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INTRODUCTION/OBJECTIVES: In clinical practice, dogs are screened for subaortic stenosis (SAS) using two-dimensional (2DE) and Doppler echocardiography. There is no accepted antemortem diagnostic criterion to distinguish between mild SAS and unaffected, therefore additional means of evaluating the left ventricular outflow tract (LVOT) and aorta may be desirable. This study sought to determine and compare LVOT and aortic orifice areas using 2DE and three-dimensional echocardiography (3DE) in apparently healthy dogs of various breeds and somatotypes. ANIMALS, MATERIALS, AND METHODS: Sixty-nine healthy, privately-owned dogs. The LVOT and aortic orifice areas were determined using 2DE aortic valve (AV) diameter-derived area; the continuity equation (CE); and 3DE planimetry of the LVOT, AV, sinus of Valsalva, and sinotubular junction. Orifice areas were indexed to body surface area (BSA). RESULTS: Obtaining 3DE images and performing planimetry were feasible in all dogs. The mean indexed area measured using the 2DE AV diameter (2.85 cm2/m2) was significantly lower than that derived from 3DE AV planimetry (3.85 cm2/m2; mean difference, 1.00 cm2/m2; P<0.001). There was poor agreement between the effective area calculated using the CE and the anatomic areas calculated using 2DE AV diameter and 3DE planimetry. The area calculated using the CE was less than all other calculations of area. Interobserver and intraobserver repeatability and reproducibility for 3DE planimetry were excellent. CONCLUSIONS: Methods for determining aortic orifice areas in dogs are not interchangeable, and this must be taken into account if these methods are investigated in the evaluation of dogs with SAS in the future.

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INTRODUCTION: To echocardiographically evaluate a large number of apparently healthy Czechoslovakian wolfdogs (CWDs) to identify possible subclinical cardiac abnormalities and to generate reference intervals. ANIMALS: One-hundred and seventeen apparently healthy client-owned CWDs. MATERIALS AND METHODS: Standard two-dimensional, M-mode, and Doppler echocardiographic measurements were obtained on non-sedated, manually restrained standing dogs. Animals with no relevant echocardiographic abnormalities were used to generate reference intervals. Echocardiographic variables were compared between males and females and between dogs with and without mitral regurgitation (MR). RESULTS AND DISCUSSION: Among the 117 CWDs, 103 dogs were used to generate reference intervals. The 14 dogs with abnormalities had more than trivial MR (12 dogs), subaortic stenosis (one dog), and equivocal subaortic stenosis (one dog). The 44 males were heavier than 59 females (P<0.001) and had a larger maximum left atrial dimension (P=0.015), left ventricular internal dimension at end-diastole (P<0.001) and systole (P<0.001), and thicker interventricular septum thickness at end-diastole (P=0.016). A positive linear correlation was identified between bodyweight and aortic root and left atrial diameters and left ventricular dimensions and between age and aortic root and left atrial diameter and peak late transmitral flow velocity. A negative linear correlation was identified between age and peak early transmitral flow velocity and the ratio between peak early and late transmitral flow velocities. No differences in echocardiographic-derived cardiac dimensions were detected between healthy dogs and dogs with more than trivial MR. CONCLUSIONS: In this population of CWDs, subclinical cardiac abnormalities were uncommon, and they were mainly classified as MR.

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OBJECTIVES: The clinical data of patients with subaortic stenosis who underwent surgical treatment in our centre in the past 12 years were reviewed. The short-term and long-term clinical outcomes were analyzed, and the long-term outcomes of different surgical methods for subaortic stenosis were compared to determine the optimal surgical treatment strategy for subaortic stenosis. METHODS: From December 2010 to December 2022, 90 patients undergoing surgical treatment for subaortic stenosis in our hospital were enrolled. There were 55 males and 35 females with a median age of 72 (46,132) months and an average surgical weight of (21.35 ± 15.84) kg. According to the operation method, 90 patients were divided into group A (50 patients with simple subaortic membrane resection) and group B (40 patients with subaortic membrane and muscle resection or modified Konno procedure). RESULTS: There were three early deaths (3.33%). One late death occurred in group B. There was no significant difference in long-term survival rate between the two groups (p = 0.904). The preoperative left ventricular outflow tract pressure gradient in group B was (91.56 ± 36.98) mm Hg, which was higher than that in group A(51.13 ± 36.04)mm Hg(p < 0.001). There was no significant difference in immediate postoperative left ventricular outflow tract pressure gradient between group B [(5.44 ± 8.43) mm Hg] and group A [(7.82 ± 13.44) mm Hg] (p = 0.343). In the long-term follow-up, left ventricular outflow tract pressure gradient in group B was (5.86 ± 9.53) mm Hg, which was not statistically significant compared with group A (8.83 ± 14.52) mm Hg (p = 0.294). Eleven patients with moderate or greater aortic regurgitation (group A/group B: 3/8) underwent simultaneous aortic valvuloplasty. In group B, moderate or greater aortic regurgitation was significantly improved immediately after operation (p = 0.013) and was not significantly aggravated in long-term follow-up (p = 0.083), and there was no significant improvement in group A after operation and long-term follow-up. CONCLUSIONS: According to the different anatomical lesions of left ventricular outflow tract, the individualised surgical treatment strategy for patients with subaortic stenosis can achieve good long-term outcomes. The long-term survival rate and freedom from reoperation due to late left ventricular outflow tract obstruction after simple subaortic membrane resection and extended left ventricular outflow tract resection are comparable. For patients with moderate or greater aortic regurgitation before extended left ventricular outflow tract resection, simultaneous aortic valvuloplasty is beneficial to improve postoperative aortic valve function.

