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Background The combination of symptomatic aortic stenosis (AS) and subaortic membrane (SAM) is rare, and the haemodynamic consequences of this combination are challenging to diagnose and manage. Case summary We present the case of a 78-year-old male who presented with symptomatic AS and chronic heart failure. On echocardiography, the combination of AS and SAM was diagnosed. Transoesophageal echocardiography (TOE) was performed to confirm the presence and severity of AS and SAM. Based on the patient's clinical profile and the high risk of surgical repair, a self-expandable transcatheter aortic valve implantation (TAVI) was performed. Three-month follow-up was uneventful. In addition, we discuss the assessment and management strategy challenges for SAM in patients with aortic regurgitation or AS. Discussion As transcatheter valve implantation expands its indications for more complex anatomy and patient's comorbidity. Self-expandable TAVI-prosthesis under TOE guidance is feasible for the treatment of symptomatic AS and SAM, especially in patients with high surgical risk. [ABSTRACT FROM AUTHOR]

Background Subaortic stenosis (SAS) is characterized by a fibromuscular membrane located just below the aortic valve, causing fixed outflow tract obstruction. There is a paucity of studies evaluating this condition. This cohort study reviewed the contemporary characteristics and outcomes of SAS in adult patients in a single large referral center. Methods and Results We retrospectively studied adult patients with SAS evaluated at our center during 2011 to 2022. The primary outcome was all‐cause mortality and heart failure hospitalizations during follow‐up, with secondary end points including recurrence of SAS and repeat surgery after initial SAS surgery. Among 484 patients with SAS, key characteristics included mean age 55±18 years, 67.5% female, left ventricular outflow tract peak velocity 352±140 cm/s and gradient 57±40 mm Hg, left ventricular ejection fraction 60%±14%, 54.8% had prior SAS surgery, and 45.1% had surgery during follow‐up. Over a median follow‐up of 5.5 (1.5–12.3) years, 11.5% (n=56) died, 6.8% (n=33) had heart failure hospitalizations, 8.0% (n=39) experienced SAS recurrence, and 14 (5.9%) underwent repeat SAS surgery. Multivariable analyses identified older age per 10‐years (hazard ratio [HR], 1.37 [95% CI, 1.12–1.68]) and baseline New York Heart Association class (HR, 2.48 [95% CI, 1.54–3.99]) to be statistically significantly associated with the primary end point; higher body mass index, New York Heart Association class, and peak left ventricular outflow tract gradient were also statistically significantly associated with SAS recurrence and redo surgery. Conclusions Almost half of patients with SAS had surgery in the past or during follow‐up, and a significant minority had mortality or morbidity events during follow‐up. Identified prognosticators warrant further research to guide management.

Abstract Background Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle into a low pressure superior (distal) chamber and a high pressure inferior (proximal) chamber. It is found in association with a ventricular septal defect in 90% cases with other associations being tetralogy of Fallot, transposition of great vessels, atrial septal defect and Ebstein’s anomaly. On the other hand, subaortic membrane is a form of discrete subaortic stenosis that is characterised by a membranous diaphragm in the subvalvular location of the left ventricular outflow tract. Both of these entities are responsible for causing subvalvular outflow tract obstruction. The occurrence of double chambered right ventricle in association with subaortic membrane is an extremely rare entity with only a few case reports available in the literature. Case report A 13-year-old male child with history of chest pain and palpitations presented to the outpatient department of a tertiary care center. Transthoracic echocardiography revealed a subaortic membrane producing a pressure gradient across the left ventricular outflow tract with dilatation of the right atrium and right ventricle which could not be fully evaluated on echocardiography. Cardiac computed tomography was then performed which additionally revealed an anomalous muscle bundle coursing across the right ventricle from the septum to the subinfundibular region creating a double chambered right ventricle. The patient was then taken up for reconstruction of right ventricular outflow tract and resection of subaortic membrane. Conclusion Right and left outflow tract obstructions are rare congenital lesions which when seen in combination, become even more infrequent. Echocardiography is a robust tool that detects turbulent flow to identify such lesions. However, poor acoustic window may sometimes result in missing these lesions and computed tomography in such situations can play an important role in detection as well as complete preoperative imaging evaluation.

