- Email: [email protected]
Successful Mitral Valve Replacement in a Patient with a Severe Form of β-Thalassaemia
Case Reports
haemostasis.2 Moreover CPB has been shown to cause immunosuppression, which may in turn may lead to enhancement of metastases.1 Finally, the tumours which require the involvement of CPB to facilitate their removal tend to have invaded major vascular structures and are often therefore, by definition, highly advanced. Despite this there are a few studies that have investigated the role of CPB in aiding surgical excision of tumours. Perrot et al.3 reported a series of seven patients who underwent lung resection on CPB for locally advanced bronchogenic carcinoma. Similarly Byrne et al.1 reported a series of fourteen patients who underwent resection of pulmonary and mediastinal tumours under CPB. However, in these series the most common indication for CPB was invasion of a major vascular structure. What distinguishes this case is the fact that CPB was used to facilitate oxygenation as opposed to maintaining circulatory integrity. Although there have been isolated reports of CPB being used to facilitate oxygenation in cases requiring tracheal or bronchial resection1,4 in this case the tumour had no intrinsic airway involvement and CPB was only required to prevent the sequelae of mechanical obstruction of the airway during mobilisation. Solitary fibrous tumours of the pleura are rare, representing only 5% of all pleural tumours.5 Although considered histologically benign, approximately 10% of solitary fibrous tumours show malignant transformation.6 In a recent large-scale study, Cardillo et al.6 recommended complete surgical excision of all solitary fibrous pleural tumours with preservation of the tumour capsule in order to prevent the possibility of recurrence. Given the large size of some of these tumours and the fact that their vascular supply may arise from the lung parenchyma, achieving
77
complete excision may require concomitant resection of underlying lung or chest wall.6 In this case although we did not anticipate the need for CPB pre-operatively, mobilisation of the tumour compromised both the ventilation and venous return of the patient necessitating its use. Although CPB is itself not without its risks, the fact that the total bypass time was limited to 18 min would have minimised the deleterious effects of CPB previously described. As such, we advocate the use of CPB as a tool to aid in the mobilisation and removal of otherwise technically inoperable pleural tumours.
References 1. Byrne JG, Leacche M, Agnihotri AK, Paul S, Bueno R, Mathisen DJ, Sugarbaker DJ. The use of cardiopulmonary bypass during resection of locally advanced thoracic malignancies: a 10-year two-center experience. Chest 2004;125(4):1581–6. 2. Ulicny Jr KS, Schmelzer V, Flege Jr JB, Todd JC, Mitts DL, Melvin DB, Wright CB. Concomitant cardiac and pulmonary operation: the role of cardiopulmonary bypass. Ann Thorac Surg 1992;54(2):289–95. 3. de Perrot M, Fadel E, Mussot S, de Palma A, Chapelier A, Dartevelle P. Resection of locally advanced (T4) non-small cell lung cancer with cardiopulmonary bypass. Ann Thorac Surg 2005;79(5):1691–6. 4. Chen HH, Pan W, An XX. Resection of tracheal tumour under cardiopulmonary bypass: a case report. Chin Med J (Engl) 2005;118(12):1047–9. 5. Desser TS, Stark P. Pictorial essay: solitary fibrous tumour of the pleura. J Thorac Imaging 1998;13(1):27–35. 6. Cardillo G, Facciolo F, Cavazzana AO, Capece G, Gasparri R, Martelli M. Localized (solitary) fibrous tumours of the pleura: an analysis of 55 patients. Ann Thorac Surg 2000;70(6):1808–12.
Successful Mitral Valve Replacement in a Patient with a Severe Form of -Thalassaemia Luca Botta, MD ∗ , Carlo Savini, MD, Sofia Martin-Suarez, MD, Andrea Dell’ Amore, MD, Nicola Camurri, MD, Giorgio Arpesella, MD and Roberto Di Bartolomeo, MD Department of Cardiac Surgery, University of Bologna, Bologna, Italy
-Thalassaemia is an inherited haemoglobin (Hb) disorder resulting in chronic haemolytic anaemia. The most anaemic patients require regular red blood cell (RBC) transfusions for survival but iron accumulation leads to multisystem dysfunction. Heart complications represent the leading cause of mortality in -thalassaemia patients. In this case report we present a successful mitral valve replacement (MVR) in a patient with a severe form of -thalassaemia. (Heart, Lung and Circulation 2008;17:62–79) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. -Thalassaemia; Heart complications; Mitral valve replacement Received 9 May 2006; received in revised form 15 September 2006; accepted 21 September 2006; available online 22 February 2007 ∗ Corresponding author at: Department of Cardiac Surgery, University of Bologna, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy. Tel.: +39 051 6363361; fax: +39 051 345990. E-mail address: [email protected] (L. Botta).
