CASE PRESENTATION
A 41-year-old Africo-Caribbean female with a body mass index of 29 kg/m2 presented to the emergency department with acute pulmonary oedema and increasing dyspnea on exertion with New York Heart Association (NYHA) class 3 symptoms. The patient initially experienced an episode of sudden onset tight central chest pain while walking, followed by four weeks of fatigue and malaise and two weeks of exertional breathlessness, orthopnea and a productive nocturnal cough. She had been treated empirically with antibiotics for a chest infection, but her symptoms deteriorated prompting presentation to her local hospital. The patient had essential hypertension with subsequent chronic kidney disease and pre-eclampsia during her pregnancy. She had no previous history of congenital disease, trauma, or positive family history for aorthopathy.
Her vital parameters were stable and she was apyrexial. She had bilateral basal lungs crepitations. The patient’s electrocardiogram was normal. Her leukocyte count was 8.4×10⁹/L, D-Dimer 2522 ng/mL, troponin 13.2 ng/L, Covid-19 swab test and blood cultures were negative. Chest radiograph showed pulmonary oedema with bilateral pleural effusions. Transthoracic echocardiography (TTE) demonstrated a normal size left ventricle with hyperdynamic systolic function and severe aortic regurgitation (AR) with a central jet. There was an appearance of a trileaflet aortic valve (AV) with failure of coaptation along the right coronary/noncoronary cusp (RCC/NCC) closure line (Figure 1). The coaptation defect was associated with a linear echogenic structure attached to the RCC, running along the closure line. This linear and thinned structure was mobile (Figure 1). No prolapse of the AV leaflets was demonstrated and the aortic root was mildly dilated with no signs of dissection flap. Transesophageal echocardiography was attempted, although the patient refused to have it. A prospectively gated computed tomography (CT) aortogram showed pulmonary oedema with an asymmetrical aortic root dilatation with increased RCC-LCC diameter (maximum 45 mm) and no features of aortic dissection or pulmonary embolus (Figure 2). Given the history of acute severe AR and treatment for chest infection, the initial concerns were for infective or non-infective endocarditis (Libman-Sacks endocarditis).
The patient was transferred to a tertiary cardiothoracic surgical center for urgent surgical treatment in view of acute AR. After median sternotomy, cardiopulmonary bypass (CPB) was initiated with cannulation of the distal ascending aorta and right atrium with mild hypothermia (34°C). Intermittent antegrade cold blood cardioplegia were delivered. After atriotomy, a supraannular aortic intimal tear starting from the RCC/NCC commissure was found, which did not extend into the ascending aorta (Figure 3). The AV on direct inspection was bicuspid with an RCC/NCC raphe involved in the intimal tear. Aortic root and ascending aorta were found dilated with a diameter up to 45 and 40 mm, respectively. Severe AR not amenable to repair was found. Bentall procedure was performed using the 23/26mm Carbomedics Carbo Seal Valsalva composite graft (Sulzer Carbomedics Inc, Austin, TX, USA). The patient was weaned from CPB with no inotropic support. CPB and clamp time were 109 and 86 minutes, respectively. Patient was anticoagulated with warfarin and had uneventful postoperative recovery. Valve tissue samples and blood cultures were negative. Histopathological examination revealed focal mucinous degeneration in the aortic root media with no connective tissues disorder. She was discharged on the 7th postoperative day with a satisfactory TTE and remained well during a 6-month follow-up. Informed consent and patient’s permission were obtained to report this case.