- Email: [email protected]
Cardiovascular evaluation in Turner Syndrome: The evidence, the reality and the challenges
Letters to the Editor
341
Cardiovascular evaluation in Turner Syndrome: The evidence, the reality and the challenges S.C. Wong a,b,⁎, S. Ehtisham c, M.M.H. Cheung d,e,f, M. Zacharin a,b a
Department of Endocrinology, The Royal Children's Hospital, Melbourne, Australia Centre for Hormone Research, Murdoch Childrens Research Institute, Australia Department of Endocrinology, Royal Manchester Children's Hospital, Melbourne, United Kingdom d Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia e Heart Research Group, Murdoch Childrens Research Institute, Australia f Dept of Paediatrics, University of Melbourne, Australia b c
a r t i c l e
i n f o
Article history: Received 17 January 2014 Accepted 9 March 2014 Available online 15 March 2014 Keywords: Aortic dilatation Bicuspid aortic valve Coarctation of aorta Aortic dissection Magnetic resonance imaging Turner syndrome
Whilst short stature and hypogonadism are common features of Turner syndrome (TS) a spectrum of associated health problems can occur throughout life. Emerging evidence suggests that cardiovascular pathology such as coarctation of aorta (CoA), bicuspid aortic valves (BAV) and aortic dilatation (AoD) significantly increase risks for aortic dissection in TS. Whilst several expert consensuses have recommended cardiovascular monitoring pathways in TS [1], inconsistencies in follow up exist. The 2010 International Consensus for Management of Patients with Thoracic Aorta Disease recommends re-evaluation of the aorta in TS every 5–10 years, even without any risk factors for AoD [1]. It however does not address several issues, including modality of imaging, site of measurements of aorta and possible management strategies. The aim of this letter is to raise awareness to these issues and to highlight the challenges in cardiovascular monitoring in TS. Aortic dissection, a potentially fatal condition, occurs approximately six times more commonly in TS than in healthy females, most frequently in the third decade although dissection can occur in children albeit rarely. Increasing numbers of women with TS are seeking pregnancy with modern reproductive technologies. Risk of aortic dissection is significantly increased during pregnancy, particularly in the third trimester, with mortality from aortic dissection during pregnancy in excess of 100 times population risk. Ongoing cardiovascular monitoring in TS is important since several patients have dissected in the first weeks postpartum [2]. Research in adult women suggests MRI to be superior to echocardiogram especially in the identification of CoA [3]. Echocardiogram may be the imaging of choice in the diagnosis of BAV, although a recent study using MRI in 208 adults with TS was able to demonstrate a spectrum of aortic valve abnormalities including BAV, partially fused aortic valves and unicuspid aortic valves [4]. In TS, aortic dimensions may be best evaluated by MRI since echocardiography may not be adequate for assessment of the entire length of the aorta. As most TS patients are relatively short, aortic dimensions must be adjusted for surface area, this corrected value known is as the aortic size index (ASI) [5]. Several studies have reported a relationship between ASI and acute dissection in TS. Reports of twenty-four patients with TS with acute dissection had an ⁎ Corresponding author at: Centre for Hormone Research, Murdoch Childrens Research Institute, Flemington Road, Parkville, Melbourne, VIC 3052, Australia. Tel.: +61 3 83416493. E-mail address: [email protected] (S.C. Wong).
