Membranous Interventricular Septum Research Articles

Predicting new-onset persistent conduction disturbance following transcatheter aortic valve replacement: the usefulness of FEOPS finite element analysis

BackgroundDespite the frequency of persistent new-onset conduction disturbances after transcatheter aortic valve replacement (TAVR), few preoperative methods of prediction exist.MethodsPatients who underwent TAVR in the Department of Cardiology of the Second Affiliated Hospital of the Army Medical University from December 2020 to September 2021 and postoperative aortic root modeling via the FEOPS finite element analysis were included in this single-center case–control study, divided into persistent conduction disturbances (PCD) and non-PCD groups according to their pre- and postoperative electrocardiograms in the first month. Risk factors affecting PCD were identified by comparing the baseline data of these two groups, including echocardiograms, computed tomography angiography of the aortic root, surgical decision-making, and FEOPS data. Independent risk factors were screened using logistic regression modeling, and the receiver operating characteristic (ROC) curve was used to test the predictive ability.ResultsA total of 56 patients were included in this study, 37 with bicuspid aortic valve (BAV) and 19 with trileaflet aortic valve (TAV), with 17 cases of PCD. The contact pressure index (CPI) of FEOPS, valve oversize ratio, differences between membranous interventricular septum length and implantation depth (ΔMSID) and valve implantation depth were statistically different (P < 0.05). CPI could be used as an independent risk factor for PCD (P < 0.05), and the ROC curve comparison showed that the CPI was more predictive (AUC = 0.806, 95% CI: 0.684–0.928, P = 0.001).ConclusionsThe CPI of FEOPS has better predictive value for new-onset conduction disturbance after TAVR compared to other known predictors.

Baseline CT-Based Risk Factors for Atrioventricular Block after Surgical AVR

We aimed to evaluate the impact of membranous interventricular septum (MIS) length and calcifications of the native aortic valve (AV), via preoperative multidetector computed tomography (MDCT) scan, on postoperative atrioventricular block III (AVB/AVB III) and permanent pacemaker implantation in surgical aortic valve replacement (SAVR). We retrospectively analyzed preoperative contrast-enhanced MDCT scans and procedural outcomes of patients affected by AV stenosis who underwent SAVR at our center (June 2016-December 2019). The study population was divided into two groups (AVB and non-AVB), and variables were compared with a Mann-Whitney's U-test or chi-square test. Data were further analyzed using point biserial correlation and logistic regression. A total of 155 (38% female) patients (mean age of 71.2 ± 6 years) were enrolled in our study: conventional stented bioprosthesis (N = 99) and sutureless prosthesis (N = 56) were implanted. A postoperative AVB III was observed in 11 patients (7.1%). AVB patients had significant greater calcifications in left coronary cusp (LCC) -AV (non-AVB = 181.0 mm3 [82.7-316.9] vs. AVB = 424.8 mm3 [115.9-563.2], p = 0.044), LCC left ventricular outflow tract (LVOT) (non-AVB = 2.1 mm3 [0-20.1] vs. AVB = 26.0 mm3 [0.1-138.0], p = 0.048), right coronary cusp (RCC) -LVOT (non-AVB = 0 mm3 [0-3.5] vs. AVB = 2.8 mm3 [0-29.0], p = 0.039), and consequently in total LVOT (non-AVB = 2.1 mm3 [0-20.1] vs. AVB = 26.0 mm3 [0.1-138.0], p = 0.02), while their MIS was significantly shorter than in non-AVB patients (non-AVB = 11.3 mm [9.9-13.4] vs. AVB = 9.44 mm [6.98-10.5]; p=0.014)). Partially, these group differences correlated positively (LCC -AV, r = 0.201, p = 0.012; RCC -LVOT, r = 0.283, p ≤ 0.001) or negatively (MIS length, r = -0.202, p = 0.008) with new-onset AVB III. We recommend including an MDCT in preoperative diagnostic testing for all patients undergoing surgical AVR for further risk stratification.

