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Myocardial infarction in sickle-cell disease
Case Report
Myocardial infarction in sickle-cell disease Jiří Pavlů,* Riaz E Ahmed,* Declan P O’Regan, John Partridge, David C Lefroy, D Mark Layton Lancet 2007; 369: 246 * Lead authors Department of Haematology (J Pavlů MD, D M Layton FRCP), Imaging Sciences Department (R E Ahmed FRCR, D P O’Regan FRCR, J Partridge FRCR), and Department of Cardiology (D C Lefroy FRCP), Imperial College London, Hammersmith Campus, Du Cane Road, London W12 0NN, UK Correspondence to: Dr Jiří Pavlů [email protected]
246
A 50-year-old woman was admitted to our hospital in December, 2005, with a 5 h history of central chest pain of sudden onset. She described dull pain across her chest with radiation to the right arm accompanied by nausea and vomiting. The pain was worse on deep inspiration but not associated with dyspnoea, and it failed to improve after glyceryl trinitrate. She had a history of homozygous sicklecell disease with frequent painful episodes; she managed most of these episodes at home with diclofenac and dihydrocodeine phosphate, although intermittently needed exchange transfusion. The presenting chest pain was different in character from her typical sickle-cell crises. She had no history of coronary heart disease, diabetes, hypertension, or dyslipidaemia, and was a non-smoker. She was apyrexial. Other than mild icterus, physical examination was unremarkable. Electrocardiography showed sinus rhythm with T-wave inversion in V1 to V3, and biphasic T-waves in V4 to V6. A chest radiograph was normal. Arterial blood gas analysis showed PaO2 of 10·6 kPa and SpO2 of 91·1% on room air. Haemoglobin concentration was similar to her steady state at 107 g/L. Serum biochemistry showed increased troponin I at 1·78 μg/L with normal creatinine and electrolytes. Acute coronary syndrome was suspected and enoxaparin sodium, aspirin, and clopidogrel bisulfate were started. Her troponin I peaked at 8·23 μg/L, 12 h after admission, by which time her pain had been superseded by dull pressure over the precordium. Coronary angiography (preceded by automated red-cell exchange to a haemoglobin S level of 19%) showed smooth coronary arteries with no occlusion. No ventilation-perfusion mismatches were shown on lung scintigraphy, and the patient proceeded to a cardiac MRI. An inversion recovery gradient echo sequence acquired 15 min after injection of intravenous gadolinium contrast demonstrated a region of subendocardial hyperenhancement in the lateral wall of the left ventricle consistent with an infarct (figure). Brightblood cine sequences showed that this anomaly was associated with a wall motion abnormality. Her chest pain resolved fully and electrocardiogram became normal within 4 days. When seen for follow-up in August, 2006, she remained asymptomatic. Sickle-cell disease was first described in 1910 by the Chicago physician J B Herrick who contemporaneously postulated that thrombosis in the coronary artery leads to myocardial infarction.1 Such thrombus generally forms where the coronary arteries are narrowed as a result of atherosclerotic plaque. Case reports of myocardial infarction in sickle-cell disease are uncommon, and in most cases coronary angiography showed no significant coronary-artery occlusion.2 An autopsy study demonstrated myocardial infarction in the absence of significant
Figure: Cardiac MRI Inversion recovery gradient echo sequence of the left ventricle in short (left) and long (right) axis shows a hyperenhancing infarct in the lateral wall (arrows).
obstructive or atherosclerotic lesions in 9·7% of patients.3 In contrast to the normal findings in major coronary vessels, the small arteries are narrow in many patients with sickle-cell disease.1 On this background, aggregation of blood cells and their interaction with coagulation factors can cause acute occlusion. Release of inflammatory mediators from leucocytes and platelets induces coronary vasospasm,1 to which scavenging of nitric oxide by haemoglobin liberated through intravascular haemolysis may contribute further. Fat embolism, secondary to bonemarrow infarction, has been implicated in occurrence of myocardial infarction during painful sickle-cell crisis.4 A strength of cardiac MRI is its ability to provide non-invasive myocardial-tissue characterisation at a high spatial resolution. Hyperenhancement of infarcted tissue occurs owing to an increased volume of distribution and delayed wash-out of contrast. Late enhancement can be seen in other causes of cardiomyopathy and in myocarditis, but ischaemic infarction is always characterised by subendocardial enhancement.5 Myocardial infarction should be included in the differential diagnosis of chest pain in sickle-cell disease despite normal coronary angiography. Acknowledgments DPO’R is in receipt of an unrestricted research grant from Schering Healthcare Ltd, which had no involvement in the present study or the paper’s submission for publication. References 1 James TN. Homage to James B Herrick. A contemporary look at myocardial infarction and at sickle cell disease. Circulation 2000; 101: 1874–99. 2 Mansi IA, Rosner F. Myocardial infarction in sickle cell disease. J Natl Med Assoc 2002; 94: 448–52. 3 Martin CR, Johnson CS, Cobb C, Tatter D, Haywood LJ. Myocardial infarction in sickle cell disease. J Natl Med Assoc 1996; 88: 428–32. 4 Dang NC, Johnson C, Eslami-Farsani M, Haywood LJ. Myocardial injury or infarction associated with fat embolism in sickle cell disease: a report of three cases with survival. Am J Hematol 2005; 80: 133–36. 5 Hunold P, Schlosser T, Vogt FM, et al. Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction between infarction scar and non-infarction-related disease. AJR Am J Roentgenol 2005; 184: 1420–26.
