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CARDIOVASCULAR ANESTHESIA

Persistent Left Superior Vena Cava in a Patient with a History of Tetralogy of Fallot

Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina

Address correspondence and reprint requests to Marek Brzezinski, MD, Department of Anesthesiology, Box 3094, DUMC, Duke University Medical Center, Durham, NC 27710. Address e-mail to brzez001{at}mc.duke.edu.

A 42-year-old female patient was referred to our institution with recent progressive dyspnea and fatigue. Her history was significant for 3 previous surgeries for repair of tetralogy of Fallot at age 3, 9, and 17. The patient was informed after the last surgery that she has a persistent left superior vena cava (PLSVC). Diagnostic work-up at our institution with chest wall echocardiogram, magnetic resonance imaging, and cardiac catheterization demonstrated severe pulmonary insufficiency, right ventricular dysfunction, and a patent foramen ovale (PFO). These imaging techniques also confirmed the presence of the PLSVC draining to the coronary sinus (CS). Electrophysiology study identified foci of ventricular tachycardia in the endocardial surface of the right ventricle. The patient was scheduled for an elective pulmonic valve replacement, closure of the PFO, and endocardial ablation for ventricular arrhythmia.

Intraoperative transesophageal echocardiography revealed a normal sized left ventricle and an enlarged right ventricle, both with a good function. The CS was enlarged. The presence of the PLSVC was confirmed by injection of agitated normal saline as echo contrast into the left arm vein. Consistent with the diagnosis of PLSVC, the contrast was seen in the CS instead of draining into the right superior vena cava (Fig. 1) (video loop; see supplemental data at www.anesthesia-analgesia.org). Other pathology confirmed included a PFO with bidirectional shunt and a severely incompetent pulmonic valve. No residual postoperative defects were demonstrated. The patient tolerated the procedure well, and her postoperative course was uncomplicated.

View larger version (65K): [in this window] [in a new window]   Figure 1. Midesophageal modified 4-chamber view predominantly showing right-sided structures. The right ventricle (RV) and the right atrium (RA) are markedly enlarged. The large coronary sinus (CS) can be seen wrapping around the left atrium. Panel A illustrates the baseline noncontrast still image. Contrast study with agitated normal saline injection into a left arm vein shown in panel B demonstrates the drainage into the CS instead of the right superior vena cava, confirming a diagnosis of left persistent superior vena cava.

Persistent LSVC, the most common anomaly of systemic venous return, is an uncommon congenital pathology with a prevalence of 0.3–0.5% in patients with normal heart and 1.3–4.5% in patients with congenital heart disease. Tetralogy of Fallot was found in up to 25% of patients with PLSVC (1). A failure of obliteration of the LSVC in late embryologic life results in a PLSVC draining the left upper extremity and left part of the head into the CS and thence into the right atrium, causing a dilation of the CS (2). Usually, a PLSVC, when not associated with other congenital anomalies, is asymptomatic and hemodynamically insignificant. However, PLSVC has important clinical implications. It may complicate placement of cardiac catheters or pacemaker leads and also represents a contraindication for a retrograde administration of cardioplegia. The diagnosis of PLSVC is classically made by injecting contrast into the left upper extremity (e.g., left basilic vein) and demonstrating drainage of the contrast into the CS. The CS on the two-dimensional echocardiography is enlarged (>1 cm in diameter). An enlarged CS can also be found in patients with right ventricular dysfunction and right atrial hypertension. On the basis of the established safety record of the saline microbubble contrast test (3), we felt that it was an acceptable procedure for demonstrating the presence of a PLSVC by echocardiography. To reduce right-to-left shunt we positioned the patient in Trendelenberg position. A PLSVC is a rare anomaly with significant implications for anesthetic management. The case presented will help familiarize anesthesiologists with the typical echocardiographic presentation, diagnostic approach, and clinical consequences.

	Footnotes

 
Supplemental data available at www.anesthesia-analgesia.org.

Accepted for publication October 29, 2004.

	References

Top References

 

1. Fraser RS, Dvorkin J, Rossall RE, Eidem R. Left superior vena cava: a review of associated congenital heart lesions, catheterization data and roentgenologic findings. Am J Med 1961;31:711–6.[Medline]
2. Gerber TC, Kuzo RS. Images in cardiovascular medicine: persistent left superior vena cava demonstrated with multislice spiral computed tomography. Circulation 2002;105:e79.[Free Full Text]
3. Nanda NC, Carstensen EL. Echo-enhancing agents: safety. In: Nanda NC Schlief R, Goldberg BB, eds. Advances in echo imaging using contrast enhancers. 2nd ed. Dordrecht, the Netherlands: Kluwer, 1997:115–31.

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This Article Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via ISI Web of Science (2) Citing Articles via Google Scholar Google Scholar Articles by Brzezinski, M. Articles by Swaminathan, M. Search for Related Content PubMed PubMed Citation Articles by Brzezinski, M. Articles by Swaminathan, M. Related Collections Cardiovascular Heart Monitoring (Cardiac)

Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins with the assistance of Stanford University Libraries' HighWire Press®. Copyright 2006 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999