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A rare case of heart failure with preserved ejection fraction in Erdheim Chester syndrome, at the crossroad of many possible causes

Session Poster Session 4

Speaker Elena-Alina Patru

Event : Heart Failure 2019

  • Topic : heart failure
  • Sub-topic : Chronic Heart Failure: Comorbidities
  • Session type : Poster Session

Authors : A-E Patru (Bucharest,RO), M Anton (Bucharest,RO), D Coriu (Bucharest,RO), M Boros (Bucharest,RO), D Deleanu (Bucharest,RO), A Bardas (Bucharest,RO), C Ginghina (Bucharest,RO), BA Popescu (Bucharest,RO), C Jurcut (Bucharest,RO), R Jurcut (Bucharest,RO)

A-E Patru1 , M Anton1 , D Coriu2 , M Boros2 , D Deleanu1 , A Bardas2 , C Ginghina1 , BA Popescu1 , C Jurcut3 , R Jurcut1 , 1Institute of Cardiovascular Diseases Prof. C.C. Iliescu - Bucharest - Romania , 2Fundeni Clinical Institute - Bucharest - Romania , 3Carol Davila Emergency Clinical Military Hospital - Bucharest - Romania ,


Introduction We present the case of a 71 year old man, asymptomatic until 2017 when he was admitted to hospital for recurrent pleurisy. CT scan shows pleurisy and pericarditis and also bilateral periaortic and perirenal dense, fibrotic tissue, highly suggestive for Erdheim Chester disease. Perirenal biopsy was made, histological examination showing histiocyte inflitrations that expressed CD68 / PGM1 fascine and factor XII.Also,he had a history of borderline coronary atherosclerosis.
Patient management In February 2018, he develops progressive shortness of breath, fatigue and severe uncontrolled hypertension. Lab test showed progressive kidney failure (creatinine level of 2.5 mg/dl, relative to 1.5 mg value in 2017). TTE showed normal size cardiac cavities, preserved systolic function (LVEF 50-55%),inferobasal hypokinesia and infiltrated pericardial layer. Contrast angioCT reveals massive infiltration in the arterial aortic wall, significant left renal artery stenosis and no flow in the right kidney artery, with subsequent renal atrophy. Renal arteriography confirmed severe left renal artery stenosis and a 7 mm stent is implanted. At 48h after stent implantation, creatinine was 1.3 mg/dl. At discharge, arterial blood pressure values are decreasing, requiring lower dose of antihypertensive drugs, and functional capacity was remarkably improved. During followup, the patient developed severe leuco-thrombocytosis, bone marrow biopsy and genetic testing established the diagnostic of chronic myeloid leukemia. Cytoreductive treatment with hydroxyurea was started, but after two weeks, general status was not improved, with frequent episodes of paroxysmal dyspnea, elevated blood pressure, markedly high NTproBNP levels (12943 pg/ml) and an echocardiographic LVEF of 50%, with diastolic dysfunction and mild pericardial effusion. Optimization of HF therapy and  stopping hydrea led to a reduction of NT proBNP levels (5292 pg/ml) and constant echocardiographic findings. However, while receiving hidroxicarbamide and imatinibe for the hematologic malignancy, several milder decompensations of heart failure episodes reoccurred.
Problems, possible differential diagnosis: Concerning the heart failure symptoms, we can make a differential diagnosis between myocardial ischemia (but with no angina, no new hypokinesia and no cardiac necrosis biomarkers), diastolic disfunction with increased filling pressure due to marked elevated arterial hypertension and chronic kidney disease, myocardial injury due to hystiocitar infiltration or cardiotoxicity of oncologic treatment for leukemia.
Conclusions : Erdheim Chester syndrome is a rare non-Langerhans cell histiocytosis that most commonly affects adults, often a fatal disease, with multi-organ damage and rapid progression, therefore diagnostic precocity is a key element. HF with preserved ejection fraction can have multiple causes in this setting (pericarditis, aortitis and medium size vasculitis, hematologic changes).

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