Primary Tricuspid Regurgitation in ACHD: Diagnosis and Treatment.

Primary tricuspid valve regurgitation includes congenital and acquired disorders, especially Ebstein anomaly and tricuspid valve dysplasia. Severe tricuspid regurgitation causes progressive right atrial and ventricular dilation, arrhythmias, exercise intolerance, impaired left ventricular filling, and eventual biventricular dysfunction. Some patients remain minimally symptomatic for decades despite progressive disease. Poor prognostic factors include severe cardiomegaly, ventricular dysfunction, cyanosis, and arrhythmias. Surgical indications include symptoms, declining exercise capacity, cyanosis, progressive right ventricular enlargement or dysfunction, recurrent arrhythmias, and, in selected asymptomatic patients, severe regurgitation with right ventricular dilation. The Cone repair procedure restores native valve competence, improves right and left ventricular interaction, promotes reverse remodeling, and has low operative risk in experienced centers. Current evidence supports individualized and earlier surgical repair, preferably during childhood and in specialized centers, to improve ventricular function, quality of life, and long-term survival.