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.