HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
70 year-old female patient admitted for the onset of heart failure.

History
No known drug allergies.
Cardiovascular risk factors: no toxic habits. Arterial hypertension (AHT) and dyslipidaemia without pharmacological treatment. No family history of sudden death or early ischaemic heart disease.
Cardiological history: assessed by the primary care physician for dyspnoea, initiating treatment with spironolactone and furosemide, pending outpatient assessment by the cardiology team.
Other antecedents: hypothyroidism on replacement therapy. Monoclonal gammopathy of uncertain significance studied by haematology in 2009, ruling out multiple myeloma.
Colonic diverticulosis. Colonic polypectomy in 2016 (polyps with low-grade dysplasia).
Partial gastrectomy for duodenal ulcer and partial adnexectomy for endometriosis.
Usual treatment: spironolactone 25 mg every 48 hours, omeprazole 20 mg, furosemide 40 mg (1-0-0), calcium carbonate/cholecarciferol.

Present illness
The patient reports that in the two months prior to admission, she began with a sensation of dyspnoea on exertion that progressively worsened until it became minimal exertion.
She also reports that since the week prior to admission she has had oedema in the lower limbs. She denies chest pain, palpitations or syncope. She reported that for several years she had been suffering from periorbital haematomas that resolved spontaneously, for which she had not consulted. Since the week prior to admission, he had again presented haematoma around both orbits. He came to the emergency department of our centre and it was decided to admit him for treatment and study.

Physical examination
On admission:
Baseline O2 saturation 89%. Blood pressure 110/70 mmHg. Heart rate 110 bpm. Jugular venous pulse (JVP) elevated, hepatojugular reflux. Cardiac auscultation: rhythmic, left parasternal systolic murmur II/VI. Pulmonary auscultation: bibasal crackles. Abdomen: soft, depressible, non-painful hepatomegaly of 2 cm. Lower extremities: oedema up to the pretibial region. Bilateral periorbital purpura, especially on the left.

COMPLEMENTARY TESTS
Electrocardiogram (ECG): sinus rhythm at 90 bpm. First degree BAV (PR 220 ms).
Narrow QRS. Anterior pseudoinfarction pattern (QS V1-V4). HBAI.
Blood tests:
General biochemistry: glucose 99 mg/dL, urea 47 mg/dL, creatinine 1.02 mg/dL, glomerular filtration rate (CKD-EPI) 56 ml/min/1.73 m2, sodium 142 mmol/l, potassium 4.1 mmol/l. AST 33 IU/l, ALT 43 IU/l, ALP 126 IU/l, GGT 127 IU/l, HbA1C 5.9%, TSH 6.3 mIU/l, free T4 1.31 ng/dl. Lipid profile: total cholesterol 211 mg/dl, triglycerides 102 mg/dl, HDL 49 mg/dl, LDL 142 mg/dl.
NT-proBNP 4828 pg/ml. Troponin T ultrasensitive 72 ng/l.
Ferrokinetics: iron 75 μg/dl, transferrin 315 mg/dl, IST 24%, ferritin 92 ng/ml.
Haemoglobin 14.6 g/dl, leucocytes 8,300/μl, platelets 292,000/μl, reticulocytes 1.5%, haptoglobin 44%.
Coagulation: INR 1.09, prothrombin rate 87%, activated partial T.T. 35.9 sec.
Immunoglobulins: serum free kappa chains 7.7 mg/l (reference values 3.3-19), serum free lambda chains 517 mg/l (5.71-26.3), free Kappa/Lambda ratio 0.01 (0.26-1.65), free Lambda/Kappa ratio 67.
Proteinogram: IgG Lambda monoclonal band in late gamma + additional monoclonal component in mid-gamma also of Lambda light chain with no clear association to IgG, IgA or IgM heavy chain.
24-hour urine: creatinine clearance 23.31 ml FG. Quantitative protein 2.4 mg/dl, urea 7.31 g/24 hours, creatinine 0.34 g/24 hours. Kappa chains 0.66 mg/dl (0-0.75), lambda chains 1.68 (0-0.41), kappa/lambda ratio 0.39. Concentration of urine x20 shows elimination of monoclonal component of lambda free light chain in gamma zone. Detection limit of the technique: anti-IgG+IgA+IgM, anti-kappa, anti-lambda 1.25 mg/l, free anti-kappa, free anti-lambda 2.5 mg/l.
Chest X-ray (PA and lateral): cardiomegaly at the expense of left cavities, flow cephalisation, Kerley lines A and B, left pleural effusion, right costophrenic sinus impingement.
Transthoracic echocardiogram: left ventricle with severe asymmetric hypertrophy (interventricular septum 13 mm, posterior wall 16 mm) with preserved ejection fraction (60% by Simpson biplanar). Hyper-refringent myocardium, suggestive of deposition cardiomyopathy. Type III diastolic dysfunction. Hypertrophic right ventricle with normal function (TAPSE 22 mm). Mild biauricular dilatation (left atrium 20 cm2, right atrium 18 cm2).
Mitral insufficiency grade 2+. Moderate tricuspid insufficiency with evidence of PHT at rest (PSAP 40 mmHg + PVC). Dilated inferior vena cava (25 mm), with respiratory variation < 50%.
Cardiac MRI: left ventricle of normal size (46 mm) with thickening of the basal anterior wall (16 m), basal lateral wall (13 mm), medial septum (14 mm) and apical septum (10 mm). No alterations in global or segmental contractility were identified. LVEF 58%. LV-DVT 109 ml (69 ml/m2). LV-STV 46 ml (29 ml/m2). LV-SVL-VI 64 ml (40 ml/m2). Right ventricle of normal size with diffuse myocardial thickening, mainly of its free wall. Function preserved. Thickening of the interatrial septum. Slight biauricular dilatation (RA 21 cm2; LA 22 cm2). After intravenous contrast administration, the myocardial signal is not annulled, identifying diffuse enhancement of the myocardial wall of the left ventricle, the right ventricle, the interatrial septum and the wall of both atria. Slight bilateral pleural effusions.
Myocardial scintigraphy with diphosphonates: the study shows no pathological uptake compatible with amyloidosis due to transthyretin deposition.
Cardiac catheterisation: coronary arteries without angiographically significant stenosis.
Fine needle puncture of abdominal subcutaneous fat: adipose tissue with the presence of nodular deposits that stain with Congo red stain and show green birefringence in the study under polarised light. Immunohistochemistry: lambda positive. Kappa, A-component, P-component and transthyretin negative. The result of the immunohistochemical study is in favour of AL amyloid.

