We read with great interest the recent case report highlighting the importance of advanced diagnostic evaluation in adults with repaired congenital heart disease (CHD) presenting with unexplained symptoms.1 The authors describe a 43-year-old woman who underwent surgical repair of an atrial septal defect (ASD) in childhood and presented with desaturation. Initial investigations at the local hospital failed to identify the underlying cause, prompting referral to a specialized CHD center. There, a previously unrecognized inferior-type sinus venosus defect with a bidirectional shunt was diagnosed, and the patient was subsequently referred for corrective surgery.
This case serves as an excellent example of the challenges faced by patients and clinicians operating in an era of limited diagnostic modalities. It underscores the importance of maintaining clinical vigilance for missed or residual defects in individuals presenting with symptoms or inappropriate cardiac remodeling during follow-up. The clinical outcome for the patient was favorable, as the correct diagnosis was ultimately established and appropriate therapy instituted.
However, we believe that the authors’ take-home message warrants reconsideration. We fully agree that patients with repaired CHD benefit from evaluation by specialized teams with expertise in congenital cardiology in case of unexplained symptoms and diagnostic uncertainty exists. Nevertheless, the conclusion that “patients with repaired CHD should be followed up by a specialized team with expertise in CHD at reasonable intervals based on the lesion and the results of the repair” does not align with the follow-up recommendations. According to the 2020 European Society of Cardiology Guidelines for the Management of Adult Congenital Heart Disease—co-authored by 2 of the same investigators—patients who underwent surgical ASD closure before the age of 25 do not require routine follow-up in the absence of symptoms or abnormal findings.2
Thus, the message conveyed in this case report is in conflict with guideline-based practice and risks promoting defensive clinical behavior leading to unnecessary investigations and increased healthcare costs, based on a single case report. This is particularly noteworthy given that the missed diagnosis originated from the “specialized” center where the initial repair had been performed. We therefore suggest refining the take-home message to emphasize the importance of clinical vigilance in evaluating patients with symptoms or evidence of inappropriate remodeling following repair of congenital heart defects, while maintaining alignment with current guidelines stating that routine follow-up is not indicated for asymptomatic patients with surgical ASD closure before the age of 25.
Footnotes
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
References
- 1.Danylenko O., Babu-Narayan S., Brida M., Heng E.L., Li W. Biventricular interatrial shunt with desaturation after atrial septal defect repair. JACC Case Rep. 2025;18 doi: 10.1016/j.jaccas.2025.105743. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Baumgartner H., De Backer J., Babu-Narayan S.V., et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563–645. doi: 10.1093/eurheartj/ehaa554. [DOI] [PubMed] [Google Scholar]