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Objective Subaortic membrane is an anatomical intracardiac anomaly that may cause discrete subaortic stenosis and aortic insufficiency. Patients requiring subaortic membrane resection may benefit from a minimally invasive approach; however, subaortic membranes are typically resected through a median sternotomy. We present our initial clinical experience of adult patients who have undergone a mini-thoracotomy subaortic membrane resection. Methods Eight patients who underwent an elective subaortic membrane resection performed through a mini-thoracotomy were retrospectively reviewed. A 5-cm mini-thoracotomy incision was made in the 2nd intercostal space; a videoscope was inserted through a separate incision within the same interspace. Cardiopulmonary bypass (CPB) was instituted via central arterial and peripheral venous cannulation and an aortotomy was made. The subaortic membrane was resected with shafted instruments. The left ventricular outflow tract was inspected and CPB was weaned. Thirty-day mortality, intensive care and hospital length of stay, ventilation time, operative times, postoperative morbidity, and need for additional procedures were evaluated. Results The median CPB and cross-clamp times were 60 and 42 minutes, respectively. The median time to extubation was 3.6 hours. The median intensive care unit and hospital stay were 22 hours and 3 days, respectively. The postoperative left ventricular outflow tract mean gradients decreased significantly (26.5 vs. 9.4 mm Hg, P = 0.001). There were no conversions to sternotomy, perioperative strokes, or 30-day mortality. Conclusions Subaortic membranes can be resected through a mini-thoracotomy approach with excellent clinical results.

WOS: 000378158900002, Background: In this study, we report our short-and mid-term surgical results in patients with discrete subaortic stenosis. Methods: A total of 72 patients (43 males, 29 females; mean age 4.6 +/- 4.5 years; range, 6 months to 11 years) who were surgically treated for discrete subaortic stenosis in our clinic between September 2000 and January 2012 were retrospectively analyzed. Limited myectomy in addition to the transaortic excision of the stenotic structure was performed in all patients. Left ventricular outflow tract gradient and aortic valve regurgitation were evaluated by echocardiography following the procedure. Results: The mean follow-up was 4.9 +/- 2.7 (range, 1 to 10) years. No mortality occurred during the early and late postoperative period. Heart block which necessitated the placement of a permanent pacemaker developed in one pediatric patient. The mean preoperative left ventricle systolic gradient which was measured as 48.4 +/- 13.7 mmHg (range, 32 to 70 mmHg), diminished to 8.6 +/- 5.6 mmHg (range, 4 to 15 mmHg) in the postoperative period. Reoperation was necessary due to recurrent stenosis in two patients (2.7%) during follow-up. Conclusion: Our study results show that limited myectomy in addition to membranectomy results in good mid-term results without increasing morbidity and mortality.

Obstruction of the left ventricular outflow can be caused by several lesions. These are classified by location as subvalvular, valvular, or supravalvular. Subvalvular aortic stenosis, which may have a dynamic component, may be due to a fibrous membrane, muscular obstruction, or a combination of the

We report a new original method of management for subaortic stenosis. When performing myectomy from the transaortic access of Morrow, the significant problem is poor visualization of the interventricular septum. The key to the successful implementation of the septal myectomy is achieving sufficient length, width and depth of resection of the hypertrophied interventricular septum. The authors introduced a new modification called “fixed needle technique” in order to visualize the entire region of septal hypertrophy and achieve safe extended myectomy of ventricular hypertrophy. Three needles of 21 gauge (0.8 mm) are introduced into the interventricular septum immidiately under the fibrous ring of the aortic valve, and reache the distal part of the hypertrophic portion. The right and left needles limit the width of resection and its depth is limited with the central needle. Each needle is a marker for the width, length and depth of resection. Needles allow to fix the interventricular septum and improve exposure of septal hypertrophy. The described technique allows to perform the required myectomy of the same thickness. The technique provides high-quality visualization of the interventricular septum and adequately removes hypertrophied part of myocardium in challenging patients with discrete subaortic stenosis and idiopathic hypertrophic cardiomyopathy.

