No abstract

Examination. Weight 7,080 Gm., temperature 40 C., pulse 160 per minute, respiration 60 per minute, blood pressure 90/50 mm. Hg in the armis and 110/60 mmii. Hg in the legs. He was pale, lethargic, hypoactive, and in moderate respiratory distress with retraction of the lower intercostal spaces. The left precordiunm bulged slightly. The heart was enlarged to the anterior axillary line in the sixth intercostal space. A systolic thrill was felt in the suprasternal notch and in the second intercostal space along the right sternal border. A harsh, ejection-type, systolic murmur was heard at the base of the heart, equally well both to the right and to the left of the sternum, and at the left lower sternal border. It was heard faintly over the neck vessels and the lung fields posteriorly. Diastole was clear except for a gallop

SUMMARYThe myocardium, all parts of the conduction system, and the S-A node contained iron in six patients with acquired iron-storage disease. [14][15][16][17][18] A clinicopathological study was undertaken by James'9 to investigate the changes present in the myocardium and conduction system in the hearts of five patients with hemochromatosis. Two had exogenous, and three had endogenous, hemochromatosis. He found extensive iron deposits in the myocardium and in the atrioventricular (A-V) node of each heart, but noted that the sinoatrial (S-A) node was spared in all cases. He postulated that the large amount of iron within the A-V node was responsible for the cellular necrosis and degeneration and that these changes could account for the antemortem conduction disturbances and arrhythmias Concerning the absence of iron in the S-A node, he suggested that a difference in local tissue metabolism resulted in the lack of iron deposition.The purpose of this paper is to report findings from a study of the conduction system and myocardium from the hearts of six young patients with acquired iron-storage disease. The patients studied had no history or anatomic evidence of primary arteriosclerotic, rheumatic, or hypertensive cardiovascular disease. Particular attention was directed to iron deposits and fibrous tissue in the S-A node, A-V node, A-V bundle, right bundle branch (RBB), left bundle branch (LBB), Purkinje fibers, autonomic nerve bundles and ganglia, and in the myocardium. We observed the relationship between the degree of iron deposition and the extent of scarring in these structures. Finally, we attempted to correlate the estimated transfusional intake of iron, the quantitatively analyzed myocardial iron content, and the severity of microscopic iron deposits in the conduction system with the frequency and severity of antemortem arrhythmias and conduction defects. MethodsFour hearts from patients with thalassemia major (cases 1 to 4) and two from patients with chronic, refractory, aregenerative anemia (cases 5 and 6) were studied both in the gross and microscopically. The microscopic changes in the myocardium and in the specialized tissues of the conduction system were studied in detail. The conduction system was dissected from the heart according to the methods described by Hudson20 and Lev and associates. 21 Step sections of the S-A node, A-V node, bundle of His, and the right and left bundle branches from each heart were made. Sections from the moderator band were taken to demonstrate Purkinje fibers22; the posterior walls of the right atria were sectioned to demonstrate autonomic nerves and ganglia.23 The sections were stained with hematoxylin and eosin, Perls' iron, and the Masson trichrome stains.All sections were evaluated with respect to the amount of iron deposited in these regions and in the myocardium. Sections of the S-A node, A-V node, bundle of His, and myocardium were evaluated with respect to the amount of scarring. The iron deposits and the extent of scarring were each described as absent, light...