40 M. Oberson, S. Chevili

Applied Cardiopulmonary Pathophysiology 14: 40-42, 2010

Cardiovascular involvement in systemic sclerosis: A case of atrial septal associated with M. Oberson1, S. Chevili2 1Department of Cardiology, University Hospital of Bern, Bern, Switzerland; 2Department of Internal Medicine, Ospedale Italiano, Lugano, Switzerland

Introduction the sequelae of pulmonary (5) which is secondary to damage in the lung. There is increasing concern about vascular There has been an increased awareness and cardiac involvement in systemic sclerosis of left ventricular abnormalities in SSc pa- (SSc). This is a systemic infiltrative disorder tients, but the occurrence of cardiac that commonly affects the cardiovascular sys- seems to be a rare tem and this case is presented to draw atten- of SSc, only few cases are illustrated in the lit- tion to the possibility of a pathophysiological erature (6). connection between SSc and cardiovascular Vascular abnormalities such as fingertip aneurysm formation. No data exist on the in- ulcers and Raynaud’s syndrome as well as in- cidence or natural history of aneurysm in pa- volvement of other organs including kidney tients with SSc and to the best of our knowl- and the gastrointestinal tract, are prominent edge, there is no other report to date of atri- features of the . Macrovascular in- al septal aneurysm associated with intracra- volvement in SSc has received relatively little nial aneurysm in SSc. attention, the prevalence of peripheral large The heart is one of the major organs in- vessel disease is increased in SSc, on the oth- volved in SSc (1,2), the involvement of which er hand, the association with vascular can be manifested by myocardial disease, aneurysm is poorly understood, few reports conduction system abnormalities and arrhyth- are sporadically described (7-9). mias, or pericardial disease. Representative is the myocardial involve- ment by patchy fibrosis (secondary to both repeated ischemia and immune-inflammatory Case report damage) that leads to ventricular diastolic dysfunction (3,4), whereas right ventricle We hereby describe an atrial septal aneurysm overload and failure may complicate pul- associated with an intracranial aneurysm in a monary hypertension. 56-year-old woman presenting with increased SSc is frequently linked to vascular and/or esophageal motility disorders and rapidly pro- parenchymal lung disease (5), determining gressive diffuse cutaneous with symptom occurrence, particularly dyspnoea induration of all extremities and Raynaud’s and fatigue. Several vasodilator approaches phenomenon. (prostacycline or NO/endothelin) may coun- During routine TTE, an hypertrophic teract the microvascular dysfunction at pe- cardiomyopathy and an asymptomatic atrial ripheral and cardiopulmonary level and fight septal aneurysm (ASA) without shunting at Cardiovascular involvement in systemic sclerosis 41

Figure 1 two-dimensional color-Doppler echocardiog- aneurysm formation. Inflammatory reactions raphy, were detected. and endothelial damage seem to be common The ASA was protruding far away into the features in the formation and growth of in- right atrium with oscillation, and maximum tracranial aneurysms, too. displacement of ASA was evaluated between SSc is also characterized by 10 and 12 mm (Fig A). Initial pulmonary ar- and fibrosis of many organs. As previously de- tery hypertension at 45 mmHg, although a scribed, the underlying mechanism appears treatment with since two years for related to microcirculatory impairment caus- a severe form of Raynaud’s phenomenon ing focal ischemic injury and irreversible fi- (Fig. B), was measured. Serum BNP concen- brosis (12,13). The histological alterations are tration was moderately increased (274 ng/L) primarily found on the intima of the vessel indicating an overload of the right and/or left wall, increasing its collagen content (14). Im- ventricle. An incidentally found irregular in- mune complex deposition in the disease is tracranial aneurysm (10 x 6 mm; not shown) extensive and leads to vascular damage (15). during a magnetic resonance imaging per- Most of the effect is on small vessels and cap- formed for aspecific malaise/migraine con- illaries. firms the possible mechanism whereby sys- Although the occurrence of atrial septal temic sclerosis may result in aneurysm forma- aneurysm associated with an intracranial tion. aneurysm as in this case, may be pure coinci- dence, an inflammatory process could be sus- pected to be a cause for vascular aneurysm Conclusion formation and the same process could be postulated for other cardiovascular structures There is evidence of generalized endothelial leading to aneurysm formation. inflammation in patients with SSc (10), and A better knowledge and awareness of car- this is thought to be a precondition for diovascular involvement is necessary be- aneurysm formation (11); endothelial injury cause it conveys a major risk for mortality; a to vessels, followed by platelet adhesion and meticulous search for aneurysms seems to be leukocyte activation, plays a primary role in important in patients with SSc. Vascular and aneurysm formation. These events cause cardiac involvement in SSc is often manifest, complementary activation and release of and nearly always present when accurately and lysosomal enzymes, with searched. additional mechanical disruption of the vessel wall. Hemodynamic stress at the sites of ves- sel disruption may result in focal wall and 42 M. Oberson, S. Chevili

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