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Ann Thorac Surg 1997;64:1456-1458
© 1997 The Society of Thoracic Surgeons

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Case Report

Single-Lung Transplantation in a Patient With Cystic Fibrosis and an Asymmetric Thorax

Departments of Thoracic and Cardiovascular Surgery and Paediatrics, Essen University Medical School, Essen, Germany

Accepted for publication July 1, 1997.

	Abstract

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We report metachronous single-lung transplantation for cystic fibrosis after contralateral pneumonectomy. Kyphoscoliosis and mediastinal shift required careful donor-lung sizing with computed tomography and was not dependent on typical parameters. Severe reperfusion injury was treated with nitric oxide, C1-esterase inhibitor, and continuous venovenous hemodialysis. The patient was extubated on the fifth postoperative day and is alive and well. We conclude that single-lung transplantation after contralateral pneumonectomy for patients with cystic fibrosis and an asymmetric chest and evident lung volume mismatch may be an acceptable functional therapeutic option.

	Introduction

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See also page 1458.

The treatment of choice for end-stage cystic fibrosis is bilateral sequential lung transplantation or heart-lung transplantation [1, 2]. Both result in improved pulmonary function and removal of all contaminated lung tissue as a source of recurrent infections. Single-lung transplantation for cystic fibrosis has only been reported twice: once with simultaneous contralateral pneumonectomy [3] and once as a result of removal of the left allograft on the tenth day after primary bilateral transplantation [4].

An 11-year old patient underwent left single-lung transplantation for advanced cystic fibrosis 8 months after removal of a nonfunctioning and very small (due to the kyphoscoliosis; long-arched 40-degree kyphosis and 12-degree left convex scoliosis) right "destroyed lung," which had caused frequent contralateral and general infections. The main problem was the assessment of the correct dimensions for the donor organ.

The history was typical for a patient with cystic fibrosis and showed increasingly frequent pulmonary infections (Pseudomonas species), nonproductive cough, and progressive loss of pulmonary function. As of 1991, frequent in-hospital courses of antibiotic therapy were necessary, and by mid-1992, the patient's parents requested consideration of transplantation. The patient was originally accepted for transplantation in June 1992 with the intention to perform a single left lung transplantation and a simultaneous right pneumonectomy. In early 1994 the periods between severe general infectious (septic) episodes became shorter and the pulmonary ventilation/perfusion scans revealed that the function of the right lung had been reduced to near zero. Because the right lung was the main source of infections leading to deterioration of the patient's general condition, a right pneumonectomy was indicated to allow a longer waiting time. The resection was performed in March 1994 and resulted in a reduction of the infectious episodes and a relatively rapid improvement of the general condition. The pneumonectomy caused an additional mediastinal shift to the right, making the left hemithorax even larger. The improvement period was short, however: recurrent infectious exacerbations accompanied by a further pulmonary function drop led to urgent transplantation in November 1994 (Table 1).

View larger version (96K): [in this window] [in a new window]   Fig 1. . Constructing an imaginary donor thorax. The left hemithorax from the recipient's chest computed tomogram (A) serves as a source for a virtual donor thorax (B). For explanation, see text.

Based on the computed tomographic scan the deviated mediastinum was considered the midline of the thorax (Fig 1A) and we drew a mirror image of the left hemithorax on the right side (Fig 1B). An imaginary thorax resulted. Its depth (a) measured 22 cm and its width (b) 26 cm. From the formula for the circumference of an ellipse, the chest circumference was calculated as 75 cm. These parameters were compared with body data from other patients who underwent chest computed tomography for other reasons. From these parameters a donor with a weight ranging from 50 to 70 kg and height between 160 and 180 cm seemed desirable.

The organ obtained by distal procurement from a 13-year-old male donor of adult body size (170 cm, 55 kg) was perfused with modified Euro-Collins solution, explanted in a slightly inflated status, and transported on ice.

Access to the recipient lung was gained via a lateral thoracotomy through the fourth intercostal space. The left hemithorax was voluminous but deformed due to prior right pneumonectomy and marked kyphoskoliosis. Because the ascending aorta was out of reach, the left subclavian artery and pulmonary artery were cannulated for cardiopulmonary bypass. After left pneumonectomy, all three anastomoses were made in typical fashion. There were no intraoperative complications. The total ischemic time was 163 minutes. After reperfusion the patient was weaned from cardiopulmonary bypass without difficulty. Total bypass time was 57 minutes. During the initial postbypass phase, the graft appeared macroscopically normal and oxygenated well (oxygen tension of 440 mm Hg and carbon dioxide tension of 57 mm Hg at an inspired oxygen fraction of 1.0, and oxygen tension of 306 mm Hg and carbon dioxide tension of 43 mm Hg at an inspired oxygen fraction of 0.5 and 8 L/min ventilation).

