Aortic Valve Bypass for Aortic Stenosis in a Patient With Paroxysmal Nocturnal Hemoglobinuria Mehrdad Ghoreishi, Maria R.

Baer, Ranjana Bhargava, Craig E. Stauffer, Bartley P. Griffith and James S. Gammie Ann Thorac Surg 2010;90:279-281 DOI: 10.1016/j.athoracsur.2009.11.047

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The Annals of Thoracic Surgery is the official journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association. Copyright 2010 by The Society of Thoracic Surgeons. Print ISSN: 0003-4975; eISSN: 1552-6259.

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Ann Thorac Surg 2010;90:279 81

CASE REPORT GHOREISHI ET AL AORTIC VALVE BYPASS FOR PNH

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References
1. Enriquez-Sarano M, Akins CW, Vahanian A. Mitral regurgitation. Lancet 2009;373:138294. 2. Geidel S, Lass M, Ostermeyer J. Restrictive mitral valve annuloplasty for chronic ischemic mitral regurgitation: a 5-year clinical experience with the Physioring. Heart Surg Forum 2008;11:E22530. 3. Aleri O, Maisano F, De Bonis M, et al. The double-orice technique in mitral valve repair: a simple solution for complex problems. J Thorac Cardiovasc Surg 2001;122:674 81. 4. Kar S. Experience with the MitraClip therapy in the EVEREST II High Risk Registry. EuroPCR, Barcelona, May 20, 2009. 5. Geidel S, Lass M, Ostermeyer J. Seven-year experience with permanent atrial brillation ablation concomitant to mitral valve surgery in 152 patients. Heart Surg Forum 2008;11(3):E175 80.

Aortic Valve Bypass for Aortic Stenosis in a Patient With Paroxysmal Nocturnal Hemoglobinuria
Mehrdad Ghoreishi, MD, Maria R. Baer, MD, Ranjana Bhargava, MD, Craig E. Stauffer, BS, Bartley P. Grifth, MD, and James S. Gammie, MD
Department of Cardiac Surgery and Department of Medicine and Greenebaum Cancer Center, University of Maryland Medical Center, Baltimore, Maryland

Fig 2. Mitral valve repair by posterior sliding plasty, repair of the anterior leaet using three 4-0 Gore-Tex sutures (Ethicon, Somerville, NJ) as neochords implanted close to the free edge of the anterior leaet (top) and ring annuloplasty (bottom) (Carpentier-Edwards Physioring, size 34 [Edwards Lifesciences LLC, Irvine, CA]).

grade 3 or 4 with excellent clinical results [1, 2]. Our case report demonstrates that there is a substantial risk to reduce the benet of conventional MV surgery by a rst-step PMVR and intervention failure. In the described case, the valvular pathology had been deteriorated signicantly by the PMVR procedure leading to an additional chordal rupture. Particularly because of encouraging early results demonstrated in the EVEREST study, it must be anticipated that an increasing number of PMVR procedures will be performed in the near future and more cases after failed intervention will occur. Therefore surgical strategies must be adapted to this situation. Although SMVR after leaet clipping represents a challenge, the procedure can be successfully performed by an experienced team, even in complex situations. However, indications for PMVR should be discussed carefully, because the likelihood of a perfect surgical MV repair predicts the patients prognosis that can be possibly impaired afterward. At present the method of leaet clipping cannot be recommended as a rst option therapy. Surgical MV repair after failed PMVR by using the MitraClip device requires the complete spectrum of MV repair techniques. Valve pathology can be severely deteriorated, thereby reducing the chance for successful surgical repair after preceding percutaneous MV intervention.
2010 by The Society of Thoracic Surgeons Published by Elsevier Inc

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired bone marrow disorder characterized by hemolytic anemia, neutropenia, thrombocytopenia, hemoglobinuria, and venous thrombosis. Cardiopulmonary bypass-induced complement activation may be associated with exacerbation of hemolysis in patients with PNH undergoing cardiac operations. We describe a 77-year-old man with PNH and critical symptomatic aortic stenosis who underwent successful aortic valve bypass operation without cardiopulmonary bypass as an alternative to conventional aortic valve replacement. (Ann Thorac Surg 2010;90:279 81) 2010 by The Society of Thoracic Surgeons

aroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired stem-cell disorder that manifests clinically as intravascular hemolysis, cytopenias, and venous thrombosis. It is caused by an acquired mutation of the phosphatidylinositol glycan anchor biosynthesis, class A (PIG-A) gene in bone marrow stem cells that result in lack of expression of glycosylphosphatidylinositol (GPI)linked proteins, including the complement regulatory proteins CD55 and CD59. Affected cells have increased susceptibility to complement-mediated hemolysis [1]. Dr Gammie discloses that he has a nancial relationship with Correx, Inc.

