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HeartWare implantation in a patient with situs inversus totalis
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COMMENTS AND OPINIONS HeartWare implantation in a patient with situs inversus totalis Ali Haitam Abdul Jabbar, MD,a Whitson B. Etheridge II, MD,b and O. H. Frazier, MDc,d From the aDivision of Cardiology, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas; bSection of Nephrology, Department of Internal Medicine, St. Luke’s Episcopal Hospital, Houston, Texas; cDepartment of Cardiopulmonary Transplantation, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas; and the dCenter for Cardiac Support, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas.
Patients with ventriculo-systemic discordance, a consequence of many congenital heart diseases, frequently develop systemic ventricular failure. This condition necessitates advanced heart failure treatment, heart transplantation, or mechanical assistance. One such congenital heart disease is situs inversus totalis. This condition is rare, but a disproportionate number of patients with this condition have complex congenital abnormalities. These abnormalities pose a set of unique surgical challenges, including those related to providing mechanical circulatory support. We report the placement of a HeartWare (HeartWare International Inc, Framingham, MA) continuous-flow ventricular assist device (VAD) in a patient with situs inversus totalis and congenitally corrected transposition of the great arteries (CCTGA). A 49-year-old man with CCTGA and situs inversus was admitted for worsening dyspnea. At age 13, he had undergone sub-pulmonic resection and surgical closure of atrial and ventricular septal defects. During the current admission, he developed cardiogenic shock and required intra-aortic balloon pump support. He was transferred to our institution for advanced heart failure treatment. The patient could not be weaned from the balloon pump. His creatinine level increased to 2.4 mg/dl, bilirubin increased to 3.2 mg/dl, aspartate aminotransferase was 65 IU/liter, and alanine aminotransferase was 100 IU/liter. On hospital Day 11, we implanted a HeartWare VAD in the right chest (Figure 1). The inlet cannula was inserted through the diaphragmatic surface of the right (systemic) ventricle, and the outflow aortic graft was anastomosed to the ascending aorta in the standard left–right plane. Post-operatively, the patient developed worsening inflammatory hepatopathy and acute kidney injury and required dialysis. However, his end-organ function subsequently recovered completely, and he was discharged on
Figure 1 An X-ray image shows the implanted HeartWare ventricular assist device in the patient’s right chest.
Day 62 on anticoagulant therapy, a b-blocker, and sildenafil. He made regular follow-up visits to our outpatient clinic and was able to return to work.
Discussion Situs inversus totalis occurs in 1:8,500 births. The incidence of congenital heart disease is disproportionately high in individuals with this disorder relative to the general population. Atrioventricular and ventricular-systemic discordance are the most common abnormalities, but CCTGA also occurs. Itself a rare disorder, CCTGA constitutes approximately 1% of all congenital heart abnormalities. Among individuals with CCTGA and associated abnormalities, 67% develop congestive heart failure by age 45. Heart transplants have been performed in patients with situs inversus totalis, but the venous anastomosis is technically difficult and carries significant risk. Munoz-Guijosa et al1 reported a transplant in a patient with situs inversus totalis, TGA, and a previous Mustard procedure. We and others have reported VAD implantation in patients with CCTGA and in patients with dextrocardia.2,3 Faggian et al4 recently reported placing a HeartMate II VAD (Thoratec Corporation, Pleasanton, CA) in a patient with situs inversus totalis. Our patient with CCTGA and situs inversus totalis had complex anatomy that made heart transplantation technically difficult, so we chose to insert a continuous-flow pump to support the failing systemic ventricle. As continuous-flow pumps become smaller and more dependable, and as we learn more about the mechanical-physiologic interaction their use entails, they increasingly provide a needed therapeutic option in the growing population of adults with
1053-2498/$ - see front matter r 2013 International Society for Heart and Lung Transplantation. All rights reserved. http://dx.doi.org/10.1016/j.healun.2012.11.021
Comments and Opinions
271
congenital heart disease and systemic ventricular failure, particularly in view of the shortage of transplantable organs and these patients’ often complex anatomy.
From the aIntensive Care Unit; and the bLung Transplant Centre, CHU UCL Mont-Godinne–Dinant, Universite Catholique de Louvain, Yvoir, Belgium.
