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Coarctation of the aorta is a leading cause of morbidity and mortality among adults with congenital heart disease (ACHD). Lifelong surveillance is mandatory to screen for possible long-term cardiovascular events. Left ventricular systolic dysfunction has been reported in association with recoarctation, and association with dilated cardiomyopathy (DCMP) is very rare. Herein, we report the case of a 19-year-old boy with coarctation of the aorta who complained of mild exertional dyspnea. Cardiac magnetic resonance revealed a moderately dilated, hypokinetic left ventricle (LV), with mildly reduced EF (45%), and residual isthmic coarctation was excluded. Genetic tests revealed a heterozygous missense variant in TNNT2 (NM_001001430.2): c.518G>A (p. Arg173Gln). This case highlights the role of careful history taking: a family history of cardiomyopathy should not be overlooked even when the clinical setting seems to suggest a predisposition to hemodynamic factors for LVSD.
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http://dx.doi.org/10.3390/biom11050696 | DOI Listing |
Acad Radiol
September 2025
Department of Nuclear Medicine, National Taiwan University Hospital, Taipei, Taiwan (J.Y.H., C.L.K., K.L.C.); College of Medicine, National Taiwan University, Taipei, Taiwan (J.Y.H., C.K.H., K.L.C., Y.W.W.); Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan (C.K
Rationale And Objectives: The prognostic implications of myocardial perfusion imaging (MPI) are imperative to provide proper management of coronary artery disease (CAD). This study aimed to quantify the long-term prognostic value of MPI under routine clinical conditions.
Materials And Methods: This single-center retrospective cohort study evaluated all-cause mortality and cause-specific survival according to MPI findings in patients with suspected or known CAD who underwent diagnostic evaluation or assessment of myocardial ischemia and viability in a tertiary referral cardiovascular center.
Stem Cell Res
September 2025
Department of Cardiology, Affiliated Hospital of Jining Medical University, Shandong, China; Shandong Provincial Key Medical and Health Discipline of Cardiology Affiliated Hospital of Jining Medical University, Shandong, China; Key Laboratory of Cell and Biomedical Technology of Shandong Province, C
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary infiltrative cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium, which may extend to the left ventricle in the advanced stages. Clinically, the condition is commonly associated with right ventricular dilation, malignant arrhythmias, and an increased risk of sudden cardiac death. In this study, we successfully established induced pluripotent stem cell (iPSC) lines from peripheral blood mononuclear cells of ARVC patients carrying a heterozygous LMNA gene mutation (c.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
September 2025
Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea.
: This study aimed to compare the long-term outcomes of a modified réparation à l'étage ventriculaire (REV) and the Rastelli repair for ventricular septal defect (VSD) and pulmonary outflow tract obstruction without ventriculoarterial concordance. : The study included 100 consecutive patients who underwent a modified REV ( = 50) or Rastelli repair ( = 50) for transposition of the great arteries, double outlet right ventricle, or double outlet left ventricle with VSD and pulmonary outflow tract obstruction. The mean ages of the patients who underwent the modified REV and Rastelli repair were 2.
View Article and Find Full Text PDFEur Heart J Cardiovasc Imaging
September 2025
Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Venusberg-Campus 1, 53127, Bonn, Germany.
Aims: Fetal circulation undergoes complex changes in congenital heart disease (CHD) that are challenging to assess with fetal echocardiography. This study aimed to assess clinical feasibility and diagnostic value of 4D flow cardiac magnetic resonance (CMR) in fetal CHD.
Methods And Results: Pregnant women in advanced third trimester pregnancy with fetal CHD were prospectively recruited for fetal CMR between 08/2021 and 11/2024.
Am J Physiol Heart Circ Physiol
September 2025
Division of Pediatric Critical Care, Department of Pediatrics, University of California, San Francisco, USA.
Right ventricular (RV) failure is the primary cause of death among patients with pulmonary arterial hypertension (PAH). Patients with congenital heart disease-associated PAH (CHD-PAH) demonstrate improved outcomes compared to patients with other forms of PAH, which is related to the maintenance of an adaptively hypertrophied RV. In an ovine model of CHD-PAH, we aimed to elucidate the cellular, microvascular, and transcriptional adaptations to congenital pressure overload that support RV function.
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