Pulmonary Valve Atresia with Intact Ventricular Septum and Severe Aortic Stenosis

Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis

“…PA/IVS has generally been difficult to manage. Among them, the combination of PA/IVS and aortic stenosis is quite rare and lethal [1]. Although successful balloon valvuloplasty for aortic stenosis and pulmonary stenosis have been reported [2], these catheter-based approaches were impractical for the current case.…”

“…A combination of pulmonary atresia with intact ventricular septum (PA/IVS) and aortic stenosis is quite rare and lethal [1]. The severities of aortic stenosis and insufficiency are accentuated by the increased transvalvular flow to maintain the pulmonary circulation.…”

A rare case of biventricular outflow obstruction with intact ventricular septum

“…1 However, PAIVS with aortic valve stenosis is an extremely rare and fatal combination; if catheter intervention is unsuccessful or impossible, it is likely to lead to dismal consequences for the patient. [2][3][4][5] Yoshimura and colleagues 6 prepared a management protocol based on their quantitative assessment of the RV morphology of PAIVS. According to that protocol, a staged Fontan strategy was appropriate for this case, wherein a systemic-pulmonary shunt was the only option for the first palliation.…”

Pulmonary atresia with intact ventricular septum associated with aortic coarctation

“…Those that have been documented include Fallot's tetralogy and aortic coarctation, [1][2][3] valvar stenosis and coarctation, [4][5][6] the combination of tricuspid and pulmonary atresia with coarctation, 7 and valvar pulmonary stenosis or atresia with aortic stenosis. 8,9 These combinations are difficult to explain in terms of embryology, but clearly do exist. Pulmonary atresia with an intact ventricular septum associated with left-sided obstructive lesions is extremely rare.…”