Sinus Bradycardia, Wolff Parkinson White, and Left Ventricular Noncompaction

• DOI: 10.1007/s00246-007-9043-9
• Publication Date: 2007-08-31T03:28:07Z
• Authors: Jack C. Salerno; Terrence U. Chun; Joe C. Rutledge
• Keywords: Left ventricular noncompaction; Abnormality; Sinus bradycardia; Sick sinus syndrome; Sinus (botany)

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.