ASI N 2.5 cm/m2. It is suggested that TS patients with ASI N 2.0 cm/m are at high risk for aortic dissection and those with ASI N 2.5 cm/m2 are at very high risk [2,6]. In TS, aortic dimensions, especially at the aortic sinuses, increase during follow up [7]. A recent prospective study of aortic dimensions using MRI in 102 adults with TS studied over mean of 4.8 years showed that CoA, BAV, age, diastolic blood pressure, body surface area and use of antihypertensives were variables that were significantly related to aortic dimensions [7]. From those data, the authors have been able to generate mathematical models which can be used in clinical practice that allows prediction of aortic dimensions and change in dimensions, taking into account other risk factors. Recent evaluations of clinical practice have, however, revealed inadequate cardiovascular monitoring in TS. A recent study demonstrated that fewer than 40% of women with TS who underwent oocyte donation for pregnancy had any form of cardiovascular screening [8]. Approximately 40% of TS women with a reportedly normal cardiovascular evaluation by a cardiologist not familiar with TS had cardiac abnormalities which preclude pregnancy, when re-evaluated by a cardiologist familiar with TS [9]. In a French cohort of TS patients, only 38.6% had evaluation with echocardiogram and MRI [10]. Careful cardiovascular evaluation, to identify vascular and valvular abnormalities in TS is crucial, at least prior to transition to adult care and preferably earlier, given reported dissection in children, for appropriate management and counselling regarding pregnancy risks. During the past five years, we have streamlined our cardiology referral process, such that adult TS patients are reviewed by a single cardiologist. MRI and echocardiogram are now performed in one centre, compared to four different centres previously. In an audit of 69 adult TS patients, median age 25 years (range 18, 66) managed in our centre by one clinician, 46/69 (66.7%) had echocardiogram and MRI. Two patients underwent CT due to contraindication for MRI. One patient was unable to tolerate MRI. Seven patients (10.1%) had previously known CoA. MRI detected five previously mild undiagnosed CoA (5/46, 10.9%). Measurements of aortic dimensions on MRI in individuals were reported in a median of 4 different anatomic sites (1, 8). Whilst it was possible to calculate the index from the reported dimensions, ASI was not consistently reported in earlier reports. Whilst our audit of TS patients managed by one single clinician revealed a higher percentage of patients who underwent echocardiogram and MRI compared with the French audit, we identified several clinical issues which were hurdles for appropriate cardiovascular follow up in these patients. These include inconsistencies of information provided from imaging (eg number of aortic sites measured, comments on aortic valves), variability in advice about cardiovascular follow up and risk when several cardiologists/clinicians were involved and challenges of engaging in long term follow up of TS patients. It is important to recognise however that even though two recent consensus statements differ in recommendations of cardiovascular risk contraindicating pregnancy in TS [11,12], local consistencies in advice are needed. In conclusion, whilst there is emerging evidence of the role of MRI and echocardiogram for cardiovascular evaluation in TS, a large proportion of TS patients have not had appropriate assessments.
342
Letters to the Editor
Hurdles and challenges to improve care outcome in these patients include engaging TS patients and ensuring ongoing follow-up, clear local clinical pathways and close working relationships between endocrinologists, gynaecologists, cardiologists and general physicians, to ensure provision of consistent advice. New mathematical modelling of aortic dimensions and other risk factors may allow early identification of TS patients with rapid growth of aortic dimension, even though this needs to be validated with clinical outcome [7]. We strongly recommend that care of patients with TS should be provided by clinicians including physicians, gynaecologists, endocrinologists and cardiologists, familiar with aspects of health risks unique to this population group. The Murdoch Childrens Research Institute is supported by the Victorian Government's Operational Infrastructure Support Program. The Heart Research Group is supported by RCH 1000, The Royal Children's Hospital Foundation. References [1] Hiratzka LF, Bakris GL, Beckman JA, et al. ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/ STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. [Erratum appears in Circulation. 2010 Jul 27;122(4):e410]Circulation 2010;121:e266–369.
