HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
53-year-old male with no known drug allergies or previous cardiological history. As cardiovascular risk factors, the patient had hypertension and type 2 DM for years and was treated with metformin 850 1/12h and irbersartan/hydrochlorothiazide 300/12.5 1/24h. The patient has a history of nephritic colic (the last one a year ago) and underwent otorhinolaryngological examination for progressive bilateral hypoacusis with a diagnosis of chronic sound trauma.
The patient consulted for progressive dyspnoea in the last few months until he became moderately laboured. He presented with orthopnoea and paroxysmal nocturnal dyspnoea in the last 15 days, accompanied by a progressive decrease in diuresis and increased oedema in the lower limbs. She denies chest pain. She does not report febrile syndrome. A week ago he was diagnosed with possible parotitis, due to bilateral parotid swelling, more marked on the right side.
On physical examination the patient is conscious, oriented and cooperative, in good general condition, with good peripheral perfusion. There was swelling of both parotid glands, the right one with a stony consistency. BP 126/55, HR 91 bpm. CBC: PVY normal. AC: rhythmic heart sounds, holodiastolic murmur in aortic focus. AP: preserved vesicular murmur. Abdomen nondescript. IIJS: oedema with fovea up to the knees.

COMPLEMENTARY TESTS
Laboratory tests on admission: biochemistry: glucose 107.0 mg/dl (74.0 - 100.0), lipase 29.0 IU/L (3.0 - 60.0), creatinine 1.19 mg/dl (0.7 - 1.2), urea 44.0 mg/dl (13.0 - 50.0), bilirubin 1,28 mg/dl (0.15 - 1.1), sodium ion 138.0 mmol/L (135.0 - 145.0), potassium ion 4.56 mmol/l (3.3 - 5.1), alanine aminotransferase (ALT) 28.0 IU/l (5,0 - 41.0), aspartate aminotransferase 38.0 IU/l (5.0 - 38.0), total protein 7.15 g/dl (6.4 - 8.3), C-reactive protein 1.71 mg/dl (0.1 - 0,5), alpha amylase 191.0 IU/l (28.0 - 100.0), renal glomerular filtration rate 69.0 ml/min/1.73 m2 (89.0 - 140.0), troponin T us 27.0 ng/l (0.0 - 14.0). Haemogram: leucocytes 4.0 10E9/L (3.6 - 11.0), neutrophils % 62.4 % (40.0 - 80.0), red cells 5.1 10E12/L (4.2 - 6.2), haemoglobin 13.9 g/dl (13.0 - 18.0), haematocrit 42.3 % (40.0 - 54.0), mean corpuscular volume (MCV) 83.3 - 83.0, haemoglobin 13.9 g/dl (13.0 - 18.0), haematocrit 42.3 % (40.0 - 54.0). Mean Corpuscular Volume (MCV) 83.3 fl (80.0 - 100.0), HDI 13.1 % (10.5 - 16.0), platelets 144.0 10E9/L (130.0 - 450.0). Coagulation: prothrombin time 12.9 sec (8.0 - 13.0), prothrombin activity rate 70.9 % (70.0 - 140.0), partial thromboplastin To 25.3 sec (22.0 - 37.0), fibrinogen 442.0 mg/dl (145.0 - 450.0). EKG: sinus rhythm at 87 bpm, PR 174 msec, signs of LV enlargement. No significant repolarisation abnormalities.
Chest X-ray: cardiothoracic index within normality. Prominence of both pulmonary hilar lobulated contours, which in the context of the patient could correspond to adenopathies. Free cardiophrenic and costophrenic sinuses.
Probable bilateral and diffuse reticulonodular interstitial involvement. I recommend completing the study with chest CT.
Echocardiogram: LA slightly increased in size (43 mm, 23 cm2). LV not dilated in high limits of normality for the patient's CS (DD 54 mm, VTD 165 ml would correspond to a mild dilatation but indexed to its body surface is 72 ml/m2, in the high limit of normality). Global EF preserved (62 % Simpson biplane). Diastole not assessable due to flow fusion with aortic insufficiency. Normal AD. Normal RV. TAPSE 25 mm. Mitral: leaflets with slight excess tissue and minimal billowing of posterior leaflet without insufficiency. Mild restriction to anterior leaflet opening due to aortic insufficiency. Aortic: trivalve, leaflets with minimal sclerosis, although poorly visualised, it appears to be right coronary leaflet prolapse leading to severe eccentric insufficiency towards the anterior mitral leaflet (diastolic reverse flow in descending thoracic aorta with end-diastolic velocity > 20 cm/sec and in abdominal aorta). Aorta: normal calibre root (sinus 33 mm). Normal descending and descending aorta. Normal tricuspid. No IT to estimate PAPs. Normal pulmonary. Normal IVC. Normal pericardium.
