Perioperative and long-term management of Fontan patients

Abstract

A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.

The effects of Treprostinil in high-risk patients after total cavo-pulmonary connection procedure

“…Total cavo-pulmonary connection is a palliative procedure to treat anatomic univentricular and univentricular physiological malformation, with the purpose of eliminating cyanosis and reducing cardiac volume load at the cost of increasing central venous pressure [1]. After total cavo-pulmonary connection procedure, post-operative complications have gradually reduced with the continuous improvement of perioperative treatment [2]. However, the post-operative recovery is far from perfect, especially in high-risk patients.…”

“…However, the post-operative recovery is far from perfect, especially in high-risk patients. Early post-operative complications such as arrhythmia, acute kidney injury, pleural effusion and thromboembolism may develop, resulting in delayed post-operative recovery and increased risk of death [3,4].…”

Long-term management of Fontan patients: The importance of a multidisciplinary approach

“…In some cases, anticoagulant therapy with heparin can help and octreotide may be effective in some patients by decreasing thoracic duct lymphatic drainage ( 39 ). Lymphatic intervention, by accessing and occluding the abnormal connection, with selective embolization, is an emerging strategy, potentially promising but still needs technical refinements ( 40 , 41 ). Currently, these invasive therapies are considered only in selected patients and the only effective cure for lymphatic dysfunction is heart transplantation.…”

Increased Risk for Thromboembolism After Fontan Surgery: Considerations for Thromboprophylaxis

“…Post-Fontan TE in the short- and long-term following surgery remains a significant complication of the procedure, and an enduring cause of morbidity and mortality ( 6 , 7 ). Thromboembolic complications arising from the Fontan circuit commonly present in the arterial circulation as intracardiac thrombosis, ischemic stroke or arterial embolism, and in the venous circulation as central venous thrombosis or pulmonary embolism ( 2 – 4 , 8 , 9 ).…”