[2] Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Cardiol 2008;23:519–26. [3] Ostberg JE, Brookes JA, McCarthy C, Halcox J, Conway GS. A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab 2004;89:5966–71. [4] Olivieri LJ, Baba RY, Arai AE, et al. Spectrum of aortic valve abnormalities associated with aortic dilation across Age groups in Turner syndrome. Circ Cardiovasc Imaging 2013;6:1018–23. [5] Cleemann L, Mortensen KH, Holm K, et al. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study. Pediatr Cardiol 2010;31:497–504. [6] Carlson M, Airhart N, Lopez L, Silberbach M. Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. Circulation 2012;126:2220–6. [7] Mortensen KH, Erlandsen M, Andersen NH, Gravholt CH. Prediction of aortic dilation in Turner syndrome — enhancing the use of serial cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2013;15:47. [8] Chevalier N, Letur H, Lelannou D, et al. Materno-fetal cardiovascular complications in Turner syndrome after oocyte donation: insufficient prepregnancy screening and pregnancy follow-up are associated with poor outcome. J Clin Endocrinol Metab 2011;96:E260–7. [9] Chalas Boissonnas C, Davy C, Marszalek A, et al. Cardiovascular findings in women suffering from Turner syndrome requesting oocyte donation. Hum Reprod 2011;26:2754–62. [10] Donadille B, Rousseau A, Zenaty D, et al. Cardiovascular findings and management in Turner syndrome: insights from a French cohort. Eur J Endocrinol 2012;167:517–22. [11] Cabanes L, Chalas C, Christin-Maitre S, et al. Turner syndrome and pregnancy: clinical practice. Recommendations for the management of patients with Turner syndrome before and during pregnancy. Eur J Obstet Gynecol Reprod Biol 2010;152:18–24. [12] Practice Committee of American Society For Reproductive M. Increased maternal cardiovascular mortality associated with pregnancy in women with Turner syndrome. Fertil Steril 2012;97:282–4.
0167–5273/$ – see front matter. Crown Copyright © 2014 Published by Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2014.03.031
Medieval roots of modern knowledge regarding carotid sinus syncope Babak Daneshfard a,b, Behnam Dalfardi b,c,⁎ a b c
Research center for Traditional Medicine and History of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran Research Office for the History of Persian Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
a r t i c l e
i n f o
Article history: Received 2 March 2014 Accepted 9 March 2014 Available online 14 March 2014 Keywords: Islamic Golden Age Medieval history Persia Vasovagal syncope
Carotid sinus syncope (CSS) is a significant condition in the field of cardiovascular medicine. Undeniably, the efforts of post-medieval physicians have played a key role in describing this disorder; nevertheless, such a clinical phenomenon was undoubtedly known and described by medieval Islamic scholars [1]. Previously presented investigations on the early descriptions of CSS enumerated explanations made by such figures as Al-Akhawayni Bukhari (?–983 AD), Haly Abbas (? 930–994 AD), and Avicenna (980– 1037 AD). In this paper, we introduce three other available historic
⁎ Corresponding author at: Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran. Tel.: +98 913 2483359; fax: +98 711 2122970. E-mail address: [email protected] (B. Dalfardi).
examples from the Islamic Golden Age period (9th–12th centuries AD) on this topic [1–3]. Our recent investigations have revealed that, in his book entitled Al-Shukuk al'a Jalinus (Doubts about Galen), Rhazes (865–925 AD) provided what is probably the oldest Muslim scientists' descriptions of loss of consciousness following carotid (sobat in Arabic) artery massage [4]. It is noteworthy that Rhazes was the teacher of the teacher of Al-Akhawayni [5]. Following Rhazes, Abū Man ūr asan ibn Nū al-Qamarī al-Bukhārī (died in about 990 AD), the author of Kitāb al-Tanvīr (The book of Enlightenment), described carotid arteries as follows: “These are two vessels located in the neck which, when compressed, the person will experience sleep” [6]. To the best of these authors' knowledge, alQamarī was one of the teachers of Avicenna, and his aforementioned book is one of the