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There is increasing recognition of the crucial role of the right ventricle (RV) in determining functional status and prognosis in multiple conditions. The normal RV is anatomically and functionally different from the left ventricle, which precludes direct extrapolation of our knowledge of left-sided physiopathology to the right heart. RV adaptation is largely determined by the level of exposure to hemodynamic overload (both preload and afterload) as well as its intrinsic contractile function. These 3 processes (pressure overload, volume overload, and RV cardiomyopathy) are associated with distinct clinical course and therapeutic approach, although in reality they often coexist in various degrees. The close relationship between the RV and left ventricle (ventricular interdependence) and its coupling to the pulmonary circulation further modulate RV behavior in different clinical scenarios. In this review, the authors summarize current knowledge of RV anatomic, structural, metabolic, functional, and hemodynamic characteristics in both health and disease.
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http://dx.doi.org/10.1016/j.jacc.2018.12.076 | DOI Listing |
JACC Case Rep
September 2025
Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois, USA; Northwestern Feinberg School of Medicine, Chicago, Illinois, USA.
Background: Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) with musculoskeletal, craniofacial, and cardiovascular features with a prevalence of approximately 1:50,000. Morbidity and mortality often occur earlier in patients with LDS compared to patients with other CTDs.
Case Summary: We present a teenager with subacute heart failure, 4/6 holosystolic murmur with diastolic rumble, facial differences, and arachnodactyly.
Circ Cardiovasc Interv
September 2025
Department of Biomedical Sciences, Humanitas University, Pieve Emanuele-Milan, Italy (F.T., G.A., M.G., K.S., D.D., G.S., M.C.).
Mitral regurgitation is the most common valve disease worldwide. Despite its wide success in inoperable or high-risk surgical patients, transcatheter edge-to-edge repair remains limited by some anatomic features and the not negligible rate of significant residual regurgitation. Transcatheter mitral valve replacement has emerged as a viable alternative that promises to overcome these issues, but its development has been progressing slowly.
View Article and Find Full Text PDFEClinicalMedicine
October 2025
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, China.
Background: Paediatric patients who underwent surgery for mitral regurgitation (MR) have a high risk of recurrence or death; however, no prediction tool has been developed to risk-stratify this challenging subpopulation.
Methods: In this multicentre cohort study, paediatric patients undergoing surgery for congenital MR in Shanghai Children's Medical Center in January 1st, 2009-December 31st, 2022 were included for analysis while those had a combination with infective endocarditis, anomalous left coronary artery from the pulmonary artery, rheumatic valvular disease, connective tissue disease, or single ventricle were excluded. A Cox regression model predictive of the primary outcome (a composite of mortality or mitral valve [MV] re-operation) was derived and converted to a point-based risk score.
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.
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