I27.84

Clinical Causes: Congenital single ventricle defects Hypoplastic left heart syndrome Tricuspid atresia Other congenital heart conditions necessitating the Fontan procedure

Key Symptoms: Tiredness and fatigue, especially during activity Swelling in the legs, abdomen, or neck (edema) Shortness of breath or difficulty breathing Arrhythmias or irregular heartbeats Liver enlargement or signs of liver disease Protein loss leading to swelling or ascites

Diagnosis Path: Diagnosis of Fontan-related circulation involves a combination of clinical evaluation and specialized imaging tests. Healthcare providers may utilize echocardiography to assess heart function, cardiac MRI for detailed anatomical imaging, and catheter-based procedures to measure pressures within the heart and blood vessels. Regular follow-up is essential to monitor for potential complications and to guide management strategies.

Treatment Protocols: Medications to control arrhythmias, reduce fluid retention, and improve heart efficiency Regular physical activity tailored to the patient's capacity Nutritional support to address growth and energy needs Monitoring and treating complications such as liver problems or protein-losing conditions In some cases, additional surgical procedures or interventions may be necessary to address specific issues Long-term follow-up with a specialized cardiology team