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.09.010
CASE REPORTS
Heart, Lung and Circulation 2008;17:62–79
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Case Reports
CASE REPORTS
Introduction

-Thalassaemia is an inherited haemoglobin (Hb) disorder caused by impaired synthesis of the beta globin chain. The excess unmatched alpha chains accumulate in the growing erythroid precursors and in adult red blood cells causing ineffective erythropoiesis and haemolysis.1 Depending on clinical severity, two forms are distinguished: thalassaemia major (TM) and thalassaemia intermedia (TI).2 The most anaemic patients require regular red blood cell (RBC) transfusions to avoid death from cardiac failure. Heart complications always represented and still remain the leading cause of death in -thalassaemia.3–5 Open heart surgery is unusual in patients with thalassaemia: few cases have been described in patients with -thalassaemia trait but no one, to the best of our knowledge, has been reported in more severe forms.6 In this case report, we present a successful mitral valve replacement (MVR) in a patient with -thalassaemia major requiring regular RBC transfusions once a week in the last five years.
Case Report A 50-year-old white male patient with a severe form of -thalassaemia was referred to our institute for a severe mitral-valve steno-insufficiency. He received regular blood transfusions every two weeks from the age of seven years till the age of 12 years when he underwent splenectomy. In the following 33 years, he refused transfusions surviving with levels of haemoglobin between 6 and 8 g/dl. In 2001, regular RBC transfusions restarted (once every 7–10 days) and iron chelation therapy with desferrioxamine (2500 mg/die for six days/week) begun. Mean ferritin level for the last four years was 2015.5 ng/ml, while mean pretransfusional haemoglobin level was 8.2 g/dl in the last year. The latest cardiac follow up examination revealed dyspnea for mild efforts, while echocardiography showed a severe mitral steno-insufficiency (MVA = 1.2 cm2 , peak/mean gradient = 17/9 mmHg). The ejection fraction (EF) was 57% and left ventricle end diastolic diameter/end systolic diameter (LV EDD/ESD) = 4, 5/3 cm; considerable pulmonary hypertension (systolic tricuspid gradient >35 mmHg and right ventricle pressure (RVP) >40 mmHg) was also present. Two months later, the patient underwent MVR with an On-X (MCRI, LLC, Austin, Texas) bileaflet mechanical valve (25/33 mm). Cardiac arrest was induced with a single dose of cold crystalloid cardioplegia solution (CUSTODIOL, HTK, F.K. Chemie, Alsbach-Hahnlein, Germany). The cardiopulmonary by-pass time and aortic cross-clamp time were 83 and 63 min, respectively. Preoperative, intraoperative (mean) and postoperative (mean) values of haemoglobin and haematocrit (Ht) were 9.2, 8.1, 9.4 gr/dl and 28.3, 24, 27.8%, respectively. In the operative room, the patient was transfused with fresh frozen plasma (FFP), RBC and platelets. The patient was estubated six hours later and transferred to the ward after an overall ICU stay of 16 hours. He enjoyed a smooth and progressive recovery not
Heart, Lung and Circulation 2008;17:62–79
requiring other transfusions and was discharged on the seventh postoperative day with values of Hb, Ht and ferritin of 9.1 g/dl, 27.2% and 2971 ng/ml, respectively. At sixmonth follow-up, a satisfactory clinical status and a good surgical result (echocardiographic control) were observed.
