Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects

2013 ◽  
Vol 80 (8) ◽  
pp. 663-674 ◽  
Author(s):  
P. Syamasundar Rao
2010 ◽  
Vol 17 (3-4) ◽  
pp. 116-122
Author(s):  
Ramunė VANKEVIČIENĖ

Background. The discovery of ultrasound has made a revolution in almost all fields of medicine. The past three decades have withessed an intensive development of fetal echocardiography methods and technique. The aim of the paper is to present a review of the results and trends of the last 10 years of fetal echocardiography in Lithuania and to show the spectrum and outcomes of prenatally detected congenital heart diseases. Materials and methods. Fetal echocardiography was performed for 1816 fetuses during the period from 1999 to 2009. Results. Cardiac pathology was diagnosed in 176 (9.7%) fetuses. Heart defects were detected in 112 (63.6%) of them, cardiac rhythm and conduction disturbances in 62 (35.2%), cardiomyopathy in 2 (1.1%) fetuses, and heart rhabdomyoma in 1 (0.6%) fetus. The general rate of the postnatal diagnosis of congenital heart defects in Lithuania was about 10%. Most of fetal cardiac diseases (70.5%) were diagnosed after 22 weeks of gestation. Because most of antenatally diagnosed congenital heart defects (74%) were critical and inconsistent with life, a large part of newborns (40.2%) died in the neonatal period, 10.7% of fetuses died in utero, and 8% of pregnancies were terminated by abortion. The data demonstrate good tendencies: the diagnosis has become earlier, a wider spectrum of diseases have been diagnosed, more newborns have survived. Our survey shows that 41.1% of newborns with prenatally diagnosed congenital heart defects have survived. Conclusions. 10% of severe congenital heart diseases are detected prenatally in Lithuania. The efficacy of antenatal diagnostics depends on the qualification of specialists, the number of tertiary care centers, on a successful collaboration among pediatric cardiologists, obstetricians and geneticists. The main problem is an insufficient preparation of obstetricians, the uncertified favor of pediatric cardiologist. Keywords: congenital heart disease, fetal echocardiography, antenatal diagnostics


Author(s):  
Tong Duy Phuc ◽  
Nguyen Sinh Hien ◽  
Nguyen Dang Hung ◽  
Vuong Hoang Dung

Abstract: Congenital airway stenosis (tracheobronchial stenosis) in children is rare, difficult to treat and become more complex when associated with congenital heart defects. In severe airway stenosis, slide tracheobronchoplasty is the most optimal strategy to manage this condition, yet really challenging. We report case series with this combined condition of airway stenosis and congenital heart diseases, which were successfully treated by slide tracheobronchoplasty with concomitant heart repair. We also discuss about the indication, surgical technique as well as postoperative care.


2021 ◽  
Vol 2021 ◽  
pp. 1-10
Author(s):  
Maryam Borjali ◽  
Mostafa Amini-Rarani ◽  
Mehdi Nosratabadi

Introduction. Mortality due to noncommunicable diseases has increased in the world today with the advent of demographic shifts, growing age, and lifestyle patterns in the world, which have been affected by economic and social crises. Congenital heart defects are one of the forms of diseases that have raised infant mortality worldwide. The objective of present study was to identify nonmedical determinants related to this abnormality from the mother’s perspectives. Methods. This research was a qualitative study and the data collection method was a semistructured interview with mothers who had children with congenital heart diseases referring to the Shahid Rajaei Heart Hospital in Tehran, Iran. A thematic analysis approach was employed to analyze transcribed documents assisted by MAXQDA Plus version 12. Results. Four general themes and ten subthemes including social contexts (social harms, social interactions, and social necessities), psychological contexts (mood disorders and mental well-being), cultural contexts (unhealthy lifestyle, family culture, and poor parental health behaviors), and environmental contexts (living area and polluted air) were extracted from interviews with mothers of children with congenital heart diseases. Conclusions. Results suggest that factors such as childhood poverty, lack of parental awareness of congenital diseases, lack of proper nutrition and health facilities, education, and lack of medical supervision during pregnancy were most related with the birth of children with congenital heart disease from mothers’ prospective. In this regard, targeted and intersectorial collaborations are proposed to address nonmedical determinants related to the incidence of congenital heart diseases.


