Patient Profile
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.
Initial echocardiography 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.
Cardiopulmonary bypass was established between the distal ascending
aorta and the vena cavae after a median sternotomy. 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. An intraoperative transesophageal
echocardiogram was also performed, revealing 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.
The patient recovered from the surgery uneventfully and remained well
upon last follow-up.