Background: Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle into a low pressure superior (distal) chamber and a high pressure inferior (proximal) chamber. It is found in association with a ventricular septal defect in 90% cases with other associations being tetralogy of Fallot, transposition of great vessels, atrial septal defect and Ebstein's anomaly. On the other hand, subaortic membrane is a form of discrete subaortic stenosis that is characterised by a membranous diaphragm in the subvalvular location of the left ventricular outflow tract. Both of these entities are responsible for causing subvalvular outflow tract obstruction. The occurrence of double chambered right ventricle in association with subaortic membrane is an extremely rare entity with only a few case reports available in the literature. Case report: A 13-year-old male child with history of chest pain and palpitations presented to the outpatient department of a tertiary care center. Transthoracic echocardiography revealed a subaortic membrane producing a pressure gradient across the left ventricular outflow tract with dilatation of the right atrium and right ventricle which could not be fully evaluated on echocardiography. Cardiac computed tomography was then performed which additionally revealed an anomalous muscle bundle coursing across the right ventricle from the septum to the subinfundibular region creating a double chambered right ventricle. The patient was then taken up for reconstruction of right ventricular outflow tract and resection of subaortic membrane. Conclusion: Right and left outflow tract obstructions are rare congenital lesions which when seen in combination, become even more infrequent. Echocardiography is a robust tool that detects turbulent flow to identify such lesions. However, poor acoustic window may sometimes result in missing these lesions and computed tomography in such situations can play an important role in detection as well as complete preoperative imaging evaluation. [ABSTRACT FROM AUTHOR]

Weill–Marchesani syndrome (WMS) is a rare genetic inherited disorder with autosomal recessive and dominant modes of inheritance. WMS is characterized by the association of short stature, brachydactyly, joint stiffness, eye anomalies, including microspherophakia and ectopia of the lenses, and, occasionally, heart defects. We investigated the genetic cause of a unique and novel presentation of heart-developed membranes in the supra-pulmonic, supramitral, and subaortic areas, creating stenosis that recurred after their surgical resection in four patients from one extended consanguineous family. The patients also presented ocular findings consistent with Weill–Marchesani syndrome (WMS). We used whole exome sequencing (WES) to identify the causative mutation and report it as a homozygous nucleotide change c. 232T>C causing p. Tyr78His in ADAMTS10. ADAMTS10 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif 10) is a member of a family of zinc-dependent extracellular matrix protease family. This is the first report of a mutation in the pro-domain of ADAMTS10. The novel variation replaces a highly evolutionary conserved tyrosine with histidine. This change may affect the secretion or function of ADAMTS10 in the extracellular matrix. The compromise in protease activity may thus cause the unique presentation of the developed membranes in the heart and their recurrence after surgery. [ABSTRACT FROM AUTHOR]

A 35-year-old pregnant female in her second trimester presented with heart failure manifestations with evidence of very severe fixed left ventricular outflow tract obstruction. The peak systolic gradient was 132 mmHg, which is the highest reported in the literature, secondary to congenital subaortic membrane. Similar case reports that we could find in the literature were reviewed to highlight the importance of such anomaly.

Abstract Background Subaortic obstruction by a membrane or systolic anterior motion of the mitral valve leaflets is usually suspected in young patients, especially if the anatomy of the aortic valve is not clearly stenotic and unexplained left ventricular hypertrophy exists in the context of high transaortic gradients. Main body In certain circumstances, some patients show both aortic and subaortic stenotic lesions of variable severity. Doppler echocardiography can help in grading severity in the case of single-level obstruction but not in patients with multilevel obstruction where the continuity equation is of no value. Three-dimensional (3D) echocardiography allows "en-face" visualization of each level of the aortic valve and subaortic tract; in addition, direct planimetry of the areas can be done using multiplanar reformatting. Conclusions Accordingly, 3D echocardiography plays a crucial role in the assessment in patients with multilevel left ventricular outflow tract obstruction as it can accurately delineate the location and size, and severity of the stenosis.