oldest known medical dictionaries. Hakim Esmail Jorjani (1042–1137 AD) also described CSS. In his book entitled Zakhireh-i Kharazmshahi (The Treasure of King Khwarazm), he explained: “When a bathhouse servant compresses the sobat [carotid] vessels, loss of consciousness occurs …, this may be a great hazard …” [7]. Among the aforenamed physicians, Avicenna discussed this condition in more detail [3]. The fact is that the science of cardiology has been dynamically improved over the passage of time, and Muslim scholars are among those who have contributed to this progression [2,8,9]. After the GrecoRoman Era, Islamic world scholars gathered the surviving knowledge of
341
Cardiovascular evaluation in Turner Syndrome: The evidence, the reality and the challenges S.C. Wong a,b,⁎, S. Ehtisham c, M.M.H. Cheung d,e,f, M. Zacharin a,b a
Department of Endocrinology, The Royal Children's Hospital, Melbourne, Australia Centre for Hormone Research, Murdoch Childrens Research Institute, Australia Department of Endocrinology, Royal Manchester Children's Hospital, Melbourne, United Kingdom d Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia e Heart Research Group, Murdoch Childrens Research Institute, Australia f Dept of Paediatrics, University of Melbourne, Australia b c
a r t i c l e
i n f o
Article history: Received 17 January 2014 Accepted 9 March 2014 Available online 15 March 2014 Keywords: Aortic dilatation Bicuspid aortic valve Coarctation of aorta Aortic dissection Magnetic resonance imaging Turner syndrome
Whilst short stature and hypogonadism are common features of Turner syndrome (TS) a spectrum of associated health problems can occur throughout life. Emerging evidence suggests that cardiovascular pathology such as coarctation of aorta (CoA), bicuspid aortic valves (BAV) and aortic dilatation (AoD) significantly increase risks for aortic dissection in TS. Whilst several expert consensuses have recommended cardiovascular monitoring pathways in TS [1], inconsistencies in follow up exist. The 2010 International Consensus for Management of Patients with Thoracic Aorta Disease recommends re-evaluation of the aorta in TS every 5–10 years, even without any risk factors for AoD [1]. It however does not address several issues, including modality of imaging, site of measurements of aorta and possible management strategies. The aim of this letter is to raise awareness to these issues and to highlight the challenges in cardiovascular monitoring in TS. Aortic dissection, a potentially fatal condition, occurs approximately six times more commonly in TS than in healthy females, most frequently in the third decade although dissection can occur in children albeit rarely. Increasing numbers of women with TS are seeking pregnancy with modern reproductive technologies. Risk of aortic dissection is significantly increased during pregnancy, particularly in the third trimester, with mortality from aortic dissection during pregnancy in excess of 100 times population risk. Ongoing cardiovascular monitoring in TS is important since several patients have dissected in the first weeks postpartum [2]. Research in adult women suggests MRI to be superior to echocardiogram especially in the identification of CoA [3]. Echocardiogram may be the imaging of choice in the diagnosis of BAV, although a recent study using MRI in 208 adults with TS was able to demonstrate a spectrum of aortic valve abnormalities including BAV, partially fused aortic valves and unicuspid aortic valves [4]. In TS, aortic dimensions may be best evaluated by MRI since echocardiography may not be adequate for assessment of the entire length of the aorta. As most TS patients are relatively short, aortic dimensions must be adjusted for surface area, this corrected value known is as the aortic size index (ASI) [5]. Several studies have reported a relationship between ASI and acute dissection in TS. Reports of twenty-four patients with TS with acute dissection had an ⁎ Corresponding author at: Centre for Hormone Research, Murdoch Childrens Research Institute, Flemington Road, Parkville, Melbourne, VIC 3052, Australia. Tel.: +61 3 83416493. E-mail address: [email protected] (S.C. Wong).
