J Forensic Sci, 2015 doi: 10.1111/1556-4029.12722 Available online at: onlinelibrary.wiley.com
CASE REPORT PATHOLOGY/BIOLOGY
Cristian D’Ovidio,1 M.D., Ph.D.; Sara Sablone,1 M.D.; and Aldo Carnevale,1 M.D., Ph.D.
Spontaneous Coronary Artery Dissection: Case Report and Literature Review
ABSTRACT: Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction and sudden cardiac death. It
occurs most commonly in otherwise healthy women during pregnancy or the postpartum period. The true incidence of SCAD is underestimated, as most cases are diagnosed at autopsy. The pathophysiology of SCAD is still not fully understood, and its management can be challenging. This report describes a 35-year-old pregnant female who presented with an acute antero-lateral ST elevation secondary to spontaneous dissection of the left anterior descending artery and the circumflex artery. The diagnosis was established by coronary artery angiography. However, the patient died following cardiac tamponade. The examination of this case represented a starting point for the reviewing of the diagnosis, clinical course, and management of SCAD, and for the placing of this in context with the existing literature. This study highlights the importance of prompt diagnosis and subsequent lifesaving treatment.
KEYWORDS: forensic science, cardiology, pregnancy, maternal death, coronary artery dissection, autopsy Cardiovascular diseases arise during 0.2% to 4% of all pregnancies in the industrialized world (1). One particular form of cardiovascular disease is spontaneous coronary artery dissection (SCAD), which is a highly unusual cause of acute coronary syndrome and sudden cardiac death that is seen especially in women in pregnancy or in the early postpartum period. The first autopsy report involved a 42-year-old woman with sudden cardiac death in 1931 (2). Subsequently, fewer than 400 cases have been reported in the literature. However, the true number of cases is no doubt much higher: Indeed, sudden cardiac death is often the first manifestation, and the majority of cases are diagnosed at autopsy, because the mortality is high, at 70% (3–5). The exact etiology and pathogenesis of pregnancy-related SCAD are uncertain, and its management can be challenging because of the lack of guidelines in terms of optimal treatment. Nevertheless, as acute myocardial infarction is a common medical emergency and a life-threatening condition, the occurrence of SCAD should be suspected as early as possible in all pregnant or peripartum women with acute coronary syndrome. Case Report The present case is based on the medical history of a previously healthy pregnant woman who experienced an episode of acute coronary syndrome and died after Cesarean delivery. Her death was considered as unusual and led to a forensic autopsy and pathological analysis.
1 Section of Legal Medicine, Department of Medicine and Aging Sciences, ‘G. d’Annunzio’ University of Chieti–Pescara, Via dei Vestini, 31, 66100 Chieti, Italy. Received 4 Nov. 2013; and in revised form 26 Feb. 2014; accepted 22 May 2014.
© 2015 American Academy of Forensic Sciences
Past Medical History The patient was aged 35 years. She had no underlying cardiovascular risk factors. The patient medical history included a prior miscarriage in the 4th month of pregnancy; moreover, arterial hypertension and hyperthyroidism had occurred during a previous pregnancy, which was her first full-term pregnancy. She reported nothing relevant in her family, except for a history of arterial hypertension. Case The woman was in the 35th week of her third pregnancy. She presented at the emergency unit of the nearest hospital with a 1h history of sudden and severe retrosternal chest pain that woke her from her sleep. On physical examination, the patient was hemodynamically stable, with a heart rate of 82 beats per min and a blood pressure of 125/70 mmHg. Her respiratory rate and rhythm were normal. There was no jugular venous distension or cervical bruits. Her abdomen was soft, nontender, and nondistended, with no organomegaly, and her bowel sounds were normal. Hepatojugular reflux was absent. Lower extremity pulses were normal, and there was no lower extremity edema. The patient had no clinical signs of Marfan syndrome or heart failure. The initial electrocardiogram demonstrated evidence of acute antero-lateral ST elevation myocardial infarction. Transthoracic echocardiography revealed cardiac anteroseptoapical akinesia, with overall good left ventricle systolic function and no pericardial effusion or valvular abnormalities. The aortic root and arch appeared intact. Fetal monitoring was normal. The patient was treated medically with nitrates and heparin, while a coronary angiography was not performed immediately because the hospital was not equipped with a catheterization laboratory. 1