A 35‑year‑old pregnant female in her second trimester presented with heart failure manifestations with evidence of very severe fixed left ventricular outflow tract obstruction. The peak systolic gradient was 132 mmHg, which is the highest reported in the literature, secondary to congenital subaortic membrane. Similar case reports that we could find in the literature were reviewed to highlight the importance of such anomaly. [ABSTRACT FROM AUTHOR]

Ventricular septal defects (VSD) are the most common congenital cardiac defect. Patients with large VSDs present early due to an increase in the volume load on the left ventricle and pressure load on the right ventricle. Few of them present late even without surgical intervention, due to partial restriction of perimembranous (PM) VSD, either by the septal leaflet of the tricuspid valve or by aortic valve cusp prolapse into the VSD. The authors observed a novel structure (ie, subaortic membrane in this case) restricting the large PM VSD in a 15-year-old child. [ABSTRACT FROM AUTHOR]

Background: Subaortic obstruction by a membrane or systolic anterior motion of the mitral valve leaflets is usually suspected in young patients, especially if the anatomy of the aortic valve is not clearly stenotic and unexplained left ventricular hypertrophy exists in the context of high transaortic gradients. Main body: In certain circumstances, some patients show both aortic and subaortic stenotic lesions of variable severity. Doppler echocardiography can help in grading severity in the case of single-level obstruction but not in patients with multilevel obstruction where the continuity equation is of no value. Three-dimensional (3D) echocardiography allows "en-face" visualization of each level of the aortic valve and subaortic tract; in addition, direct planimetry of the areas can be done using multiplanar reformatting. Conclusions: Accordingly, 3D echocardiography plays a crucial role in the assessment in patients with multilevel left ventricular outflow tract obstruction as it can accurately delineate the location and size, and severity of the stenosis. [ABSTRACT FROM AUTHOR]

Background Patients with hereditary hemorrhagic telangiectasia have liver vascular malformations that can cause high‐output cardiac failure (HOCF). Known sequelae include pulmonary hypertension, tricuspid regurgitation, and atrial fibrillation. Methods and Results The objectives of this study were to describe the clinical, echocardiographic, and hemodynamic characteristics and prognosis of hereditary hemorrhagic telangiectasia patients with HOCF who were found to have a subaortic membrane (SAoM). A retrospective observational analysis comparing patients with and without SAoM was performed. Among a cohort of patients with HOCF, 9 were found to have a SAoM in the left ventricular outflow tract by echocardiography (all female, mean age 64.8±4.0 years). The SAoM was discrete and located in the left ventricular outflow tract 1.1±0.1 cm below the aortic annular plane. It caused turbulent flow, mild obstruction (peak velocity 2.8±0.2 m/s, peak gradient 32±4 mm Hg), and no more than mild aortic insufficiency. Patients with SAoM (n=9) had higher cardiac output (12.1±1.3 versus 9.3±0.7 L/min, P=0.04) and mean pulmonary artery pressures (36±3 versus 28±2 mm Hg, P=0.03) compared with those without SAoM (n=19) during right heart catheterization. Genetic analysis revealed activin receptor‐like kinase 1 mutations in each of the 8 patients with SAoM who had available test results. The presence of a SAoM was associated with a trend towards higher 5‐year mortality during follow‐up. Conclusions SAoM with mild obstruction occurs in patients with hereditary hemorrhagic telangiectasia and HOCF. SAoM was associated with features of more advanced HOCF and poor outcomes.

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

Background: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. Materials and Methods: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. Results: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age

Recently, ADAMTS19 was identified as a novel causative gene for autosomal recessive heart valve disease (HVD), affecting mainly the aortic and pulmonary valves. Exome sequencing and data repository (CentoMD) analyses were performed to identify patients with ADAMTS19 variants (two families). A third family was recognized based on cardiac phenotypic similarities and SNP array homozygosity. Three novel loss of function (LoF) variants were identified in six patients from three families. Clinically, all patients presented anomalies of the aortic/pulmonary valves, which included thickening of valve leaflets, stenosis and insufficiency. Three patients had (recurrent) subaortic membrane, suggesting that ADAMTS19 is the first gene identified related to discrete subaortic stenosis. One case presented a bi‐commissural pulmonary valve. All patients displayed some degree of atrioventricular valve insufficiency. Other cardiac anomalies included atrial/ventricular septal defects, persistent ductus arteriosus, and mild dilated ascending aorta. Our findings confirm that biallelic LoF variants in ADAMTS19 are causative of a specific and recognizable cardiac phenotype. We recommend considering ADAMTS19 genetic testing in all patients with multiple semilunar valve abnormalities, particularly in the presence of subaortic membrane. ADAMTS19 screening in patients with semilunar valve abnormalities is needed to estimate the frequency of the HVD related phenotype, which might be not so rare. [ABSTRACT FROM AUTHOR]

