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Daniel Tobler, Matthias Greutmann, Erwin Oechslin, The answer lies in the bubbles: a patient with superior sinus venosus defect, persistent left superior vena cava, and absent innominate vein, European Heart Journal, Volume 31, Issue 3, February 2010, Page 317, https://doi.org/10.1093/eurheartj/ehp502
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Extract
A 38-year-old woman with atypical chest pain and exertional dyspnoea was referred to our service for a diagnostic work-up. Transthoracic echocardiography showed an enlarged coronary sinus (CS) and enlarged right-sided heart chambers without obvious atrial septal defect (Panel A). After injection of agitated saline contrast into the left cubital vein, immediate appearance of bubble contrast was noted in the CS and the right atrium consistent with drainage of a persistent left superior vena cava into the CS (Panel B). This finding left the dilatation of the right-sided heart chamber unexplained. Additional injection of agitated saline into the right cubital vein was the critical diagnostic hint, when bubbles appeared simultaneously in both atria (Panel C). This finding is almost pathognomonic for a superior sinus venosus defect. The absence of bubble contrast in the left atrium after injection into the left cubital vein is consistent with the absence of an innominate vein, which was subsequently confirmed on cardiac magnetic resonance imaging (Panel D).
Comments
We wish to thank White and colleagues for their interest in our Cardiovascular Flashlight about the pattern of bubble contrast appearance in the setting of a superior sinus venosus defect.1 White et al criticize that in the setting of a superior sinus venosus defect simultaneous appearance of bubble contrast after injection into the right cubital vein would only occur in the setting of Eisenmenger-syndrome with shunt reversal or in an optimally timed release of the Valsalva manoeuvre. We disagree with this opinion. A sinus venosus defect is characterized by a deficiency of the infolding of the atrial roof and the key anatomical criterion for the diagnosis of sinus venosus defects is overriding of the mouth of the superior caval vein across the intact muscular border of the oval fossa. The interatrial communication is outside the confines of the oval fossa (extraseptal interatrial communication). Thus, the superior vena cava is committed to both atria and typically overrides the true interatrial septum. 2, 3 These specific patho-anatomic features of this uncommon defect explain the unique streaming pattern of blood from the superior vena cava and thus explain the simultaneous and immediate appearance of bubble contrast in both atria after injection into the right antecubital vein. This occurs without Valsalva manoeuvre and is not dependent on the presence of pulmonary hypertension. We agree that a transpulmonary shunt is a common cause for appearance of bubble contrast within the left atrium. The hallmark of a transpulmonary shunt is, however, delayed appearance in the left atrium, which is in sharp contrast of the finding of immediate, simultaneous appearance of bubble contrast in both atria in the setting of a superior sinus venosus defect. We completely agree that the finding of simultaneous bubble contrast in both atria in the setting of an unexplained dilatation of the right sided heart chambers should prompt further investigations, typically with transesophageal echocardiography or cardiac magnetic resonance imaging. However these tests should be performed at centres with experience and expertise in diagnosis and treatment of congenital heart disease by specifically trained cardiologists and radiologists familiar with the anatomy and pathophysiology of congenital heart lesions.4, 5 Concomitant defects, such as anomalous pulmonary venous connection(s) are common and surgeons rely on optimal preoperative information for the planning of successful surgical repair. Bubble contrast echocardiography with the pathognomonic findings as described in our case vignette is a cheap and reliable screening test to diagnose a superior sinus venosus defect for the general cardiologist confronted with the unexplained dilatation of the right sided heart chambers.
References
1. Tobler D, Greutmann M, Oechslin E. The answer lies in the bubbles: a patient with superior sinus venosus defect, persistent left superior vena cava, and absent innominate vein. Eur Heart J. 2010;31(3):317.
2. Oliver JM, Gallego P, Gonzalez A, Dominguez FJ, Aroca A, Mesa JM. Sinus venosus syndrome: atrial septal defect or anomalous venous connection? A multiplane transoesophageal approach. Heart. 2002;88(6):634-638.
3. al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML. Anatomical criteria for the diagnosis of sinus venosus defects. Heart. 1997;78(3):298-304.
4. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP, Jr., Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD, Smith SC, Jr., Jacobs AK, Adams CD, Anderson JL, Antman EM, Buller CE, Creager MA, Ettinger SM, Halperin JL, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Page RL, Riegel B, Tarkington LG, Yancy CW. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(23):e1-121.
5. Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A, Sorenson K, Kaemmer H, Thilen U, Bink-Boelkens M, Iserin L, Daliento L, Silove E, Redington A, Vouhe P, Priori S, Alonso MA, Blanc JJ, Budaj A, Cowie M, Deckers J, Fernandez Burgos E, Lekakis J, Lindahl B, Mazzotta G, Morais J, Oto A, Smiseth O, Trappe HJ, Klein W, Blomstrom-Lundqvist C, de Backer G, Hradec J, Parkhomenko A, Presbitero P, Torbicki A. Management of grown up congenital heart disease. Eur Heart J. 2003;24(11):1035-1084.
Conflict of Interest:
None declared
We read with interest the Cardiovascular Flashlight from Tobler et al. who, quite rightly, emphasize that the finding of right heart dilatation should always prompt an extensive search for an atrial septal defect or left to right shunt.
They draw particular attention, however, to the finding that bubble contrast appearing simultaneously in the right and left atria is 'almost pathognomonic for a superior sinus venosus defect'. We feel that this observation may not be helpful for readers approaching this diagnostic problem in a clinical setting.
As can be seen in the TOE image we provide (figure 1.) , in a case of ours with the same congenital anatomical variation, there is a clear left to right shunt - blue colour flow Doppler. Only if there were to be a forced and well timed release of Valsalva at the time of bubble contrast entering the right atrium, or if the patient has a right to left shunt e.g. classically with Eisenmenger syndrome (and therefore with much more advanced disease), may bubbles be seen in the left heart chambers. The authors do not make comment of this in their case.
Furthermore, a more common, and therefore more misleading cause of bubbles in the left heart in this situation may be because of transpulmonary shunting of bubble contrast. This occurs more often with gelofusine (or similar agents) than with saline, but remains a further confounding factor.
We would propose that readers enter into an exhaustive search for a left to right shunt in cases of unexplained right heart dilatation but that they should apply no reliance on bubble contrast in the left heart. Further imaging techniques such as TOE or CMR should then be employed for definitive diagnosis.
Please click here to see figure 1
Figure 1. Large sinus venosus atrial septal defect on TOE imaging in the bi-caval view. Colour flow Doppler analysis confirms a large blue jet of flow away from the probe and therefore a left to right shunt. In the normal physiological state without Valsalva bubble contrast would not shunt from right to left.
Conflict of Interest:
None declared