Abstract 13195: A Case of Ebstein Anomaly Diagnosed Following Tricuspid and Pulmonic Valve Infective Endocarditis in an Adult Patient

Background: We present a rare case of infective endocarditis (IE) involving both tricuspid (TV) and pulmonic valves (PV) in the setting of newly diagnosed Ebstein anomaly (EA) with isolated, small membranous VSD. Case: A 48 yo woman with no known medical history presented with two weeks of fever, flu-like symptoms, and dyspnea. Vitals showed fever, tachycardia, and tachypnea. Poor dentition, grade III systolic murmur, and S3 gallop were noted on physical exam. EKG showed sinus tachycardia and right bundle branch block. Laboratory studies showed leukocytosis and elevated CRP. A chest CT revealed bilateral lung infiltrates in a rounded configuration concerning for septic emboli. Blood cultures grew Streptococcus mitis , and IV antibiotics were initiated. TTE revealed 3.2cm apical displacement of the TV resulting in right ventricular atrialization, diagnostic for EA. TTE also found a 26x17mm vegetation on the septal and anterior leaflets of the TV with moderate tricuspid regurgitation and an 11x6mm vegetation on the PV, confirmed by TEE. Cardiac MRI also noted a focal aneurysm of the membranous interventricular septum with a hemodynamically insignificant shunt. Discussion: While interatrial communications are seen in a majority of EA patients (80-94% of cases), this patient presented solely with a small membranous VSD and absence of ASD and PFO. Moreover, this patient had no PDA to depend on for pulmonary flow. Remarkably, she had an asymptomatic course with EA until the development of endocarditis. It is believed the presence of an isolated VSD in this patient’s severe EA likely promoted forward pulmonic flow in an already inadequate right ventricle. Right-sided IE is itself rare (5-10% of cases) with isolated PV involvement being even rarer (2% of cases). This patient’s uncorrected EA with redundant TV and PV leaflet tissue likely contributed as a nidus for simultaneous infection of both valves.

Anatomical study of the cardiac conduction system in swine hearts.

The cardiac conduction system (CCS) is crucial for regulating heartbeats; therefore, clinicians and comedicals involved in cardiovascular medicine treatment must have a thorough understanding of the CCS structure and function. However, anatomical education of the CCS based on actual dissection and observation is uncommon, although such educational methodology promotes three-dimensional structural understanding of the observed object. Based on previous studies, we examined the CCS structure in the heart of a swine (pig, Sus scrofa domestica) which has been used in the biological, medical and anatomical curricula as science teaching materials, by using macroscopic dissection procedures. Most CCS structures in a young pig heart were successfully identified and illustrated on a macroscopic scale. The atrioventricular bundle (His bundle) was located on the lower edge of the membranous interventricular septum and was clearly distinguished from the general myocardial fibres by its colour and fibre arrangement direction. Following the atrioventricular bundle towards the atrium or ventricle with properly removing the endocardium and myocardium, the atrioventricular node or the right and left bundles appeared respectively. In contrast, the sinoatrial node was not identified. The anatomy of the CCS in young pig hearts was essentially similar to that previously reported in humans and several domestic animals. Our findings of the CCS in young pig hearts are expected to be useful for medical and anatomical education for medical and comedical students, young clinicians and comedical workers.

Permanent pacemaker implantation after transcatheter self-expanding aortic valve replacement: data from a single-center registry