CLINICAL EVOLUTION
Patient with no known cardiological history, who had previously presented with periorbital purpura, and who consulted for clinical symptoms of progressive dyspnoea of weeks' evolution.
She attended the emergency department and was admitted to the cardiology hospital ward for the first episode of heart failure with evidence of systemic and pulmonary congestion. After an echocardiogram suggestive of deposit disease, it was decided to complement the study with magnetic resonance imaging, cardiac scintigraphy and light chains in plasma and urine. After obtaining the results, a referral was made to the haematology department, which requested an abdominal subcutaneous fat puncture. From the clinical point of view, she responded well to intravenous diuretic treatment; however, she had a tendency to hypotension, and in view of the electrocardiogram with baseline conduction disorder, it was decided not to start treatment with ACE inhibitors or beta-blockers.
She was discharged stable, in NYHA functional class II/IV, pending anatomopathological results of subcutaneous fat.
Two weeks after admission, she returned to the emergency department for chest pain and decompensation of heart failure, and was again admitted to the cardiology ward. Cardiac catheterisation was performed, ruling out associated coronary disease. The anatomopathological result of the subcutaneous fat was in favour of AL amyloid. Diagnosed with AL amyloidosis, haematology started treatment with cyclophosphamide, bortezomib and dexamethasone.
She remained at home, stable from the cardiovascular point of view, with outpatient monitoring by haematology.

Three months after his first admission, he had an ischaemic stroke of the left hemisphere in the left middle cerebral artery with an image compatible with a small thrombus at the level of the left M1, and was admitted to neurology. Paroxysmal atrial fibrillation was detected and anticoagulation with heparin and acenocoumarol was started, with lower gastrointestinal bleeding. A gastroscopy was performed with no relevant findings and colonoscopy revealed haemorrhoids. During admission she developed critical patient neuropathy and was transferred to a rehabilitation centre for treatment with low molecular weight heparin, pending outpatient percutaneous closure of the appendage.

DIAGNOSIS
AHA/ACC stage C congestive heart failure, with pulmonary and systemic congestion.
AL amyloidosis with cardiac involvement.
Left hemispheric stroke of probable cardioembolic origin.
Paroxysmal atrial fibrillation.
Polyneuropathy in critically ill patients.
Preserved left ventricular systolic function. Restrictive pattern diastolic dysfunction.
Mitral insufficiency grade 2+.
Stage 3a chronic kidney disease.
