Search Results (4)

Background and Clinical Significance: Subvalvular aortic stenosis (SAS) is the second most common form of aortic stenosis after valvular disease and predominantly affects male patients. It is frequently associated with other congenital cardiac anomalies, such as ventricular septal defect, and is rarely diagnosed during infancy. Instead, SAS typically manifests during childhood or adulthood as a progressive left ventricular outflow tract obstruction, leading to left ventricular hypertrophy and, in many cases, aortic regurgitation. Case Presentation: The first patient was a 61-year-old man presenting with progressive dyspnea, in whom echocardiography revealed severe subaortic stenosis and computed tomography demonstrated aneurysmal dilatation of the ascending aorta. Intraoperatively, the aortic valve was found to be dystrophic with mixed stenotic and regurgitant disease; therefore, subaortic membrane resection, mechanical aortic valve replacement, and ascending aortic replacement with a synthetic graft were performed. The second patient was a 31-year-old man with exertional dyspnea and a discrete subaortic membrane associated with mild ascending aortic dilatation. Surgical treatment consisted of complete membrane resection and aortic valve repair, while the ascending aorta was preserved. Both patients had an uneventful postoperative course and were discharged on the fourth postoperative day. At 3-month follow-up, both were asymptomatic, in normal sinus rhythm, and demonstrated satisfactory echocardiographic findings without residual left ventricular outflow tract obstruction. Conclusions: Surgical intervention remains the definitive treatment for subvalvular aortic stenosis when clinically indicated. Concomitant cardiac or aortic pathology should be addressed during the same procedure to optimize outcomes. When performed with meticulous technique and appropriate patient selection, surgical correction is associated with excellent early recovery and favorable mid-term results, although long-term follow-up remains essential due to the risk of recurrence. Full article

Subvalvular aortic stenosis (SAS) is a frequent cause of left ventricular outflow tract (LVOT) obstruction in children. Surgical resection is the standard treatment, yet recurrence requiring reintervention remains a significant concern. We aim to identify risk factors associated with surgical reintervention. This retrospective study included 76 patients under 18 years who underwent surgical resection for SAS between 2000 and 2020. Preoperative, intraoperative, and postoperative data were analyzed. The mean age at initial surgery was 5.8 years. In addition to subaortic membrane resection, myomectomy was performed in 73.7% of patients. Reintervention was required in 13.1% of cases, with reintervention-free survival at 5 and 10 years of 93.4% and 89.5%, respectively. Significant predictors of reintervention included younger age and shorter stature at surgery. Myomectomy reduced postoperative gradients but was not protective against recurrence and was associated with a higher rate of conductive disorders requiring pacemakers. While surgical resection of SAS is associated with excellent survival, recurrence remains a concern, especially in younger patients and those with tunnel-like lesions. Individualized surgical planning is essential to balance recurrence risk with potential complications. Full article

Subaortic stenosis (SAS) is a rare heart disease in adults with an unclear etiology and variable clinical presentation. In some cases, SAS appears as hypertrophic cardiomyopathy with obstruction due to the accompanying systolic anterior motion of the mitral valve. A 46-year-old male with dizziness for several months presented in the outpatient department. Two-dimensional transthoracic echocardiography demonstrated a slightly hypertrophic left ventricle with normal systolic function without wall-motion abnormalities. Just below the aortic valve, a linear structure protruding from the septum side and the left-ventricular outflow tract (LVOT) side of the mitral valve was confirmed, which was causing a significant pressure gradient (mean and maximum of 91 mmHg and 138 mmHg, respectively). A diagnosis of SAS with subaortic membrane was made, and surgical myomectomy and subaortic membrane removal surgery were performed. Postoperative transthoracic echocardiography did not show flow acceleration through the LVOT, nor a significant pressure gradient across the aortic valve. This case report highlights the clinical significance of SAS with subaortic membrane, which can be confused with aortic stenosis of other etiology. Full article

A persistent left superior vena cava (PLSVC) is the most frequent anomaly of the venous drainage system. While both a right and left superior vena cava (SVC) are usually present, a unique, left-sided SVC, also known as an isolated PLSVC, accounts for only 10–20% of cases. It is frequently associated with arrhythmias and other congenital cardiac anomalies. Though it is usually an asymptomatic condition, it may pose significant problems whenever central venous access is needed. We report a case of an isolated PLSVC that was diagnosed incidentally during pacemaker implantation for sinus node dysfunction. The venous anomaly was associated with subvalvular aortic stenosis determined by a subaortic membrane; this particular association of congenital cardiovascular anomalies is a rare finding, with only a few cases reported in the literature. We aim to highlight the clinical and practical implications of this condition, as well as to discuss the embryonic development and diagnostic methods of this congenital defect. Full article