Background: The combination of subaortic membrane (SAM) and patent ductus arteriosus is very rare. Subaortic stenosis is the second most common form of left ventricular outflow tract (LVOT) obstruction after valvular aortic stenosis. We are reporting the largest case series of SAM and PDA. Methods: We included all patients that were diagnosed with the combination of SAM and PDA at our cardiac center. We have reviewed patients echocardiographic studies, cardiac catheterizations, surgical notes and all the outpatients notes. Results: We have a total of 7 patients. The age at presentation was in the early childhood with 3 patients diagnosed in infancy. Four patients had severe and moderate LVOT obstruction with SAM being very close to the aortic valve and all required surgical intervention. The last three patients had mild LVOT obstruction 2 of them with the SAM being > 4mm away from the aortic valve. Six out of the seven patients had intervention while the last one is under clinical follow up currently. PDA closure did not change the outcome. There were no other postoperative complication like developing new AI or developing complete heart block. There was no relation between gender, height, weight or age at diagnosis to the SAM clinical course. Conclusion: SAM and PDA association is very rare. The underlying pathophysiology is not well understood. When the SAM is closer to aortic valve (≤ 4mm), it carries higher risk of progressive LVOT obstruction. The interventions for SAM and PDA were safe procedures. [ABSTRACT FROM AUTHOR]

A case of 30 years' male, a farmer by occupation who was a diagnosed case of a ventricular septal defect (VSD) since the age of seven, but refused for surgical closure back then, came in the emergency room (ER) with decompensated congestive cardiac failure and peripheral edema. After initial stabilization, he underwent transthoracic echocardiography (TTE) that showed small perimembranous VSD with the left to right shunt, a subaortic membrane with severe left ventricular outflow tract (LVOT) obstruction and moderate aortic and mitral regurgitation. TTE was followed by Computed Tomography (CT) thoracic aortogram that revealed small VSD with significant aortic regurgitation (AR). Subaortic membrane, aneurysmal dilated aortic root and ascending aorta and aneurysmal dilated pulmonary arteries. Cardiac surgeons were taken onboard and advised for VSD closure with aortic valve replacement, subaortic membrane resection and aortic root replacement. Patient and family were counseled regarding the procedure but refused again for surgical closure. [ABSTRACT FROM AUTHOR]

Background: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. Materials and Methods: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. Results: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. Conclusions: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life. [ABSTRACT FROM AUTHOR]

Left ventricular outflow tract obstruction is a serious complication of mitral valve surgery (repair and replacement) and transcatheter mitral valve replacement. An appreciation of the various mechanisms which cause outflow obstruction in these settings is critical to avoiding this complication and to initiating appropriate treatment. This article discusses the mechanisms, pathophysiology, and imaging of left ventricular outflow tract obstruction which can arise following insertion of a variety of mitral valve prosthetics. [ABSTRACT FROM AUTHOR]

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed. [ABSTRACT FROM AUTHOR]

Aims - Fixed subaortic stenosis (SAS) can be classifi ed into a complex form when it is associated with other defects involving the left ventricular (LV) infl ow and outfl ow tract and a simple form when this is not the case. Previous studies have suggested that these two forms have different outcomes and might represent different pathologies altogether. Our purpose was to study the characteristics and outcomes of operated and unoperated complex SAS and simple SAS patients in a single tertiary centre of general cardiology. Methods - A total of 93 consecutive patients were retrospectively identifi ed between 2003 and 2016. Propensity score matching was used to obtain comparable subgroups of complex SAS (n=24) and simple SAS (n=20) in terms of age (mean 26 years), gender (52% female), functional class (class III 9%, class II 75%) and LV ejection fraction (mean 61%) at diagnosis. Results - LV diastolic diameter (51 vs. 47 mm, p=0.08) and interventricular septum thickness (12 vs. 10 mm, p=0.08) seemed to have higher values in the complex SAS group, despite a similar mean aortic gradient in the two subgroups (40 vs. 45 mmHg, p=0.23). There was no signifi cant difference regarding LV ejection fraction (60% vs. 61%, p=0.4). There were more patients with moderate to severe mitral regurgitation in the complex SAS group (25% vs. 10%), whereas moderate to severe aortic regurgitation was equally noticed in both groups (54% vs. 55%). There were more patients who received surgical treatment (54% vs. 25%, p=0.05), with a higher usage of aortic valve replacement in the complex SAS group (38% vs. 0%, p=0.15). Mortality seemed higher in the complex SAS group when compared to simple SAS group (16% vs. 10%, p=0.4), both in operated (15% vs. 0%, p=0.5) and unoperated patients (18% vs. 13%, p=0.6), without reaching the statistical signifi cance due to small sample size. Mean subaortic gradient correlated signifi cantly with left ventricular end-diastolic diameter (negative correlation, R2=0.22, p=0.04), interventricular thickness (positive correlation, R2=0.22, p=0.03) and posterior wall thickness (positive correlation, R2=0.25, p=0.02) in the complex SAS group, with no signifi cant correlations in the simple SAS group. Conclusion - Complex form of subaortic stenosis is associated with a more important left ventricular remodelling probably due to the role played by associated cardiac defects and seems to have a less favourable outcome, both in surgically managed and conservatively treated patients, independent of NYHA functional class and left ventricular ejection fraction at initial diagnosis, with the limitation of the small dimension of the studied group. Further larger and prospective studies are needed in order to confi rm these aspects. [ABSTRACT FROM AUTHOR]