Discussion Normal adult haemoglobin (Hb A) is a tetramer of two alpha and two beta globin chains. In -thalassaemia, the defect in normal beta globin synthesis leads to the accumulation and deposition of the excess unmatched alpha chains which in turn cause haemolysis and ineffective erythropoiesis. The consequences of the resulting chronic anaemia and tissue hypoxia include bone marrow expansion, extramedullary haemopoiesis, spleen and liver enlargement, growth retardation, increased iron absorption, susceptibility to infections and hypercoagulability.1–3 TM and TI differentiates from one another for the severity of the clinical phenotype. TM is rapidly fatal unless adequate transfusions are started early enough. In contrast, TI is generally characterised by a mild clinical picture, better prognosis and better chance for survival. Appropriate transfusion support allows normal growth and development and prevents congestive heart failure, severe bony deformities and endocrinopathies even if iron overload is inevitable. Iron primarily accumulates in the liver, heart and endocrine organs finally leading to multisystem dysfunction. Iron chelation therapy result compulsory.1,2 Heart disease is the leading cause of mortality and one of the main causes of morbidity in -thalassaemia patients. Myocardial iron overload resulting from multiple transfusions and the high output state caused by chronic anaemia are the two main pathogenetic factors involved. Cardiac involvement consists primarily of ventricular dysfunction and pulmonary hypertension both resulting in subsequent congestive heart failure and cardiogenic death.1–5 Pulmonary hypertension is now recognised as a major issue, particularly in splenectomised patients. The underlying cause is increased pulmonary vascular resistance which has a multifactorial origin: mechanical damage of pulmonary vascular endothelium caused by the high CO, lung injury caused by respiratory tract infections, chest wall deformities, extramedullary intrapulmonary haemopoietic masses and age related diffuse elastic tissue disorders. Moreover, an important role can be played by an hypercoagulable state whose causes are thought to be thrombocytosis, defects of erythrocyte and platelet membrane phospholipids, increased blood levels of activation peptides (prothrombin fragment1.2) and decreased levels of anti thrombotic proteins.1–3 Acute pericarditis and abnormalities of cardiac valves including left-sided valve regurgitation, rupture of chordae tendinae, tricuspid regurgitation, endocardial calcification and thickening of leaflets have also been reported in these patients.3–5 Open heart surgery is unusual in patients with -thalassaemia: few cases have been described in patients with -thalassaemia trait but no one, to the best of our knowledge, has been reported in more severe forms except for some cases of cardiac transplantation in TM
patients affected by iron overload end-stage cardiomyopathy. The erythrocytes of thalassaemic patients are more susceptible to haemolysis than are those of normal individuals; a greater degree of haemolysis is much more evident after open heart operations because of the use of the heart–lung machine for extracorporeal circulation.6,8 In order to reduce this problem, packed RBC were used in priming solution for cardiopulmonary bypass and a non pulsatile flow was administered using a centrifugal pump in our patient. Moreover, cardioplegic solution was completely recovered from the right atrium and two aspirators were used as selective pulmonary veins vents. Since life perspective of these patients is still unclear as the bioprosthetic valves’ durability, we have chosen a mechanical prosthetic heart valve according to the patient’s age. According to De Chiara,7 right ventricular dysfunction and abnormal heart rate variability may represent the early features of cardiac disease in thalassaemic patients with no evidence of ventricular dysfunction at routine evaluation. This could allow to identify patients who will probably develop significant heart disease earlier, trying to avoid, as far as possible, the serious complications. This experience shows that open heart surgery is feasible and safe even in patients with severe forms of -thalassaemia if a shrewd, specific perioperative and intraoperative management is performed.
Case Reports
79
References 1. Schrier SL, Angelucci E. New strategies in the treatment of the thalassaemias. Annu Rev Med 2005;56:157–71. 2. Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, et al. Thalassaemia heart disease: a comparative evaluation of thalassaemia major and thalassaemia intermedia. Chest 2005;127:1523–30. 3. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, et al. Cardiac involvement in thalassaemia intermedia: a multicenter study. Blood 2001;97(11): 3411–6. 4. Farmakis D, Deftereos S, Giakoumis A, Polymeropoulos E, Aessopos A. Rupture of chordae tendineae in patients with b-thalassaemia. Eur J Haematol 2004;72:296–8. 5. Wu KH, Chang JS, Su BH, Peng CT. Tricuspid regurgitation in patients with beta-thalassaemia major. Ann Hematol 2004;83(12):779–83. 6. Cokkinou V, Katsiyanni A, Orkopoulou M, Michalis A, Tolis G, Cokkinos DV. Evidence of increased haemolysis after open heart surgery in patients heterozygous for beta-thalassaemia. Tex Heart Inst J 1988;15:35–8. 7. De Chiara B, Crivellaro W, Sara R, Ruffinini L, Parolini M, Fesslova` V, et al. Early detection of cardiac dysfunction in thalassemic patients by radionuclide angiography and heart rate variability analysis. Eur J Haematol 2005;74:517–22. 8. Caines AE, Kpodonu J, Massad MG, Chaer R, Evans A, Lee JC, et al. Cardiac transplantation in patients with iron overload cardiomyopathy. J Heart Lung Transplant 2005;24(4):486–8.
