Fifty-Five Years Follow-Up of 111 Adult Survivors After Biventricular Repair of PAIVS and PS

Residual Restrictive Right Ventricular Physiology after One-and-a-Half Ventricular Repair Conversion in Pulmonary Atresia with Intact Ventricular Septum

“…Although long-term survival is relatively good in BVR patients, 4 restrictive RV physiology after BVR is often observed, 5 and it is associated with elevated RA pressure, RA dysfunction, 7 and increased central venous pressure, leading to end-organ damage such as liver congestion. At our institution, according to our accumulated experiences 1 and previous reports, 8 1.5VR conversion is performed for patients with PA-IVS with failed BVR who fulfill the following criteria: (1) heart failure symptoms, (2) significant PR, (3) elevated RA pressure (>10 mm Hg), and (4) liver damage assessed by ultrasound. In the present case, after successful 1.5VR conversion concomitant with pulmonary valve replacement, RA pressure and serum B-type natriuretic peptide were significantly decreased, and liver congestion was markedly improved.…”

Prognostic Implications of Right Atrial Dysfunction in Adults With Pulmonary Atresia and Intact Ventricular Septum

“…[3][4][5] Long-term outcomes studies show that adults with PA-IVS who underwent biventricular repair in childhood have significantly impaired exercise capacity, high burden of atrial arrhythmias, and cardiovascular death, and the long-term outcomes in these patients are similar to those in patients who received univentricular or 1.5 ventricular palliation. [3][4][5][6][7] This is attributed to severe RV diastolic dysfunction, which is often exacerbated by concomitant tricuspid and pulmonary valve disease. 8,9 There are limited data to guide risk stratification in this population because, in contrast to other patients with RV outflow tract disease, the underlying haemodynamic problem is impaired RV compliance rather than RV volume overload.…”