Aneurysm Of Membranous Septum Research Articles

Abstract 12143: Hearing is Believing: A Case of a Ruptured Sinus of Valsalva Aneurysm

A 36-year-old previously active and healthy male was referred to our Adult Congenital Heart Disease clinic with worsening exertional dyspnea and intermittent palpitations over the past 6 months. He had recently been diagnosed with a membranous ventricular septal defect (VSD) and membranous septal aneurysm at another hospital, although images were not available for review.On physical exam, a continuous cardiac murmur was noted which prompted reconsideration of the diagnosis, with highest suspicion of ruptured sinus of Valsalva aneurysm (SVA). An urgent cardiac CT was obtained, which showed a large, mobile right SVA protruding into and partially obstructing the right ventricular outflow tract (RVOT), with pulmonic regurgitation and dynamic closure of a supracristal VSD (Figure 1A). A margin of the SVA was ill-defined and suspicious for rupture, although no definite communication was seen. An expedited TTE showed findings consistent with SVA rupture (Figure 1B), and he was admitted for surgery soon thereafter. Intra-operatively (Figure 1C), SVA rupture into the RVOT was confirmed by direct inspection; the SVA tissue was resected, the defect closed with a fresh autologous pericardial patch, and the VSD was closed with pledgeted sutures. Post-operatively, there was no leakage around the patch margin and only trace aortic regurgitation.A ruptured SVA causes continuous shunting from the aorta into an adjacent cardiac chamber and if left unrecognized, can lead to right heart failure, arrhythmia, and potentially sudden death, emphasizing the need for early identification. Echocardiography may show an aneurysmal sinus of Valsalva with a continuous (with accentuation in diastole) high-velocity, turbulent flow-jet into an adjacent cardiac chamber, and holodiastolic aortic flow reversal; a VSD is often present. Cross-sectional imaging is also important for diagnosis and surgical planning. A comprehensive physical exam is crucial to mitigate the risk of misdiagnosis.

Perimembranous ventricular septal defect with left ventricular volume overload: Features of patients in the French Cohort Study (FRANCISCO)

Management of perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload (Zscore of end diastolic LV diameter (LVEDD) > 2) without pulmonary arterial hypertension remains controversial. Our objective was to describe features of patients in the FRANCISCO registry, a prospective nationwide cohort study. Data were collected at inclusion. Echocardiographic data were cross-checked by a Core-lab. A total of 212 patients with a median age of 4-year-old [1; 13] have been included in 38 centres. LV was very dilated (Zscore of LVEDD > 3) in 38.6% of the study population with a relationship with maximum pmVSD size ( P < 0.01). Maximum pmVSD size was correlated with Zscore of LVEDD ( P < 0.01) and left atrial area ( P < 0.001). In patients aged 1 to 2 yo ( n = 87; 41%), dyspnoea (26%) was associated with a lower weight ( P < 0.001), left atrial area ( P < 0.01), medical therapy ( P < 0.01), a trend to a higher LVEDD Zscore ( P = 0.19) but not with pm VSD size. In 25.5% of cases, pmVSD were closed by catheterization or surgery, with management varying among French regions ( P < 0.001). PmVSD closure was performed in 19 children aged 1 to 2 yo (22%), in 23 children aged 3 to 15 yo (27%) and in 12 patients over 15 yo (29%), P = 0.6 ( Fig. 1 ). Factors associated with pmVSD closure were maximum pmVSD size in all patients ( P < 0.001); dyspnoea in children aged 1 to 2 yo ( P = 0.021); minor to mild aortic regurgitation ( P < 0.01), inlet or outlet pmVSD extension ( P < 0.01) and height of the membranous septal aneurysm ( P < 0.01) in children aged 3 to 15 yo; aortic cusp prolapse in patients over 15 yo ( P = 0.017). In France, pmVSD with LV overload are not systematically closed in patients above one year-old. Closure is mainly indicated in case of symptoms or associated anatomical lesions. Long-term outcomes are awaited to refine the therapeutic algorithm.

Membranous septal aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy.

The online version contains supplementary material available at 10.1007/s12055-021-01287-x.

Perimembranous ventricular septal defect closure via ultra-minimal trans intercostal incision in children.

