Ann Thorac Surg 2009;88:1002-1003. doi:10.1016/j.athoracsur.2009.01.060
© 2009 The Society of Thoracic Surgeons
Case Reports
Heart-Lung Transplantation In Situs Inversus Totalis
Tobias Deuse, MD*,
Bruce A. Reitz, MD
Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, California
Accepted for publication January 22, 2009.
* Address correspondence to Dr Deuse, Department of Cardiothoracic Surgery, Stanford University School of Medicine, 300 Pasteur Dr, CVRB MC 5407, Stanford, CA 94305 (Email: deuse{at}stanford.edu).
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Abstract
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Situs inversus totalis is a condition with left-to-right reversal of the viscera combined with dextrocardia. It has long been regarded a contraindication for thoracic transplantation. Reconstruction of the mirror-image systemic venous pathways to accommodate normal donor organs remains the main surgical challenge. Here we present our simplified surgical technique for combined heart-lung transplantation and provide a concise review of the literature.
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Introduction
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Situs inversus totalis describes an anatomical malposition with left-to-right reversal of the viscera combined with dextrocardia [1]. The cardiac situs is determined by the atrial location, and in situs inversus, the morphologic right atrium is on the left and the morphologic left atrium is on the right. Viscero-atrial concordance with the liver and gallbladder on the left and the spleen and stomach on the right is most common [2], but not necessarily compelling [3]. The pulmonary anatomy is also reversed with a wide and short, steeply descending left bronchus and a long and more narrow, more horizontally descending right bronchus. Also, the left lung has three lobes and the right lung has two lobes. Because situs inversus is a rare anomaly and had been regarded a contraindication for heart-lung transplantation in the early years, it was not until 1989 that the first combined heart-lung transplantation was performed in a patient with situs inversus and Kartagener's disease [4].
Heart-lung transplantation for situs inversus totalis is surgically challenging, mainly because it requires reconstruction of the mirror-image systemic venous pathways. The patient described was a 31-year-old woman with complex congenital heart disease, including situs inversus, dextrocardia, single ventricle, transposition of the great vessels, and pulmonic stenosis. Progressive cyanosis developed in the patient, and she underwent Waterston shunt placement at 8 years old. Over the years, she developed Eisenmenger's physiology (Fig 1A).
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Fig 1. (A) Pre-transplant chest x-ray (left) and computed tomography (right). (B) An intraoperative image showing a patient with situs inversus and dextrocardia. (C) Three systemic venous connections to the donor heart-lung bloc were made: recipient left-sided superior vena cava (rLSVC) to donor superior vena cava (dSVC) and innominate vein (behind the aorta [Ao]), recipient right-sided superior vena cava (rRSVC) to donor right atrial appendage, and direct inferior vena cava (IVC) to IVC. (D) Post heart-lung transplant chest x-ray (left) and computed tomography (right); the heart is in its normal levocardia position.
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The donor heart-lung bloc was harvested as previously described [5]. An extra length SVC-innominate vein was taken. No additional procedures were necessary to prepare the donor organ bloc for implantation. A median sternotomy incision was performed, and the pericardium and both pleural cavities were opened (Fig 1B). Adhesions, especially around the aorta, were divided, and the heart and both lung hilae were dissected free. The aorta, both superior vena cavae, and the left-sided inferior venae cavae (IVC) were cannulated and cardiopulmonary bypass was initiated. The posterior aorta was dissected above the Waterston shunt, and the aorta was cross clamped. Recipient cardiectomy was performed, leaving a generous systemic atrial cuff in continuation of the IVC and full length of the SVCs (Fig 2A). The aorta at the site of the Waterston shunt was primarily repaired. Both lungs were excised by dividing the pulmonary arteries and veins and stapling off the main bronchi. The trachea was mobilized and transected above the carina. The donor heart-lung bloc was placed in the chest. The tracheal anastomosis was performed above the carina. It was possible to directly anastomose the donor IVC to the recipient IVC, which shifted the heart mildly to the left. The donor innominate vein was connected to the recipient left-sided SVC and the right-sided SVC was directly anastomosed to the right atrial appendage. Finally, the aortic anastomosis was performed end-to-end, using extra length to allow mild aortic bowing over the underlying innominate vein (Figs 1C and 2B). The recipient pericardium was widely opened on the left to allow levocardia position of the heart, as seen in postoperative imaging studies (Fig 1D).
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Fig 2. Technique for heart-lung transplantation. (A) The recipient situs is prepared to accommodate the donor heart-lung bloc. (B) Direct anastomoses obviate the need for creation of intracardiac or extracardiac tunnels for systemic venous reconstruction.
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Comment
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The tracheal and aortic anastomoses to an anatomically normal donor heart-lung bloc can usually be made easily in patients with situs inversus. Reconstructing the systemic venous return presents the challenge. Intracardiac and extracardiac channels for re-routing of the systemic venous return into the donor right atrium have been described.
The initial systemic venous reconstruction described by Miralles and colleagues [4] implemented the creation of a large single atrium by resecting the entire interatrial septum and closing the pulmonary vein orifices. The left anterior portion of this single atrial cavity was closed with the external wall of the left-sided right atrium and was anastomosed to the donor right atrium. One inconvenience was the large atrial size that would facilitate blood stasis and thrombosis [6]. Rabago and colleagues [6] later reported a modification of this technique as he positioned the lungs anterior to the phrenic nerves and performed the right atrium anastomosis after clockwise rotation of the heart to allow the right-sided pulmonary veins to pass cephalic to the right atrial anastomosis. Parry and colleagues [7] used bi-caval connections for the heart-lung bloc. He fashioned a common conduit out of the recipient right atrial remnant that would drain both the hepatic vein and the left IVC, and would allow direct anastomosis to the donor IVC. However, a greater saphenous vein spiral graft was necessary to connect the donor innominate vein with the recipient left SVC. The bi-caval technique described in this article did not necessitate the use of graft interposition, because an extra-length donor SVC-innominate vein was taken. This obviates the need for creation of intracardiac or extracardiac tunnels for systemic venous reconstruction.
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References
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- Rao PS. Dextrocardia: systematic approach to differential diagnosis Am Heart J 1981;102:389-403.[Medline]
- Maldjian PD, Saric M. Approach to dextrocardia in adults: review AJR 2007;188:S39-S49.[Abstract/Free Full Text]
- Macartney F, Partridge J, Shinebourne E, Tynan M, Anderson R. Identification of the atrial situsIn: Anderson RH, Shinebourne EA, editors. Pediatric cardiology. Edinburgh, NY: Churchill Livingston; 1977. pp. 16-26.
- Miralles A, Muneretto C, Gandjbakhch I, et al. Heart-lung transplantation in situs inversus: a case report in a patient with Kartagener's syndrome J Thorac Cardiovasc Surg 1992;103:307-313.[Abstract]
- Reitz B. Heart and lung transplantationIn: Baumgartner WA, Reitz BA, Aschuff SC, editors. Heart and heart-lung transplantation. Philadelphia, PA: Saunders; 1990. pp. 319-346.
- Rabago G, Copeland JG, Rosapepe F, et al. Heart-lung transplantation in situs inversus Ann Thorac Surg 1996;62:296-298.[Abstract/Free Full Text]
- Parry AJ, O'Fiesh J, Wallwork J, Large SR. Heart-lung transplantation in situs inversus and chest wall deformity Ann Thorac Surg 1994;58:1174-1176.[Abstract/Free Full Text]
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Abstract
Introduction
