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Reoperations can be challenging for patients who experience left and right atrioventricular valve failure after repair of a complete atrioventricular septal defect. Herein, we present a case of a 10-year-old boy who developed dysfunction in left and right atrioventricular valve following surgery for a complete atrioventricular septal defect. The patient underwent successful replacement of his left atrioventricular valve with a mechanical valve due to severe stenosis. Additionally, his right atrioventricular valve, in which the bridging leaflets adhered to the ventricular septal defect patch, causing the significant regurgitation, was repaired. The bridging leaflets were augmented using the autologous pericardium treated with ethanol after detaching the leaflets and chordae from the patch. Postoperative echocardiography showed that the prosthetic valve was functioning well, with no significant regurgitation in the right atrioventricular valve. This technique could benefit patients experiencing complex valve failures following atrioventricular septal defect surgery.

Acute rupture of the chordae tendineae of the mitral valve could lead to severe mitral regurgitation and circulatory collapse in infants. Mitral valve replacement may be often challenging because of the valve-annulus size mismatch in small infants when mitral valve repair cannot be accomplished. We present an infant with acute massive rupture of the chordae tendineae of the mitral valve who successfully underwent supra-annular mitral valve replacement using the short composite valve of an expanded polytetrafluoroethylene( ePTFE) graft and a mechanical valve. His mechanical valve has been functioning without complications such as thrombosis and pulmonary venous obstruction for 20 months after surgery. This technique could be helpful even infants with acute rupture of the chordae tendineae of the mitral valve whose left atrium may not be dilated.

Recently, number of adult congenital heart disease(ACHD) patients are rapidly increasing, but statistical evidences in adult congenital heart surgery are not sufficient because of its short history in addition to the complicated pathophysiology of ACHD. Therefore, a "heart team" including surgeons, pediatricians, and cardiologists are required, and blood flow imaging and 4-dimensional (4D) imaging plays an essential role in perioperative patient management. Enhanced 3-dimensional computed tomography( 3D-CT) scan is useful to determine cardiopulmonary bypass strategies in reoperation, and 4D flow magnetic resonance imaging( MRI) is useful in assessment of complicated hemodynamics. Diastolic function of systemic ventricle is very important in right side heart valve surgery or univentricular heart surgery, echocardiography is essential to examine the valve structure, and electrophysiological mapping is useful in concomitant arrhythmia surgery. This article describes the indications, procedures, and perioperative managements of surgery in the right ventricular outflow tract, tricuspid valve, aortic root, Fontan circulation, and systemic right ventricle.

In the recent years, the outcomes of treatment for hypoplastic left heart syndrome (HLHS) is improved because of perioperative management and procedure. We adopt a strategy of the primary Norwood procedure basically for neonates with HLHS, however bilateral pulmonary artery banding precede in patients with risk factors. In the Norwood procedure, aortic arch is reconstructed without artificial prostheses and pulmonary blood flow is supplied from the Blalock-Taussig shunt or the right ventricle-pulmonary artery shunt by case. We administer high dose vasodilators and nitric oxide gas with low resistance strategy after the Norwood procedure. The survival rate of patients with HLHS after 2005 is 84.2% in this strategy.