Ann Thorac Surg 1995;60:194-197
© 1995 The Society of Thoracic Surgeons
Case Reports
Novel Approach for Orthotopic Heart Transplantation in Visceroatrial Situs Inversus
Robert E. Michler, MD,
Aqeel A. Sandhu, MD
Division of Cardiothoracic Surgery, Department of Surgery, Columbia University, College of Physicians and Surgeons, New York, New York
Accepted for publication November 30, 1994.
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Abstract
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Orthotopic heart transplantation was performed successfully in a 7-year-old girl with visceroatrial situs inversus. Creation of two autologous left-sided atrial tissue baffles tunneled the left superior vena cava and inferior vena cava to the right of the pulmonary veins. The reconstructed caval tunnels remain widely patent more than 4 years after transplantation. The ability of autologous tissue to grow with the patient has distinct advantages in the pediatric transplant population.
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Introduction
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Although transplantation for visceroatrial situs inversus has been described previously, these repairs incorporate either extracardiac connections that rely on excess donor tissue or complex intracardiac baffling procedures. We describe a simplified technique using autologous tissue for orthotopic heart transplantation in patients with visceroatrial situs inversus.
A 7-year-old girl with complex congenital heart disease was admitted to the Babies and Children's Hospital of Columbia Presbyterian Medical Center with recurrent attacks of supraventricular tachycardia accompanied by palpitations, pallor, diaphoresis, and weakness. The patient was diagnosed at birth to have a single ventricle, atrioventricular valve regurgitation, pulmonary stenosis, and visceroatrial situs inversus. Cardiac catheterization performed 2 months before admission demonstrated a cardiac index of 3.4 L • min-1 • m-2, mean arterial blood pressure of 70 mm Hg, mean pulmonary artery pressure of 22 mm Hg, and pulmonary vascular resistance of 1.2 Wood units. The patient was admitted acutely with a junctional tachycardia and hemodynamic decompensation requiring inotropic support and intravenous antiarrhythmic therapy. She was functionally in New York Heart Association class IV and considered at prohibitive risk for a Fontan procedure; therefore, she underwent heart transplantation.
The aorta was anterior and to the right of the main pulmonary artery. The pulmonary veins entered the posterior midline of the atrium rightward of the left superior vena cava and inferior vena cava. The orifice of the left superior vena cava measured 1.5 cm in diameter and approximated the size of the right superior vena cava.
The aorta, right and left superior, and inferior venae cavae were cannulated in standard fashion. The patient was cooled to 24°C. The heart was removed along the atrioventricular groove, leaving the entire recipient atrium intact to allow for sufficient tissue to be used as a baffle (Fig 1
).
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Fig 1. . Patient's anatomy after recipient heart excision. Excessive tissue is trimmed and flaps are created from the left atrium (dotted lines).
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Rerouting of systemic blood from the left sided superior and inferior venae cavae to the right atrium was managed by creating baffles using autologous left-sided atrial tissue. The free wall of the recipient atrium was incised along the left-sided crista terminalis from the orifice of inferior vena cava caudad to the orifice of the left superior vena cava cephalad (see Fig 1
). This tissue was divided, enabling us to fashion two trap door flaps or baffles. The inferior margin of the superior baffle was sutured to the back wall of the atrium above the entrance of the pulmonary veins. The inferior baffle was sutured similarly to the back wall of the atrium below the entrance of the pulmonary veins (Fig 2).
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Fig 2. . Intracardiac baffles fashioned from left-sided atrial tissue reroute systemic venous return from the left superior vena cava and inferior vena cava to the right of the pulmonary vein orifices.
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The donor heart was prepared in standard fashion. The left atrial anastomosis consisted of suturing the donor left atrial cuff to the recipient lateral left atrial free wall, then to the margins of the newly created atrial baffles superiorly and inferiorly, and then to the free atrial wall medially (Fig 3). The right atrial anastomosis was completed by suturing the septal portion of the donor right atrium to the newly created septum and the remaining right atrial cuff to the recipient right atrial free wall (Fig 4). This permitted both the left and right superior venae cavae and the inferior vena cava to drain into a large newly reconstructed right atrium.
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Fig 3. . Left atrial anastomosis. The donor left atrium is sutured to the recipient's free left atrial margin and the intracardiac baffles. The remaining atrial cuff is anastomosed to the recipient atrium right of the pulmonary veins.
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Fig 4. . Right atrial anastomosis. The donor atrial septum is sutured to the newly created recipient atrial septum and the remaining right atrial cuff is anastomosed to the recipient right atrial free wall.
