Background/Purpose: There is no specific treatment for primary cardiac involvement in SSc. Even if heart transplantation is an option, only 1 case have been reported¹. The aim of the study was to collect data of patients with SSc and a primary cardiac involvement requiring a heart transplantation in order to establish the clinical course and expectable outcomes for this procedure.

Methods: Retrospective chart review of patients with SSc and a primary cardiac involvement requiring a heart graft in one of the major transplantation centers in France.

Results: A national survey allowed us to identify 6 patients fulfilling ACR/EULAR 2013 classification criteria for SSc. They had a history of primary cardiac involvement with an unequivocal indication for heart transplantation. 4/6 patients were women, 50% had lcSSc and 1 patient had an overlap with RA (Table 1). The median age at SSc diagnosis was 28 years and time to cardiac dysfunction diagnosis was 2.5 years. All patients had at least another systemic involvement, mostly gastrointestinal and/or musculoskeletal. Immunosuppressive treatment excluding corticosteroids has been prescribed to 3 patients.

In the year before transplantation, all patients were classified NYHA functional capacity III or IV and 5 of them required at least 1 hospitalisation. Median time from cardiac dysfunction diagnosis to transplantation was 4 years. The leading indication was heart failure requiring intravenous vasopressors except for 1 patient who was transplanted for recurrent ventricular arrhythmia. Cardiac pre-transplantation structural, functional and hemodynamic data are presented in Table 2.

The histopathology specimen of the explanted heart revealed myocardial fibrosis compatible with SSc primary cardiac involvement in all patients. Infectious complications occurred in 4 patients, 2 patients had ischemic lesions and 1 patient died from an unexplained graft failure. Median intensive care unit stay after the surgery was 22 days. During a median follow-up of 2.8 years, 4 patients had at least one acute cellular rejection, mainly of mild grade. Mild heart allograft vasculopathy occurred after a median of 2 years in 3 of 4 patients in whom coronary arteries were explored.

Conclusion: Symptomatic cardiac involvement in SSc has a bad prognosis. Heart transplantation is a relatively safe life-saving procedure in carefully chosen SSc patients with primary cardiac involvement manifesting with progressive dysfunction and/or arrhythmic complications.

Reference:

1. Martens E. Transplantation. 2012

Table 1: Patients’ characteristics

Patient

Sex

SSc subset

Age at SSc diagnosis (years)

Antibody profile

SSc diagnosis to cardiac involvement (years)

Other systemic manifestations

Previous IS

Indication for heart transplantation

Post-op infectious complications

Post-op ischemic complications

Post-transplantation IS (ongoing)

Post-transplantation follow-up (years)

Acute rejection (number, intensity)

Time to allograft vasculopathy (years)

Vital status up to May 2014

1

F

DcSSc

15

Anti-Scl70+

5

Upper GI, arthritis, myositis

MMF, CS

Recurrent ventricular tachycardia

0

0

Tacrolimus, Everolimus

0,5

No

Alive

2

F

DcSSc

12

ANA+

12

Upper + lower GI, arthritis, myositis

D-Pen

Global heart failure

5

Yes

MMF, Everolimus

3

1, severe

2

Alive

3

F

LcSSc

35

ANA+

2

Upper GI, arthritis, myositis

CS

Global heart failure

1

Yes

MMF, Tacrolimus

0,1

No

Deceased

4

H

LcSSc

46

ANA-

2

Upper GI

No

RV heart failure

1

0

MMF, CSA

10

2, mild

7

Alive

5

H

LcSSc

32

ANA-

3

Upper GI

No

Global heart failure

0

0

CSA, Everolimus

12

2, mild

10

Alive

6

F

RA overlap

24

ANA+, RF+, anti-CCP+

1

Arthritis

TCZ, MTX, CS

Global heart failure

1

0

MMF, CSA

2,5

1, mild

1

Alive

Median

28

2,5

2,75

Table 2: Cardiac structural and hemodynamic values before transplantation

Patient	LVEF (%)	LV filling pressures	RV dysfunction	MRI – Gadolinium enhancement	mPAP (mmHg)	RAP (mmHg)	Wegde pressure (mmHg)	PVR (dyn x s x cm-5)	CI (L/min/m2)
1	35	N	Yes	T1 Biventricular	7	5	5	56	1,70
2	25	↑	Yes		32	12	22	192	2,60
3	17	N	Yes	No	30	11	25	190	1,40
4	27		Yes		22	20	18	145	1,10
5	15		Yes	No	12	7	8	72	2,90
6	15		Yes	No	20	1	10	149	3,23
Median	21				21	9	14	147	2,15

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