Discussion
Congenital TV abnormalities resulting in isolated TR are uncommon and
often become clinically significant in the setting of other associated
congenital heart diseases3. TVr is the preferred
surgical treatment for TR, particularly in congenital TV
disease4. Severe TR can precipitate arrythmias and
induce RV dilatation, dysfunction, and ultimately right heart failure.
Studies have demonstrated that isolated moderate to severe TR is an
independent risk factor for morbidity and
mortality5,6. However, despite this, there is a
paucity of outcome studies in this patient population, thus the timing
and indications for surgical intervention remain an area of ongoing
discussion. Currently, the American College of Cardiology and American
Heart Association guidelines recommend TV surgery to be considered in
patients with severe primary TR and progressive RV dilation, even in
those who are asymptomatic7,8. Therefore, while the
surgical approach to TVr is challenging and unique to each patient’s TV
morphology, the overall treatment of TR irrespective of valve pathology
has shifted towards an emphasis on early surgical intervention to
preserve RV function.
In our case, the patient did not have any additional associated
congenital heart diseases and chordal elongation was believed to be
responsible for prolapse of the anterior TV leaflet. TVr for congenital
TR secondary to chordal abnormalities is challenging. We elected to
perform the repair using the Alfieri edge-to-edge method given the
patient’s valve pathology and safety and feasibility of the technique.
In a recently published retrospective study of 237 adult patients with
moderate-to-severe TR who underwent TVr using the edge-to-edge
technique, Lee et al. demonstrated favorable long-term outcomes,
emphasizing three important advantages of this technique: ease and
feasibility, flexibility to accommodate additional approximation
stitches if necessary, and ability to be performed without an
annuloplasty9. One major concern of the edge-to-edge
repair is the risk for tricuspid stenosis. In order to mitigate this, we
performed a stepwise evaluation of the repair and only when acceptable
coaptation was achieved and confirmed with a saline test was the suture
tied. Additionally, at the completion of the repair, intraoperative TEE
demonstrated a satisfactory mean pressure gradient across the valve of 1
mmHg. In conclusion, isolated congenital TR is extremely rare and highly
variable and we advocate for early referral for surgical evaluation and
TVr in isolated congenital TR prior to the development of severe RV
dysfunction.