Subaortic stenosis (SAS) is a rare congenital heart defect (CHD), accounting for 1%-2% of all CHD and the second most common type of left ventricular outflow tract (LVOT) obstruction. SAS ranges from localized obstructive membranes to complex tunnel narrowing and often coexists with other CHD like ventricular septal defect, interrupted aortic arch, and aortic coarctation. Despite generally successful surgeries, SAS recurs in 5%-30% of patients, necessitating reoperation. Recurrence factors include younger age at initial surgery, higher preoperative LVOT gradients, and complex anatomical presentations. This report discusses an 11-year-old girl with recurrent SAS who underwent a modified Konno operation. It emphasizes the need for meticulous surgical technique and vigilant postoperative follow-up to manage SAS and reduce recurrence risk effectively. The case highlights the challenges of this CHD and underscores the importance of personalized, comprehensive care strategies.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11879096 | PMC |
http://dx.doi.org/10.1093/jscr/rjaf100 | DOI Listing |
Fabry disease (FD) is a lysosomal disorder due to alpha-galactosidase-A enzyme deficiency, accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) which lead to proinflammatory effects. Males develop progressive hypertrophic cardiomyopathy (HCM) followed by fibrosis; females develop nonconcentric hypertrophy and/or early fibrosis. The inflammatory response to Gb3/lyso-Gb-3 accumulation is one of the suggested pathogenic mechanisms in FD cardiomyopathy when the secretion of inflammatory and transforming growth factors with infiltration of lymphocytes and macrophages into tissue promotes cardiofibrosis.
View Article and Find Full Text PDFSci Rep
March 2025
Department of Cardiovascular Ultrasound, The First Affiliated Hospital of China Medical University, 155 North Nanjing Street, Heping District, Shenyang, 110001, China.
Contemporary classification of hypertrophic cardiomyopathy (HCM) was mainly based on the site of myocardial hypertrophy and left ventricular outflow tract obstruction. A complementary classification based on left ventricular function could provide a powerful tool to identify individuals with high risk of adverse cardiovascular outcomes and guide individualized managements. Multi-dimensional echocardiographic parameters of left ventricular function derived from conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography were obtained in 266 HCM patients and 169 healthy controls (HCs).
View Article and Find Full Text PDFJACC Case Rep
March 2025
Klinik für Thorax- und Kardiovaskularchirurgie, Herz-Diabetes-Zentrum Nordrhein-Westphalen, Bad Oeynhausen, Germany.
The case concerns a 20-year-old patient with Canadian Cardiovascular Society class II angina who was initially referred for aortic valve replacement because of a suspected high-grade aortic valve stenosis with increased transvalvular gradients (max/mean: 70/40 mm Hg) measured by Doppler echocardiography. Examinations using transesophageal echocardiography and computed tomography showed a sufficiently opening bicuspid aortic valve, excluded supra- and subvalvular stenoses, and measured a narrow aorta (diameter: 2 cm). The explanation for the highly increased gradients across the aortic valve was the pressure recovery (PR) phenomenon, which cannot be detected by Doppler gradients.
View Article and Find Full Text PDFMultimed Man Cardiothorac Surg
March 2025
Northwell Cardiovascular Institute, New York, NY, USA.
This 27-year-old female patient is undergoing a reoperation for a recurrent subaortic membrane causing significant left ventricular outflow obstruction. The re-sternotomy is uneventful and cardiopulmonary bypass is established. The left ventricular outflow tract is accessed through the previously implanted semi-calcified homograft aortic root.
View Article and Find Full Text PDFEur Heart J Case Rep
March 2025
Department of Cardiovascular Surgery, Ayase Heart Hospital, 2-16-7 Yanaka, Adachi-Ku, Tokyo 120-0006, Japan.
Background: We report a rare case of trans-catheter aortic valve implantation (TAVI) in an elderly male with membranous ventricular septal defect (VSD), sub-aortic band, and severe aortic stenosis (AS). We discuss the safety and efficacy of the technique.
Case Summary: An 86-year-old male was admitted to our hospital with congestive heart failure due to low-flow low-gradient severe AS, a membranous VSD, a sub-aortic band, and a double-chambered right ventricle (RV).
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