ASI N 2.5 cm/m2. It is suggested that TS patients with ASI N 2.0 cm/m are at high risk for aortic dissection and those with ASI N 2.5 cm/m2 are at very high risk [2,6]. In TS, aortic dimensions, especially at the aortic sinuses, increase during follow up [7]. A recent prospective study of aortic dimensions using MRI in 102 adults with TS studied over mean of 4.8 years showed that CoA, BAV, age, diastolic blood pressure, body surface area and use of antihypertensives were variables that were significantly related to aortic dimensions [7]. From those data, the authors have been able to generate mathematical models which can be used in clinical practice that allows prediction of aortic dimensions and change in dimensions, taking into account other risk factors. Recent evaluations of clinical practice have, however, revealed inadequate cardiovascular monitoring in TS. A recent study demonstrated that fewer than 40% of women with TS who underwent oocyte donation for pregnancy had any form of cardiovascular screening [8]. Approximately 40% of TS women with a reportedly normal cardiovascular evaluation by a cardiologist not familiar with TS had cardiac abnormalities which preclude pregnancy, when re-evaluated by a cardiologist familiar with TS [9]. In a French cohort of TS patients, only 38.6% had evaluation with echocardiogram and MRI [10]. Careful cardiovascular evaluation, to identify vascular and valvular abnormalities in TS is crucial, at least prior to transition to adult care and preferably earlier, given reported dissection in children, for appropriate management and counselling regarding pregnancy risks. During the past five years, we have streamlined our cardiology referral process, such that adult TS patients are reviewed by a single cardiologist. MRI and echocardiogram are now performed in one centre, compared to four different centres previously. In an audit of 69 adult TS patients, median age 25 years (range 18, 66) managed in our centre by one clinician, 46/69 (66.7%) had echocardiogram and MRI. Two patients underwent CT due to contraindication for MRI. One patient was unable to tolerate MRI. Seven patients (10.1%) had previously known CoA. MRI detected five previously mild undiagnosed CoA (5/46, 10.9%). Measurements of aortic dimensions on MRI in individuals were reported in a median of 4 different anatomic sites (1, 8). Whilst it was possible to calculate the index from the reported dimensions, ASI was not consistently reported in earlier reports. Whilst our audit of TS patients managed by one single clinician revealed a higher percentage of patients who underwent echocardiogram and MRI compared with the French audit, we identified several clinical issues which were hurdles for appropriate cardiovascular follow up in these patients. These include inconsistencies of information provided from imaging (eg number of aortic sites measured, comments on aortic valves), variability in advice about cardiovascular follow up and risk when several cardiologists/clinicians were involved and challenges of engaging in long term follow up of TS patients. It is important to recognise however that even though two recent consensus statements differ in recommendations of cardiovascular risk contraindicating pregnancy in TS [11,12], local consistencies in advice are needed. In conclusion, whilst there is emerging evidence of the role of MRI and echocardiogram for cardiovascular evaluation in TS, a large proportion of TS patients have not had appropriate assessments.
342
Letters to the Editor
Hurdles and challenges to improve care outcome in these patients include engaging TS patients and ensuring ongoing follow-up, clear local clinical pathways and close working relationships between endocrinologists, gynaecologists, cardiologists and general physicians, to ensure provision of consistent advice. New mathematical modelling of aortic dimensions and other risk factors may allow early identification of TS patients with rapid growth of aortic dimension, even though this needs to be validated with clinical outcome [7]. We strongly recommend that care of patients with TS should be provided by clinicians including physicians, gynaecologists, endocrinologists and cardiologists, familiar with aspects of health risks unique to this population group. The Murdoch Childrens Research Institute is supported by the Victorian Government's Operational Infrastructure Support Program. The Heart Research Group is supported by RCH 1000, The Royal Children's Hospital Foundation. References [1] Hiratzka LF, Bakris GL, Beckman JA, et al. ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/ STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. [Erratum appears in Circulation. 2010 Jul 27;122(4):e410]Circulation 2010;121:e266–369.