This study aimed to investigate the safety, feasibility and, availability of perimembranous ventricular septal defect (PmVSD) closure via a left parasternal ultra-minimal trans intercostal incision in children. From January 2015 to January 2019, 131 children with restrictive PmVSDs were enrolled in this study and successfully done in 126 patients (96.18%). PmVSDs were occluded via an ultra-minimal trans intercostal incision (≤1 cm), and the entire occlusive process was guided and monitored by TEE. A pericardium hanging technique was employed without sternal incision. PmVSDs were closed through a short delivery sheath assembled using a concentric occluder device. All patients were followed up for a period ranging from18 months to 24 months. Thirteen patients with PmVSD had aneurysm of membranous septum (AMS). Multistream (≥2) PmVSDs with AMS were found in 11 cases. After the operation, mild residual shunt beside the amplatzer occluder in one patient was found and had self-healing result during the 5-month follow-up period. Five patients transferred to ventricular septal defect repair operation under direct visualization with a cardiopulmonary bypass. One reason was ventricular fibrillation when guidewire passed the PmVSD, another was device dislocation, and others were the guidewire cannot pass through the PmVSD. PmVSDs closure using a concentric occluder via a left parasternal ultra-minimal trans intercostal incision under TEE guidance is feasible, safe, and effective in children. This approach can be considered as an alternative treatment to open-heart surgery for restrictive PmVSDs.

Echocardiographic Classification of Perimembranous Ventricular Septal Defect Guides Selection of the Occluder Design for Their Transcatheter Device Closure.

BACKGROUNDPerimembranous ventricular septal defects (VSDs) has proximate relation to the aortic and tricuspid valves as well as the conduction tissues. Transcatheter closure utilizes various off-label device designs.METHODSPerimembranous VSD without aortic margin were classified as group A, with thick aortic margin as group B, with membranous septal aneurysm as group C and defects restricted by tricuspid valve attachments as group D. The proposed ideal design was asymmetric device in group A; duct occluder I (ADOI) and muscular ventricular septal occluder (MVSO) in group B; thin profile duct occluder II (ADOII) in group C and ADOI in group D. Device was 0–2 mm larger than the defect.RESULTSEighty patients with VSD measuring 6.83 ± 2.87mm underwent successful closure. Device was retrieved before release in one group A and one group C patient due to aortic regurgitation. Asymmetric device was used in 16 group A defects. Among group B defects, ADOI was used in 5, ADOII in 5, MVSO in one and asymmetric device in 3. Group C defects were closed with ADOI in 7, ADOII in 10 and asymmetric device in 3. Three patients with multiple exits had 2 ADOII devices. Group D defects were closed using ADOI in 20 and ADOII in 10 patients. There was no late aortic regurgitation or heart block on a follow-up exceeding 7 years.CONCLUSIONSThis echocardiographic classification helps device selection in every single patient. While asymmetric device is uniquely suited for group A defects, different designs are appropriate in the other groups.

An interventricular membranous septal aneurysm obstructing the right ventricle outflow tract in a five-year-old boy: a case report

BackgroundWhile the aneurysms of the membranous septum (AVS) are rare, the possibility that they lead to obstruction is even rarer. To the best of our knowledge, 11 similar cases have been reported since 1982.Case presentationInitially, the five-year-old boy was evaluated for dyspnoea that had been present since birth. He did not receive any medical treatment until the previous year. At the age of four, the transthoracic echocardiography showed a large aneurysm extending to the right ventricular outflow tract (RVOT) and causing RVOT stenosis. Complete surgical resection of the aneurysmal tissue was performed, and the boy was discharged home in satisfactory condition.ConclusionsAs the occurrence of RVOT obstruction by a membranous ventricular septal aneurysm is very rare, we are reporting the second case in which an aneurysm of the membranous septum dynamically obstructed the RVOT in a child. We are also reviewing all the previously reported similar cases in the literature. Further studies are needed to obtain a more comprehensive understanding of aneurysms of the membranous septum (AVS).

Right Ventricular Outflow Obstruction by a Membranous Septal Aneurysm in a Ventricular Septal Defect

Right Ventricular Outflow Obstruction by a Membranous Septal Aneurysm in a Ventricular Septal Defect

Dynamic right ventricular outflow tract obstruction caused by alarge interventricular membranous septal aneurysm.

