HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

BACKGROUND:
63-year-old male. No known drug allergies. Personal history: exobabies, type 2 diabetes mellitus, dyslipidaemia, myelodysplastic syndrome subtype CRDM (refractory cytopenias with multilinear dysplasia) followed up by haematology. Ischaemic heart disease of debut in 1996 in the form of acute myocardial infarction with ST-segment elevation (STEMI) posteroinferior. Inferior re-infarction in 1999 and in June 2006 with syncope and lacunar cerebrovascular accident (CVA). On admission in 2006 he was diagnosed with multivessel coronary artery disease with slightly depressed left ventricular systolic function and severe aortic stenosis, and it was decided to perform surgical coronary revascularisation and aortic valve replacement. In December 2006, aortic valve replacement with mechanical prosthesis and triple aortocoronary bypass with revascularisation of the LAD with IMA and PD and 1st marginal with two saphenous vein fragments.
Follow-up in outpatient cardiology clinics in usual New York Heart Association (NYHA) functional class II. In the last year and a half, progressive deterioration of functional class with progressively decreasing dyspnoea on exertion and oedematisation of the lower limbs (lower extremities).
Last echocardiogram in November 2016: left ventricle (LV) of normal size.
Mild septal hypertrophy, preserved systolic function with left ventricular ejection fraction (LVEF) around 55%, akinesia of the inferior wall, the rest of the segments without contractility alterations. Normofunctioning mitral valve (MV). Mechanical prosthesis in aortic position with adequate opening, moderate periprosthetic insufficiency. Mild tricuspid insufficiency (TI). Estimated systolic pulmonary artery pressure (PAPs) of 45 mmHg. No pericardial effusion.
Under home treatment with: acenocoumarol according to guideline, atorvastatin 10 mg every 24 hours, omeprazole 20 mg every 24 hours, glimepiride 4 mg every 24 hours, metformin/vildagliptin 850/50 mg every 12 hours, ezetimibe 10 mg every 24 hours, ferrous sulphate 80 mg every 24 hours, ivabradine 5 mg every 12 hours, furosemide 40 mg every 24 hours, spironolactone 50 mg every 24 hours.

CURRENT ILLNESS:
The patient comes to the ED for frank clinical worsening in the last month with dyspnoea on minimal effort, bendopnoea, increased abdominal perimeter and oedematisation of EEII. No clear orthopnoea or DPN. He also reported the appearance of oppressive, continuous precordial pain, of about three days' evolution, without irradiation or accompanying vegetative cortex. No dietary transgressions or apparent intercurrent infectious process.

PHYSICAL EXAMINATION:
Blood pressure (BP) 115/60 mmHg, heart rate 72 bpm, oxygen saturation 93% with GN at 2 bpm. Afebrile. Normohydrated with icteric staining of skin and conjunctivae. Conversational dyspnoea, no respiratory work at rest. Jugular ingurgitation. Cardiac auscultation: rhythmic, prosthetic sounds with diastolic murmur in aortic and left parasternal focus.
Pulmonary auscultation: discrete bibasal hypophonesis, without crackles or other added pathological sounds. Abdomen was globular, with collateral circulation, no clear ascitic surge and hepatomegaly of 3 traverses. The lower extremities showed oedema with fovea up to the middle third of both legs.

