Case report
We report the clinical case of a young 50-year-old woman, affected by severe regurgitation of bicuspid aortic valve associated to aortic aneurysm. In the past she had radio and chemotherapy for cancer of the right breast. She was symptomatic for dyspnea for moderate effort (NYHA II) and she had no other cardiac symptom. Transesophageal echocardiography (TEE) showed normal ejection fraction (EF), normal left ventricle diameters and a severely incontinent type I bicuspid aortic valve: the rafe was between right and left coronary cusp and the non-coronary cusp was prolapsing (Figure 1). A coronary artery CT scan angiography reported the absence of coronary stenosis and aortic dilation (49.6 mm as aortic root diameter and 47 mm diameter of the ascending aorta) (Figure 2). According to the current guidelines1, surgical indication for aortic valve repair and root- ascending aorta replacement was posed. The operation was performed though median sternotomy and cardiopulmonary by-pass (CPB) was established through right atrium and distal aortic arch cannulation. Cristalloid cold cardioplegia (Custodiol) was administered directly in the coronary ostia. No anomalies in the origin of the coronary arteries was found and the ostia were normally positioned. Operative inspection confirmed the echocardiographic data: aortic valve was bicuspid, with a coronary cusp resulting from the fusion of the right and the left coronary cusp and a normal non-coronary cusp. Aortic annulus was dilated and measured with Hegar dilator at 28 mm. Aortic valve repair was performed through the excision of the rafe on the coronary cusp; then, plicating stitches on the free edge were put, in order to realign the free edges, aiming for an effective height of 9 mm2. Remodelling of the aortic root with subvalvular external annuloplasty (Cardioroot bulged graft 28 mm and CORONEO® ring 27 mm) was performed2. Weaning from CBP was uneventful and TEE showed a normal functional valve with no insufficiency and no pathological gradients. After weaning, hemodynamical instability suddenly developed: episodes of ventricular fibrillation (FV) were repeatedly shocked and ST elevation was reported in the inferior leads. Hemodynamic deterioration was not improved by intra-aortic balloon pump (IABP) and CBP was restarted. A venous graft was used to bypass the right coronary artery, but unsuccessfully. As the operative theatre was hybrid, coronary angiogram was logistically easy and it was promptly performed. Angiography showed massive coronary spasm (Figure 3), not responsive to intracoronary nitrates, so balloon angioplasty and drug eluted stents were placed in the right, circumflex and anterior descending coronary arteries (Figure 4). CPB weaning was then possible with inotropic support and the patient was moved to the ICU. During the first postoperative day the maximum troponin release was 2732 ng/l (14-50 cut off). Hemodynamical conditions gradually improved and in second postoperative day IABP removal and extubation were possible. Inotropic drug support was step by step reduced e definitely stopped at the 72nd postoperative hour. In fourth postoperative day the patient was moved to the ward and discharged home in a few days. The echocardiography before discharge showed no incompetence, no pathological gradients on the aortic valve and a normal ejection fraction (Figure 5), a coronary artery CT scan angiography showed excellent result with no residual coronary artery stenoses (Figure 6). At follow-up outpatient visits, at one and six months, the patient was fine and asymptomatical.