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The patient was discharged 19 days after transplantation. A magnetic resonance image of the heart performed after discharge demonstrated the reconstructed intracardiac conduits to be widely patent (Figs 5, 6). Cardiac catheterization performed 3
years later revealed a right atrial pressure of 4 mm Hg, mean pulmonary artery pressure of 12 mm Hg, pulmonary capillary wedge pressure of 9 mm Hg, left ventricular end-diastolic pressure of 10 mm Hg, and cardiac index of 3.4 L • min-1 • m-2. She is now 4 years after cardiac transplantation and she continues to grow normally for her age and is attending school.
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Fig 5. . Posttransplantation nuclear magnetic resonance study in a patient with visceroatrial situs inversus. Patent intracardiac baffles (arrows) reroute anomalous systemic venous return to the right atrium (RA). (L = liver; PA = pulmonary artery; RV = right ventricle.)
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Fig 6. . Posttransplantation nuclear magnetic resonance study: reconstructed pulmonary venous return demonstrates the pulmonary veins draining into the donor left atrium (LA). Note the right-sided stomach (S) and aorta (Ao) and the left-sided liver (L) and inferior vena cava (IVC). (C = colon; LV = left ventricle; RV = right ventricle.)
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Comment
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Cardiac transplantation for congenital heart disease involves not only replacement of intracardiac malformations but often repair of extracardiac abnormalities [1]. These procedures can be technically exacting and require careful preoperative planning of (1) the technical details of reconstruction, (2) cannulation sites for cardiopulmonary bypass, and (3) conduct and timing of the donor/recipient operations. Reported experience with these procedures has taught us about the constancy of certain anatomic relationships and the need for surgical creativity. For example, Doty and associates [2] described the constant relationship at the pericardial reflection between the aorta and pulmonary artery regardless of their malposed or transposed position with respect to ventricular anatomy. In addition, Chartrand [3] illustrated the use of pericardial and synthetic patches for intracardiac venous rerouting.
Orthotopic heart transplantation for situs inversus is a formidable technical challenge, and at one time situs inversus was considered a contraindication to orthotopic heart transplantation. Reconstruction of the mirror-image systemic and pulmonary venous pathways remains the essence of the repair in these patients. Reported methods have described a variety of extracardiac and intracardiac channels to correct recipient systemic and pulmonary venous return [2, 4–6]. These approaches rely on harvesting accessory donor tissue and are often cumbersome, involving construction of complex baffles from synthetic materials or autologous pericardium. Potential problems exist with these techniques. The lumen of baffles created with pericardium or prosthetic material may become stenotic as the pericardium thickens or an intimal peel forms on the prosthesis [7]. Extracardiac conduits are vulnerable to external compression and potential obstruction. Our technique eliminates the need for additional donor tissue and creates conduits that are lined with endothelium and have the ability to grow with the patient.
Systemic and pulmonary venous rerouting using two intraatrial baffles created from the free wall of the left-sided atrium is a straightforward and reproducible technique for orthotopic heart transplantation in patients with visceroatrial situs inversus.
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Footnotes
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Address reprint requests to Dr Michler, Cardiac Transplant Service, Columbia Presbyterian Medical Center, MHB 7-435, 177 Ft Washington Ave, New York, NY 10032.
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References
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- Michler RE, Rose EA. Pediatric heart and heart-lung transplantation. Ann Thorac Surg 1991;52:708–9.[Medline]
- Doty DB, Renlund DG, Caputo GR, Burton NA, Jones KW. Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 1990;99:493–9.[Abstract]
- Chartrand C. Pediatric cardiac transplantation despite atrial and venous return anomalies. Ann Thorac Surg 1991;52:716–21.[Abstract]
- Monties JR, Goudard A, Blin D, et al. Atypical heart transplantations: technical aspects (based on a case report). Ann Chir 1989;43:591–5.[Medline]
- Mayer JE, Perry S, O'Brien P, et al. Orthotopic heart transplantation for complex congenital heart disease. J Cardiovasc Surg 1990;99:484–92.
- Reitz BA, Jamieson SW, Gaudiani VA, Oyer PE, Stinson EB. Method for cardiac transplantation in corrected transposition of the great arteries. J Cardiovasc Surg 1982;23:293–6.[Medline]
- Spray TL, Huddleston CB, Canter CE. Technique of transplantation for hypoplastic left heart syndrome with left superior vena cava. Ann Thorac Surg 1993;55:779–81.[Abstract]
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Abstract
Introduction
