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The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome

^a Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Department of Pediatrics, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
^c Department of Surgery, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA

View larger version (97K): [in a new window]   Figure 1 (A) Transthoracic echocardiographic image of type A atrial morphology. The distinction between septum primum (prim) and septum secundum (sec) can be seen. Arrows denote the decompression pathway from the left atrium (LA) to the innominate vein, right superior vena cava and right atrium (RA). This pathway is unobstructed. (B) Doppler flow in the ascending limb of the decompression shows low velocity, phasic flow confirming the absence of significant obstruction.

View larger version (93K): [in a new window]   Figure 2 Type B atrial morphology. The atrial septum is thickened without distinction between septum primum and secundum. The left atrium (LA) appears muscular. Arrows point to atrial septum. RA = right atrium.

View larger version (109K): [in a new window]   Figure 3 Fetal echocardiographic image of type C atrial morphology. The left atrial appendage and left atrium (LA) are markedly dilated secondary to severe mitral regurgitation. Arrows point to atrial septum. LV = left ventricle; RA = right atrium.

View larger version (133K): [in a new window]   Figure 4 Elastin stain of pulmonary veins (x400 reduced by 50%). (A) Control patient with hypoplastic left heart syndrome and an atrial septal defect measuring 3 to 4 mm. The pulmonary vein is thin. There is little elastin staining within the wall and no distinct elastic lamina noted. (B) Infant with type B atrial morphology and obstructed decompression pathway. The pulmonary vein is thick walled with multiple elastic laminae noted ("arterialization" of the pulmonary vein).