www.thelancet.com Vol 369 January 20, 2007
Myocardial infarction in sickle-cell disease Jiří Pavlů,* Riaz E Ahmed,* Declan P O’Regan, John Partridge, David C Lefroy, D Mark Layton Lancet 2007; 369: 246 * Lead authors Department of Haematology (J Pavlů MD, D M Layton FRCP), Imaging Sciences Department (R E Ahmed FRCR, D P O’Regan FRCR, J Partridge FRCR), and Department of Cardiology (D C Lefroy FRCP), Imperial College London, Hammersmith Campus, Du Cane Road, London W12 0NN, UK Correspondence to: Dr Jiří Pavlů [email protected]
246
A 50-year-old woman was admitted to our hospital in December, 2005, with a 5 h history of central chest pain of sudden onset. She described dull pain across her chest with radiation to the right arm accompanied by nausea and vomiting. The pain was worse on deep inspiration but not associated with dyspnoea, and it failed to improve after glyceryl trinitrate. She had a history of homozygous sicklecell disease with frequent painful episodes; she managed most of these episodes at home with diclofenac and dihydrocodeine phosphate, although intermittently needed exchange transfusion. The presenting chest pain was different in character from her typical sickle-cell crises. She had no history of coronary heart disease, diabetes, hypertension, or dyslipidaemia, and was a non-smoker. She was apyrexial. Other than mild icterus, physical examination was unremarkable. Electrocardiography showed sinus rhythm with T-wave inversion in V1 to V3, and biphasic T-waves in V4 to V6. A chest radiograph was normal. Arterial blood gas analysis showed PaO2 of 10·6 kPa and SpO2 of 91·1% on room air. Haemoglobin concentration was similar to her steady state at 107 g/L. Serum biochemistry showed increased troponin I at 1·78 μg/L with normal creatinine and electrolytes. Acute coronary syndrome was suspected and enoxaparin sodium, aspirin, and clopidogrel bisulfate were started. Her troponin I peaked at 8·23 μg/L, 12 h after admission, by which time her pain had been superseded by dull pressure over the precordium. Coronary angiography (preceded by automated red-cell exchange to a haemoglobin S level of 19%) showed smooth coronary arteries with no occlusion. No ventilation-perfusion mismatches were shown on lung scintigraphy, and the patient proceeded to a cardiac MRI. An inversion recovery gradient echo sequence acquired 15 min after injection of intravenous gadolinium contrast demonstrated a region of subendocardial hyperenhancement in the lateral wall of the left ventricle consistent with an infarct (figure). Brightblood cine sequences showed that this anomaly was associated with a wall motion abnormality. Her chest pain resolved fully and electrocardiogram became normal within 4 days. When seen for follow-up in August, 2006, she remained asymptomatic. Sickle-cell disease was first described in 1910 by the Chicago physician J B Herrick who contemporaneously postulated that thrombosis in the coronary artery leads to myocardial infarction.1 Such thrombus generally forms where the coronary arteries are narrowed as a result of atherosclerotic plaque. Case reports of myocardial infarction in sickle-cell disease are uncommon, and in most cases coronary angiography showed no significant coronary-artery occlusion.2 An autopsy study demonstrated myocardial infarction in the absence of significant
Figure: Cardiac MRI Inversion recovery gradient echo sequence of the left ventricle in short (left) and long (right) axis shows a hyperenhancing infarct in the lateral wall (arrows).
obstructive or atherosclerotic lesions in 9·7% of patients.3 In contrast to the normal findings in major coronary vessels, the small arteries are narrow in many patients with sickle-cell disease.1 On this background, aggregation of blood cells and their interaction with coagulation factors can cause acute occlusion. Release of inflammatory mediators from leucocytes and platelets induces coronary vasospasm,1 to which scavenging of nitric oxide by haemoglobin liberated through intravascular haemolysis may contribute further. Fat embolism, secondary to bonemarrow infarction, has been implicated in occurrence of myocardial infarction during painful sickle-cell crisis.4 A strength of cardiac MRI is its ability to provide non-invasive myocardial-tissue characterisation at a high spatial resolution. Hyperenhancement of infarcted tissue occurs owing to an increased volume of distribution and delayed wash-out of contrast. Late enhancement can be seen in other causes of cardiomyopathy and in myocarditis, but ischaemic infarction is always characterised by subendocardial enhancement.5 Myocardial infarction should be included in the differential diagnosis of chest pain in sickle-cell disease despite normal coronary angiography. Acknowledgments DPO’R is in receipt of an unrestricted research grant from Schering Healthcare Ltd, which had no involvement in the present study or the paper’s submission for publication. References 1 James TN. Homage to James B Herrick. A contemporary look at myocardial infarction and at sickle cell disease. Circulation 2000; 101: 1874–99. 2 Mansi IA, Rosner F. Myocardial infarction in sickle cell disease. J Natl Med Assoc 2002; 94: 448–52. 3 Martin CR, Johnson CS, Cobb C, Tatter D, Haywood LJ. Myocardial infarction in sickle cell disease. J Natl Med Assoc 1996; 88: 428–32. 4 Dang NC, Johnson C, Eslami-Farsani M, Haywood LJ. Myocardial injury or infarction associated with fat embolism in sickle cell disease: a report of three cases with survival. Am J Hematol 2005; 80: 133–36. 5 Hunold P, Schlosser T, Vogt FM, et al. Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction between infarction scar and non-infarction-related disease. AJR Am J Roentgenol 2005; 184: 1420–26.
www.thelancet.com Vol 369 January 20, 2007