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.