2| CASE PRESENTATION
A 35-year-old male previously diagnosed to have TOF at the age of 5 but did not undergo any intervention for an unknown reason, presented with dyspnea after physical exercises and occasional cyanosis of the lips. He reported no symptoms of chest pain, palpitation, amaurosis, syncope, swelling of limbs, or squatting position. His other past medical history included pulmonary tuberculosis that was fully treated at the age of 25, and an abdominal angioma diagnosed approximately 4 weeks prior to his hospitalization in our institution.
The physical examination revealed evidence of cardiac arrhythmia, tremors, grade 3/6 systolic ejection murmur at the second intercostal space and the left margin of the sternum, grade 4/6 systolic blowing murmur at the fourth intercostal space and the left margin of the sternum. Echocardiography was performed which showed unoperated TOF. A dilated right atrium measuring 52 mm in diameter was evident, while the right ventricle appeared hypertrophic with a thickness of 14 mm. The infundibulum of the right ventricular outflow tract appeared severely narrowed with a diameter of 8 mm. The pulmonary artery measured 18 mm in diameter and the pulmonary artery valves were slightly restrictive. The left ventricle appeared normal in size and the ejection fraction was 73%. The aorta seemed to have moved forward and 50% of its area covered the interventricular septum. Furthermore, there was dilation of the aortic root (55.4 mm) (Fig.1) and a mild central AR (VC = 5.7 mm) (Fig.2) . The right coronary artery showed evidence of an anomaly with a diameter of 7.5mm. Moreover, there was a secundum atrial septal defect showing bidirectional low-speed shunt and a 15 mm perimembranous ventricular septal defect (VSD). Given the above findings, surgery was recommended to correct the above abnormalities.
In surgery, a median sternotomy was performed and a total cardiopulmonary bypass was established between the distal ascending aorta and the vena cavae. Myocardial protection strategies including systemic hypothermia at 32℃, antegrade cold cardioplegia, and consistent retrograde cardioplegia through coronary sinus were applied. The right ventricular outflow tract was incised longitudinally and the excessive infundibular muscle was resected. The VSD extending towards the infundibular and perimembranous portion of the right ventricle was clearly visualized. An autologous pericardium was harvested for the closure of the VSD and secured with continuous sutures. The aortic root was then transected to explore the aortic annulus and aortic valves. There was no thickening or calcification found, and all the leaflets appeared equally. Following that, both the coronary arteries were freed, and all the aortic sinuses with the entire ascending aorta were resected. A 3 mm high aortic valvuloplasty ring was made out of a 26 mm artificial vessel. Then, the valvuloplasty ring, aortic root, and the proximal end of the artificial vessel were assembled by continuous suturing to reconstruct the Valsalva sinus. An end-to-end anastomosis was performed between the ascending aorta and the distal end of the artificial vessel with running sutures. Openings of 8 mm in size were made on the artificial vessel and both the coronary arteries were then anastomosed to the openings with continuous suturing using 5-0 Prolene. An autologous pericardium was utilized as a patch to enlarge the pulmonary artery trunk. Partial closure of the atrial septal defect was performed using running sutures, leaving a small remaining 3 mm opening. Upon completion of the surgical procedures, patient rewarming, lung recruitment, discontinuation of cardiopulmonary bypass, and cardiac resuscitation were uneventful. An intraoperative transesophageal echocardiogram was performed, which revealed trivial central AR and an unobstructed left ventricular outflow tract. Postoperatively, the right ventricular systolic pressure measured 45 mmHg, the pulmonary arterial pressure was 18 mmHg, and the peripheral blood pressure was 88/45 mmHg.