To the Editor:
We appreciate the comments of Dr. Kasouridis and would like to address some of his concerns. Case reports are limited by strict word counts, which made in-depth discussion of complex embryology and valvular anatomy difficult. Although his view about nomenclature in the report is valid, we elected to limit our discussion to relevant details about the closure of the primum atrial septal defect (ASD) via a percutaneous approach, which was facilitated by intraprocedural transesophageal echocardiogram (TEE) guidance.
The patient was initially sent to us with a diagnosis of Lutembacher's syndrome from a secundum ASD and rheumatic mitral stenosis. The patient adamantly refused surgical therapy and requested a nonsurgical option due to significant worsening of symptoms. The structural heart team, which included experts in treating adult congenital heart diseases, was consulted and developed a plan to provide the patient symptomatic relief, without compromising subsequent surgical interventions if the patient changed their mind in the future. The team decided to proceed with balloon valvuloplasty of the mitral valve with possible ASD closure as the anatomy seemed reasonable based on two-dimensional transthoracic echocardiography.
The intraprocedural catheterization and TEE findings were demonstrated in our report. It became apparent by three-dimensional TEE that balloon valvuloplasty of the left atrioventricular valve was not an option. We then decided to focus our efforts to address the ASD as its closure continued to align with the predetermined procedural goal to improve symptoms while leaving future treatment options available. We understood that the left-sided pressures would likely increase with removal of the shunt, so repeated measurements were performed prior to deploying the device. An outpatient transthoracic echocardiography 1 month later demonstrated a gradient of 8 mm Hg without significant intracardiac shunt, as mentioned in our report. Importantly, the patient has had remarkable symptomatic improvement and does not wish to pursue additional therapy at this time.
Interventionalists have begun to introduce alternative treatment options for specific patients. Closing select superior sinus venosus ASDs in the catheterization laboratory, previously considered feasible only through surgical repair, represents one of the latest advancements in managing congenital heart disease. This challenges the conventional gold standard of surgical intervention, a practice long upheld by guidelines. It is paramount to realize guidelines based on lower levels of evidence, as are frequently encountered in rare diseases, are inevitably dynamic and only altered and progressed by attempting innovative therapies. We provided a creative solution to address a congenital pathology where conventional guidelines were not applicable and the patient had no therapeutic options for treatment.
Footnotes
Conflicts of interest: J.M.M. serves as a consultant for Edwards Lifesciences, Abbott Laboratories, Medtronic, Boston Scientific, and Shockwave and holds equity in Excision Medical. The remaining authors have nothing to disclose.