Case Report Singapore Med J 2011, 52(3) e48 Combined heart -liver transplantation with extended cardiopulmonary bypass Marriott A J, Hwang N C, Lai F 0, Tan C K, Tan Y M, Lim C H, Boey S K, Tay S M, Cheow P C, Lim Y P, Chan T, Loh K, Kwok B, Chung A, Sivathasan C ABSTRACT We report a case of combined heart and liver transplantation for familial amyloid polyneuropathy. This is the first such combined transplant performed in Asia, and differs from previously described cases, in that cardiopulmonary bypass was continued at partial flow during liver transplantation in our case. This was done in order to provide haemodynamic support to the cardiac graft and to protect it from the impending reperfusion insult that frequently accompanies liver transplantation. The utility of this management course is discussed, along with its actual and potential complications. We also describe the impact of a lung -protective ventilation strategy employed during cardiac transplantation. Keywords: cardiopulmonary bypass, familial amyloid polyneuropathy, heart transplantation, liver transplantation, reperfusion injury Singapore Med J 201 1; 52(3): e48 -e51 INTRODUCTION Our patient, a 57 -year -old man, underwent combined heart and liver transplantation (CHLT) for familial amyloid polyneuropathy (FAP) non-transthyretin Met30 variant. This autosomal dominant disease is characterised by abnormal hepatic amyloidogenesis, with progressive amyloid deposition in the extracellular matrix of peripheral and autonomic nerves, and to a varying degree within the heart, gastrointestinal tract, kidneys and eyes. Life expectancy without transplantation is approximately ten years from the onset of symptoms.(') This condition also carries the risk of subsequent cardiac failure despite isolated liver transplantation,(2) especially in patients with cardiac amyloidosis at the time of transplantation. Herlenius et al suggested that if cardiac involvement is present in patients with non-transthyretin Met30 FAP, then combined liver and heart transplantation may be justified.°) We add our experience to the series of case reports of CHLT and detail the factors that compelled us to continue cardiopulmonary bypass (CPB) during the liver implantation phase, along with its attendant implications for coagulation and witnessed benefits at the time of hepatic reperfusion. CASE REPORT Our patient's first symptoms, diarrhoea and urinary incontinence, appeared in mid 2004 at the age of 52, followed by a gradual reduction in exercise tolerance over the next six months. He was a non-smoker, non-drinker, and his only medical history was that of childhood asthma. He was assessed by a neurologist in 2005. Physical examination revealed orthostatic hypotension and mildly reduced pinprick sensation in the extremities, and further questioning elicited a family history of paternal death due to a cardiomyopathy of unknown aetiology. Nerve conduction studies and electromyography showed a generalised sensorimotor and autonomic polyneuropathy. Congo red -stained preparates from neural biopsies exhibited birefringence in polarised light, denoting presence of amyloid fibrils. Subsequent genetic testing confirmed mutation of the transthyretin gene and a diagnosis of FAP was made. The patient was referred to our liver transplant service in September 2006. Cardiac biopsy revealed the presence of amyloid deposition. The decision was made in conjunction with the patient and his family to proceed with wait-listing for combined heart and liver transplantation. Preoperative evaluation proceeded in accordance with local guidelines for heart and liver transplantation. Electrocardiography showed borderline low voltages in frontal leads, which was consistent with infiltrative heart disease. Plain chest radiography and spirometry were normal for age. Transthoracic echocardiography revealed mild concentric increase in left ventricular thickness, with ejection fraction of approximately 60% and pseudonormal filling. Right heart catheterisation revealed a central venous pressure of 11 mmHg, pulmonary arterial pressure 26/14 mmHg, mean pulmonary arterial pressure 19 mmHg, pulmonary artery capillary wedge pressure 18 mmHg, cardiac index 3.92 L/min/m2 and systemic vascular resistance 978 dyne/ sec/cm'. There was only mild derangement of hepatic transaminases. The patient's serum bilirubin concentration Department of Anaesthesiology, Singapore General Hospital, Outram Road, Singapore 169608 Marriott AJ, MBBS Medical Officer Hwang NC, MBBS, FFARCSI Senior Consultant Lai FO, MBBS, MMed Senior Consultant Boey SK, MBBS, MMed Senior Consultant Tay SM, MBBS, FFARCS Senior Consultant Loh K, MBBS Registrar Liver Transplant Programme Tan CK, MBBS, MRCP Senior Consultant Tan YM, MBBS, FRCSE Senior Consultant Cheow PC, MMed, FRCSE Consultant Chung A, MBBS, FRCSE Senior Consultant Heart and Lung Transplant Programme, National Heart Centre, 17 Third Hospital Ave, Singapore 168752 Lim CH, MBBS, FRCSE Senior Consultant Lim YP, FRCSG, FRCSE Senior Consultant Chan T, BSc, GDip Chief Perfusionist Kwok B, MMed, MRCPE Senior Consultant Sivathasan C, FRCSE, FRCS Visiting Consultant Correspondence to: A/Prof Hwang Nian Chih Tel: (65) 6321 4220 Fax: (65) 6326 6301 Email: hwang.nian. chih@sgh.com.sg