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• W2220104088 abstract "PresentationAn 82-year-old woman presented to the emergency department after being found unresponsive by bystanders. While driving she felt a band-like chest pressure, epigastric pain, and general malaise, causing her to pull over. She was under emotional duress after visiting her son in the hospital; he was being treated for an acute aortic dissection. By the time she arrived at the emergency department, her chest pressure had resolved. Other comorbidities include hypothyroidism and atrial fibrillation, for which she was anticoagulated with rivaroxiban.AssessmentOn arrival at the emergency department, blood pressure was 60/37 mm Hg, heart rate 80 beats per minute, respiratory rate of 12 breaths per minute, and afebrile. She was alert and oriented and only complaining of generalized weakness. Heart sounds were distant but normal rate, and results from lung examination were unremarkable; jugular venous pressure was elevated >20 cm water, though she had received 6 L of intravenous physiologic saline by the time of our initial evaluation. Pulsus paradoxus was 7 mm Hg. The electrocardiogram showed sinus rhythm at 87 beats per minute and left axis deviation without acute ST changes.Initial blood pressure was fluid responsive and, after 4 L of fluid, she was stable to undergo a computed tomography (CT) scan to assess for aortic dissection or pulmonary embolism. Pertinent laboratory data included cardiac troponin T <0.01 ng/mL, normal chemistry panel, liver and thyroid functions, and complete blood count. Lactate was 3.6 mM. The chest CT scan revealed a non-aneurysmal aorta (Figure 1A), a moderate to large pericardial effusion, and significant left ventricular (LV) thickening with small LV cavity size, suggesting hypertrophic obstructive cardiomyopathy (Figure 1B). The radiologist recommended cardiology evaluation on the basis of initial imaging data.A surface echocardiogram showed a small, under-filled, thickened and hyperdynamic LV with a moderate- to large-sized circumferential pericardial effusion (Figure 2) with increased echogenicity in the pericardial effusion, suggesting blood or exudate. It showed borderline impaired diastolic filling (right ventricular diastolic collapse as well as enlarged inferior vena cava of 3.2 cm) but without right atrial collapse or augmentation of trans-mitral or trans-tricuspid blood flow by continuous wave Doppler imaging throughout the respiratory cycle. There was a 3.8-cm enlarged ascending mid-tubular aorta (Figure 3).Figure 2Subcostal echocardiographic view of the heart. The pericardial space shows an echo-lucent and echo-dense signal concerning for fresh and coagulated blood. LV = left ventricle; RV = right ventricle.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Parasternal long axis view of the heart. A large collection of pericardial fluid is noted, with marked compression of the right ventricle (RV). The aortic root is markedly dilated. LV = left ventricle.View Large Image Figure ViewerDownload Hi-res image Download (PPT)DiagnosisThe patient continued to have persistent hypotension after 8 L of physiologic saline, with systolic blood pressures in the range of 60-70s mm Hg. A few findings were suggestive of pericardial tamponade, including persistent shock and borderline impaired diastolic filling. However, findings against tamponade included a normal pulsus paradoxus, without right atrial or right ventricular diastolic collapse, or respiratory variation in trans-mitral and trans-tricuspid blood flow.The other concern was pericardial effusion etiology. There was a clear distinction within the pericardial fluid of an echo-bright substance adjacent to the pericardium and an echo-dark fluid that looked more like typical pericardial fluid. A major concern was impending hemodynamic collapse if the pericardial material was coagulated blood, because percutaneous pericardiocentesis may dislodge a stable thrombus, and so a surgical approach would be a safer option.ManagementThe patient remained in shock during a time of night when ancillary surgical services were not immediately available and so, contrary to what guidelines suggest, a decision was made to perform an urgent ultrasound-guided pericardiocentesis from an apical approach. Forty milliliters of serous fluid were removed, after which no more fluid could be removed. Serous rather than sanguinous fluid initially was reassuring for the absence of tamponade due to acute coronary or vascular pathology. However, the patient remained unstable, so a subxyphoid approach was performed with needle placement in the pericardial space confirmed by agitated saline injection. A total of 150 mL of bloody fluid was removed, and a pericardial drain was inserted. It was thought that the initial serous fluid was from the pleural cavity. She had immediate blood pressure improvement after pericardiocentesis. A transesophageal echocardiogram (TEE) was then performed and revealed a dilated ascending aorta with sub-intimal hematoma. Reviewing