COMPLEMENTARY TESTS
ECG: sinus rhythm at 83 bpm, PR 190 ms, known LBBB with secondary repolarisation alterations.
ANALYTICS on admission: ProBNP 1,919 pg/ml, glucose 72 mg/dl, urea 40 mg/dl, creatinine 0.97 mg/dl, sodium 134 mEq/l, potassium 3.8 mEq/l. Leukocytes 3,400/ml (76.8% neutrophils), Hb 11.4 gr/dl, Hto 35.5%, platelets 59,000/mm3. INR 3.96.
THORAX X-RAY: global cardiomegaly. Aortic valve prosthesis. Mid sternotomy sutures. Increase of interstitial tissue of probable vascular origin. No consolidative foci or pleural effusion. Dorsal spondylosis with calcification of the anterior vertebral ligament.
Transthoracic echocardiogram on admission: LV of normal size and moderate concentric hypertrophy. Normal global LV systolic function; flattened septal motion probably related to previous surgery and right overload, inferobasal and medial inferior akinetic eschar. Restrictive diastolic pattern, with data suggestive of elevated LVEDP. Slight biauricular dilatation. Right ventricle (RV) of preserved size and contractility. Aortic root and visualised portion of proximal ascending aorta of normal size. MV: thin leaflets, not limited in its opening and mild central mitral insufficiency (MI). Aortic prosthesis: opening of occluders, gradients similar to previous studies, normal Doppler velocity index (IVT TSVI/Ao ratio: 0.47) and wide periprosthetic reflux, eccentric, running parallel to the interventricular septum, originating in the theoretical right coronary sinus (around 11:00 hours), short THP, holodiastolic inversion in descending thoracic aorta with end-diastolic velocity < 20 cm/s (18 cm/s) and only protodiastolic inversion in abdominal aorta, for all these reasons it is classified as grade III-IV/IV. A semilunar image is observed adjacent to the theoretical right coronary sinus, echodense, where the leak originates, which could be the origin of the leak. Light TI. Dilated inferior vena cava (IVC) with absence of inspiratory collapse.
Severe pulmonary hypertension (PHT). Mild PE allowing estimation of a mean PAP of 51 mmHg (31 mmHg + 20 mmHg DBP). Perihepatic fluid. Absence of pericardial effusion and intracavitary masses via this access route. Conclusions: moderate concentric left ventricular hypertrophy (LVH). Normal LVEF, with segmental asymmetries. Restrictive diastolic pattern. Mild MI. Grade III-IV/IV aortic periprosthetic leak. Severe PHT.
Transesophageal echocardiogram: left atrium without thrombus inside. Left atrial appendage without thrombus. Atrial septum intact. Aortic prosthesis: correct movement of hemidiscs, gradients similar to previous studies, Doppler velocity index within normality and two physiological intraprosthetic refluxes for prosthesis. A crescentic image was observed between the theoretical right coronary sinus and non-coronary sinus, between 9:00 and 12:00 hours, of about 0.5 cm2 area, suggesting prosthetic dehiscence at this level and from which originates aortic periprosthetic leak with wide jet that runs parallel to the interventricular septum and deepens in LV reaching papillary muscles, with short THP, holodiastolic inversion in descending thoracic aorta with end-diastolic velocity > 20 cm/s, without observing reverse flow in abdominal aorta, for all these reasons it is classified as grade III-IV/IV. Mitro-aortic junction and periaortic region with normal echoic characteristics, without images suggesting abscesses or other periannular complications. Other findings superimposable to transthoracic study.
Conclusions: grade III-IV/IV aortic periprosthetic leak. Moderate LVH. Normal LVEF, with segmental asymmetries. Restrictive diastolic pattern. Severe PHT.
ECHOCARDIOGRAM after percutaneous closure procedure: aortic prosthesis: gradient improvement, currently maximum of 32 mmHg. Several periprosthetic leaks are seen (one at 11 hours and another at 3 hours) overall of somewhat lower grade than in the previous study but still grade III/IV (THP 280-310 ms, holodiastolic reversal of flow in isthmus and left femoral artery). Mild TR allowing estimation of PAPs 47 mmHg. IVC currently not dilated with normal collapse. Mild PI allowing an estimated mean PAP of 25 mmHg.
Current absence of pericardial effusion. Rest of study unchanged from previous.
Conclusions: Moderate eccentric LVH. Preserved LVEF. Inferobasal akinesia. AIo III/IV. Moderate PHT.

CLINICAL EVOLUTION
Clinical improvement during admission with loss of 13 kg of weight, decrease in abdominal perimeter and disappearance of oedema under intravenous perfusion of loop diuretic and inotropes.
Given the persistence of jaundice together with sustained high bilirubin levels and slight elevation of GGT, FA and LDH, an abdominal ultrasound was performed showing hepatomegaly suggestive of chronic liver disease and splenomegaly.
The echocardiogram showed severe aortic insufficiency which was confirmed by TEE (performed after depletive treatment), visualising a grade IIIIV/IV periprosthetic aortic leak with severe pulmonary hypertension. In view of these findings, the case was presented at a medical-surgical session and, given the patient's comorbidity, percutaneous closure of the periprosthetic leak was considered the best option.
During admission, closure of the aortic paravalvular leak was performed. The procedure was very difficult due to the difficulty in advancing the guidewires through the leak, achieving partial closure of the leak by means of a ductus closure device. There were no complications in the vascular access and the patient remained haemodynamically stable at all times. Subsequent evolution was good, and a transthoracic echocardiogram was performed which showed an improvement in aortic regurgitation, although a grade II/IV periprosthetic leak persisted with a decrease in pulmonary pressure, for which reason he was discharged. The patient is currently under outpatient follow-up by the heart failure team, having required only one hospital admission since then for decompensated heart failure in the context of respiratory infection.

DIAGNOSIS
Right-sided congestive heart failure. Periprosthetic aortic leak with grade III-IV/IV insufficiency, with partial closure by percutaneous procedure.
Chronic ischaemic heart disease.
Myelodyspastic syndrome (anaemia + thrombopenia).
