Repair of Double-Chamber Right Ventricle and Subvalvular Pulmonic Stenosis in a 78-Year-Old With Repaired Tetralogy of Fallot: Anesthetic Considerations and Review of the Literature.

BACKGROUND

Double-chambered right ventricle (DCRV) is a rare congenital anomaly characterized by hypertrophied muscular bundles dividing the right ventricle into high- and low-pressure chambers. It is frequently associated with congenital heart disease, particularly tetralogy of Fallot (TOF), but rarely presents in late adulthood.

CASE PRESENTATION: We report the case of a 78-year-old man with a history of prior pulmonary valvotomy who presented with progressive dyspnea and was found to have severe subvalvular right ventricular outflow tract obstruction. Multimodal imaging and cardiac catheterization confirmed DCRV with near-systemic right ventricular pressures and an anomalous left anterior descending coronary artery arising from the right coronary cusp. The patient underwent surgical resection of hypertrophied infundibular muscle bundles with patch augmentation of the main pulmonary artery. Intraoperative transesophageal echocardiography guided management and confirmed successful relief of obstruction. The patient had an uncomplicated postoperative course with resolution of symptoms.

DISCUSSION: This case highlights the late presentation of DCRV as a sequela of incompletely repaired TOF and underscores the importance of comprehensive imaging and careful surgical planning in the presence of anomalous coronary anatomy. Anesthetic management focused on maintaining right ventricular preload and coronary perfusion while avoiding dynamic right ventricular outflow tract obstruction.

CONCLUSION

DCRV can present late in life in patients with prior congenital heart disease. Successful management requires a multidisciplinary approach with careful perioperative hemodynamic optimization.