Sinus bradycardia, Wolff Parkinson White, and left ventricular noncompaction: an embryologic connection?
Left ventricular noncompaction (LVNC) is an uncommon disorder that has recently been recognized as a distinct cardiomyopathy. LVNC is thought to result from an arrest in the normal process of myocardial compaction. The association of Wolff-Parkinson-White with noncompaction of the left ventricle is well recognized. Sinus bradycardia has also been associated with LVNC, although less frequently than that of Wolff-Parkinson-White. We report an infant with LVNC, Wolff-Parkinson-White, and progressive sinus bradycardia who had a myocardial vascular abnormality in the region of the sinus node evident on autopsy. We propose that the progressive nature of the conduction system abnormality was as a result of abnormal angiogenesis.
Salerno
Jack C
JC
Department of Pediatrics, Children's Hospital and Regional Medical Center and University of Washington School of Medicine, Seattle, WA 98105, USA. jack.salerno@seattlechildrens.org
Chun
Terrence U
TU
Rutledge
Joe C
JC
D001344:Autopsy; D001919:Bradycardia / Q000196:embryology*; D009202:Cardiomyopathies / Q000151:congenital / Q000473:pathology*; D017809:Fatal Outcome; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008297:Male; D018487:Ventricular Dysfunction, Left / Q000151:congenital / Q000196:embryology*; D014927:Wolff-Parkinson-White Syndrome / Q000196:embryology*