Thelansis

 Hypoplastic Left Heart Syndrome (HLHS) is a multifaceted cardiac malformation, likely originating from numerous developmental errors during the initial stages of cardiogenesis. If not treated, it is invariably fatal. HLHS is characterized by an array of chest radiographic findings, including an enlarged cardiac silhouette (with a notably prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. In patients with HLHS, pulmonary venous blood enters the left atrium, but the atretic or stenotic mitral valve prevents the atrial systole from pushing blood into the hypoplastic left ventricle. HLHS predominantly affects males and is known to have a genetic predisposition in certain families. However, the complete genetic etiology remains elusive—approximately 12% of infants with HLHS present with associated extracardiac anomalies. Genetic disorders linked with HLHS include Turner syndrome, Holt-Oram syndrome, Smith-Lemli-Opitz syndr...

 Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably result from multiple developmental errors in the early stages of cardiogenesis and, if left untreated, invariably prove fatal. Various chest radiographic findings are seen in patients with HLHS, including an enlarged cardiac silhouette (notably a prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. In HLHS, pulmonary venous blood enters the left atrium, but atrial systole cannot push blood across the atretic or stenotic mitral valve into the hypoplastic left ventricle. Consequently, left atrial blood is shunted across interatrial communication. The interatrial defect can range from a highly restrictive pinhole-sized foramen ovale to a wide, nonrestrictive atrial septal defect. Most commonly, the foramen ovale restricts left-to-right shunting. This restricted flow, coupled with decreased flow through the mitral valve, c...

  Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably results from multiple developmental errors in the early stages of cardiogenesis and that, if left untreated, invariably prove fatal. A variety of chest radiographic findings is seen in patients with HLHS, including an enlarged cardiac silhouette (notably a prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. In HLHS, pulmonary venous blood enters the left atrium, but atrial systole cannot push blood across the atretic or stenotic mitral valve into the hypoplastic left ventricle. Consequently, left atrial blood is shunted across interatrial communication. The interatrial defect can range from a highly restrictive pinhole-sized foramen ovale to a wide, nonrestrictive atrial septal defect. Most commonly, the foramen ovale restricts left-to-right shunting. This restricted flow, coupled with decreased flow through the mitr...