Background: Adults with congenital heart disease and ventricular dysfunction are prone to liver congestion, leading to fibrosis or cirrhosis but little is known about the prevalence of liver disease in atrial switch patients. Liver impairment may develop due to increased systemic venous pressures. This prospective study aimed to assess non-invasively hepatic abnormalities in adults who underwent Senning or Mustard procedures.
Methods: Hepatic involvement was assessed non-invasively clinically by laboratory analysis, hepatic fibrotic markers, sonography, and liver stiffness measurements [transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)].
Results: Overall, 24 adults who had undergone atrial switch operation (13 Senning, 11 Mustard; four female; median age 27.8 years; range 24-45 years) were enrolled. In liver stiffness measurements, only three patients had values within the normal reference. All other patients showed mild, moderate or severe liver fibrosis or cirrhosis, respectively. Using imaging and laboratory analysis, 71% of the subjects had signs of liver fibrosis (46%) or cirrhosis (25%).
Conclusions: Non-invasive screening for liver congestion, fibrosis or cirrhosis could be meaningful in targeted screening for hepatic impairment in patients with TGA-ASO. As expert knowledge is essential, patients should be regularly controlled in highly specialised centres with cooperations between congenital cardiologists and hepatologists.
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http://dx.doi.org/10.21037/cdt.2019.07.10 | DOI Listing |
Cardiovasc Diagn Ther
December 2024
Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary.
Background: Dextro-transposition of the great arteries (dTGA) stands out as a prevalent cyanotic congenital heart defect (CHD), characterized by an intricate reversal in the arrangement of the major arteries. In the past, several surgical procedures have been used to treat dTGA, including the atrial switch. Although the method is no longer used, survivors of the procedure still living among us.
View Article and Find Full Text PDFAndes Pediatr
October 2024
Departamento de Cardiopatías Congénitas y Pediátricas, Fundación Cardiovascular de Colombia, Santander, Colombia.
Unlabelled: Transposition of the great arteries (Dextro-TGA), repaired with physiological correction techniques (atrial switch - Mustard or Senning surgery), can present as a complication the failure of the right ventricle that acts as systemic and, at the same time, deconditioning of the left ventricle, leading to congestive heart failure. In these patients, treatment and recovery options are very limited.
Objective: To describe successful late anatomical correction after ventricular retraining.
Int J Cardiol Congenit Heart Dis
March 2024
Service of Cardiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Since the late 1980s, the standard approach for treating D-transposition of the great arteries has been the arterial switch operation (ASO), replacing the Mustard/Senning procedure. Although ASO has shown impressive long-term survival rates, recent case series have revealed late complications such as neoaortic dilation and coronary artery stenosis. New findings emphasize the need for comprehensive evaluation of coronary risk and a deeper understanding of the mechanisms leading to coronary artery stenosis and myocardial ischemia over the long term.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
December 2024
Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium.
Background: The Mustard and Senning operations for dextro-transposition of the great arteries (D-TGA) establish a biventricular physiology with a subaortic right ventricle (sRV). While prolonged QRS has been associated with worse prognosis in these patients, current echocardiographic tools fall short in adequately assessing the (mal)performance and function decline of the sRV during follow-up. The present study is the first to characterize Myocardial Work (MW) indices of the sRV in D-TGA patients after Mustard/Senning repair.
View Article and Find Full Text PDFJ Clin Med
September 2024
Department of Clinical Physiology, Faculty of Medicine, Medical University of Lodz, Mazowiecka 6/8, 92-215 Lodz, Poland.
Congenitally corrected transposition of the great arteries (ccTGA) is an infrequent and complex congenital malformation, which accounts for approximately 0.5% of all congenital heart defects. This defect is characterized by both atrioventricular and ventriculoarterial discordance, with the right atrium connected to the morphological left ventricle (LV), ejecting blood into the pulmonary artery, while the left atrium is connected to the morphological right ventricle (RV), ejecting blood into the aorta.
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