Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum

1999 ◽  
Vol 9 (1) ◽  
pp. 99-103 ◽  
Author(s):  
B Sarubbi ◽  
L M Gerlis ◽  
S Y Ho ◽  
J Somerville
Keyword(s):  
Ventricular Septal Defect ◽  
Right Bundle Branch Block ◽  
Septal Defect ◽  
Atrioventricular Node ◽  
Post Mortem ◽  
Unexpected Death ◽  
Bundle Branch Block ◽  
The Right ◽  
Membranous Septum ◽  
Apparently Healthy

AbstractAn apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.

The effects of surgically induced right bundle branch block on left ventricular function after closure of the ventricular septal defect

2008 ◽  
Vol 18 (4) ◽  
pp. 430-436 ◽  
Author(s):  
Thais A. L. Pedersen ◽  
Niels H. Andersen ◽  
Mette R. Knudsen ◽  
Thomas D. Christensen ◽  
Keld E. Sørensen ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Left Ventricular Function ◽  
Right Bundle Branch Block ◽  
Septal Defect ◽  
Left Ventricular ◽  
Surgical Correction ◽  
Control Group ◽  
Bundle Branch Block ◽  
Surgical Closure ◽  
Control Subjects

AbstractObjectiveTo determine the long-term significance of right bundle branch block on left ventricular systolic and diastolic function in children subsequent to surgical closure of ventricular septal defect.MethodsWe studied 26 children who underwent surgical closure of a ventricular septal defect 11 ± 2 years postoperatively by use of conventional and tissue Doppler echocardiography, comparing the findings to those obtained from a control group. Of those having surgical correction 14 had postoperative right bundle branch block.ResultsIrrespective of the presence of right bundle branch block, the peak systolic velocity of the mitral ring was lower in those undergoing surgical correction, with values of 5.2 ± 1.4 cm/s in those with right bundle branch block, 5.4 ± 1.2 cm/s in those without right bundle branch block after surgical correction, and 6.6 ± 1.0 cm/s in the control subjects (p < 0.01). In terms of diastolic function, the early septal velocity of transmitral inflow divided by the early diastolic mitral annular velocity was significantly higher in children with right bundle branch block, at 12 ± 3.0 cm/s compared to 8.4 ± 1.5 cm/s in the control subjects (p < 0.01), but not significantly higher in the children without right bundle branch block after correction compared to the control group. The fractional shortening percentage was similar in both patients and control subjects. The changes noted in left ventricular function were not significantly related to age at surgery, the period of follow-up, or the surgical method.ConclusionsSystolic long axis function is significantly reduced in children after surgical closure of ventricular septal defects, irrespective of the presence of right bundle branch block. Diastolic dysfunction, in contrast, was observed primarily in children with post-operative right bundle branch block.

scholarly journals ETIOLOGY OF RIGHT BUNDLE BRANCH BLOCK (RBBB) FOLLOWING CLOSURE OF VENTRICULAR SEPTAL DEFECT (VSD)

1974 ◽  
Vol 8 (4) ◽  
pp. 353-353 ◽  
Author(s):  
Edwin O Okoroma ◽  
Barbara Guller ◽  
James D Maloney ◽  
William H Weidman ◽  
Ralph E Smith
Keyword(s):  
Ventricular Septal Defect ◽  
Right Bundle Branch Block ◽  
Septal Defect ◽  
Bundle Branch Block

The Gerbode Defect: The Significance of a Left Ventricular to Right Atrial Shunt

2009 ◽  
Vol 19 (S2) ◽  
pp. 96-99 ◽  
Author(s):  
Angela M. Kelle ◽  
Luciana Young ◽  
Sunjay Kaushal ◽  
C. Elise Duffy ◽  
Robert H. Anderson ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Tricuspid Valve ◽  
Right Atrium ◽  
Septal Defect ◽  
Left Ventricular ◽  
Right Atrial ◽  
Surgical Closure ◽  
The Mean ◽  
The Right ◽  
Membranous Septum

