Cardiovascular involvement in systemic sclerosis: A case of atrial septal aneurysm associated with intracranial aneurysm

There is increasing concern about vascular and cardiac involvement in systemic sclerosis (SSc). This is a systemic infiltrative disorder that commonly affects the cardiovascular system and this case is presented to draw attention to the possibility of a pathophysiological connection between SSc and cardiovascular aneurysm formation. No data exist on the incidence or natural history of aneurysm in patients with SSc and to the best of our knowledge, there is no other report to date of atrial septal aneurysm associated with intracranial aneurysm in SSc. The heart is one of the major organs involved in SSc (1,2), the involvement of which can be manifested by myocardial disease, conduction system abnormalities and arrhythmias, or pericardial disease. Representative is the myocardial involvement by patchy fibrosis (secondary to both repeated ischemia and immune-inflammatory damage) that leads to ventricular diastolic dysfunction (3,4), whereas right ventricle overload and failure may complicate pulmonary hypertension. SSc is frequently linked to vascular and/or parenchymal lung disease (5), determining symptom occurrence, particularly dyspnoea and fatigue. Several vasodilator approaches (prostacycline or NO/endothelin) may counteract the microvascular dysfunction at peripheral and cardiopulmonary level and fight the sequelae of pulmonary hypertension (5) which is secondary to damage in the lung. There has been an increased awareness of left ventricular abnormalities in SSc patients, but the occurrence of cardiac aneurysms seems to be a rare complication of SSc, only few cases are illustrated in the literature (6). Vascular abnormalities such as fingertip ulcers and Raynaud’s syndrome as well as involvement of other organs including kidney and the gastrointestinal tract, are prominent features of the disease. Macrovascular involvement in SSc has received relatively little attention, the prevalence of peripheral large vessel disease is increased in SSc, on the other hand, the association with vascular aneurysm is poorly understood, few reports are sporadically described (7-9).