We report a 15-year-old female patient with an unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation. [ABSTRACT FROM AUTHOR]

A 10 years old boy diagnosed as subaortic membrane with patent ductus arteriosus presented with history of occasional fever, repeated respiratory tract infection since childhood and poor gain weight.

Background: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute.Methods: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken.Results: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%.Conclusions: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence. [ABSTRACT FROM AUTHOR]

This article presents a case review and literature review focused on the challenges of managing subaortic membranes (SAMs) in young adult patients with mild aortic regurgitation (AR) or aortic stenosis (AS). The study aims to discuss the diagnosis of SAM, the imaging studies used for assessment, the management strategies in young patients, the risk of valvular damage, and the controversy surrounding prophylactic resection in mild AR. The management of SAM in adults poses challenges due to limited treatment options and potential complications, necessitating further investigation into the progression of AS and AR in asymptomatic SAM patients. The case presentation describes a 40-year-old male with muscular dystrophy who presented with symptoms and was diagnosed with SAM. Various imaging techniques, including CT chest, transthoracic echocardiogram (TTE), and transesophageal echocardiogram (TEE), were used to confirm the presence and severity of SAM. Based on the patient's clinical profile and the absence of surgical indications, medical therapy was initiated, and regular outpatient follow-up was recommended to monitor disease progression. The discussion highlights the challenges in diagnosing SAM, the importance of imaging studies, and the potential complications associated with SAM in young patients. The article also explores the management options for SAM, emphasizing surgical resection as the definitive treatment, while acknowledging the limited success rates of alternative approaches. Close monitoring and prompt intervention for complications are crucial in the management of SAM. The concluding statement emphasizes the need for further research to explore alternative treatments for SAM in young patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Asif et al.)

Subaortic stenosis secondary to subaortic membrane is the second most common form of left ventricular outflow tract obstruction. We present the case of a 70-year-old male patient who presented with a 6-week history of progressive signs of heart failure. Multimodality imaging was required to confirm the presence of a subaortic membrane. ( Level of Difficulty: Beginner. )., Competing Interests: Dr Kleiman has served as a local principal investigator in trials sponsored by Boston Scientific, Medtronic, Abbott, and Edwards Lifesciences. Dr Reardon has served as a consultant for Medtronic, Boston Scientific, Abbott, and W.L Gore & Associates. Dr Goel has served as a consultant for Medtronic and W.L. Gore & Associates; and has served on the Speakers Bureau for Abbott Structural Heart. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Double-chambered right ventricle (DCRV) is a rare congenital anomaly in which the right ventricle is divided into two compartments with varying pressures due to an anomalous muscle bundle. Here, we describe a case of an adolescent male with DCRV with associated ventricular septal defect and subaortic membrane. Two-dimensional and three-dimensional transthoracic echocardiography with color flow clearly outlined all the three cardiac anomalies as well as their relationship with each other. The diagnosis was confirmed by cardiac catheterization. The patient underwent successful surgical resection of the anomalous muscle bundle along with repair of the associated anomalies. [ABSTRACT FROM AUTHOR]

