Disease Severity Score of Damy et al for Hereditary Transthyretin Amyloidosis (ATTRv): Cardiac and Cardiovascular Involvement

Description

Damy et al reported a disease severity score for a patient with hereditary transthyretin amyloidosis (ATTRv). A group of findings relates to cardiac and cardiovascular involvement. The authors are from multiple institutions in Europe.

Patient selection: hereditary transthyretin amyloidosis

Parameters for cardiovascular involvement:

(1) orthopnea

(2) dyspnea

(3) edema (pitting edema of ankles)

(4) orthostatic hypotension on tilt table testing

(5) AV first degree block on ECG

(6) left anterior hemiblock on ECG

(7) left posterior hemiblock on ECG

Parameters for cardiac disease:

(1) heart failure

(2) diastolic LV wall thickness on echocardiography

(3) interventricular wall thickness on echocardiography

(4) end-diastolic interventricular septum thickness of echocardiography

(5) heart valve function on echocardiography

(6) diastolic function on echocardiography

(7) left ventricular ejection fraction on echocardiography

(8) left ventricular longitudinal strain on echocardiography

(9) brain natriuretic peptide (BNP)

(10) high-sensitivity cardiac troponin T concentration

Parameter	Finding	Points
orthopnea	absent	0
	present	1.26
dyspnea	absent	0
	present	1.21
edema	absent	0
	present	0.89
orthostatic hypotension	absent	0
	present	1.18
AV first degree block	absent	0
	present	0.56
left anterior hemiblock	absent	0
	present	0.46
left posterior hemiblock	absent	0
	present	0.46

subscore for cardiovascular involvement =

= SUM(points for all of the parameters)

Parameter	Finding	Points
heart failure	absent	0
	present	1.53
diastolic LV wall thickness	normal	0
	abnormal	0.81
interventricular wall thickness	normal	0
	abnormal	0.91
end-diastolic interventricular septum	normal	0
	abnormal	0.97
heart valve function	normal	0
	abnormal	0.54
diastolic function	normal	0
	abnormal	0.91
left ventricular ejection fraction	normal	0
	abnormal	1.32
left ventricular longitudinal strain	absent	0
	present	1.21
serum BNP	normal	0
	elevated	1.16
serum high-sensitivity troponin T	normal	0
	elevated	1.08

subscore for cardiac disease =

= SUM(points for all of the parameters)

Interpretation:

• minimum subscores: 0

• maximum subscore for cardiovascular involvement: 6.02

• maximum subscore for cardiac disease: 10.44 (10.43 in paper)

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Disease Severity Score of Damy et al for Hereditary Transthyretin Amyloidosis (ATTRv): Cardiac and Cardiovascular Involvement

Description

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