Anomalous connection of the superior vena cava to the morphologic left atrium is a rare congenital systemic venous abnormality. As opposed to pulmonary venous anomalies, the significant right-to-left shunt in these patients warrants a correction early in life. Optimal repair technique for combined pulmonary and systemic venous anomalies is not defined yet. Herein, we describe a neonate with such a diagnosis treated with cavoatrial anastomosis, known as Warden procedure with excellent results

Fonseca Escalante, Elizabeth

Gerrah, Rabin

Gorenflo, Matthias

Loukanov, Tsvetomir

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Case ReportThe Cavoatrial AnastomosisProcedure in Anomalous Connectionof Superior Vena Cava to Left AtriumRabin Gerrah, MD1, Elizabeth Fonseca Escalante, MD1,Matthias Gorenflo, MD1, and Tsvetomir Loukanov, MD11Division of Pediatric Cardiac Surgery, Department of CardiacSurgery, Heidelberg University, Heidelberg, GermanyDOI: 10.1177/2150135119878102AbstractAnomalous connection of the superior vena cava to the morphologicleft atrium is a rare congenital systemic venous abnormality. Asopposed to pulmonary venous anomalies, the significant right-to-leftshunt in these patients warrants a correction early in life. Optimalrepair technique for combined pulmonary and systemic venousanomalies is not defined yet. Herein, we describe a neonate with sucha diagnosis treated with cavoatrial anastomosis, known as Wardenprocedure with excellent results.IntroductionThe cavoatrial anastomosis, known as Warden procedure, was origi-nally designed to repair the anomalous connection of the pulmonaryvein (PV) to the superior vena cava (SVC) when baffling of the PV tothe left atrium (LA) is a challenge. Herein, we present a case of aneonate in whom the cavoatrial anastomosis technique was used torepair a rare systemic venous drainage along with pulmonary venousanomaly.Case ReportA one-week-old, 3-kg full-term newborn was admitted with cyanosisand hypoxemia (oxygen saturation 69%). Further workup revealedanomalous connection of the right-sided SVC to the LA, presenceof a superior sinus venosus defect with left-to-right shunt, patent fora-men ovale, and patent ductus arteriosus in a situs solitus heart with noother malformations.On day of life 25, the patient was taken to operating room forcorrection of his combined anomalous systemic and pulmonaryvenous drainage. The anatomy was remarkable for anomalous con-nection of the SVC to the LA, anomalous connection of threeminuscule PVs to anterolateral aspect of the SVC, and septaldefects as described by echocardiogram (Figure 1; supplementalvideo). In this setting, the decision to perform a Warden procedureseemed the most appropriate. Initially, the ductus arteriosus wasligated. The SVC was mobilized and transected above the junctionof the highest connecting PV and distal stump was over sewn. Theright atrial (RA) appendage was explored with a horizontal inci-sion and a direct anastomosis was constructed between the prox-imal end of the SVC and the RA appendage. This anastomosiscould accommodate a 10-mm Hegar dilator easily.Trabeculations were resected to ensure unobstructed flow to theRA and to avoid late stenosis. This anastomosis diverted the SVC flowto the RA in a normal fashion. The sinus venosus septal defect wasclosed using an autologous pericardial patch, which baffled the flowfrom the anomalous PVs to the LA. The patent foramen ovale wasclosed primarily. This combined strategy of both Warden and patchrepair achieved a normal anatomy of this complex dual venous drai-nage anomaly.Upon completion of this repair, normal sinus rhythm, fulloxygenation with saturation of 100%, and normal RA pressureswere observed. The postoperative course was remarkable for rightdiaphragmatic paresis with mild respiratory effect with no need forfurther treatment. The patient was discharged home on postopera-tive day 13 in good condition. In a six-month follow-up, thepatient was in good condition; on echo, the SVC diameter wasmeasured as 4 mm and the anastomosis was open with a diameterof 7 mm.CommentsAnomalous connection of the SVC to the LA is extremely rare,first reported in 19561 and then in other reports.2–4 Unlike pulmon-ary venous anomalies that create a left-to-right shunt and often arediagnosed later in life, the anomalous SVC to LA creates a largeright-to-left shunt by diverting the majority of upper body systemicvenous drainage to the LA, and potentially cyanosis. The exactetiology of this lesion is not known; however, it is believed tobe related to abnormal development of sinus venosus. During theembryogenesis, the abnormal resorption of the cephalic portion ofthe right sinus venosus valve possibly creates a seal that preventsthe SVC connection to the RA, resulting in this anomaly.4 Due tophysiologic significance of