Scleroderma

  A systemic disease with multiple organ involvement.

  Cardiac manifestations of SSc can affect all structures of the heart and may result in pericardial disease,

      arrhythmias, conduction system abnormalities, direct myocardial disease such as myositis,

      cardiac failure, cardiac fibrosis, coronary artery diseases and, rarely, primary valvular involvement.

  An abnormal ECG is present in approximately 50% of patients with SSc and

      is considered an independent predictor of mortality.




ECG findings in Scleroderma

  Nonspecific ST-T wave abnormalities (12.1%)

      significantly associated with elevated right ventricular systolic pressure on echocardiogram

 
Septal infarction pattern (10%) related to abnormal perfusion in the septum and anteroseptal wall

      in the absence of extramural coronary artery disease (probably due to underlying myocardial fibrosis).

 
First-degree atrioventricular block (8%),

      significantly associated with elevated right ventricular systolic pressure on echocardiogram

  Complete
right bundle branch block with or without left anterior hemiblock (2.6%)

      It has been suggested that
right bundle branch block is an independent predictor of mortality,

      and should be considered a marker of disease severity in SSc (possibly because that RBBB is either

      a surrogate for primary cardiac involvement [myocardial fibrosis] or undiagnosed pulmonary

      hypertension in SSc patients).

  Second- and third-degree
atrioventricular block (<2%)

 
Left bundle branch block (1%)

 
QTc prolongation

  Increased number of
Ventricular ExtraSystoles (VES, VPS, VPC) on Holter recordings.



References (including links to abstracts and some free full-text articles)

  Rheumatology 2014;53:1172-1177.

      The Scleroderma Clinical Trial Consortium (SCTC) Cardiac Subcommittee

  Ann Intern Med 1981;94:38-40.

  Am J Med 1988;84:1007-1015.

  PLoS One 2013 Oct 31;8(10):e78808.

  Scand J Rheumatol 2014;43:221-225.





ECG 1.
Third degree (complete) AV block in a 68 years-old woman with Systemic Sclerosis (Scleroderma).
Multidetector CT coronary angiography showed normal coronary arteries.

Click here for a more detailed ECG

The ECG above has been used with the permission of Anatolian Journal of Cardiology and AVES Publishing.

Click here to see the related article of Dr. Canpolat et al.





ECG 2. The ECG above belongs to a 54 years-old woman.
She has had Scleroderma for almost 8 years.
Left bundle branch block pattern is seen. Limb leads show
low voltage.

Click here for a more detailed ECG





ECG 3. The ECG above belongs to a woman with
Scleroderma and pulmonary hypertension.
PR interval prolongation (
first-degree AV block) and P wave widening (140 milliseconds, interatrial block) are seen.
Right bundle branch block is seen. The tall R wave in V1 is suggestive of right ventricular hypertrophy.

Dr. Peter Kukla has donated the above ECG to our website.

Click here for a more detailed ECG