Related Experiment Videos
Severe involvement of the conduction system in a patient with sclerodermal heart disease. An electrophysiological
Insights
Progressive systemic sclerosis can cause syncopal symptoms due to heart conduction disorders, not always diffuse myocardial damage. Electrophysiological evaluation is crucial for managing sudden death risk in scleroderma heart disease.
Area of Science:
- Cardiology
- Rheumatology
- Electrophysiology
Background:
- Progressive systemic sclerosis (PSS) is an autoimmune disease that can affect multiple organs, including the heart.
- Cardiac involvement in PSS, known as scleroderma heart disease, can manifest in various ways, including conduction abnormalities.
- Syncopal symptoms in PSS patients warrant thorough investigation to rule out cardiac causes.
Observation:
- A case of PSS presenting with syncope and an initial electrocardiogram (ECG) pattern suggestive of anterior myocardial infarction is described.
- Despite the ECG findings, coronary angiography excluded significant coronary atherosclerotic heart disease.
- The patient's ECG evolved to show advanced right bundle branch block with left posterior fascicular block.
Findings:
- Advanced cardiac conduction abnormalities, specifically a markedly prolonged H-V interval on His bundle electrogram, were identified.
- Echocardiography, heart catheterization, and angiographic studies revealed no significant mechanical impairment of ventricular function.
- This suggests that intraventricular conduction disorders in scleroderma heart disease may arise from localized conduction system involvement rather than diffuse myocardial disease.
Implications:
- This case highlights that conduction system disease in scleroderma heart disease can occur independently of widespread myocardial damage.
- The presence of significant conduction abnormalities indicates a potential risk for sudden cardiac death.
- Electrophysiological evaluation is recommended for selected PSS patients with cardiac involvement to assess and manage this risk.
Abstract:
A case of progressive systemic sclerosis with syncopal symptoms is reported. The presenting ECG pattern was that of an anterior myocardial infarction. The clinical history and the coronary angiography excluded significant coronary atherosclerotic heart disease. The ECG pattern evolved from the infarctual pattern associated with right bundle branch block to probably major degree of right bundle branch block associated with left posterior fascicular block. M-mode echocardiography, heart catheterization and angiographic studies did not reveal significant mechanical impairment of the left or right ventricle function. His bundle electrogram documented a markedly prolonged H-V interval, confirming an advanced impairment of distal conducting system. This case supports the suggestion that intraventricular conduction disorders in sclerodermal heart disease are not always related to diffuse myocardial involvement. The risk of sudden death justifies accurate electrophysiological evaluation in selected patients with sclerodermal cardiopathy.