2
JOURNAL OF FORENSIC SCIENCES
Following urgent multidisciplinary discussions among the patient, her family and the treating obstetrics and cardiology units, the patient was transferred to a nearby hospital that was better equipped, and on presentation there she had a blood pressure of 80/40 mmHg, while pulmonary auscultation revealed bilateral crepitations. Beta-adrenergic blockers (atenolol) and analgesics (morphine) were immediately administered. The patient was rapidly referred for coronary angiography to determine whether revascularization was necessary. This investigation showed a normal left main stem flow, but highlighted a radiolucent flap consistent with left anterior descending (LAD) artery and circumflex artery dissection (see Fig. 1). The right coronary artery appeared normal. Percutaneous transluminal coronary angioplasty with stent placement was performed and normal flow was restored to the circumflex artery. Before revascularization in the field of the LAD artery was performed, intra-aortic balloon pump assistance was provided by the left femoral approach, as the patient was in cardiogenic shock. Trans-esophageal echocardiography confirmed the anteroseptoapical akinesia and revealed left ventricular systolic dysfunction, with depressed left ventricular ejection fraction, at 35%. It also showed mild aortic regurgitation, caused by a slight dilatation of the left ventricle, and mild pericardial effusion. However, the LAD dissection was not amenable to reperfusion by percutaneous intervention. Therefore, the patient was urgently transferred to an operating theater for Cesarean section delivery of a baby girl with cardiac arrest, which necessitated cardiopulmonary resuscitation and hospitalization in a pediatric intensive care unit. The initial evolution was favorable: postoperative trans-esophageal echocardiography revealed a moderate improvement of the left ventricular ejection fraction, at 40% to 45%. On this basis, no ventricular assistance device was applied, such as extracorporeal membrane oxygenation, and the patient was hemodynamically stable when transferred to the cardiac intensive care unit. Nevertheless, cardiac arrest occurred half an hour later. Transthoracic echocardiography revealed a hemopericardium with cardiac tamponade. Despite prolonged advanced life support and surgical opening of the pericardium to evacuate the accumulated blood, the patient died.
FIG 1––Coronary angiography showing spontaneous dissection of the LAD artery and the circumflex artery (arrows).
Further Medico-Legal Investigations Autopsy Findings—An autopsy was requested by the investigating agency, which submitted the case to the Institute of Legal Medicine in Chieti (Italy). On external examination, lesions were noted due to the surgical management: There was a recent suture line of the lower abdomen, from the Cesarean section; an additional recent suture line was noted at the subxiphoid area of the thorax, as a result of the access that was created to evacuate the hemopericardium. Upon entering the thorax, the previously dissected pericardial sac contained partially clotted residual blood. After isolating the heart, the findings at coronary angiography were confirmed: There was a SCAD that involved the circumflex artery and the LAD artery. The circumflex artery included a stent extending the length of the dissection. The LAD artery had an intramural hematoma that obstructed the lumen at a distance of about 1 cm from its origin (see Fig. 2 and 3). Furthermore, the heart showed a transmural perforation on the anterolateral wall of the left ventricle, near the apex (see Fig. 4). The perforation had a transverse diameter of 0.2 cm, with a circular shape and a surrounding eccentric area of hemorrhagic infiltration. Following a complete autopsy, no abnormalities were noted in the other organs. Pathology Analysis—Several heart areas were examined. The histological specimens were stained with hematoxylin–eosin. Microscopically, the LAD artery transverse section showed a dissecting hematoma that had pushed the separated intima-media layer toward the true lumen of the vessel, which was affected by an eccentric stenosis (see Fig. 5). The dissected layer showed a thickened area of subendothelial connective tissue, which appeared to be affected by hyperelastosis, according to Weigert staining. The histology slides of the longitudinal and oblique LAD artery sections from the distal course of the vessel showed a circumferential extension of dissecting clefts. Myocardium surrounding the ventricle perforation was affected by a transmural infarct which was
CASE REPORT PATHOLOGY/BIOLOGY
Cristian D’Ovidio,1 M.D., Ph.D.; Sara Sablone,1 M.D.; and Aldo Carnevale,1 M.D., Ph.D.
