Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation.

Potential Role of Mechanical Stress in the Etiology of Pediatric Heart Disease: Septal Shear Stress in Subaortic Stenosis

https://www.jtcvs.org/article/S0022-5223(99)70286-2/pdf

Surgical treatment of subaortic stenosis: a seventeen-year experience.

The discovery of the first heart field (FHF) and the second heart field (SHF) led us to understand how cardiac lineages and structures arise during development. However, it remains unknown how they are specified. Here, we generate precardiac spheroids with pluripotent stem cells (PSCs) harboring GFP/RFP reporters under the control of FHF/SHF markers, respectively. GFP+ cells and RFP+ cells appear from two distinct areas and develop in a complementary fashion. Transcriptome analysis shows a high degree of similarities with embryonic FHF/SHF cells. Bmp and Wnt are among the most differentially regulated pathways, and gain- and loss-of-function studies reveal that Bmp specifies GFP+ cells and RFP+ cells via the Bmp/Smad pathway and Wnt signaling, respectively. FHF/SHF cells can be isolated without reporters by the surface protein Cxcr4. This study provides novel insights into understanding the specification of two cardiac origins, which can be leveraged for PSC-based modeling of heart field/chamber-specific disease.

/pdf/precardiac-organoids-form-two-heart-fields-via-bmp-wnt-y382lmccir.pdf

Precardiac organoids form two heart fields via Bmp/Wnt signaling.

Echocardiographic morphometry and geometry of the left ventricular outflow tract in fixed subaortic stenosis.

Rheologic genesis of discrete subvalvular aortic stenosis: A Doppler echocardiographic study

Hypoplasia of the right ventricle, unassociated with severe pulmonary or tricuspid valvar malformations, is a primary congenital abnormality in which the trabeculated sinus portion of the ventricle fails to develop. An atrial septal defect or a stretched foramen ovale serves as an escape valve. The clinical, hemodynamic, and angiocardiographic features of this rare type of cyanotic heart disease are described in two children and reviewed in 13 previously reported cases. The clinical spectrum varies considerably in accordance with the degree of hypoplasia. Severe forms of hypoplasia, usually presenting in infancy, closely resemble tricuspid or pulmonary atresia. The electrocardiogram in hypoplasia, which shows right atrial enlargement and absence of right ventricular electrical forces, may be indistinguishable from an electrocardiogram in atresia. Lesser degrees of hypoplasia should be differentiated from Ebstein's malformation and anomalies of the systemic venous return. Both our patients, who had only mi...

Luc Van der Hauwaert

Papers

Rheologic genesis of discrete subvalvular aortic stenosis: A Doppler echocardiographic study

Isolated Right Ventricular Hypoplasia

Transcatheter occlusion of high flow Blalock-Taussig shunts with a detachable balloon.

Transcatheter occlusion of high flow Blalock-Taussig shunts with a detachable balloon