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Hypertrophic obstructive cardiomyopathy (HOCM) and subaortic membrane (SAS) are distinct cardiac conditions, but their coexistence presents complex diagnostic challenges. We report the case of a 52-year-old male with HOCM and a concurrent subaortic membrane, highlighting the intricacies of diagnosis and management. The patient's presentation included symptoms of dyspnea and chest tightness, and diagnostic evaluations revealed a unique combination of dynamic left ventricular outflow tract (LVOT) obstruction from HOCM and fixed obstruction from the subaortic membrane. This case emphasizes the importance of a comprehensive diagnostic workup to guide appropriate treatment decisions when managing multiple cardiac abnormalities.

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Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

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Subaortic stenosis is a CHD that can lead to left ventricular hypertrophy, heart failure, and aortic valve damage if left untreated. The gold standard treatment for subaortic stenosis is septal myectomy. However, there is no clear consensus on the surgical margins required for adequate muscle resection. In this retrospective study, we reviewed the records of 83 patients who underwent subaortic stenosis surgery between 2012 and 2020 to investigate the effect of early troponin levels on prognosis. We excluded patients with additional cardiac pathologies, hypertrophic obstructive cardiomyopathy, and valvular aortic stenosis.Troponin levels were recorded in the early post-operative period, and patients were monitored for complications such as ventricular arrhythmia, left ventricular systolic dysfunction, infective endocarditis, and pacemaker implantation. The troponin levels were significantly higher in the patients who had septal myectomy. The degree of myectomy affected the risk of complications in the early post-operative period and recurrence in the later period. However, when the gradient was substantially or completely removed by myectomy, patients experienced significant symptom improvement in the early post-operative period, and their late survival was equivalent to that of healthy individuals of the same age.Our findings suggest that monitoring troponin levels in patients undergoing septal myectomy may be beneficial in predicting the risk of complications. However, further studies are needed to establish the optimal surgical technique and extent of muscle resection required for subaortic stenosis treatment. Our study adds to the existing knowledge of the benefits and risks associated with septal myectomy as a treatment option for subaortic stenosis.

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Subvalvular aortic stenosis (SAS) is the most common congenital heart disease (CHD) in dogs and is also prevalent in human children. A fibrous ridge below the aortic valve narrows the left ventricular outflow tract (LVOT) and increases blood flow velocity, leading to devastating side effects in diseased patients. Due to the similarities in presentation, anatomy, pathophysiology, cardiac development, genomics, and environment between humans and dogs, canine SAS patients represent a critical translational model of human SAS. Potential adverse outcomes of SAS include arrhythmias, left-sided congestive heart failure, endocarditis, exercise intolerance, syncope, and sudden cardiac death. The greatest divergence between canine and human SAS clinical research has been the standard of care regarding treatment of these outcomes, with pharmacological intervention dominating best practices in veterinary medicine and surgical intervention comprising the standard practice for human SAS patients. Regardless of the species, the field has yet to identify a treatment option to prevent disease progression or permanently remove the fibrous ridge, but historical leaps in SAS research support a continued translational approach as the most promising method for achieving this goal.

