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Rare Presentation of Gerbode Defect in a 4-Month-Old Nigerian and a Review of the Literature

DOI: 10.1155/2013/564786

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A Gerbode defect is a very rare congenital anomaly which presents as a direct or an indirect type. We report a 4 month old Nigerian child who presented with poor feeding and failure to thrive and whose echocardiography confirmed an indirect Gerbode with a direct left ventricle to right atrial shunt due to adherent leaflets of the tricuspid valve. This was confirmed by surgery done outside the country. 1. Introduction The Gerbode ventriculoatrial defect is a very rare congenital anomaly [1]. It is described as a communication between the left ventricle and the right atrium. There are 2 types known, a direct and an indirect as reported by Gerbode et al. [2]. In a patient with a perimembranous ventricular septal defect (VSD) and a defect in the tricuspid valve, the shunt is from left ventricle to right ventricle then through the tricuspid valve into the right atrium. The communication thus occurs below the tricuspid valve. This is referred to as an indirect left ventricular—to—right atrial shunt while in a true or direct Gerbode, the blood in the left ventricle goes through the small area of the membranous septum where there is the potential for a left ventricular—to—right atrial shunt. This communication is above the tricuspid valve. There are contrasting reports of this defect being first reported in 1857 by Meyer [3] and by Thurman in the 19th century [4]. The first successful closure of such a defect was reported by Kirby et al. (using hypothermia and inflow occlusion) at the Hospital of the University of Pennsylvania in 1956 [5]. Gerbode (after whom the defect was named) was a noted surgeon at Stanford University who successfully operated on a series of patients with a left ventricular—to—right atrial shunt in 1958 [2]. According to the STS Congenital Heart Nomenclature and Database Project [6], the definition of a Gerbode defect is a true left ventricular—to—right atrial communication. We report a rare form of Gerbode defect in which a perimembranous VSD is restricted by the adherent septal and anterior tricuspid leaflet at the anterocommisural area resulting in an LV to RA jet, though through the tricuspid valve. 2. Case Report A four-month-old female infant was brought to a private paediatric hospital in Port Harcourt, Nigeria; by her mother with complaints of poor feeding and poor weight gain, weighing 4.5?kg (<3rd percentile) at presentation and 2.8?kg at birth. There was no history of fast breathing, cough, cyanosis; or loose excessive sweating. She is the only child of a nonconsanguineous couple delivered by emergency caesarean section at term

References

[1]  R. McKay, S. A. Battistessa, J. L. Wilkinson, and J. P. Wright, “A communication from the left ventricle to the right atrium: a defect in the central fibrous body,” International Journal of Cardiology, vol. 23, no. 1, pp. 117–123, 1989.
[2]  F. Gerbode, H. Hultgren, D. Melrose, and J. Osborn, “Syndrome of left ventricular-right atrial shunt. Successful repair of defect in five cases, with observation of bradycardia on closure,” Annals of Surgery, vol. 148, pp. 433–446, 1958.
[3]  H. Meyer, “Ueber angeborene enge oder verschluss der lungenarterienbahn,” Virchows Archiv, vol. 12, article 532, 1857.
[4]  J. Thurman, “Aneurysms of the heart,” Transactions of Medical Society of London, vol. 21, article 187, 1838.
[5]  C. K. Kirby, J. Johnson, and H. F. Zinsser, “Successful closure of a left ventricular-right atrial shunt,” Annals of Surgery, vol. 145, no. 3, pp. 392–394, 1957.
[6]  J. P. Jacobs, R. P. Burke, J. A. Quintessenza, and C. Mavroudis, “Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect,” Annals of Thoracic Surgery, vol. 69, no. 4, sypplement, pp. S25–S35, 2000.
[7]  J. J. Silbiger, M. Kamran, S. Handwerker, N. Kumar, and M. Marcali, “The gerbode defect: left ventricular to right atrial communication—anatomic, hemodynamic, and echocardiographic features,” Echocardiography, vol. 26, no. 8, pp. 993–998, 2009.
[8]  T. A. Riemenschneider and A. J. Moss, “Left ventricular-Right atrial communication,” The American Journal of Cardiology, vol. 19, no. 5, pp. 710–718, 1967.
[9]  O. M. Cheema, A. A. Patel, M. C. Su, and D. J. Shah, “Gerbode ventricular septal defect diagnosed at cardiac MR imaging: case report,” Radiology, vol. 252, no. 1, pp. 50–52, 2009.
[10]  P. Panduranga and M. Mukhaini, “A rare type of Gerbode defect,” Echocardiography, vol. 28, no. 6, pp. e118–e120, 2011.

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