scholarly journals Surgical Options for Pulmonary Valve Pathology in the Current Era

2021 ◽  
Author(s):  
Sameh M. Said
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Clinical Profile ◽  
Surgical Options ◽  
Valve Pathology

Pulmonary valve pathology occurs mostly in the settings of congenital heart disease whether primary or as the result of repair of a variety of congenital heart defects. Acquired pulmonary valve disorders, albeit rare, can occur in the settings of endocarditis, tumors, carcinoid syndrome, or rheumatic fever. Surgical options include repair and replacement of pulmonary valve. Several options for replacement are available, which can be tailored based on the patient’s clinical profile and the primary valve pathology. In this chapter, we present the surgical options that are currently available for pulmonary valve disorders and the current outcomes.

scholarly journals Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Genetic Diagnosis ◽  
Single Nucleotide Variants ◽  
Personalized Approach

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.

Lecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease

2019 ◽  
Vol 10 (5) ◽  
pp. 558-564
Author(s):  
Christina L. Greene ◽  
Richard D. Mainwaring ◽  
Douglas Sidell ◽  
Michal Palmon ◽  
Frank L. Hanley
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Surgical Procedure ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Pulmonary Atresia ◽  
Absent Pulmonary Valve ◽  
Airway Compression ◽  
Absent Pulmonary Valve Syndrome

Purpose: Children with congenital heart disease may present with severe airway compression prior to any surgical procedure or may develop airway compression following their surgical procedure. This combination of congenital heart defect and airway compression poses a significant management challenge. The purpose of this study was to review our experience with the Lecompte procedure for relief of severe airway compression. Methods: This was a retrospective review of ten patients who underwent a Lecompte procedure for relief of severe airway compression over the past nine years (2010-2018). Three patients with absent pulmonary valve syndrome presented with severe symptoms prior to any surgical procedure. Seven patients presented with symptoms of airway compression following repair of their congenital heart defects (one with absent pulmonary valve syndrome, three patients had repair of pulmonary atresia with ventricular septal defect, and three patients had undergone aortic arch surgery). The median age at presentation was two years (range: one day to seven years). Results: The ten patients underwent a Lecompte procedure without any significant complications or operative mortality. The median interval between the surgical procedure and extubation was 9.5 days. No patients have required any further interventions for relief of airway obstruction. Conclusions: The Lecompte procedure is a surgical option for young children who present with severe airway compression. The patients in this series responded well to the Lecompte procedure as evidenced by clinical relief of airway compression.

Cause of death in adults with congenital heart disease — An analysis of the German National Register for Congenital Heart Defects

2016 ◽  
Vol 211 ◽  
pp. 31-36 ◽  
Author(s):  
Claudia C. Engelings ◽  
Paul C. Helm ◽  
Hashim Abdul-Khaliq ◽  
Boulos Asfour ◽  
Ulrike M.M. Bauer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Cause Of Death ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
National Register

scholarly journals Morphological characteristics of the thymus gland in children with congenital heart disease

1986 ◽  
Vol 67 (4) ◽  
pp. 265-268
Author(s):  
I. F. Matyushin ◽  
I. K. Okhotin ◽  
V. Ya. Ovsyanikov
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Marginal Zone ◽  
Heart Defects ◽  
Morphological Characteristics ◽  
Thymus Gland ◽  
Thoracic Cavity ◽  
Biopsy Specimens

We have undertaken a study of thymus gland structure in children with congenital heart defects aged 3 to 10 years. We analyzed thymus biopsy specimens obtained from the marginal zone of the gland during surgical correction of cardiac malformations after thoracic cavity dissection. The biopsy size was 1.5 cm3.

scholarly journals The incidence of congenital heart defects in the world regarding the severity of the defect

2016 ◽  
Vol 73 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Vesna Miranovic
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Methodological Approach ◽  
Live Births ◽  
Significant Difference ◽  
The World

Bacground/Aim. Congenital heart defects (CHDs) are structural or functional abnormalities of the heart present at birth even if they are detected much later. Their importance lies in the fact that, depending on the severity, they change the quality of life, and may be life threating. In addition, we should not ignore the high costs of treating people with congenital heart disease. The aim of this study was to analyze the incidence of congenital heart disease in relation to the severity in the world based on the available literature. Methods. All the available literature on the incidence of CHD cases regarding the severity of CHD published from 1955 to 2012 was analyzed. The researcher was able to read the titles and abstracts of 128 papers on the subject. Due to methodological inconsistency, 117 of the papers were rejected. Based on the criteria of reliability, availability and comparability, our analysis included 11 studies testing CHD incidence regarding the severity of the defect conducted all over the world. The Yates' ?2-test was used to compare the observed incidences. Results. The frequency of severe congenital heart defects, ranged from 0.414 to 2.3/1,000 live births, the incidence of moderate congenital heart defects from 0.43 to 2.6/1,000 live births while in the group of minor congenital heart defects the incidence ranged from 0.99 to 10.3/1000 live births. There were no statistically significant differences in the incidence of mild, moderate and severe CHDs. Conclusion. The results obtained studying of the available data suggest that no statistically significant difference in the incidence of mild, moderate and severe congenital heart defects. A universal methodological approach to the incidence of CHD is essential.

Prevalence of congenital heart defects among offspring of parents with congenital heart disease

1969 ◽  
Vol 23 (1) ◽  
pp. 131
Author(s):  
J.J. Nora ◽  
Paul F. Dodd ◽  
R.D. Leachman ◽  
R.J. Sommerville ◽  
D.G. McNamara
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects
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