A 29-year-old woman, who had been diagnosed with Ebstein's anomaly associated with paroxysmal supraventricular tachycardia due to Wolff-Parkinson-White (WPW) syndrome, was referred to our hospital for treatment of congestive heart failure and tachycardia. She had undergone a catheter ablation for WPW syndrome at the age of 28 years. Subsequently, surgical treatment for Ebstein's anomaly was indicated because of persistent symptoms of heart failure due to tricuspid regurgitation (TR). The echocardiogram and pathologic findings corresponded to Ebstein's anomaly of the Carpentier type B classification, with severe displacement of the septal and posterior leaflets resulting in moderate TR. A mobile anterior leaflet of sufficient size without a cleft enabled us to successfully perform Hetzer's procedure. In this procedure, the large mobile anterior leaflet was approximated to the opposing true tricuspid annulus with a mattress suture of 3-0 polypropylene passed from the anterior leaflet annulus to the true tricuspid annulus at the site of atrialized right ventricle near the coronary sinus. The postoperative course was uneventful, and the cardiothoracic ratio reduced from 56% to 48% with mild TR. In this adult case of Carpentier's type B adult Ebstein's anomaly, Hetzer's procedure allowed reconstruction of the tricuspid valve mechanism of “leaflet-to-septum” coaptation at the level of the true annulus by approximating the anterior leaflet. This was, effective in reducing the patient's moderate TR. We conclude that this procedure is a simple and reproducible method for repairing the tricuspid valve in Ebstein's anomaly, especially for cases with a large mobile anterior leaflet.

A 6-month-old baby boy had undergone the Jatene procedure at 4 days. Four months later, catheter intervention (balloon angioplasty) was performed because of severe stenosis at the bifurcation of the pulmonary arteries. Twenty days later, several episodes of cyanosis occurred and he was readmitted. The existence of shunt flow between the sinus of valsalva and the pulmonary bifurcation was detected by echocardiography and examination by 16-row MDCT revealed 2 holes at this site. Under a diagnosis of aortopulmonary (AP) window, the patient was placed on cardiopulmonary bypass and the pulmonary artery was opened after aortic clamping. There was a ridge between the bifurcation of the pulmonary arteries. After removing it, 2 holes were visualized that resembled the findings on 16-row MDCT. These holes were closed with Xenomedica patches and the main pulmonary artery was also extended with a Xenomedica patch. AP window is a rare complication after balloon angioplasty for pulmonary stenosis, but we must take great care to prevent this complication.

We present a rare case of mitral valve prolapse associated with congenital bicuspid aortic valve, followed by abrupt left chordae tendineae rupture resulting in severe left heart failure and cardiac arrested. The patient, a 43-year-old man who had been admitted because of sudden orthopnea suffered cardiac arrest on arrival in the emergency unit. After successful cardiopulmonary resuscitation, emergency double-valve replacement (SJM 25mm for the aortic valve and Carbomedics 31mm for the mitral valve) was performed; his postoperative course was uneventful. Concerning the pathogenesis of the acute rupture of the chordae tendineae in this patient with no evidence of infective endocarditis, it was likely that chronic and progressive left ventricular volume overload due to aortic regurgitation caused by congenital bicuspid aortic valve was the causative factor of abrupt rupture of the chordae tendineae during the course of mild mitral valve prolapse.

Preoperative evaluation of cardiac anatomy is essential to determine the correct surgical procedure for congenital heart disease. Multi-detector row CT (MDCT) is a useful alternative imaging modality to cardiac catheterization and echocardiography. Sixteen patients (12 with total anomalous pulmonary venous return (TAPVR) and 4 with aortic arch anomalies) underwent 16-slice multi-detector row CT scanning. Three-dimensional reconstruction by MDCT was useful to determine the type of TAPVR and the presence of pulmonary venous obstruction (PVO) in TAPVR patients, as well as to detect postoperative PVO in patients who underwent intracardiac repair. In 2 patients who had asplenia associated with TAPVR III and I a, MDCT enabled an intra-atrial approach for TAPVR repair by precise preoperative determination of the relationship between the common PV chamber and single atrium. In patients with aortic arch anomalies, MDCT was useful to determine the type of anomaly, the presence of arch hypoplasia, and any associated rare vascular anomalies (including isolated subclavian artery, and the right-sided descending aorta with left aortic arch). In conclusion, MDCT provides reliable preoperative evaluation of pulmonary venous return and aortic arch anatomy, and therefore is extremely useful for surgical management of congenital heart disease.

We describe a case of postoperative aortic valve regurgitation (AR) after arterial awitch operation (ASO) successfully managed by the modified Konno procedure. A 4-year-old girl with complete transposition of the great arteries (TGA, Type II) had undergone the ASO (LeCompte maneuver) at 10 days of age. Because of progression of moderate AR 4 years after ASO, the modified Konno procedure with aortic valve replacement (SJM 21mm) was successfully performed. She remains in good clinical condition at the last follow-up at 5 years.