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Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Double-outlet right ventricle (DORV) is defined as “a type of ventriculoarterial connection in which both great vessels arise either entirely or predominantly from the right ventricle” [13]. DORV accounts for 1% of all CHD [14] and is often associated with other anomalies such as coarctation, heterotaxy, and chromosomal mutations such as trisomy 13, trisomy 18, and chromosome 22q11 deletion. Surgical approach to DORV, and thus the associated residua and sequelae, depend on the relationship of the VSD to the great arteries, the size of the VSD, the size of the great arteries, size of the ventricles, and the atrioventricular valves. Ventricular-to-pulmonary artery conduits and baffles are typically used in two-ventricle repairs, and are associated with conduit stenosis or regurgitation, and baffle stenoses or leaks. Single-ventricle repairs are discussed below.
Cardiology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
This lesion is also called univentricular heart and typically both inlet valves open into a ‘single’ ventricle, although it is rarely single and a posterior hypoplastic ventricle is usually identifiable on detailed scanning. The management is rather like tricuspid atresia and depends on the arterial connections (i.e. which are from the hypoplastic ventricle), the size of the VSD and any associated lesions, which, just like tricuspid atresia, may limit pulmonary or systemic flow. There may be associated pulmonary stenosis and systemic outflow obstruction or coarctation.
Cardiac surgery
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
In some situations a single ventricle circulation is created surgically where the ventricle supports the systemic circulation and pulmonary blood flow comes directly from the systemic veins. The single ventricle can be a right or a left ventricle. Lesions requiring a Fontan strategy include hypoplastic left heart syndrome and tricuspid atresia.
Velocity vector imaging for the assessment of segmental ventricular function in children with a single right ventricle after cavopulmonary anastomosis
Published in Current Medical Research and Opinion, 2019
Li-Jun Chen, Yu-Qi Zhang, Sheng-Fang Bao, Shu-Wen Zhong, Ai-Min Sun, Zhi-Fang Zhang
There are a number of limitations of this study that should be considered. The first is the relatively small size of the groups. A single ventricle anomaly is very uncommon, and acquiring a large number of patients is difficult. Only longitudinal ventricular mechanics was examined; radial and circumferential strains of the SRV were not evaluated. While, radial and circumferential strains are important, longitudinal ventricular mechanics is the most important3, and thus we believe that using this measure is the first step towards validating VVI for evaluation of a single ventricle. We did not observe a relationship between the type of single ventricle and heart function due to the small number of single ventricle patients. All indices studied were of systolic function; diastolic function was not evaluated. As with ventricular mechanics, systolic function is more of a concern than diastolic function.
Overview and surgical aspects of Uhls anomaly
Published in Expert Opinion on Orphan Drugs, 2020
Kotturathu Mammen Cherian, Lydia Jeris W, Mohamed Thayub
The various surgical options available are Single ventricle strategy (Bidirectional cavopulmonary anastomosis, atrial septectomy, and disconnection of main pulmonary artery);One and a half ventricle repair with partial right ventriculectomy;Fontan procedure; andCardiac transplantation.
Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2022
Meredith C. G. Broberg, Ira M Cheifetz, Sarah T Plummer
There is significant variation in medication use during the interstage period. This is partly due to lack of evidence for or against the use of many of these medications, but also due to the need to address the symptoms and morbidity these patients encounter during this vulnerable time period. Infants with single ventricle congenital heart disease have significant symptomology ranging from irritability to respiratory distress and poor weight gain. Well-intentioned clinicians treat these infants relying primarily on collective experience and evidence from other disease states and other patient populations.