We report the case of a female patient, 3-years-old, with a history of bileaflet aortic valve, critical aortic valve stenosis, hypoplasia of the aortic arch, coarctation of the aorta, perimembranous ventricular septal defect and a patent ductus arteriosus. During newborn period, at 23 days of age, a balloon aortic valvuloplasty was performed to manage critical aortic stenosis; 6 days later, reconstruction of the ascending aorta and arch was performed. At 2 months of age, pulmonary artery banding was performed to manage pulmonary overflow due to the different left-to-right shunts,.
After a period of 24 months without institutional follow-up, she presented once more to our institution with symptoms of atypical chest pain, dysphonia, hoarseness and worsening functional class. Due to her history, she was admitted for studies and a chest x-ray showed significant mediastinal widening, a cardiothoracic index of 0.65, and an enlarged pulmonary hilum with cephalic redistribution (See Fig. 1).
An echocardiogram was performed with findings of dysplastic aortic valve with bileaflet opening and severe dilation of the ascending aorta. Measurements included an aortic root of 16 mm (Z score + 0.13), a sinotubular junction of 20 mm, a proximal ascending aorta of 15 mm (Z score + 0.63) and an apparent ascending aorta aneurysm of 40x70 mm with an inner-thrombosed area and spontaneous contrast. (see Fig. 2).
A chest angiotomography was performed where an intimal tear hole measuring 16 mm in diameter (type A aortic dissection) was found 40 mm above the valvular plane, with false lumen perfusion that generated a saccular pseudoaneurysmal dilation measuring 53x40x70 mm. The pseudoaneurysm produced compression of the pulmonary artery, left main bronchus and left upper lobe bronchus with signs of air trapping (see Fig. 3).
After these diagnostic studies, it was decided to undergo surgical correction. In the operating room, cervical cannulation was started, systemic heparinization was initiated and carotid cannulation was performed. Cardiopulmonary bypass circulation was started and 20°C hypothermia was achieved; after the sternotomy was performed, an aneurysmal sac rupture was observed and supraortic vessels are repaired. Antegrade cerebral perfusion was initiated. A competent dysplastic bicuspid aortic valve was observed, a Hegar passage 11 Z Score 0 is performed, the aorta was reconstructed with a 22 mm supracoronary Dacron tube, anastomosis is continued with a Dacron tab, reconstructing the arch, and the supraortic arches were anastomosed with an island technique (see Fig. 4). The biopsy revealed diffuse interstitial degenerative changes in the tunica media. Medical therapy was adjusted and she was discharged with strict follow-up by Pediatric Cardiology, Genetics and Pediatrics.