CASE REPORTS
Heart, Lung and Circulation 2008;17:62–79
haemostasis.2 Moreover CPB has been shown to cause immunosuppression, which may in turn may lead to enhancement of metastases.1 Finally, the tumours which require the involvement of CPB to facilitate their removal tend to have invaded major vascular structures and are often therefore, by definition, highly advanced. Despite this there are a few studies that have investigated the role of CPB in aiding surgical excision of tumours. Perrot et al.3 reported a series of seven patients who underwent lung resection on CPB for locally advanced bronchogenic carcinoma. Similarly Byrne et al.1 reported a series of fourteen patients who underwent resection of pulmonary and mediastinal tumours under CPB. However, in these series the most common indication for CPB was invasion of a major vascular structure. What distinguishes this case is the fact that CPB was used to facilitate oxygenation as opposed to maintaining circulatory integrity. Although there have been isolated reports of CPB being used to facilitate oxygenation in cases requiring tracheal or bronchial resection1,4 in this case the tumour had no intrinsic airway involvement and CPB was only required to prevent the sequelae of mechanical obstruction of the airway during mobilisation. Solitary fibrous tumours of the pleura are rare, representing only 5% of all pleural tumours.5 Although considered histologically benign, approximately 10% of solitary fibrous tumours show malignant transformation.6 In a recent large-scale study, Cardillo et al.6 recommended complete surgical excision of all solitary fibrous pleural tumours with preservation of the tumour capsule in order to prevent the possibility of recurrence. Given the large size of some of these tumours and the fact that their vascular supply may arise from the lung parenchyma, achieving
77
complete excision may require concomitant resection of underlying lung or chest wall.6 In this case although we did not anticipate the need for CPB pre-operatively, mobilisation of the tumour compromised both the ventilation and venous return of the patient necessitating its use. Although CPB is itself not without its risks, the fact that the total bypass time was limited to 18 min would have minimised the deleterious effects of CPB previously described. As such, we advocate the use of CPB as a tool to aid in the mobilisation and removal of otherwise technically inoperable pleural tumours.
References 1. Byrne JG, Leacche M, Agnihotri AK, Paul S, Bueno R, Mathisen DJ, Sugarbaker DJ. The use of cardiopulmonary bypass during resection of locally advanced thoracic malignancies: a 10-year two-center experience. Chest 2004;125(4):1581–6. 2. Ulicny Jr KS, Schmelzer V, Flege Jr JB, Todd JC, Mitts DL, Melvin DB, Wright CB. Concomitant cardiac and pulmonary operation: the role of cardiopulmonary bypass. Ann Thorac Surg 1992;54(2):289–95. 3. de Perrot M, Fadel E, Mussot S, de Palma A, Chapelier A, Dartevelle P. Resection of locally advanced (T4) non-small cell lung cancer with cardiopulmonary bypass. Ann Thorac Surg 2005;79(5):1691–6. 4. Chen HH, Pan W, An XX. Resection of tracheal tumour under cardiopulmonary bypass: a case report. Chin Med J (Engl) 2005;118(12):1047–9. 5. Desser TS, Stark P. Pictorial essay: solitary fibrous tumour of the pleura. J Thorac Imaging 1998;13(1):27–35. 6. Cardillo G, Facciolo F, Cavazzana AO, Capece G, Gasparri R, Martelli M. Localized (solitary) fibrous tumours of the pleura: an analysis of 55 patients. Ann Thorac Surg 2000;70(6):1808–12.
Successful Mitral Valve Replacement in a Patient with a Severe Form of -Thalassaemia Luca Botta, MD ∗ , Carlo Savini, MD, Sofia Martin-Suarez, MD, Andrea Dell’ Amore, MD, Nicola Camurri, MD, Giorgio Arpesella, MD and Roberto Di Bartolomeo, MD Department of Cardiac Surgery, University of Bologna, Bologna, Italy
-Thalassaemia is an inherited haemoglobin (Hb) disorder resulting in chronic haemolytic anaemia. The most anaemic patients require regular red blood cell (RBC) transfusions for survival but iron accumulation leads to multisystem dysfunction. Heart complications represent the leading cause of mortality in -thalassaemia patients. In this case report we present a successful mitral valve replacement (MVR) in a patient with a severe form of -thalassaemia. (Heart, Lung and Circulation 2008;17:62–79) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. -Thalassaemia; Heart complications; Mitral valve replacement Received 9 May 2006; received in revised form 15 September 2006; accepted 21 September 2006; available online 22 February 2007 ∗ Corresponding author at: Department of Cardiac Surgery, University of Bologna, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy. Tel.: +39 051 6363361; fax: +39 051 345990. E-mail address: [email protected] (L. Botta).