Background. Transcatheter aortic valve replacement is a safe and effective procedure in patients with severe aortic stenosis who are at high surgical risk. One of the most commonly diagnosed complications after transcatheter aortic valve replacement is postoperative complete heart block, requiring in most cases the permanent pacemaker implantation.Aim. The study aims to identify independent prognostic factors of permanent pacemaker implantation after transcatheter aortic valve replacement.Methods. This retrospective study included 336 patients who underwent transcatheter self-expanding aortic valve replacement between 2015 and 2021. 28 patients (8.3%) after procedure required the permanent pacemaker implantation. Patients with a previously implanted permanent pacemaker, surgical aortic valve replacement, transcatheter aortic valve replacement in history and patients after conversion of endovascular intervention to surgical replacement were excluded from the study.Results. The mean age of patients was 75.4 ± 7.4 years. Diabetes mellitus (50.0 versus 28.6%, p = 0.03), baseline right bundle-branch block (32.1 versus 3.9%, p &lt; 0.05), and smaller length of the interventricular membranous septum (5.5 ± 2.2 and 6.9 ± 1.8 mm, p &lt; 0.05) were more common in the permanent pacemaker implantation patient group. The groups were comparable in terms of other clinical, demographic and instrumental characteristics. There were no statistically significant differences between the groups in major procedural complications. Independent prognostic factors of permanent pacemaker implantation were baseline complete right bundle-branch block (OR 21.01, 95% CI 5.86–75.42, p &lt; 0,001), mean transcatheter aortic valve implantation depth below the aortic annulus (OR 1.42, 95% CI 1.16–1.74, p = 0.001) and the length of the interventricular membranous septum (OR 0.7, 95% CI 0.50–0.95, p = 0.02).Conclusion. The frequency permanent pacemaker implantation after transcatheter aortic valve replacement procedure depends on some initial clinical factors, procedural aspects, and postoperative conduction disorders. In this registry, independent prognostic factors of a permanent pacemaker implantation were the baseline complete right bundle-branch block, mean transcatheter aortic valve implantationdepth and the length of the interventricular membranous septum. Received 19 March 2022. Revised 18 April 2022. Accepted 19 April 2022. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest. Contribution of the authorsConception and study design: A.A. Baranov, O.V. Krestyaninov, D.A. Khelimskii, A.G. Badoian, R.U. Ibragimov, A.P. Gorgulko, S.N. Manukian, Sh.Sh. ZaynobidinovData collection and analysis: A.A. Baranov, O.V. Krestyaninov, D.A. Khelimskii, A.G. BadoianStatistical analysis: A.A. Baranov, A.G. Badoian, D.A. KhelimskiiDrafting the article: A.A. Baranov, O.V. Krestyaninov, D.A. Khelimskii, A.G. Badoian, R.U. Ibragimov, A.P. Gorgulko, S.N. Manukian, Sh.Sh. ZaynobidinovCritical revision of the article: A.A. Baranov, O.V. Krestyaninov, D.A. Khelimskii, A.G. Badoian, R.U. Ibragimov, A.P. Gorgulko, S.N. Manukian, Sh.Sh. ZaynobidinovFinal approval of the version to be published: A.A. Baranov, O.V. Krestyaninov, D.A. Khelimskii, A.G. Badoian, R.U. Ibragimov, A.P. Gorgulko, S.N. Manukian, Sh.Sh. Zaynobidinov

Presumed congenital Gerbode defect in an American Domestic Shorthair cat

A five-month-old, intact female Domestic Shorthair cat presented to the Kansas State Veterinary Health Center for evaluation of a murmur and exercise intolerance. Physical exam revealed a grade V/VI right, parasternal, holosystolic murmur. On echocardiogram, there was an abnormality in the membranous interventricular septum and tricuspid valve, allowing blood to shunt from the left ventricle to the right atrium. This lesion is consistent with an infravalvular Gerbode defect. The Gerbode defect is a rare, left ventricle-to-right atrial shunt that can be congenital or acquired, and in cats, has only been reported once. At the time of submission, the patient is alive and is maintaining a good quality of life.

