HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
A 39-year-old man presented with dyspnoea.

BACKGROUND:
Family history: paternal grandmother, paternal uncles and father suffered from dilated cardiomyopathy and died of sudden death. He has two living sisters diagnosed with dilated cardiomyopathy. Cardiovascular risk factors: active smoker of 1 pack a day, obese, dyslipidaemia type hypertriglyceridaemia. He was also a moderate drinker, with no alcohol consumption at present.
Cardiological history: diagnosed in 2012 with dilated cardiomyopathy with severe biventricular systolic dysfunction, non-ischaemic, with criteria of non-compaction cardiomyopathy on magnetic resonance imaging after admission to cardiology for heart failure (HF) (videos 1-2). It was reported as: dilated left ventricle (LV) (end-diastolic volume [EDV] 195 ml/m2 and end-systolic volume [ESV] 161 ml/m2). Left ventricular ejection fraction (LVEF) of 17% due to diffuse severe hypokinesia. Lateral and apical predominant hypertrabeculation meeting radiological criteria for non-compaction. Small area of intramyocardial late enhancement at the basal insertion of both ventricles. Dilated right ventricle (RV) (RVT 141 ml/m2 and STV 106 ml/m2). Right ventricular ejection fraction (RVEF) of 25% with global hypocontractility. Severely dilated left atrium (LA) and right atrium (RA). Dilatation of pulmonary artery, inferior vena cava and suprahepatic veins. Normal pericardium. During this admission, a coronary angiography was also performed, which showed right dominance and coronary arteries without significant lesions. Followed in cardiology outpatient clinic since then. Stable, with adequate compliance with hygienic-dietary measures. Angiotensin converting enzyme inhibitor (ACE inhibitor), beta-blocker (BB) and aldosterone inhibitors up to maximum tolerated doses, together with ivabradine. Ergospirometry was performed in June 2013 with the following result: forced vital capacity (FVC) 4.48 l (76%), forced expiratory volume in one second (FEV1) 4.22 l (83%), FEV1% 87%. Suggestive of restrictive disorder due to obesity. VO2 max 21 ml/kg/min, coincides with the threshold. VE max 106 l/min Eq CO2 at threshold 30. Maximum heart rate (HR) 149 bpm (80% maximum theoretical heart rate [MTHRF]). Exercise time 10.3 min. Implantable cardioverter defibrillator (ICD) implantation was decided for primary prevention after imaging studies showed persistent LVEF < 30%. Last check-up 6 months before admission, in New York Heart Association (NYHA) functional class II. Other history: sleep apnoea-hypopnoea syndrome (under treatment with continuous positive airway pressure [CPAP]). Usual treatment: enalapril 10 mg (1 tablet at breakfast), carvedilol 25 mg (1 tablet at breakfast and 1 tablet at dinner), spironolactone 25 mg (2 tablets at dinner), ivabradine 5 mg (1 tablet at breakfast and 1 tablet at dinner), furosemide 40 mg (1 tablet at breakfast).

CURRENT DISEASE:
In NYHA functional class II until 5 weeks ago, refers progressive worsening due to increased dyspnoea until becoming minimal effort, with orthopnoea and paroxysmal nocturnal dyspnoea. The patient, in view of the functional worsening, increased the dose of oral furosemide from 40 mg to a dose of 160 mg per day (2 tablets at breakfast and 2 tablets at snack time). As he continued with dyspnoea on minimal exertion without improvement, he consulted the emergency department. Due to his work situation, he reported non-compliance with hygiene and dietary measures in recent weeks, with dietary transgressions. The rest of the medical history was negative.

PHYSICAL EXAMINATION:
On examination he is conscious and oriented, well perfused. He weighs 110 kg. Blood pressure (BP) was 120/90 mmHg. Jugular venous pressure is not measurable due to obesity. Cardiac auscultation is rhythmic at 80 beats per minute, without murmurs. Pulmonary auscultation shows good ventilation in all fields. The abdomen is soft and slightly painful hepatomegaly of about 2 centimetres is palpable. The lower extremities are oedematous up to mid-leg. Distal capillary refill is good.


