European Heart Journal (1993) 14, 1717-1718 Aneurysm of the interventricular membranous septum with thrombo-embolism — an indication for surgical repair? D. THOMAS*, J. SALLOUM* AND G. RANCURELt * Department of Cardiology, fDepartment of Neurology, Groupe Hospitalier Pitie-Salpetriere, 47 bdde VHopital 75651 Paris Cedex 13 France KEY WORDS: Aneurysm of the interventricular membranous septum, thrombo-embolism, surgery. A 40-year-old woman with a significant neurological history presented with right hemiparesia, paraesthesia of the right upper member and of the hemiface. Computer tomography scanning revealed hypodensity along the right lateral ventricle which corresponded to the left hemiplegia which had developed when she was 20 years old. Arteriography of the four cervical axes was normal. Eclwcardiography visualized an aneurysm of the membranous septum free of thrombosis without ventricular septal defect. The embolism was thought to be ofcardiac origin and a decision was taken to correct it by surgery. Ten years after surgical repair no other neurological event has occurred. Introduction Aneurysm of the interventricular membranous septum can spontaneously close a ventricular septal defect1'-21. Complications such as rupture, endocarditis or thrombosis are unusual'1"1. We report the occurrence of repetitive brain embolism in a patient with aneurysm of the membranous septum. Ten years after surgical repair no further occurrence of embolism has been observed. Case report A 40-year-old woman was admitted to our hospital for right hemiparesia, paresthaesia of the right upper member and of the right side of the face. Clinical examination revealed a blood pressure of 110/80 mmHg, a heart rate of 80 beats. min~' and a respiratory rate of 15min~'. Cardiac examination was normal, as were chest X-ray and electrocardiogram. Motor function of the right side was diminished. Paresthaesia of the left upper member with reduced power was also noted; it probably consisted of sequelae as a result of a previous neurological accident. This patient already had a long neurological history: regressive right hemiplegia and aphasia at the age of 3 months; an episode of vertigo, diplopia with occipital headaches at 19 years of age; left hemiplegia, anosognosia, left lateral homonymous hemianopsia and decreased sensitivity at 20 years of age. Occlusion of the right sylvian artery was revealed by arteriography. Cardiovascular examination, cerebro-spinal fluid (CSF) and coagulation tests were normal. Recovery of the left upper member function was only partial. Multiple episodes of transient right hemiparesia occurred at the age of 20. Despite anticoagulant therapy, using acenocoumarol with the dosage titrated to maintain a prothrombin time 1-5 to 2-5 the normal control times, she presented another neurological event with central vertigo, diplopia, right paresthaesia, Submitted for publication on 22 March 1993, and in revised form 7 July 1993. Correspondence: Pr Daniel Thomas, Department of Cardiology Groupe Hospitalier Pitie-Salpelriere, 47 bdde I'Hopital 75651 Paris cedex 13 France. 0195-668X/93/I2I717 + O2 $08.00,0 Figure I Left ventricular angiography in left anterior oblique view: a pouch-like structure bulges into the right ventricle during the systole (arrows). right thermoalgesic hypoaesthesia and left lateral nystagmus at the age of 35. CSF and coagulation tests were normal. Transmitral echocardiography revealed a lack of continuity between the aorta and the interventricular septum. Angiography revealed a pouch-like structure with distinct margins bulging into the right ventricle during systole and indicated the diagnosis of aneurysm of the membranous interventricular septum (AMS) (Fig. 1). Computed tomographic scanning was performed and revealed a hypodensity along the right lateral ventricle which was dilated and corresponded to the previous left hemiplegia at 20 years of age. Cervical echo-Doppler was normal, as was arteriography of the four cervical axes. Two-dimensional echocardiography showed the AMS to be free of thrombus. The cardiac origin of the embolism was confirmed and it was decided to proceed to surgery. A spherical aneurysmal pouch 1 cm in diameter was visualized protruding into the right ventricular outflow © 1993 The European Society of Cardiology 1718 D. Thomas et al. tract. There was no ventricular septal defect and the aneurysm was free of thrombus. Obliterating endoaneurysmorraphy was performed. After surgery, anticoagulant therapy was instituted for 6 months and then stopped. Ten years after surgical repair no other neurological events have been observed. Discussion Aneurysm of the membranous septum is almost always associated with a ventricular septal defect (VSD) and may be a marker for a defect that is likely to become smaller and close in the future'1-21. Diagnosis is made by twodimensional echocardiography and by angiography'31. Thrombo-embolic complications are very rare. Bush et al.li] reported the case of a 57-year-old patient presenting symptoms of congestive heart failure. Echocardiogram, magnetic resonance imaging, cardiac catheterization and cardiac surgery demonstrated an AMS containing a large thrombus associated with aortic stenosis. Pernot et a/.'21 reported right hemiplegia in a 3-year-old child known to have a VSD. The diagnosis of a large VSD was made at 5 months of age; it then became smaller. Repeat catheterization revealed AMS with a small VSD; at cardiac surgery the AMS was free of thrombosis. Soyer et a/.'4' reported syncope and regressive right hemiplegia in a 10-year-old boy who had a VSD diagnosed at 10 months of age. Echocardiogram, catheterization and surgery revealed an AMS free of thrombosis without any shunt. In our patient, the AMS was small and was not associated with VSD and repetitive cerebro-vascular embolism occurred. Thrombo-embolic complications in AMS are very rare but may be underestimated, as in the past with aneurysm of the interatrial septum which was not considered to be a potential cause of thrombo-embolism'51. In previously reported cases of AMS with thrombo-embolism, there was no VSD in one case'"1 and a small VSD in two cases'231; in our case there was no VSD. In large VSDs the blood flow through the AMS is important and blood stasis does not occur. Conversely, in cases with small or no VSDs, conditions are favourable for blood stasis and thrombosis. The size of the AMS does not seem to be a risk factor for thrombo-embolism; it was small in two cases'23' (as in our case) and large in one case'4'. Rhythm disturbances have been reported in association with AMS'61 but there were no cardiac arrhythmias in any of the reported cases of AMS with thromboembolism. The history of our patient was suggestive of repetitive cerebral embolism. Ten years after surgical repair no other neurological event had occurred. We believe that surgical repair for AMS is indicated when cerebral embolism occurs despite anticoagulant therapy in order to prevent other neurological accidents. References [1] Beerman LB. ParkSC. Fisher DR, etal. Ventricular septal defect associated with aneurysm of the membranous septum. J Am Coll Cardiol 1985; 5: 118-23. [2] Pernot C. Hoeffel JC. Henry M, Worms AM. Mentre B, Fhzot M. L'anevrysme du septum membraneux a propos de 20 observations Coeuret Med Int 1976; 2: 199-213. [3] Bush HS, Perin E, Massumi A, Klima T, Hall RJ. Detection of thrombus in an aneurysm of the ventricular septum. Am J Cardiol 1989; 63: 1533-5. [4] Soyer R, Bouchart F, David N, el al. Anevrysme du septum membraneux, a propos de 4 cas operes. Arch Mai Coeur 1990; 83: 85-90. [5] Schneider B, Hanrath P, Vogel P, Meinertz T. Improved morphologic characterization ofatrial septal aneurysm by transesophageal echocardiography: relation to cerebrovascular events J Am Coll Cardiol 1990; 16: 1000-9. [6] Langenfeld H, Meesmann M, Schanzenbacher P. Angeborenes Aneurysma des membranosen interventrikularen Septums Dtsch Med Wochenschr 1990; 115: 622-5