Impact of Interventricular membranous septum length on pacemaker need with different Transcatheter aortic valve implantation systems

BackgroundThe need for new permanent pacemaker implantation (PPI) after Transcatheter Aortic Valve Implantation (TAVI) remains a critical issue. Membranous Septum (MS) length is associated with PPI after TAVI. The aim of this study was to identify different MS thresholds for the contemporary THV-platforms.MethodsThis retrospective, case-control study enrolled all patients who underwent a successful TAVI procedure with contemporary THV-platforms in the Erasmus University Medical Center between January 2016 and March 2020. The follow-up period for new PPI was 30 days. MS-length was determined by Computed Tomography.ResultsThe study consisted 653 TAVI patients with median age 80.6 years (IQR 74.7–84.8). New PPI occurred in 120 patients (18.4%). Patients with new PPI had a shorter MS-length (2.9 mm (IQR 2.3–4.3) vs. 4.2 mm (IQR 2.9–5.7), p < 0.001). MS-length < 3 mm identified a high-risk phenotype with 30.3% PPI-rate (OR 6.5 [95%CI 2.9–14.9]), MS-length 3–6 mm an intermediate-risk phenotype with 15.4% PPI-rate (OR 2.7 [95%CI 1.2–6.2]) and MS > 6 mm a low-risk phenotype with a 6.3% PPI-rate (reference). For the Lotus valve, there was no significant difference in PPI-rates between the high-risk (45.8%, OR 3.5 [95%CI 0.8–15.1]) and low-risk group (20%).By multivariate analysis MS-length, Agatston-score, use of Lotus valve, and ECG with first-degree AV block, RBBB or bifascular block were independent predictors for new PPI.ConclusionMS-length was an independent predictor for new PPI post-TAVI. Three phenotypes were found based on MS-length. MS < 3 mm was universally associated with a high risk for new PPI (>30%). MS > 6 mm represented a low-risk phenotype with PPI-rate < 10%. PPI-rate varied per THV type in the intermediate phenotype. PPI-rate with Lotus was high regardless of MS-length.

Aneurysmal and obstructive lesions of the left ventricular outflow: evaluation on multidetector computed tomography angiography

The left ventricular outflow is an anatomically complex region situated between the anterior leaflet of the mitral valve and the left ventricular aspect of the muscular and membranous interventricular septum. It gives rise to the aorta, provides support to the aortic valvular cusps, and houses important components of the conduction system. The left ventricular outflow handles high pressures and pressure variations and is subsequently affected by a variety of aetio-pathological conditions. Diseases involving the left ventricular outflow can be intraluminal, mural, or extramural, and the consequent complications of the lesions can be local, loco-regional, or even systemic. Appropriate evaluation requires comprehensive multimodality imaging with each modality contributing to assessment of different aspects of diagnosis, lesion characterization, local extension, prognostication for systemic complications and mortality, and the decision for the approach and type of intervention and aggressive follow-up in case non-interventional management is decided. In this review, we briefly describe the relevant anatomy and the gamut of structural abnormalities pertaining to the left ventricular outflow on multidetector computed tomography angiography.

Pseudoaneurysm of interventricular membranous septum: a rare late complication after repeated valve replacements

A 39-year-old female patient who had aortic and mitral valve replacement due to rheumatic heart disease was also found to have cystic mass in the right ventricle on echocardiography during late routine control. The cystic mass identified on echocardiography was shown to be a pseudoaneurysm that develops from the membranous section of the interventricular septum and grows toward the right heart by ECG-triggered cardiac computed tomography performed for differential diagnosis. Because the patient did not accept the surgery, regular follow-ups were recommended.

P2275Left-sided deviation and fibrous-fatty infiltration of the right bundle branch in the elderly: implication for transcatheter aortic valve implantation (TAVI) procedure