Disclosure statement
A 57-year-old man with terminal usual interstitial pneumonia, treated by corticosteroids, was transferred to our intensive care unit after worsening respiratory distress that required tracheal intubation and mechanical ventilation. Rapidly, the patient underwent bilateral lung transplantation under extracorporeal membrane oxygenation. The transplant lung was ABO-compatible: the recipient’s blood group was Aþ and the donor was Oþ. Extracorporeal membrane oxygenation was weaned after 1 day and mechanical ventilation after 2 days. Post-operative induction immunosuppression was based on methylprednisolone, azathioprine, cyclosporine, and basiliximab. On Post-operative Day (POD) 10, intermittent hemodialysis was started due to anuric renal failure (urea, 311 mg/dl; creatinine, 2.6 mg/dl). On POD 11, the patient developed a hemolytic anemia (hemoglobin, 6.7 g/dl; lactate dehydrogenase, 4658 UI/l; bilirubin, 24.5 mg/dl; and haptoglobin, o 22 mg/dl; Figure 1). Because cyclosporinerelated microangiopathy was raised, cyclosporine was replaced by tacrolimus. However, this diagnosis was not confirmed because the schistocytes count was within the normal reference range (o 0.l%). Despite transfusions of Aþ red blood cells (RBCs), the anemia worsened secondary to an increase in hemolysis. Therefore, the passenger lymphocyte syndrome (PLS)
None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.
References 1. Mun˜oz-Guijosa C, Ginel A, Montiel J, Padro JM. Orthotopic heart transplantation in a patient with situs invs, transposition of the great arteries and Mustard operation. Eur J Cardiothorac Surg 2008;34:219-21. 2. Southard RE, Nelson JC, Joyce DL, et al. Placement of a left ventricular assist device in a patient with dextrocardia. J Heart Lung Transplant 2005;24:338-9. 3. Sugiura T, Kurosawa H, Shin’oka T, Kawai A. Successful explantation of ventricular assist device for systemic ventricular assistance in a patient with congenitally corrected transposition of the great arteries. Interact Cardiovasc Thorac Surg 2006;5:792-3. 4. Faggian G, Forni A, Luciani GB. LVAD in situs viscerum inversus totalis. J Heart Lung Transplant 2011;30:1420-1.
Passenger lymphocyte syndrome mimicking hemolytic uremic syndrome after lung transplantation Geoffrey Horlait, MD,a Pierre Bulpa, MD,a,b and Patrick Evrard, MD, PhD,a,b
Figure 1 Post-operative timeline detailing passenger lymphocyte–mediated hemolysis. Arrows indicate units of packed red blood cells (RBCs), with k representing Aþ blood (6 units) given until exclusion of microangiopathy and m representing Oþ blood (12 units). The period of donor-identical blood group transfusion requirement spanned 29 days. LDH, lactate dehydrogenase.
COMMENTS AND OPINIONS HeartWare implantation in a patient with situs inversus totalis Ali Haitam Abdul Jabbar, MD,a Whitson B. Etheridge II, MD,b and O. H. Frazier, MDc,d From the aDivision of Cardiology, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas; bSection of Nephrology, Department of Internal Medicine, St. Luke’s Episcopal Hospital, Houston, Texas; cDepartment of Cardiopulmonary Transplantation, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas; and the dCenter for Cardiac Support, the Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas.
Patients with ventriculo-systemic discordance, a consequence of many congenital heart diseases, frequently develop systemic ventricular failure. This condition necessitates advanced heart failure treatment, heart transplantation, or mechanical assistance. One such congenital heart disease is situs inversus totalis. This condition is rare, but a disproportionate number of patients with this condition have complex congenital abnormalities. These abnormalities pose a set of unique surgical challenges, including those related to providing mechanical circulatory support. We report the placement of a HeartWare (HeartWare International Inc, Framingham, MA) continuous-flow ventricular assist device (VAD) in a patient with situs inversus totalis and congenitally corrected transposition of the great arteries (CCTGA). A 49-year-old man with CCTGA and situs inversus was admitted for worsening dyspnea. At age 13, he had undergone sub-pulmonic resection and surgical closure of atrial and ventricular septal defects. During the current admission, he developed cardiogenic shock and required intra-aortic balloon pump support. He was transferred to our institution for advanced heart failure treatment. The patient could not be weaned from the balloon pump. His creatinine level increased to 2.4 mg/dl, bilirubin increased to 3.2 mg/dl, aspartate aminotransferase was 65 IU/liter, and alanine aminotransferase was 100 IU/liter. On hospital Day 11, we implanted a HeartWare VAD in the right chest (Figure 1). The inlet cannula was inserted through the diaphragmatic surface of the right (systemic) ventricle, and the outflow aortic graft was anastomosed to the ascending aorta in the standard left–right plane. Post-operatively, the patient developed worsening inflammatory hepatopathy and acute kidney injury and required dialysis. However, his end-organ function subsequently recovered completely, and he was discharged on
Figure 1 An X-ray image shows the implanted HeartWare ventricular assist device in the patient’s right chest.
Day 62 on anticoagulant therapy, a b-blocker, and sildenafil. He made regular follow-up visits to our outpatient clinic and was able to return to work.