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OBJECTIVES: We reviewed the mid- and long-term surgical outcomes of patients with subaortic stenosis (SAS). METHODS: Patients operated for SAS from April 1990 to August 2016 were reviewed retrospectively. Patients with major associations such as aortic arch obstruction were excluded. Time to reintervention and predictors of recurrence were assessed using Kaplan-Meier analysis, log-rank test and uni/multivariable Cox regression. RESULTS: 120 patients at a median age of 4.7 years (interquartile range 2.9, 8.1) underwent primary operation (median peak preoperative left ventricular outflow tract gradient 52.5 mmHg, interquartile range 40, 70) involving fibrous tissue excision (n = 120) with septal myectomy (93%; n = 112) as the procedure of choice.At median follow-up of 13 years (interquartile range 7, 18), freedom from reintervention at 1, 3, 5 and 10 years was 99% (95% confidence interval 94%, 99%), 94% (87%, 97%), 93% (86%, 96%) and 90% (82%, 94%), respectively. Recurrence occurred in 18% (n = 20) with 15 patients undergoing reinterventions, 13 of whom required radical reoperation. Multivariable analysis revealed higher preoperative peak left ventricular outflow tract gradient (hazard risk 1.06, confidence interval 1.03, 1.09, P < 0.001), and presence of bicuspid aortic valve (hazard risk 14.13, confidence interval 3.32, 60.1, P < 0.001) as predictors for reintervention. Mild/moderate aortic regurgitation occurred in 49% (n = 55) of patients at the most recent follow-up. CONCLUSIONS: Reintervention for recurrent SAS is common, predicted by higher preoperative peak left ventricular outflow tract gradient, and presence of bicuspid aortic valve, and frequently involves a radical procedure. Aortic regurgitation is a major consequence of SAS, but its severity usually remains low. CLINICAL REGISTRATION NUMBER: SCHN HREC reference number 2019/ETH02729, approved on 09 July 2019.

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Hypertrophic obstructive cardiomyopathy (HOCM) describes a pathologic state in which the subaortic region of the interventricular septum undergoes significant hypertrophy and fibrosis, resulting in septal bowing into the left ventricle. The reduced left ventricular chamber size and altered cardiac function impair diastolic filling, stroke volume, and cardiac output. This case report evaluates the cardiac tissue of a 36-year-old, formalin-embalmed cadaver affected by HOCM, with the goal of providing a comprehensive overview of the gross and pathologic findings associated with the condition. This donor's heart was found to be larger than average, weighing 510.1 g, which is 52% heavier than the predicted value of 335.6 g for a male of similar stature. The thickness of the interventricular septum, right ventricular free wall, and left ventricular free wall was comparable to other reports of HOCM. However, asymmetrical thickening of the left ventricular walls, which is characteristic of HOCM, was less prominent than expected. Histologic staining of the cadaveric tissue, with hematoxylin and eosin, trichrome, and desmin, further bolstered the diagnosis. Importantly, this also showed that histologic examination of embalmed tissue is effective and diagnostic, even 11 months after embalming. The report herein demonstrates that morphologic and histologic analysis of cadaveric cardiac tissue is sufficient to support a diagnosis of HOCM. To the researchers' knowledge, this is the first case report evaluating HOCM in a cadaver donated for medical education.

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Subaortic stenosis (SubAS) is a common cause of left ventricular outflow tract obstruction. It may be focal or diffuse, leading to the development of a subaortic tunnel. Considered for a long time as a congenital anomaly, SubAS has been established lately as an acquired anomaly and secondary to a pre-existing anatomic alteration in the interventricular septum and mitral valve apparatus. It is a progressive disease that is often confused with obstructive hypertrophic cardiomyopathy, which can lead to several complications. Case presentation: In this paper, the authors report two cases of SubAS secondary to different mitral valvar anomalies. The analysis of echocardiographic data was a milestone in raising this diagnosis and identifying its mechanisms. Conclusion: This work highlights a rare situation, often underdiagnosed, in which the evolution can be marked by an important risk of recurrence even after surgical cure.

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Bicuspid aortic valve is the most common congenital cause for the development of aortic valve calcification and stenosis. Calcification cause valvular stenosis or valvular insufficiency due to coaptation failure. We report a unique case of calcification of bicuspid valve was extending to left ventricular outflow tract and attached to interventricular septum which caused subvalvular stenosis.

