Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 year

Althaus, Ulrich

Berdat, Pascal A.

Carrel, Thierry

Mohacsi, Paul

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Case reportSuccessful heart transplantation in a patient with Ivemark syndrome combinedwith situs inversus, single atrium and ventricle after totalcavo-pulmonary connectionPascal A. Berdata, Paul Mohacsib, Ulrich Althausa, Thierry Carrela,*aClinic for Thoracic and Cardiovascular Surgery, University Hospital, CH-3010 Berne, SwitzerlandbDivision of Cardiology, University Hospital, Berne, SwitzerlandReceived 17 August 1998; accepted 30 September 1998AbstractHeart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. Wereport the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwentseveral prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technicalmodifications was performed successfully at the age of 19 years. Ó 1998 Elsevier Science B.V. All rights reservedKeywords: Situs inversus; Orthotopic cardiac transplantation; Congenital heart disease1. IntroductionEnd-stage heart failure patients suffering from congen-ital heart disease accounts for less than 5% of the overallnumber of cardiac transplant candidates. There may be aslightly increasing incidence in future years due to anexpected rising of patients with failure of Fontan circulationand of patients with late systemic ventricular failure follow-ing atrial repair because of transposition of the greatarteries.Ivemark syndrome is a multiple organ syndrome asso-ciated with splenic abnormalities, complex cardiac pathol-ogy and abnormality of the abdominal organs. Theincidence of Ivemark syndrome is thought to be approxi-mately 1/6000 deliveries and the cause is unknown. Theprognosis depends mainly on the degree of malformationof the heart [1].We present a patient with complex cardiac malformationand situs inversus associated to Ivemark syndrome. Heunderwent successful orthotopic heart transplantationafter several previous palliative interventions, despite anaccidentally transplanted ABO-incompatible cardiac allo-graft.2. Case reportA 19-year-old patient presented with Ivemark syndromeand end-stage heart failure. The cardiac malformation in-cluded a situs inversus totalis with single atrium, singleventricle, transposition of the great arteries, persistentright superior vena cava and a fistula between the rightcoronary artery and the pulmonary artery. Main symptomswere dyspnea NYHA functional class III and pre-syncopes.Treatment of heart failure included digoxin, diuretics, a CE-inhibitor and a b-blocker at low dosage.2.1. Previous surgical historyAt the age of 11 years (in 1989), the patient presentedwith increasing cyanosis and a modified Blalock–Taussigshunt was constructed between the right subclavian arteryEuropean Journal of Cardio-thoracic Surgery 14 (1998) 631–6341010-7940/98/$19.00 Ó 1998 Elsevier Science B.V. All rights reservedPII S1010-7940(98)00244-9* Corresponding author. Tel.: +41-31-632-9268; fax: +41-31-382-0279;e-mail: thierry.carrel Ó insel.chand the right pulmonary artery, using a Gore-Tex shunt of 5mm in diameter. The child recovered well until 3 years later,when cyanosis returned because of shunt occlusion. At thattime, cardiac catheterism showed still normal pulmonarypressure and resistance, a normal function of the singleventricle with absent regurgitation of the atrio-ventricularvalve. Therefore, a Fontan-like palliation was considered. Atwo-stage total cavo-pulmonary connection was performedin 1992 included a bilateral, bidirectional cavo-pulmonaryanastomosis and a connection between the inferior venacava and the main pulmonary artery using a 18 mm Gore-Tex fenestrated (4 mm) tube graft lying within the singleatrium (Fig. 1).2.2. Present historyAfter uneventful recovery, medication could be stoppeduntil increasing dyspnoe and rapidly progressive heart fail-ure occurred in 1997. Echocardiography showed severe sys-temic atrio-ventricular valve regurgitation and severelyimpaired left ventricular function (ejection fraction 30%).Since any additional drug treatment nor surgical approachcould have improved intractable heart failure, the patientwas considered a cardiac transplant candidate and under-went transplantation in May 1997.2.3. Surgical techniqueThe ascending aorta, both superior venae cavae and theright iliac vein were cannulated and cardiopulmonarybypass conducted in mild hypothermia (28 ° C). Extendedcardiectomy was performed, removing the prior bilateralsuperior cavo-pulmonary anastomosis. The single atriumwas opened and the intraatrial Gore-Tex graft was comple-tely excised.Donor cardiectomy was modified in order to includecomplete inflow and outflow tissue in the explant. Trans-plantation started with a running suture connecting the sin-gle, systemic atrium of the recipient with the left atrium ofthe donor heart; this suture was performed in a way toexteriorize the left-sided inferior vena cava out of the singleatrium. Due to exceeding tissue, the inferior vena cavacould be anastomosed end-to-end without tension. Twoseparate pulmonary artery anastomoses were performedrather peripherally with running suture of 5.0 polypropy-lene. Then, both superior venae cavae were anastomosed,the innominate vein of the donor heart assuming restorationof the continuity to the left recipient superior vena cava.Since the innominate donor vein was lying between theaorta and the main pulmonary artery, a short ring-reinforcedGore-Tex graft was interponed to avoid compression.Finally end-to-end aortic anastomosis was performed andwarm blood reperfusion cardioplegia was instilled beforeaortic declamping (total cold ischemic time was 85minutes). Fig. 2 summarizes the most important steps ofcardiac grafting. Sinus rhythm appeared rapidly duringreperfusion and weaning from extracorporeal circulationwas realized under minimal inotropic