Author(s):  
A. V. Tsepokina ◽  
A. V. Ponasenko ◽  
A. V. Shabaldin

The article presents data on the study of gene interconnections between the xenobiotics detoxification genes in various phases.Materials and methods. The study involves 131 children with congenital heart diseases (CHD) and 103 conditionally healthy children. The genotyping was performed by RT-PCR method using TaqMan probes. Intergenic bonds were analyzed via MDR v.3.0.2.Results and conclusion. We discovered no statistically significant differences in the genotype distribution in children with CHD and conditionally healthy donors. The analysis of intergenic interactions helped to develop a five-locus model characterized by the highest reproducibility, sensitivity and specificity: GSTP1 rs1793068 – GSTP1 rs6591256 – GSTP1 rs1871042 – CYP1A1 rs1048943 – CYP1A2 rs762551. This model was used to determine a number of protective and risky combinations of congenital heart defects-associated genotypes in children.


2007 ◽  
Vol 5 (3) ◽  
pp. 0-0
Author(s):  
Virgilijus Lebetkevičius ◽  
Virgilijus Tarutis ◽  
Rita Sudikienė ◽  
Daina Liekienė ◽  
Kęstutis Lankutis ◽  
...  

Virgilijus Lebetkevičius1, Virgilijus Tarutis1, Rita Sudikienė1, Daina Liekienė1, Kęstutis Lankutis1, Giedrė Nogienė1, Vidmantas Jonas Žilinskas1, Vytautas Sirvydis1, Kęstutis Versockas2, Žydrė Jurgelienė2, Asta Bliūdžiūtė21 Vilniaus universiteto Širdies chirurgijos centras, Santariškių g. 2, LT-08661 Vilnius2 Vilniaus universiteto ligoninės Santariškių klinikų Anesteziologijos,intensyviosios terapijos ir skausmo gydymo centras, Santariškių g. 2, LT-08661 VilniusEl paštas: [email protected] Tikslas Apžvelgti Vilniaus universiteto Širdies chirurgijos centro naujagimių įgimtų širdies ydų chirurgijos rezultatus, juos įvertinti ir padaryti išvadas. Metodai Nuo 1964 iki 2006 metų Vilniaus universiteto Širdies chirurgijos centre atlikta 5066 įgimtų širdies ydų operacijos vaikams iki 18 metų. Naujagimių dalis – 307 operacijos (6,1%). Korekcijos naujagimiams atliktos naudojant dirbtinę kraujo apytaką (DKA) ir be jos. Rezultatai Įgimtų širdies ydų operacijos suskirstytos į keturis etapus. 1974–1990 metai (1974 m. atlikta pirmoji širdies operacija naujagimiui). Tuo laikotarpiu mirštamumas buvo 100%. 1991–1995 metais – mirštamumas 71%, 1996–2000 metais – mirštamumas 68%, 2001–2006 metais – mirštamumas 38%, 2006 metais mirštamumas – 22%. Išvados Analizuojant naujagimių širdies chirurgijos rezultatus, pažymėtina, kad per pastaruosius metus mirštamumas sumažėjo iki priimtinų dydžių, kurie artėja prie kitų pasaulio širdies chirurgijos klinikų rezultatų. Gerėjant visam kompleksui tyrimo, gydymo, slaugymo priemonių visose grandyse (kardiologija, anesteziologija-reanimatologija, chirurgija), mirštamumą įmanoma sumažinti iki minimalaus. Pagrindiniai žodžiai: įgimtos širdies ydos, širdies chirurgija, naujagimiai Corrective surgery of congenital heart defects: experience of Vilnius University Heart Surgery Centre Virgilijus Lebetkevičius1, Virgilijus Tarutis1, Rita Sudikienė1, Daina Liekienė1, Kęstutis Lankutis1, Giedrė Nogienė1, Vidmantas Jonas Žilinskas1, Vytautas Sirvydis1, Kęstutis Versockas2, Žydrė Jurgelienė2, Asta Bliūdžiūtė21 Vilnius University, Heart Surgery Centre, Santariškių str. 2, LT-08661 Vilnius2 Vilnius University Hospital „Santariškių klinikos“, Anaesthesiology Intensive Careand Pain Management Centre, Santariškių str. 2, LT-08661 VilniusE-mail: [email protected] Objective To access the outcome and mortality trend in newborns undergoing corrective surgery for congenital heart defect. Methods We reviewed the hospital records on 307 neonates under 30 days of life, who had congenital heart defects operated on at the Heart Surgery Center of Vilnius University, Lithuania, in 1994 through 2006. Early and late mortality results were analyzed. Results From January 1974 to 2006, 307 neonates with congenital heart disease underwent surgical repair at Heart Surgery Clinic of Vilnius University. It came to 6.1% of all 5066 procedures of the congenital heart diseases performed. The number of neonate operations considerably increased in the later years, because a special department with proper technique was established. 144 neonates underwent surgery repair with cardiopulmonary bypass and 163 neonates were operated on without cardiopulmonary bypass. The mean age of the patients was 11.8 ± 0.28 days. There were 113 girls and 194 boys. Conclusions Mortality from 71% in 1991–1995 dropped to 38% in 2001–2006 and 22% in 2006. Key words: congenital heart diseases, cardiosurgery, neonates


