIALS AND METHODS: We reviewed literature available on HLHS, specifically its anatomy, embryology and pathophysiology, and treatment. The Pubmed and ClinicalKey databases were searched using the key words hypoplastic left heart syndrome, foetal aortic valvuloplasty, foetal septoplasty, Norwood procedure, bidirectional Glenn procedure, Fontan procedure, hybrid procedure. The relevant literature was reviewed and included in the article. We reported a case from Children's Clinical University Hospital, Riga, to illustrate treatment tactics in Latvia. RESULTS: There are three possible directions for therapy in newborns with HLHS: orthotopic heart transplantation, staged surgical palliation and palliative non-surgical treatment or comfort care. Another treatment mode - foetal therapy - has arisen. Staged palliation and full Fontan circulation is a temporary solution, however, the only means for survival until heart transplantation. Fifty to 70% of patients who have gone through all three stages of palliation live to the age of five years. CONCLUSIONS: The superior mode of treatment is not yet clear and the management must be based on each individual case, the experience of each clinic, as well as the financial aspects and will of the patient's parents.publishersversionPeer reviewe

Pulmonary arterial hypertension in repaired congenital heart disease: a multicentre study

This doctoral thesis aims to investigate the demographics, treatment patterns and prognosis of paediatric pulmonary hypertension (PH) and the emerging group of children and adults with PH in the setting of repaired congenital heart disease (CHD). I have conducted three studies, each with a distinct focus. The first is a retrospective, longitudinal study of ten CHD centres across the UK that assesses the clinical characteristics and treatment patterns of adults with a Fontan-type circulation receiving pulmonary vasodilators. I have compared treated patients with a matched cohort of Fontan patients who are not receiving pulmonary vasodilator therapy, complemented by an expert survey to determine current practice and the goals of therapy. In the second study, I created a 20-year national UK registry of paediatric PH and derived estimates of incidence and prevalence for all groups of paediatric PH in different age categories. I determined the natural history of paediatric PH and performed survival analysis. I then focused on patients with CHD and described the changes in demographics, with a substantial increase in patients with previously repaired CHD, who now form the largest PAH-CHD subgroup. The third study focuses on this latter group of repaired PAH-CHD. I highlighted the heterogeneity in terms of severity and onset of PAH. I identified prognostic markers and variables associated with PH resolution and developed a novel risk score for predicting adverse clinical outcomes in this group. This score will form the basis for the risk stratification of this high-risk population, to inform prognostication and guide treatment.Open Acces

Track 1: Surgery, anaesthesia and intensive care

Surgery, anaesthesia and intensive care

Track 2: Catheter interventions from fetus to adult

Catheter interventions from fetus to adult

Narrative Review of Single Ventricle: Where Are We after 40 Years?

BACKGROUND AND OBJECTIVE: Key medical and surgical advances have been made in the longitudinal management of patients with functionally single ventricle physiology, with the principles of Fontan circulation applied to other complex congenital heart defects. The purpose of this article is to review all of the innovations, starting from fetal life, that led to a change of strategy for single ventricle. METHODS: Our literature review included all full articles published in English language on the Cochrane, MedLine, and Embase with references to single ventricle and univentricular hearts , including the initial history of the treatments for this congenital heart defects as well as the innovations reported within the last decades. KEY CONTENT AND FINDINGS: All innovations introduced have been analyzed, including: (I) fetal diagnosis and interventions, in particular to prevent or reduce brain damages; (II) neonatal care; (III) post-natal diagnosis; (IV) interventional cardiology procedures; (V) surgical procedures, including neonatal palliations, hybrid procedures, bidirectional Glenn and variations, Fontan completion, biventricular repair; (VI) peri-operative management; (VII) Fontan failure, with Fontan take-down and conversion, and mechanical circulatory support; (VIII) transplantation, including heart, heart and lung, heart and liver; (IX) exercise; (X) pregnancy; (XI) adolescents and adults without Fontan completion; (XII) future studies, including experimental studies on animals, computational studies, genetics, stem cells and bioengineering. CONCLUSIONS: These last 40 years have certainly changed the course of natural history for children born with any form of functionally single ventricle, thanks to the improvement in diagnostic and treatment techniques, and particularly to the increased knowledge of the morphology and function of these complex hearts, from fetal to adult life. There is still much left unexplored and room for improvement, and all efforts should be concentrated in collaborations among different institutions and specialties, focused on the same matter

Track 5: Cardiology and the imaging revolution - Part I

Cardiology and the imaging revolution