Subaortic obstruction due to systolic anterior motion ( SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy ( HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms. Identification requires a high index of suspicion and raises important management considerations, including need for surgical myectomy for definitive relief of obstruction with associated excellent outcomes, as the alternative invasive treatment option with percutaneous alcohol septal ablation would be ineffective. [ABSTRACT FROM AUTHOR]

A subaortic membrane is an uncommon cause for left ventricular outflow tract obstruction. Hypertrophic cardiomyopathy with dynamic left ventricular outflow tract obstruction would mask the presence of the subaortic membrane on transthoracic echocardiography and cause a false diagnosis. We report a patient with subaortic stenosis due to flail subaortic membrane misdiagnosed as obstructive hypertrophic cardiomyopathy on transthoracic echocardiography, identified on transesophageal echocardiography and cardiac catheterization. [ABSTRACT FROM AUTHOR]

Subaortic Membrane is the most common type of subaortic stenosis. That, coexisting with hypertrophic obstructive cardiomyopathy (HOCM) is an extremely rare combination and clinically underappreciated. In this report, we will discuss an 18-year-old male patient who presented with chest pain and dyspnea due to fixed (sub-aortic membrane), as well as dynamic (HOCM), left ventricular outflow tract obstruction (LVOT) obstruction., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Farhan et al.)

Anomalous origin of the right coronary artery (ARCA) represents <3% of congenital coronary anomalies, while the subaortic membrane represents 6.5% of congenital heart anomalies. Symptomatic co-occurrence of ARCA and subaortic membrane in an adult is rare. A 68-year-old man developed a non-ST-elevation myocardial infarction necessitating percutaneous coronary intervention (PCI) four years prior to presentation at our hospital. In the years after his PCI, he developed progressive exertional breathlessness. Following a positive treadmill EKG, he underwent coronary CT angiography that indicated RCA dominance with ARCA arising from the left coronary sinus and coursing between the ascending aorta and pulmonary artery, causing 50-60% intraluminal narrowing at rest without atherosclerotic plaque. Echo showed normal left ventricular ejection fraction (LVEF) and a surprise finding of the subaortic membrane, with a modest gradient. He underwent successful resection of the subaortic membrane and unroofing of the anomalous RCA tunnel with tract marsupialization. The post-operative period was complicated by arrhythmias necessitating electrical cardioversion. At discharge, he was sent home on apixaban, bisoprolol, aspirin, atorvastatin, and an amiodarone taper. The subaortic membrane would not have required intervention independently because it was not associated with a severe gradient. However, surgery is recommended for symptomatic ARCA or subaortic membrane; hence, our patient underwent surgical management. Atrial fibrillation and flutter are the most common arrhythmias following cardiac surgery. Due to the patient's increased risk of complications, cardioversion and anticoagulation were pursued. Although ARCA is congenital, our patient had been asymptomatic for most of his life, suggesting that the development of the subaortic membrane might have triggered symptom onset, combining a modest subaortic gradient with previously asymptomatic exercise-induced right coronary ischemia. Clinicians should consider evaluating for secondary structural heart conditions in newly symptomatic adult patients with ARCA due to the risk of sudden cardiac death, to provide the most complete treatment., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Ogunleye et al.)

Introduction: Subvalvular aortic stenosis, which may have a dynamic component, may be due to a fibrous membrane, muscular obstruction, or a combination of the 2. About 50% of patients with a subaortic membrane also have leakage of the aortic valve. Left ventricular outflow tract (LVOT) obstructive lesions account for approximately 6 percent of cases of congenital heart disease in children; the incidence was estimated to be 6 in 10,000 live births. Subvalvar aortic stenosis (AS) is the second most common form of AS. Among children with congenital AS, subvalvar AS accounts for 10 to 14 percent of cases. Subvalvar AS is more common in males, who account for 67 to 75 percent of cases. Patients with severe or untreated subaortic stenosis may be at risk for sudden cardiac death. An echocardiogram will show the level and severity of the obstruction. It will also show if the left ventricle is thickened or enlarged. The progression is often very slow. This is especially true in people whose obstructions are not detected until they are adults. Surgery may be necessary to stop the progression of subaortic stenosis. Case 1: 25-year-old female patient was admitted on our department, resuscitated and afterward intubated due to ventricular fibrillation. Her condition was additionally complicated by allergy reaction and signs of acute respiratory distress syndrome. In the age of 6 verified CHA. TTE show hypertrophic obstructive cardiomyopathy (HOCM) with PGmean 76mmHg and PGmax 142mmHg in LVOT. Two months later she underwent surgical operation of septal myectomy. 10 years later she is healthy and gave two births. Case 2. 36-years-old female patient, growth with developmental difficulties, adipose with amaurose. Within the preoperative preparation for cholecystectomy operation was diagnosed for HOCM (Figure 1, Figure 2), subvalvular subaortic membrane (Figure 3), with high gradients of subaortic stenosis, PG mean 54mmHg in the LVOT and mild aortic valve regurgitation (Figures 4-6). After cholecystectomy operation, she was scheduled for surgical correction of HOCM. [ABSTRACT FROM AUTHOR]

