PERSONAL HISTORY
No known allergies. Family history: mother with depressive syndrome. No cardiovascular risk factors. Previous illnesses: migraine, asthma. No surgical history of interest. Previous medication: formoterol, budesonide, ipratropium bromide, paroxetine, rizatriptan. Social and professional history: independent for basic activities of daily living until the onset of the disease one year ago, with progressive worsening of her dyspnoea. Lives alone in her flat. She has worked as a social worker until the onset of this episode.

CURRENT ILLNESS
A 36-year-old woman presents with progressive exertional dyspnoea of three to four months' duration, which has become minimal effort in the last two weeks. She refers to having attributed the symptoms to her asthma and now consults because of the lack of improvement despite bronchodilator treatment. He also complains of oedema in the lower limbs and increased abdominal circumference with a feeling of fullness, nausea and vomiting in the last few days. He denies orthopnoea, paroxysmal nocturnal dyspnoea and oliguria. Refers bendopnoea. No anginal chest pain. Occasional palpitations on exertion, never at rest.

PHYSICAL EXAMINATION
Blood pressure 114/82 mmHg, heart rate 110 bpm. Baseline SpO2 98%. Afebrile. Cardiac auscultation: rhythmic, tachycardic, systolic murmur in the left parasternal border (EIB) and mitral focus. Pulmonary auscultation: preserved vesicular murmur, minimal crackles in the right base. Oedema with fovea in both malleoli. Abdomen: distended, without signs of peritoneal irritation. Not painful. No hepatomegaly.

COMPLEMENTARY TESTS
Electrocardiogram (ECG) on admission: sinus tachycardia at 110 bpm. Normal PR. Narrow QRS, normal axis. No acute repolarisation alterations. ECG at discharge: RS at 60 bpm. Normal PR. Narrow QRS. BIRDHH. T negative in II, III, aVF, V4- V6.
CBC 26/12/2018: haemoglobin 12.1 g/dl (12-15.6), haematocrit 39.3% (35.5-45.5), leukocytes 7.7, x10e3/μl (3.9-10.2), neutrophils 60.7% (42-77), platelets 258 x10e3/μl (150-370), INR 1.2 (0.8-1.2), fibrinogen 296 mg/dl (150-450), AST 35 IU/l (15-37), ALT 60 IU/l (13-56), GGT 35 IU/l (5-55), c-reactive protein 7.5 mg/l (0-3), glucose 102 mg/dl (74-106), urea 36 mg/dl (15-39), creatinine 1.08 mg/dl (0.55-1.02), glomerular filtration rate (CKD EPI) 66,2 ml/mn/1.73 m2 (60-999999), sodium 138.7 mmol/l (136-145), potassium 3.80 mmol/l (3.5-5.1), chlorine 107.0 mmol/l (98-107), NT-proBNP 4300. CBC at discharge: red blood cells 4.31 x10e6/μl (3.9-5.2), haemoglobin 12 g/dl (12-15.6), haematocrit 40% (35.5-45.5), leucocytes 6.3 x10e3/μl (3.9 - 10.2), neutrophils 49% (42-77), lymphocytes 40.1 % (20-44), platelets 271 x10e3/μl (150-370), creatinine 1,00 mg/dl (0.55-1.02), sodium 134.3 mmol/l (136-145), potassium 3.80 mmol/l (3.5-5.1), chlorine 97.0 mmol/l (98-107), NT-proBNP 2813 pg/ml (0-125).
Chest X-ray on admission: alteration of the morphology of the cardiomediastinal silhouette with growth mainly of the left atrium (LA) and right ventricle (RV). Vascular redistribution in upper lung fields to be assessed with the patient's baseline cardiac function. Pulmonary parenchyma with non-specific alterations.
Transthoracic echocardiogram: moderately dilated left ventricle (DTDVI 60 mm), somewhat spherical, and with systolic dysfunction that appears mild by this technique. The end of the apex is not adequately defined, which, although it could be explained by a certain degree of obliteration of the apex, may also be related to the ultrasound window. There is mild eccentric hypertrophy, with some linear hyperechogenicity of the septum. Pseudonormal diastolic pattern with shortened deceleration time (0.08 seconds). Slightly dilated right ventricle (RVOT 45 mm), with systolic function parameters at the limit of normality (TAPSE 17 mm, shortening fraction 34%, systolic wave 9.4 cm/s). Moderate-severe dilatation of the LA and mild-moderate dilatation of the right atrium (RA). Mitral valve with leaflets not clearly thickened or calcified, with preserved opening and moderate-severe insufficiency, reaching the roof of the atrium with coanda effect although it is more important in proto-mesosystolic. Morphologically and functionally normal aortic valve. Slightly eccentric tricuspid insufficiency, of mild-moderate degree, which allows estimating a PSAP of around 60-65 mmHg. Minimal amount of pericardial fluid. Visualised segments of the aorta and pulmonary arteries without alterations.  Cardiac magnetic resonance (CMR): 1. Morphological study: the LV is of normal size with high cardiac output (7l/min). It is not hypertrophic. Its function is at the low limit of normality (LVEF 51%). Eccentric mitral regurgitation (MR) towards the posterior aspect of the LA is at least moderate. After contrast administration, a laminar thrombus is observed along the entire LV apex, with a maximum thickness of 5 mm. Diffuse subendocardial perfusion defect with subendocardial late enhancement (<25%) is seen throughout the LV wall. The right ventricle is of normal size with moderate dysfunction (RVEF 38%) with global hypokinesia. There is rectification of the interventricular septum suggestive of RV pressure overload. Tricuspid insufficiency (TI), which appears moderate. No significant enhancement was observed after contrast administration. Biauricular dilatation. LA 65 x 60 x 58 mm. RA 68 x 58 mm. Intact atrial septum. Small amount of bilateral pleural and pericardial effusion. Mild hepatomegaly.
Dilatation of the inferior vena cava (IVC). Cystic image in the right breast. 2. Ventricular function study (calculated on short axis) left ventricle: end-diastolic volume 144 ml (86 ml/m2). Telesystolic volume 70 ml (42 ml/m2). Systolic volume 74 ml. Cardiac output 7 l/min. Ejection fraction (EF %) 51%. Mass 103 g (62 g/m2). Right ventricle: end-diastolic volume 130 ml (78 ml/m2). End-systolic volume 81 ml (49 ml/m2). Systolic volume 49 ml. Cardiac output 4.6 l/min. Ejection fraction (EF %) 38%. 3. Flow quantification: pulmonary aortic flow. Systolic volume 39.8 ml. Anterograde volume 40.8 ml. Retrograde volume 1 ml. Regurgitant fraction 2.4%. Maximum velocity 1 m/s. Systolic volume 33.8 ml. Anterograde volume 35.6 ml. Retrograde volume 1.8 ml. Regurgitant fraction 5%. Mean velocity 7.2 cm/s.
Conclusion: LV neither dilated nor hypertrophic with function at the lower limit of normality and high cardiac output. Subendocardial enhancement in all segments with laminar thrombus in the apex. RV neither dilated nor hypertrophic with moderate dysfunction. Biauricular dilatation. MR and TR at least moderate. The findings suggest restrictive cardiomyopathy with the most likely cause being endomyocardial fibrosis.
Endomyocardial biopsy: study of four samples showing findings compatible with endomyocardial fibrosis with the presence of extensive fibrosis and severe endocardial thickening, as well as images compatible with thrombus. Right catheterisation: pressure study. RA pressure 12 mmHg, systolic right ventricular pressure 65 mmHg, pulmonary artery pressure 64/28 (40) mmHg, pulmonary capillary pressure 20 mmHg, transpulmonary gradient 20 mmHg, cardiac output 2.6 bpm, cardiac index 1.5 bpm/m2. Pulmonary vascular resistance 7.7 UW. Abdominal ultrasound: conclusion: hepatic venous congestion.
Microbiology: blood cultures (2/1): E. cloacae. Urine culture (4/1): contaminated urine. Blood cultures (18/1): sterile. Urine culture (19/1) negative. Serology: Syphilis IgG negative, toxoplasma IgG negative, rubella IgG positive, HSV IgG negative, IgM negative, varicella IgG doubtful, cytomegalovirus IgG positive, IgM negative, HAV IgM negative, IgG positive, HBs Ag negative, anti-HBs Ag negative, anti-HBs borderline positive, HAV IgG positive, HIV 1/2 negative, measles IgG positive, mumps IgG positive.