2018 ◽  
Vol 2 (Issue 2) ◽  
pp. 56 ◽  
Author(s):  
Irina Akhmedova ◽  
Gulzada Imanalieva ◽  
Damirbek Abibillaev ◽  
Taalaibek Kudaiberdiev

Objective: The aim of this paper is to understand the structure of congenital heart diseases (CHD) and the age of the first diagnosis in a population referred to specialized cardiac surgery center from different regions of Kyrgyz Republic. Methods: In 2017, 7213 people of different ages, from Kyrgyz Republic were examined by using transthoracic echocardiogram at cardiac surgery center aimed to detection of congenital heart disease Results: In total, 478 new patients with primary diagnosis of CHD were identified; 413 (86%) patients had a delayed diagnosis. The total detectability was 6.75%. The structure of congenital heart disease has a large difference depending on the age group. The proportion of complex heart defects predominates in newborn children and significantly decreases with age. Conclusion: A study of the prevalence and structure of heart defects in the country will help to find the right decision in planned care for patients with CHD, in cases of limited resources. Keywords: newborn, congenital heart defects, diagnostics, prevalence, echocardiography


2014 ◽  
Vol 995 ◽  
pp. 85-112
Author(s):  
Naznin Sultana ◽  
Nobuhiro Nakamura ◽  
Shigehisa Hirose ◽  
Koichi Kutsuzawa ◽  
Toshihiro Akaike ◽  
...  

Heart development is a precisely harmonized process of cellular proliferation, migration, differentiation, and integrated morphogenetic interactions, and therefore it is extremely vulnerable to developmental defects that cause congenital heart diseases (CHD). One of the major causes of CHD has been shown to be the mutations in key cardiac channel-forming proteins namely, connexins (Cxs). Cxs are tetra-spanning transmembrane proteins that form gap junction channels and hemichannels on cellular membrane. They allow passage of small molecules or ions between adjacent cells or between cells and the extracellular environment. Studies have revealed that the spatiotemporal expression of Cxs mainly, Cx31.9, Cx40, Cx43, and Cx45 is essentially involved in early developmental events, morphogenetic transformations, maturation, and functional significance of heart. Our lab and others have shown that mutations in gap junction proteins could result in impaired trafficking, misfolding, and improper channel function of these proteins. It has also been shown that differential expressions of cardiac Cxs are associated with pathophysiological conditions of heart. Collectively, these conditions are coupled with abrogated or modified functionality of relevant channels in cardiac tissue, which are associated with many pathological situations, including CHD. Since CHD are a major cause of morbidity, therefore recovery of such kind of heart defects associated with Cxs is extremely important but remains highly challenging. In this review, we will summarize the role of Cxs in development, morphogenesis, maturation, normal function, and pathology of heart, and propose possible bioengineering techniques to recover defects in cardiac tissues related to the modified functions of Cxs.