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Subaortic stenosis (SAS) is a common congenital heart disease that can cause significant morbidity and mortality if not treated promptly. Patients with heart valve disease are prone to complications after replacement surgery, and the existence of SAS can accelerates disease progression, so timely diagnosis and treatment are required. However, the effects of subaortic stenosis on mechanical heart valves (MHV) are unknown. This study aimed to investigate flow characteristics in the presence of subaortic stenosis and computationally quantify the effects on the hemodynamics of MHV. Through the numerical simulation method, the flow characteristics and related parameters in the presence of SAS can be more intuitively observed. Based on its structure, there are three types of SAS: Tunnel-type SAS (TSS); Fibromuscular annulus SAS (FSS); Discrete SAS (DSS). The first numerical simulation study on different types of SAS found that there are obvious differences among them. Among them, the tunnel-type SAS formed a separated vortex structure on the tunnel-type narrow surface, which exhibits higher wall shear force at a low obstacle percentage. However, discrete SAS showed obvious differences when there was a high percentage of obstacles, forming high peak flow, high wall shear stress, and a high-intensity complex vortex. The presence of all three types of SAS results in the formation of high-velocity jets and complex vortices in front of the MHV, leading to increased shear stress and stagnation time. These hemodynamic changes significantly increase the risk of MHV dysfunction and the development of complications. Despite differences between the three types of SAS, the resultant effects on MHV hemodynamics are consistent. Therefore, early surgical intervention is warranted in SAS patients with implanted MHV.

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Background: Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention. Case summary: A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *109/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band. Conclusion: Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy.

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Objective:To investigate the prognosis and risk factors for children diagnosed with all types of subaortic stenosis(SAS) who developed recurrent left ventricular outflow tract obstruction after surgical treatment.Methods:The study retrospectively included patients aged 0-18 years old who underwent open heart SAS surgery at Fuwai Hospital from 2016-2019. Children with hypertrophic obstructive cardiomyopathy were excluded. Detailed operative notes, medical records and ultrasound information, and follow-ups were extracted. Recurrent SAS was defined as left ventricular outflow tract gradient 30 mmHg(1 mmHg=0.133 kPa) 1 month after SAS surgical treatment.Results:A total of 137 children were included in this study. The medium age of children at the time of SAS surgery was 4.6 years old(3 months-17.8 years old). After a median follow-up of 4.36 years(3.2-5.7 years), a total of 30 patients developed recurrent LVOTO, with a recurrence rate of 21.9%, and 7(5.1%) underwent a second surgery. Compared to the non-recurrent group, children in the recurrent group were younger at the time of surgery( P=0.0443), had a smaller body surface area( P=0.0485), and a longer length of stay( P=0.0380). In Cox analysis, when only considering preoperative variables, the independent risk factor for LVOTO recurrence were a peak left ventricular outflow tract gradient higher than 50 mmHg( HR=5.25, P=0.001), a BSA less than 0.9( HR=2.5, P=0.023), and a length of SAS 5 mm( HR=2.29, P=0.050). When both preoperative and intraoperative variables were considered, preoperative peak left ventricular outflow tract gradient 50 mmHg( HR=4.91, P=0.002) and peeling from the aortic valve( HR=3.23, P=0.010) were independent risk factors for postoperative recurrence. Conclusion:Recurrent LVOTO after SAS surgical repair is common, and regular postoperative follow-up is crucial to evaluate whether a secondary intervention is required. Regular postoperative follow-up is needed for children at high risk.

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@#Objective     To summarize the characteristics of children diagnosed with secondary subaortic stenosis after the surgical closure for ventricular septal defect and explore its potential mechanism. Methods     We retrospectively collected patients aged from 0 to 18 years, who underwent ventricular septal defect closure and developed secondary subaortic stenosis, and subsequently received surgical repair from 2008 to 2019 in Fuwai Hospital. Their surgical details, morphological features of the subaortic stenosis, and the follow-up information were analyzed. Results     Six patients, including 2 females and 4 males, underwent the primary ventricular septal defect closure at the median age of 9 months (ranging from 1 month to 3 years). After the first surgery, patients were diagnosed with secondary subaortic stenosis after 2.9 years (ranging from 1 to 137 months). Among them, 2 patients underwent the second surgery immediately after diagnosis, and the other 4 patients waited 1.2 years (ranging from 6 to 45 months) for the second surgery. The most common type of the secondary subaortic stenosis after ventricular septal defect closure was discrete membrane, which located underneath the aortic valve and circles as a ring. In some patients, subaortic membrane grew along with the ventricular septal defect closure patch. During the median follow-up of 8.1 years (ranging from 7.3 to 8.9 years) after the sencond surgery, all patients recovered well without any recurrence of left ventricular outflow tract obstruction. Conclusion     Regular and persistent follow-up after ventricular septal defect closure combining with or without other cardiac malformation is the best way to diagnose left ventricular outflow tract obstruction in an early stage and stop the progression of aortic valve regurgitation.