Discrete subaortic stenosis (DSS) is a congenital heart disease that results in the formation of a fibro-membranous tissue, causing an increased pressure gradient in the left ventricular outflow tract (LVOT). While surgical resection of the membrane has shown some success in eliminating the obstruction, it poses significant risks associated with anesthesia, sternotomy, and heart bypass, and it remains associated with a high rate of recurrence. Although a genetic etiology had been initially proposed, the association between DSS and left ventricle (LV) geometrical abnormalities has provided more support to a hemodynamic etiology by which congenital or post-surgical LVOT geometric derangements could generate abnormal shear forces on the septal wall, triggering in turn a fibrotic response. Validating this hypothetical etiology and understanding the mechanobiological processes by which altered shear forces induce fibrosis in the LVOT are major knowledge gaps. This perspective paper describes the current state of knowledge of DSS, articulates the research needs to yield mechanistic insights into a significant pathologic process that is poorly understood, and proposes several strategies aimed at elucidating the potential mechanobiological synergies responsible for DSS pathogenesis. The proposed roadmap has the potential to improve DSS management by identifying early targets for prevention of the fibrotic lesion, and may also prove beneficial in other fibrotic cardiovascular diseases associated with altered flow.

Background: Recurrence after surgical resection of discrete subvalvar aortic stenosis in children often requires repeat operation. Risk factors for recurrence are poorly understood. We sought to determine potential risk factors for recurrence and postoperative comorbidities in the long term. Methods: Retrospective chart review was performed on all pediatric patients who underwent surgical resection of discrete subaortic stenosis at our institution. Demographics, perioperative findings, and clinical data were analyzed for predisposing factors. Results: From 1991 to 2015, a total of 104 patients underwent primary surgical resection of discrete subaortic stenosis. There were no postoperative deaths. Three (2.9%) patients required pacemaker implantation. Nine (8.4%) patients required repeat resection for recurrence of subaortic membrane over a median follow-up of 8.5 years (interquartile range: 5.9-13.5 years). Actuarial freedom from repeat resection was 100%, 94%, and 82% at one, five, and ten years, respectively. Repeat resection occurred more frequently in patients with genetic disease (37.5% vs 10.7%; P = .033) and preoperative mitral regurgitation (MR; 25% vs 1.2%; P < .001). Postoperative aortic insufficiency (AI) that was moderate or worse was associated with older age at the time of first resection (relative risk [RR]: 1.54, P < .05), moderate or severe preoperative AI (RR: 1.84, P = .002), and repeat resection of subaortic stenosis (RR: 1.90, P < .001). Conclusion: The majority of children who undergo surgical resection of subaortic stenosis will not experience recurrence in childhood and those who do require repeat resection may have a higher incidence of genetic disease and preoperative MR. Postoperative AI is associated with repeat resection, older age at the time of surgery, and degree of preoperative AI.

Since 2007, in addition to a lower mid-line mini-sternotomy and a right anterior mini-thoracotomy, a right posterior-lateral mini-thoracotomy has also been offered as a surgical option. Depending on the type of congenital heart disease (CHD), we may enter into the chest by using the fourth or the fifth intercostal space. In details, we enter the chest in the fifth intercostal space for treating more simple CHDs as ASD II and partial-AVSD, usually with the use of induced ventricular fibrillation. In contract, we chose to enter in the fourth intercostal space for more complex CHDs as partial anomalous pulmonary venous connection (PAPVC), discrete subaortic stenosis, ventricular septal defects, and so on. Through this approach the aorta can be easily visualized and cross-clamped. As in right anterior mini-thoracotomy (RAMT), when aortic cannulation is required (patients with a body weight Chapter 5 ). This cannula has a long stilet, which make easy aortic cannulation.

