scholarly journals Deep Phenotypic Analysis for Transposition of the Great Arteries and Prognosis Implication

Author(s):  
Huayan Shen ◽  
Qiyu He ◽  
Xinyang Shao ◽  
Shoujun Li ◽  
Zhou Zhou

Background Transposition of the great arteries (TGA) consists of about 3% of all congenital heart diseases and 20% of cyanotic congenital heart diseases. It is always accompanied by a series of other cardiac malformations that affect the surgical intervention strategy as well as prognosis. In this study, we comprehensively analyzed the phenotypes of the patients who had TGA with concordant atrioventricular and discordant ventriculoarterial connections and explored their association with prognosis. Methods and Results We retrospectively reviewed 666 patients with a diagnosis of TGA with concordant atrioventricular and discordant ventriculoarterial connections in Fuwai Hospital from 1997 to 2019. Under the guidance of the Human Phenotype Ontology database, patients were classified into 3 clusters. The Kaplan‐Meier method was used to analyze the prognosis, and the Cox proportional regression model was used to investigate the risk factors. In this 666‐patient TGA cohort, the overall 5‐year survival rate was 94.70% (92.95%–96.49%). Three clusters with distinct phenotypes were obtained by the Human Phenotype Ontology database. Kaplan‐Meier analysis revealed a significant difference in freedom from reintervention among 3 clusters ( P <0.001). To eliminate the effect of surgeries, we analyzed patients who only received an arterial switch operation and still found a significant difference in reintervention ( P =0.019). Conclusions We delineated a big cardiovascular phenotypic profile of an unprecedentedly large TGA cohort and successfully risk stratified them to reveal prognostic significance. Also, we reported the outcomes of a large TGA population in China.

2020 ◽  
Vol 63 (4) ◽  
pp. 151-156
Author(s):  
Hee Sun Baek ◽  
Youngok Lee ◽  
Hea Min Jang ◽  
Joonyong Cho ◽  
Myung Chul Hyun ◽  
...  

Background: Acute kidney injury (AKI) is one of the most significant postoperative complications of pediatric cardiac surgery. Because serum creatinine has limitations as a diagnostic marker of AKI, new biomarkers including neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and interleukin-18 (IL-18) are being evaluated to overcome these limitations and detect AKI at an early stage after cardiac surgery.Purpose: This study aimed to investigate the clinical usefulness of these biomarkers in young children.Methods: Thirty patients with congenital heart diseases who underwent cardiac surgery using cardiopulmonary bypass (CPB) were selected, and their urine and blood samples were collected at baseline and 6, 24, and 48 hours after surgery. Serum creatinine and blood urea nitrogen levels as well as NGAL, KIM-1, and IL-18 levels in urine samples were measured, and clinical parameters were evaluated.Results: Of the 30 patients, 12 developed AKI within 48 hours after cardiac surgery. In the AKI group, 8 of 12 (66.6%) met AKI criteria after 24 hours, and urine KIM-1/creatinine (Cr) level (with adjustment of urine creatinine) peaked at 24 hours with significant difference from baseline level. Additionally, urine KIM-1/Cr level in the AKI group was significantly higher than in the non-AKI group at 6 hours. However, urine NGAL/Cr and IL-18/Cr levels showed no specific trend with time for 48 hours after cardiac surgery.Conclusion: It is suggested that urine KIM-1/Cr concentration could be considered a good biomarker for early AKI prediction after open cardiac surgery using CPB in young children with congenital heart diseases.


