Case Study
Percutaneous coronary intervention in a case of afibrinogenemia
Asian Cardiovascular & Thoracic Annals 21(3) 358–359 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312455180 aan.sagepub.com
Homa Falsoleiman, Mehdi Hasanzadeh Daloee, Mashalla Dehghani, Atoosheh Rohani and Baktash Bayani
Abstract Congenital afibrinogenemia is a rare autosomic recessive blood disorder. A 30-year-old lady, known to have congenital afibrinogenemia, presented with acute anterior myocardial infarction. We managed her with dual antiplatelet therapy and atorvastatin, but her chest pain did not subside and she was transferred to the catheterization laboratory. A proximal left anterior descending artery occlusion was crossed with a floppy wire. Angioplasty was performed successfully with a bare metal stent, and her symptoms resolved completely.
Keywords Afibrinogenemia, angioplasty, blood coagulation disorders, inherited, myocardial infarction, stents
Introduction Congenital afibrinogenemia is a rare autosomic recessive blood disorder that can manifest with excessive bleeding from birth; however, thromboembolic complications may also occur. Management of these patients is very problematic. Anticoagulant drugs are contraindicated so the patient loses the chance of therapeutic options such as thrombolytic therapy and percutaneous coronary interventions (PCI), and coronary artery bypass grafting is impossible.
Case report A 30-year-old lady who was known to have afibrinogenemia since birth, presented with acute anterior myocardial infarction. She had chest pain for 10 h before admission. Her family history was significant because one of her brothers died in childhood because of excessive bleeding after circumcision, but she did not have any risk factors for coronary artery disease. Physical examination revealed no fever, pulse rate 100 beatsmin 1, and blood pressure 110/70 mmHg. Cardiovascular examination was normal, but her electrocardiogram showed anterior ST-elevation myocardial infarction (MI). Her troponin I was elevated to 20 ngL 1. Liver function tests and lipid profile (high density cholesterol: 41 mgdL 1; triglycerides: 1 80 mgdL ; low density cholesterol: 138 mgdL 1)
were normal, but the fibrinogen level was 50 s, (control: 13 s), activated partial thromboplastin time >240 s, (control: 32 s), and international normalized ratio >7. Transthoracic echocardiography estimated her left ventricular ejection fraction as 52% with hypokinesia of the anterolateral wall. It has been recommended that antiplatelets can be used safely in these patients; therefore, we managed her with dual antiplatelet therapy (aspirin plus clopidogrel) and atorvastatin, but her chest pain did not subside and we transferred her to catheterization laboratory. After replacement therapy with cryoprecipitate and normalization of her international normalized ratio, urgent coronary angiography revealed a significant proximal left anterior descending thrombotic lesion up to 90%; the other coronaries were normal. Due to anterior MI and severe chest pain, we decided to perform angioplasty on the left anterior descending artery, and the patient was given eptifibatide (two 180-mgkg 1 bolus doses 10 min
Mashhad Cardiac Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Corresponding author: Atoosheh Rohani, Mashhad Cardiac Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Email: [email protected]
Downloaded from aan.sagepub.com at University of Birmingham on June 2, 2015
Falsoleiman et al.
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apart combined with a continuous infusion of 2.0 mgkg 1min 1) and heparin in the catheterization laboratory. The proximal left anterior descending artery occlusion was crossed with a floppy wire. Angioplasty was performed with a bare metal stent (Medtronic) at 16 atm and yielded a satisfactory result and TIMI3 flow. The patient’s symptoms resolved completely. We used a bare metal stent because of the shorter duration of antiplatelet therapy and faster endothelialization, we also used eptifibatide for management of this patient because we could not give her appropriate amounts of heparin to reduce the risk of acute cardiac ischemic events. We discharged her in a stable condition after 5 days on aspirin 80 mg and clopidogrel 75 mg for one month. After one year, she was admitted again with unstable angina and chest discomfort, and we performed another angiography; 50% in-stent restenosis was seen, and we continued medical treatment with dual antiplatelet therapy (aspirin plus clopidogrel), atorvastatin, and nitrates, and her chest pain subsided. Two years after the primary event, she was alive, on aspirin and metolazone, and in a stable clinical condition.
