Ann Thorac Surg 1995;59:1575-1577
© 1995 The Society of Thoracic Surgeons
Case Report
Arterial Switch and Resection of Hepatic Hemangioendothelioma
Robert C. Robbins, MD,
Clifford Chin, MD,
Kwok L. Yun, MD,
Gerald J. Berry, MD,
Daniel Bernstein, MD,
Bruce A. Reitz, MD
Department of Cardiothoracic Surgery, Division of Pediatric Cardiology, and Department of Pathology, Stanford University School of Medicine, Stanford, California
Accepted for publication November 1, 1994.
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Abstract
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We report on the management of a neonate undergoing arterial switch for transposition of the great arteries and concomitant resection of a hepatic infantile hemangioendothelioma. A preoperative aortogram demonstrated the arterial supply of the hepatic hemangioendothelioma. Pulmonary artery hypertension and myocardial ischemia were noted after separation from cardiopulmonary bypass. Resection of the hepatic malformation produced an immediate reduction in pulmonary hypertension and resolution of the myocardial ischemia. The patient had an uneventful postoperative recovery.
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Introduction
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Infantile hemangioendothelioma of the liver is a rare anomaly. This report details the management of a neonate with hemangioendothelioma of the liver in association with transposition of the great arteries.
A 3.3-kg girl had profound cyanosis at birth requiring endotracheal intubation. The diagnosis of D-transposition of the great arteries with intact ventricular septum was made with two-dimensional echocardiography in the first hours of life. The infant was initially stabilized after commencement of a prostaglandin E2 infusion. A balloon atrial septostomy was done because of poor mixing. An aortogram was performed to investigate for coarctation of the aorta. A large artery arising from the superceliac aorta was observed to perfuse a malformation in the left lobe of the liver (Fig 1
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Fig 1. . (A) Aortogram demonstrating the arterial supply of a large mass in the left lobe of the liver. (B) The venous phase of the injection shows the extent of the cavernous malformation.
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We performed an arterial switch operation and closure of the atrial septal defect on the second day of life. The coronary artery pattern was Yacoub type A, with the left anterior descending and circumflex arteries arising from the right facing sinus and the right coronary artery from the left facing sinus (1LCx,2R). The procedure was uncomplicated, and excellent perfusion of the entire myocardium was observed after removal of the aortic clamp. Satisfactory perfusion pressure was maintained throughout cardiopulmonary bypass. The patient was separated from cardiopulmonary bypass without difficulty. The right atrium, right ventricle, and pulmonary artery were noted to be volume loaded with systemic pulmonary artery pressures. Right ventricular dysfunction soon developed as the result of myocardial ischemia. The right coronary artery was observed to be distorted by the enlarged pulmonary artery. The ischemia immediately resolved with anterior and leftward traction of the anteriorly positioned neopulmonary artery, but returned once the traction was relaxed.
We extended the midline incision inferiorly and performed an exploratory laparotomy while anterior and leftward traction of the neopulmonary artery was maintained to prevent right coronary ischemia. We found a 3 x 3 cm mass in the left lobe of the liver. A vigorous thrill was palpable in the mass, and with manual compression of the mass pulmonary artery pressure was reduced to one-half of systemic pressure. We performed a hepatic resection, including the mass, without difficulty. The pulmonary hypertension and volume overload of the right heart immediately diminished. No further coronary ischemia was observed after the hepatic resection. We did not administer protamine sulfate because of increased pulmonary artery reactivity. The activated clotting time normalized at the completion of the hepatic resection, and hemostasis was satisfactory. The patient was extubated on the second postoperative day and was discharged home on the seventh postoperative day. She continues to do well 7 months after operation.
The surgical specimen displayed a solitary, circumscribed, but unencapsulated, soft mass composed of solid and blood-filled spongy areas. Microscopic examination revealed a vascular proliferation supported by a loose, edematous connective tissue stroma. The intercommunicating, small vascular channels with collapsed and slit-like lumina were lined by uniform, plump, and oval endothelial cells (Fig 2). Ectatic blood-filled spaces resembling a cavernous hemangioma were found within the lesion. This constellation of findings was characteristic of infantile hemangioendothelioma.
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Fig 2. . Infantile hemangioendothelioma. (A) Irregular vascular channels of varying size. (Hematoxylin and eosin; original magnification x30.) (B) High magnification showing the interconnecting vessels lined by a continuous single layer of uniform endothelial cells. (Hematoxylin and eosin; original magnification, x200.)
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Comment
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Arteriovenous malformations can result in left-to-right shunting and volume overload of the right heart. We were concerned that this malformation would complicate the conduct of cardiopulmonary bypass because of inability to maintain adequate perfusion pressure. We considered preoperative percutaneous coil embolization of the malformation, but this was abandoned because of the absence of heart failure. Right coronary ischemia resulted from distortion of the transposed right coronary artery by the anteriorly positioned, volume-overloaded neopulmonary artery. The ischemia immediately resolved with the elimination of the left-to-right shunt through the malformation.
Infantile hemangioendothelioma is a benign lesion and the most common tumor of the liver in the first year of life [1]. Patients can present with hepatomegaly and congestive heart failure [2]. Treatment has consisted of surgical resection, decongestive medications, glucocorticoid therapy, transarterial embolization, and transplantation [3–6]. Recent advances in the diagnosis and treatment of these patients has significantly reduced the mortality previously associated with these malformations [7].
Neonates with transposition of the great arteries and poor mixing may require balloon atrial septostomy with either echocardiographic or fluoroscopic guidance. Aortography was done in this case to evaluate for coarctation of the aorta, and the hepatic malformation was discovered. The presence of excessive hepatomegaly and congestive heart failure in neonates with transposition of the great arteries, especially with intact ventricular septum, may be an indication for ultrasound screening of the liver. Large arteriovenous malformation of the liver, confirmed with angiography, should be embolized percutaneously or ablated surgically before arterial switch operation.
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Footnotes
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Address reprint requests to Dr Robbins, Department of Cardiothoracic Surgery, Falk Cardiovascular Research Center, Stanford University School of Medicine, Stanford, CA 94305-5247.
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References
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- Stocker JT. Hepatic tumors. In: Balistreri WF, Stocker JT, eds. Pediatric hepatology. New York: Hemisphere, 1990:399.
- Larcher VF, Howard ER, Mowat AP. Hepatic hemangiomata: diagnosis and treatment. Arch Dis Child 1981;56:7–14.[Abstract/Free Full Text]
- Somppi E, Niemi K, Ruuskanen O, Arajarvi P, Lauslahti K. Cavernous hepatic hemangioma in the newborn infant: case report of a successful resection. J Pediatr Surg 1974;9:239–41.[Medline]
- Mattioli L, Lee KR, Holder TM. Hepatic artery ligation for cardiac failure due to hepatic hemangioma in the newborn. J Pediatr Surg 1974;9:859–62.[Medline]
- Roccini AP, Rosenthal A, Issenberg HJ, Nadas AS. Hepatic hemangioendothelioma: hemodynamic observations and treatment. Pediatrics 1976;57:131–5.[Abstract/Free Full Text]
- Touloukian RJ. Hepatic hemangioendothelioma during infancy: pathology, diagnosis and treatment with prednisone. Pediatrics 1970;45:71–6.[Abstract/Free Full Text]
- Luks FI, Yazbeck S, Brandt ML, Bensoussan AL, Brochu P, Blanchard H. Benign liver tumors in children: a 25-year experience. J Pediatr Surg 1991;26:1326–30.[Medline]
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Abstract
Introduction