Accepted for publication Nov 17, 2009. Address correspondence to Dr Gammie, University of Maryland Medical Center, 22 S Greene St, N4W94, Baltimore, MD 21201; e-mail: jgammie@ smail.umaryland.edu.

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CASE REPORT GHOREISHI ET AL AORTIC VALVE BYPASS FOR PNH

Ann Thorac Surg 2010;90:279 81

Cardiopulmonary bypass (CPB) in patients with PNH undergoing cardiac operations can cause complement activation and induce hemolytic crises. In addition, PNHinduced granulocytopenia and thrombocytopenia increases the risk of postoperative infection and bleeding [2]. Aortic valve bypass (AVB) with an apicoaortic conduit allows treatment of aortic stenosis by bypassing the stenotic valve with a valve-containing conduit constructed between the ventricular apex and the descending thoracic aorta [3]. AVB is performed without CPB, cardioplegic cardiac arrest, or sternotomy. A 77-year-old man was referred to our hospital with symptoms of progressive dyspnea, dizziness, and intermittent left chest pain. He had been found to be anemic and leukopenic 9 years previously, and his cytopenias had persisted over time and worsened in recent months. Flow cytometry 3.5 years before our evaluation had demonstrated a leukocyte population with absent CD55 expression, and the diagnosis of PNH was made. He had no history of hemoglobinuria or thrombosis. The patient was transfusionindependent until 6 months before admission, when worsening anemia developed along with dyspnea and dizziness. He underwent bare-metal coronary stent implantation 3 months before admission to treat a 75% left anterior descending stenosis. He complained of progressive dyspnea with minimal exertion. On physical examination, there was a 3/6 mid-peaking harsh systolic murmur at the upper sternal border and also at the apex. Transthoracic echocardiography showed severe aortic valve stenosis, with estimated valve area of 0.8 cm2, peak and mean pressure gradients of 56 and 36 mm Hg, respectively, and a left ventricle ejection fraction of 50%. Laboratory tests demonstrated hemoglobin, 11 mmol/L (transfused); white blood cell count, 2.4 109/L; absolute neutrophil count, 1.1 109/L; platelet count, 139 109/L; reticulocytes, 5.52%; haptoglobin, 8.2 mg/dL, total bilirubin, 1.7 mg/dL; direct bilirubin, 0.3 mg/dL; lactate dehydrogenase, 200 U/L; and creatinine, 1.2 mg/dL. Peglgrastim (6 mg) was administered 2 days before the operation to prevent perioperative exacerbation of neutropenia and increased risk of infection. At operation, the AVB conduit was constructed on the back table and included a 14-mm apical connector (Model 140, Medtronic Inc, Minneapolis, MN) anastomosed to a 19-mm Freestyle porcine stentless valve (Medtronic Inc) and an 18-mm straight Dacron (DuPont, Wilmington, DE) vascular graft. A small (4-inch) lateral thoracotomy in the sixth interspace was performed. The inferior pulmonary ligament was mobilized. Intravenous heparin (5000 U) was administered, and a partial occlusion clamp placed on the descending thoracic aorta. The distal anastomosis between the conduit and the aorta was performed with running 4-0 monolament suture. The pericardium was opened and a site for distal anastomosis was identied 2 cm lateral to the apex of the heart. Circumferential 3-0 Prolene (Ethicon, Somerville, NJ) pledgeted horizontal mattress sutures were placed deep in the apical myocardium and brought through the sewing
Fig 1. Distribution of blood ow during cardiac cycle (divided into 30 cine images) in the ascending aorta (black line), within the conduit (thick dashed line), the descending aorta proximal (preconduit, thin line) and distal (postconduit, thin dashed line) to the conduit insertion.