Discussion Due to consanguineous marriage in our country, the prevalence of afibrinogenemia is 10-times more frequent than in western countries.1 We record all cases in the Iranian National Registry of Inherited Bleeding Disorders.2 There are a few case reports of afibrinogenemia with myocardial infarction.3–5 All of the patients were treated with medical therapy, but the optimal strategy is not clear. The mechanism of thrombosis is also not understood and unrelated to replacement therapy. To the best of our knowledge, this is the first report of stent deployment and use of eptifibatide in an
afibrinogenemia patient. Fortunately our patient is still alive and free of symptoms. However, angioplasty is not recommended as a routine therapeutic option in these cases, but when medical treatment has failed, and if the expected benefit of coronary angioplasty outweighs the risk of bleeding, it is the last life-saving method that can be used in this setting. We report this case to share our experience and to elicit discussion on the therapeutic option in these gravely ill patients. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Lak M, Sharifian RA, Karimi K, Mansouritorghabeh H. Prevalence and management of inherited bleeding disorders in Iran. J Thromb Haemost 2009;7(Suppl 2) Abstract PP-MO-552 Available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2009.03473_2.x/pdf. Accessed June 27, 2012. 2. Mannucci PM, Duga S and Peyvandi F. Recessively inherited coagulation disorders. Blood 2004; 104: 1243–1252. 3. Kumar N, Padma Kumar R, Ramesh B and Garg N. Afibrinogenaemia: a rare cause of young myocardial infarct. Singapore Med J 2008; 49: e104–e106. 4. Mghaieth F, Mizouni H, Mbarki S, Ayari J, Trabelsi R, Moussa NB, et al. Acute myocardial infarction in a patient with hypofibrinogenemia: a case report. J Med Case Rep 2011; 5: 582. 5. Rohani A, Akbari V, Moradian K and Malekzade J. Combining white blood cell count and thrombosis for predicting in-hospital outcomes after acute myocardial infraction. J Emerg Trauma Shock 2011; 4: 351–354.
Downloaded from aan.sagepub.com at University of Birmingham on June 2, 2015
Percutaneous coronary intervention in a case of afibrinogenemia
Asian Cardiovascular & Thoracic Annals 21(3) 358–359 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312455180 aan.sagepub.com
Homa Falsoleiman, Mehdi Hasanzadeh Daloee, Mashalla Dehghani, Atoosheh Rohani and Baktash Bayani
Abstract Congenital afibrinogenemia is a rare autosomic recessive blood disorder. A 30-year-old lady, known to have congenital afibrinogenemia, presented with acute anterior myocardial infarction. We managed her with dual antiplatelet therapy and atorvastatin, but her chest pain did not subside and she was transferred to the catheterization laboratory. A proximal left anterior descending artery occlusion was crossed with a floppy wire. Angioplasty was performed successfully with a bare metal stent, and her symptoms resolved completely.
Keywords Afibrinogenemia, angioplasty, blood coagulation disorders, inherited, myocardial infarction, stents
Introduction Congenital afibrinogenemia is a rare autosomic recessive blood disorder that can manifest with excessive bleeding from birth; however, thromboembolic complications may also occur. Management of these patients is very problematic. Anticoagulant drugs are contraindicated so the patient loses the chance of therapeutic options such as thrombolytic therapy and percutaneous coronary interventions (PCI), and coronary artery bypass grafting is impossible.