Rupture of the sinus of Valsalva (RSOV) is an uncommonly encountered condition. It can present with wider manifestations ranging from an asymptomatic murmur to cardiogenic shock. The case discussed in this report also had subaortic membrane which usually presents with subvalvular aortic stenosis, left ventricular hypertrophy, myocardial ischemia and sudden death. Corrective cardiac surgery was advised, but due to financial constraints, the patient could not be operated and he died. Here, the authors report for the first time an unusual presence of both RSOV and subaortic membrane and the patient presented with thromboembolic complication resulting from infective endocarditis. [ABSTRACT FROM AUTHOR]

A fixed subaortic membrane is a rare cause for left ventricular outflow tract obstruction. This case report describes an unusual echocardiographic presentation of a subaortic membrane in which the membrane, initially not seen, was identified only after a linear shadow posterior to the membrane was seen on a transesophageal echocardiogram. This represents an unusual demonstration of an ultrasound beam ‘dropout’ of a subaortic membrane. [ABSTRACT FROM PUBLISHER]

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder leading to left ventricular outflow tract (LVOT) obstruction. It can present with chest pain, syncope, breathlessness, or it may cause sudden cardiac death in some cases. The echocardiography in most cases while cardiac CT or cardiac MRI in selected cases are the important diagnostic modalities to make the diagnosis of HCM. In this case report, we discuss a case of a young female patient previously diagnosed with HCM and presented with palpitations, chest pain, and shortness of breath. Her echocardiography revealed severe asymmetrically hypertrophied left ventricle (LV) with normal function, the systolic anterior motion of the mitral valve was present and a subvalvular aortic membrane was also seen. The CT was also performed showing severe asymmetrical hypertrophied septum and thickened trileaflet tricommissural aortic valve with no calcification or significant valvular aortic stenosis but there was a subaortic membrane (concentric only sparing anteriorly). The presence of subaortic membrane with HCM is a rare finding and it can be a diagnostic challenge and untreated cases are susceptible to progressive heart failure and worsening of the symptoms by further increasing LVOT obstruction. A thorough investigation and planning before surgical intervention is required to achieve optimal results., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Mushtaque et al.)

Background: The resection of a subaortic membrane remains far from a curative operation. We sought to examine factors associated with reoperation and the degree of aortic valve regurgitation as a potential long-term source for reoperation., Methods: All patients who underwent resection of an isolated subaortic membrane between 1995 and 2018 were included. Patients who underwent other procedures were excluded. Paired categorical data were compared using McNemar's test. Univariate time-to-event analyses were performed using Kaplan-Meier methods with log-rank tests for categorical variables and univariate Cox models for continuous variables., Results: A total of 84 patients (median age 6.6, 31% females) underwent resection of isolated subaortic membrane. At a median follow-up of 9.3 years (interquartile range 0.6-22.5), 12 (14%) patients required one reoperation and 1 patient required two reoperations. Median time to first reoperation was 4.6 years. The degree of aortic valve regurgitation improved post-operatively from pre-operatively (p = 0.0007); however, the degree of aortic valve regurgitation worsened over the course of follow-up (p = 0.010) to equivalence with pre-operative aortic valve regurgitation (p = 0.18). Performance of a septal myectomy was associated with longer freedom from reoperation (p = 0.004)., Conclusions: In patients with isolated subaortic membranes, performance of a septal myectomy can minimise risk for reoperation. Patients should be serially monitored for degradation of the aortic valve, even if aortic regurgitation is not present post-operatively.