Heparan sulfate storage in the cardiac conduction system triggers atrioventricular block

• Published in: Brain & development (Tokyo. 1979) Vol. 39; no. 5; pp. 418 - 421
• Main Authors: Kato, Rie, Miyahara, Hiroaki, Kawano, Tatsuya, Matsuzuka, Atsuko, Noda, Kimiko, Izumi, Tatsuro
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.05.2017
• Subjects: Adult; Atrioventricular block; Atrioventricular Block - etiology; Atrioventricular Block - pathology; Cardiac conduction system; Electrocardiography; Electroencephalography; Heart Conduction System - physiopathology; Heparan sulfate; Heparitin Sulfate - metabolism; Humans; Long-surviving patient; Longitudinal Studies; Male; Myocytes, Cardiac - metabolism; Neurology; Sanfilippo syndrome; Heparan sulfate; Cardiac conduction system; Long-surviving patient; Sanfilippo syndrome; Atrioventricular block
• ISSN: 0387-7604; 1872-7131
• DOI: 10.1016/j.braindev.2016.12.008

Abstract Objective To elucidate the novel biological functions of heparan sulfate (HS) by clinic-pathologically studying a patient with paroxysmal atrioventricular (AV) block. Patient A long-surviving male patient with Sanfilippo syndrome type A presented with paroxysmal AV block at age 33 years. He then survived another 2.5 years after the onset of paroxysmal AV block and pacemaker implantation. Methods and results His cardiac histopathological examination at autopsy showed HS storage in the cardiac conduction system (CCS), especially in the atrioventricular node (AVN)-His bundle branches. Conclusion HS storage in the CCS might trigger AV block, arising from below the AVN-His bundle branches. This is the first description to indicate that HS might be an essential constituent of life-long CCS plasticity and that its storage in the CCS results in AV block.