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Ann Thorac Surg 1998;65:823-825
© 1998 The Society of Thoracic Surgeons

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Case Reports

Combined Heart and Single-Lung Transplantation in Complex Congenital Heart Disease

Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, California, USA,
Section of Cardiothoracic Surgery, Veterans Affairs Palo Alto Health Care System, Palo Alto, California, USA

Accepted for publication October 27, 1997.

Dr Fann, Department of Cardiothoracic Surgery, Stanford University Medical Center, 300 Pasteur Dr, Stanford, CA 94305.

	Abstract

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We present a patient with a history of tricuspid and pulmonary atresia who underwent a classic Glenn shunt and a Potts shunt during childhood, resulting in different right and left pulmonary physiology. Because of progression of cardiopulmonary disease and the fact that the right lung was "protected," the patient underwent combined heart–left single-lung transplantation. The postoperative course was uneventful. Potential early and late advantages of this approach include simplifying of the operative procedure and mitigating the potential effects of obliterative bronchiolitis.

	Introduction

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Combined heart-lung transplantation is an established therapeutic modality for end-stage cardiopulmonary disease, such as congenital heart disease and Eisenmenger’s syndrome and primary pulmonary hypertension and secondary heart failure [1][2][3][4][5]. In these cases, isolated cardiac transplantation is contraindicated, because the elevated pulmonary vascular resistance would result in acute right heart failure; similarly, single-lung or double-lung transplantation would not provide adequate relief of the underlying disease process. We present a case of a progressively symptomatic patient with congenital heart disease who previously underwent a classic Glenn shunt and a Potts shunt resulting in differential right and left pulmonary physiology; the treatment was thus directed at the underlying cardiac disorder and the left pulmonary pathology.

The patient was a 33-year-old man with tricuspid atresia and pulmonary atresia. He underwent a classic Glenn shunt (end-to-end superior vena caval to right pulmonary arterial anastomosis) at age 1 year and a Potts shunt (descending aorta to the left pulmonary arterial anastomosis) at age 7 years. He presented with progressive dyspnea, hypoxia, and worsening cardiac failure. He did not have any active infections and had no history of cigarette smoking. His only medication was metachlorpramide for nausea. On examination, the patient was markedly cyanotic with clubbing. He was afebile, heart rate was 100 beats/min, and blood pressure was 110/70 mm Hg. Lung examination showed clear lung fields bilaterally. Cardiac examination demonstrated a systolic ejection murmur at the left precordium. His abdominal and neurologic examinations were unremarkable. Laboratory tests showed a white blood cell count of 4.8 x 10⁹/L, hematocrit of 53%, and normal liver function tests. Chest radiograph showed enlarged cardiac silhouette with increased pulmonary vasculature on the left. The patient was initially evaluated 3 years ago at the University Hospital and accepted as a candidate for heart-lung transplantation. At that time, pulmonary function test demonstrated a forced vital capacity of 3.03 L (63% of predicted) and a forced expiratory volume in 1 second of 2.50 L (64%); arterial blood gases showed pH of 7.40, carbon dioxide tension of 38 mm Hg, oxygen tension of 45 mm Hg, and HCO₃ level of 23.2 mEq/L. Transthoracic echocardiography 3 years ago demonstrated enlarged and hypertrophic left ventricle with markedly reduced systolic function, mild aortic insufficiency, and moderate mitral regurgitation. Magnetic resonance imaging and high-resolution computed tomography showed diminutive right ventricle with enlarged left ventricle and patent ventricular septal defect. Both Glenn and Potts shunts were patent, and the left pulmonary artery was enlarged. Ten years before evaluation for heart-lung transplantation, the patient had undergone cardiac catheterization demonstrating a superior vena caval pressure of 16 mm Hg, pulmonary artery pressure of 40/25 mm Hg with a mean of 29 mm Hg, left pulmonary capillary wedge pressure of 18 mg, aortic pressure of 136/80 mg, and an estimated left ventricular ejection fraction of 0.25.

After general anesthesia was achieved, a median sternotomy was performed. The ascending aorta was dissected from the atretic pulmonary artery; the superior vena cava was exposed to the level of the anastomosis to the right pulmonary artery (Glenn shunt). The inferior vena cava was isolated. The left pleural adhesions were lysed and the left lung was mobilized. After cannulation of the ascending aorta (22F cannula) and the superior (24F angled cannula) and inferior venae cavae (28F cannula), cardiopulmonary bypass was instituted. The Potts shunt was divided and its connection to the aorta oversewn. The atretic pulmonary artery was divided and oversewn. After the aorta was cross-clamped, the proximal ascending aorta was transected. The heart was explanted just proximal to the superior vena caval–right pulmonary arterial anastomosis; an inferior vena caval cuff and a right pulmonary vein cuff were created. The left lung was removed by dividing the left main bronchus (Fig 1). The donor heart-lung block was prepared. The right donor lung was removed, leaving a long pulmonary artery cuff attached to the donor heart; the right donor pulmonary veins were divided, leaving a left atrial cuff. The heart–single-lung block was placed in the chest cavity and the left bronchial anastomosis was performed with continuous 4-0 polypropylene suture. The heart was brought into the pericardial cavity posterior to the phrenic nerve. The native right pulmonary vein was anastomosed to the left atrium using 4-0 polypropylene suture. The inferior vena caval anastomosis was performed with 4-0 polypropylene suture. The native right pulmonary artery was disconnected from the superior vena cava and anastomosed in an end-to-end fashion to the donor right pulmonary artery using 5-0 polypropylene suture. The superior vena caval anastomosis was performed using 5-0 polypropylene suture. The aortic anastomosis was performed using 4-0 polypropylene suture. After deairing maneuvers, the aortic cross-clamp was removed and the patient was weaned from cardiopulmonary bypass. The cardiopulmonary bypass time was 3 hours 2 minutes, the aortic cross-clamp time was 2 hours 34 minutes, and the graft ischemic time was 3 hours 22 minutes.

