Hypoplastic coronary artery disease, as a cause of sudden death

Abstract

Hypoplastic coronary artery disease (HCAD) is a rare coronary artery anomaly that may be the cause of sudden death. It can involve a single or all coronary arteries. This anomaly may cause circulatory insufficiency leading to myocardial infarction. HCAD has no symptoms or may exhibit cardiovascular signs like syncope, dyspnea, chest discomfort, or dizziness. It is often diagnosed at autopsy, and early diagnosis is made with a coronary angiogram. We report HCAD as the cause of the sudden death of a 25-year-old female with a history of loss of consciousness following exertion. On autopsy, all the coronary arteries’ lumen was narrowed with thin vessel walls. Histopathological examination shows an underdeveloped and missing muscular layer of the left anterior descending and circumflex coronary arteries’ vascular wall. Many cases of HCAD diagnosed by radiographic imaging in living patients have been reported in the literature, but a structural anomaly of coronaries leading to HCAD has not been reported. We report a case of HCAD describing the histopathological examination findings of the vascular wall of coronary vessels illustrating the structural difference.

Hypoplastic and Congenital Absence of Coronary Arteries and Its Correlation with Clinical Implications of Cardiac Circulation: A Systematic Review and Meta-Analysis

“…Eighty-eight studies were excluded due to discrepancies between their primary and secondary results compared to those of this review, or because they failed to meet the established criteria for good data extraction. Therefore, 39 articles [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 ] were selected for this study ( n = 140,137) (including patients, images, and cadavers) ( Figure 1 ).…”

“…Of the 39 studies included in this review (as listed in Table 1 and Table 2 ), 19 articles are from Asia [ 11 , 12 , 13 , 19 , 20 , 21 , 23 , 27 , 33 , 35 , 36 , 37 , 39 , 41 , 43 , 45 , 46 , 47 , 48 ], 10 are from Europe [ 13 , 16 , 17 , 22 , 26 , 30 , 31 , 32 , 34 , 40 ], 8 are from North America [ 10 , 15 , 18 , 24 , 25 , 29 , 38 , 44 ], 1 is from South America [ 28 ], and 1 is from Oceania [ 42 ]. Therefore, the total number of subjects is 140,137, among whom 811 presented coronary artery variants, with an average age of diagnosis of 41.4 years.…”

“…The hypoplasia of the LMCA presented fewer incidences, being studied in only four studies with four subjects [ 14 , 17 , 25 , 35 ]. Hypoplastic left coronary artery (H-LMCA) refers to the underdevelopment of the LMCA, causing an insufficient blood supply to the heart that can or cannot be compensated by the right coronary system or the aberrant origin of the LCx or LAD.…”

Novel Anatomical Variation of Left Coronary Artery Origin at the Sinotubular Junction Level With Coeval Hypoplastic Left Circumflex, Superdominant Right Coronary Artery, and Obstructive Coronary Artery Disease: A Case Report

“…In the second case, a nine-year-old girl died when her LCx and LAD, which measured 0.8 mm in diameter (normal: 1.8 mm), showed microscopic evidence of ischemia associated with HCAD [21][22][23]. Sangita et al's latest study in 2023 [24] included the case of a female 25-year-old who had previously experienced loss of consciousness after exertion. Her autopsy and histology examinations revealed that the lumen of all coronary arteries was constricted, with thin vessel walls and an undeveloped and absent muscle layer in the vascular walls of the LAD and LCx arteries, respectively.…”

Casting Light on The Hidden Prevalence: A Novel Perspective on Hypoplastic Coronary Artery Disease

“…Coronary artery anatomy assessment remains a standard procedure for patients exhibiting a constellation of clinical manifestations and risk factors, with some variability owing to local protocols or availability of medical procedures. Another source of bias may be the method of assessment-post-mortem procedures versus coronary angiography versus CCTA [9,16]. There is data supporting better detection when using CCTA compared to other methods in detecting not only hypoplasia, but also other arterial anomalies.…”