Cardiac catheterisation: coronary arteries without significant lesions.
Complete laboratory tests: biochemistry: TSH-Tyrotropin 2.71 μIU/ml (0.25 - 4.22), alkaline phosphatase 85.0 IU/L (40.0 - 129.0), gamma-glutamyltransferase (GGT) 141.0 IU/l (3.0 - 71.0), albumin 3.78 g/dl (3.5 - 5.2), globulins 2,85 g/dl (1.0 - 4.5), albumin/globulins 1.3 (1.0 - 2.1), 4116 albumin-corrected calcium 2.90 mmol/l (2.15 - 2.5), 116 albumin-corrected calcium 11.61 mg/dl (8.62 - 10.02) glucose 106,0 mg/dl (74.0 - 100.0), creatinine 1.87 mg/dl (0.7 - 1.2), urea 69.0 mg/dl (13.0 - 50.0), bilirubin 0.84 mg/dl (0.15 - 1.1), urate 8.09 mg/dl (3.4 - 7.0), sodium ion 137.0 mmol/l (135,0 - 145.0), potassium ion 4.29 mmol/l (3.3 - 5.1), creatine kinase 48.0 IU/l (24.0 - 195.0), alanine aminotransferase (ALT) 30.0 IU/l (5.0 - 41.0), aspartate aminotransferase 33.0 IU/l (5.0 - 38,0), total protein 6.63 g/dl (6.4 - 8.3), L-lactate dehydrogenase 159.0 IU/l (135.0 - 225.0), C-reactive protein 0.74 mg/dl (0.1 - 0.5), alpha amylase 223.0 IU/l (28.0 - 100.0), renal glomerular filtration rate 40.0 ml/min/1.73 m2 (89.0 - 140.0), total calcium 2.86 mmol/l (2.15 - 2.5), total calcium 11.44 mg/dl (8.62 - 10.02), phosphate 1.02 mmol/l (0.81 - 1.45), phosphate 3.16 mg/dl (2,5 - 4.5) intact parathormone 9.0 pg/ml (15.0 - 65.0), ACE-angiotensin converting enzyme* 175.0 U/l (20.0 - 70.0), vitamin D (25hydroxychalciferol) 9.0 ng/ml, albumin 4.06 g/dl (3.5 - 5,2), globulins 2.73 g/dl (1.0 - 4.5), albumin/globulins 1.5 (1.0 - 2.1), albumin-corrected calcium 2.89 mmol/l (2.15 - 2.5), albumin-corrected calcium 11.58 mg/dl (8.62 - 10.02).
CBC: leucocytes 3.5 10E9/L (3.6 - 11.0), sedimentation rate 23.0 mm (0.0 - 20.0), neutrophils 55.2 % (40.0 - 80.0), red cells 4.9 10E12/L (4.2 - 6.2), haemoglobin 13.8 g/dl (13.0 - 18.0), haematocrit 41.2 % (40.0 - 54.0), mean corpuscular volume (MCV) 83.7 mm (0.0 - 7.0). mean corpuscular volume (MCV) 83.7 fl (80.0 - 100.0), platelets 125.0 10E9/L (130.0 - 450.0).
Coagulation: prothrombin time 12.4 sec (8.0 - 13.0), prothrombin activity rate 76.5 % (70.0 - 140.0), partial thromboplastin To 26.2 sec (22.0 - 37.0), fibrinogen 297.0 mg/dl (145.0 - 450.0).
Neck ultrasound: bilateral and symmetrical enlargement of both parotid glands, with smooth, well-defined borders and an inhomogeneous and reduced echostructure, with diffuse hypoechoic pseudonodular punctate images. The glandular vascularisation is normal and symmetrical.
There are no distinct intraparotid focal lesions. The described findings suggest inflammatory/infectious pathology as a first possibility.
Bilateral submaxillary and laterocervical adenopathies, all of them subcentimetric and non-specific. Normal sized submaxillary glands, without focal lesions. Normal size thyroid, with the right lobe measuring 20 x 19 x 46 mm (AP x T x CC) and the left lobe measuring 13 x 15 x 46 mm. In the right lobe there is a heterogeneous, predominantly cystic lesion with a thick wall and multiple thick septa inside, measuring 15 x 15 x 15 x 20 mm. In the left lobe there is a mixed, predominantly solid, isoechogenic nodule with some peripheral gross calcifications and small cystic areas, measuring 7 x 8 x 9 mm; a mixed nodule in the inferior pole, heterogeneous, predominantly solid, measuring 9 x 13 x 14 mm; and a gross calcification of 3 mm. A directed study and FNA are recommended.
Abdominal ultrasound: liver with increased echogenicity, homogeneous, with no evidence of space-occupying lesions. Normal gallbladder and bile duct. Portal vein permeable. Homogeneous splenomegaly of 16 cm. Retroperitoneum visualised without pathological findings. Kidneys without dilatation of the excretory tract or other alterations.