2021 ◽  
Vol 3 (2) ◽  
pp. 10
Author(s):  
Amira S. E. Hussein ◽  
Wafaa E. A. Ouda ◽  
Randa M. Adly

Contents: Families of children with congenital heart disease face frequent healthcare encounters due to their child's care trajectory. With an emphasis on assuring caring in healthcare, it is necessary to understand parents’ perceptions of healthcare providers’ actions when their child undergoes heart surgery. Aim:  This study aimed to evaluate the effect of family-centered care intervention on maternal coping and participation in care for their children with congenital heart diseases. Methods: A quasi-experimental design was used. The study was carried out at the pediatric inpatient department in Cardiovascular Hospital, affiliated to Ain Shams University Hospitals. A purposive sample of 50 children undergoing heart surgery and their accompanying mothers. The study was used a structured interview questionnaire, the family-centered care scale, the index of mothers' participation, mothers’ practice observational checklists, and the coping process scale to collect the study’s data. Results: The study reveals a statistically significant difference in the mothers' total coping pattern after family-centered care intervention compared to before intervention at p=0.000. A statistically significant difference was also revealed regarding the total mothers' participation in the care of their children with congenital heart disease at p=0.000. There was a strong positive correlation between total mothers' coping patterns, participation, and mothers' total actual practices regarding their children's care suffering from congenital heart diseases after the intervention. Conclusion: The family-centered care intervention effectively improved mothers' coping patterns and participation in care for their children with congenital heart diseases. Emphasizing the importance of family (usually the mother) participation in care for their children with congenital heart diseases during hospitalization for heart surgery. Designing and carrying out educational programs for pediatric nurses to promote family-centered care in clinical practice.


2017 ◽  
Vol 2 (2) ◽  
pp. 106-111 ◽  
Author(s):  
Niaz Kamal ◽  
Nasih Othman ◽  
Aso Salih

Congenital heart diseases (CHD) are common causes of cardiovascular morbidity and mortality among young children and adolescents. It is the most common form of structural congenital defects. Little is known about incidence and type of these disorders in Sulaimani. Therefore, this study was undertaken to determine the incidence of congenital heart diseases and their types in Sulaimani Governorate. The study was conducted in Sulaimani Pediatric Teaching Hospital and the Maternity Hospital during January 2015 to December 2016. A sample of 400 consecutive cases of CHD in children aged 0-12 years was recruited attending the echocardiographic department of the hospital for diagnosis or follow-up purposes. Demographic data was collected through a face-to-face interview with the mothers of cases. A total number of new live births for 2015-2016 and those with congenital heart diseases were obtained from the medical records of the department of statistics of Sulaimani Maternity Teaching Hospital. The overall incidence of all types of congenital heart diseases was 1.7/1000, 1.6/1000 live births for the year 2015 and 2016 respectively. There was a statistically significant difference in incidence between males and females over the two years, male to female risk ratio 1.83 (95% CI 1.09-3.14, p 0.007). The commonest types of cyanotic congenital heart diseases were tetralogy of Fallot (38.9% of all cyanotic defects), complete atrioventricular canal (22.1%), and transposition of great arteries (18.2%), while the most common types of non-cyanotic congenital heart disease were atrial septal defect (34.1% of all non-cyanotic defects), ventricular septal defect (31.6%), and the other defects are less common. In conclusion, tetralogy of Fallot, atrial septal defects, and ventricular septal defects are the commonest congenital heart deformities in Sulaimani Governorate; the incidence is lower than other countries but this could be an underestimation.


Author(s):  
M.V. Medvedev, M.V. Kubrina

Main prenatal ultrasound differential patterns of congenital heart diseases including interventricular septal defect and dextroposed aorta are presented.


2020 ◽  
Vol 16 ◽  
Author(s):  
Farhan Bajwa ◽  
Syed M Jafri ◽  
Karthik Ananthasubramaniam

: The advancement in corrective surgical procedures and anaesthesia technology has resulted in the increase survival of patients with Congenital Heart Diseases (CHD). Most of the surviving CHD patients have successfully reached adulthood and those surviving adults now outnumber the infants born with the CHD. Unfortunately, the surviving adults with CHD do not get proper care due to either inconsistent follow up or not getting care from a specialist in the field of CHD. It is imperative for general practicing clinicians to be aware of the congenital diseases as well as the current clinical recommendations. This manuscript reviews some of the common congenital diseases seen in adults such as cardiac shunts, left heat obstructive lesions and aortopathies.


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