China
Africa
Explore chapters and articles related to this topic
Cardiac diseases in pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Saravanan Kuppuswamy, Sudarshan Balla
Many children with complex cyanotic congenital heart disease die during infancy and childhood, and do not reach child-bearing age, but with greatly improved surgical techniques, a larger number of these patients are surviving into adulthood. Specific congenital lesions of interest include (i) tetralogy of Fallot (PS, VSD, overriding aorta, and right ventricular hypertrophy); (ii) Ebstein’s anomaly (displacement of the tricuspid valve into the right ventricular cavity, resulting in a small right ventricle and poor forward output, often associated with right-to-left shunting through an ASD; (iii) truncus arteriosus (single outflow tract and outflow valve distal to both ventricles, often associated with a VSD); (iv) transposition of the great vessels (separate pulmonary and systemic circulations operating in parallel with communication via a VSD; and (v) tricuspid atresia (absent tricuspid orifice, small nonfunctional right ventricle, and a connection between the pulmonary and systemic circulations). These lesions represent many of the important cyanotic congenital heart lesions, and individual lesions may be variable in severity.
Congenital heart disease
Published in Swati Gupta, Alexandra Marsh, David Dunleavy, Kevin Channer, Cardiology and the Cardiovascular System on the move, 2015
Swati Gupta, Alexandra Marsh, David Dunleavy, Kevin Channer
It describes a syndrome with four cardiac manifestations: Infundibular muscular pulmonary stenosisVSDOverriding aortaRV hypertrophy
Congenital heart disease
Published in Andrew R. Houghton, MAKING SENSE of Echocardiography, 2013
Tetralogy of Fallot (ToF) accounts for 3.5 per cent of cases of congenital heart disease and, as the word ‘tetralogy’ suggests, consists of four key abnormalities (Fig. 28.6): VSDoverriding aortaRVOT obstructionRV hypertrophy.
The challenges of an aging tetralogy of Fallot population
Published in Expert Review of Cardiovascular Therapy, 2021
Jennifer P. Woo, Doff B. McElhinney, George K. Lui
Significant advancements in medicine and cardiac surgery have allowed children with congenital heart disease (CHD) to survive into adulthood [1–3]. This achievement has transformed the field of CHD such that there are now more adults than children living with CHD in the United States [4–7]. Tetralogy of Fallot (TOF) is the most common form of cyanotic CHD, with an incidence of 0.33 to 0.36 per 1000 live births [8,9]. The anterior displacement of the infundibular septum during cardiac development results in the four main defects associated with TOF – a malalignment ventricular septal defect (VSD), overriding aorta, right ventricular outflow tract (RVOT) obstruction, and right ventricular (RV) hypertrophy. The disease encompasses a wide spectrum ranging from mild forms with a small VSD and minor RVOT obstruction to severe forms with complete AV canal defects and complete pulmonary atresia. Improvements and substantial progress in management since the first surgical repair was performed in the 1950s have led to a dramatic increase in infant survival and overall life expectancy, with a 90% 20 to 30-year survival in recent outcome studies [10–13]. Cardiac surgery, however, is not curative and decades after repair, adults are often faced with the long-term complications and sequalae from the underlying cardiac anomalies as well as the surgical and other interventional treatments.
Absent Pulmonary Valve Syndrome in a Fetus: A Case Report and Literature Review
Published in Fetal and Pediatric Pathology, 2019
Wan-Ying Zhou, Yue-Yi Li, Xiao-Qin He, Yi-Bin Wang
Absent pulmonary valve syndrome (APVS) occurs in about 0.2%–0.4% of live born infants with congenital heart disease [1]. The main characteristics of APVS include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and aneurysmal dilatation of the pulmonary trunk and its branches. APVS is commonly divided into two types [2, 3]. The first type is characterized as APVS with a ventricular septal defect, overriding aorta, and absence of ductus arteriosus. This is the more common type of APVS, which shares many similarities with Tetralogy of Fallot (TOF) – also known as TOF-type APVS [3]. The other less common type of APVS has an intact ventricular septum, a lower degree of pulmonary artery dilatation, and a patent ductus arteriosus, with or without tricuspid atresia – also called non-TOF-type APVS [4]. APVS has poor prognosis: apart from heart complications, the aneurysmal dilatation of the pulmonary trunk and its branches can compress the bronchus and esophagus, leading to bronchomalacia and polyhydramnios. According to a previous study [2], mortality of APVS was 75%, including intrauterine death and postnatal death, only 25% were alive after postnatal surgery; therefore, prenatal diagnosis of APVS is significant.
Association between the promoter methylation of the TBX20 gene and tetralogy of fallot
Published in Scandinavian Cardiovascular Journal, 2018
Xiaofei Yang, Qingyu Kong, Zhenghao Li, Min Xu, Zhifeng Cai, Cuifen Zhao
The tetralogy of Fallot(TOF) is the most common kind of cyanotic congenital heart disease and accounts for 10% of congenital heart disease cases. Its pathological features consist of ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy [1]. Epidemiological studies showed that genetic defects play an important role in the pathogenesis of TOF [2–5], but the mechanisms underlying this role are undetermined [6]. In recent studies, Epigenetic factors were reported to alter cardiac development-related genes expression in signaling pathways and contribute to the occurrence of TOF [7–12].