Successful repair of a rare case of congenital low lying secundum atrial septal defect with unroofed coronary sinus

Tóm tắt

Background

Congenital low lying atrial septal defect (ASD) with unroofed coronary sinus is an extremely rare condition, with an estimated incidence of less than 1% among all congenital heart defects. Repairing unroofed coronary sinus in adult patients presents unique challenges due to the complexity of the defect and limited data on optimal management. This unique case report emphasizes the rarity of this condition, the infrequency of its surgical repair in adults, and the challenges encountered in the repair process, particularly in the Philippine setting

Methods

We present a case of a 54-year-old Filipino male, non-hypertensive, diabetic, and dyslipidemic who was incidentally diagnosed with paroxysmal atrial fibrillation and an ASD with an unroofed coronary sinus. Following thorough evaluation, including pre-operative transesophageal echocardiography (TEE) findings, the decision to proceed with open heart surgery for ASD repair was made. Post-operative TEE findings were also assessed to evaluate the surgical outcome.

Results

The decision to proceed with surgical repair was based on the patient's symptoms of easy fatigability and palpitations on exertion, along with the pre-operative TEE findings. The TEE revealed a low lying atrial septal defect measuring 1.6 cm in diameter and an area of 1.51 cm2, consistent with predominantly left-to-right shunting. Additionally, the presence of an unroofed coronary sinus was confirmed, posing unique challenges in the repair process. The decision to proceed with surgery was further supported by post-operative TEE findings, which showed successful closure of the defect with no residual shunting or complications.

Conclusion

The presented case report describes a unique instance of congenital low lying ASD with unroofed coronary sinus, emphasizing its rarity in adults and the successful surgical repair in a Filipino patient. The incorporation of pre-operative and post-operative TEE findings in the decision-making process strengthens the assessment of the patient's cardiac anatomy and guides the surgical repair strategy. This report sheds light on the challenges encountered in repairing unroofed coronary sinus in adult patients, highlighting the need for further research and collaborative efforts to improve the management of similar cases, particularly in the Philippine healthcare setting.