Ann Thorac Surg 2004;78:e30-e33
© 2004 The Society of Thoracic Surgeons
Case report
Open-heart surgery in pediatric patients with end-stage liver disease
Emile A. Bacha, MD*a,
Joel Hardin, MDb,e,
David C. Cronin, MD, PhDc,
Lynda Brady, MDd,
Michael J. Millis, MDc,
Joanne P. Starr, MDa,
Peter Koenig, MDb,
Suanne Daves, MDe,
Madelyn Kahana, MDe
a Cardiothoracic Surgery, Pediatric and Congenital Cardiac Surgery, Chicago, IL, USA
b Section of Pediatric Cardiology, The University of Chicago Children's Hospital, Chicago, IL, USA
c Section of Transplantation Surgery, The University of Chicago Children's Hospital, Chicago, IL, USA
d Section of Pediatric Hepatology, The University of Chicago Children's Hospital, Chicago, IL, USA
e Section of Pediatric Critical Care, The University of Chicago Children's Hospital, Chicago, Illinois, USA
Accepted for publication November 25, 2003.
* Address reprint requests to Dr Bacha, Pediatric and Congenital Cardiac Surgery, The University of Chicago Children's Hospital, 5841 S Maryland Ave, Chicago, IL 60637, USA.
e-mail: ebacha{at}surgery.bsd.uchicago.edu
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Abstract
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Little is known about the safety of pediatric cardiac surgery in children with end-stage liver disease. We reviewed our experience with 4 patients with biliary atresia or Alagille's syndrome who underwent repair of ventricular septal defect and tricuspid regurgitation, atrioventricular canal, subaortic stenosis, or supravalvular aortic stenosis. One patient died on postoperative day 2. All other patients survived to discharge. At follow-up, 1 patient died at home awaiting liver transplantation and the remaining patients are doing well. One patient received a successful liver transplant. Pediatric cardiac surgery in children with end-stage liver disease can be done safely, albeit with a higher mortality.
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Introduction
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Both Allagille's syndrome (arteriohepatic dysplasia) and biliary atresia are associated with complex congenital heart disease (CHD) in 85% [1] and 15% [2] of cases, respectively. Conversely, complex congenital heart disease (eg, such as left atrial isomerism) is known to be associated with biliary atresia in 10% of cases [3]. Outcomes in the management of children with end-stage liver disease (ESLD) or severe cholestasis have improved [4]. Cardiopulmonary anomalies are a relative contraindication to orthotopic liver transplantation, and right ventricular hypertension in particular is an absolute contraindication to orthotopic liver transplantation [4]. Consequently, congenital heart surgeons will be called on more frequently to operate on such patients or on those patients with residual or recurrent heart disease after liver transplantation. This report analyzes our experience with this patient population.
Between May 2000 and June 2003, 4 children with ESLD underwent open-heart surgery at the University of Chicago Children's Hospital. A retrospective chart review was performed, and follow-up was obtained by direct patient contact as well as contact with the primary cardiologist and hepatologist.
Table 1 lists the pertinent preoperative clinical data. Our usual perioperative protocol was maintained, including preoperative administration of 30 mg/kg intrave-nous methylprednisolone in the pump prime, an aprotinin infusion at a loading dose of 140 U/m2 and an infusion of 70 U/m2, avoidance of low-flow cardiopulmonary bypass, keeping an on bypass hematocrit of 25% to 30%, insuring excellent drainage from the inferior vena cava, and moderate hypothermia (25°C to 32°C). Blood products were given as indicated.
Table 2 lists the pertinent postoperative clinical data. One patient died in the postoperative period. This was a 2-year-old child with Allagille's syndrome and severe biventricular outflow tract obstruction. She died on postoperative day 2 after a profound vasodilation with preserved cardiac function that began in the operating room. This vasodilation (systolic blood pressure in the 40s) was unresponsive to vasopressin, norepinephrine, or support by extracorporeal membrane oxygenation. Good biventricular function and the absence of any significant residual cardiac disease were documented. The autopsy was unrevealing. The infant with biliary atresia, Ebstein's disease, and ventricular septal defects had a protracted hospital course mainly due to nutritional issues. He died suddenly at home several months after discharge while awaiting a liver transplantation. Postoperative hemostasis for all 4 cases was not particularly difficult. Both survivors are doing well at 3 and 4 years postoperatively, and they are both asymptomatic. One child received a liver transplantation at 1 year postoperatively and the other child has nonprogression of Allagile's syndrome.
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Comment
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Review of the literature provides no references addressing the perioperative management of children with congenital heart disease who have ESLD or severe cholestasis. A few reports exist in the adult cardiac literature [5–7] that highlight the higher morbidity and mortality experienced when operating on patients with ESLD or cirrhosis. Postoperative bleeding and transfusion requirements were higher. Higher mortality was experienced mostly in child's B patients [7]. Keeping the cardiopulmonary bypass times short was the main recommendation. The present retrospective review analyzes our experience with open-heart surgery in a pediatric patient population with liver disease. Because of the small number of patients reviewed, statistical analysis was not performed.
In review, the patient who had died postoperatively had the highest preoperative total bilirubin value in the group. Other risk factors included more advanced cardiac disease and longest cardiopulmonary bypass and myocardial ischemic times. All of these factors may have contributed to the postoperative death. Isolated acute liver failure was unlikely the cause of the severe vasodilation that occurred, because laboratory data were more consistent with multiorgan failure.
Based on this small but unique series, we conclude that congenital heart surgery for complex problems in patients with ESLD or severe cholestasis can be performed, albeit at a higher morbidity and mortality. Although patients with advanced complex congenital heart disease and ESLD are at increased risk, intervention represents the only reasonable option for a chance at orthotopic liver transplantation or long-term survival, or both.
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References
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- Alagille D., Estrada A., Hadchouel M., Gautier M., Odievre M., Dommergues J.P. Syndromic paucity of interlobar bile ducts (Allagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 1987;110:195-200.[Medline]
- Fischler B., Haglund B., Hjern A. A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia. J Pediatr 2002;141:217-222.[Medline]
- Gilljam T., McCrindle B.W., Smallhorn J.F., Williams W.G., Freedom R.M. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000;36:908-916.[Abstract/Free Full Text]
- Marino I.R., Chap P., Esquivel C.O., et al. Liver transplantation for arteriohepatic dysplasia (Allagille's syndrome). Transpl Int 1992;5:61-64.[Medline]
- Nimomiya M., Takamoto S., Kotsuka Y., Ohtsuka T. Indication and perioperative management for cardiac surgery in patients with liver cirrhosis. Our experience with 3 patients. Jpn J Thorac Cardiovasc Surg 2001;49:391-394.[Medline]
- Hirata N., Sawa Y., Matsuda H. Predictive value of preoperative serum cholinesterase concentration in patients with liver dysfunction undergoing cardiac surgery. J Card Surg 1999;14:172-177.[Medline]
- Kaplan M., Cimen S., Kut M.S., Demirtas M.M. Cardiac operations for patients with chronic liver disease. Heart Surg Forum 2002;5:60-65.[Medline]
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Abstract
Introduction