Abstract Introduction Permanent and irreversible damage to the conduction tissue is one of the most common complications of TAVI. Detailed knowledge of the anatomy of the atrioventricular (AV) bundle is crucial to minimize the potential for injury to the His bundle branch block or complete AV block. Purpose Preexisting anatomic location and damage of the right bundle branch (RBB) may have important unrecognized clinical implications. Methods The myocardial arrangement and the presence of fibrous and fatty tissue infiltration of the AV conduction axis and right bundle branch were examined by dissection techniques and histological sections in 57 structurally normal human heart specimens (48 males, 77±7 years). Results The AV conduction axis enters the AV component of the membranous septum and is encircled by the fibrous tissue of the central fibrous body. The AV bundle is divided in a non-branching portion and a branching portion. After a short distance of the non-branching component along the septal crest, the RBB arises at the end of the branching portion of the conduction axis and is located superficially in the muscular ventricular septum. The RBB takes off from the bundle at the level of origin of the superior fascicle of the left bundle, passing then through the thickness of the ventricular septum to emerge beneath the medial papillary muscle of the tricuspid valve. In 22 hearts (49%) in which we found to have a relatively left-sided deviation of the AV bundle in relation to the interventricular membranous septum the RBB runs intramyocardial along the muscular interventricular septum. In the remaining 51% of the hearts the RBB runs subendocardially in the crest of the interventricular muscular septum. We found connective tissue and fatty infiltration along the right bundle branch in 23 hearts (40%) from its origin to its distal part. A significant correlation was found between age and the presence of RBB fibrosis (85% of hearts from individuals with age &gt;80 years). These 2 conditions may make the RBB very vulnerable to self-expanding aortic valves during or after TAVI (figure). RBB and its critical region Conclusions The presence of an intramyocardial location of the RBB with a lef-sided deviation and the fibro-fatty infiltration found in the mayority of senescent hearts are relevant anatomic determinants that may increase the risk of complete AV block following a TAVI procedure.

P6015Histological pattern of penetrating His bundle division: implications for atrioventricular conduction damage during and following transcatheter aortic valve implantation

Abstract Background Severe damage to the atrioventricular conduction system is one of the most common complications of transcatheter aortic valve implantation (TAVI) and can be linked to important comorbidity, increased healthcare expense, need for long-term monitoring and pacemaker implantation. Purpose To provide a detailed description of the His bundle (HB) arrangement within the left ventricular outflow tract. Methods We examined by dissection techniques and histological sections the course of the AV conduction axis (penetrating, non-branching and branching HB) in relation with the membranous and muscular interventricular septum in 57 structurally normal human heart specimens (48 males, 77±7 years) Results The AV conduction axis is located along the inferior edge of the membranous septum (MS). The MS is divided into AV and interventricular components and is located at the base of the interleaflet triangle between the right and non-coronary leaflets of the aortic valve. The conduction axis enters the AV component of the MS and is encircled by the fibrous tissue of the central fibrous body to reach the left ventricular outflow tract. The MS showed in cadaveric hearts variable dimensions in length (4.6±1.5 mm) with a range bewteen 1 to 9mm. In 17.5% of specimens the MS length was ≤2 mm. After penetrating the AV membranous septum it has a non-branching component that in 85.5% of cases runs only for a short distance (1–3 mm) along the septal crest before giving rise to the fascicles of the left bundle (LB) on the septal surface (Type A). The most anterior fibers of the LB originate at the end of the branching portion located underneath the inferior edge of the MS. In 5 hearts (9%) the HB division was found before it reached the interventricular MS (Type B) and in 3 cases (5.5%)2–3.5 mm distal to the crest (Type C). 22 hearts (49%) were shown to have a relatively left-sided deviation of the AV bundle with the anterior part of the bundle closely related to the nadir of attachment of the right coronary leaflet of the aortic valve. In the remaining 51% of hearts, the bundle coursed centrally or with a right-sided deviation. The HB measures were (mm, mean±SD (range)): length: 3±0.6 (2–4.5), width: 3.7±1.4 (2.5–5.6), thickness: 1.4±0.5 (0.5–2.2), HB to endocardium: 0.7±0.3 (0.2–1.5). His bundle position types Conclusions A shorter membranous septum length, the variable HB dimensions and the left-sided deviation of the AV conduction axis are extremely relevant anatomic features that are linked to the frequent injury to the HB branch or complete AV block following TAVI.

Cardiac T2* magnetic resonance analysis of membranous interventricular septum in assessment of cardiac iron overload in pediatric thalassemia patients: A pilot study.

