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Cardiovascular system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
8.19. Which of the following is/are found in children with tetralogy of Fallot?The heart appears enlarged on chest X-ray.Cyanosis is invariably present by the first birthday.Pulmonary valve stenosis must be present to make the diagnosis.A right aortic arch is present in more than 15% of cases.The aortic valve is smaller than usual.
Relationship between body mass index and the degree of mitral valve stenosis: Supporting evidence for the obesity paradox phenomenon
Published in Ade Gafar Abdullah, Isma Widiaty, Cep Ubad Abdullah, Medical Technology and Environmental Health, 2020
A.N. Lestari, I.R. Alie, M.R. Akbar
In a guideline of echocardiographic examination of valve stenosis, Baumgartner et al. (2009) stated that there are other valve abnormalities that can be caused by mitral valve stenosis due to its rheumatic nature. Tricuspid valve disease, which is often associated with this case, is functional tricuspid regurgitation. Pulmonary valve stenosis can be very rare, although it is related to the rheumatic process. In mitral valve regurgitation due to rheumatism, the main mechanism is the restriction of movement of the valve leaflets (Baumgartner et al. 2009).
Congenital heart disease in the neonatal period
Published in Janet M Rennie, Giles S Kendall, A Manual of Neonatal Intensive Care, 2013
Janet M Rennie, Giles S Kendall
Cross-sectional echocardiography shows an enlarged right atrium. In 85% of babies the RV is very small but thick walled, often with endocardial fibroelastosis. The tricuspid valve is often small and dysplastic. The pulmonary valve is usually an imperforate membrane with a good-sized main pulmonary artery, but at the other extreme there may be absence of the outlet portion of the RV and of most of the pulmonary trunk. Colour Doppler often shows marked tricuspid incompetence. The absence of flow across the pulmonary valve differentiates the condition from severe pulmonary valve stenosis. Immediate transfer to a specialist centre, with prostaglandin infusion to maintain duct patency, is essential.
An Unguarded Tricuspid Valve Orifice Diagnosed by Autopsy
Published in Fetal and Pediatric Pathology, 2023
Xiaoxue Zhou, Ye Zhang, Yihua He
The family chose termination of the pregnancy. Chromosomal malformation and known congenital heart defect genes were excluded. Exploration of organs such as lungs, liver, gallbladder, and appendix confirmed the normal position and development of internal organs on fetal autopsy. The number of lobes in the lungs were two lobes on the left and three lobes on the right. Marked cardiomegaly was due to the dilated RA on fetal autopsy (Fig. 4). The right ventricle and the left-sided chambers were concordant and of normal size. There was a partial absence of tricuspid septal leaflets and tricuspid septal annulus (Fig. 5). Stenotic pulmonary valve, hypoplasia of the main pulmonary artery and branches were found (Fig. 6). An atrial secundum defect (Fig. 7) with an intact interventricular septum was also present. The final diagnosis on autopsy confirmed UTVO, pulmonary valve stenosis, and secundum atrial septal defect.
Care of the adult woman with Turner syndrome
Published in Climacteric, 2018
S. Shah, H. H. Nguyen, A. J. Vincent
Up to 70% of TS females have congenital heart disease (CHD) (Figure 1) and the presence of bicuspid aortic valve (BAV) should prompt karyotype assessment for TS8. The most common abnormality is BAV, occurring in 15–34% of TS patients (compared with 1–2% in the general population)52. BAV is a risk factor for valvular dysfunction, aortic dilation, aortic dissection and infective endocarditis. Coarctation of the aorta, the second most common form of CHD, occurs in up to 17% of patients (compared with 0.04% in the general population) and may coexist with BAV and abnormal aortic phenotype. Other identified abnormalities include: partial anomalous pulmonary venous drainage, transverse aortic arch, atrial septal defects, ventricular septal defects, persistent arterial duct, interrupted inferior vena cava with azygous continuation, and pulmonary valve stenosis52.
Management of congenitally corrected transposition from fetal diagnosis to adulthood
Published in Expert Review of Cardiovascular Therapy, 2023
All the surgical options mentioned above leave the morphologically RV in the systemic position. A completely new approach to surgical management of ccTGA was proposed at the turn of the 1980s and 1990s [34–36]. The aim of so-called anatomical correction or double-switch procedure is to restore the morphologically left ventricle into the subaortic position. It can be achieved by atrial switch (Mustard/Senning procedure) in combination with arterial switch operation. In patients with pulmonary valve stenosis, the arterial switch procedure cannot be performed. In such cases, the Rastelli procedure, the Nikaidoh procedure and the REV procedure are the options [37]. The long-term survival of anatomic repair is acceptable (20-year survival of 83%) [38], and the long-term complication of the arterial switch is the neoaortic regurgitation [39]. Of importance, this surgical option is available only for infants and young children in whom the morphologically left ventricle is still capable to sustain systemic pressure. In patients without subpulmonary ventricular flow obstruction, the pulmonary artery banding has been proposed as a way to adapt the left ventricle to work as a systemic ventricle. The results of pulmonary artery banding as a bridge to double switch procedure are inconsistent [40–42]. An intention-to-treat analysis of pulmonary artery banding showed that patients above 16 years of age are unlikely to achieve anatomic repair [43]. Some authors noted that individuals with palliative pulmonary artery band had better survival than the anatomic repair group [44], whereas others observed the lowest transplant-free survival at 10 years in this group of patients [45].