Dynamic right ventricular outflow tract obstruction caused by alarge interventricular membranous septal aneurysm.

A single-center experience with 12 consecutive cases of pregnancy among patients with membranous ventricular septal aneurysm

BackgroundMembranous ventricular septal aneurysm (MVSA) is a rare cardiac anomaly that can occur as an isolated entity or being associated with other cardiac malformations. Complications of MVSA include thromboembolism, arrhythmia, rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and atrioventricular valve diseases.The success rate of pregnancy and delivery in patients with MVSA has not been reported in the literature. This study was to assess the clinical implications of this condition from our center’s experience.MethodsThis was a retrospective study for consecutive 12 pregnancies in women with MVSA, who delivered at a tertiary care center in west China between May 2008 and March 2015.ResultsAll patients with MVSA delivered via caesarian section. One patient with severe pulmonary arterial hypertension expired from pulmonary infection and heart failure after delivery. One patient terminated pregnancy in the second trimester- necessitated by cardiogenic shock. The other mothers had varying degrees of cardiac morbidity, but survived. Ten of thirteen newborns survived. Congenital heart disease and small-for-gestational-age (SGA) of newborn occurred in two cases (one twin and one single gestation). Two of these babies expired.ConclusionsMaternal and neonatal risk appeared associated with heart functional classifications, pulmonary hypertension and histories of cardiac events such as serious cardiac arrhythmia. Accurate diagnosis and care by a multidisciplinary team is recommended for pregnant woman with MVSA.

Bacterial Endocarditis in an Intravenous Drug User with a Ventricular Septal Aneurysm

An aneurysm of the membranous portion of the ventricular septum (AMS) is a common complication of spontaneous ventricular septal defect closure. They have been shown to increase the incidence of cardiac pathology notably bacterial endocarditis. Because of such a severe threat, it may be an important anomaly to assess in high risk patients. A 45-year-old woman with a history of intravenous drug use presented in septic shock with end organ damage requiring intubation, broad spectrum antibiotics, and vasopressors. Physical examination displayed a 2/6 systolic ejection murmur heard loudest at the left lower sternal border. Echocardiography confirmed a mobile tricuspid vegetation; as well as, an aneurysmal deformity. During her initial history the patient stated that her VSD had been diagnosed in childhood. A cardiac catheterization showed a membranous ventricular septal aneurysm protruding from the left ventricle into the right ventricle with no evidence of ventricular septal defect. This case exemplifies a patient predisposed to endocarditis who was at higher risk due to her actions. The objective contribution of the AMS to the resultant endocarditis is unknown at this point. This sentiment raises the question if AMS detection would warrant preventative intervention for associated pathologies.

Interventricular Membranous Septal Aneurysm Incidentally Diagnosed during Computed Tomographic Angiography in a Patient with Infrequent Supraventricular Tachycardia

Interventricular membranous septal (IVMS) aneurysm is a rare condition with no accurate incidence. It is known to be associated with 0.3 % of congenital heart disease and 19 % of ventricular septal defects. IVMS aneurysm is often asymptomatic but can be complicated with right ventricular obstruction, rupture, thromboembolism, and conduction defects. Clinicians and radiologist should be aware about this rare clinical entity and evaluation of any possible underlying cardiac abnormalities should be performed. Conservative management with close follow up is the mainstay of management in the absence of complication.

Diagnosis of the membranous septal aneurysm with multi-slice CT:2 cases report

Diagnosis of the membranous septal aneurysm with multi-slice CT:2 cases report

PP-143 An Incidentally Detected Giant Membranous Interventricular Septal Aneurysm Secondary to Previous Surgery for Subaortic Valvular Stenosis

PP-143 An Incidentally Detected Giant Membranous Interventricular Septal Aneurysm Secondary to Previous Surgery for Subaortic Valvular Stenosis

Septic Pulmonary Emboli and Right Ventricular Obstruction in a Membranous Interventricular Septal Aneurysm

Although the majority of patients with perimembranous ventricular septal defect and septal aneurysm remained asymptomatic, some of them presented with serious complications during adulthood and thus required high risky surgery. In accordance with other rare condition, the incidence and natural history have not been well documented. This case describes the occurrence of a septic pulmonary emboli associated with right ventricular outflow tract obstruction in a young child.

