History, current illness and physical examination
Reason for consultation: dyspnoea Personal history: No known drug allergies. Diabetes mellitus secondary to pancreatoduedectomy performed in 2005 due to a mass in the head of the pancreas with suspected ampuloma and corresponding to chronic sclerosing pancreatitis; spenectomy in the same surgical act (also cholecystectomy and appendectomy). Multicentric Castleman's disease, plasmacytic variant, diagnosed in January 2006. Initially treated with CHOP (8 cycles) with good response. Due to gastric parietal thickening, a gastroscopy was performed which revealed grade 2 oesophageal varices, without fundic varices and a biopsy of the gastric body compatible with chronic gastritis without significant atrophy. In November of the same year, due to persistent PET-CT activity, treatment was started with chlorambucil and later rituximab, which was maintained intermittently due to supra- and infradiaphragmatic metabolic progression until January 2013. She continued to be monitored by the haematology outpatient clinic, with suspicion of deep vein thrombosis of the left lower limb, which was ruled out by normal Doppler ultrasound.
Baseline situation: IABVD. NYHA functional class I. Previous treatment: pantoprazole 40 mg (1-0-0), pancreatin 10000 U (3-3-3), insulin as prescribed, paracetamol if necessary.

Present illness: 62-year-old patient attended the emergency department for progressive exertional dyspnoea of 1 month's evolution until becoming resting in the last few days, orthopnoea and paroxysmal nocturnal dyspnoea; nocturnal decubitus cough and nocturia. He also reported an increase in abdominal perimeter and oedema of the lower limbs, discomfort in the right hypochondrium (suggestive of hepatic congestion in the anamnesis). No chest pain. She did report occasional palpitations during the last 20 days lasting up to 30 minutes. Uncontrolled blood pressure at home. Denies non-compliance with treatment. No fever. No expectoration. No nausea, no vomiting, no alteration in stool frequency or stool characteristics.

Physical examination: BP 136/57 mmHg, HR 68 bpm, afebrile. Weight 67 kg, height 170 cm. BEG. Normal colour, normoperfused and normohydrated. Head and neck: jugular ingurgitation. AC: rhythmic tones, no murmurs. PA: semiology of pleural effusion in the lower half of the right hemithorax. Abdomen: soft, depressible, pain in the right hypochondrium without peritoneal irritation or defence, no masses or visceromegaly. Extremities: oedema with fovea up to the root of the thighs, no signs of DVT, positive pedal pulses.

