A patient with myocardial hypertrophy

1. A patient with myocardial hypertrophy Ρ?Ρ?΄Κ»¨Κ»¨κ» ΡΟ[ΠΟΠΟΙΘ Ρ ΡΙ ΡΙΤΣΑΤΟΣ ΚΩΝ/ΝΟΣ RitsatosKonstantinos

2. Case • 30 years old patient • Atypical symptoms • Clinical examination normal • Basic laboratory data normal • Abnormal ECG • Abnormal TransthoracicEchocardiogram

3. ECG

10. Ε Normal diastolic function E/Ea :6 ε

11. Laboratory -Workup • Complete Blood Count • Hct 43 Hb14.3 MCV 88.5 MCH 29.5 • WBC 7500/μl (P 62%/L 26%/M 10%/E 1%) • PLT 240000/μl • Glc 90mg/dl • Urea 33mg/dl, Cr 1.1mg/dl, • K 4.1mmol/l, Na 141mmol/l, Mg 2.1mg/dl, Ca 9.8mg/dl, P 3.2mg/dl • BILD 1.3mg/dl, BILT 0.46mg/dl • SGOT 18 U/L, SGPT 20 U/L, ALP 66U/L, γGT 18U/L • UA 5.5mg/dl • CPK 89U/L, MB 9.0U/L • LDH 188U/L • Amylase 104U/L • Total Protein 7.8g/dl, Albumin 4.7g/dl • ESR 12,CRP 3.89mg/dl • TSH 1.77μIU/ml • BNP 13pg/ml • TnI 11.9pg/ml (<34) • Serum Protein Electrophoresis (N) • C3, C4 (N)

12. 24h ECG • Sinus Rhythm • Max HR 153bpm, Min HR 51bpm, Mean HR 71bpm • 2 Single VPBs • 2 Single SVPBs • No pauses

13. ΤΤΕ performed 5 years ago No findings mentioned

14. Pedigree

15. Uncle's ECG & TTE

16. Differential diagnosis • Cardiac tumors • vs • Atypical fοrm of HCM

19. CMR

20. DIMENSIONS

21. LGE

22. CMR Report • Mass dimensions > 5mm • LV Inferior wall • T1w/ Cine isointense • T2w hypointense, heterogenic • Rapid first pass perfusion, heterogenic NON CONTRACTILE

23. RCA Angiography,Left Ventriculography

24. Mass Characteristics MRI TTE 35x49mm Basal Inferior wall Contrast infusion (++) Non contractile Non compatible with HCM features: ↑Size Atypical location Non contractile Hypointense T2w Isoitense T1W • Large mass • LV Inferior wall • T1w/ Cine isointense • T2w hypointense, heterogenic Angiography • Normal coronary vessels • Inferior wall

25. PET Scan • Intensely metabolically active mass • Most likely malignant aetiology • Sarcoma type neoplasia • Alternative: Cardiac lymphoma

26. MR imaging of cardiac tumors and masses: a review of methods and clinical applications. Motwani M, et al. Radiology. 2013 Jul;268(1):26-43

27. Differential Diagnosis The LGE (++) heterogenic by CMR, the intense Vascular Network the positive Uptake at PET/SCAN could suggest: ·Sarcoma (angio?) ·Lymphoma ·Angioma ·Fibroma

28. Biopsy not performed Patient denied

29. 2 YEARS LATER 2015 2017

30. 22015 22017

31. ECG 2017

32. Exercise test • Treadmill, Bruce protocol • 12min • 13,5METS • Max HR 181bpm (96% predicted) • Inotropic & chronotropiccompetance • No complaints • No arrhythmias

33. 24h Rhythm • Sinus Rhythm • Max HR 160bpm, Min HR 48bpm, Mean HR 81bpm • 14 Single VPBs • 1 Single SVPBs • No pauses

34. Ευχαριστώ πολύ