Background:Cardiac iron deposition in transfusion-dependent thalassemia patients is patchy in distribution.Purpose:The purpose of this study is to assess the correlation between T2* matrices of membranous interventricular septum (MIVS) and T2* values of muscular interventricular septum (IVS) on magnetic resonance imaging (MRI) and to evaluate the relationship of myocardial T2* at these two locations with MRI-estimated liver iron concentrations (LIC) and electrocardiographic (ECG) parameters.Material and Methods:MRI of heart and liver was performed in 16 consecutive pediatric patients of transfusion-dependent thalassemia major to calculate liver iron concentration and T2* time of membranous and muscular IVS. ECG parameters of these patients were charted and correlated with MRI parameters.Results:No significant correlation between T2* values of muscular IVS and MIVS was observed. Mean T2* of MIVS (9.8 ms) was significantly lower than that of muscular IVS (26.9 ms). T2* of MIVS correlated strongly with LIC where as a weak correlation was observed between T2* of IVS and LIC. Significantly higher mean QTc (corrected QT interval) value (439.86 ms) was seen in patients with T2* IVS <20 ms.Conclusion:Addition of T2* analysis of MIVS to the existing MRI protocol, consisting of muscular IVS analysis, may offer a more sensitive estimation of cardiac iron overload.

Abstract 575: Primary Cilia of Cardiac Neural Crest Cells Orchestrate Multiple Aspects of Cardiovascular Development

Cardiac neural crest cells (CNCC) are a specialized population of migratory neural crest cells that contribute to the developing cardiac outflow tract (OFT) and endocardial cushions (ECC). Like many eukaryotic cells, CNCC also possess primary cilia, which function as membrane-bound sensory organelles involved in intra- and extra-cellular communications in developing tissues. Previous work in our laboratory has shown that primary cilia of cardiac progenitor populations are critical orchestrators of both the septation and alignment of the developing OFT and interventricular septum. Our current in vivo models have refined the elimination of primary cilia to CNCC of the embryonic mouse heart using multiple neural crest-specific Cre drivers ( Wnt1 :Cre-Danielian et al,1998; Wnt1 :Cre2-Soriano et al, 2013) crossed with the Ift88 flox/flox mouse (Haycraft et al, 2007), resulting in elimination of primary cilia from CNCC during early embryonic heart development. Additionally, we have utilized a Td-Tomato (Tdt) reporter mouse line to track the migration of CNCC during various embryonic timepoints. Use of this reporter line revealed transient Tdt-positive CNCC contributions to the ventricular portion of the developing myocardium, in addition to the expected CNCC contributions to in the OFT and ECC regions. The resultant mutant phenotypes included a high incidence of outflow tract defects, impaired septation of the membranous interventricular septum, non-compaction of the developing ventricular myocardium, and perinatal lethality. Our data combine various immunohistochemical approaches to document, characterize, and quantify the novel presence of CNCC in the developing embryonic ventricular myocardium. These data suggest a potential modulatory role for primary cilia of CNCC in the development and maturation of the ventricular myocardium. Primary cilia-mediated signal transduction events that could account for the descried phenotypes are currently being elucidated.

Electrophysiological Consequences of Congenital Heart Defects Shaped by Primary Cilia‐Dependent Cardiac Developmental Processes

