Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
We report a case of 24-year-old man with single ventricle congenital heart disease and concomitant familial Fabry disease who developed progressive subaortic stenosis 22 years after Fontan procedure. Despite stable ventricular volumes, subaortic stenosis developed due to Fabry disease-related myocardial hypertrophy. He underwent pulmonary arterial trunk reconstruction with valved conduit and Damus-Kaye-Stansel (DKS) anastomosis. Postoperatively, smooth outflow tracts and stable valve function were observed. This case highlights the impact of Fabry disease on ventricular outflow tract obstruction and shows the efficacy of the DKS anastomosis in treating subaortic stenosis.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1177/02184923251356574 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Hypertrophic Cardiomyopathy: Mechanisms of Pathogenicity.
Biomed Environ Sci
August 2025
Department of Cardiology, Fuwai Central China Cardiovascular Hospital, Henan Provincial People's Hospital Heart Center, Zhengzhou 451464, Henan, China.
Hypertrophic cardiomyopathy (HCM) is a major contributor to cardiovascular diseases (CVD), the leading cause of death globally. HCM can precipitate heart failure (HF) by causing the cardiac tissue to weaken and stretch, thereby impairing its pumping efficiency. Moreover, HCM increases the risk of atrial fibrillation, which in turn elevates the likelihood of thrombus formation and stroke.
View Article and Find Full Text PDFSubaortic Membrane With a Patent Ductus Arteriosus in an Adult Patient: A Challenging Diagnostic Dilemma.
Cureus
August 2025
Cardiology, Faculty of Medicine and Pharmacy of Rabat, Mohammed V University, Rabat, MAR.
Subaortic membrane (SAM) is a subtype of left ventricular outflow obstruction, rarely seen in adults. In some cases, SAM may be associated with other congenital defects. The association of patent ductus arteriosus (PDA) and SAM is the rarest, especially in adult patients.
View Article and Find Full Text PDFMavacamten in Symptomatic Nonobstructive Hypertrophic Cardiomyopathy.
N Engl J Med
September 2025
Cleveland Clinic Coordinating Center for Clinical Research, Heart Vascular Thoracic Institute, Cleveland Clinic, Cleveland.
Background: Mavacamten is approved to treat adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM). However, its effects in nonobstructive HCM remain uncertain.
Methods: We conducted a phase 3, international, double-blind, placebo-controlled, clinical trial to determine whether mavacamten improves functional capacity and patient-reported health status among adults with symptomatic nonobstructive HCM.
Aficamten or Metoprolol Monotherapy for Obstructive Hypertrophic Cardiomyopathy.
N Engl J Med
September 2025
Massachusetts General Hospital, Harvard Medical School, Boston.
Background: Beta-blockers have been the initial treatment for symptomatic obstructive hypertrophic cardiomyopathy (HCM) despite limited evidence of their efficacy. Aficamten is a cardiac myosin inhibitor that reduces left ventricular outflow tract gradients, improves exercise capacity, and decreases HCM symptoms when added to standard medications. Whether aficamten as monotherapy provides greater clinical benefit than beta-blockers as monotherapy remains unknown.
View Article and Find Full Text PDFKnowledge, attitude, and practice toward hypertrophic cardiomyopathy of patients with primary hypertension: a cross-sectional study.
Sci Rep
August 2025
National Traditional Chinese Medicine Clinical Research Base, Affiliated Traditional Chinese Medicine Hospital, Southwest Medical University, Luzhou, 646000, China.
Primary hypertension plays a crucial role in cardiovascular health and is potentially associated with hypertrophic cardiomyopathy (HCM). This study aimed to investigate the knowledge, attitude, and practice (KAP) of patients with primary hypertension toward HCM. This cross-sectional study was conducted among patients with primary hypertension at the Hospital between November 2023 and December 2023, using a self-designed questionnaire.
View Article and Find Full Text PDF