support (epinephrine5 mg/kg per min).2.4. ABO-MismatchBefore starting reperfusion, we received informationfrom the transplant coordinator that ABO-mismatch hadhappened accidentally, the transplant candidate (O-group)receiving a B-group graft. The following precautions weretaken: in vivo absorption of the preformed anti-B antibodiesby transfusion of a B-blood group red cells concentratefollowed by intraoperative exchange plasmapheresis.Early postoperatively specific immunoabsorption was per-formed combined to intravenous infusions of IgG and IgMimmunoglobulins and C1-inhibitor infusions. Anti-B titerswere assessed isotype-specifically by ELISA throughout thetreatment.2.5. Present statusOne and a half years after transplantation, the systolic anddiastolic ventricular function are excellent, coronary angio-graphy and endovascular ultrasound shows absence of graftatherosclerosis and the coronary flow reserve is fully nor-mal. The anti-B antibody titer remains constant at 130–150% of the pre-transplant value. The patient continues toreceive a triple immunosuppressive therapy and enjoys anormal life quality.3. DiscussionIncreased use of the Fontan’s principle has permitted thecorrection of anomalies previously thought to be inoperable,like various forms of univentricular hearts, tricuspid atresiaand double inlet left ventricle. Recent series suggest that amodified Fontan operation (so called total cavo-pulmonaryconnection) can be performed with a low mortality and thatthe early results are encouraging in the majority of patients[2,3]. Careful attention to operation indication and completepreoperative assessment have contributed to better early andlong-term results. Nevertheless, late failure of the Fontancirculation is still difficult to predict and will happen in asubstantial number of patients.Adolescents and adult patients undergoing cardiac trans-plantation for congenital heart disease remain exceptionalcases. In several series published, this subset of patientsrepresent a very small group of candidates for transplanta-tion [4–8]. Obviously, the perioperative risk may beincreased by several factors in this patient’s group, suchas laborious dissection, increased bleeding, correction ofcongenital anomalies or re-establishment of a normal anat-omy to fit with the cardiac allograft, as well as modificationsof the transplantation technique that may extend the opera-tive time and the graft ischemic period.632 P.A. Berdat et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 631–634This case demonstrates that cardiac transplantation can beperformed with excellent anatomical and functional resultsand with a low surgical risk, even in presence of most com-plex anomalies of venous and pulmonary inflow and outflowand multiple previous surgical palliations. The operativetechnique must be adapted to the malformation and includesusually bicaval anastomosis as well as extensive reconstruc-tion of the pulmonary arteries [9].The present observation shows also that accidental ABO-mismatch can be rescued by comprehensive application ofmodern immunological methods, including selective bind-ing of the anti-donor antibodies, extracorporeal immunoab-sorption and selective blockade of the complement system.In this case, ABO-mismatch did fortunately not compromiseearly and mid-term result of the complex surgical proce-dure.References[1] Roth P, Agnani G, Arbez F, Roux C, Martin A, Colette C. Ivemark-syndrome: 2 case reports and a review of the literature. J GynecolObstet Biol 1993;22:771–775.[2] Malm T, Pawada A, Karl T, Mee RB. Recent results with the modifiedFontan operation. Scand J Thorac Cardiovasc Surg 1993;27:65–70.[3] Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, DanielsonGK. Five- to fifteen-year follow-up after Fontan operation. Cir-culation 1992;85:469–496.[4] Carrel T, Neth J, Pasic M. et al. Should cardiac transplantation forcongenital heart disease be delayed until adult age. Eur J Cardio-thorac Surg 1994;8:462–469.[5] Chartrand C, Guerin R, Kanagh R, Stanley P. Pediatric heart trans-plantation: surgical considerations for congenital heart disease. JHeart Transplant 1990;9:608–617.[6] Cooper MM, Fuzesi L, Addonizio LJ, Hsu DT, Smith CR, Rose EA.Pediatric heart transplantation after operations involving the pulmon-ary arteries. J Thorac Cardiovasc Surg 1991;102:386–395.Fig. 1. The artist’s drawing demonstrates the pre-transplant operative situs with the total cavo-pulmonary connection including a bilateral, bidirectional Glennshunt and the intra-atrial Gore-Tex tube graft to connect the inferior vena cava to the pulmonary circulation. The proximal segment of the main pulmonaryartery has been sutured just above the pulmonary valve.Fig. 2. Schematic representation of the modified orthotopic heart transplantation technique. Exceeding donor tissue allowed a tension-free anastomosis to therecipient inferior vena cava which was lying on the left side. The superior left vena cava was reconstructed using the innominate vein of the donor and thepersistent superior right vena cava was anastomosed with the donor superior vena cava. Note the separate rather distal reconstruction of the pulmonaryarteries.633P.A. Berdat et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 631–634[7] Menkis AH, McKenzie FN, Novick RJ. et al. Expanding applicabil-ity of transplantation after multiple prior palliative procedures. AnnThorac Surg 1991;52:722–726.[8] Hsu DT, Quaegebeur JM, Michler RE. et al. Heart transplantation inchildren with congenital heart disease. J Am Coll Cardiol1995;26:743–749.[9] Bailey LL. Heart transplantation techniques in complex congenitalheart disease. J Heart Lung Transplant 1993;12:168–175.634 P.A. Berdat et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 631–634

Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

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