Paediatric cardiac anaesthesia is a specialized area of anaesthesiology that deals with children with congenital or acquired heart diseases. Congenital heart defects indicate a disorder in the structure and/or function of the heart that exists from birth. They are among the most common and severe anomalies of the organ system and are the most common cause of death in the perinatal and early neonatal period. The pathophysiology of defects will dictate the clinical presentation and the approach to treatment and anaesthesia. When considering pathophysiology, it is necessary to consider the presence of shunting and/or obstruction, the presence of cyanosis, changes in hemodynamic, and the consequences of these changes on the cardiovascular system. Basic diagnostics approach to heart defects includes X-rays, ECG, and echocardiography. Treatment depends on the type and severity of the defect and is conducted pharmacologically and surgically. An essential part of surgical treatment is an adequate anaesthetic procedure. Each aesthetic procedure begins with a thorough preoperative assessment, which includes assessing functional status, taking a medical history, and reviewing medical documentation. anaesthetic management in paediatric cardiac surgery consists of maintaining hemodynamic stability (balancing systemic and pulmonary resistance and flow), adjusting mechanical ventilation, and providing sufficient analgesia and sedation. Induction can be performed using benzodiazepines (midazolam), opioids (fentanyl), or dexmedetomidine. Maintenance is usually carried out using inhalational (sevoflurane) or intravenous (opioids, ketamine) anaesthetics. During the maintenance phase of anaesthesia, all basic and extended multimodal monitoring is necessary (invasive measurement of arterial and venous pressures, hourly diuresis, TTE, measurement of cerebral oxygenation and blood flow in the brain). Maintaining hemodynamic stability is of utmost importance, and its maintenance often requires the use of vasoactive drugs, adjustment of ventilation, and careful selection and titration of anaesthetics. The transition from the operating room to the intensive care unit marks the beginning of the postoperative phase. This period is characterized by monitoring clinical status and timely recognition and management of potential complications such as arrhythmias or heart failure.