Personal history
We present the case of a 56-year-old Caucasian woman, administrative profession. She had no known cardiovascular risk factors, except obesity. Her cardiological history included an admission one year earlier for an episode of unstable angina with cardiac catheterisation that revealed a critical lesion of the ostial left main coronary artery (LMCA), on which a drug-eluting stent was implanted, with no significant lesions in the rest of the coronary arteries. The transthoracic echocardiogram (TTE) performed during this admission showed a left ventricle of normal size and thickness, with normal global systolic function and segmental contractility and no significant valvulopathies. His medical history included a diagnosis of autoimmune hepatitis since 1998, for which he was receiving chronic treatment with immunosuppressants (azathioprine) in addition to bisoprolol 2.5mg/24 hours, lansoprazole, acetylsalicylic acid, clopidogrel and atorvastatin 20 mg.

Current illness and physical examination
In the last few months, the patient reported symptoms of asthenia and dyspnoea, without chest pain, with progressive worsening of the functional grade up to NYHA functional grade III/IV, for which reason a stress test had been requested. The exercise test was stopped early due to hypertensive crisis with dyspnoea and electrical positivity. That same day, hours after the exercise stress test, he attended the Emergency Department of our hospital due to a sudden episode of dyspnoea and acute pulmonary oedema. On arrival at the ED he had a BP in MSI of 95/60 and in MSD of 146/70 mmHg, a heart rate of 85 bpm, a baseline saturation of 92% and was afebrile. On cardiac auscultation he had rhythmic heart sounds, with aortic diastolic murmur III/IV and mitral systolic murmur III/VI. He had bilateral crackles up to the upper fields with no oedema in the lower limbs. In the upper limbs, there was a decrease in radial and brachial pulses in the left upper limb (MSI) compared to the right upper limb (MSD).

COMPLEMENTARY TESTS
Blood count: Hb 12.9g/dl, leukocytes 10,5000 (57% N), platelets 480,000. Coagulation: PT 98%, FD 700. Urgent blood biochemistry: Glc 158 mg/dl, U 39 mg/dl, Cr 0.98 mg/dl, Na 139 mmol/l, K 4 mmol/l, CK 172 U/l, TnT (ultrasensitive, upper limit 35 ng/l): 27 ng/l. Complete biochemistry: Glc 89 mg/dl, U 46 mg/dl, Cr 0.83 mg/dl, eGFR >60ml/min/1.7, urate 7 mg/dl, Na 141 mmol/l, K 4 mmol/l, Ca 2.36 mmol/l, phosphate 1.51 mmol/l, CK 65 U/l, LDH 178 U/l, FA 57 U/l, ALT 11U/L, BrT <1 mg/dl, total protein 66 g/l, creatinine 133 mg/dl, CRP 2.2 mg/dl. Immunological study: positive ANAs autoantibodies at 1/320 titre, with nucleolar pattern. The rest was normal.

Electrocardiogram: sinus rhythm, left anterior hemiblock and subepicardial ischaemia in the anterolateral region.
Chest X-ray: compatible with acute pulmonary oedema.
Transthoracic and transesophageal echocardiogram: left ventricle slightly dilated and hypertrophic, with moderately depressed systolic function (LVEF estimated by Simpson at 42%) at the expense of a global hypokinesia, more marked in anterior and lateral regions. The aortic valve was trivalve, with no evidence of vegetation or prolapse and presented severe aortic insufficiency with a central regurgitant jet originating at the level of the entire commissure between the non-coronary and left coronary leaflets with coaptation defect of the leaflets, suggesting leaflet retraction at this level as the underlying mechanism. The aortic root was of normal size. At mitral valve level a mitral insufficiency (MI) grade 2 was detected and at tricuspid level a severe tricuspid insufficiency (TI) which estimated a pulmonary artery systolic pressure of approximately 80 mmHg.
Cardiac catheterisation: occlusion of the right coronary artery at the ostial level which was filled by collateral circulation. The patency of the stent previously implanted at the level of the left main coronary artery persisted.
Angio-CT of the thoracic aorta: thickening of the arterial wall of the common carotid arteries and of the left subclavian artery, which is occluded with filiform tracts affecting the occlusion at the origin of the left vertebral artery, and of the superior mesenteric artery, which is also occluded with hypertrophy of the pancreaticoduodenal arcade. There is no calcium deposit in any of the lesions, ulcerations, dissection or aneurysm.
Infectious serology: HBsAg, anti-HCV, HIV Ag+Ac and Syphilis G+M negative.
Biopsy (aortic valve): the sample submitted as aortic valve with presence of myxoid degenerative changes and chronic lymphocytic inflammatory infiltrate.

EVOLUTION
We present the case of a 56-year-old woman with a cardiac history of ostial LMCA disease revascularised percutaneously with a drug-eluting stent one year earlier, who was admitted with acute pulmonary oedema. Physical examination of the patient revealed the presence of murmurs in aortic and mitral foci, suggestive of valve disease at these levels, which were not present in the echocardiogram of a year earlier, as well as a clear decrease in pulses in MSI together with a significant discordance between systolic blood pressure between both upper limbs (BP in MSI of 95/60 and in MSD of 146/70 mmHg). In addition to a more marked global hypokinesia in the anterior and lateral regions, the presence of severe aortic insufficiency (AIo), the mechanism of which appeared to be a coaptation defect of the non-coronary and left coronary leaflets due to their retraction, with a normal-sized aortic root. Given the ischaemic alterations in the ECG on admission and the segmental alterations in contractility in the echocardiogram, coronary angiography was performed in the following 24 hours, which showed the previous stent in the IVC to be permeable and without restenosis, and occlusion of the ostial right coronary artery, which at that time was pending a decision on revascularisation strategy. Given the findings consisting of asymmetry in pulses and blood pressure in both upper limbs, ostial coronary lesions and severe aortic insufficiency of subacute onset, together with a history of autoimmune involvement and in a patient without CVRF, an aortic CT angiography was requested with suspicion of aortitis, in addition to complete immunological studies, acute phase reactants (APR) and serology. Angio-CT showed thickening of the arterial wall of both common carotid arteries and left subclavian, left vertebral and superior mesenteric arteries. A diagnosis of aortitis in the context of large vessel vasculitis was established. Treatment was started with glucocorticoids at a dose of 1.5 mg/kg with a subsequent tapering regimen, and the dose of azathioprine that the patient was taking was doubled. After presenting her case at a medical-surgical session (Heart Team), surgical treatment was decided once the patient was stabilised, with resolution of the acute pulmonary oedema. After 10 days of treatment with oral glucocorticoids, the aortic valve was replaced with a metallic prosthesis no. 21 and coronary revascularisation by means of aorto-coronary bypass from the saphenous vein to the middle anterior descending artery and proximal right coronary artery. During surgery, the aorta was also affected, with marked thickening of both the aortic wall and the wall of the coronary arteries, as well as friability of these tissues and the aortic annulus. After the procedure, she presented haemodynamic instability, requiring high-dose amines and intra-aortic balloon counterpulsation. 24 hours later the patient was in cardiogenic shock related to perioperative infarction. She presented a slow evolution in the following days with poor tolerance to amine weaning and removal of the balloon with severe haemodynamic instability and progression to multi-organ failure, and finally exitus.

DIAGNOSIS
Vasculitis of large vessels of the Takayasu arteritis type.
