ƯNG DUNG CÔNG HƯƠNG TƯ

TIM TRONG CHÂN ĐOAN BÊNH

CƠ TIM THOAI HOA DANG BÔT

BS TRÂN THI XUÂN ANH

KHOA NÔI TIM MACH

BÊNH VIÊN ĐAI HOC Y DƯƠC

TRƯƠNG HƠP LÂM SANG

BN nam 36 tuôi , nhâp viên vi kho thơ , giam kha năng găng sưc tiêntriên trong 3 thang.

• Sinh hiêu : M: 101 bpm.NT: 23/min. HA: 70/50mmHg.

• SpO2: 89% khi phong

• TMC cô nôi/ 45 đô (+)

• Tim nhanh, đêu.

• Phôi giam thông khi hai đai phôi.

• Gan to 3cm dươi HSP

• Phu chân tơi gôi.

• NT pro BNP: 27743 ng/L

Xquang

ngưc

thăng

ECG

CINE CMR

LGE tăng tin hiêu muôn

Abdominal fat pad : lambda chain in apple – green color - polarized light- Red Congo stain.

SAU 13 CHU KY ĐIÊU TRI

• DEXAMETHASONE+ MELPHALAN / 4 TUÂN

• CHI SÔ NT PRO-BNP:

05/02/2018 15/6/2018 20/11/2018 28/1/2019 08/04/2019 13/5/2019 17/6/2019

27743 ng/L 22011 ng/L 7067 ng/L 5567 ng/L 4919 ng/L 4341 ng/L 5421 ng/L

2D M MODE DOPPLER

IVSd 26CM Ao 3.0 MV E VEL 0.69 M/S

LVIDd 1.7CM LA 4.1 MV DecT 199 MS

IVPWd 4.0CM MV Decslope 3.5m/s2

IVSs 1.7CM MV A VEL 0.41 M/S

LVIDs 2.1CM E/A 1.7

LVPWs 3.1CM

EDV( TEICH) 69ML

ESV( TEICH) 37ML

EF(TEICHOL)

46

% FS 23

SV( TEICH) 32

AMYLOIDOSIS• Môt bênh ly hiêm

• Sinh ly bênh la sư tâm nhuân cac protein dangsơi, amyloid, trong cac mô co liên quan.

• Co thê anh hương tơi nhiêu cơ quan: thân, tim, gan, hê hô hâp, tiêu hoa…

• Tiên lương xâu du hoa tri hay điêu tri băng cacliêu phap sinh hoc, ghep tuy.

• MRI cho thây nhiêu lơi thê trong hô trơ chânđoan khi viêc sinh thiêt không co săn.

CƠ TIM THOAI HOA DANG BÔT• Cơ tim thoai hoa dang bôt la tinh trang cac sơi

protein không hoa tan amyloid tâm nhuântrong tê bao cơ tim gây nên tinh trang phi đaicơ tim.

• BN co suy tim vơi EF bao tôn, rôi loan nhip, rôiloan dân truyên.

• Chân đoan xac đinh dưa trên mô hoc vơi sư xuât hiên ma cac protein mau xanh tao (apple green birefringence ) dươi anh sang phân cưckhi nhuôm đo Congo.

CAC DANG AMYLOIDOSIS

• Co hơn 30 loai protein amyloid, hiên đươc chia thanh 3 nhom chinh: – AL: light chain:

• chuôi nhe, immunoglobulin light-chains (λ,κ) tư tương bao ( plasma cells).

• Chiêm đa sô 90% amyloidosis• 50% anh hương tơi tim• Điên di miên dich cô đinh.

– ATTR: di truyên( mutation) va lơn tuôi ( wide-types) , Transthyretin.

– AA :• qua trinh viêm, thư phat: bênh thâp, loc mau thân nhân tao… • serum amyloid A protein

CAC DÂU CHƯNG LÂM SANG GƠI Y

• Hôi chưng ông cô tay hai bên

• Dê bâm da, măt gâu truc

• Suy tim vơi EF bao tôn, suy chưc năng tâmtrương năng , điên thê ECG thâp, gia nhôi mau

• Dây thanh tim, cac la van tim.

• Ha ap tư thê, HA thâp.

Examples of patient assessments showing cardiac involvement.

Martha Grogan et al. Heart 2017;103:1065-1072

Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.

Rodney H. Falk et al. JACC 2016;68:1323-1341American College of Cardiology Foundation

Diagnostic algorithm for cardiac amyloidosis.

Martha Grogan et al. Heart 2017;103:1065-1072

Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.

N = 21pt LGE 76%

Late gadolinium enhancement (LGE)

quantification

CoComparison between the contoured (a-d) and source (e-h) LGE images in the same slice position to illustrate the quantification method and the resulting difference in the burden of LGE from the basal (a and e) towards the apical segments (d and h). For each of the contoured slices (a-d) the endocardial (red) and epicardial contours (green) as well as the reference area of non-enhanced myocardium (blue) have been definedmparison between the contoured (a-d) and source (e-h) LGE images in the same slice position to illustrate the quantification method and the resulting difference in the burden of LGE from the basal (a and e) towards the apical segments (d and h). For each of the contoured slices (a-d) the endocardial (red) and epicardial contours (green) as well as the reference area of non-enhanced myocardium (blue) have been defined

Representative examples of VVI strain analysis and curves in the 2, 3,

and 4-chamber long axis views in a patient with cardiac amyloidosis.

The lower panel demonstrates regional strain curves, with the coloured curves corresponding to the segments in the upper panel. In each of the views, the basal segments are represented by the blue and green curves, the mid-ventricular segments by the white and yellow curves, and the apical segments by the turquoise and pink curves. Note the highest regional strain values are consistently seen in the apical segments (highlighted by the black arrows) in all three long-axis views

Possible biopsy sites include2:

• Salivary gland

• Subcutaneous fatty tissue of abdominal wall

• Rectal mucosa

• Gastric

• Kidney

• Sural nerve

• Tenosynovial tissue

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