HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
The patient is a 61-year-old male who consults for dyspnoea.

History
No known drug allergies. Ex-smoker. Ex-drinker. Hyperuricaemia with gouty crisis. HYPERTENSION. Dyslipidaemia. Long-standing type 2 diabetes mellitus, under treatment with oral anti-diabetics and insulin, with good control. Stage III chronic kidney disease. Cholecystectomy in 2012 for acute calculous cholecystitis. Repeated nephritic colic, requiring admission to the ICU in one of the episodes. Anxious-depressive syndrome being monitored by Psychiatry, with one admission for decompensation in 2005, and subsequently good clinical control with medication.
In 2010, he was referred to Pneumology for dyspnoea and a pathological chest X-ray, being diagnosed with sarcoidosis after completing the study with a high-resolution thoracic axial tomography (TACAR) and a biopsy of the mediastinal adenopathies by mediastinoscopy. He was treated for years with oral corticosteroids, which were subsequently withdrawn due to stabilisation of the disease both clinically and radiologically.
Follow-up since 2006 in Cardiology Outpatients, being diagnosed with dilated cardiomyopathy with severe systolic dysfunction. Coronary angiography was performed at that time, which ruled out significant coronary stenosis.
The patient admitted to consuming about 40 grams of alcohol per day, which was attributed to the origin of his heart disease. Successive follow-up visits were made; eight months after giving up the alcohol habit, the left ventricular ejection fraction (LVEF) was found to have recovered to 55%. At that time, he presented an electrocardiogram (ECG) with complete left bundle branch block (LBBB) and a QRS of 120 msec.
In 2011, he was admitted to Cardiology for chest pain with typical characteristics.
Coronary angiography was repeated, with no significant stenosis in the coronary tree.
An echocardiogram was performed, which showed a slightly dilated left ventricle with mild systolic dysfunction (LVEF 45%). On admission, after registering bouts of paroxysmal atrial fibrillation and atrial flutter, anticoagulant treatment was started with acenocoumarol; subsequently, due to a low rate of controls in therapeutic range with dicoumarinic drugs, he was switched to apixaban, without incident.
In the following years, during outpatient follow-up, there was evidence of progressive deterioration of LVEF, which fell to 30%, as well as the appearance of signs and symptoms of incipient heart failure. Treatment with angiotensin-converting enzyme inhibitors (ACE inhibitors), beta-blockers and aldosterone antagonists was initiated. From then on, in the successive ECGs obtained, he alternates complete right bundle branch block (RBBBH), with other recordings showing a non-specific intraventricular conduction disorder, and QRS between 140 and 150 msec.
Usual treatment on admission: spironolactone 25 mg/24 hours, ramipril 2.5 mg/12 hours, carvedilol 12.5 mg/12 hours), allopurinol 100 mg/24 hours, apixaban 5 mg/12 hours, atorvastatin 20 mg/24 hours, digoxin 0.25 mg/24 hours, furosemide 80 mg/24 hours (1-1-0), insulin glargine 16 IU/24 hours, metformin 850 mg-vildagliptin 50 mg/12 hours, omeprazole 20 mg/24 hours, duloxetine 60 mg/24 hours, trazodone 100 mg/24 hours, alprazolam 0.25 mg/24 hours.

Current illness
In 2013, she came to the Emergency Department with a 20-day history of progressive increase in her usual dyspnoea (ambulatory class II/IV), until it became minimal effort, with episodes in the last week of orthopnoea, paroxysmal nocturnal dyspnoea and bendopnoea limiting any physical activity. He also admits a subjective decrease in diuresis and occasional palpitations at rest, with no history of dizziness or syncope.

Physical examination
Normoperfused. Tachypnoea at rest, with use of accessory muscles. Elevation of jugular venous pressure, with positive hepatojugular reflux. O2 saturation 93% with oxygen supply at 3 litres per minute through nasal goggles. Blood pressure 130/70 mmHg. Arrhythmic heart sounds at 100 beats per minute (bpm), no murmurs. Pulmonary crackles up to midfields. Perimalleolar oedema with fovea in both lower limbs. Abdomen soft, depressible, non-painful, without masses or megaliths, with hydro-aerial sounds present.

