Single ventricle physiology offers limited options with significant morbidity and mortality. The Glenn shunt is a mid-stage procedure for the Fontan circulation. With persistent left Superior Vena Cava (SVC) and Azygos continuation of the Inferior Vena Cava (IVC) to the SVC, needs the development of bilateral bidirectional Glenn shunts. Fifteen patients underwent Bidirectional Glen shunt procedure over a period of ten years. Of these two were found to have left SVC along with interrupted IVC and its Azygos continuation to the SVC. These two patients underwent the Kawashima operation. A seven years old girl was admitted via emergency with severe cyanosis. She had tricuspid atresia, azygos continuation of IVC with single ventricle physiology. She had a complicated postoperative course with prolonged hospital stay after bilateral bidirectional Glen shunt (Kawashima operation). She successfully has completed Fontan after 5 years. The second case ofa 7 year old girl had elective surgery for DORV (double outlet right ventricle), pulmonary atresia and azygos continuation of IVC and persistent left SVC. She had an uneventful postoperative course. The Kawashima operation is an established surgical procedure to deal with bilateral SVCs. The procedure is safe; outcome is favourable and prepares the patient for total cavo-pulmonary shunt

Amanullah, Muneer

Atiq, Mehnaz

Fatimi, Saulat

Shahabuddin, Syed

eCommons@AKU

eCommons@AKUSection of Cardiothoracic Surgery Department of SurgeryJanuary 2009Kawashima operation: functional modification ofbidirectional Glen shunt with left superior venacava in single ventricular morphologySyed ShahabuddinAga Khan UniversitySaulat FatimiAga Khan UniversityMehnaz AtiqAga Khan UniversityMuneer AmanullahAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_cardiothoracicPart of the Surgery CommonsRecommended CitationShahabuddin, S., Fatimi, S., Atiq, M., Amanullah, M. (2009). Kawashima operation: functional modification of bidirectional Glenshunt with left superior vena cava in single ventricular morphology. Journal of the Pakistan Medical Association, 59(1), 43-5.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_cardiothoracic/22convalescent phase starts when all the clinical signs haveresolved and ends when the laboratory abnormalities havereturned to normal usually in four to six weeks after theonset of disease.5The two most commonly missing symptoms inatypical cases are cervical lymphadenopathy andpolymorphous rash while mucosal changes are nearlyalways found.6 These symptoms were also missing in ourpatient, although our patient had a history of conjunctivitisin the beginning of the illness. Some of the other clinicalfeatures which may be present are aseptic meningitis, facialpalsy, pleural effusion, acute renal failure, hepatitis,pancreatitis and jaundice. Mucous membrane changes aremost common, occurring in 90% of typical and incompleteKawasaki disease. The laboratory findings in incompletecases are the same as the typical ones.7 Early recognition ofthese cases is important as these children may suffer frommyocardial infarction or unexplained death years later andsome cases have actually been diagnosed at autopsy. Theprincipal cause of death in Kawasaki disease is due tomyocardial infarction.8Recovery is usually complete in children who do nothave detectable coronary vasculitis and second attacksoccur only rarely. Intravenous immune globulin is known tobe safe and its early use in patients with suspectedincomplete Kawasaki disease is appropriate.9 The frequencyof coronary artery abnormalities can be reduced from 25%to less than 5% by early treatment with intravenous immuneglobulin. ConclusionA diagnosis of incomplete Kawasaki disease shouldbe considered in all febrile children in whom some but notall features of Kawasaki disease are present. It has beenrecommended that in children less than six months of agewith fever greater than 7 days and children older than 6months with fever of more than 5 days should beinvestigated for Kawasaki disease. Early recognition andtreatment of Kawasaki disease can reduce the developmentof potentially life threatening coronary abnormalities, andinstitute prompt treatment. References1. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantileacute febrile mucocutaneous lymph syndrome (MLNS) prevailing in Japan.Pediatrics   1974; 54:271-6. 2. Taubert KA, Shulman ST. Kawasaki Disease. Am Fam Physician 1999;59:3093-102.3. Rowley AH, Gonalez-Crussi F, Gidding SS, Duffy CE, Schulman ST.Incomplete Kawasaki disease with coronary artery involvement. J Pediatr1987; 110: 409-13.4. Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, et al.Kawasaki Disease: an evidence based approach to diagnosis, treatment, andproposal for future research. Arch Dis Child 2002; 86:286-90.5. J Harry Baumer. Kawasaki disease: What to do with incomplete cases? ArchDis Child.Ed.Pract.2005; 90; 102-104.6. Joffe A, Kabani A,  Jadavji T. Atypical and complicated Kawasaki disease ininfants. Do we need criteria? West J Med 1995 ; 162 :322-7.7. Kucinska B, Wroblewska-Kaluzewska M. What do we know about Kawasakidisease? Med Sci Monit 2000; 6 :1227-318. Oates-Whitehead RM, Baumer JH, Hainess L, S Love, IK Machonochie, AGupta, K roman, JS Dua, I Flynn. Intravenous immunoglobulin for thetreatment of Kawasaki disease in children. Cochrane Database of SystematicReviews 2003, issue 4.9. Freeman AF, Shulman ST. Recent developments in Kawasaki disease. CurrOpin Infect Dis 2001 ; 14 :357-61.10. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC,et al. Diagnosis, treatment, and long- term management of Kawasaki disease:a statement for health professionals from the Committee on RheumaticFever, Endocarditis, and Kawasaki disease, Council on CardiovascularDisease in the Young, American Heart Association. Pediatrics 2004;114:1708-33.Vol. 59, No. 1, January 2009 43Case ReportKawashima operation: Functional modification of Bidirectional Glenshunt with left superior vena cava in single ventricular morphologySyed Shahabuddin, Saulat Fatimi, Mehnaz Atiq, Muneer AmanullahDivision of Congenital Cardiac Surgery, Department of Surgery, The Aga Khan University Hospital, Karachi.AbstractSingle ventricle physiology offers limited optionswith significant morbidity and mortality. The Glenn shunt isa mid-stage procedure for the Fontan circulation. Withpersistent left Superior Vena Cava (SVC) and Azygoscontinuation of the Inferior Vena Cava (IVC) to the SVC,needs the development of bilateral bidirectional Glennshunts. Fifteen patients underwent Bidirectional Glen shuntprocedure over a period of ten years. Of these two werefound to have left SVC along with interrupted IVC and itsAzygos continuation to the SVC. These two patientsunderwent the Kawashima operation.A seven years old girl was admitted via emergencywith severe cyanosis. She had tricuspid atresia, azygoscontinuation of IVC with single ventricle physiology. Shehad a complicated postoperative course with prolongedhospital stay after bilateral bidirectional Glen shunt(Kawashima operation). She successfully has completedFontan after 5 years. The second case of a 7 year old girl hadelective surgery for DORV (double outlet right ventricle),pulmonary atresia and azygos continuation of IVC andpersistent left SVC. She had an uneventful postoperativecourse. The Kawashima operation is an established surgicalprocedure to deal with bilateral SVCs. The procedure issafe; outcome is favourable and prepares the patient for totalcavo-pulmonary shunt.  IntroductionSingle ventricular physiology is associated with ashorter life expectancy. It includes variety of complexcongenital heart diseases where only one ventricle isresponsible for supporting both systemic and pulmonarycirculation. However some of the anatomical biventricularhearts require to be managed on principles of singleventricle.The role of Bidirectional Glen (superior vena cava topulmonary artery) shunt has been well established as anintermediate palliation in patients with single ventricularphysiology.1 We have already established the safety ofbidirectional Glen shunt in a single ventricle.2 Themanagement of such patients is even more challenging forcongenital cardiac surgeons in presence of bilateral SVC's.Two children underwent bilateral bidirectional cavopulmonary shunt operation for tricuspid atresia and avariant of double outlet right ventricle. The first childunderwent salvage bilateral bidirectional Glen shunt withexpected stormy postoperative course. The second child hadan elective uneventful surgery. These children had bilateralsuperior vena cavae, interrupted inferior vena cava withAzygos continuation of IVC. This modified bilateralbidirectional Glen shunt is also known as the Kawashimaoperation.3 We describe our surgical strategy in two caseswith presence of left superior vena cava and azygoscontinuation of the IVC in patients with a single ventricularmorphology.Case-1A seven year old girl was diagnosed to haveTricuspid Atresia with Ventricular Septal Defect (VSD),pulmonary stenosis, Azygos continuation of IVC, and asingle ventricle physiology. She had a modified BlalockTaussig (BT) shunt, that is, subclavian artery to pulmonaryartery in infancy. She presented with progressivelyincreasing cyanosis and failure to thrive. She was advisedsurgical intervention abroad. On her way to the airport shebecame unwell and was brought to the emergency, room ofAga Khan Hospital with severe cyanosis. While she wasbeing wheeled to the operating room for a BT shuntoperation, she had a cardiopulmonary arrest necessitatingcardiopulmonary resuscitation (CPR). While CPR wasbeing performed, cardiopulmonary bypass (CPB) wasestablished. This was done with aortic and right atrialvenous cannulae after full systemic heparinization(300U/Kg). Instead of a BT shunt a bilateral Glen shunt(BDG) was thought to be more beneficial. In the presence ofbilateral SVC's three venous cannulae were used. The rightand left SVC's were divided at their junction with atria andanastomosed to right and left pulmonary artery respectivelyFig-1a & 1b. She was weaned off cardiopulmonary supportwith heavy doses of inotropic support. She had re-exploration of the mediastinum for bleeding and wasextubated on first post-operative day. She had a prolongedstay in the Intensive Care Unit (ICU) to optimize cardiac44 J Pak Med AssocFigure 1a: Line diagram to show additional (third) left SVC cannula.Figure 1b: Line diagram showing bilateral cavopulmonary shunt.function, and