A Borghi

A Loforte

AC Zomer

J Belohlavek

J Billett

T Bom Van der

V Bautista-Hernandez

WJ Ko

English

R. J. Uilkema

L. C. Otterspoor

Crossref

CASE REPORTExtracorporeal membrane oxygenation in adult patientswith congenital heart diseaseR. J. Uilkema & L. C. OtterspoorPublished online: 13 February 2014# The Author(s) 2014. This article is published with open access at Springerlink.comBackgroundDue to improved diagnostic and therapeutic methods, theprognosis of patients with congenital heart disease has im-proved dramatically [1, 2]. Therefore, a considerable numberof these patients survives to adulthood. As a consequence, anincreased number of them will eventually develop complica-tions, varying from mild rhythm disturbances to decompen-sated heart failure or cardiogenic shock. Furthermore, becauseof improved survival of patients with congenital heart disease,more re-operations are necessary [3].Extracorporeal membrane oxygenation (ECMO) is a sup-port system that consists of a pump and an oxygenator(Fig. 1). Blood is pumped from a large vein through anoxygenator, and back into an artery. Therefore, the systemfunctions as a complete cardiac and pulmonary serial bypass.The cannulas can be inserted into either a central or peripheralvein or artery (Fig. 1)In acute cardiogenic shock, ECMO can be used to providecirculatory and respiratory support and serves as a bridge-to-decision.Furthermore, because of improved survival to adulthood ofpatients with congenital heart disease, more re-operations arenecessary [3]. ECMO can be used as bridge-to-surgery, butalso to provide circulatory and respiratory support inpostcardiotomy cardiogenic shock. ECMO can particu-larly be useful in this patient group who often have adiminished right ventricular function or even a Fontancirculation, given that the right ventricle can be fullybypassed.In this article we describe the use of ECMO in two adultpatients with congenital heart disease and shock. The avail-able literature on the use of ECMO in this particular patientgroup is reviewed.Case report 1A 33-year-old female was admitted to our hospital with acuteright-sided heart failure caused by severe outflow tract steno-sis. As a child she underwent a correction of a tetralogy ofFallot with closure of the ventricular septum defect and cor-rection of the right ventricular outflow tract. Other medicalhistory revealed bleeding disorders, partially explained byfactor V Leiden and hepato-splenomegaly. She rapidly devel-oped severe shock for which a peripheral ECMO was placed(Fig. 1). This stabilised her circulation and allowed carefulplanning of valve surgery.Shortly after ECMO implantation, leakage and haematomaat the arterial cannula ensued. This led to a low flow state andsubsequent multi-organ failure, including acute-on-chronickidney failure requiring dialysis. The next day, balloon dila-tation of the pulmonary tract and pulmonary valve replace-ment were performed. Due to new-onset left ventricular fail-ure, probably due to stunning, the patient remained dependenton ECMO.Six days after correction of the pulmonary stenosis, theECMO system could be removed. She was almost ready fortransfer to the ward when arterial bleeding occurred at the oldinsertion site of the femoral cannula. Transfusion of bloodproducts and a surgical correction could not prevent her fromdying of haemorrhagic shock.R. J. UilkemaDivision Vital Functions, Intensive Care Centre, University MedicalCentre, Utrecht, the NetherlandsPresent Address:L. C. Otterspoor (*)Department of Cardiology, Catharina Hospital, Eindhoven, theNetherlandse-mail: luuk.otterspoor@cze.nlNeth Heart J (2014) 22:520–522DOI 10.1007/s12471-014-0526-zCase report 2A 38-year-old male with a Fontan circulation after previouscorrection of a congenital tricuspid atresia, suffered from recur-rent atrial tachyarrhythmias. Hewas admitted to our hospital fora MAZE operation and partial resection of the atria. Due to anincrease in intrathoracic pressure caused by mechanical venti-lation, the passive pulmonary blood flow of the Fontan circuitwas postoperatively impaired, after which he became hypoxicand hypotensive. In order to optimise pulmonary blood flow,treatment with sedatives, inodilators and nitric oxide ventilationwas initiated. Progressive shock and multi-organ failure need-ing dialysis developed. Eight days after ICU admission anECMO support system was inserted. After installing ECMO,his haemodynamic condition stabilised, although he was still inneed of high-dose vasopressins and fluids. Due to progres-sive multi-organ failure it was decided to withdraw theECMO after which he died.DiscussionIn this case report the use of extracorporeal membrane oxy-genation in two patients with congenital heart disease andshock is described. In the first patient, ECMO was primarilyused to optimise the clinical condition before