ring of the apical connector. A stab wound was made in the apex. A Foley catheter was inserted in the ventricle and inated, and a circular coring knife was used to remove a plug of myocardium. The apical connector was tied in place and ow was established through the AVB conduit after air was removed. Intraoperative conduit ow was 2.2 L/min and simultaneous cardiac output was 7.2 L/min. Procedural time was 2 hours. Blood loss volume was 400 mL, and no blood products were transfused during the operation. Postoperatively, the patient was treated with aspirin (325 mg daily). He was discharged home on day 9. Tests results before discharge included hemoglobin, 10.5 mmol/L; white blood cell count, 5.1 109/L; platelet count, 135,000; haptoglobin, 9 mg/dL; lactate dehydrogenase, 214 u/L; and creatinine, 1.1 mg/dL. The predischarge echocardiography showed an ejection fraction of 50% and conduit ow as a percentage of total cardiac output of 55%. Figure 1 demonstrates distribution of instantaneous blood ow during the cardiac cycle in cardiac magnetic resonance imaging of distinct regions of the aorta and conduit performed 1 week after the operation.

FEATURE ARTICLES

Comment
Few case reports have been published of cardiac operations with CPB in patients with PNH [4]. Contact of blood with foreign material surfaces during cardiac operations with CPB can induce complement activation and exacerbate hemolysis in patients with PNH. Hypoxemia, hypoperfusion, and hypercarbia associated with CPB can also contribute to accelerated hemolysis. PNH-induced thrombocytopenia increases the risk of bleeding, and the risk of postoperative infection is increased by PNH-induced granulocytopenia [5, 6]. Prophylactic administration of antibiotics and granulocyte colony-stimulating factor is recommended before elective operations in patients with PNH. We administered peglgrastim before the operation to prevent exacerbation of neutropenia. Others have recommended the preoperative transfusion of group-specic fresh blood to a normal hemoglobin level to decrease the concentration of GPI-decient red blood cells and the risk of hemolysis. Adequate uid administration and the use of diuretics are recommended to prevent renal complications [2].

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Ann Thorac Surg 2010;90:281 4

CASE REPORT DI LUOZZO ET AL CHYLOUS ASCITES IN TAA PATIENT

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As a result of our extensive experience performing AVB procedures in high-risk aortic stenosis patients, we reasoned that relief of left ventricle outow tract obstruction with an AVB operation would be associated with a lower risk of postoperative hemolysis, infection, and renal complications [3]. Key advantages of AVB operations include the absence of CPB, aortic cross-clamping, and nonpulsatile perfusion, all of which are recognized risk factors for postoperative complications in patients with PNH. Our patient enjoyed excellent hemodynamic relief of aortic stenosis through a small left thoracotomy without CPB or cardioplegic arrest. His rapid recovery and absence of postoperative hemolysis or other complications suggest that the AVB procedure may be an optimal approach for patients with PNH and aortic stenosis.
We thank Dr Laszlo Leb, Fallon Clinic, Worcester Medical Center, Worcester, Massachusetts, for helpful information, and Mary J. Byron, PA-C, for superb patient care.

is very specic to the etiology and clinical status of the patient. The mortality from chylous ascites is not negligible, and aggressive measures are often needed. A voluminous chylous ascites developed in a 38-year-old woman after an open abdominal aortic aneurysm repair that was refractive to initial therapeutic interventions. We used an extracorporeal peritoneovenous shunt to control the abdominal ascites and improve the patients immunologic and nutritional status. (Ann Thorac Surg 2010;90:281 4) 2010 by The Society of Thoracic Surgeons

References
1. Parker CJ. Bone marrow failure syndrome: paroxysmal nocturnal hemoglobinuria. Hematol Oncol Clin North Am 2009; 23:333 46. 2. Knobloch K, Lichtenberg A, Leyh RG, Schubert J. Aortic valve replacement and coronary revascularization in paroxysmal nocturnal hemoglobinuria. Interact Cardio Vasc Thorac Surg 2003;2:6479. 3. Gammie JS, Krowoski LS, Brown JM, et al. Aortic valve bypass surgery: midterm clinical outcomes in a high-risk aortic stenosis population. Circulation 2008;118:1460 6. 4. Dinesh D, Baker B, Carter JM. Cardiopulmonary bypass surgery in a patient with paroxysmal nocturnal haemoglobinuria. Transfus Med 2006;16:206 8. 5. Ascione R, Lloyd CT, Underwood MJ, Lotto AA, Pitsis AA, Angelini GD. Inammatory response after coronary revascularization with or without cardiopulmonary bypass. Ann Thorac Surg 2000;69:1198 204. 6. Kathirval S, Prakash A, Lokesh BN, Sujatha P. The anesthetic management of a patient with paroxysmal nocturnal hemoglobinuria. Anesth Analg 2000;91:1029 31.