AbstractBackgroundThe so-called Gerbode ventriculo-atrial defect is a rare defect that permits shunting from the left ventricle to the right atrium. It takes 2 forms, either a deficiency of the atrioventricular membranous septum, or shunting initially through a ventricular septal defect, with atrial shunting occurring through a deficiency in the septal leaflet of the tricuspid valve. In this review, we describe the natural history and outcomes of surgical repair for the variant with shunting through a deficiency at the site of the atrioventricular membranous septum.MethodsFrom 1990 to 2008, we identified 6 patients from our departmental database who had undergone surgical closure of a congenital defect of the atrioventricular component of the membranous septum. Median age at repair was 1.6 years, with a range, from 0.4 to 19 years. All patients were symptomatic, with 3 having congestive cardiac failure, 2 failing to thrive, and 2 having intolerance to exercise. All had a dilated right atrium demonstrated by echocardiogram, with a mean preoperative gradient measured by echocardiogram to be 109 millimetres of mercury, with a range from 65 to 150 millimetres of mercury.ResultsThere was no operative or late mortality. The mean size of the defect was 6.2 ± 2.0 millimetres, with a range from 4 to 8 millimetres. All were closed by insertion of a patch. The mean period of cardiopulmonary bypass was 90.5 ± 11.3 minutes, the mean time of aortic cross-clamping 54.8 ± 6.9 minutes, and the mean length of stay in hospital 4.3 ± 1.0 days. No patient had a residual defect, and only trivial regurgitation of the tricuspid valve was evident by postoperative echocardiography. There were no complications or reoperations.ConclusionThe membranous ventriculo-atrial defect can be recognized echocardiographically on the basis of dilation of the right atrium in the setting of an unusually high Doppler echocardiogram gradient compared to the ventricular septal defect with shunting only at ventricular level. Since all patients in our series were symptomatic, we recommend surgical closure of all these defects.

scholarly journals P1491 Aneurysm of the membranous septum after spontaneous closure of ventricular septal defect combined with bicuspid aortic valve

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Drakopoulou ◽  
E Nyktari ◽  
S Soulaidopoulos ◽  
G Oikonomou ◽  
K Toutouzas ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Ventricular Septal Defect ◽  
Right Ventricular ◽  
Bicuspid Aortic Valve ◽  
Septal Defect ◽  
The Body ◽  
Heart Team ◽  
Severe Aortic Regurgitation ◽  
The Right ◽  
Membranous Septum

Abstract An 18-year-old asymptomatic patient with a cardiac background of ventricular septal defect (VSD) and bicuspid aortic valve diagnosed in early childhood, was referred to our Adult Congenital Heart Disease (ACHD) outpatient clinic for routine assessment. Imaging by transthoracic echocardiography and Cardiac Magnetic Resonance (CMR) showed a well-developed multilobulated appendiform saccular formation (34x20mm) arising from the right ventricular side of the membranous septum inferior to the anterior aortic cusp and just beneath the septal leaflet of the tricuspid valve, protruding into the right ventricular outflow tract and the body of right ventricle. The fibrous quality and the absence of myocardium in this structure led to the formation of an aneurysm of the membranous septum (AMS) with the characteristic outpouching or ‘windsock’ appearance from its distention during ventricular systole (Figure). There was no shunt between the ventricles. The aortic valve was true bicuspid with severe aortic regurgitation and an eccentric jet towards the anterior leaflet of the mitral valve. The left ventricle was dilated with preserved systolic function. On the basis of the above information the Heart Team decided for surgical management.Both the presence of a true bicuspid valve (embryologically linked to VSD) as well as the pre-existing left-to-right shunting (until the spontaneous VSD closure) seem to have contributed to aortic valve dysfunction in this case. An interesting physical phenomenon concerning fluid dynamics, known as the ‘Venturi effect’, occurs in patients with ‘aneurysmal transformation’ of the ventricular septum, where the VSD becomes smaller creating thus a low-pressure zone that affects the adjacent aortic valve cusp, causing prolapse and, hence, aortic valve regurgitation. Non-invasive imaging evaluation of patients with AMS is required for optimal diagnosis and treatment as well as for follow-up examinations. Echocardiography is an effective tool for diagnosing AMS, mainly as an incidental finding in asymptomatic subjects whereas CMR is capable of three-dimensional anatomical assessment and provides functional data about the blood flow into the aneurysm and integrity of the ventricular membranous septum. Abstract P1491 Figure

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