operation. Thisstrategy has been used previously, mainly in patients awaitingcardiac or lung transplantation, but also in children awaitingsurgery for congenital heart disease [4]. In this case, ECMOwas needed to overcome heart failure, one of the major prob-lems in adults with congenital heart disease and the secondcause of death in these patients [1, 5].In the second case, ECMO was used to treat a patient withcardiogenic shock after cardiac surgery. Although there ismore experience in children [6], its use has also beendescribed in adult patients after operations related to congen-ital heart disease [7]. Although ECMO is promising forstabilising postcardiotomy cardiogenic shock, survival is lim-ited due to possible complications, of which haemorrhage isone of the most important. Prevention by withholding antico-agulants increases the risk of circuit clotting and thromboem-bolism [6]. Our first patient had a pre-existing coagulopathy,which made this consideration even more difficult.In the second patient ECMO was started to treat multi-organ failure and as a bridge-to-decision. The use of ECMO inpatients with multi-organ failure is not associated with apositive outcome, probably due to severe organ damage priorto the initiation of ECMO. Our patient suffered from acuterenal failure at day one, while ECMOwas started at day eight.Earlier initiation of ECMO may improve survival, as this canprevent organ damage [6].As both cases illustrate, the use of ECMO is not withoutcomplications. Haemorrhagic problems, but also sepsis,mediastinitis and thrombosis, are frequent causes of death [6].Inserting peripheral cannulas may damage vessels, makingfuture interventions more difficult. Patients who have alreadyundergone surgery have the disadvantage of scarring and chang-es in anatomy, which makes it more difficult to insert a centralECMO. Scarring due to the ECMO cannulas can precipitatenew arrhythmias, which is the most frequent long-term compli-cation and leading cause of death in this patient group [1, 5].The use of extracorporeal membrane oxygenation may pro-vide a valuable tool in stabilising patients with congenital heartdisease who present with cardiogenic shock, especially when thisis due to right ventricular failure [8]. The system can be used as abridge-to-decision or as a bridge-to-surgery. Furthermore, it canbe applied to overcome postcardiotomy cardiogenic shock.Clinicians who apply ECMO should be aware of its seriousrisks and complications. If manifest multi-organ failure isalready present, it is not recommended to start ECMO.Funding None.Conflict of interest None declared.Open Access This article is distributed under the terms of the CreativeCommons Attribution License which permits any use, distribution, andreproduction in any medium, provided the original author(s) and thesource are credited.References1. Borghi A, Ciuffreda M, Quattrociocchi M, et al. The grown-up con-genital cardiac patient. J Cardiovasc Med (Hagerstown). 2007;8(1):78–82.2. Billett J, Majeed A, Gatzoulis M, et al. Trends in hospital admissions,in-hospital case fatality and population mortality from congenital heartdisease in England, 1994 to 2004. Heart. 2008;94(3):342–8.Fig. 1 Schematic drawing of a veno-arterial ECMO system. Blood ispumped from a large vein to an artery. The system contains a pump (blackarrow) and an oxygenator (white arrow). A heater is needed to prevent theextracorporeal blood from cooling offNeth Heart J (2014) 22:520–522 5213. Zomer AC, Verheugt CL, Vaartjes I, et al. Surgery in adults withcongenital heart disease. Circulation. 2011;124(20):2195–201.4. Bautista-Hernandez V, Thiagarajan RR, Fynn-Thompson F, et al.Preoperative extracorporeal membrane oxygenation as a bridge tocardiac surgery in children with congenital heart disease. AnnThorac Surg. 2009;88(4):1306–11.5. Van der Bom T, Zomer AC, Zwinderman AH, et al. The changingepidemiology of congenital heart disease. Nat Rev Cardiol. 2011;8(1):50–60. Review.6. Loforte A, DelmoWalter EM, Stiller B, et al. Extracorporeal membraneoxygenation for intraoperative cardiac support in children with congen-ital heart disease. Interact Cardiovasc Thorac Surg. 2010;10:753–8.7. KoWJ, Lin CY, Chen RJ, et al. Extracorporeal membrane oxygenationsupport for adult postcardiotomy cardiogenic shock. Ann Thorac Surg.2002;73(2):538–45.8. Belohlavek J, Rohn V, Jansa P, et al. Veno-arterial ECMO in severeacute right ventricular failure with pulmonary obstructive hemody-namic pattern. J Invasive Cardiol. 2010;22(8):365–9.522 Neth Heart J (2014) 22:520–522

Netherlands Heart Journal

Extracorporeal membrane oxygenation in adult patients with congenital heart disease

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Extracorporeal membrane oxygenation in adult patients with congenital heart disease

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