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Fig. 2.12 The rst historical description of myocardial bridge made in 1834 by Leopoldo and Floriano Caldani, Professors of Anatomy at the University of Padua
• Myocardial bridge with a short, not deep intramural course of a coronary artery branch (mostly descending coronary artery), observed in 30% of individuals [33–38] (Fig.2.12).
• Separate origin of the left anterior descend­ing and left circumex arteries, in the absence of left coronary trunk [33, 39].
• Double left anterior descending coronary artery [2, 33] (Fig.2.13).
• Left anterior descending coronary artery taking origin from the right coronary artery and crossing the pulmonary infun­dibular, at risk during surgical infundibu­lotomy [39].
S. Rizzo et al.
(b) Uncertain morbid signicance
• High take off-ostium located >2.5 mm from the sino-tubular junction [40, 41] (Fig.2.14)
• Origin of the right coronary artery from the left sinus
• Single coronary artery (Fig.2.15)
• Valve-like ridge in front of a coronary ostium [42] (Fig.2.16)
Fig. 2.13 Double left anterior descending coronary artery
2 Congenital Anomalies ofCoronary Arteries: Anatomy, Embryology andRisk ofSudden Death
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• Short and supercial myocardial bridge in HCM [43–47] (Fig.2.17)
• Congenital coronary aneurysm
(c) Anomalies at risk of sudden death (as sole
explanation of fatal outcome)
• Origin from the pulmonary artery [48, 49] (Fig.2.18)
• Origin of the left coronary artery from wrong right aortic sinus [50–55] (Fig.2.19)
Fig. 2.14 High take off
27
a
b
c
Fig. 2.15 Single right coronary artery. (a) Drawing of gross picture (b) and (c). AO aorta, PA pulmonary trunc, RA right atrium, SCV superior caval vein
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S. Rizzo et al.
ba
Fig. 2.16 Valve-like ridge obstructing the left coronary ostium. (a) gross view; (b) histology, Azan-Mallory stain
a
b
c
Fig. 2.17 The heart of a boy with Hypertrophic Cardiomyopathy. A cross-section of the heart (a) remark­able asymmetric septal hypertrophy (25-mm septal thick­ness vs 7-mm thickness of the left ventricular free wall); in the center is a dark red area. (b) the anterior view of the heart. The proximal middle tract of the left anterior
descending coronary artery show a deep and long intra­mural course. The region of the rst and second septal perforators corresponds to the location of the myocardial bridging. A panoramic histologic view (c) shows coagula­tion and reperfusion necrosis of the septal myocardium (trichrome–Heidenhain stain). From Gori F etal. [47]
2 Congenital Anomalies ofCoronary Arteries: Anatomy, Embryology andRisk ofSudden Death
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Fig. 2.18 Origin of the left coronary artery from the pul­monary artery. From Thiene, G., Corrado, D., Basso, C. (2016). Coronary Artery Disease. In: Sudden Cardiac
• Myocardial bridge, deep >5 mm, long >2.5cm with a myocardial circular sleeve with disarray [56, 57] (Fig.2.20)
• Left circumex from right sinus coronary artery, with retro-aortic course [56, 58,
59] (Fig.2.21)
Death in the Young and Athletes. Springer, Milano. https://
doi.org/10.1007/978- 88- 470- 5776- 0_3
• Coronary ostia sequestration [60] (Fig.2.22).
• Coronary stula
30
Rt cor
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Aortic valve
S. Rizzo et al.
Circ
Pulmonic valve
Fig. 2.19 Origin of the left coronary artery from the wrong right aortic sinus. From Thiene, G., Corrado, D., Basso, C. (2016). Coronary Artery Disease. In: Sudden
Cardiac Death in the Young and Athletes. Springer, Milano. https://doi.org/10.1007/978- 88- 470- 5776- 0_3
ab
2 Congenital Anomalies ofCoronary Arteries: Anatomy, Embryology andRisk ofSudden Death
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a b
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Fig. 2.20 Myocardial bridge with deep intramural course: the coronary segment is completely surrounded by a myocardial sleeve. Gross (a) and histological views (b). Azan Mallory stain. From Thiene, G., Corrado, D., Basso,
LAD
LCX
RCA
LCX
LAD
RCA
Fig. 2.21 LCx from RCA (a) Diagram with origin of the left circumex artery from the right coronary artery and retro-aortic course. From Roberts [39]. (b) Gross view of the aortic root. Arrow indicates the retro-aortic course of the anomalous left circumex artery. From Thiene, G.,
C. (2016). Coronary Artery Disease. In: Sudden Cardiac Death in the Young and Athletes. Springer, Milano. https://
doi.org/10.1007/978- 88- 470- 5776- 0_3
Corrado, D., Basso, C. (2016). Coronary Artery Disease. In: Sudden Cardiac Death in the Young and Athletes. Springer, Milano. https://doi.
org/10.1007/978- 88- 470- 5776- 0_3
32
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Fig. 2.22 Coronary ostia sequestration in Williams syndrome
S. Rizzo et al.
Coronary Artery Anomalies andSudden Cardiac Death
Coronary artery anomalies account for a signi­cant burden of sudden cardiac deaths in the young and particularly in athletes [61–70]. Of 75 consecutive cases of sudden death in athletes, studied pathologically in the time interval 1981– 2014 in the Veneto Region, Italy, 12 (16%) (Fig.2.23) were ascribed to congenital malfor­mations of coronary arteries, either of origin or
course [71]. They represent the third morbid entity as the sole explanation of the fatal out­come, following arrhythmogenic cardiomyopa­thy (27%) and coronary atherosclerosis (24%). Clearly, their detection at pre-participation screening for competitive sports eligibility plays a fundamental role in sudden death prevention [72]. It represents a great challenge, since it requires clinical imaging, ECG (both 12 leads basal and stress test) having a scarce sensibility to raise the suspicion.
Sudden Cardiac Death in Athletes
aortic dissection
DC
2 Congenital Anomalies ofCoronary Arteries: Anatomy, Embryology andRisk ofSudden Death
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Veneto Region, Italy, 1981-2014 total n. 75
normal heart PE 10%
1%
cond system
M
1%
7%
1%
mvp
myocarditis
4%
3%
HCM
5%
CAD, ATH 24%
CCA 16%
A(RV)C
27%
33
Fig. 2.23 Causes of Sudden Cardiac Death in 75 ath­letes, Veneto Region Registry, Italy (1981–2014). Coronary artery anomalies accounted for 16% of cases. A(RV)C arrhythmogenic right ventricular cardiomyopa-
Causal Relationship Between Coronary Artery Anomalies andSCD
According to the autopsy guidelines for the study of SCD cases of the Association for European Cardiovascular Pathology [73, 74]:
1. Only the origin from the pulmonary trunk should be considered as a certain cause of SCD.
2. The origin of left coronary artery from the opposite wrong right sinus of Valsalva has been classied as a highly probable cause of SCD.
3. Other forms (right from left sinus, left circum­ex from right sinus and retro-aortic course, high take off, and myocardial bridging) are classied as uncertain cause.
4. If uncertain, in the absence of other structural abnormalities, molecular investigation together with family genetic study cascade should be carried to rule out pathogen muta­tions for channelopathies.
thy, CAD coronary artery disease, ATH atherosclerosis, CCA congenital coronary anomalies, DCM dilated cardio­myopathy, HCM hypertrophic cardiomyopathy, mpv mitral valve prolapse, PE pulmonary embolism
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