was discharged on the 17th post-operativeday. She did well at follow up and subsequently underwentcompletion of Fontan operation after 5 years. Case-2Another seven years old girl was diagnosed to haveDORV, Pulmonary atresia and azygos continuation of IVCinto a persistent left SVC. She had presented at birth withcyanosis and underwent construction of a modified BTshunt at four days of age. As she grew older her cyanosisworsened. She was evaluated regarding further surgicalintervention and her anatomical lesions were foundunsuitable for biventricular repair. It was planned to treather in line of single ventricle. A decision was made toperform Bidirectional Glenn shunt to improve pulmonaryblood flow and prepare her for future Fontan circulation.She underwent elective bilateral bidirectional Glen shunt oncardiopulmonary bypass. She came off CPB on small doseof inotropes. Her postoperative course was uneventful. Shewas extubated on first postoperative day and was dischargedon 6th post-op day. She has been asymptomatic on followup. She will be requiring completion of Fontan operation inthe future. DiscussionFontan in Bordeaux (France) in 1968 performedtotal cavo-pulmonary shunt by diverting SVC and IVCblood to the pulmonary artery for right heart bypass and hasremained of great value in patients with single ventriclephysiology.4 However, a group of children withuniventricular heart are at high risk for Fontan procedure.These children require interim palliation with bidirectionalGlen shunt which in turn prepares these children forcompletion of Fontan at a later stage.  We described twocase reports about children with heterotaxy syndrome5having intra-cardiac defects, right and left SVC along withazygos continuation of IVC managed with staged surgicalprocedure. The majority of patients entering theuniventricular heart protocol have to undergo various formsof surgical procedures to balance and optimize pulmonaryand systemic blood flow. Initial palliations to increasepulmonary blood flow in the presence of severely reducedpulmonary perfusion include aorto-pulmonary shuntprocedures similar to our patients who underwent modifiedBT shunts in the immediate post-natal period. Subsequentlyboth of them underwent bilateral bidirectional Glenn shunt,facilitating completion of Fontan and one of the patient hashad this procedure and the other patient will be requiring thesame in the near future. It is questionable; whether in thepresence of IVC continuation of azygos vein, completion ofFontan is necessary? It is important to have completionFontan, as pulmonary arterio-venous fistula develops andcollaterals from SVC and the hepatic veins, drain into theright atrium contributing to substantial cyanosis andhypoxaemia specially with exercise.6 The ultimate goal is tooff load the systemic ventricle and completely divert thevenous return to pulmonary circulation. In the Indian Sub-continent, severe complexcongenital cardiac problems are considered uncorrectableand hence several children are deprived of surgicalcorrection or palliation. Our report of two cases is an effortto provide palliation to such children and make themparticipate in the society at a better functional level. To conclude, in patients with this unusual anatomy,the Kawashima operation is safe and associated with goodoutcome. It also prepares the patient for future Fontancirculation by establishing a near total cavo-pulmonaryshunt.AcknowledgementWe are grateful to Dr. Muneer Amanullah for the linediagrams.FootnotesPresented at 6th Biennial International Conferenceof Pakistan Society of Cardiovascular and ThoracicSurgeons, Karachi, Pakistan, 14 - 17,  December, 2006.References1. Lamberti JJ, Spicer RL, Waldman JD, Grehl TM, Thomson D,  George L,et al.The bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg 1990; 100:22-30.2. Khan G, Ali SS, Fatimi SH. Bidirectional cavopulmonary shunt for cyanoticheart diseases: surgical experience from a developing country. J Pak MedAssoc 2003 ; 53:506-9. 3. Kawashima Y, Kitamura S, Matsuda H, Shimazaki Y,  Nakano S, Hirose H.Total cavopulmonary shunt operation in complex cardiac anomalies: A newoperation. J Thorac Cardiovasc Surg 1984; 87: 74-81. 4. Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstructionof the right ventricular outflow tract in tricuspid atresia: a report of 5 patients.Ann Thorac Surg 1980; 29:317-215. Bartram U, Wirbelauer J, Speer CP. Heterotaxy Syndrome - asplenia andpolysplenia as indicators of visceral malposition and complex congenital heartdisease. Biol Neonate 2005; 88:278-90.6. McElhinney DB, Kreutzer J, Lang P, Mayer JE Jr, del Nido PJ, Lock JE.Incorporation of the hepatic veins into the cavopulmonary circulation inpatients with heterotaxy and pulmonary arteriovenous malformations after aKawashima procedure. Ann Thorac Surg 2005; 80: 1597 - 603.Vol. 59, No. 1, January 2009 45

Kawashima operation: functional modification of bidirectional Glen shunt with left superior vena cava in single ventricular morphology

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Kawashima operation: functional modification of bidirectional Glen shunt with left superior vena cava in single ventricular morphology

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