her previous studies, a TEE conducted 6 months before assess for intracardiac thrombus revealed a small ulcerated plaque in the aortic arch that extended proximally to the aortic valve.The patient tolerated TEE well and became hypertensive in the coronary care unit, which was controlled using oral labetalol, 300 mg three times daily. A few hours later the cardiothoracic surgery team evaluated the patient and determined she had a contained aortic rupture. A repeat chest CT study 36 hours after presentation showed an interval increase in ascending aortic aneurysm intramural hematoma, though she remained clinically stable. After holding her rivaroxiban for 48 hours, she was taken urgently to the operating room to surgically manage her expanding intramural hematoma. She was found to have a Stanford type A dissection from the aortic root extending to her right innominate artery and underwent replacement of the ascending aorta with Dacron graft. She recovered rapidly and was discharged to a skilled nursing facility. Interestingly, 24 hours before presentation, her son had presented with an acute Stanford type A aortic dissection that required emergent surgery by the same cardiothoracic surgeon. The family later revealed that the patient's sister died of an aortic dissection a few years prior, raising the possibility of genetic predisposition to aortopathies.DiscussionAcute aortic syndromes present as three clinical scenarios: aortic dissection, penetrating atherosclerotic ulcer (PAU), and intramural hematoma (IMH). Aortic dissections are classified as either ascending (Stanford type A) or descending (Stanford type B) dissections, with type A dissections necessitating urgent surgical repair. The incidence of thoracic aortic aneurysm rupture is 3 per 100,000 person years.1Clouse W.D. Hallett Jr., J.W. Schaff H.V. et al.Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture.Mayo Clin Proc. 2004; 79: 176-180Abstract Full Text Full Text PDF PubMed Scopus (420) Google ScholarThe etiology can be either heritable or sporadic. Because of her strong family history of aortopathy, it is thought that there could be a genetic component. There is an increasingly recognized familial syndrome in up to 20% of patients with aortic dissections, called familial thoracic aortic aneurysm and dissection, that is associated with α-actin smooth muscle mutations (ACTA2 gene). They display an autosomal dominant mode of inheritance, and hence all first-degree relatives should be screened. Our patient's physical appearance was not consistent with the phenotypic manifestation of Marfans, Turners, Ehler-Danlos, or Loeys-Dietz syndrome. She also did not have a bicuspid aortic valve.In our patient, it is thought that her known PAU at the aortic arch caused an entry tear leading to an ascending dissection, pericardial tamponade, and subsequently a contained rupture. That would explain her bloody layering pericardial effusion causing tamponade. It is interesting that neither the CT scan nor TEE showed an aortic dissection, but both showed a thickened enlarged aorta, suggestive of dissection. This is an unusual presentation because PAU is typically present in the mid or distal descending thoracic aorta with penetration of the internal elastic lamina, leading to hematoma and associated with extensive atherosclerosis. Intramural hematomas are not associated with intimal tears, and it is unclear how they arise, but they may be associated with cystic medial necrosis. They are thought to be precursors to, rather than the result of, a dissection. Both PAU and IMH were associated with worse 1-year prognosis than type A or type B aortic dissections,2Coady M.A. Ikonomidis J.S. Cheung A.T. et al.Surgical management of descending thoracic aortic disease: open and endovascular approaches: a scientific statement from the American Heart Association.Circulation. 2010; 121: 2780-2804Crossref PubMed Scopus (134) Google Scholar possibly because PAU and IMH are diseases of the elderly.Current recommendations argue against placement of a pericardial drain in a patient with concomitant Stanford type A dissection and pericardial tamponade because that can lead to exsanguination into the pericardial space. Surgery is the treatment of choice. A case review showed that pericardiocenteses in patients presenting with hemopericardium from aortic dissection lead to death in 3 of 4 patients treated with successful pericardiocentesis.3Isselbacher E.M. Cigarroa J.E. Eagle K.A. Cardiac tamponade complicating proximal aortic dissection. Is pericardiocentesis harmful?.Circulation. 