Complementary tests
CBC: leukocytes 8.7 thousands/mcL, haemoglobin 15.3 g/dL, haematocrit 47.7%, MCV 98 fl, platelets 191 thousands/mcL. ESR (1 hour) 64 mm. Activ. Prot. 84%, APTT 37 sec, Fng 453 mg/dL, Gluc 145 mg/dL, CRP 0.27 mg/dL, Creat. 1.07 mg/dL, urea 39 mg/dL, sodium 137 mmol/L, potassium 4.6 mmol/L, calcium 8.5 mg/dL, phosphorus 4.4 mg/dL, GOT 24 IU/L, GPT 16 IU/L, GGT 25 IU/L, FA 109 IU/L, total cholesterol 123 mg/dL, LDH 509 IU/L, triglycerides 52 mg/dL, HDL 46 mg/dL, total protein 7.5 g/dL. TSH 2.12 mcU/mL. ʟʟ Auto antibodies: ANA and ANCA negative.
Serology: HBV and HCV negative.
Total proteins 8.80 g/dl, albumin 42.9% 3.78 g/dl, Alpha-1-globulins 3.5% 0.31 g/dl, Alpha-2-globulins 10.7% 0.94 g/dl, ß-globulins 9.2% 0.81 g/dl, Gamma-globulins 33.7% 2,97 g/dl, polyclonal hypergammaglobulinaemia, serum immunoglobulin G 3120 mg/dl, immunoglobulin A 134 mg/dl, immunoglobulin M 40.4 mg/dl, serum Beta-2-microglobulin 4.59 μg/ml.
Baseline arterial blood gases: pH 7.39, pCO2 31 mmHg, pO2 68 mmHg. ʟʟ 24-hour urine: clearance 59 ml/min, total proteinuria 219 mg/24 hours, Ca 73 mg/24 hours. Bence Jones proteinuria (kappa and lambda light chains in urine) negative.
Chest X-ray: cardiothoracic index at the upper limit of normality, mediastinum and both hilar normal, thickening of the greater fissure, bilateral pleural effusion, predominantly in the right hemithorax.
Electrocardiogram: sinus rhythm with first degree AV block and left anterior hemiblock. Isolated ventricular extrasystoles.
During the hospital stay, he had several episodes of palpitations, and the electrocardiogram showed paroxysmal atrial fibrillation with ventricular response at 130lpm and spontaneous reversion to sinus rhythm after a strategy of ventricular rate control with beta-blockers and anticoagulation.
Transthoracic echocardiogram: left ventricle slightly dilated (VTD 145 ml, VTD/SC 81mL/m2), not hypertrophied, with hypokinesia of all segments at baseline and moderate systolic dysfunction. Grade III diastolic dysfunction (mean E/e" =16, observing restrictive mitral filling pattern that pseudonormalises with respiratory movements). Estimated global LVEF moderately depressed LVEF 41%. Slightly dilated left atrium with slightly dilated aortic root and ascending aorta. Non-dilated right ventricle with preserved systolic function. Mitral valve with thin leaflets without stenosis and with mild insufficiency. Trivalve aortic valve without significant stenosis or insufficiency. No tricuspid insufficiency that would allow estimation of PSAP. Inferior cava not dilated with normal inspiratory collapse. Intact interatrial septum. Minimal pericardial effusion. Bilateral pleural effusion.
Whole body PET-CT fluorodeoxyglucose-F18: the images obtained still show a localised adenopathy in the left internal mammary chain, with no significant metabolic or morphological change with respect to the previous study, current maximum SUV of 1.2 (the same as the previous one). At the infradiaphragmatic level, some lymph node formations are visualised in both external iliac chains with slight metabolic detectability (SUV max around 1.8, similar to previous) and without significant variations in their size or number. An adenopathy is seen in the right obturator region with slight detectability (SUV max of 1.5) which was not visualised before. In addition, there are still innumerable nodular lesions, already seen in the previous study, in the mesentery, although with an overall decrease in their metabolism and size. The lesion previously described in the mesentery, at the right pelvic level, close to the midline, is not currently visible, which suggests a probable reactive/inflammatory nature as there is no evidence of new treatments since the last PET scan.
In the localising CT scan, bilateral pleural effusion is seen, predominantly on the right, without metabolic detectability; multiple pseudonodular infiltrates are also seen, predominantly in the bases, with slight metabolic detectability.
Conclusion: slight metabolic improvement with respect to the previous control of 1 year ago with persistence of detectable supra- and infradiaphragmatic metabolic lymphadenopathies with predominance of the latter location.
Ergometry-SPECT: inconclusive ergometry due to not reaching submaximal heart rate (HR). Up to the load reached (5 METS) no signs of ischaemia were observed. Chronotropic incompetence. Slow HR recovery. Moderately reduced functional capacity (64% of predicted). ʟʟ Gated SPECT of myocardial perfusion is performed after ergometric stress and injection of the radiopharmaceutical at the time of maximum stress. Delayed resting study with injection of the same activity. Two-day protocol. The images obtained post-stress show a slight perfusion defect in the basal and middle segment of the inferior wall that remains with similar characteristics when the radiological attenuation correction technique is applied, improving moderately in the resting acquisition. There is also an increase in the size of the left ventricle in the post-stress study, which reaches a STV of approximately 101 ml, with global hypokinesia in the stress study, which decreases significantly at rest, also improving LVEF. In the GATED study, the following functional values were obtained post-stress/rest: LVEF 29%/52%, STV 101 mL/62 mL, TDV 142 mL/131 mL. VTD/SC 80 mL/m2 at stress and 74 mL/m2 at rest.
Conclusion: findings compatible with mild-moderate and moderately extensive stress-induced lower wall ischaemia, with transient ventricular dysfunction at post-stress compared to rest as an indirect sign of severity. abdominal ultrasound: liver of normal size with homogeneous echogenicity and regular contours without evidence of focal lesions. Portal vein of normal calibre. No dilatation of the suprahepatic veins. Absence of gallbladder and spleen due to previous surgery. Retroperitoneal region not adequately assessed due to intestinal gas interposition. Both kidneys are of normal size, with preserved cortices, without dilatation of the excretory tract or images suggestive of lithiasis. No free fluid was seen in the abdominal cavity.

Clinical course
Given the presence of cardiovascular risk factors, it was decided to request a diagnostic coronary angiography. The patient showed clinical improvement of heart failure with treatment. Coronary angiography: coronary arteries without significant stenosis. Ventriculography showed a slightly dilated left ventricle with moderately depressed systolic function (LVEF 38%) and global hypokinesia. Absence of mitral insufficiency. Given that the coronary angiography was normal and the possible aetiology of coronary macroagiopathy was ruled out as the cause of the ventricular dysfunction, it was decided to request an outpatient MRI to rule out infiltrative lesions probably related to Castleman's disease, the results of which are currently pending.

Diagnosis
Main diagnosis at discharge: congestive heart failure with moderate systolic dysfunction and grade III diastolic dysfunction.

Secondary diagnoses:
non-valvular paroxysmal atrial fibrillation. CHA2DS2-VASc Score = 2 (high embolic risk). HAS-BLED Score = 0 (low bleeding risk).
Multicentric Castleman's disease.
Diabetes mellitus secondary to pancreatectomy.

Treatment: salt- and fat-free diet. Daily exercise.

Medication:
Pancreatin 10,000 U: 3 tablets at breakfast, 3 tablets at lunch and 3 tablets at dinner.
Continue with the usual regimen of novomix 26 IU at breakfast, 14 IU at lunch and 18 IU at dinner.
Furosemide 40 mg 1 tablet at breakfast and half a tablet at lunch.
Bisoprolol 2.5 mg 1/2 tablet at breakfast.
Rivaroxaban 20 mg 1 tablet at breakfast.
Spironolactone 100 mg 1/2 tablet at breakfast.
Ramipril 2.5 mg every 12 hours.
Pantoprazole 40 mg 1 tablet at breakfast.
