Intracardiac conduction intervals in children with congenital heart disease: comparison of His bundle studies in 41 normal children and 307 patients with congenital cardiac defects.

Two families are described in which there is possible monogenic inheritance of congenital cardiac defects within the spectrum of faulty conotruncal septation (CTS). Evidence for a genetic control of conotruncal septation arises from genetic and embryologic studies of similar defects in the Keeshond dog model, the excess of sibship pairs with conotruncal septation defects in sibship pairs with congenital heart disease, and previously reported pedigrees of families with multiple affected individuals with conotruncal septation defects. It is suggested that in the small number of cases of congenital cardiac defects in which there is a strong family history for CTS defects, a higher recurrence risk should be considered rather than the usual polygenic recurrence risk of 3% that is usually given in such situations.

Download Full-text

Book ReviewAtlas of Two-Dimensional Echocardiography in Congenital Cardiac Defects Congenital Heart Disease: Morphologic echocardiographic correlations

New England Journal of Medicine ◽

10.1056/nejm198405173102030 ◽

1984 ◽

Vol 310 (20) ◽

pp. 1339-1339

Author(s):

Warren G. Guntheroth

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Two Dimensional ◽

Cardiac Defects ◽

Congenital Cardiac Defects ◽

Dimensional Echocardiography ◽

Two Dimensional Echocardiography

Download Full-text

Pattern of Congenital Heart Disease In Children Attending Central Teaching Pediatric Hospital, Baghdad

AL-Kindy College Medical Journal ◽

10.47723/kcmj.v15i2.170 ◽

2020 ◽

Vol 15 (2) ◽

pp. 82-86

Author(s):

Husam T Al-Zuhairi

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Ductus Arteriosus ◽

Cardiac Defects ◽

Common Cause ◽

Congenital Cardiac Defects ◽

Modes Of Presentation ◽

Patent Ductus

Background: Congenital cardiac defects have a wide spectrum of severity in infants. About 30-40% of patients with congenital cardiac defects will be symptomatic in the 1st year of life, while the diagnosis was established in 60% of patients by the 1st month of age. Objectives: To identify the occurrence of specific types of CHD among hospitalized patients and to evaluate of growth of patients by different congenital heart lesions. Methods: A retrospective study, done on ninety-six patients (51 male and 45 female) with congenital heart disease (CHD) admitted to central teaching hospital of pediatrics, Baghdad from 1st September 2009 to 30th of August 2010. Results: The most common congenital heart diseases (CHD) were ventricular septal defect (VSD), tetralogy of Fallot (TOF), patent ductus arteriosus (PDA), transposition of great arteries (TGA), pulmonary Stenosis( PS), and Atrial septal defect (ASD).The most common modes of presentation were respiratory infection and heart failure in acyanotic patients and cyanosis in cyanotic groups. The study showed that only patent ductus arteriosus and atrial septal defects were more common in female while all other lesions were equal male to female ratio or slightly more common in male. The effect of acyanotic congenital heart disease on growth (Wt) is more common than cyanotic congenital heart disease. Conclusions: VSD is the most common type of congenital heart disease and the most common cause of morbidity, while ASD is the least common cause of morbidity in patients with congenital heart disease. The most common modes of presentation of patients with CHD are respiratory infection and heart failure in acyanotic CHD while cyanosis is the most common presentation in cyanotic group.

Download Full-text

Trazodone-Associated Syncope in a Patient with Congenital Cardiac Anomalies

Journal of Pharmacy Technology ◽

10.1177/875512259801400210 ◽

1998 ◽

Vol 14 (2) ◽

pp. 78-81

Author(s):

Vinod S Bhatara ◽

Sami Awadallah

Keyword(s):

Risk Factors ◽

Congenital Heart Disease ◽

Heart Disease ◽

Noonan Syndrome ◽

Congenital Heart ◽

Cardiac Defects ◽

Congenital Cardiac Defects ◽

Cardiac Anomalies ◽

Case Summary ◽

Congenital Cardiac Anomalies

Objective: To report a case of trazodone-associated syncope in a 19-year-old woman with congenital cardiac anomalies and to review risk factors and mechanisms involved in this case. Case Summary: A 19-year-old woman diagnosed with Noonan syndrome with congenital cardiac anomalies tolerated sertraline, but had a syncopal episode when trazodone was used. There was no recurrence of syncope after the trazodone was discontinued. Discussion: The syncopal episode could have resulted from either trazodone or congenital heart disease, but it most likely resulted from a trazodone–congenital heart disease interaction. Trazodone is not cardiotoxic in most patients; our patient was probably vulnerable to the cardiotoxic effects of trazodone due to congenital cardiac defects. Conclusions: Trazodone can precipitate arrhythmias or syncope in susceptible patients with heart disease and should be prescribed cautiously in patients with congenital heart disease. In such cases, it may be safer to use a selective serotonin-reuptake inhibitor.