1994; 90: 2375-2378Crossref PubMed Scopus (108) Google Scholar Another small study showed that patients with type A aortic dissection and signs of preoperative cardiac tamponade without palpable pulses had a 16-fold increased risk of poor outcome and especially death as compared with patients with type A dissection and impending cardiac tamponade with palpable pulses.4Bayegan K. Domanovits H. Schillinger M. et al.Acute type A aortic dissection: the prognostic impact of preoperative cardiac tamponade.Eur J Cardiothorac Surg. 2001; 20: 1194-1198Crossref PubMed Scopus (41) Google Scholar However, we hypothesized that she was clinically unstable from her tamponade and that bleeding was self-contained, arguing for the need for emergent pericardiocentesis.In summary, this is an unusual presentation of an acute aortic syndrome with complicated diagnosis and management. The exact etiology of the patient's IMH, tamponade, and eventual dissection is unclear, though we suspect both PAU and familial aortopathy are responsible. No one diagnostic test led to a definitive diagnosis, requiring us to utilize multiple modalities quickly in this unstable patient. After synthesis of the information, an informed decision that differed from the guidelines produced a successful outcome to stabilize the patient and allow for a positive surgical outcome. PresentationAn 82-year-old woman presented to the emergency department after being found unresponsive by bystanders. While driving she felt a band-like chest pressure, epigastric pain, and general malaise, causing her to pull over. She was under emotional duress after visiting her son in the hospital; he was being treated for an acute aortic dissection. By the time she arrived at the emergency department, her chest pressure had resolved. Other comorbidities include hypothyroidism and atrial fibrillation, for which she was anticoagulated with rivaroxiban. An 82-year-old woman presented to the emergency department after being found unresponsive by bystanders. While driving she felt a band-like chest pressure, epigastric pain, and general malaise, causing her to pull over. She was under emotional duress after visiting her son in the hospital; he was being treated for an acute aortic dissection. By the time she arrived at the emergency department, her chest pressure had resolved. Other comorbidities include hypothyroidism and atrial fibrillation, for which she was anticoagulated with rivaroxiban. AssessmentOn arrival at the emergency department, blood pressure was 60/37 mm Hg, heart rate 80 beats per minute, respiratory rate of 12 breaths per minute, and afebrile. She was alert and oriented and only complaining of generalized weakness. Heart sounds were distant but normal rate, and results from lung examination were unremarkable; jugular venous pressure was elevated >20 cm water, though she had received 6 L of intravenous physiologic saline by the time of our initial evaluation. Pulsus paradoxus was 7 mm Hg. The electrocardiogram showed sinus rhythm at 87 beats per minute and left axis deviation without acute ST changes.Initial blood pressure was fluid responsive and, after 4 L of fluid, she was stable to undergo a computed tomography (CT) scan to assess for aortic dissection or pulmonary embolism. Pertinent laboratory data included cardiac troponin T <0.01 ng/mL, normal chemistry panel, liver and thyroid functions, and complete blood count. Lactate was 3.6 mM. The chest CT scan revealed a non-aneurysmal aorta (Figure 1A), a moderate to large pericardial effusion, and significant left ventricular (LV) thickening with small LV cavity size, suggesting hypertrophic obstructive cardiomyopathy (Figure 1B). The radiologist recommended cardiology evaluation on the basis of initial imaging data.A surface echocardiogram showed a small, under-filled, thickened and hyperdynamic LV with a moderate- to large-sized circumferential pericardial effusion (Figure 2) with increased echogenicity in the pericardial effusion, suggesting blood or exudate. It showed borderline impaired diastolic filling (right ventricular diastolic collapse as well as enlarged inferior vena cava of 3.2 cm) but without right atrial collapse or augmentation of trans-mitral or trans-tricuspid blood flow by continuous wave Doppler imaging throughout the respiratory cycle. There was a 3.8-cm enlarged ascending mid-tubular aorta (Figure 3).Figure 3Parasternal long axis view of the heart. A large collection of pericardial fluid is noted, with marked compression of the right ventricle (RV). The aortic root is markedly dilated. LV = left ventricle.View Large Image Figure ViewerDownload Hi-res image Download (PPT) On arrival at the emergency department, blood pressure was 60/37 mm Hg, heart rate 80 beats per minute, respiratory rate of 12 breaths per minute, and afebrile. She was alert and oriented and only complaining of generalized weakness. Heart sounds were distant but normal rate, and results from lung examination were unremarkable; jugular venous pressure was elevated >20 cm water, though she had received 6 L of intravenous physiologic saline by the time of our initial evaluation. Pulsus paradoxus was 7 mm Hg. The electrocardiogram showed sinus rhythm at 87 beats per minute and left axis deviation without acute ST changes. Initial blood pressure was fluid responsive and, after 4 L of fluid, she was stable to undergo a computed tomography (CT) scan to assess for aortic dissection or pulmonary embolism. Pertinent laboratory data included cardiac troponin T <0.01 ng/mL, normal chemistry panel, liver