HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
In May 2017, an 84-year-old man was admitted to the Cardiology Department of the CAULE due to syncope. His personal history included long-standing hypertension under treatment, dyslipidaemia, and chronic kidney disease being monitored by Nephrology. He denies drug allergies and previous cardiological history. On regular antihypertensive treatment with torasemide 10 (1-0-0). He came to the Emergency Department of our hospital due to a syncopal episode on the night of admission, while sitting, with a slight sensation of blurred vision beforehand, and fell to the floor. Complete recovery within seconds. Subsequently presented with dizziness lasting 20 minutes. He denied chest pain or other accompanying symptoms. He reported increased dyspnoea with moderate exertion in the previous days.

Physical examination showed: BP 130/89, baseline SATO2 94%, HR 103 bpm, AC: arrhythmic at 110 bpm, systolic murmur in aortic focus II/VI with 2nd noise preserved. AP: bibasal crackles. Lower extremities without oedema, positive and symmetrical peripheral pulses.

COMPLEMENTARY TESTS
ECG: AF at 115 bpm, narrow QRS with negative T wave from V4 to V6, II, III and aVF. CBC: leukocytes 4,700 (87 % neutrophils), haemoglobin 1.3, haematocrit 34.4 %, platelets 383,000, prothrombin rate 83 %, INR 1.1, glucose 163, urea 74, creatinine 1.38, glomerular filtration rate 49, Na 141, K 4.4, Cl 104, Nt-proBNP 9233. Chest X-ray: cardiomegaly, bilateral interstitial pattern, free costophrenic sinuses. Transthoracic echocardiogram: LV with severe hypertrophy, non-dilated with apical akinesia with preserved global systolic function. Elevated filling pressures. Moderate LA and mild RA dilatation (suggesting cardiac amyloidosis). Mild mitral insufficiency. Aortic sclerosis without significant gradient and mild insufficiency. Mild tricuspid insufficiency with moderate-severe pulmonary hypertension. Mild-moderate pericardial effusion with no evidence of haemodynamic compromise. Haemodynamic study: coronary arteries with tortuosity and irregularities, without angiographically significant stenosis. Right dominance. Normal sized LV, slightly hypertrophic, with preserved LVEF (58%), with very localised apical akinesia. Mild mitral insufficiency. LVEDP 13 mmHg. Slight calcification of aortic valve leaflets, especially the left coronary, without significant transvalvular gradient. Magnetic resonance imaging (MRI): LV of normal size (38 mm) with asymmetric thickening of septal predominance in the middle and apical segments. Signal hyperintensity on T2-weighted sequences in the apical wall suggesting oedema. Heterogeneous late enhancement, transmural in the inferior wall and in subendocardial location in the anterior wall. There is also enhancement in both atria and the interatrial septum. Severe pericardial effusion. Slight bilateral pleural effusions. The findings described suggest amyloidosis.

Myocardial scintigraphy with 99mTc-HDMP: chest study (late phase, planar and tomographic). Diffuse and homogeneous activity deposit of remarkable intensity is observed in the wall of both cardiac ventricles. Intense foci of uptake at the anterior ends of several ribs on both sides. Conclusion: findings consistent with the diagnosis of ATTR type cardiac amyloidosis.

EVOLUTION
The patient was admitted to the Cardiology hospital ward with telemetry monitoring on suspicion of syncope with a cardiogenic profile and clinical signs of heart failure, for the study of structural heart disease. Telemetry monitoring showed AF with MVR around 140 bpm, without significant pauses or arrhythmic events. A transthoracic echocardiographic study was started and completed with a haemodynamic study due to suspicion of a possible ischaemic aetiology. The echocardiographic study describes concentric hypertrophy with normal LV size, without segmental alterations of contractility, with severe LA dilatation and mild mitral and aortic insufficiency. Catheterisation showed coronary arteries without angiographically significant stenosis and localised apical akinesia. At this point, the clinical evolution during admission and test findings suggest CHF with preserved ejection fraction, with AF with rapid MVR and diastolic dysfunction. Diuretic treatment was intensified and MRI with gadolinium was requested to continue the study, given the suspicion of cardiomyopathy, in which normal-sized left ventricle with asymmetric thickening of predominantly septal in the middle and apical segments, with heterogeneous late enhancement, transmural in the inferior wall and in the subendocardial location in the anterior wall, suggestive of amyloidosis, was reported. In view of these results, myocardial scintigraphy with 99mTc-HDMP was requested, and the findings were consistent with the diagnosis of ATTR type cardiac amyloidosis. In the following days, the patient progressively improved his clinical symptoms of heart failure, so he was discharged home after pharmacological adjustment for outpatient clinical follow-up in cardiology consultations.

DIAGNOSIS
Syncope in the probable context of arterial hypotension.
Heart failure with preserved EF.
ATTR cardiac amyloidosis.
Coronary arteries without angiographically significant stenosis.
