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Ann Thorac Surg 1996;62:296-298
© 1996 The Society of Thoracic Surgeons

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How to Do It

Heart-Lung Transplantation in Situs Inversus

Department of Cardiovascular and Thoracic Surgery, University of Arizona, Tucson, Arizona

Accepted for publication March 5, 1996.

	Abstract

Top Footnotes Abstract Introduction Method Comment References

 
Reconstruction was accomplished in 2 heart-lung recipients with situs inversus resulting in a left-sided systemic venous atrium. We created a large common atrium that was closed on the left side, leaving an atrial cuff on the inferior right quadrant. To this we anastomosed the donor right atrium, which had been opened laterally between the cavae. This resulted in some clockwise rotation of the ventricles and anterior positioning of the apex. The right pulmonary veins passed superior to the atrial anastomosis and posterior to the donor right atrium. Cardiopulmonary function was excellent in both cases.

	Introduction

Top Footnotes Abstract Introduction Method Comment References

 
Patients with situs inversus syndrome who require heart-lung transplantation present a major anatomic challenge for the surgeon. Combined heart-lung transplantation in situs inversus has been described by the La Pitie group [1]. Recently we have used a modification of this technique in 2 patients, one with a Kartagener's syndrome and a second one with Eisenmenger's syndrome secondary to congenital heart disease with situs inversus.

Our first patient is a 45-year-old man who had a reported history in his childhood of pneumonia and sinusitis and was diagnosed in 1979 with Kartagener's syndrome. He had atrial inversion and the apex of his heart pointed to the right. The second patient is a 17-year-old man who had a common atrium with right-sided entry of pulmonary veins, left-sided heart, pulmonary stenosis, and normal position of the great arteries with respect to each other. The patient presented with four venae cavae: superior and inferior venae cavae on both sides; the largest of the four was the left inferior.

	Method

Top Footnotes Abstract Introduction Method Comment References

 
Donor Procurement
Donor organs were harvested according to the technique described by Reitz [2]. The lungs were preserved by administration of prostacyclin through the main pulmonary artery immediately before pulmonary artery flush with modified Collins (60 mL/kg). The heart was protected with infusion of 1 L of modified Krebs high/low-potassium cardioplegia solution (500 mL with potassium 25 mEq/L followed by 500 mL with potassium of 4.5 mEq/L) through the aortic root. The organs were stored in a sterile container with saline solution at 4°C for the transport.

Surgical Procedure
A median sternotomy incision was performed. The pericardium, as well as the pleural cavities were opened. We dissected both hilae before bypass, then bypass was established. We first removed the patient's heart, leaving a generous atrial cuff on both sides and a generous piece of aorta (Fig 1). Once the heart was out, exposure improved. We dissected the entire hilum on the left side free from the phrenic nerve and stapled with 4.8 staples using a TA90 stapler and cut the lung away. A similar procedure was used on the right side. We then closed the pulmonary veins intraatrially. Next the trachea was transected and removed just above the carina. At this point of the operation careful hemostasis in both hilar areas and the mediastinum was obtained with electrocautery and metallic clips.

View larger version (56K): [in this window] [in a new window]   Fig 1. . After removal of both ventricles we leave a generous atrial cuff on both sides to be able to construct the large right atrium.

View larger version (64K): [in this window] [in a new window]   Fig 2. . Section of the atrial septum allowing blood flow from the right to the left atrium. Pulmonary veins on the right side are closed.

View larger version (53K): [in this window] [in a new window]   Fig 3. . Construction of the anterior wall of the recipient right atrium using part of the free wall of the right and left atrium.

View larger version (72K): [in this window] [in a new window]   Fig 4. . Complexion of the transplantation anastomosing the donor right atrium with the megaatrium of the recipient formed with both right and left atrium. If necessary a pericardial patch can be used to enlarge this anastomosis and allow a better blood flow.

View larger version (43K): [in this window] [in a new window]   Fig 5. . Lateral view of the heart-lung transplantation showing how this technique is going to pull the apex anteriorly.

View larger version (66K): [in this window] [in a new window]   Fig 6. . Clockwise rotation of the left atrium and its relation to the right atrium. For better understanding both ventricles and right donor atrium have been removed.

