Perioperative and long-term management of Fontan patients.
May Al-ShawkAdeolu BanjokoAriana AxiaqKiran AminAmer HarkyPublished in: Cardiology in the young (2021)
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.
Keyphrases
- pulmonary artery
- venous thromboembolism
- left ventricular
- end stage renal disease
- magnetic resonance
- ejection fraction
- acute kidney injury
- pulmonary hypertension
- coronary artery
- healthcare
- chronic kidney disease
- cardiac surgery
- newly diagnosed
- heart failure
- atrial fibrillation
- adipose tissue
- magnetic resonance imaging
- type diabetes
- metabolic syndrome
- squamous cell carcinoma
- pulmonary arterial hypertension
- patient reported outcomes
- mitral valve
- primary care
- mesenchymal stem cells
- liver failure
- lymph node
- minimally invasive
- direct oral anticoagulants
- inferior vena cava
- mechanical ventilation
- small molecule
- acute respiratory distress syndrome
- glycemic control
- insulin resistance
- hepatitis b virus
- aortic dissection
- extracorporeal membrane oxygenation
- protein protein
- electronic health record