Download Full-text

GENETICS OF CARDIAC MALFORMATIONS

Fetal and Maternal Medicine Review ◽

10.1017/s0965539508002155 ◽

2008 ◽

Vol 19 (2) ◽

pp. 105-118

Author(s):

SHA TANG ◽

TAOSHENG HUANG

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Heart Defects ◽

Fetal Life ◽

Genetic Syndromes ◽

Cardiac Defects ◽

Psychological Burden ◽

Congenital Cardiac Defects ◽

Child Healthcare

ABSTRACTCongenital heart disease is one of the most common major malformations in humans, contributing substantially to the financial and psychological burden of child healthcare. About one percent of children are born with heart defects, and every year, more children die from congenital heart disease than are diagnosed with cancer. A diagnosis of congenital heart disease is frightening for parents, particularly when it affects a fragile newborn. The heart is the first organ to be matured in a human fetus and if a particular congenital heart defect is compatible with fetal life, the child will be born with a defective heart. More than 300 genetic syndromes are associated with congenital cardiac defects. In this review, we will discuss the genetics of congenital heart disease, how to carry out a diagnosis of the genetic causes and how to provide counseling for families with congenital heart disease.

Download Full-text

ABNORMAL CAPILLARY MORPHOLOGY IN CONGENITAL HEART DISEASE

PEDIATRICS ◽

10.1542/peds.37.2.316 ◽

1966 ◽

Vol 37 (2) ◽

pp. 316-322

Author(s):

Stella B. Kontras ◽

JoAnn G. Bodenbender

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Chronic Hypoxia ◽

Normal Children ◽

Vascular Abnormalities ◽

Definitive Surgery ◽

Vascular Compartment

Capillary morphology has been studied in 111 normal children. In 98% of these cases, straight hairpin forms make up over 80% of the capillaries examined. Thirty children with congenital heart disease showed 80% abnormal capillary patterns consisting of dilated, tortuous, and branched loops. These were most marked in cyanotic tetralogy of Fallot and ventricular septal defect with pulmonary hypertension. These cases also were associated with microscopic hemorrhages, increased capillary fragility and actual postoperative hemorrhagic complications. Definitive surgery in two cases, though improving the patient, did not result in changes in the abnormal capillary patterns. It is suggested that the role of the capillary structure in rheology of blood in the microvasculature has largely been ignored and that abnormalities in this vascular compartment may affect perfusion of tissues. The association of abnormalities in coagulation studies, hemorrhage, and thrombosis with congenital heart disease may in part be related to the morphologic vascular abnormalities. The abnormal capillary findings may be due to maturational arrest or dysmaturity of this portion of the cardiovascular system or may be the result of chronic hypoxia.

Download Full-text

CT and MRI Findings of Cardiac Defects in Congenital Heart Disease

Radiology Illustrated: Pediatric Radiology - Radiology Illustrated ◽

10.1007/978-3-642-35573-8_17 ◽

2013 ◽

pp. 553-572

Author(s):

Hyun Woo Goo

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Mri Findings ◽

Cardiac Defects ◽

Ct And Mri

Download Full-text

Mismatch between self-estimated and objectively assessed exercise capacity in patients with congenital heart disease varies in regard to complexity of cardiac defects

Cardiology in the Young ◽

10.1017/s1047951120003406 ◽

2020 ◽

pp. 1-7

Author(s):

Julius M. Woile ◽

Stefan Dirks ◽

Friederike Danne ◽

Felix Berger ◽

Stanislav Ovroutski

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Oxygen Uptake ◽

Exercise Capacity ◽

Congenital Heart ◽

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Uptake ◽

Subjective Rating ◽

Cardiac Defects ◽

Cardiopulmonary Exercise

Abstract Aim: Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity. Methods: We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8–68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient’s self-estimated exercise capacity with focus on differences between complex and non-complex defects. Results: Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho −0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho −0.11, p < 0.255). Conclusion: The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD. Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Download Full-text