hylous ascites is a rare condition that requires hospital admission [1, 2]. The diagnosis may be increasing secondary to aggressive intraabdominal and retroperitoneal operations and in oncologic patients with increased survival. The major causes of chylous ascites are categorized as congenital or acquired. Acquired conditions of chylous ascites include inammatory processes, neoplasm, postoperative, and trauma [2]. The prognosis of chylous ascites depends on the specic cause, with mortality rates as high as 40% to 70% [3]. However, postoperative lymphatic ascites can be resolved in approximately 60% of patients [3]. A few case series have described the management of persistent chylous leakage after an abdominal aneurysm repair. Chylous ascites is most commonly a result of the division of major lymphatic channels or innumerous minor channels during extensive retroperitoneal dissection. We report a 38-year-old patient with Marfan syndrome and previous repair of a Crawford type I thoracoabdominal aneurysm who subsequently required repair of an infrarenal abdominal aortic aneurysm. A largevolume chylous ascites developed that was refractory to initial conservative management, surgical ligation, and Denver shunt implantation. Our patient is a 38-year-old woman with a history of autoimmune hepatitis, hepatitis C, Marfan syndrome, hypertension, and Stanford type B aortic dissection during pregnancy. The autoimmune hepatitis was suppressed with azathioprine and prednisone. She underwent repair of a thoracoabdominal aortic aneurysm in 2006. During the initial operation, the aorta was replaced from the subclavian artery to the level of the renal arteries, with reimplantation of the mesenteric and renal arteries. Her recovery from this operation was uneventful. During the next 2 years, the infrarenal abdominal aorta expanded from 2.5 to 5.5 cm. In 2008, she underwent an abdominal aortic aneurysm repair through a midline approach. Dense adhesions around the previous aortic anastomosis required extensive dissection in preparation for the abdominal aortic reconstruction. The aortic aneurysm sac was closed over a Hemashield graft (Boston Scientic, Natick, MA) after the aortic replacement. At the completion of the operation, there was minimal lymphatic leak in the retroperitoneal area. Two drains were placed in the retroperitoneal space around the aortic aneurysm sac.
0003-4975/$36.00 doi:10.1016/j.athoracsur.2009.12.020

Extracorporeal Peritoneovenous Shunt for the Management of Postaneurysmectomy Chylous Ascites in a Marfan Patient
Gabriele Di Luozzo, MD, Corey Scurlock, MD, Jeffrey I. Mechanick, MD, and Randall B. Griepp, MD
Departments of Cardiothoracic Surgery, Anesthesiology, and Medicine, Mount Sinai School of Medicine, New York, New York

Chylous ascites is an uncommon complication after aortic and other retroperitoneal surgeries. The management
Accepted for publication Dec 2, 2009. Address correspondence to Dr Di Luozzo, Department of Cardiothoracic Surgery, Mount Sinai Medical Center, 1190 Fifth Ave, Box 1029, New York, NY 10029; e-mail: gabriele.diluozzo@mountsinai.org.

2010 by The Society of Thoracic Surgeons Published by Elsevier Inc

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Aortic Valve Bypass for Aortic Stenosis in a Patient With Paroxysmal Nocturnal Hemoglobinuria Mehrdad Ghoreishi, Maria R. Baer, Ranjana Bhargava, Craig E. Stauffer, Bartley P. Griffith and James S. Gammie Ann Thorac Surg 2010;90:279-281 DOI: 10.1016/j.athoracsur.2009.11.047
Updated Information & Services References Subspecialty Collections including high-resolution figures, can be found at: http://ats.ctsnetjournals.org/cgi/content/full/90/1/279 This article cites 6 articles, 3 of which you can access for free at: http://ats.ctsnetjournals.org/cgi/content/full/90/1/279#BIBL This article, along with others on similar topics, appears in the following collection(s): Valve disease http://ats.ctsnetjournals.org/cgi/collection/valve_disease Requests about reproducing this article in parts (figures, tables) or in its entirety should be submitted to: http://www.us.elsevierhealth.com/Licensing/permissions.jsp or email: healthpermissions@elsevier.com. For information about ordering reprints, please email: reprints@elsevier.com

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