Ann Thorac Surg 2000;69:1270-1271
© 2000 The Society of Thoracic Surgeons
CASE REPORTS
Switch from a BIVAD to a LVAD in a boy with Kawasaki disease
Christoph Schmitz, MDa,
Armin Welz, MDa,
Oliver Dewald, MDa,
Rainer Kozlik-Feldmann, MDb,
Heinrich Netz, MDb,
Bruno Reichart, MDa
a Department of Cardiac Surgery, Grosshadern Medical Center, Ludwig-Maximilians-University, Munich, Germany
b Department of Pediatric Cardiology, Grosshadern Medical Center, Ludwig-Maximilians-University, Munich, Germany
Address reprint requests to Dr Schmitz, Department of Cardiac Surgery, University of Bonn, Sigmund-Freud-Str 25, D-53105 Bonn, Germany
e-mail: c.schmitz{at}uni-bonn.de
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Abstract
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A 9-year-old boy with Kawasaki disease survived after two severe myocardial infarctions. Thereafter pharmacologically untreatable ventricular arrhythmia and rapidly deteriorating heart failure developed in the patient. After 19 days of biventricular and a further 27 days of left univentricular mechanical circulatory support with the Berlin Heart (Cardiotechnica, Berlin, Germany) assist device the boy successfully underwent heart transplantation. At a follow-up of 54 months, the boy is leading an active and unrestricted life.
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Introduction
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Over the past decade bridging to cardiac transplantation has become a viable option for transplant candidates whose conditions deteriorate while awaiting a suitable donor heart [1]. Most of the patients have been of adult age suffering from end-stage heart disease. Infants and small children who suffer from ventricular dysfunction, however, have fewer treatment options, mainly due to size constraints. More recently, a small number of pediatric patients have been supported with extracorporeal centrifugal and pneumatic devices, particularly after cardiac operations [2].
A 9-year-old boy (140 cm, 28 kg) with known Kawasaki disease since 2 years and history of previous posterior wall infarction (1 year before) was referred to our pediatric cardiac surgical unit with an acute anterior wall infarction. Emergency cardiac catheterization now revealed occlusion of the left anterior descending artery (Fig 1) and severe left ventricular dysfunction (ejection fraction [EF] 0.19). Lysis with streptokinase failed to reopen the vessel. During the following 24 hours the left ventricular function remained severely compromised and the boy developed severe ventricular arrhythmia. Despite maximal inotropic support and various antiarrhythmic treatment he became progressively hypotensive and oliguric. After he had to be intubated due to ventricular fibrillation requiring defibrillation nearly every 10 minutes, the decision was made to stabilize his circulation with a mechanical circulatory assist device.
The Berlin Heart (Cardiotechnica, Berlin, Germany) is a pneumatically driven heart assist system that can be used either as a univentricular or a biventricular assist device. As the child suffered from severe ventricular rhythm disorder frequently degenerating to ventricular fibrillation the device was primarily set up for biventricular assistance. After a midline sternotomy cardiopulmonary bypass was instituted. For left ventricular support, the inflow cannula was placed in the apex of the left ventricle and the outflow cannula anastomosed to the aorta. For right ventricular support the inflow cannula was put through the right atrial appendage and the outflow cannula placed on the pulmonary trunk. The exit sites of the cannulas were just below the xiphoid. Both pumps were heparin coated with a maximal stroke volume of 30 mL on the left and 25 mL on the right side. After cardiopulmonary bypass was stopped heparin was partially antagonized. The chest was closed, and the boy was returned to our pediatric cardiac intensive care unit with minimal inotropic support. Pump rate ranged from 60 to 90 beats/min providing flow rates between 1.5 and 2.5 L/min. Atrial pressures showed a perfect drainage of both sides of the heart.
Postoperative anticoagulation consisted of intravenous heparin with an activated clotting time between 150 and 200 seconds, and a partial thrombin time about 1.5 to 2 times control. Antithrombin III was given if levels decreased to less than 80%. Under this regimen thromboembolism did not develop in the boy, although thrombotic material was found in both pump chambers after removal.
