HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

History
No family history of heart disease or sudden death. Personal history:
Cardiovascular risk factors (CVRF): grade I obesity. No known hypertension, dyslipidaemia or diabetes mellitus.
Cardiological history:
On outpatient follow-up for cardiac amyloidosis type AL (diagnosed in December 2018) in NYHA functional class I/IV.
Pyrophosphate cardiac scan (Tc99m-DPD): pathological radiotracer deposit was observed, with biventricular distribution and intensity greater than that of the adjacent bone (grade 3), compatible with cardiac amyloidosis.
Transthoracic echocardiogram: slightly hypertrophic left ventricle with preserved LVEF. Mild mitral insufficiency without other significant valvulopathies. No pericardial effusion.

Other history:
Known chronic hepatitis C since 1991. Genotype 1b. Has received paritaprevir/ritonavir/ombitasvir 150/100/25 mg one tablet daily plus dasabuvir 250 mg twice daily in May-August/2015, with sustained virological response (cured infection).
AL amyloidosis with histological confirmation. Diagnosis in December 2018 with cardiac and renal involvement; 6 cycles of bortezomib, cyclophosphamide and dexamethasone are planned, on admission she is on her fourth cycle. A renal biopsy was performed, with the result of AL amyloidosis. After renal biopsy, complication with retroperitoneal haematoma requiring embolectomy.

Usual medication:
Omeprazole 20 mg 1-0-0, acyclovir 800 1-0-0, hydroferol 1/15 days, allopurinol 100 1-1-1, formoterol/budesonide 160/4.5 mcg 1-0-1, furosemide 40 mg 1-1-0.
Chemotherapy treatment of myeloma with: CyBorDex (cyclophosphamide + bortezomib + dexamethasone).

Current disease
A 63-year-old male patient with the aforementioned history, who while admitted to the haematology department for febrile syndrome, under active treatment for multiple myeloma, presented with a condition characterised by palpitations. When vital signs were taken, an arrhythmic and rapid pulse curve was observed, and an electrocardiogram (ECG) was requested, confirming the suspected diagnosis. In view of the ECG findings, a cardiology assessment was requested. She denied current or previous chest pain.

Physical examination
Conscious and oriented in person, time and place. Collaborative, eupneic at rest and afebrile.
Cardiac auscultation: arrhythmic heart sounds, tachycardic, systolic murmur II/VI in mitral focus not radiating.
Pulmonary auscultation: preserved vesicular murmur without added sounds.
Abdomen: soft and depressible. No pain on superficial or deep palpation, no visceromegaly. Preserved hydro-aerial sounds.
Lower extremities: no signs of deep vein thrombosis (DVT). Palpable paedial pulses. No oedema.

COMPLEMENTARY TESTS
Blood count: haemoglobin 14.2 g/dl. Red blood cells 5.47 x10/μl. Haematocrit 42.3%. MCV. 84.3 fl.
Leukocytes 3.24 x10 /μ. Neutrophils (blood-%) 58.7%. Lymphocytes (%) 33.1%. Platelets 123 x10 .
Biochemistry: glucose 111 mg/dl. Urea 91 mg/d. Creatinine 1.52 mg/dl. Estimated glomerular filtration rate 48 ml/min/m . Sodium ion 142 mmol/. Potassium ion: 4.1 mmol/l. NT-proBNP 5,174 ng/d. (VN <350). C-reactive protein 0.2 mg/dl.
Serial myocardial damage markers during admission (48 hours): creatine kinase: 61--> 49 U/l. Ultrasensitive troponin T: 24--> 26 ng/l.
Coagulation: T. prothrombin 13.00 sec. T. partial thromboplastin (R. 0.98 T. prothrombin (%) 82.0%. Derived fibrinogen 477. Prothrombin T. (ratio 1.14). INR 1.14 T. partial thromboplastin 30.90 sec.
ECG: AF at 150 L/min, narrow QRS, low voltages in limb leads, abnormal R wave progression in precordial leads, normal axis, inverted T waves in high lateral face.
Chest X-ray: normal cardiothoracic index, no pinched costophrenic angles or increased vascular pattern.
Transthoracic echocardiogram: normal sized left ventricle with mild hypertrophy (interventricular septum thickness 14mm), mild global hypokinesia resulting in an
LVEF 40%. Right ventricle (RV) slightly dilated with mild hypokinesia of the free wall, moderate low functional mitral insufficiency and moderate tricuspid insufficiency with estimated PSAP of 33mmHg + PVC.

Previous complementary tests of interest: pyrophosphate scintigraphy (Tc99m-DPD) (image 2): a pathological deposit of radiotracer was observed, with biventricular distribution and intensity greater than that of the adjacent bone (grade 3), compatible with cardiac amyloidosis.

CLINICAL EVOLUTION
A 63-year-old male patient was admitted to the haematology department for febrile syndrome, being at the time of admission under active treatment for multiple myeloma with multiple organ involvement and presenting with an episode of atrial fibrillation. The patient commented that the symptoms had started suddenly 2 hours prior to our assessment. An ECG was performed showing AF with a previously unknown rapid ventricular response. Given that the patient clearly noticed the onset of symptoms and that vital parameters had been within normal limits in the previous 24 hours (heart rate around 60-80 per minute), it was decided to manage the patient with rhythm control.
After bolus and infusion amiodarone, the patient recovered sinus rhythm about 5 hours later. Treatment with oral amiodarone and beta-blockers was discontinued. An echocardiogram was performed (already in SR) showing a slightly hypertrophic left ventricle with normal size and function, with left atrial enlargement, normal right chambers, low moderate mitral regurgitation and minimally elevated PSAP. The patient progressed satisfactorily and did not develop signs or symptoms of congestive heart failure.
The management of anticoagulation with this patient, having a CHADS2-VA2SC of 1 (CHF) was a challenge, as the patient is not adequately represented in current clinical practice guidelines. Being a patient with this score on the CHADS2-VA2SC scale and having AL cardiac amyloidosis as comorbidity, it was decided, in consensus with the haematology service, to initiate anticoagulation treatment with rivaroxaban, as he was a patient at high thromboembolic risk.

DIAGNOSIS
AL cardiac amyloidosis.
Paroxysmal atrial fibrillation, pharmacological cardioversion. CHADS2-VA2SC 1 point. Moderately impaired LVEF.
Moderate low mitral insufficiency.
Multiple myeloma with cardiac and renal involvement in active treatment. Febrile syndrome in patient with mild bicytopenia.
Chronic renal disease secondary to monoclonal gammopathy.