Congenital heart defects (CHD) affect approximately 8 out of every 1,000 newborns, resulting in the birth of 35,000 babies with CHD annually in the United States. Primary cilia are small, finger‐like structures that extend from almost all eukaryotic cells and have been found to play a pivotal role in both development and maturation of embryonic and perinatal heart. Our laboratory presents a novel cardiac phenotype resulting from the elimination of primary cilia from cardiac neural crest cells (cNCC) of the embryonic mouse heart. This phenotype combines a high incidence of outflow tract defects, including double‐outlet right ventricle, with severe damage to the membranous interventricular septum, non‐compaction of the developing ventricular myocardium, and perinatal lethality. The purpose of this ongoing project is to develop a reliable method for obtaining electrocardiograms (ECG) in perinatal mice, to perform a comprehensive and diagnostic analysis of the combined anatomical and physiological data and to better understand the perinatal lethality resulting from primary cilia elimination. A Cre recombinase‐expressing mouse (Wnt1:Cre) was crossed with the Ift88flox/flox mouse, resulting in elimination of primary cilia from cNCC during mid embryonic heart development. Extensive histological and imaging analyses were previously performed to characterize and reconstruct the anatomical abnormalities. Perinatal electrocardiography (ECG) was then performed on 34 pups within the first day of life (P1), where mice remained conscious in the prone position. Modified gel‐coated ECG electrodes were placed in the right axilla of the left lower quadrant of the abdomen, allowing for a two‐lead electrophysiological evaluation. ECGs were collected for a total of 5 minutes, after which, pups were euthanized for further histological evaluation. ECG analysis included heart rate (HR; minimum, maximum and mean), QRS duration, P wave presence, PR interval, and overall ECG interpretation. Mutant mice (n=26) demonstrated a significantly lower HR when compared to littermate wildtype (WT) (n=8) mice (233±24 beats per minute (bpm) vs 30±2 bpm, p&lt;0.0001). QRS duration was significantly lengthened in the mutant mice compared with WT (0.020±0.001 milliseconds (ms) vs 0.014±0.001 ms, p=0.018). P wave activity was absent in 54% of mutant mice when compared to 100% appearance in WT mice. Combined incidence of bradycardia, HR irregularity and the absence of P wave activity seen in the mutant mice suggests that the QRS complexes demonstrated were not sinus in nature, rather of a junctional or ventricular origin. Whether this is due to a defect in SA node development, communication between the atria and ventricles, or hypoxia is currently under investigation. Increased QRS duration seen in mutant mice is suggestive of additional depolarization abnormalities. Collectively, these results characterize a relationship between the anatomical phenotype resulting from primary cilia elimination and abnormal embryological development of cardiac electrical conduction pathways.Support or Funding InformationGenerous support for this project was made possible by the Saving tiny Hearts Foundation and the Peter Morgane Fellowship Program at the University of New England.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

A case of ST-segment elevation acute myocardial infarction with an aneurysm of the interventricular membranous septum and a hypoplastic left atrial appendage

A case of ST-segment elevation acute myocardial infarction with an aneurysm of the interventricular membranous septum and a hypoplastic left atrial appendage

Clinical Cases: Valvular heart disease142A sub-aortic valve mass in a rheumatoid arthritis patient: an unconventional mechanism of aortic regurgitation143Symptomatic severe aortic regurgitation with coronary obstruction due to chronic type a dissection144Mitral valve prolapse and ventricular tachycardia: a long-lasting love story

Clinical Cases: Valvular heart disease142A sub-aortic valve mass in a rheumatoid arthritis patient: an unconventional mechanism of aortic regurgitation143Symptomatic severe aortic regurgitation with coronary obstruction due to chronic type a dissection144Mitral valve prolapse and ventricular tachycardia: a long-lasting love story

