De subitaneis mortibus. XXVII. Histological abnormalities in the sinus node, atrioventricular node and His bundle associated with coarctation of the aorta.

Abstract

A twenty-year-old carpenter died suddenly and unexpectedly nine years after surgical treatment of coarctation of the aorta. Both before and after surgery he had paroxysmal atrial fibrillation, T wave inversions in the ECG and persisting cardiac hypertrophy. At postmortem examination there was focal fibromuscular dysplasia narrowing the sinus node artery, but other small coronary arteries were normal and there was no focal fibrosis of the ventricular myocardium. Within the sinus node there were several small glomera surrounding branches of the sinus node artery. Pericardial fibrosis was present over much of the heart, including margins of the sinus node. The central fibrous body was thickened, particularly on the left, and the His bundle was smaller than normal in cross section. The His bundle appeared displaced toward the right. The artioventricular (A-V) node was split into an upper and lower half tenuously connected through the central fibrous body which divided it. In its lower half the A-V node was directly continuous with ordinary myocardial cells of the interventricular septum. Possible developmental relationships between these unusual anatomical findings in the conduction system and coarctation of the aorta are discussed. How these findings might relate to the known electrophysiological disturbances and some causes for his sudden death are considered.