Ventricular Septal Defect With Secondary Left Ventricular–to–Right Atrial Shunt Is Associated With a Higher Risk for Infective Endocarditis and a Lower Late Chance of Closure

Abstract

OBJECTIVE. Although ventricular septal aneurysm may diminish or even close the shunt through the ventricular septal defect (VSD), developing a left ventricular–to–right atrial (LV-RA) shunt may be unfavorable. This study sought to clarify this issue on the basis of an extended observation of such patients. METHODS. Sixty-eight patients (1201 patient-years) who had small perimembranous VSD and LV-RA shunt and were not operated on before 6 years of age were studied. RESULTS. The onset age of LV-RA shunt was 5.8 ± 3.3 years, with clinical improvement later observed in 23 (34%). The murmur disappeared and showed spontaneous closure in 5 (7%). Seven episodes of infective endocarditis occurred in 6 (8.7%, or 58 per 10000 patient-years), with 2 receiving surgery. Another 4 received surgery during follow-up. With echocardiography, aneurysmal transformation involving the anterior and septal leaflets of tricuspid valve (double sac) was found in 56 (85%), whereas only the septal leaflet (single sac) was involved in 10. Patients with double sac were less likely to show improvement, whereas patients who had superior QRS axis and were female showed clinical improvement more frequently. CONCLUSIONS. VSD with secondary LV-RA shunt is associated with a higher risk for infective endocarditis but still has a low chance for late improvement and even closure.

Reappraisal of Left Ventricular to Right Atrial (LV-RA) Shunt in Pediatric Patients With Isolated Perimembranous Ventricular Septal Defect

“…Wu et al reported that VSD with secondary LV-RA shunt is associated with a higher risk for infective endocarditis, but still had a low chance for late improvement and even closure. 5 In the present series, the event-free probability was not significantly different in both groups. LV-RA shunt may not aggravate the hemodynamic changes in patients with mild VSD, but may contribute to congestive heart failure and the need for surgical intervention in patients with a large VSD.…”

Expert review Left ventricular to right atrial shunt (Gerbode defect): congenital versus acquired

“…In the surgical operations, a patch repair should be performed on the right atrial side in order to prevent atrioventricular block [ 59 , 94 ]. In long-term follow-up (up to 19.7 years) 17% of the LV-RA shunts spontaneously closed, while 8.5% developed IE during follow-up [ 105 ]. The nature of spontaneous closure was proven, by surgical exploration, to be aneurysmal transformation of the tricuspid septal leaflet [ 58 ].…”

A Rare Type of Gerbode Defect

“…They recommend closing essentially all Gerbode defects. In a more recent follow-up study from same group, Wu et al 8 reclassified the aneurysmal transformation in patients with LV-RA shunts, and reported that the aneurysmal transformation involving the anterior and septal leaflets of tricuspid valve (double sac) was found in 85%, whereas only the septal leaflet (single sac) was involved in 15%. Perimembranous VSD may diminish in size or spontaneously close by "aneurysmal transformation" (i.e., adherence of septal or ATL tissue of the tricuspid valve or adjacent tissue onto the VSD), but this may cause LV-RA shunts, as well as subaortic ridges.…”

“…7 Type A morphology was closely associated with the development of LV-RA shunt while types C and D were most commonly seen in those spontaneously closed defects and those with only interventricular shunt. In a more recent follow-up study from same group, Wu et al 8 reclassified the aneurysmal transformation in patients with LV-RA shunts, and reported that the aneurysmal transformation involving the anterior and septal leaflets of tricuspid valve (double sac) was found in 85%, whereas only the septal leaflet (single sac) was involved in 15%. In the case presented, a single sac of ATL forming an aneurysm, covering the VSD and causing LV-RA shunt has been described.…”