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.09.010
CASE REPORTS
Heart, Lung and Circulation 2008;17:62–79
78
Case Reports
CASE REPORTS
Introduction

-Thalassaemia is an inherited haemoglobin (Hb) disorder caused by impaired synthesis of the beta globin chain. The excess unmatched alpha chains accumulate in the growing erythroid precursors and in adult red blood cells causing ineffective erythropoiesis and haemolysis.1 Depending on clinical severity, two forms are distinguished: thalassaemia major (TM) and thalassaemia intermedia (TI).2 The most anaemic patients require regular red blood cell (RBC) transfusions to avoid death from cardiac failure. Heart complications always represented and still remain the leading cause of death in -thalassaemia.3–5 Open heart surgery is unusual in patients with thalassaemia: few cases have been described in patients with -thalassaemia trait but no one, to the best of our knowledge, has been reported in more severe forms.6 In this case report, we present a successful mitral valve replacement (MVR) in a patient with -thalassaemia major requiring regular RBC transfusions once a week in the last five years.
Case Report A 50-year-old white male patient with a severe form of -thalassaemia was referred to our institute for a severe mitral-valve steno-insufficiency. He received regular blood transfusions every two weeks from the age of seven years till the age of 12 years when he underwent splenectomy. In the following 33 years, he refused transfusions surviving with levels of haemoglobin between 6 and 8 g/dl. In 2001, regular RBC transfusions restarted (once every 7–10 days) and iron chelation therapy with desferrioxamine (2500 mg/die for six days/week) begun. Mean ferritin level for the last four years was 2015.5 ng/ml, while mean pretransfusional haemoglobin level was 8.2 g/dl in the last year. The latest cardiac follow up examination revealed dyspnea for mild efforts, while echocardiography showed a severe mitral steno-insufficiency (MVA = 1.2 cm2 , peak/mean gradient = 17/9 mmHg). The ejection fraction (EF) was 57% and left ventricle end diastolic diameter/end systolic diameter (LV EDD/ESD) = 4, 5/3 cm; considerable pulmonary hypertension (systolic tricuspid gradient >35 mmHg and right ventricle pressure (RVP) >40 mmHg) was also present. Two months later, the patient underwent MVR with an On-X (MCRI, LLC, Austin, Texas) bileaflet mechanical valve (25/33 mm). Cardiac arrest was induced with a single dose of cold crystalloid cardioplegia solution (CUSTODIOL, HTK, F.K. Chemie, Alsbach-Hahnlein, Germany). The cardiopulmonary by-pass time and aortic cross-clamp time were 83 and 63 min, respectively. Preoperative, intraoperative (mean) and postoperative (mean) values of haemoglobin and haematocrit (Ht) were 9.2, 8.1, 9.4 gr/dl and 28.3, 24, 27.8%, respectively. In the operative room, the patient was transfused with fresh frozen plasma (FFP), RBC and platelets. The patient was estubated six hours later and transferred to the ward after an overall ICU stay of 16 hours. He enjoyed a smooth and progressive recovery not
Heart, Lung and Circulation 2008;17:62–79
requiring other transfusions and was discharged on the seventh postoperative day with values of Hb, Ht and ferritin of 9.1 g/dl, 27.2% and 2971 ng/ml, respectively. At sixmonth follow-up, a satisfactory clinical status and a good surgical result (echocardiographic control) were observed.