PLoS ONE ◽  
2021 ◽  
Vol 16 (3) ◽  
pp. e0248894
Author(s):  
Chi-Son Chang ◽  
Sir-yeon Hong ◽  
Seo-yeon Kim ◽  
Yoo-min Kim ◽  
Ji-Hee Sung ◽  
...  

Objective To investigate the prevalence of extracardiac anomalies (ECA) in prenatally diagnosed congenital heart diseases (CHD), and to provide more information for counseling of women with prenatally diagnosed fetal CHD. Methods This was a retrospective cohort study of 791 cases of fetal CHD diagnosed by prenatal ultrasound from January 2005 to April 2018. Associated ECAs included extracardiac structural malformation (ECM), chromosomal anomaly, and 22q11.2 microdeletion. CHD was classified into 10 groups according to a modified anatomic and clinical classification of congenital heart defects. Results The overall prevalence of ECA in our CHD cohort was 28.6% (226/791): ECM, 25.3%; chromosomal anomaly, 11.7%; and 22q11.2 microdeletion, 5.5%. For those with ECM, ventricular septal defect (VSD) had the highest prevalence (34.5%), followed by anomalies of atrioventricular junctions and valves (28.8%) and heterotaxy (26.9%). For those with chromosomal anomaly, anomalies of atrioventricular junctions and valves had the highest prevalence (37.5%), followed by anomalies of atria and interatrial communications (25.0%) and VSD (22.9%). 22q11.2 microdeletion was detected only in those with anomalies of extrapericardial arterial trunks (14.3%) or ventricular outflow tracts (6.4%). Conclusion ECM, chromosomal anomaly, and 22q11.2 microdeletion have different prevalence according to the type of CHD.


2021 ◽  
Author(s):  
Arash Bordbar ◽  
Mandana Kashaki ◽  
Maryam Vafapour ◽  
Amir A. Sepehri ◽  
Elaheh Partovi

Abstract Background: It is vital to screen all critical congenital heart defects as early as possible. Screening for congenital heart diseases in neonates is essential for saving children's lives and reducing undetected adult congenital heart diseases. Unfortunately, over 50% of neonates with heart malformations are unrecognized at birth. Accurate screening for congenital heart malformations can be accomplished using the certified and internationally patented digital intelligent phonocardiography machine. The objective of this study is 2-fold: assess the actual prevalence of newborn heart defects in our well-baby nursery and evaluate the digital intelligent phonocardiogram screening for critical congenital heart diseases. Methods: In this study, we have accomplished "The Neonates Cardiac Monitoring Research Project" (ethics approval IR-IUMS-FMD. REC.1398.098) at Shahid Akbarabadi Maternity Hospital. Eight hundred forty neonates from our well-baby nursery have undergone both the standard clinical examinations at birth and the digital intelligent phonocardiogram examination in a double-blinded format. The median age of the neonates was 30,85 hours, and the median weight was 3.12 Kg. In addition, the pediatric cardiologist has performed echocardiography diagnosis for each neonate classified as abnormal either by the intelligent machine or by the standard medical examinations of a neonate. Detailed dataset of the eight hundred neonates’ examinations and printouts of the intelligent machine are available online.Results: We have found that the prevalence of heart malformations in our well-baby nursery is 5%. 44% of the heart malformations are unrecognized at birth through the standard medical examination of the hospital, including neonates with CCHD, Whereas the intelligent phonocardiogram has recognized 100% of neonates with heart malformations.Conclusions: We have accomplished screening for congenital heart malformations in all neonates accurately and cost-effectively in our hospital using the digital intelligent phonocardiogram. The intelligent machine has detected CCHD, where we have not detected it through the hospital's standard medical examinations, including pulse oximetry and the heart sound auscultation. In addition, the intelligent machine interprets innocent murmurs as healthy heart sounds.


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