Spontaneous Coronary Artery Dissection: Case Report and Literature Review
ABSTRACT: Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction and sudden cardiac death. It
occurs most commonly in otherwise healthy women during pregnancy or the postpartum period. The true incidence of SCAD is underestimated, as most cases are diagnosed at autopsy. The pathophysiology of SCAD is still not fully understood, and its management can be challenging. This report describes a 35-year-old pregnant female who presented with an acute antero-lateral ST elevation secondary to spontaneous dissection of the left anterior descending artery and the circumflex artery. The diagnosis was established by coronary artery angiography. However, the patient died following cardiac tamponade. The examination of this case represented a starting point for the reviewing of the diagnosis, clinical course, and management of SCAD, and for the placing of this in context with the existing literature. This study highlights the importance of prompt diagnosis and subsequent lifesaving treatment.
KEYWORDS: forensic science, cardiology, pregnancy, maternal death, coronary artery dissection, autopsy Cardiovascular diseases arise during 0.2% to 4% of all pregnancies in the industrialized world (1). One particular form of cardiovascular disease is spontaneous coronary artery dissection (SCAD), which is a highly unusual cause of acute coronary syndrome and sudden cardiac death that is seen especially in women in pregnancy or in the early postpartum period. The first autopsy report involved a 42-year-old woman with sudden cardiac death in 1931 (2). Subsequently, fewer than 400 cases have been reported in the literature. However, the true number of cases is no doubt much higher: Indeed, sudden cardiac death is often the first manifestation, and the majority of cases are diagnosed at autopsy, because the mortality is high, at 70% (3–5). The exact etiology and pathogenesis of pregnancy-related SCAD are uncertain, and its management can be challenging because of the lack of guidelines in terms of optimal treatment. Nevertheless, as acute myocardial infarction is a common medical emergency and a life-threatening condition, the occurrence of SCAD should be suspected as early as possible in all pregnant or peripartum women with acute coronary syndrome. Case Report The present case is based on the medical history of a previously healthy pregnant woman who experienced an episode of acute coronary syndrome and died after Cesarean delivery. Her death was considered as unusual and led to a forensic autopsy and pathological analysis.
1 Section of Legal Medicine, Department of Medicine and Aging Sciences, ‘G. d’Annunzio’ University of Chieti–Pescara, Via dei Vestini, 31, 66100 Chieti, Italy. Received 4 Nov. 2013; and in revised form 26 Feb. 2014; accepted 22 May 2014.
© 2015 American Academy of Forensic Sciences
Past Medical History The patient was aged 35 years. She had no underlying cardiovascular risk factors. The patient medical history included a prior miscarriage in the 4th month of pregnancy; moreover, arterial hypertension and hyperthyroidism had occurred during a previous pregnancy, which was her first full-term pregnancy. She reported nothing relevant in her family, except for a history of arterial hypertension. Case The woman was in the 35th week of her third pregnancy. She presented at the emergency unit of the nearest hospital with a 1h history of sudden and severe retrosternal chest pain that woke her from her sleep. On physical examination, the patient was hemodynamically stable, with a heart rate of 82 beats per min and a blood pressure of 125/70 mmHg. Her respiratory rate and rhythm were normal. There was no jugular venous distension or cervical bruits. Her abdomen was soft, nontender, and nondistended, with no organomegaly, and her bowel sounds were normal. Hepatojugular reflux was absent. Lower extremity pulses were normal, and there was no lower extremity edema. The patient had no clinical signs of Marfan syndrome or heart failure. The initial electrocardiogram demonstrated evidence of acute antero-lateral ST elevation myocardial infarction. Transthoracic echocardiography revealed cardiac anteroseptoapical akinesia, with overall good left ventricle systolic function and no pericardial effusion or valvular abnormalities. The aortic root and arch appeared intact. Fetal monitoring was normal. The patient was treated medically with nitrates and heparin, while a coronary angiography was not performed immediately because the hospital was not equipped with a catheterization laboratory. 1