Aorta of normal calibre.
CT scan: performed without VSD due to the patient's poor renal function. However, abundant mediastinal adenopathies of pathological size in the different mediastinal compartments, bilateral hilar, subcarinal, aortopulmonary window, prevascular, etc., are identified. Widespread peribronchial thickening. Interstitial pattern predominantly in apexes and bases with thickening of the interlobular septa.
No pleural effusion is identified. Hepatosplenomegaly. Spleen 17 x 7 cm (in hepatopathy, sarcoidosis, etc.). Normal gall bladder. FNA: Benign type 2. Cystic colloid nodules Parotid FNA: samples consisting of haematic material and very occasional epithelial cells without atypia or evidence of malignancy. Thyroid FNA: cytological smears showing foamy histiocytes and follicular cells without atypia arranged in a dissociated manner on a colloid background. Diagnosis Colloid nodule with cystic-like changes. FNA of mediastinal adenopathies by bronchoscopy with confirmation of material compatible with sarcoidosis.
Cardiac MRI: LV and RV images were obtained in cine sequences, late enhancement after gadolinium administration and flow quantification in pulmonary and aortic valves. LV: mildly dilated, with TVV of 96 ml/m2 (range 47-92 ml/m2) and STV of 39 ml/m2 (range 13-30 ml/m2). EF 59%, cardiac output 8.3 l/min and stroke volume 128 ml. Normal segmental contractility. Normal myocardial mass of 88 g/m2 (range 70-113 g/m2). Maximum septal thickness of 13 mm. Mild concentric hypertrophy. No areas of non-compaction. Subepicardial hyperenhancement of contrast in basal posterior segment compatible with myocardial fibrosis in the clinical context of sarcoidosis. Mechanical aortic valve prosthesis. RV: normal size, without morphological alterations, with TVD of 74 ml/m2 (range 55-105 ml/m2) and STV of 18 ml/m2 (range 15-43 ml/m2). EF of 75%, cardiac output 8.3 l/min and stroke volume 127 ml. Normal contractility. Jet of tricuspid regurgitation. LA: Area 20 cm2, normal size. RA: area of 24 cm2, slightly dilated. Ascending aorta of normal calibre (2.9 x 2.9 cm). Pulmonary artery of normal calibre (2.9 x 2.3 cm). Pericardium: No pericardial thickening is observed. Mild-moderate pericardial effusion with a maximum thickness of 1.2 cm in the lateral aspect of the LV. Paratracheal mediastinal adenopathies (2 cm short axis), prevascular (1.5 cm), aortopulmonary window (1.2 cm), subcarinal (1.9 cm), in left hilum (1.8 and 2.1 cm), in right hilum (2.2 and 1.8 cm) compatible with sarcoidosis. Middle sternotomy wires.

EVOLUTION
The transthoracic echocardiogram showed a slightly dilated and hypertrophic LV with preserved systolic function and the existence of a trivalve aortic valve with severe insufficiency at the expense of the right coronary leaflet that appears to present a flail. On the other hand, given the findings of the examination, serological studies were requested, which ruled out an infectious origin. Laboratory data showed hypercalcaemia and elevated ACE (angiotensin-converting enzyme) levels as the most relevant findings and, together with the chest X-ray showing a prominence of both pulmonary hilums compatible with lymphadenopathy, a diagnosis of sarcoidosis with parotid and cardiac involvement was established. Chest CT showed multiple mediastinal, hilar and subcarinal adenopathies, as well as a predominant interstitial pattern in the vertex and bases. To confirm the diagnosis, it was decided to perform a parotid FNA and finally fibrobronchoscopy with biopsy of one of the lymph nodes with the presence of non-caseating granulomas, confirming the diagnosis of sarcoidosis.
The patient underwent aortic valve replacement. The valve was described as a very thin, sagging right coronary leaflet, with no aortic annulus in that area, with prolapse of the left leaflet and retraction of the non-coronary leaflet, for which reason it was decided to implant a mechanical prosthesis. The surgical specimen was sent for anatomopathological examination, which confirmed the existence of non-caseating granulomas compatible with sarcoidosis.
Two months after surgery, cardiac MRI showed a slightly dilated and hypertrophic LV with pathological Gadolinium uptake in the subepicardial region, predominantly in the posterobasal segment, compatible with cardiac involvement in the context of systemic sarcoidosis.
At present, the patient is asymptomatic from the cardiovascular point of view with functional grade I/IV in outpatient follow-up by internal medicine and cardiology and with treatment with corticoids without having presented new episodes of decompensation since hospital discharge.

DIAGNOSIS
Systemic sarcoidosis with cardiac involvement.
Severe aortic insufficiency due to sarcoid valve involvement.
Aortic valve replacement by mechanical prosthesis.
