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Heart failure aggravated by beta blockers. Could this suggest the etiology?

Session Poster Session 4

Speaker Robert-Daniel Adam

Event : Heart Failure 2019

  • Topic : arrhythmias and device therapy
  • Sub-topic : Hypertrophic Cardiomyopathy
  • Session type : Poster Session

Authors : R-D Adam (Bucharest,RO), A Jercan (Bucharest,RO), S Badelita (Bucharest,RO), AG Fruntelata (Bucharest,RO), R Ciudin (Bucharest,RO), BA Popescu (Bucharest,RO), C Ginghina (Bucharest,RO), M Draghici (Bucharest,RO), C Stan (Bucharest,RO), D Coriu (Bucharest,RO), R Jurcut (Bucharest,RO)

Authors:
R-D Adam1 , A Jercan2 , S Badelita2 , AG Fruntelata3 , R Ciudin1 , BA Popescu1 , C Ginghina1 , M Draghici4 , C Stan5 , D Coriu2 , R Jurcut1 , 1Institute of Cardiovascular Diseases Prof. C.C. Iliescu, Cardiology - Bucharest - Romania , 2Fundeni Clinical Institute, Hematology - Bucharest - Romania , 3Monza Hospital, Cardiology - Bucharest - Romania , 4Fundeni Clinical Institute, Neurology - Bucharest - Romania , 5Fundeni Clinical Institute, Nuclear Medicine - Bucharest - Romania ,

Citation:

Cardiomyopathies (CM) are a heterogenous group of myocardial diseases associated with mechanical and/or electrical dysfunction due to various causes, most frequently genetic. Cardiac amyloidosis (CA) is a restrictive CM characterized by extracellular accumulation of misfolded protein fragments, most frequently classified by the amyloid precursor. Transthyretin amyloidosis (ATTR) results from accumulation of a protein (transthyretin), produced by the liver and is subdivided in 2 different types – wild type (wt) and hereditary (mt).

We present the case of a 42 yo patient with family history of heart failure (HF) and death at young age, evaluated in our clinic for aggravated dyspnea and fatigue in the last few weeks. The patient was recently diagnosed with HCM, and was started on beta-blocker (BB) therapy, which led to further worsening of his symptoms. The physical examination was unremarkable and the laboratory workup showed mild renal involvement and high levels of NTproBNP. The ECG showed synus rhythm with a 2:1 atrioventricular block (AVB), major RBBB and normal voltage, leading to interruption of BB, the patient reverting to normal conduction the next day (80 bpm). The patient had a 48 h ECG Holter monitoring that showed multiple and prolonged episodes of high grade and even complete AVB with a heart rate of 28 bpm, as well as episodes of nonsustained VT. The echocardiography showed severe biventricular hipertrophy, normal global systolic function (LVEF), but important longitudinal dysfunction with apical sparing and severe diastolic dysfunction with restrictive filling pattern.

Cardiac imaging, conduction disturbances as well as the patient’s origin in a country region which has a high incidence of ATTRmt raised the suspicion of CA. Thus, his workup was completed by a neurologic evaluation that showed bilateral carpal tunnel syndrome and a bone-avid phosphate-based isotope nuclear scintigraphy with important myocardial uptake of the isotope which is highly suggestive for ATTR.

The patient was implanted a  bicameral ICD, with favorable evolution. He also received a low dose of furosemide and spironolactone, with no symptoms at discharge. Family screening included 2 maternal cousins with normal echocardiography.

Although a rare cause of HF, CA remains one of the main phenocopies in the differential diagnosis of HCM, patients often presenting important and dramatic symptoms. Awareness of this entity as a cause of cardiac hypertrophy is of paramount importance for following the lead. Clear recommendations for the choice between pacemaker vs ICD are not yet released, therefore the individual decision should be made based on available data and risk stratification.  We stress the importance of a "red flags" based diagnosis (echocardiographic aspect, associated rhythm and conduction dysorders, poor tolerance of BB, neurologic involvement) and the importance of a careful family screening in rare genetic cardiovascular diseases.

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