scholarly journals Sudden Death in Congenital Heart Disease: The Role of the Autopsy in Determining the Actual Cause

2020 ◽  
Vol 7 (4) ◽  
pp. 58
Author(s):  
Mary N. Sheppard

Congenital heart defects (CHDs) have undergone a large change in epidemiology due to prenatal screening and improved outcomes with surgery and percutaneous procedures. In patients with complex CHD there is an increased risk of sudden cardiac death (SCD) and up to 11% of all SCDs in the young occur in people with CHD. It is essential for clinicians to be aware of the risk factors, and for all patients to be followed up in specialised centres. When an SCD occurs, it is important that an autopsy is done and for the pathologist to have an in-depth knowledge of the particular defect and the corrective surgical techniques employed, as well as any complications due to these procedures. Both pathologist and cardiologist should work closely together to explain the cause of death to the family. A terminal cardiac arrhythmia explains many of the SCD cases, often with underlying cardiac fibrosis due to previous procedures. SCD may also be the first presentation of CHD, so great care is required when examining such cases and referral for a detailed expert opinion is recommended in all CHD-SCD cases.

2010 ◽  
Vol 29 (5) ◽  
pp. 817-821 ◽  
Author(s):  
Reem S. Abu-Rustum ◽  
Linda Daou ◽  
Sameer E. Abu-Rustum

2021 ◽  
Vol 10 (2) ◽  
pp. 113-124
Author(s):  
D. V. Borisenko ◽  
A. A. Ivkin ◽  
D. L. Shukevich

Highlights. The article discusses the pathophysiological aspects of cardiopulmonary bypass and the mechanisms underlying the development of the systemic inflammatory response in children following congenital heart surgery. We summarize and report the most relevant preventive strategies aimed at reducing the systemic inflammatory response, including both, CPB-related methods and pharmacological ones.The growing number of children with congenital heart defects requires the development of more advanced technologies for their surgical treatment. However, cardiopulmonary bypass is required in almost all surgical techniques. Despite the tremendous progress and recent advances in cardiopulmonary bypass techniques, the systemic inflammatory response syndrome associated with these surgeries remains unresolved. The review summarizes the causes and mechanisms underlying its development. The most commonly used preventive strategies are reported, including standard and modified ultrafiltration, leukocyte filters, and pharmacological agents (systemic glucocorticoids, aprotinin, and antioxidants).The role of cardioplegia and hypothermia in the reduction of systemic inflammation is defined. Cardiac surgery centers around the world use a variety of techniques and pharmacological approaches, drawing on the results of randomized clinical studies. However, there are no clear and definite clinical guidelines aimed at reducing the systemic inflammatory response during cardiopulmonary bypass in children. It remains a significant problem for pediatric intensive care by aggravating their postoperative status, prolonging the length of the in-hospital stay, and reducing the survival rates.


PeerJ ◽  
2020 ◽  
Vol 8 ◽  
pp. e10502
Author(s):  
Huan Guo ◽  
Xinke Zhao ◽  
Haixiang Su ◽  
Chengxu Ma ◽  
Kai Liu ◽  
...  

Background Radiation exposure of the thorax is associated with a greatly increased risk of cardiac morbidity and mortality even after several decades of advancement in the field. Although many studies have demonstrated the damaging influence of ionizing radiation on cardiac fibroblast (CF) structure and function, myocardial fibrosis, the molecular mechanism behind this damage is not well understood. miR-21, a small microRNA, promotes the activation of CFs, leading to cardiac fibrosis. miR-21 is overexpressed after irradiation; however, the relationship between increased miR-21 and myocardial fibrosis after irradiation is unclear. This study was conducted to investigate gene expression after radiation-induced CF damage and the role of miR-21 in this process in rats. Methods We sequenced irradiated rat CFs and performed weighted correlation network analysis (WGCNA) combined with differentially expressed gene (DEG) analysis to observe the effect on the expression profile of CF genes after radiation. Results DEG analysis showed that the degree of gene changes increased with the radiation dose. WGCNA revealed three module eigengenes (MEs) associated with 8.5-Gy-radiation—the Yellow, Brown, Blue modules. The three module eigengenes were related to apoptosis, G2/M phase, and cell death and S phase, respectively. By blocking with the cardiac fibrosis miRNA miR-21, we found that miR-21 was associated with G2/M blockade in the cell cycle and was mainly involved in regulating extracellular matrix-related genes, including Grem1, Clu, Gdf15, Ccl7, and Cxcl1. Stem-loop quantitative real-time PCR was performed to verify the expression of these genes. Five genes showed higher expression after 8.5 Gy-radiation in CFs. The target genes of miR-21 predicted online were Gdf15 and Rsad2, which showed much higher expression after treatment with antagomir-miR-21 in 8.5-Gy-irradiated CFs. Thus, miR-21 may play the role of fibrosis and G2/M blockade in regulating Grem1, Clu, Gdf15, Ccl7, Cxcl1, and Rsad2 post-irradiation.


