European Journal of Echocardiography Advance Access originally published online on August 30, 2008
European Journal of Echocardiography 2009 10(1):160-162; doi:10.1093/ejechocard/jen226
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Obliteration of left superior caval vein draining to the left atrium during spontaneous closure of ventricular septal defect
Department of Paediatric Cardiology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK
Received 15 July 2008; accepted after revision 1 August 2008; online publish-ahead-of-print 30 August 2008.
* Corresponding author. Tel: +44 20 7352 8121, Fax: +44 20 7351 8503. Email address: j.kaski{at}ucl.ac.uk
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Abstract |
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Persistent left-sided superior caval veins (SVC) are present in 0.4% of the population. In the majority of cases, the persistent left SVC drains into the right atrium via the coronary sinus, but direct connection to the left atrium is also recognized. Previous reports have described re-opening of persistent left SVCs in patients with congenital heart disease following bidirectional cavopulmonary connection or Fontan-type procedures, suggesting that the lumen of the left SVC obliterates during embryological development, rather than disappears. The case described in this report is, to our knowledge, the first description of obliteration of the left SVC in post-natal life, associated with spontaneous closure of a ventricular septal defect. Our observation lends further support to the hypothesis that venous structures obliterate but do not completely disappear in foetal life.
Keywords: Echocardiography; Left superior vena cava; Paediatric; Congenital heart disease
A premature girl born at 28 weeks' gestation was found to have a murmur on Day 12 of life. She received a 6 day course of indomethacin for a presumed persistent arterial duct; however, the murmur persisted and she was transferred to our institution for evaluation. On examination, she was fully saturated with no evidence of heart failure and normal peripheral pulses. Auscultation revealed normal heart sounds with a grade 3/6 holosystolic murmur at the left lower sternal edge. Echocardiogram demonstrated situs solitus, normal intra-cardiac connections, and a 6 mm membranous ventricular septal defect (VSD) shunting left to right with a Doppler-derived pressure drop of 55 mmHg (Figure 1A). The left ventricle (LV) was dilated with impaired systolic function (fractional shortening 23%) (Figure 1B). There was a large left superior vena cava (SVC) draining to the roof of the left atrium with bidirectional flow (Figure 1C), a smaller right-sided SVC draining into the right atrium and no bridging innominate vein (Figure 1D).
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Treatment was commenced with angiotensin-converting enzyme inhibitor and diuretics, and she was reviewed at our institution 2 months later. At this time, she was thriving and asymptomatic with clinical features of a small VSD. Repeat echocardiogram showed that the VSD was almost entirely occluded by tricuspid valve tissue, with only a small residual left-to-right jet seen (Figure 2A). The LV was no longer dilated and systolic function was normal (Figure 2B). There was no longer any blood flow from the left SVC to left atrium (Figure 2C); instead, the left SVC drained into a bridging innominate vein, and thence to the right SVC (Figure 2D).
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During embryological development, the bilateral anterior cardinal venous system usually develops into a unilateral, right-sided system. In up to 0.4% of the general population, the left-sided system, manifest as the left SVC, persists.1,2 In the majority of cases, the persistent left SVC drains into the right atrium via the coronary sinus, but direct connection to the left atrium is also recognized.3 Previous reports have described re-opening of persistent left SVCs in patients with congenital heart disease following bidirectional cavopulmonary connection or Fontan-type procedures.1,4 It has been speculated that altered haemodynamics resulting from increased pressure gradients between the pulmonary and systemic venous circuit following establishment of cavopulmonary connections may account for this.1 These findings suggest that the lumen of the left SVC obliterates during embryological development, rather than disappears. The case described in this report is, to our knowledge, the first description of obliteration of the left SVC in post-natal life. We speculate that patency of the left SVC was maintained by elevated left atrial pressure, resulting from the VSD and impaired LV function. As the VSD became smaller and as LV function improved, left atrial pressure fell, leading to obliteration of the left SVC, and re-opening of the bridging innominate vein. The latter observation lends support to the hypothesis that venous structures obliterate but do not completely disappear in foetal life.1
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Acknowledgements |
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The authors are grateful to the patient's parents for consenting to the publication of this report.
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References |
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TopAbstractReferences |
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- Filippini LH, Ovaert C, Nykanen DG, Freedom RM. Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections. Heart (1998) 79:509–512.
[Abstract/Free Full Text] - Nsah EN, Moore GW, Hutchins GM. Pathogenesis of persistent left superior vena cava with a coronary sinus connection. Pediatr Pathol (1991) 11:261–269.[CrossRef][Medline]
- Mazzucco A, Bortolotti U, Stellin G, Gallucci V. Anomalies of the systemic venous return: a review. J Card Surg (1990) 5:122–133.[Medline]
- Magee AG, McCrindle BW, Mawson J, Benson LN, Williams WG, Freedom RM. Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors. J Am Coll Cardiol (1998) 32:502–508.
[Abstract/Free Full Text]
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