COMPLEMENTARY TESTS
ELECTROCARDIOGRAM (ECG) (on admission): sinus rhythm at 100 bpm. PR interval of normal duration and QRS of 110 ms. Scarce R wave progression in precordials and asymmetric negative T waves in DI, aVL, V5 and V6. THORAX RADIOGRAPHY: cardiomegaly and slight vascular redistribution to upper fields. No parenchymal condensation. ANALYSIS (during admission): haemoglobin 11.7 g/dl, leukocytes 10,000 /mm3 (normal formula), platelets 255.000 /mm3, glucose 87 mg/dl, creatinine 0.8 mg/dl, urea 45 mg/dl, sodium 137 mEq/dl, potassium 4.4 mEq/l, total cholesterol 112 mg/dl, LDL cholesterol 75 mg/dl, uric acid 9.3 mg/dl, gamma-GT 74 U/l, total bilirubin 1.6 mg/dl, ferritin 52 ng/dl, transferrin saturation index 4.3%, glycosylated haemoglobin 6.0%. Normal TSH. amino-terminal pro-brain natriuretic peptide (NT-proBNP) fraction on admission 1,607 pg/dl, on discharge 858 pg/dl.

CLINICAL EVOLUTION
The patient was admitted to the cardiology ward for the first episode of decompensation after diagnosis of HF. Intravenous diuretic treatment was started with improvement of the signs of congestion and symptoms. The patient lost 4 kg of weight during admission. Hygiene and dietary measures were emphasised and the patient was included in the HF consultation, and an early visit (one week after discharge) was scheduled. On discharge, the previous neurohormonal treatment was maintained with a daily dose of 160 mg of oral furosemide. At the first review, the patient was in NYHA functional class II and in euvolemia, so the dose of furosemide had been reduced to 80 mg per day. With BP of 125/70 mmHg and given the patient's profile, with risk of readmission and progression of the disease, it was decided to suspend enalapril and after 36 hours, treatment was started with sacubitril/valsartan 24/26 mg every 12 hours. A new check-up was scheduled after 3 weeks. On the second visit to the clinic, the patient was found to be euvolaemic and with BP levels around 120/60 mmHg. The dose of sacubitril/valsartan was increased to 49/51 mg every 12 hours. Five days after the consultation, the patient came to the emergency department for severe dizziness. Hypotension of 90/50 mmHg was noted. When assessed by the cardiology ward, it was decided to maintain the dose of sacubitril/valsartan at 49/51 mg every 12 hours, but the dose of furosemide was reduced to 40 mg, given that he showed no signs of congestion. At the next visit, systolic BP was around 110-120 mmHg, with no signs of congestion. Prior to the last dose titration of sacubitril/valsartan, it was decided to reduce the dose of furosemide 40 mg to half a tablet at breakfast. The patient was titrated to the maximum dose of sacubitril/valsartan 97/103 mg without symptomatic hypotension. Iron deficiency was also corrected with the administration of iron-carboxymaltose in the day hospital. At a review after 2 months, the patient was found to be in functional class I in a situation of euvolemia. Treatment after consultation follow-up: sacubitril/valsartan 97/103 mg (1 tablet every 12 hours), carvedilol 25 mg (1 tablet every 12 hours), ivabradine 5 mg (1 tablet every 12 hours), furosemide 40 mg (1/2 tablet at breakfast) spironolactone 25 mg (2 tablets at dinner) omeprazole 20 mg (1 tablet at breakfast).

DIAGNOSIS
HF in a patient with non-ischaemic dilated cardiomyopathy with severe biventricular systolic dysfunction and criteria for non-compaction cardiomyopathy. NYHA functional class I. Euvolemic state. ICD carrier in primary prevention.