A 54-year-old man, ex smoker with high blood pressure and a history of possible Wolff-Parkinson-White syndrome was admitted for presenting an episode suggestive of acute coronary syndrome with immediate syncope and left bundle branch block, while performing physical activity. Angioplasty and a drug-eluting stent were performed in the left circumflex artery. Subsequently, Doppler echocardiography disclosed an image suggestive of a subaortic membrane. Given these findings, the patient underwent a 3D transesophageal echocardiogram and a magnetic resonance study, which confirmed the diagnosis of a subaortic membrane. In turn, in the Holter monitoring a paroxysmal pattern of Wolff-ParkinsonWhite was observed. The patient presented three possible causes of syncope. A stress echocardiogram elicited a gradient of 126 mm Hg, which could be possibly related to the syncopal episode that the patient suffered.Paciente masculino de 54 años, ex fumador, hipertenso y con el antecedente no confirmado de síndrome de Wolff-Parkinson-White, que ingresó por haber presentado, mientras realizaba actividad física, un cuadro sugestivo de síndrome coronario agudo con inmediato episodio de síncope y bloqueo de rama izquierda, por lo que se le realizó una angiografía coronaria con posterior angioplastia y la colocación de un stent liberador de drogas en la arteria circunfleja. En el ecocardiograma Doppler se observó una imagen compatible con membrana subaórtica. Ante estos hallazgos se realizó un ecocardiograma transesofágico 3D y una resonancia magnética cardiaca que confirmaron el diagnóstico. A su vez se evidenció en el monitoreo Holter y de forma paroxística el patrón de Wolff-Parkinson-White. De esta manera, el paciente presentó tres posibles causas de síncope. Se realizó un ecocardiograma de esfuerzo, en el que el gradiente intraesfuerzo alcanzó los 126 mm Hg, lo que podría justificar el episodio del síncope.

Double-chambered right ventricle with discrete subaortic stenosis without ventricular septal defect is rare in adults. This report shows incremental value of 3D echocardiography in delineating the pathoanatomy of these lesions.

Discrete subaortic stenosis (DSS) is a progressive obstruction of left ventricular outflow tract (LVOT) resulting in the development of aortic regurgitation in its natural course. Hence, early intervention is recommended. Percutaneous balloon dilation of DSS has been reported and can substantially reduce LVOT pressure gradient. The response to balloon dilation of DSS is transient and may complicate with chordal or papillary muscle rupture. Hence, balloon dilation of DSS should be discouraged.

A 28-year-old man with mild dyspnea on exertion presents for evaluation. He has a history of subaortic stenosis (SAS), bicuspid aortic valve (BAV), and coarctation of the aorta (CoA; Figure 1). He underwent repair of his CoA as a neonate with patch aortoplasty. At age 9, he was discovered to have SAS and had surgical resection of a subaortic membrane (Figure 2). His primary care provider referred him for evaluation after noting arterial hypertension and hearing a to-and-fro murmur on examination. Blood pressures suggested a recurrent coarctation with a 40 mm Hg gradient between the right arm and right leg. An echocardiogram demonstrated severe aortic regurgitation (AR; Figure 3) and recurrent SAS with a mean gradient of 31 mm Hg across the left ventricular outflow tract (LVOT). Cardiac magnetic resonance (CMR) confirmed recurrent CoA and estimated the aortic regurgitation fraction to be 30% (Figures 4 and 5). The patient underwent stenting of his CoA (Figure 6). Evaluation 6 months later revealed a mean gradient of 42 mm Hg across the LVOT with unchanged AR. Consequently, the patient underwent surgical aortic valve (AoV) replacement and SAS resection. Figure 1. Aortic coarctation by echocardiography. Echocardiogram in a 2-wk-old neonate with coarctation of the aorta. The 2-dimensional image ( A ) shows a tight stenosis at the isthmus (asterisk) with a posterior shelf (arrow) and mild hypoplasia of the aortic arch. The left common carotid artery (LCC) is dilated. The color Doppler image ( B ) demonstrates flow acceleration at the site of coarctation. Movie in the Data Supplement. AAo indicates ascending aorta; IA, innominate artery; and LSCA, left subclavian artery. Figure 2. Transesophageal echocardiogram of a discrete subaortic membrane. Transesophageal echocardiogram in the long-axis orientation showing a discrete subaortic membrane on the septal surface of the left ventricular (LV) outflow tract, 6 mm from the aortic valve leaflets. A , Two dimensional …

DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood. One patient underwent liver and kidney transplant at three yr of age for hereditary polycystic disease, and the other had liver transplant at three months of age due to uncontrollable high-output failure secondary to a large hepatic hemangioma. Both patients developed mild dynamic LV outflow tract obstruction and systemic hypertension soon after transplant, both of which resolved by the time of discharge. Transient LV hypertrophy was observed in both cases. Corticosteroids, cyclosporine A, and tacrolimus were used as initial immunosuppressants. Both patients developed DSS long after the regression of the initial dynamic LV outflow tract obstruction. Herein, we report DSS as an atypical late cardiovascular complication after solid organ transplant and postulate that transient post-transplant hyperdynamic LV outflow tract obstruction and chronic immunosuppressive treatment may lead to subendocardial remodeling resulting in a fibromuscular membranous ridge in susceptible subjects.