this anomaly, an early repair is war-ranted. Furthermore, beyond the problem of shunting, this lesionentails a potential of paradoxical embolism. For this reason, priorto correction of this anomaly, use of intravenous line in the upperextremity should be avoided to minimize the risk of systemic,especially cerebral emboli.The Warden procedure is an adjunct in repair of sinus venosusdefect when the high drainage of PV to SVC excludes the option ofan easy repair with baffling the pulmonary venous flow to the LA bymeans of a patch that closes the defect.5 Performing a Warden proce-dure has generally been reserved for limited indications due to itspotential for complications such as late stenosis (usually a result ofinsufficient resection and clearance of the trabeculations in the RAappendage) and sinoatrial node dysfunction with consequentdysrhythmia.For this anomaly, the Warden procedure seems to be the mostintuitive and reasonable repair, achieving nearly normal anatomy. Thecase of a four-year-old with some similarities to ours was reported byRecto et al; however, in that case, the SVC did not drain exclusively tothe LA.6 Yerebakan described an unusual case in which the right SVChad connections to both atria; some elements of a Warden procedurewere used to repair that anomaly.7 Braudo et al presented a case inwhich the right SVC pursued a tortuous course to connect to the LA,Submitted April 11, 2019; Accepted August 16, 2019.Corresponding Author:Rabin Gerrah, Division of Pediatric Cardiac Surgery, Department of Pediatricand Congenital Cardiac Surgery, Heidelberg University, INF 110, Heidelberg69120, Germany.Email: rgerrah@gmail.comand it was repaired by direct anastomosis of SVC to an L-shapedincision in the RA.4 Our case presents an ideal indication for theWarden procedure, and therefor expands its usage beyond the usualsetting of repair of sinus venosus defects. On the latest postoperativefollow-up, six months after the surgery, patient is doing well andechocardiography shows wide open anastomosis between the SVCand RA (Figure 2).Authors’ NoteBoth parents granted consent for publication of this case report.Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect tothe research, authorship, and/or publication of this article.FundingThe author(s) received no financial support for the research, author-ship, and/or publication of this article.Supplemental MaterialSupplemental material for this article is available online.Anomalous connecon of superior vena cava to le atrium, Before repairAer repair with Cavoatrial AnastomosisAo AoRVRASVCSVCPVsRAAPAPAIVIVLA LACAAFigure 1. Surgical view ofthe anomaly before andafter repair with cavoatrialanastomosis. Note theconnection of the superiorvena cava to thesuperoposterior aspect ofthe left atrium. Ao indicatesaorta; CAA, cavoatrialanastomosis; IV, innominatevein; LA, left atrium; PA,pulmonary artery; PVs,pulmonary veins; RA, rightatrium; RV, right ventricle;SVC, superior vena cava.Superior Vena CavaRight AtriumFigure 2. Postrepairechocardiographic view of the repairat follow-up showing a wide openconnection between the superior venacava and right atrium.804 World Journal for Pediatric and Congenital Heart Surgery 10(6)References1. Wood P. Diseases of the Heart and Circulation. 2nd ed. Philadel-phia, PA: Lippincott; 1956:457-458.2. Aminololama-Shakeri S, Wootton-Gorges SL, Pretzlaff RK, ReyesM, Moore EH. Right-sided superior vena cava draining into the leftatrium: a rare anomaly of systemic venous return. Pediatr Radiol.2007;37(3): 317-320.3. Van Praagh S, Geva T, Lock JE, Nido PJ, Vance MS, Van PraaghR. Biatrial or left atrial drainage of the right superior vena cava:anatomic, morphogenetic, and surgical considerations—report ofthree new cases and literature review. Pediatr Cardiol. 2003;24(4):350-363.4. Braudo M, Beanlands DS, Trusler G. Anomalous drainage of theright superior vena cava into the left atrium. Can Med Assoc J.1968;99(14): 715-719.5. Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternativemethod for repair of partial anomalous pulmonary venous connectionto the superior vena cava. Ann Thorac Surg. 1984;38(6): 601-605.6. Recto MR, Sobczyk WL, Yeh T. Right superior vena cava drainingpredominantly into the left atrium causing cyanosis in a youngchild. Pediatr Cardiol. 2004;25(2): 163-164.7. YerebakanC, FrankL,HibinoN, Jonas RA. Partial anomalous pulmon-ary venous connection with anomalous connection of the superior venacava to the left atrium. J Thorac Cardiovasc Surg. 2012;144(1): e1-e3.Gerrah et al 805

The Cavoatrial Anastomosis Procedure in Anomalous Connection of Superior Vena Cava to Left Atrium

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The Cavoatrial Anastomosis Procedure in Anomalous Connection of Superior Vena Cava to Left Atrium

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