	Comment

Top Footnotes Abstract Introduction Method Comment References

Two different approaches have been described in heart transplantation with situs inversus and no pulmonary disease. Doty and colleagues [4] used extracardiac reconstruction of the systemic venous return to the heart after total excision of the right atrium. They reconstructed the superior vena cava using the innominate vein on the right side of the recipient and the superior vena cava of the donor. Rerouting of the inferior vena cava required the construction of a composite conduit using the right atrium and the in situ pericardium over the diaphragm. A second approach proposed by Michler and Sandhu [5] consists of the creation of two autologous left-sided atrial tissue baffles tunneling the left superior vena cava and inferior vena cava to the right of the pulmonary veins.

The increased propensity for infection in patients with Kartagener's syndrome contributes to the development of bronchitis and bronchiectasis, and finally pulmonary failure. Heart-lung or double-lung transplantation have been the two surgical approaches for these patients. Double-lung transplantation is advised in patients with end-stage pulmonary disease without pulmonary hypertension and with normal heart function [6]. Both procedures require modifications of the standard transplant techniques.

In the case of double-lung transplantation, the surgical approach is by sternotomy or bilateral thoracotomy. All patients reported by Macchiari and co-workers [6] presented inverse lung airway position. That is the main technical problem, which requires a double bronchial anastomosis with the increased risk of bronchial dehiscence or stenosis [7]. One of the reported patients required right lower lobectomy because of dextrocardia precluding expansion of the right lobe.

Heart-lung transplantation, on the other hand, presents the challenge of the right atrial anastomosis with no special problems in connecting the aorta and trachea. Because the vena cavae are located on the left side, the creation of a large atrium including the native right and left atrium (resection the atrial septum), are necessary to establish a connection in between the right recipient atrium and right donor atrium. One inconvenience is the large atrial size that could facilitate blood stasis and thrombosis. In 2 years of follow-up, there is no evidence of pulmonary thromboembolic events in our 2 patients. One advantage of the heart-lung technique is a single tracheal anastomosis decreasing the risk of stenosis or dehiscence. Furthermore, the heart is positioned in a sightly rotated and anterior pointing position but causes no compression of the lung. The exposure of the peritracheal area is excellent, providing opportunity for control of the bronchial arteries and therefore decreased risk of postoperative bleeding. In our first patient we enlarged the right atrium with a pericardial patch allowing a better blood flow through the atrial conduit and decreased tension on the atrial suture line, but this enlargement was not required in the second patient. In both patients the lungs were positioned in the chest passing anterior to the phrenic nerves allowing less manipulation of these structures, thus decreasing the risk of phrenic paralysis.

We have been following up these patients for 2 years and found no thromboembolic episodes or venous stasis. We conclude that heart-lung transplantation remains an effective modality for the treatment of patients with situs inversus associated with pulmonary end-stage disease, in spite of the major atrial modification that this required.

	Footnotes

Top Footnotes Abstract Introduction Method Comment References

 
Address reprint requests to Dr Copeland, Department of Cardiovascular and Thoracic Surgery, The University of Arizona Health Sciences Center, 1501 N Campbell Ave, Tucson, AZ 85724.

	References

Top Footnotes Abstract Introduction Method Comment References

 

1. 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;103:307–13.[Abstract]
2. Reitz BA. Heart and lung transplantation. In: Baumgartner WA, Reitz BA, Achuff SC, eds. Heart and heart-lung transplantation. Philadelphia: Saunders, 1990:319-46.
3. Tabeta H, Hiroshima K. A case of Kartagener's syndrome with bilateral superior vena cava and absent inferior vena cava. Nippon Kyobu Shikkan Gakkai Zasshi 1991;29:507–11.
4. Doty DB, Renlund DG, Caputo GR, Burton NA, Jones KW. Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 1990;99:493–9.[Abstract]
5. Michler RE, Sandhu AA. Novel approach for orthotopic heart transplantation in visceroatrial situs inversus. Ann Thorac Surg, 1995;60:194–7.[Abstract/Free Full Text]
6. Macchiarini P, Chapelier A, Vouhé P, et al. Double lung transplantation in situs inversus with Kartagener's syndrome. J Thorac Cardiovasc Surg 1994;108:86–91.[Abstract/Free Full Text]
7. Patterson GA, Todd TR, Cooper JD, Pearson FG, Winton TL, Maurer J. Airway complications after double lung transplantation. J Thorac Cardiovasc Surg 1990;99:14–21.[Abstract]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Gregorio Rábago Jack G. Copeland, III Felice Rosapepe Andrew C. Tsen David A. Arzouman Francisco A. Arabia Gulshan K. Sethi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rábago, G. Articles by Sethi, G. K. Search for Related Content PubMed PubMed Citation Articles by Rábago, G. Articles by Sethi, G. K.