Invited Commentary

In this issue of The Annals, Wang and colleagues [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] reported follow-up data of a large cohort of consecutively treated patients who underwent isolated aortic valve replacement (AVR) surgery for bicuspid aortic valve (BAV) disease. The authors should be congratulated for their efforts to determine the fate of proximal aortopathy in BAV patients stratified on the pattern of aortic valve dysfunction, namely, BAV–aortic stenosis versus BAV–aortic insufficiency (AI). Indeed, there is an emerging evidence of the coexistence of distinct clinical entities of BAV disease, namely, those presenting with predominant aortic valve stenosis (ie, BAV stenosis phenotype) and those presenting with predominant aortic insufficiency and dilation of the aortic root (ie, BAV root phenotype). Given the evident paucity of homogeneous phenotype-specific reporting of outcomes in BAV disease, the current report [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] is of great clinical value and should be a good example for future BAV studies. The major focus of this paper is on a cohort of BAV patients presenting with aortic valve insufficiency and aortic root involvement (ie, root phenotype). Wang and colleagues [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] found a significantly increased ascending aortic growth rate and higher prevalence of aortic events in BAV-AI patients, which is in line with our previous data [2Girdauskas E. Disha K. Rouman M. Espinoza A. Borger M.A. Kuntze T. Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: echocardiographic follow-up study.Eur J Cardiothorac Surg. 2015; 48: e71-e76Crossref PubMed Scopus (41) Google Scholar, 3Girdauskas E. Rouman M. Disha K. et al.Aortic dissection after previous aortic valve replacement for bicuspid aortic valve disease.J Am Coll Cardiol. 2015; 66: 1409-1411Crossref PubMed Scopus (47) Google Scholar] and therefore indicate the genetic origin of BAV-AI associated aortopathy. In retrospect, these data show convincingly that the historically reported increased risk of aortic events in BAV disease is mainly due to the mixing of such root phenotype patients in the analyzed heterogeneous BAV cohorts. The congenital nature of aortopathy in root phenotype patients is further supported by common phenotypic features (ie, young male BAV patients) and involvement of adjacent cardiac structures—marked enlargement of aortoventricular junction, involvement of anterior mitral leaflet (ie, WAMBIRE complex [4Sievers H.H. Invited commentary.Ann Thorac Surg. 2009; 87: 82Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar]), defects/aneurysms of membranous interventricular septum, and coexistent coronary anomalies. Similar to other well-known connective tissue disorders, several noncardiac manifestations (eg, skeletal deformities, recurrent groin hernia, and cerebral arterial aneurysms) have been previously reported in BAV insufficiency cohorts. Prospective trials aiming to clarify the underlying gene expression and biomarker patterns in BAV root phenotype patients are under way, and their results are awaited eagerly. The proof of congenital background of BAV-AI associated aortopathy will undoubtedly change our current understanding of BAV disease complex and make this distinct entity to become a firm part in the novel BAV nomenclature. Impact of Aortic Insufficiency on Ascending Aortic Dilatation and Adverse Aortic Events After Isolated Aortic Valve Replacement in Patients With a Bicuspid Aortic ValveThe Annals of Thoracic SurgeryVol. 101Issue 5PreviewAberrant flow pattern and congenital fragility bestows bicuspid aortic valve (BAV) with a propensity toward ascending aorta dilatation, aneurysm, and dissection. Whether isolated aortic valve replacement (AVR) can prevent further dilatation in BAV ascending aorta and what indicates concurrent aortic intervention in the case of valve operation remain controversial. Full-Text PDF

Interventricular membranous septal aneurysm: CT and MR manifestations.

Advanced cardiac imaging is a valuable method to investigate cardiac malformations. The detection of the interventricular membranous septum has clinical significance due to thrombogenic and arrythmogenic predisposition, as well as a role in obstructing the pulmonary flow. This review describes six clinical presentations in which advanced cardiac imaging has been the tool for evaluation, with special emphasis in CT angiography and cardiac MRI sequences.Teaching Points• The interventricular membranous septum can predispose patients to thrombogenic and arrythmogenic events.• Subpulmonic stenosis relates to the protrusion of the aneurysm into the right ventricle• During surgery, ventricular pressures of the opened heart become balanced, making the aneurysm less evident.

Massive Interventricular Septal Aneurysm and Stroke in a Healthy Young Patient: Guilt by Association?

Aneurysm of the membranous interventricular septum is an uncommon cardiac defect that is, on rare occasions, associated with embolic stroke. We describe here the case of an otherwise healthy, 41-year-old man who presented to the hospital with acute-onset confusion and left-sided body weakness attributed to a right middle cerebral artery ischemic stroke. He experienced a nearly complete resolution of deficits following systemic thrombolytic therapy. After an extensive workup, the presumed mechanism of stroke was a thromboembolus that originated in a massive aneurysm of the patient's membranous interventricular septum. Due to a perceived risk of surgical morbidity, the patient was managed conservatively with anticoagulation. He denied further events and reported nearly full function at follow-up.

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.