Discussion Situs inversus totalis occurs in 1:8,500 births. The incidence of congenital heart disease is disproportionately high in individuals with this disorder relative to the general population. Atrioventricular and ventricular-systemic discordance are the most common abnormalities, but CCTGA also occurs. Itself a rare disorder, CCTGA constitutes approximately 1% of all congenital heart abnormalities. Among individuals with CCTGA and associated abnormalities, 67% develop congestive heart failure by age 45. Heart transplants have been performed in patients with situs inversus totalis, but the venous anastomosis is technically difficult and carries significant risk. Munoz-Guijosa et al1 reported a transplant in a patient with situs inversus totalis, TGA, and a previous Mustard procedure. We and others have reported VAD implantation in patients with CCTGA and in patients with dextrocardia.2,3 Faggian et al4 recently reported placing a HeartMate II VAD (Thoratec Corporation, Pleasanton, CA) in a patient with situs inversus totalis. Our patient with CCTGA and situs inversus totalis had complex anatomy that made heart transplantation technically difficult, so we chose to insert a continuous-flow pump to support the failing systemic ventricle. As continuous-flow pumps become smaller and more dependable, and as we learn more about the mechanical-physiologic interaction their use entails, they increasingly provide a needed therapeutic option in the growing population of adults with
1053-2498/$ - see front matter r 2013 International Society for Heart and Lung Transplantation. All rights reserved. http://dx.doi.org/10.1016/j.healun.2012.11.021
Comments and Opinions
271
congenital heart disease and systemic ventricular failure, particularly in view of the shortage of transplantable organs and these patients’ often complex anatomy.
From the aIntensive Care Unit; and the bLung Transplant Centre, CHU UCL Mont-Godinne–Dinant, Universite Catholique de Louvain, Yvoir, Belgium.
Disclosure statement
A 57-year-old man with terminal usual interstitial pneumonia, treated by corticosteroids, was transferred to our intensive care unit after worsening respiratory distress that required tracheal intubation and mechanical ventilation. Rapidly, the patient underwent bilateral lung transplantation under extracorporeal membrane oxygenation. The transplant lung was ABO-compatible: the recipient’s blood group was Aþ and the donor was Oþ. Extracorporeal membrane oxygenation was weaned after 1 day and mechanical ventilation after 2 days. Post-operative induction immunosuppression was based on methylprednisolone, azathioprine, cyclosporine, and basiliximab. On Post-operative Day (POD) 10, intermittent hemodialysis was started due to anuric renal failure (urea, 311 mg/dl; creatinine, 2.6 mg/dl). On POD 11, the patient developed a hemolytic anemia (hemoglobin, 6.7 g/dl; lactate dehydrogenase, 4658 UI/l; bilirubin, 24.5 mg/dl; and haptoglobin, o 22 mg/dl; Figure 1). Because cyclosporinerelated microangiopathy was raised, cyclosporine was replaced by tacrolimus. However, this diagnosis was not confirmed because the schistocytes count was within the normal reference range (o 0.l%). Despite transfusions of Aþ red blood cells (RBCs), the anemia worsened secondary to an increase in hemolysis. Therefore, the passenger lymphocyte syndrome (PLS)
None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.
References 1. Mun˜oz-Guijosa C, Ginel A, Montiel J, Padro JM. Orthotopic heart transplantation in a patient with situs invs, transposition of the great arteries and Mustard operation. Eur J Cardiothorac Surg 2008;34:219-21. 2. Southard RE, Nelson JC, Joyce DL, et al. Placement of a left ventricular assist device in a patient with dextrocardia. J Heart Lung Transplant 2005;24:338-9. 3. Sugiura T, Kurosawa H, Shin’oka T, Kawai A. Successful explantation of ventricular assist device for systemic ventricular assistance in a patient with congenitally corrected transposition of the great arteries. Interact Cardiovasc Thorac Surg 2006;5:792-3. 4. Faggian G, Forni A, Luciani GB. LVAD in situs viscerum inversus totalis. J Heart Lung Transplant 2011;30:1420-1.
Passenger lymphocyte syndrome mimicking hemolytic uremic syndrome after lung transplantation Geoffrey Horlait, MD,a Pierre Bulpa, MD,a,b and Patrick Evrard, MD, PhD,a,b
Figure 1 Post-operative timeline detailing passenger lymphocyte–mediated hemolysis. Arrows indicate units of packed red blood cells (RBCs), with k representing Aþ blood (6 units) given until exclusion of microangiopathy and m representing Oþ blood (12 units). The period of donor-identical blood group transfusion requirement spanned 29 days. LDH, lactate dehydrogenase.