CLINICAL COURSE
A 36-year-old woman was admitted for the first episode of heart failure. Depletive treatment was started with good clinical evolution. An echocardiogram was performed showing restrictive cardiomyopathy with mild left ventricular dysfunction and moderate-severe MI with severe pulmonary hypertension. Treatment for heart failure was started with good tolerance, titrating low doses of enalapril due to a tendency to hypotension. He subsequently presented signs of low cardiac output, with discrete deterioration of renal function and elevation of liver enzymes and coagulation alteration. A course of levosimendan was administered with clinical improvement. She presented a fever peak on the fifth day of admission with shivering and haemodynamic worsening, requiring admission to the coronary unit and inotropic support with intravenous noradrenaline in the context of bacteraemia due to Enterobacter cloacae, which was treated with a course of intravenous meropenem. In the following days the patient improved clinically, and the noradrenaline was withdrawn and diuretic doses were reduced. A CMR was performed which showed findings compatible with endomyocardial fibrosis and the presence of a laminar thrombus in the apex. In view of the suspicion of endomyocardial fibrosis, an endomyocardial biopsy was performed which confirmed the diagnosis.
A right heart catheterisation was also performed, showing pulmonary hypertension (group 2) with increased pulmonary vascular resistance and low cardiac output. Due to the presentation in cardiogenic shock and the poor prognostic data, a pre-transplant cardiac study was initiated.  

DIAGNOSIS
First episode of heart failure Restrictive cardiomyopathy: endomyocardial fibrosis. Moderate-severe mitral insufficiency (MI). Moderate tricuspid regurgitation (TR). Severe pulmonary hypertension group 2. LV apex thrombus. Cardiogenic shock and septic shock associated with Enterobacter cloacae bacteraemia. Adaptive syndrome with anxious-depressive symptoms.
