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New coronary congenital diseases classification aids identification of secondary defects

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A scheme outlined in a novel European Society of Cardiology (ESC) position paper has revealed that a new classification of coronary congenital diseases is set to aid surgeons identify secondary defects in the operating theater. The details of the same were published recently in Cardiovascular Research. This will also help clinical cardiologists to know what to look for on cardiovascular images. The stressful environment in the operating theater can make it difficult for doctors to spot further defects.

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Professor Cristina Basso, chairperson of the ESC Development, Anatomy and Pathology Working Group said that while coronary congenital diseases affect less than 1% of newborns but they are an important cause of myocardial infarction and sudden death particularly in children and competitive athletes. As atherosclerotic coronary artery disease is more common, these coronary congenital conditions are often forgotten in the clinical setting. The heart muscles are nourished by the coronary arteries. If there is any disruption of coronary development during the development of an embryo, coronary congenital defects can occur. Such defects change the blood flow and can adversely affect cardiovascular health.

In the new classification of coronary artery anomalies launched by the paper common points of origin between various coronary defects are explained. If the origin of a primary defect is identified, doctors can evaluate the probability of finding specific secondary defects with an origin mechanistically related to the main anomaly. Professor Basso opined that the paper contains practical information to help clinicians diagnose coronary artery anomalies and prevent devastating complications which includes sudden death.

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Dr. José María Pérez-Pomares, first author of the paper said that they have established links between coronary congenital diseases sharing a common mechanism.  He added that when surgeons operate on a certain diagnosed coronary anomaly, it can be often difficult for them to spot further defects in the stressful environment of the operating theatre. So, it can be of significant help to have an idea of the anomalies one can. Another benefit of the new classification is that it will help clinicians using imaging to diagnose coronary artery anomalies and prevent future complications.

Dr. Pérez-Pomares is of the opinion that they have been able to produce this classification because they understand more about how coronary arteries develop in the embryo and how they relate to major diseases. Cardiovascular research scientists can avail of new, updated information on the complex embryonic development of coronary arteries through this classification. It will also throw light on the aetiology of coronary congenital anomalies.

Even though the embryonic origin of the coronary endothelium is a controversial topic in cardiovascular developmental biology, the authors give their expert opinion on it. Since long scientists have wanted to identify a single and unique source for coronary endothelial cells but it is now known that they come from different sources that merge together explained Dr. Pérez-Pomares. It seems that has an impact on what happens to coronary vessels during embryonic development and perhaps also in the adult. Even though it is not mentioned clearly in the paper, the authors seems to be of the opinion that since the origin of cells in the endothelium is heterogeneous, it may play a role in the development of adult coronary disease, as endothelial cells with different origins might respond differentially to pathological stimuli.

The embryonic development of the coronary vascular system is mosaic-like which reflects in the complex spectrum of coronary artery anomalies. Dr. Pérez-Pomares remarked that the more they dig in, the more they have the impression that important events happening in the embryo, such as the activation of regulatory gene networks, signalling molecular pathways and specific cellular mechanisms do have a clear function in adult responses to pathological stimuli.

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References:

https://medicalxpress.com/news/2016-01-coronary-congenital-disease-classification-aids.html

https://www.sciencedaily.com/releases/2016/01/160111092409.htm