2
JOURNAL OF FORENSIC SCIENCES
Following urgent multidisciplinary discussions among the patient, her family and the treating obstetrics and cardiology units, the patient was transferred to a nearby hospital that was better equipped, and on presentation there she had a blood pressure of 80/40 mmHg, while pulmonary auscultation revealed bilateral crepitations. Beta-adrenergic blockers (atenolol) and analgesics (morphine) were immediately administered. The patient was rapidly referred for coronary angiography to determine whether revascularization was necessary. This investigation showed a normal left main stem flow, but highlighted a radiolucent flap consistent with left anterior descending (LAD) artery and circumflex artery dissection (see Fig. 1). The right coronary artery appeared normal. Percutaneous transluminal coronary angioplasty with stent placement was performed and normal flow was restored to the circumflex artery. Before revascularization in the field of the LAD artery was performed, intra-aortic balloon pump assistance was provided by the left femoral approach, as the patient was in cardiogenic shock. Trans-esophageal echocardiography confirmed the anteroseptoapical akinesia and revealed left ventricular systolic dysfunction, with depressed left ventricular ejection fraction, at 35%. It also showed mild aortic regurgitation, caused by a slight dilatation of the left ventricle, and mild pericardial effusion. However, the LAD dissection was not amenable to reperfusion by percutaneous intervention. Therefore, the patient was urgently transferred to an operating theater for Cesarean section delivery of a baby girl with cardiac arrest, which necessitated cardiopulmonary resuscitation and hospitalization in a pediatric intensive care unit. The initial evolution was favorable: postoperative trans-esophageal echocardiography revealed a moderate improvement of the left ventricular ejection fraction, at 40% to 45%. On this basis, no ventricular assistance device was applied, such as extracorporeal membrane oxygenation, and the patient was hemodynamically stable when transferred to the cardiac intensive care unit. Nevertheless, cardiac arrest occurred half an hour later. Transthoracic echocardiography revealed a hemopericardium with cardiac tamponade. Despite prolonged advanced life support and surgical opening of the pericardium to evacuate the accumulated blood, the patient died.
FIG 1––Coronary angiography showing spontaneous dissection of the LAD artery and the circumflex artery (arrows).
Further Medico-Legal Investigations Autopsy Findings—An autopsy was requested by the investigating agency, which submitted the case to the Institute of Legal Medicine in Chieti (Italy). On external examination, lesions were noted due to the surgical management: There was a recent suture line of the lower abdomen, from the Cesarean section; an additional recent suture line was noted at the subxiphoid area of the thorax, as a result of the access that was created to evacuate the hemopericardium. Upon entering the thorax, the previously dissected pericardial sac contained partially clotted residual blood. After isolating the heart, the findings at coronary angiography were confirmed: There was a SCAD that involved the circumflex artery and the LAD artery. The circumflex artery included a stent extending the length of the dissection. The LAD artery had an intramural hematoma that obstructed the lumen at a distance of about 1 cm from its origin (see Fig. 2 and 3). Furthermore, the heart showed a transmural perforation on the anterolateral wall of the left ventricle, near the apex (see Fig. 4). The perforation had a transverse diameter of 0.2 cm, with a circular shape and a surrounding eccentric area of hemorrhagic infiltration. Following a complete autopsy, no abnormalities were noted in the other organs. Pathology Analysis—Several heart areas were examined. The histological specimens were stained with hematoxylin–eosin. Microscopically, the LAD artery transverse section showed a dissecting hematoma that had pushed the separated intima-media layer toward the true lumen of the vessel, which was affected by an eccentric stenosis (see Fig. 5). The dissected layer showed a thickened area of subendothelial connective tissue, which appeared to be affected by hyperelastosis, according to Weigert staining. The histology slides of the longitudinal and oblique LAD artery sections from the distal course of the vessel showed a circumferential extension of dissecting clefts. Myocardium surrounding the ventricle perforation was affected by a transmural infarct which was