Precise anatomical analysis of stenotic lesions of the left ventricular outflow tract (LVOT) in discrete subaortic membranous stenosis (DSS) and hypertrophic obstructive cardiomyopathy (HOCM) is challenging due to their complex nature [(1,2)][1]. In the present study, we clarified the difference of

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

Item does not contain fulltext AIMS: Discrete subaortic stenosis (DSS) is often diagnosed early in life and known for its sometimes rapid haemodynamic progression in childhood and strong association with aortic regurgitation (AR). However, data about the evolution of DSS in adulthood are scarce. Therefore, we aimed to evaluate the natural history of DSS, and identify risk factors for the progression of DSS, AR, and intervention-free survival. METHODS AND RESULTS: Conservatively managed adult DSS patients were included in this retrospective multicentre cohort study. Mixed-effects and joint models were used to assess the progression of DSS and AR, and intervention-free survival. Longitudinal natural history data were available for 149 patients [age 20 (IQR: 18-34) years, 48% male]. Sixty patients (40.3%) had associated congenital heart defects (CHDs). The median follow-up duration was 6.3 (IQR: 3.0-12.4) years. The baseline peak left ventricular outflow tract (LVOT) gradient was 32.3 +/- 17.0 mmHg and increased by 0.8 +/- 0.1 mmHg/year. While the baseline LVOT gradient (P = 0.891) or age (P = 0.421) did not influence the progression rate, the presence of associated CHD was associated with faster progression (P = 0.005). Mild AR was common (58%), but did not significantly progress over time (P = 0.701). The median intervention-free survival was 16 years and associated with the baseline LVOT gradient [hazard ratio (HR) = 3.9 (95% CI: 2.0-7.6)], DSS progression [HR = 2.6 (95% CI: 2.0-3.5)], and AR [HR = 6.4 (95% CI 2.6-15.6)]. CONCLUSION: In contrast to children, DSS progresses slowly in adulthood. In particular, patients with associated CHD are at risk for faster progression and should be monitored cautiously. Discrete subaortic stenosis progression is not influenced by the baseline LVOT gradient or age. Mild AR is common, but non-progressive over time.

Background— Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. Methods and Results— Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th–75th percentile, 18.4–31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th–75th percentile, 6.2–20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively ( P P =0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time ( P =0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02–2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31–1.62). Additional myectomy did not reduce the risk for reoperation ( P =0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P =0.005). Conclusions— Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely.

Although acquired left ventricular outflow obstruction has been reported in a variety of conditions, there are scant reports of its occurrence following mitral valve replacement (MVR). This study describes two female patients, who developed severe discrete subaortic stenosis, five years following MVR. In both cases, the mitral valve was replaced by a porcine Carpentier-Edwards 27-mm bioprosthesis with preservation of mitral valve leaflets. The risk of very late left ventricular outflow tract obstruction after bio-prosthetic MVR with preservation of subvalvular apparatus needs to be kept in mind in symptomatic patients.

Discrete subaortic stenosis is an unusual cause of the left ventricular outflow tract obstruction in the adults and characterized by a discrete subaortic membrane. A 52-year-old female patient presented with chief complaints of progressive dyspnoea, chest pain and fatigue. Echocardiographic study showed a discrete fibromembranous ridge located in the subaortic region, which resulted in severe subaortic stenosis, with a mild aortic regurgitation and a mean gradient of 65 mmHg. She underwent surgical resection of the subaortic membrane without any complications. The postoperative course was uneventful, and she was discharged from hospital on the 7th postoperative day. At one-year postoperative follow-up, the patient was doing well without recurrence on echocardiogram. A close follow-up is mandatory for a possible recurrence despite sufficient surgical resection.

We report a case of tricuspid atresia with transposed great arteries and rudimentary right ventricle owing to which the patient developed severe subaortic stenosis and restrictive bulboventricular foramen 5 years after her extracardiac Fontan operation. She underwent a successful modified Damus–Kaye–Stansel operation using her native pulmonary valve. Spiral cardiac computed tomography with three-dimensional reconstructions was instrumental in pre-operative surgical planning.

a b s t r a c t Aim: Percutaneous transluminal balloon dilation is an alternative to surgery in discrete subaortic stenosis (DSS). Methods: We included 75 patients with percutaneously treated DSS, who were followed up for a mean period of 16 ± 6 years.