View larger version (38K): [in this window] [in a new window]   Diagram of the operative field after explantation of the heart and left lung. Cannulas were placed in the ascending aorta, superior vena cava, and inferior vena cava. The atretic main pulmonary artery was oversewn. Implantation of the heart–single-lung block was accomplished by performing the left bronchial anastomosis; the heart was placed in the pericardial cavity with the left hilum posterior to the phrenic nerve. The remaining anastomoses were performed in the following sequence: right pulmonary vein–left atrial anastomosis, inferior vena caval anastomosis, pulmonary arterial anastomosis, superior vena caval anastomosis, and aortic anastomosis.

	Comment

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In this patient with tricuspid and pulmonary atresia, two previous shunt operations were performed during childhood, resulting in different right and left pulmonary physiology. The right lung could be considered to be in a "protected" environment with inflow exclusively from the superior vena cava; the left lung, on the other hand, sustained progressive pulmonary vascular disease and hypertension as a result of the systemic blood flow through the Potts shunt. Because of continued cardiopulmonary deterioration, the patient underwent combined heart-lung transplantation. Because the native right lung was relatively undiseased, salvage of that lung seemed attractive and thus a combined heart–single-lung (left) transplantation was performed.

Advantages of this approach of combined heart–single-lung transplantation include operative considerations, such as limiting the amount of dissection in the right chest, thereby decreasing the risk of coagulopathy, which is a known risk factor for postoperative complications [2][4]. Other real and potential benefits of this approach include long-term issues, such as preservation of native lung tissue, thereby decreasing the potential effects of obliterative bronchiolitis, and possible protection from cardiac events or rejection by undergoing the combined transplantation. Obliterative bronchiolitis, likely the result of chronic immune injury, affects more than 50% of all transplant recipients and remains the major complication in the long-term follow-up after lung transplantation or heart-lung transplantation [1][2][5]. Should obliterative bronchiolitis develop in this patient, it would be limited to the transplanted left lung; thus, the clinical effects would be somewhat mitigated because of his native right lung.

Rejection of the heart (either isolated or simultaneous with lung rejection) in patients who underwent combined heart-lung transplantation is less frequent than in patients with isolated heart transplantation [1][4]. At 5 years, 37% of heart-lung transplant recipients were free from heart rejection compared with 7.2% of heart transplant recipients [4]. Graft coronary artery disease also occurred less frequently in patients with combined heart-lung transplantation compared with those with isolated heart transplantation [4]. In the case presented, we suspect that the patient’s transplanted heart may be somewhat protected from cardiac rejection and graft coronary artery disease because the transplantation was performed using a heart-lung block: this concept, however, remains speculative.

In summary, we present a case of a patient with an uncommon pulmonary physiology who underwent a combined heart–single-lung transplantation for progressive cardiopulmonary failure. Potential early and late advantages of this approach include simplifying the operative procedure, mitigating the effects of obliterative bronchiolitis, and possibly decreasing the risk of cardiac rejection and graft coronary artery disease.

	References

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1. McGregor CGA, Jamieson SW, Baldwin KC, et al. Combined heart-lung transplantation for end-stage Eisenmenger’s syndrome. J Thorac Cardiovasc Surg 1986;91:443-450.[Abstract]
2. Harjula A, Baldwin JC, Starnes VA, et al. Proper donor selection for heart-lung transplantation: the Stanford experience. J Thorac Cardiovasc Surg 1987;94:874-880.[Abstract]
3. Reitz BA Heart-lung transplantation: consensus, experience, or both?. Ann Thorac Surg 1993;56:208.[Free Full Text]
4. Sarris GE, Smith JA, Shumway NE, et al. Long-term results of combined heart-lung transplantation: the Stanford experience. J Heart Lung Transplant 1994;13:940-949.[Medline]
5. Reichenspurner H, Girgis RE, Robbins RC, et al. Stanford experience with obliterative bronchiolitis after lung and heart-lung transplantation. Ann Thorac Surg 1996;62:1467-1473.[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): James I. Fann Michael K. Wilson Bruce A. Reitz Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Fann, J. I. Articles by Reitz, B. A. Search for Related Content PubMed PubMed Citation Articles by Fann, J. I. Articles by Reitz, B. A.