Aortic outflow obstruction in visceral heterotaxy: A study based on twenty postmortem cases

• Published in: The American heart journal 1997-05, Vol.133 (5), p.558-569
• Main Authors: Van Praagh, Stella, Geva, Tal, Friedberg, David Z., Oechler, Herb, Colli, Anna, Frigiola, Alessandro, Van Praagh, Richard
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - pathology; Aortic Valve - abnormalities; Child; Child, Preschool; Female; Heart Defects, Congenital - pathology; Humans; Infant; Infant, Newborn; Male; Spleen - abnormalities; Syndrome; Viscera - abnormalities
• ISSN: 0002-8703; 1097-6744
• DOI: 10.1016/S0002-8703(97)70151-0

Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia ( n = 4) or polysplenia ( n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001). (Am Heart J 1997;133:558-68.)