Ventricular Septal Defects in an Adult With Giant Membranous Septal Aneurysm Accompanying Stroke Onset

A 49-year-old woman presented with episodes of transient loss of power in her right leg. Computed tomography (CT) revealed brain stem infarction, and an electrocardiogram showed complete atrioventricular block. Echocardiography revealed membranous septal aneurysms (MSAs) of 42 × 37 mm attached to the left ventricle (Fig 1). Aneurysm covered with calcification identified by CT in the right side of the heart oppressed the inferior vena cava (Fig 2). We speculated that the patient's symptoms were associated with thrombus in the MSA and planned a surgical treatment.

The Windsock Syndrome: Subpulmonic Obstruction by Membranous Ventricular Septal Aneurysm in Congenitally Corrected Transposition of Great Arteries

Anomalies of the membranous portion of the interventricular septum include perimembranous ventricular septal defect and/or membranous septal aneurysm (MSA). In congenitally corrected transposition of the great arteries (L-TGA in sinus solitus), the combination of ventricular inversion and arterial transposition creates a unique anatomic substrate that fosters subpulmonic left ventricular outflow tract obstruction by an MSA. The combination of an L-TGA with subpulmonic obstruction by an MSA is referred to as the windsock syndrome. We report a case of windsock syndrome in a 25-year-old man which is to our knowledge the first three-dimensional echocardiographic description of this congenital entity.

Electro-Anatomical Approach to Membranous Septal Aneurysm: Potential as a Focus of Critical Ventricular Arrhythmias

Membranous septal aneurysm (MSA) is a rare anomaly and known to cause ventricular tachycardia and atrioventricular block. However, underlying mechanisms have not been addressed in its long history. We report first 3-D electro-anatomical mapping of MSA during and three years following the surgery. An elderly patient underwent a surgery for MSA. In the mapping, we located the His bundle near MSA and observed delayed potentials around MSA. Our report showed that electrophysiological character of myocardium was changed around MSA and this change might be a reason for ventricular tachycardia and atrioventricular block. An ordinary surgery for MSA might not resolve this problem since we still observed delayed potentials three years after the surgery.

Membranous Septal Aneurysm: An Unusual Case for Sub-Pulmonary Obstruction in cTGA

cTGA; Membranoz Septal Anevrizma; Pulmoner Stenoz Abstract Aneurysm of the membranous portion of the interventricular septum is an uncommon congenital cardiac malformation that is rarely diagnosed during life. Perimembranous ventricular septal defects are the most common type of the ventricular septal defects and originates from morphologically membranous interventricular septum. Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the tricuspid septal leaflet carries a risk of tricuspid valve insufficiency and conduction disturbances. The current study presents a case with membranous septal aneursym with congenitally corrected transposition of the great arteries and sub-pulmonary obstruction, which was surgically corrected.

Interactive Pathologies of Mitral Prolapse and True Membranous Septal Aneurysm With a Valve-Like Structure

A 70-year-old man was admitted to our hospital for surgical treatment of mitral insufficiency. A preoperative echocardiogram revealed a prolapsed anterior mitral leaflet, membranous septal aneurysm (MSA), and unusual chordae extending from the ventricular septum to the anterior mitral leaflet (Movies I and II in the online-only Data Supplement). In surgery, the aneurysm was examined through the tricuspid valve before the heart was arrested (Figure 1A). On further inspection, when the MSA was pushed with forceps, a large quantity of blood suddenly …

Left ventricle–right atrium communication along with a membranous septum aneurysm

aCardiology Department, Tepecik Training and Research Hospital bCardiology Department, Ege University, Izmir, Turkey Correspondence to Oner Ozdogan, MD, Cardiology Department, Tepecik Training and Research Hospital, 264 S. No. 1/15, 35360 Hatay, Izmir, Turkey Tel: +90 533 3676977; e-mail: [email protected] Received 9 September, 2009 Revised 27 January, 2010 Accepted 20 June, 2012