Increased type I collagen synthesis in victims of sudden cardiac death due to idiopathic myocardial fibrosis

Figures & data

Figure 1. The collagen synthesis pathway.

Table I. Detailed patient characteristics.

									More detailed location of fibrosis
	Sex	Age	BMI	Heart weight (g)	Description of the fibrosis and other information	Prior cardiac history	Family history of SCD	Cardiac medication	LV anterior wall	Septum	LV lateral wall	LV posterior wall	RV
Patient 1	Male	38	25.4	409	Focal fibrosis in LV	No	No	No	Yes	No	Yes	No	No
Patient 2	Female	65	23.4	331	Patchy fibrosis in septum	No	No	No	No	Yes	No	No	No
Patient 3	Female	30	22.8	335	Patchy fibrosis in LV	No	Yes	No	Yes	No	No	No	No
Patient 4	Female	14	23.3	260	Focal fibrosis in LV posterior wall, diffuse fibrosis in RV, LMNA mutation	No	Yes	No	No	No	No	Yes	Yes
Patient 5	Female	43	17.8	212	Perivascular fibrosis and diffuse fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 6	Male	50	29.7	410	Diffuse fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 7	Male	43	26.3	416	Diffuse fibrosis in LV	Alcohol abuse-related atrial fibrillation	No	No	Yes	No	No	No	No
Patient 8	Male	37	22.1	418	Severe patchy fibrosis in whole myocardium	No	No	No	Yes	Yes	Yes	Yes	Yes
Patient 9	Male	75	21.0	363	Patchy perivascular fibrosis and diffuse fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 10	Female	36	30.3	322	Diffuse fibrosis in LV and perivascular adipose tissue/fat cell infiltration	Small VSD	No	No	Yes	No	No	No	No
Patient 11	Female	73	26.7	390	Patchy fibrosis in whole myocardium	No	No	No	Yes	Yes	Yes	Yes	Yes
Patient 12	Female	65	27.7	402	Patchy fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 13	Male	64	29.8	354	Patchy and interstitial fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 14	Female	20	19.5	229	Patchy fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 15	Male	53	30.4	416	Diffuse fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 16	Female	66	27.1	320	Diffuse fibrosis in LV	No	No	No	Yes	No	No	No	No
Patient 17	Male	50	32.6	269	Patchy fibrosis in whole myocardium	No	No	No	Yes	Yes	Yes	Yes	Yes
Patient 18	Male	35	23.0	386	Mild diffuse fibrosis	No	No	No	Yes	Yes	Yes	Yes	Yes

BMI = body mass index; LV = left ventricle; RV = right ventricle; SCD = sudden cardiac death; VSD = ventricular septal defect.

Table II. Characteristics of idiopathic myocardial fibrosis (IMF) cases and controls.

Characteristic	IMF cases (n = 18)	Controls (n = 27)	P
Age, y	46.8 (± 17.3)	45.1 (± 16.9)	NS
Sex, male	11/18 (55.0)	19/27 (70.4)	NS
BMI	25.8 (± 4.3)	25.1 (± 4.8)	NS
Hypertension	1/18 (5.6)	0 /27 (0.0)	NS
Smoking	2/18 (11.1)	6/27 (22.2)	NS
Hypercholesterolemia	0/18 (0.0)	0/27 (0.0)	NS
Diabetes	0/18 (0.0)	0/27 (0.0)	NS
Heart, g	386.1 (± 59.6)	354.0 (± 53.5)	NS
PINP-positive collagen area (%)	27.2 (± 6.5)	8.6 (± 2.2)	0.007

Values are expressed as mean (SD) or number of subjects (%). PINP-positive collagen area is expressed in percentages of the section area. Probability values refer to chi-square and two-sided t test analyses between groups.

BMI = body mass index.

Figure 2. Immunohistochemical staining of PINP in myocardium of idiopathic fibrosis case (A) and in control case (B). Magnification × 200. The amount of PINP (stained brown) was increased in the fibrotic tissue of the IMF case in comparison with control sample.

Figure 3. Immunohistochemical staining of ICTP in myocardium of idiopathic fibrosis case (A) and in control case (B). Magnification × 200. ICTP reflecting collagen type I degradation did not differ between the IMF case and control myocardium.

Figure 4. Immunohistochemical staining of PIIINP in myocardium of lamin A/C mutation case (A) and in control case (B). Magnification × 200. The amount of PIIINP (stained brown) was slightly increased in the IMF case in comparison with the control case.

Figure 5. Immunohistochemical staining of IIINTP in myocardium of idiopathic fibrosis case (A) and in control case (B). Magnification × 200. The amount of IIINTP did not differ between the IMF case and control myocardium.