A 34-year-old woman with Down syndrome with a history of an atrioventricular canal repair and a mechanical mitral valve replacement came to our hospital due to worsening symptoms of heart failure. Echocardiography and computed tomography imagings revealed a thickened bicuspid aortic valve without

Transesophageal echocardiography (TEE) plays an important role for congenital cardiac surgery, such as measurement of residual shunt, residual regurgitation, residual pressure gradient (PG), and so on. For discrete subaortic stenosis, it could be a simple and effective option to check residual PG by direct intraoperative periaortic retrograde left ventricular catheterization if infantile TEE is not available.

Background— Transluminal balloon tearing of the membrane in a thin discrete subaortic stenosis is an alternative to membrane surgical resection. However, the long-term outcome of patients with isolated thin discrete subaortic stenosis treated by transluminal balloon tearing remains unknown. Methods and Results— This 25-year study describes findings from 76 patients with isolated thin discrete subaortic stenosis who underwent percutaneous transluminal balloon tearing of the membrane and were followed up for a mean period of 16±6 years. The age at presentation had a wide range (2–67 years). The mean age at treatment was 19±16 years. Immediately after treatment, the subvalvular gradient decreased from 70±27 to 18±12 mm Hg ( P Conclusions— Most patients (77%) with isolated thin discrete subaortic stenosis treated with transluminal balloon tearing of the membrane had sustained relief at subsequent follow-ups without restenosis, the need for surgery, progression to muscular obstructive disease, or an increase in the degree of aortic regurgitation.

Objectives The purpose of this study was to determine the prevalence of valvular aortic stenosis requiring surgery in patients with a pre-existing diagnosis of subaortic stenosis. Background Classic teachings emphasize aortic regurgitation as the most common complication associated with discrete subaortic stenosis. We hypothesized that significant aortic stenosis may also be an important valve lesion associated with this condition. Methods Clinical outcomes in patients with subaortic stenosis were examined. The primary outcome of interest was the prevalence of valvular aortic stenosis requiring surgery (surgical valvotomy or valve replacement). Logistic regression was used to identify variables associated with the need for surgery for aortic stenosis. Results One hundred twenty-one adults with subaortic stenosis (median age 32 years) were evaluated in our clinic. Associated lesions were common: 23% had bicuspid valves and 21% had coarctation of the aorta. Seventy-nine percent of the patients had at least 1 surgical resection of subaortic tissue (median age 12 years). Moderate to severe aortic regurgitation was present in 16% of patients (19 of 121), 3 of whom required surgical intervention in adulthood. Twenty-six percent of patients (32 of 121) required surgery for valvular aortic stenosis. Valve surgery for aortic stenosis was more common in patients with concomitant bicuspid aortic valve disease (p = 0.008), coarctation of the aorta (p = 0.03), and supravalvular stenosis (p = 0.02). Conclusions Valvular aortic stenosis is a surprisingly common finding in patients with discrete subaortic stenosis. Careful clinical follow-up of this population to monitor aortic valve status continues to be warranted even after a successful surgical resection.

OBJECTIVE: The term “subaortic stenosis” includes a variety of obstructions of the left ventricular outflow tract (LVOT), ranging from a short (discrete) subvalvular membrane to long, tunnel-like narrowing. An association with other congenital lesions is frequent. We reviewed the reported literature and describe our results, analyzing the nomenclature of and risk factors for restenosis after surgical treatment. METHODS: From 1994 to 2009, 81 children (53 males, 28 females; median age: 57 months, range [ R]: 5–204) underwent surgical relief of a subaortic stenosis. Patients were divided, according to pathology, into short segment (group A, n = 42) and complex obstructions (group B, n = 39), with the latter including long segment stenosis and/or associated anomalies such as aortic coarctation, interrupted aortic arch or Shone's complex. RESULTS: Surgery resulted in a significant reduction of the gradient between the left ventricle and the aorta in both groups (Δ P group A: 51 ± 28 mmHg, group B: 46 ± 25 mmHg). There was no operative mortality. One patient died in the early postoperative period due to pericardial tamponade. Median follow-up was 90 months ( R = 0.5–187). Twenty-five (31 %) patients required reoperation because of recurrent stenosis after a median of 43 months ( R = 0.5–128). Seven (16 %) patients belonging to group A developed restenosis, and 18 (46 %) in group B. Freedom from reoperation for all patients was 60 % after 10 years. 10 (40 %) of the patients of group B were ultimately treated with a Ross-Konno reconstruction of the LVOT. CONCLUSION: Despite adequate surgical resection, recurrence of subaortic stenosis within several years after initial surgical treatment is frequent, especially in patients with complex lesions. In cases requiring reoperation, the surgical therapy is often extensive, and even includes Ross-Konno reconstruction of the LVOT.