[2] Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Cardiol 2008;23:519–26. [3] Ostberg JE, Brookes JA, McCarthy C, Halcox J, Conway GS. A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab 2004;89:5966–71. [4] Olivieri LJ, Baba RY, Arai AE, et al. Spectrum of aortic valve abnormalities associated with aortic dilation across Age groups in Turner syndrome. Circ Cardiovasc Imaging 2013;6:1018–23. [5] Cleemann L, Mortensen KH, Holm K, et al. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study. Pediatr Cardiol 2010;31:497–504. [6] Carlson M, Airhart N, Lopez L, Silberbach M. Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. Circulation 2012;126:2220–6. [7] Mortensen KH, Erlandsen M, Andersen NH, Gravholt CH. Prediction of aortic dilation in Turner syndrome — enhancing the use of serial cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2013;15:47. [8] Chevalier N, Letur H, Lelannou D, et al. Materno-fetal cardiovascular complications in Turner syndrome after oocyte donation: insufficient prepregnancy screening and pregnancy follow-up are associated with poor outcome. J Clin Endocrinol Metab 2011;96:E260–7. [9] Chalas Boissonnas C, Davy C, Marszalek A, et al. Cardiovascular findings in women suffering from Turner syndrome requesting oocyte donation. Hum Reprod 2011;26:2754–62. [10] Donadille B, Rousseau A, Zenaty D, et al. Cardiovascular findings and management in Turner syndrome: insights from a French cohort. Eur J Endocrinol 2012;167:517–22. [11] Cabanes L, Chalas C, Christin-Maitre S, et al. Turner syndrome and pregnancy: clinical practice. Recommendations for the management of patients with Turner syndrome before and during pregnancy. Eur J Obstet Gynecol Reprod Biol 2010;152:18–24. [12] Practice Committee of American Society For Reproductive M. Increased maternal cardiovascular mortality associated with pregnancy in women with Turner syndrome. Fertil Steril 2012;97:282–4.
0167–5273/$ – see front matter. Crown Copyright © 2014 Published by Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2014.03.031
Medieval roots of modern knowledge regarding carotid sinus syncope Babak Daneshfard a,b, Behnam Dalfardi b,c,⁎ a b c
Research center for Traditional Medicine and History of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran Research Office for the History of Persian Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
a r t i c l e
i n f o
Article history: Received 2 March 2014 Accepted 9 March 2014 Available online 14 March 2014 Keywords: Islamic Golden Age Medieval history Persia Vasovagal syncope
Carotid sinus syncope (CSS) is a significant condition in the field of cardiovascular medicine. Undeniably, the efforts of post-medieval physicians have played a key role in describing this disorder; nevertheless, such a clinical phenomenon was undoubtedly known and described by medieval Islamic scholars [1]. Previously presented investigations on the early descriptions of CSS enumerated explanations made by such figures as Al-Akhawayni Bukhari (?–983 AD), Haly Abbas (? 930–994 AD), and Avicenna (980– 1037 AD). In this paper, we introduce three other available historic
⁎ Corresponding author at: Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran. Tel.: +98 913 2483359; fax: +98 711 2122970. E-mail address: [email protected] (B. Dalfardi).
examples from the Islamic Golden Age period (9th–12th centuries AD) on this topic [1–3]. Our recent investigations have revealed that, in his book entitled Al-Shukuk al'a Jalinus (Doubts about Galen), Rhazes (865–925 AD) provided what is probably the oldest Muslim scientists' descriptions of loss of consciousness following carotid (sobat in Arabic) artery massage [4]. It is noteworthy that Rhazes was the teacher of the teacher of Al-Akhawayni [5]. Following Rhazes, Abū Man ūr asan ibn Nū al-Qamarī al-Bukhārī (died in about 990 AD), the author of Kitāb al-Tanvīr (The book of Enlightenment), described carotid arteries as follows: “These are two vessels located in the neck which, when compressed, the person will experience sleep” [6]. To the best of these authors' knowledge, alQamarī was one of the teachers of Avicenna, and his aforementioned book is one of the oldest known medical dictionaries. Hakim Esmail Jorjani (1042–1137 AD) also described CSS. In his book entitled Zakhireh-i Kharazmshahi (The Treasure of King Khwarazm), he explained: “When a bathhouse servant compresses the sobat [carotid] vessels, loss of consciousness occurs …, this may be a great hazard …” [7]. Among the aforenamed physicians, Avicenna discussed this condition in more detail [3]. The fact is that the science of cardiology has been dynamically improved over the passage of time, and Muslim scholars are among those who have contributed to this progression [2,8,9]. After the GrecoRoman Era, Islamic world scholars gathered the surviving knowledge of