On the first postoperative day the chest was reopened due to inflow problems of the right pump inflow cannula. This time the sternum was left open, and a Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) surgical membrane was used for temporary skin closure. During the following days the patch was reopened several times to remove blood clots compromising right pump inflow. After 19 days sinus rhythm stabilized, allowing removal of the right pump and closure of the sternum. This led to markedly decreased blood loss through the drains. On the 32nd postoperative day the boy was extubated and mobilization started. Daily echocardiograms revealed no improvement in left ventricular function. The day before transplantation calculated left ventricular EF was still 0.22. After 46 days of mechanical circulatory support uneventful heart transplantation took place. Immunosuppression consisted of FK506, azathioprin, and for 3 months corticosteroids. The boy was discharged from hospital on the 24th day after transplantation. So far (54 months after heart transplantation) no severe complications have occurred.
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Comment
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This is a case of a primary pediatric bridge to transplantation. Mechanical circulatory assist systems for children are also used for postcardiotomy circulatory support, either as a bridge to recovery or transplantation [3].
We describe a pediatric case with left apical cannulation. Most authors place the left inflow cannula through the right superior pulmonary vein into the left atrium [4]. Our approach with an adult-sized left apical cannulation provides considerably better flow to the left pump than can be achieved with a left atrial cannula. The disadvantage of a ventriculotomy obviously has no importance if the patient will later undergo transplantation.
Thromboembolic complications remain one of the main problems in the long-term application of extracorporeal pneumatically driven assist systems [5]. In this case we used a strong anticoagulation regimen and accepted several reexplorations in the early postoperative phase. Due to the restricted space in a small child’s chest, small amounts of hemorrhage may compromise filling of the pumps through tamponade-like compression of the venae cavae. With four cannulas, obligatory for biventricular support, we were not able to close the chest permanently in the demonstrated case. After removal of the right ventricular pump and cannulas the chest could be closed and the bleeding stopped. For this reason we advise—whenever possible—the primary use of a univentricular device in small children. If biventricular support is unavoidable, once instituted daily echocardiographic examinations should be done to assess whether both pumps continue to be needed. The switch from biventricular to univentricular support should be accomplished as early as hemodynamically possible.
Our case shows the feasibility of treating children with severe heart failure using an extracorporeal pneumatically actuated circulatory support system as a bridge to cardiac transplantation. The 9-year old boy in this case received adequate circulatory support for 46 days, which allowed us to find a suitable donor heart for him. No major complications occurred during or after the operations. At a follow-up of 54 months, the boy is leading an active and unrestricted life, with no long-term device-related sequelae.
Our case demonstrates that extracorporeal mechanical circulatory support can be used effectively to sustain a pediatric patient while awaiting heart transplantation. This might help to overcome problems arising from the donor shortage, particularly in this age group.
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References
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Vetter H.O., Kaulbach H.G., Schmitz C., et al. Experience with the Novacor left ventricular assist system as a bridge to cardiac transplantation, including the new wearable system. J Thorac Cardiovasc Surg 1995;109:74-80.[Abstract/Free Full Text]
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Kaye M. The Registry of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 1993;12:541-548.[Medline]
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Hetzer R., Loebe M., Potapov E.V., et al. Circulatory support with pneumatic paracorporeal ventricular assist device in infants and children. Ann Thorac Surg 1998;66:1498-1506.[Abstract/Free Full Text]
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Konertz W., Hotz H., Schneider M., et al. Clinical experience with the MEDOS HIA-VAD system in infants and children. Ann Thorac Surg 1997;63:1138-1144.[Abstract/Free Full Text]
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Martin J., Sarai K., Schindler M., et al. MEDOS HIA-VAD biventricular assist device for bridge to recovery in fulminant myocarditis. Ann Thorac Surg 1997;63:1145-1146.[Abstract/Free Full Text]
Accepted for publication September 13, 1999.
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Mechanical circulatory support for infants and children with cardiac disease
Ann. Thorac. Surg.,
May 1, 2002;
73(5):
1670 - 1677.
[Abstract]
[Full Text]
[PDF]
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Abstract
Introduction