Background: Discrete subaortic stenosis (DSS) is a progressive condition. Controversy still rumbles on as to whether the subaortic membrane causes aortic regurgitation (AR) and whether membrane resection reduces AR severity. We investigated the association between the left ventricular outflow tract peak gradient (LVOT-PG) and AR severity preoperatively and changes in AR severity and obstruction recurrence after surgery in DSS patients. Methods: Twenty patients were evaluated before and after surgery for DSS (mean follow-up time: 13.60±9.61 months). The patients were evaluated via transthoracic echocardiography and transesophageal echocardiography, if necessary. The cut-off point for surgery was LVOT-PG ≥50 mmHg or the presence of progressive AR. Results: The mean age of the patients was 28.55±15.23 years, and 35% of them were male. LVOT-PG decreased from a mean of 80.83±42.72 mmHg preoperatively to 19.14±14.03 mmHg postoperatively and to 25.47±16.10 at follow-up. AR was identified in 15 (75%) patients preoperatively: mild in 8 (40%) and moderate in 7 (35%). The postoperative change in AR severity was insignificant. The correlation between preoperative LVOT-PG and the incidence and severity of preoperative AR was not significant. AR severity had no correlation with age. Membrane recurrence occurred in 25% of the patients. Conclusion: Our results indicated no relationship between AR severity and LVOT-PG and the patient’s age. Patient selection for surgery can, therefore, be carried out on the basis of LVOT-PG or AR severity separately. Subaortic resection may reduce AR severity in some patients, but this reduction is not significant. Future studies are required to elucidate whether or not the presence of the AR is an indication for surgery.

We present a case of 61-year-old man that was evaluated for possible aortic stenosis but did not show a left ventricular outflow gradient on invasive assessment in the catheterization laboratory. Transthoracic echocardiography showed subaortic stenosis secondary to a discrete membranous structure in the left ventricular outflow tract. This is the first case in the literature of a patient with discrete subaortic stenosis missed by invasive hemodynamic assessment.

Aims Associated left ventricular structures may play a role in progression and recurrence of discrete subaortic stenosis. The availability of a new 3D echocardiography tool, multiplanar review (MPR), allows comprehensive analysis of datasets in infinite planes, and detailed examination of anatomy. We sought to evaluate the role of MPR in defining the morphology of subaortic stenosis. Methods Consecutive patients underwent detailed 2 and 3D echocardiographic examination using MPR. Results Sixteen patients aged 0.7–15.9 years (median 4.57) with diagnosis as follows: isolated subaortic stenosis in nine, additional defects in seven (coarctation of aorta, VSD, mitral, or aortic stenosis). Position and extent of subaortic stenosis was clearly described by multiplanar revew in all patients. Additional MPR findings were: abnormalities of mitral valve leaflet or chordal apparatus attachments (14 patients), abnormal ventricular muscle band (11), abnormal increased aorto-mitral separation (two). The aortoseptal angle was significantly decreased in subaortic stenosis, mean 141 ± 12°, vs. normal subjects, mean 153 ± 6°, P = 0.02. Surgical findings correlated well with MPR findings. Conclusions MPR analysis of 3D datasets is a sensitive and accurate mode for delineation of morphological details of discrete subaortic stenosis, providing additional information to 2D echocardiography.