Case report A 30-year-old lady who was known to have afibrinogenemia since birth, presented with acute anterior myocardial infarction. She had chest pain for 10 h before admission. Her family history was significant because one of her brothers died in childhood because of excessive bleeding after circumcision, but she did not have any risk factors for coronary artery disease. Physical examination revealed no fever, pulse rate 100 beatsmin 1, and blood pressure 110/70 mmHg. Cardiovascular examination was normal, but her electrocardiogram showed anterior ST-elevation myocardial infarction (MI). Her troponin I was elevated to 20 ngL 1. Liver function tests and lipid profile (high density cholesterol: 41 mgdL 1; triglycerides: 1 80 mgdL ; low density cholesterol: 138 mgdL 1)
were normal, but the fibrinogen level was 50 s, (control: 13 s), activated partial thromboplastin time >240 s, (control: 32 s), and international normalized ratio >7. Transthoracic echocardiography estimated her left ventricular ejection fraction as 52% with hypokinesia of the anterolateral wall. It has been recommended that antiplatelets can be used safely in these patients; therefore, we managed her with dual antiplatelet therapy (aspirin plus clopidogrel) and atorvastatin, but her chest pain did not subside and we transferred her to catheterization laboratory. After replacement therapy with cryoprecipitate and normalization of her international normalized ratio, urgent coronary angiography revealed a significant proximal left anterior descending thrombotic lesion up to 90%; the other coronaries were normal. Due to anterior MI and severe chest pain, we decided to perform angioplasty on the left anterior descending artery, and the patient was given eptifibatide (two 180-mgkg 1 bolus doses 10 min
Mashhad Cardiac Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Corresponding author: Atoosheh Rohani, Mashhad Cardiac Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Email: [email protected]
Downloaded from aan.sagepub.com at University of Birmingham on June 2, 2015
Falsoleiman et al.
359
apart combined with a continuous infusion of 2.0 mgkg 1min 1) and heparin in the catheterization laboratory. The proximal left anterior descending artery occlusion was crossed with a floppy wire. Angioplasty was performed with a bare metal stent (Medtronic) at 16 atm and yielded a satisfactory result and TIMI3 flow. The patient’s symptoms resolved completely. We used a bare metal stent because of the shorter duration of antiplatelet therapy and faster endothelialization, we also used eptifibatide for management of this patient because we could not give her appropriate amounts of heparin to reduce the risk of acute cardiac ischemic events. We discharged her in a stable condition after 5 days on aspirin 80 mg and clopidogrel 75 mg for one month. After one year, she was admitted again with unstable angina and chest discomfort, and we performed another angiography; 50% in-stent restenosis was seen, and we continued medical treatment with dual antiplatelet therapy (aspirin plus clopidogrel), atorvastatin, and nitrates, and her chest pain subsided. Two years after the primary event, she was alive, on aspirin and metolazone, and in a stable clinical condition.
Discussion Due to consanguineous marriage in our country, the prevalence of afibrinogenemia is 10-times more frequent than in western countries.1 We record all cases in the Iranian National Registry of Inherited Bleeding Disorders.2 There are a few case reports of afibrinogenemia with myocardial infarction.3–5 All of the patients were treated with medical therapy, but the optimal strategy is not clear. The mechanism of thrombosis is also not understood and unrelated to replacement therapy. To the best of our knowledge, this is the first report of stent deployment and use of eptifibatide in an
afibrinogenemia patient. Fortunately our patient is still alive and free of symptoms. However, angioplasty is not recommended as a routine therapeutic option in these cases, but when medical treatment has failed, and if the expected benefit of coronary angioplasty outweighs the risk of bleeding, it is the last life-saving method that can be used in this setting. We report this case to share our experience and to elicit discussion on the therapeutic option in these gravely ill patients. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Lak M, Sharifian RA, Karimi K, Mansouritorghabeh H. Prevalence and management of inherited bleeding disorders in Iran. J Thromb Haemost 2009;7(Suppl 2) Abstract PP-MO-552 Available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2009.03473_2.x/pdf. Accessed June 27, 2012. 2. Mannucci PM, Duga S and Peyvandi F. Recessively inherited coagulation disorders. Blood 2004; 104: 1243–1252. 3. Kumar N, Padma Kumar R, Ramesh B and Garg N. Afibrinogenaemia: a rare cause of young myocardial infarct. Singapore Med J 2008; 49: e104–e106. 4. Mghaieth F, Mizouni H, Mbarki S, Ayari J, Trabelsi R, Moussa NB, et al. Acute myocardial infarction in a patient with hypofibrinogenemia: a case report. J Med Case Rep 2011; 5: 582. 5. Rohani A, Akbari V, Moradian K and Malekzade J. Combining white blood cell count and thrombosis for predicting in-hospital outcomes after acute myocardial infraction. J Emerg Trauma Shock 2011; 4: 351–354.
Downloaded from aan.sagepub.com at University of Birmingham on June 2, 2015
