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Cardiac catheterization for the adult with complex congenital heart disease
Published in Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead, Cardiovascular Catheterization and Intervention, 2017
Dextro-transposition of the great arteries (D-TGA) is a malalignment of the great vessels with the pulmonary artery arising from the morphologic left ventricle (LV) and the aorta arising from the morphologic RV. It causes cya- notic congenital heart disease. Currently, all newborns and many teenagers have been treated for this with the arterial switch operation, the Jatene procedure, described by Dr. Adib Jatene. [23] Unfortunately, most young adults were pre- viously treated with an atrial rather than arterial switch, such as a Mustard or Senning operation, depending on if the atrial baffle was made of pericardial tissue or Dacron material. [24] The main hemodynamic concerns in adulthood are the function of the RV as the systemic ventricle (it is unable to support such increased workload); baffle leaks predominately within the connection of the SVC baffle to the RA, resulting in a right-to-left shunt; systemic hypox- emia or systemic embolic events; and baffle obstruction resulting in SVC syndrome or enlargement of the azygous vein as it “pops” off to the IVC and arrhythmias, especially sick sinus syndrome and the need for a pacemaker inser- tion. Diagnostic catheterization is primarily reserved for hemodynamic interpretation of the failing systemic RV to obtain an end diastolic pressure, filling pressures, and pul- monary vascular resistance. These data are critical prior to proceeding for possible heart transplantation.
Deficiency of the Wnt receptor Ryk causes multiple cardiac and outflow tract defects
Published in Growth Factors, 2018
Kumudhini Kugathasan, Michael M. Halford, Peter G. Farlie, Damien Bates, Darrin P. Smith, You Fang Zhang, James P. Roy, Maria L. Macheda, Dong Zhang, James L. Wilkinson, Margaret L. Kirby, Donald F. Newgreen, Steven A. Stacker
Dextro-transposition of the great arteries (dTGA; a dextroventrally positioned aorta and ventriculoarterial discordance) with parallel alignment of the aorta and PT was observed in 22% of E18.5 Ryk−/− mice (Figure 3(I–L); Table 1). All but one of the Ryk−/− embryos with dTGA also had a subpulmonary membranous VSD (8 of 9). The arch of the aorta was observed at a more cranial level relative to the heart in E12.5 Ryk−/− mice with DORV and dTGA (Figure 3(H,L); 9 of 11) compared to littermates (Figure 3(D)). Isolated subpulmonary membranous VSD was observed in a small proportion of E18.5 Ryk−/− mice (11%; Table 1).