ObjectivesThis study aimed to identify independent predictors of reoperation after successful resection of discrete subaortic stenosis (DSS).BackgroundRecurrence of DSS has been reported to range from 0% to 55% of patients. Factors associated with recurrence have not been adequately defined.MethodsPatients were included if they had a diagnosis of DSS, normal segmental cardiac anatomy, previous resection of DSS, and at least 36 months’ follow-up. Demographic, surgical, and echocardiographic data were analyzed. Primary outcome was repeat resection of DSS in patients after successful primary resection.ResultsOf 111 subjects who had successful surgical resection of DSS, 16 patients (14%) required reoperation. Median follow-up time was 8.2 years. Form of DSS and gender did not differ significantly between those with reoperation and those without. In multivariate analysis, independent predictors of reoperation that would be available before first surgery were

Discrete subaortic stenosis (DSS) is a well-described cause of isolated left ventricular outflow tract obstruction( LVOTO) in children. But prevalence, rate of progression and postoperative data in adults are limited. We report a case of a 30-year-old woman, who was referred to our institution because of chest pain and loss of consciousness. Echocardiography revealed DSS with LVOTO (peak gradient 81 mmHg) and mild aortic regurgitation. Increased age at the time of diagnosis, female sex and preoperative left ventricular outflow tract(LVOT) gradient ≥80 mmHg were thought to be predictors for reoperation, therefore the obstructing membrane was circumferentially excised and concomitant localized myectomy of the ventricular septum was performed to achieve complete relief of the LVOT obstruction. Her postoperative course was uneventful, and she was discharged on the 5th postoperative day.

Background: The optimal surgical procedure for treatment of fibromembranous subaortic stenosis has been a subject of debate. We report our experience with patients treated for membranous subaortic stenosis using membrane resection alone and membrane resection plus aggressive septal myectomy. Methods: Patients followed in the pediatric cardiology clinic of a university hospital, who had undergone surgery for subaortic stenosis between 2002 and 2013 were reviewed. Recurrence of subaortic membrane, residual left ventricular outflow gradient, and aortic valve function were analyzed. Results: Forty-six patients underwent surgery for subaortic membrane. Of these, 19 had membrane resection plus aggressive septal myectomy, while 27 had membrane resection alone. Mean age at surgery for the membrane resection group was 7.7 ± 3.9 years and 10.9 ± 3.6 years for the membrane resection plus aggressive myectomy group. Preoperative subaortic gradient for the membrane resection group was 75.5 ± 26.7 mm Hg and 103.2 ± 39.7 mm Hg for the membrane resection plus aggressive myectomy group. The mean follow-up left ventricular outflow tract gradient was 42.3 ± 31.3 mm Hg in the membrane resection group, while it was 11.6 ± 6.3 mm Hg in the aggressive septal myectomy group. Nine patients from the membrane resection group had significant regrowth of the subaortic membrane during the follow-up period, while none of the aggressive septal myectomy group had detectable membrane on echocardiography. Seven of the nine patients with recurrence of the subaortic membrane underwent subsequent membrane resection plus aggressive septal myectomy. Intraoperative finding in all these redo cases was recurrence (growth) of a subaortic membrane. Conclusion: Aggressive septal myectomy offers less chance of recurrence, freedom from reoperation, and an improved aortic valve function. This is especially important in sub-Saharan settings where a chance of getting a second surgery is unpredictable.

Furthermore, it is difficult for individual cardiovascularsurgeons to gain sufficient experience outside high-volume centers because of the low incidence of surgicalcases. To perform this difficult surgical technique moreeasily,weusemade-to-ordersurgicalinstrumentsforresec-tionofthemembraneormuscleinpatientswithSAS.Thesesurgical instruments are custom-made for the depth andtargetangleinthetransaorticapproach.Wereporttheiruse-fulness in a sample surgical case of SAS.TECHNIQUE AND RESULTSInstrumentsHere we describe the special features of surgical instru-ments for resection of the subaortic region (Figure 1). Thescalpel has a total length of 22 cm and an angle of 140 at3 cm from the tip. It is used by attaching an edge to thetip. The scissors has a total length of 25 cm and an angleof160 rightinthemiddle.Theretractorshaveatotallengthof 30 cm and an angle of 120 at 5 cm from the tip with agroove.CaseAn 11-year-old girl had a diagnosis of discrete SAS withconstrictivepericarditis.Althoughshecouldnotreportsub-jective symptoms because of severe mental disability, weelectedtoperformpericardiotomywithresectionofthesub-aorticmembraneinlightofthehighpeakinstantaneous leftventricular outflow tract (LVOT) gradient of 80 mm Hg.Theoperationwasperformedthroughamediansternotomy.Afterpericardiotomy,thepatientwascooledto32 Cforce-rebral protection under cardiopulmonary bypass andsubsequentcardiacarrest.Afteraortotomy,thesubaorticre-gion was excellently exposed with angled retractors with agroove (Figure 2, A). The obstructive fibrous membrane,which appeared on the LVOT (Figure 2, B), and a part ofthe septal muscle were resected with a scalpel and scissorsangled to enter the septal muscle at a shallow angle(Figure 2, C and D). We confirmed passage of the diameterdilator(15mm).Afterweconfirmedthattherewasnoaorticregurgitation and no injury of the aortic valve, the incisionof the aorta was closed. The postoperative peak instanta-neous LVOT gradient was decreased, and this patient wasdischarged from the hospital on postoperative day 11.DISCUSSIONAlthoughearlysurgicalrepairofSASisassociatedwithasignificant recurrence risk and aortic regurgitation progres-sion,surgicalinterventionisrequiredinpatientswithahighLVOTgradient.