Author(s):  
Brian A. McCrossan ◽  
Frank A. Casey

Paediatric cardiology is a subspecialty ideally suited to telemedicine. A small number of experts cover large geographical areas and the diagnosis of congenital heart defects is largely dependent on the interpretation of medical imaging. Telemedicine has been applied to a number of areas within paediatric cardiology. However, its widespread uptake has been slow and fragmentary. In this chapter the authors examine the current evidence pertaining to telemedicine applied to paediatric cardiology, including their own experience, the importance of research and, in particular, economic evaluation in furthering telemedicine endeavours. Perhaps most importantly, they discuss the issues relating transitioning a pilot project into a sustainable clinical service.


Medicina ◽  
2008 ◽  
Vol 44 (2) ◽  
pp. 139
Author(s):  
Virginija Dulskienë ◽  
Vilija Malinauskienë ◽  
Ada Azaravièienë ◽  
Renata Kuèienë

The objective of this study was to determinate the incidence and diagnostics of congenital heart defects in Kaunas infant population in 1999–2005 and to compare the data obtained with the data of years 1995–1998. Subject and methods. The study population comprised all newborns born in Kaunas city during 1999– 2005. Congenital heart defects were registered based on clinical diagnosis after its verification using the data from consulting centers and pediatric outpatient departments. To assess the incidence of congenital heart malformations in newborn population, we conducted a validated newborn register based on maternal residential district. Modern epidemiological methods were used for data analysis. Results. In 1999–2005, there were 24 069 live births in Kaunas: 2231 newborns were born with congenital anomalies, 198 had congenital heart malformations. The incidence of congenital heart defects was 8.2 per 1000 live newborns. The majority of congenital heart malformations were diagnosed in delivery units (93.94%). We have analyzed the relationship between birth weight and gestational age of newborns with congenital heart malformations. Newborns with low birth weight were at significantly higher risk of congenital heart malformation than newborns with normal birth weight (OR=3.52, 95% CI, 2.25–5.47). Our data also showed that newborns born before 32 weeks of gestation had a 5-fold increased risk of congenital heart malformation (OR=5.20; 95% CI, 2.50–10.84) and infants born before 37 weeks of gestation had a 4-fold increased risk (OR=4.08; 95% CI, 2.68–6.19) compared with newborns born after 37 weeks of gestation. Conclusions. This study shows that incidence of congenital heart anomalies in Kaunas newborn population was 8.2 cases per 1000 live newborns in 1999–2005. It was determined that during 1999–2005, the number of above-mentioned anomalies diagnosed in delivery units increased by 23%.


2008 ◽  
Vol 29 (11) ◽  
pp. 1424-1431 ◽  
Author(s):  
L. M.J.W. van Driel ◽  
H. P.M. Smedts ◽  
W. A. Helbing ◽  
A. Isaacs ◽  
J. Lindemans ◽  
...  

2018 ◽  
Vol 482 ◽  
pp. 185-190
Author(s):  
Lizhu Chen ◽  
Johnny Guan ◽  
Qiuju Wei ◽  
Zhengwei Yuan ◽  
Mo Zhang

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