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Objectives: Subvalvular aortic stenosis (SAS) can occur as discrete or tunnel-like obstruction of the left ventricular outflow tract and as progressive disease often leads to aortic valve regurgitation. We report our 30-year single-center experience after surgical repair of SAS. Methods: A retrospective chart review of all patients aged < 18 years, who underwent surgical repair of SAS from May 1985 to April 2020, was conducted. Mortality was cross-checked with the national health insurance database (93.8% complete mortality follow-up in April 2020). Survival and competing risks analysis were used to analyze the primary endpoints survival and incidence of reoperations. Results: From May 1985 until April 2020 103 patients (median age 5.5 years) underwent surgical repair of SAS. Survival was 90.8% at 10 years and 88.7% at 20 and 30 years. Age < 1 year at time of surgery, Shone's complex, mitral stenosis and concomitant mitral valve surgery were associated with mortality. The cumulative incidence of reoperation for SAS was 21.6% at 10 years, 28.2% at 20 and 30 years. The incidence of reoperation for SAS did not differ between the myectomy, membrane resection and combined myectomy and membrane resection groups. The cumulative incidence of reoperation on the aortic valve was 13.5% at 20 years. Conclusion: Recurrence rate of SAS is not to be neglected, though surgical repair of subaortic stenosis has good long-term results. Patients who needed a combined membrane resection and septal myectomy are not more prone to recurrence than patients who underwent solitaire myectomy or membrane resection.

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INTRODUCTION: While only a few cases have been reported in pediatrics, subaortic stenosis (SAS) is a gradually progressive disorder rarely seen at birth and infancy, however, it is the most common type of aortic stenosis. It obstructs the blood flow across the left ventricular outflow tract (LVOT). Although the cause is still not well known, different etiologies have been suggested by the literature. While surgical resection is the definitive treatment, recurrence is observed in many patients, nonetheless, LVOT gradient usually progresses over years of follow-up. CASE PRESENTATION: We report the clinical and diagnostic course of a 41-months-old Saudi boy, asymptomatic child who was found to have progressive recurrent subaortic stenosis within a few months which required two redo sternotomy for sub-aortic membrane resection throughout a period of two years. DISCUSSION: SAS is usually detected incidentally in asymptomatic patients requiring an echocardiogram to assess other accompanying congenital heart defects (CHD), or rather potentially arising after repair of CHD. Patient close monitoring is important aspect given the nature of disease progression, re-operation for recurrence demonstrate significant increase over years, re-resection rate was 0 % after one year, 6 % after five years, and 8 % after 10 years. CONCLUSION: Recurrence of LVOT obstruction following sub-aortic membrane resection is common. Long-term follow-up care in postoperative patients is crucial. Majority of patients will need re-operation for recurrence at certain point during course of the disease.

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Decision-making in congenital cardiac care, although sometimes appearing simple, may prove challenging due to lack of data, uncertainty about outcomes, underlying heuristics, and potential biases in how we reach decisions. We report on the decision-making complexities and uncertainty in management of five commonly encountered congenital cardiac problems: indications for and timing of treatment of subaortic stenosis, closure or observation of small ventricular septal defects, management of new-onset aortic regurgitation in ventricular septal defect, management of anomalous aortic origin of a coronary artery in an asymptomatic patient, and indications for operating on a single anomalously draining pulmonary vein. The strategy underpinning each lesion and the indications for and against intervention are outlined. Areas of uncertainty are clearly delineated. Even in the presence of "simple" congenital cardiac lesions, uncertainty exists in decision-making. Awareness and acceptance of uncertainty is first required to facilitate efforts at mitigation. Strategies to circumvent uncertainty in these scenarios include greater availability of evidence-based medicine, larger datasets, standardised clinical assessment and management protocols, and potentially the incorporation of artificial intelligence into the decision-making process.

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The vulnerability of chromosome 22q11.2 region to rearrangement is due to several low copy repeat (LCR) sequences. These rearrangements are involved in syndromes that share similar phenotypic features. The rearrangements of the 22q11.2 chromosomal region are common, specifically, duplications and deletions associated with congenital anomalies and developmental disabilities disorders. However, the features associated with this chromosomal rearrangement remain largely unknown. We present, to the best of our knowledge, the third patient affected by triplication of the 22q11.2 chromosome region, who presents with Peters anomaly, global developmental delay, patent ductus arteriosus, and subaortic stenosis. This case highlights a new phenotypic feature associated with triplication of this genomic region.