Adult congenital heart disease: Surgical correction of total anomalous pulmonary venous connection with severe pulmonary hypertension at 16 years of age - Perspective of anaesthetic management

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease with an incidence of 1%–3% of all congenital heart disease patients. The pulmonary veins returning oxygenated blood from the lungs are draining into the right side (right atrium or great veins), causing systemic hypoxia and cyanosis. TAPVC patients are mostly present at an early stage and need surgical correction to sustain life and prevent complications. However, some subsets of patients with TAPVC in whom the mixing of blood is happening may progress into adult life and have associated pulmonary arterial hypertension (PAH) and right ventricular (RV) dysfunction. We report a case of a young adult lady who presented to our hospital at 16 years of age with TAPVC along with severe PAH and RV dysfunction. During the perioperative period, the patient was successfully managed with pulmonary vasodilators and inotropic support. The objective of the case report was to describe the management strategy followed in the successful treatment of the patient. Due to congestive heart failure, low cardiac output status, and other factors, these individuals will also experience comorbidities such as PAH, RV dysfunction, and organ dysfunction. Patients require care in an environment that has experience handling a variety of problems and a PAH crisis. PAH can be controlled with the aid of pulmonary vasodilators such as sildenafil, bosentan, milrinone, NTG, and inhaled nitric oxide. Mechanical hyperventilation to lower pulmonary vascular resistance will also aid in treatment. When combined with intraoperative transesophageal echocardiography monitoring, the balanced anesthetic method can be used to successfully manage the majority of cases.