Pulmonary Atresia With Ventriculocoronary Arterial Connections and a Large Conoventricular Septal Defect

Jakamy, Réda; Séguéla, Pierre‐Emmanuel; Valdeolmillos, Estíbaliz; Kara, Mansour Mostefa; Mouton, Jean-Baptiste; Iriart, Xavier; Thambo, Jean-Benoît

doi:10.1016/j.jaccas.2019.09.008

Cited by 2 publications

(3 citation statements)

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“…In the case of coronary sinusoids, the coronary arteries form abnormal connections with the intertrabecular spaces of the ventricle, effectively connecting the epicardial coronary vessels with the ventricular lumen through defects in the myocardial architecture [ 29 ]. These defects have been mainly associated with PA/IVS and HLHS AA/MS subtype, although they can also be present in other CHD [ 30 ]. Coronary sinusoids have been hypothesized to arise from two distinct mechanisms.…”

Section: Discussionmentioning

confidence: 99%

Possible Association of Pulmonary Atresia with In-Utero Coxsackievirus B Exposure

et al. 2022

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Gestational viral infection has been associated with congenital heart disease (CHD). Few studies, however, have studied the potential role of gestational Coxsackievirus B (CVB) exposure in the pathogenesis of CHD. We prospectively enrolled women with pregnancies affected by CHD to explore possible associations with in utero CVB exposure. Serum samples were obtained from 122 women referred for fetal echocardiography between 2006 and 2018. We quantified CVB IgG and IgM levels, with titers ≥ 15.0 U/mL considered positive and measured neutralizing antibodies for three CVB serotypes: CVB1, CVB3, and CVB4. Using data from the national enterovirus surveillance system, we compared the annual exposure rates for each serotype in our cohort to infections reported across the United States. 98 pregnancies with no genetic defects were included. Overall, 29.6% (29/98) had positive IgG and 4.1% (4/98) of women had positive CVB IgM titers. To explore first-trimester CVB exposure, we focused exclusively on the 26 women with positive IgG and negative IgM titers. 61.5% (16/26) had neutralizing antibodies against a single serotype and 38.5% (10/26) against multiple CVB serotypes. CVB4 neutralizing antibodies were the most common (65.4%, 17/26), followed by CVB3 (53.9%, 14/26) and CVB1 (30.8%, 8/26). Among these, 30.8% of babies presented pulmonary valve anomalies: 19.2% (5/26) pulmonary atresia, and 11.5% (3/26) pulmonary stenosis. 23.1% (6/26) of babies had coronary sinusoids. CVB exposure in our cohort mirrored that of reported infections in the United States. Our results suggest a possible association between gestational CVB exposure and specific CHD, particularly pulmonary valve anomalies and coronary sinusoids. Supplementary Information The online version contains supplementary material available at 10.1007/s00246-021-02805-9.

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Section: Discussionmentioning

confidence: 99%

Possible Association of Pulmonary Atresia with In-Utero Coxsackievirus B Exposure

et al. 2022

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“…Blood from the right ventricle flows directly to the coronary microvasculature and vasculature (right and left coronary arteries), which suffer from increased blood pressure conducted from the ventricle via coronary sinusoids. The coronary vasculature wall remodels under this increased pressure and the smooth muscle layer thickens to compensate (Jakamy et al, 2019) (Figure 7b).…”

Section: Congenital Heart Diseases and Coronary Microvasculaturementioning

confidence: 99%

“…The right ventricle is connected to the right (RCA) as well as to the left coronary artery (LCA). Scheme drawn by J. Kacvinský based on data from Gittenberger‐de Groot et al (2010), Ozyilmaz et al (2014), and Jakamy et al (2019).…”

Section: Congenital Heart Diseases and Coronary Microvasculaturementioning

confidence: 99%

Development and diseases of the coronary microvasculature and its communication with the myocardium

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Olejníčková

Naňka

et al. 2022

WIREs Mechanisms of Disease

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We review the current understanding of formation and development of the coronary microvasculature which supplies oxygen and nutrients to the heart myocardium and removes waste. We emphasize the close relationship, mutual development, and communication between microvasculature endothelial cells and surrounding cardiomyocytes. The first part of the review is focused on formation of microvasculature during embryonic development. We summarize knowledge about establishing the heart microvasculature density based on diffusion distance. Then signaling mechanisms which are involved in forming the microvasculature are discussed. This includes details of cardiomyocyte—endothelial cell interactions involving hypoxia, VEGF, NOTCH, angiopoietin, PDGF, and other signaling factors. The microvasculature is understudied due to difficulties in its visualization. Therefore, currently available imaging methods to delineate the coronary microvasculature in development and in adults are discussed. The second part of the review is dedicated to the importance of the coronary vasculature in disease. Coronary microvasculature pathologies are present in many congenital heart diseases (CHD), especially in pulmonary atresia, and worsen outcomes. In CHDs, where the development of the myocardium is impaired, microvasculature is also affected. In adult patients coronary microvascular disease is one of the main causes of sudden cardiac death, especially in women. Coronary microvasculature pathologies affect myocardial ischemia and vice versa; myocardial pathologies such as cardiomyopathies are closely connected with coronary microvasculature dysfunction. Microvasculature inflammation also worsens the outcomes of COVID‐19 disease. Our review stresses the importance of coronary microvasculature and provides an overview of its formation and signaling mechanisms and the importance of coronary vasculature pathologies in CHDs and adult diseases. This article is categorized under: Cardiovascular Diseases > Stem Cells and Development Congenital Diseases > Molecular and Cellular Physiology Cardiovascular Diseases > Molecular and Cellular Physiology

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Pulmonary Atresia With Ventriculocoronary Arterial Connections and a Large Conoventricular Septal Defect

Cited by 2 publications

References 9 publications

Possible Association of Pulmonary Atresia with In-Utero Coxsackievirus B Exposure

Possible Association of Pulmonary Atresia with In-Utero Coxsackievirus B Exposure

Development and diseases of the coronary microvasculature and its communication with the myocardium

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