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Persistent left superior vena cava
Published in Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček, Congenital Heart Disease in Adults, 2008
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček
If the persistent left superior vena cava is associated with a rare coronary sinus defect, it empties via this defect into the left atrium causing a right-to-left shunt (unroofed coronary sinus). Less often it may enter the left atrium directly. A persistent left superior vena cava is usually also present in the visceral heterotaxy syndrome (see Chapter 23).
Challenges in percutaneous transvenous mitral commissurotomy
Published in Neeraj Parakh, Ravi S. Math, Vivek Chaturvedi, Mitral Stenosis, 2018
Ravi S. Math, Cholenahally Nanjappa Manjunath
A dilated coronary sinus (CS) may pose challenges to PTMC. A mildly dilated CS is often encountered in patients in congestive cardiac failure and is of little consequence in the performance of PTMC. A markedly dilated CS is encountered in the presence of a persistent left superior vena cava (PLSVC). When this is combined with absence of the right superior vena cava, the CS becomes aneurysmally dilated46 (Figure 13.16). This distorts the cardiac anatomy leading to technical difficulties in PTMC. In the absence of RSVC, the usual technique of withdrawing the Brockenbrough needle from the RSVC and observing for the two jumps during TSP is not possible. The aneurysmal CS pushes the IAS upwards. Each time TSP is attempted, the Brockenbrough needle–Mullins sheath will slip and fall into the aneurysmal CS, leading to a risk of CS perforation. This is compounded by the fact that the IAS bulge towards the RA due to high LA pressure in severe MS. In such cases, the margins of the CS os can be delineated by contrast injection into the CS (Figure 13.17, Videos 13.41 through 13.44). In the RAO view, the IAS is viewed en face with the CS os located anteroinferiorly with respect to the fossa ovalis. As usual, the TSP site should be posterior to the noncoronary aortic cusp. However, the puncture site is higher than the usual site when the CS is dilated. The site is just above the lower end of the pigtail in the posterosuperior aspect of the FO in the RAO view midway between the pigtail and spine46 (Figure 13.19c). The correct site can further be confirmed by septal staining (Figure 13.19b) and by TTE. As the TSP site is higher, difficulties may be encountered during LV entry. As such, the OTW technique may come in handy (Figure 13.19d).
Coronary Sinus Defect, Premature Restriction of Foramen Ovale and Cysto-Colic Peritoneal Band
Published in Fetal and Pediatric Pathology, 2023
The coronary sinus is formed by coalescence of venous tributaries comprised of a small, middle, great, and oblique cardiac vein; the left marginal vein; and the left posterior ventricular vein [5,6]. Together with the vena cavae (superior and inferior), the coronary sinus delivers deoxygenated blood to the right atrium [6]. Unroofed coronary sinus is a congenital cardiac anomaly first described by Raghib et al [1]. There is an overwhelming association of this anomaly with persistent left superior vena cava that drains the left internal jugular and subclavian veins into the coronary sinus [7]. A persistent left superior vena cava occurs in 0.1–0.5% of the general population, with 8% draining into the left atrium [2]. The morphologic type of unroofed coronary sinus have been classified as Kirklin and Barratt-Boyes types whereby (1) type I is completely unroofed with persistent left superior vena cava; (2) type II is completely unroofed but without persistent left superior vena cava; (3) type III shows partially unroofed midportion; and (4) type IV shows partially unroofed terminal portion [2,8,9].
Update on shunt closure in neonates and infants
Published in Expert Review of Cardiovascular Therapy, 2021
Karim A. Diab, Younes Boujemline, Ziyad M. Hijazi
Systemic venous anomalies can result in significant cyanosis if they connect anomalously to the left sided cardiac structures. This can occur at any level of the systemic venous system including the inferior vena cave, right superior vena ccava, or persistent left superior vena cava when they connect anomalously to the pulmonary venous system or left atrium [127–130]. Although these lesions might not be clinically apparent early in life, they can present in the newborn period with significant cyanosis. Figure 5 depicts such a case of an anomalous connection of a persistent left superior vena cava to the left atrium in an infant who presented with severe cyanosis. Although rare with few cases reported in the literature, percutaneous or surgical correction is indicated to prevent hypoxemia and volume overload.
Cardiac Findings in Fetal and Pediatric Autopsies: A 15-Year Retrospective Review
Published in Fetal and Pediatric Pathology, 2019
Considering all defects, persistent left superior vena cava was the single most common malformation, either as an isolated finding or associated with other cardiac defects. The frequency of persistent left superior vena cava is 0.1–0.5% in the general population and is associated with 4–11% in patients with congenital heart defects, in particular right and left atrial isomerism [16,20,21]. Other studies have reported a lower frequency, which in part may be related to the fact that persistent left superior vena cava has sometimes been considered a variation rather than a malformation with potentially functional significance [8,22]. Still other studies have considered persistent left superior vena cava as a part of complex cardiac defects such as heterotaxy syndrome (right and left atrial isomerism) and have not counted this defect as a separate entity [4,11]. Taken together, this might explain why the frequency of persistent left superior vena cava was higher in our study. The known association of this defect with extracardiac malformations and chromosome abnormalities suggests that, while generally not clinically significant as an isolated finding, a persistent left superior vena cava does imply abnormal cardiac development and, if present, should prompt a search for cardiac and extracardiac malformations, including at post mortem examination [3,16,21].