The postoperative course was complicated by severe reperfusion injury, which developed on the first postoperative day. Fluid retention of 4,650 mL in the first 2 days in spite of high-dose furosemide led to extreme interstitial pulmonary edema. Despite adequate ventilation with an inspired oxygen fraction gradually increased from 0.3 to 1.0, positive end-expiratory pressure from 7 to 12 mbar, and inspiratory pressure from 28 to 50 mbar, the oxygen tension diminished to 100 mm Hg and carbon dioxide tension rose to 60 mm Hg.

We managed this condition successfully using nitric oxide insufflation (20 ppm) and C1 esterase inhibitor (Berinert; Centeon Pharma GmbH, Marburg, Germany) application (first dose, 6,000 IU intravenously; second dose, 3,000 IU intravenously; third and fourth doses, 1,500 IU intravenously given 12 hours apart) on the first and second days, and continuous venovenous hemodialysis (flow 1 L/h) from the second to the fourth postoperative days. After each C1 esterase inhibitor dose we observed a rise in urine output and in the dialysis fraction. From the second to the fourth postoperative days, 5,300 mL of fluid was eliminated. Pulmonary function recovered gradually from the second postoperative day on, and the patient was extubated on the fifth postoperative day (Table 2). Antibiotic therapy was continued without interruption using ceftazidim, amikacin, and ciprofloxacin as dictated by the preoperative bacterial sensitivities. However, by the eighth postoperative day, Pseudomonas aeruginosa was again isolated from the patient's tracheal secretions.

	Comment

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End-stage cystic fibrosis used to be treated by bilateral sequential lung transplantation [5]. In these patients single-lung transplantation would inevitably lead to infectious complications of the allograft or to the development of contralateral empyema after simultaneous pneumonectomy [4]. Because of chest deformity and lung mismatch, bilateral lung transplantation was not feasible in this case.

Clinical deterioration forced us to perform a "two-stage" procedure: a right pneumonectomy first and left single-lung transplantation later. The pneumonectomy was well tolerated because the right lung was without function, as demonstrated by perfusion scanning. The course thereafter was uneventful; the pneumonectomy space filled up adequately without empyema or bronchial stump insufficiency.

A particular problem in this case was to estimate the required size for the donor organ. This was resolved by making exact measurements on chest computed tomography and by deriving parameters for an "oversized" organ compared with the standard body size measurements.

The transplanted "oversized" lung fit very well. Our method to estimate the donor parameters in such cases proved to be precise.

Although this "two-stage" approach was born out of a predicament, it proved to be feasible in a patient with only one functioning lung. We consider this approach safer than simultaneous pneumonectomy, because it avoids the risk of bronchial stump dehiscence as well as pleural empyema in the early immunosuppresive course. The exact determination of the size of the donor lung, as described above, makes it possible to obtain a very well matched organ.

	Addendum

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During the peer-review process, 2 years after transplantation, a bronchial anastomotic stenosis developed in the patient on the basis of chondromalacia. During attempted stent placement the bronchus was perforated. The patient was transferred to our hospital. An emergency operation with extracorporeal circulation was performed to repair the bronchus lesion. The patient died 4 weeks later of pulmonary failure.

	Acknowledgments

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The skillful technical assistance of Hasan Bushnag is appreciated.

	Footnotes

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Address reprint requests to Dr Piotrowski, Klinik für Thorax- und Kardiovaskuläre Chirurgie, Universitätsklinik, Hufelandstr 55, 45122 Essen, Germany.

	References

Top Footnotes Abstract Introduction Comment Addendum Acknowledgments References

 

1. De Hoyos AL, Patterson GA, Maurer R, et al. Pulmonary transplantation. Early and late results. J Thorac Cardiovasc Surg 1992;103:295–306.[Abstract]
2. De Leval MR, Smyth R, Whitehead B, et al. Heart and lung transplantation for terminal cystic fibrosis. J Thorac Cardiovasc Surg 1991;101:633–42.[Abstract]
3. Forty M, Hasa A, Gould FK, et al. Single lung transplantation with contralateral pneumonectomy for cystic fibrosis. J Heart Lung Transplant 1994;13:727–30.[Medline]
4. Shennib H, Massard G, Gauthier R, et al. Single lung transplantation for cystic fibrosis: is it an option? J Heart Lung Transplant 1993;12:288–93.[Medline]
5. Shennib H, Noirclerc M, Ernst P, et al. Double lung transplantation for cystic fibrosis. Ann Thorac Surg 1992;54:27–32.[Abstract/Free Full Text]

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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): Jarowit A. Piotrowski Fred H. Splittgerber Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Piotrowski, J. A. Articles by Zerkowski, H.-R. Search for Related Content PubMed PubMed Citation Articles by Piotrowski, J. A. Articles by Zerkowski, H.-R. Related Collections Related Article