Discussion Normal adult haemoglobin (Hb A) is a tetramer of two alpha and two beta globin chains. In -thalassaemia, the defect in normal beta globin synthesis leads to the accumulation and deposition of the excess unmatched alpha chains which in turn cause haemolysis and ineffective erythropoiesis. The consequences of the resulting chronic anaemia and tissue hypoxia include bone marrow expansion, extramedullary haemopoiesis, spleen and liver enlargement, growth retardation, increased iron absorption, susceptibility to infections and hypercoagulability.1–3 TM and TI differentiates from one another for the severity of the clinical phenotype. TM is rapidly fatal unless adequate transfusions are started early enough. In contrast, TI is generally characterised by a mild clinical picture, better prognosis and better chance for survival. Appropriate transfusion support allows normal growth and development and prevents congestive heart failure, severe bony deformities and endocrinopathies even if iron overload is inevitable. Iron primarily accumulates in the liver, heart and endocrine organs finally leading to multisystem dysfunction. Iron chelation therapy result compulsory.1,2 Heart disease is the leading cause of mortality and one of the main causes of morbidity in -thalassaemia patients. Myocardial iron overload resulting from multiple transfusions and the high output state caused by chronic anaemia are the two main pathogenetic factors involved. Cardiac involvement consists primarily of ventricular dysfunction and pulmonary hypertension both resulting in subsequent congestive heart failure and cardiogenic death.1–5 Pulmonary hypertension is now recognised as a major issue, particularly in splenectomised patients. The underlying cause is increased pulmonary vascular resistance which has a multifactorial origin: mechanical damage of pulmonary vascular endothelium caused by the high CO, lung injury caused by respiratory tract infections, chest wall deformities, extramedullary intrapulmonary haemopoietic masses and age related diffuse elastic tissue disorders. Moreover, an important role can be played by an hypercoagulable state whose causes are thought to be thrombocytosis, defects of erythrocyte and platelet membrane phospholipids, increased blood levels of activation peptides (prothrombin fragment1.2) and decreased levels of anti thrombotic proteins.1–3 Acute pericarditis and abnormalities of cardiac valves including left-sided valve regurgitation, rupture of chordae tendinae, tricuspid regurgitation, endocardial calcification and thickening of leaflets have also been reported in these patients.3–5 Open heart surgery is unusual in patients with -thalassaemia: few cases have been described in patients with -thalassaemia trait but no one, to the best of our knowledge, has been reported in more severe forms except for some cases of cardiac transplantation in TM
patients affected by iron overload end-stage cardiomyopathy. The erythrocytes of thalassaemic patients are more susceptible to haemolysis than are those of normal individuals; a greater degree of haemolysis is much more evident after open heart operations because of the use of the heart–lung machine for extracorporeal circulation.6,8 In order to reduce this problem, packed RBC were used in priming solution for cardiopulmonary bypass and a non pulsatile flow was administered using a centrifugal pump in our patient. Moreover, cardioplegic solution was completely recovered from the right atrium and two aspirators were used as selective pulmonary veins vents. Since life perspective of these patients is still unclear as the bioprosthetic valves’ durability, we have chosen a mechanical prosthetic heart valve according to the patient’s age. According to De Chiara,7 right ventricular dysfunction and abnormal heart rate variability may represent the early features of cardiac disease in thalassaemic patients with no evidence of ventricular dysfunction at routine evaluation. This could allow to identify patients who will probably develop significant heart disease earlier, trying to avoid, as far as possible, the serious complications. This experience shows that open heart surgery is feasible and safe even in patients with severe forms of -thalassaemia if a shrewd, specific perioperative and intraoperative management is performed.
Case Reports
79
References 1. Schrier SL, Angelucci E. New strategies in the treatment of the thalassaemias. Annu Rev Med 2005;56:157–71. 2. Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, et al. Thalassaemia heart disease: a comparative evaluation of thalassaemia major and thalassaemia intermedia. Chest 2005;127:1523–30. 3. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, et al. Cardiac involvement in thalassaemia intermedia: a multicenter study. Blood 2001;97(11): 3411–6. 4. Farmakis D, Deftereos S, Giakoumis A, Polymeropoulos E, Aessopos A. Rupture of chordae tendineae in patients with b-thalassaemia. Eur J Haematol 2004;72:296–8. 5. Wu KH, Chang JS, Su BH, Peng CT. Tricuspid regurgitation in patients with beta-thalassaemia major. Ann Hematol 2004;83(12):779–83. 6. Cokkinou V, Katsiyanni A, Orkopoulou M, Michalis A, Tolis G, Cokkinos DV. Evidence of increased haemolysis after open heart surgery in patients heterozygous for beta-thalassaemia. Tex Heart Inst J 1988;15:35–8. 7. De Chiara B, Crivellaro W, Sara R, Ruffinini L, Parolini M, Fesslova` V, et al. Early detection of cardiac dysfunction in thalassemic patients by radionuclide angiography and heart rate variability analysis. Eur J Haematol 2005;74:517–22. 8. Caines AE, Kpodonu J, Massad MG, Chaer R, Evans A, Lee JC, et al. Cardiac transplantation in patients with iron overload cardiomyopathy. J Heart Lung Transplant 2005;24(4):486–8.
CASE REPORTS
Heart, Lung and Circulation 2008;17:62–79