Persistan sol süperior vena kavanın sağ vena kava süperior yokluğuyla beraberliği viseroatriyal situs solituslu hastalarda oldukça nadir gözlenen bir konjenital anomalidir. Bu anomalinin herhangi bir klinik semptomu olmamasına karşın, cerrahi ve diger invazif prosedürler öncesi tanı konması, transvenöz pacemaker veya defibrilatör yerleştirilmesi, intraoperatif yada monitarizasyon için postoperatif pulmoner arter kateteri yerleştirilmesi, ekstrakorporeal sirkülasyon veya kardiopulmoner bypass için sistemik venöz kanülasyon, kavapulmoner anastomoz ve ortotopik kalp transplantasyonunu içeren bir çok durumda yönetim zorluklarından kaçınmak için önemlidir. Biz bu makalede süperior vena kava yokluğu, persistan sol süperior vena kava ile diskret subaortik membran birlikteliği bulunan ve başarılı cerrahi uygulanan bir hastamızı sunduk. Anahtar kelimeler: Süperior Vena Kava; Sistemik Venöz Dönüş Anomalisi; Diskret Subaortik Stenoz; Kalpdamar Cerrahisi., Persistent left superior vena cava accompanied by an absent right superior vena cava in visceroatrial situs solitus is an extremely rare congenital anomaly. Although absence of RSVC alone in patients with visceroatrial situs solitus is of no hemodynamic significance, its diagnosis before surgery or other invasive procedure is important to avoid various management difficulties, which include complications such as the implantation of a transvenous pacemaker or defibrillator, placement of pulmonary artery catheter for intraoperative or intensive care unit monitoring, systemic venous cannulation for cardiopulmonary bypass or extracorporeal circulation, cavopulmonary anastomosis, and orthtopic heart transplantation. We present a case with the absence of RSVC along with PLSVC in association with a discrete subaortic membrane, at the end of which the patient was operated successfully. Key words: Superior Vena Cava; Systemic Venous Return Anomaly; Discrete Subaortic Stenosis; Cardiovascular Surgery.

Left ventricular (LV) false tendons were first described in 1893 by the British anatomist and surgeon Sir William Turner [1]. Turner proposed that they reduce the severity of functional mitral regurgitation by stabilizing the position of the papillary muscles as the left ventricle enlarges and promoting membrane formation in discrete subaortic stenosis .LV false tendons are found in about half of hearts examined at autopsy and occur with equal frequency in normal hearts and in those with congenital malformations [2]. Autopsy and surgical series have demonstrated a slight male preponderance. False tendons containing conduction tissue have been identified as substrates of intracavitary ventricular tachycardia [3] and have been successfully ablated. We report a 48 years old lady presented to the emergency department with history of sudden onset palpitations associated with fatigue .Twelve leads electrocardiogram was suggestive of ventricular tachycardia with right bundle branch block (RBBB) morphology (Figure 1), Patient was hemodynamically unstable so electrically cardioverted. Subsequently 2D transthoracic echocardiogram revealed dilated left ventricle with moderate LV dysfunction and showing false tendon attached from left ventricular free wall to intraventricular septum (Figure 2), which could be the focus of ventricular tachycardia in our patient.