PMC:6580293 / 9447-14758 JSONTXT

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identified 211 hearts within the archive having tetralogy as currently defined. After exclusions, 164 (77.7%) specimens were retained for the current study, of which 69 (42.1%) were classified as unrepaired, whereas 95 (57.9%) had undergone intracardiac surgical procedures. The retained hearts had been added to the collection between 1939 and 1994 and weighed from 3.80 g to 319.80 g [median 61.1 g; interquartile range (IQR) 26.4–104.6]. Donors were mostly male (88, 58.3%) and ranged from birth to 17 years at the time of death.\n\nCoronary arteries\nMultiple anomalies were observed in the orifices, origin and course of the coronary arteries, as shown in Table 1. The aortic root was accessible in 154 hearts, and while most of these specimens had 2 coronary arterial orifices (131, 85.1%), 20 (13.0%) hearts had more than 2 orifices, whereas 3 (1.9%) had a single coronary orifice. Of the overall number, 61 orifices originated at or above the sinutubular junction, with 49 of these positioned at or above the junction of the left coronary sinus and 12 from above the right coronary sinus (Fig. 2). In 7 (4.3%) hearts, a coronary artery was seen to cross the right ventricular outflow tract; this was the anterior interventricular artery in 6 (3.7%) and the right coronary artery in 1 (0.6%) (Fig. 2).\nFigure 2: Variation in the orifice position and the course of anomalous coronary arteries crossing the right ventricular outflow tract. (A) The high location of the right and left coronary orifices above the sinutubular junction (yellow dashed line). (B) An anterior interventricular artery originating from the right coronary artery and coursing towards the apex. (C) An operated heart, in which the anterior interventricular artery has been dissected, again originating from the right coronary artery, repaired using the 2-patch technique. (D) The aortic root lies to the left of the pulmonary trunk and the right coronary artery crosses the right ventricular outflow tract to achieve its anticipated position in the right atrioventricular groove; a patch has been placed beneath the right coronary artery to avoid incising it.\nTable 1: Variation in the number of coronary arterial orifices, position and anomalous coronary arteries seen crossing the right ventricular outflow tract\nAnatomical variant n (%)\nNumber of coronary orifices 154\n 1 3 (1.9)\n 2 131 (85.1)\n 3 19 (12.3)\n 4 1 (0.6)\nHigh coronary orifice position 154\n Left coronary orifice at STJ 21 (13.6)\n Right coronary orifice at STJ 0\n Left coronary orifice above STJ 28 (18.2)\n Right coronary orifice above STJ 12 (7.8)\nAnomalous coronary artery crossing RVOT 164\n Anterior interventricular artery 6 (3.7)\n Right coronary artery 1 (0.6)\nRVOT: right ventricular outflow tract; STJ: sinutubular junction.\n\nRelative position of the great arteries\nThe position of the aortic valve in relation to the pulmonary valve is illustrated in Fig. 3. In most (99, 61.9%) hearts, the aortic valve was located posterior and to the right of the pulmonary valve. The aortic valve was positioned side-by-side to the right of the pulmonary valve in 56 (35.0%) hearts, anterior and to the right in 2 (1.3%), directly posterior in 2 (1.3%) and side-by-side to the left in 1 (0.6%).\nFigure 3: Variation in the position of the aortic root (red) with reference to the pulmonary root (blue).\n\nAortic override\nThe extent of aortic override of the right ventricle ranged from 31.0% to 100% (median 59.5%, IQR 52.7–65.5) using our linear method (Fig. 4) and 33.3% to 100% (median 58.3%, IQR 50.0–66.7) with the leaflet proportion method. Comparisons of the 3 methods for determining aortic override are shown in Fig. 5. There was no difference between the linear and leaflet proportion methods (P = 0.22), but the qualitative method significantly underestimated the degree of override compared with both the linear and leaflet proportion methods (both P \u003c 0.001) and had much greater variability.\nFigure 4: Histogram demonstrating the range of aortic override using the linear method.\nFigure 5: Bland–Altman plots and histograms of difference for measurements of the degree of aortic override comparing (A) leaflet proportion versus linear methods, (B) leaflet proportion versus qualitative methods and (C) linear versus qualitative methods.\n\nVentricular septal defects\nThe differing morphology of the ventricular septal defects is illustrated in Fig. 6. Most defects were perimembranous (139, 84.8%), with 3 (2.2%) of these accompanied by an additional muscular ventricular septal defect. In 14 (8.5%) hearts, there was an isolated muscular outlet defect, produced by a muscular posteroinferior rim interposing between the leaflets of the aortic and tricuspid valves. Of the remaining hearts, in 7 (4.3%), the defect was the ventricular component of an atrioventricular septal defect, whereas in 2 (1.2%), the defect was doubly committed and juxta-arterial, due to fibrous continuity between the leaflets of the aortic and pulmonary valves.\nFigure 6: Morphology of the various types of ventricular septal defect, as seen from the right ventricle: perimembranous (A), muscular posteroinferior rim (B), doubly committed and juxta-arterial (C) and atrioventricular septal defect (D). The yellow lines show the septomarginal trabeculation."}