COMPLEMENTARY TESTS
CBC: haemogram: leucocytes 9,000 10E3/mL (no left shift), haemoglobin 13 g/dL, haematocrit 39%, MCV 92 fl, platelets 163,000 10E3/mL.
Coagulation: INR 1.3, PT 60 %, TTPa 32 seconds.
Biochemistry: glucose 116 mg/dl, urea 70 mg/dl, creatinine 1.6 mg/dl, FGR (MDMR) 49 ml/min/1.73 m2, sodium 134 mEq/L, potassium 4.6 mEq/L, liver function tests normal, protein 7.4 g/dl, albumin 4.7 g/dl, cholesterol 132, triglycerides 129, HDL-cholesterol 31, LDL-cholesterol 75, HbA1c 6 %, thyroid hormones normal, proBNP 5900 pg/ml, ultrasensitive troponin T 14 ng/l.
Chest X-ray on admission: increased cardiothoracic index. Bilateral hilar thickening. Alveolar-interstitial oedema in both lung fields. Bilateral costophrenic sinus impingement. Fluid in the fissure.
Electrocardiogram: sinus rhythm at 90 bpm, PR 180 msec, QRS 150 msec. There was a non-specific intraventricular conduction disturbance that did not fully meet the criteria for HRBBB. Block of the left anterior subdivision of the His bundle (LADB). Frequent supraventricular extrasystoles.
Transthoracic Doppler colour echocardiogram: severely dilated left ventricle (end-diastolic diameter of 68 mm), with normal wall thicknesses, and severely depressed systolic function (LVEF 25%) at the expense of generalised hypokinesia, with no segmental contractility abnormalities identified.
Right ventricle of normal size and function. No significant valvulopathies were detected. No pericardial effusion. No tricuspid insufficiency or other data suggestive of pulmonary hypertension at rest. Ascending aorta of normal size and Doppler flow. No intracardiac shunts were observed.
Cardiac magnetic resonance imaging (MRI): left ventricular dilatation (LVEDV 260 ml, 140 ml/m2; LVESV 180 ml, 100 ml/m2) with severe deterioration of systolic function (LVEF 30%) due to global hypokinesia. Abnormal movement of the interventricular septum typical of bundle branch block. Perfusion study without pathological findings. Late gadolinium enhancement of intramyocardial distribution in basal and middle segments of the interventricular septum. Right ventricle of normal size and morphology, without abnormalities in global and segmental contractility.
Dilated left atrium (36 cm2) and right atrium of normal size (20 cm2).
Chest X-ray on admission: increased cardiothoracic index.
Improvement of signs of heart failure with respect to the image on admission, with persistent alveolar-interstitial oedema in the lung bases and bilateral hilar congestion. ICD-CRT device in cardiac cavities.

EVOLUTION
After initiating diuretic and intravenous vasodilator treatment at high doses, progressive improvement of the signs and symptoms of heart failure was achieved.
Once clinical stabilisation was achieved, the study was completed with the echocardiogram and cardiac MRI described above. The findings of these tests, together with the histological characterisation of the biopsy of mediastinal adenopathies performed years earlier, pointed to cardiac sarcoidosis as the most plausible cause of our patient's dilated cardiomyopathy.
Due to the severe conduction disturbances (with alternating LBBB and non-specific intraventricular conduction disturbance-LBBB-CCRDHH), the occurrence of non-sustained ventricular arrhythmias during monitoring, and the presence of non-ischaemic dilated cardiomyopathy with severe ventricular dysfunction, in NYHA functional class III/IV, and with a QRS of 150 msec (despite not having left bundle branch block morphology at the time of admission), it was decided to implant an ICD-CRT device (cardiac resynchronisation therapy) for primary prevention and to optimise the treatment of heart failure. After titration of the doses of beta-blockers, ACE inhibitors and Aldosterone receptor antagonists to the maximum tolerated doses, and confirmation of the patient's euvolemia and clinical stability, he was discharged for outpatient follow-up.
In the first months after admission, the patient's response to the established therapeutic plan was confirmed, and he remained in stable functional class I-II/IV for two years. In the ECG at follow-up, biventricular pacing rhythm, alternating atrial tachycardia / atrial flutter / baseline sinus rhythm, and with a QRS of 110 msec.
However, recently, he required a new admission to the Cardiology hospital ward for decompensated heart failure, as well as several visits to the Emergency Department for intravenous diuretic treatment. Therefore, treatment with sacubitrilo-valsartan was started after suspending ACE inhibitors for 36 hours. The response to the new treatment was partial, so a study has been started with a view to a possible heart transplant.

DIAGNOSIS
Cardiac and pulmonary sarcoidosis.
Dilated cardiomyopathy with severe left ventricular systolic dysfunction.
Heart failure with reduced ejection fraction, stage D.
