Unusual Combination of Holt-Oram Syndrome and Persistent Left Superior Vena Cava

Thal, Sergio; Boyella, Ravichandra; Arsanjani, Reza; Thai, Hoang; Juneman, Elizabeth; Movahed, Mohammad Reza; Goldman, Steven

doi:10.1111/j.1747-0803.2011.00594.x

Cited by 5 publications

(3 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Reports in the literature concerning persistent LSVC are centered on difficulties, in patient without congenital heart disease, in placing central venous catheter or pacemaker lead insertions or related complications [12,15,17,22]. In contrast, there are many reports of persistent LSVC associated with rare combination of congenital heart disease or reports on persistent LSVC and associated anomalies of the coronary sinus [2,3,21]. The global impact of persistent LSVC has, to our knowledge, never been evaluated during the perioperative and postoperative period of cardiac surgery for congenital heart disease in children.…”

Section: Introductionmentioning

confidence: 99%

Persistent Left Superior Vena Cava in Cardiac Congenital Surgery

et al. 2013

View full text Add to dashboard Cite

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.

show abstract

Section: Introductionmentioning

confidence: 99%

Persistent Left Superior Vena Cava in Cardiac Congenital Surgery

et al. 2013

View full text Add to dashboard Cite

show abstract

“…It can occur in isolation or in association with other CHDs; the associated right-to-left shunt presents clinically as desaturation with some patients occasionally presenting with signs of systemic embolisation. Previously reported genetic associations with persistent left-sided caval vein and a normal coronary sinus include the Holt–Oram and Coffin–Siris syndromes; 12 however, the associations with these two syndromes and a left caval vein to a de-roofed coronary sinus have not yet been reported.…”

Section: Discussionmentioning

confidence: 93%

Anatomical considerations for the management of a left-sided superior caval vein draining to the roof of the left atrium

Saundankar

Salmon

et al. 2016

Cardiol Young

View full text Add to dashboard Cite

Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

show abstract

“…ASDs and VSDs are the most commonly described heart lesions. Occasionally, other forms of CHD have been reported, including hypoplastic left heart, persistence of the left superior vena cava, mitral valve prolapse, pulmonary stenosis, tetralogy of Fallot, truncus arteriosus, coarctation of the aorta, total anomalous pulmonary venous return, patent ductus arteriosus, tricuspid atresia, and AVSDs (Smith et al 1979;Ruzic et al 1981;Sahn et al 1981;Glauser et al 1989;Basson et al 1994;Newbury-Ecob et al 1996;Bruneau et al 2001;Patel et al 2012;Thal et al 2012). Missense, insertion, deletion, and chromosomal translocation mutations have all been reported.…”

Section: T-box Transcription Factors and Chdmentioning

confidence: 99%