and thyroid functions, and complete blood count. Lactate was 3.6 mM. The chest CT scan revealed a non-aneurysmal aorta (Figure 1A), a moderate to large pericardial effusion, and significant left ventricular (LV) thickening with small LV cavity size, suggesting hypertrophic obstructive cardiomyopathy (Figure 1B). The radiologist recommended cardiology evaluation on the basis of initial imaging data. A surface echocardiogram showed a small, under-filled, thickened and hyperdynamic LV with a moderate- to large-sized circumferential pericardial effusion (Figure 2) with increased echogenicity in the pericardial effusion, suggesting blood or exudate. It showed borderline impaired diastolic filling (right ventricular diastolic collapse as well as enlarged inferior vena cava of 3.2 cm) but without right atrial collapse or augmentation of trans-mitral or trans-tricuspid blood flow by continuous wave Doppler imaging throughout the respiratory cycle. There was a 3.8-cm enlarged ascending mid-tubular aorta (Figure 3). DiagnosisThe patient continued to have persistent hypotension after 8 L of physiologic saline, with systolic blood pressures in the range of 60-70s mm Hg. A few findings were suggestive of pericardial tamponade, including persistent shock and borderline impaired diastolic filling. However, findings against tamponade included a normal pulsus paradoxus, without right atrial or right ventricular diastolic collapse, or respiratory variation in trans-mitral and trans-tricuspid blood flow.The other concern was pericardial effusion etiology. There was a clear distinction within the pericardial fluid of an echo-bright substance adjacent to the pericardium and an echo-dark fluid that looked more like typical pericardial fluid. A major concern was impending hemodynamic collapse if the pericardial material was coagulated blood, because percutaneous pericardiocentesis may dislodge a stable thrombus, and so a surgical approach would be a safer option. The patient continued to have persistent hypotension after 8 L of physiologic saline, with systolic blood pressures in the range of 60-70s mm Hg. A few findings were suggestive of pericardial tamponade, including persistent shock and borderline impaired diastolic filling. However, findings against tamponade included a normal pulsus paradoxus, without right atrial or right ventricular diastolic collapse, or respiratory variation in trans-mitral and trans-tricuspid blood flow. The other concern was pericardial effusion etiology. There was a clear distinction within the pericardial fluid of an echo-bright substance adjacent to the pericardium and an echo-dark fluid that looked more like typical pericardial fluid. A major concern was impending hemodynamic collapse if the pericardial material was coagulated blood, because percutaneous pericardiocentesis may dislodge a stable thrombus, and so a surgical approach would be a safer option. ManagementThe patient remained in shock during a time of night when ancillary surgical services were not immediately available and so, contrary to what guidelines suggest, a decision was made to perform an urgent ultrasound-guided pericardiocentesis from an apical approach. Forty milliliters of serous fluid were removed, after which no more fluid could be removed. Serous rather than sanguinous fluid initially was reassuring for the absence of tamponade due to acute coronary or vascular pathology. However, the patient remained unstable, so a subxyphoid approach was performed with needle placement in the pericardial space confirmed by agitated saline injection. A total of 150 mL of bloody fluid was removed, and a pericardial drain was inserted. It was thought that the initial serous fluid was from the pleural cavity. She had immediate blood pressure improvement after pericardiocentesis. A transesophageal echocardiogram (TEE) was then performed and revealed a dilated ascending aorta with sub-intimal hematoma. Reviewing her previous studies, a TEE conducted 6 months before assess for intracardiac thrombus revealed a small ulcerated plaque in the aortic arch that extended proximally to the aortic valve.The patient tolerated TEE well and became hypertensive in the coronary care unit, which was controlled using oral labetalol, 300 mg three times daily. A few hours later the cardiothoracic surgery team evaluated the patient and determined she had a contained aortic rupture. A repeat chest CT study 36 hours after presentation showed an interval increase in ascending aortic aneurysm intramural hematoma, though she remained clinically stable. After holding her rivaroxiban for 48 hours, she was taken urgently to the operating room to surgically manage her expanding intramural hematoma. She was found to have a Stanford type A dissection from the aortic root extending to her right innominate artery and underwent replacement of the ascending aorta with Dacron graft. She recovered rapidly and was discharged to a skilled nursing facility. Interestingly, 24 hours before presentation, her son had presented with an acute Stanford type A aortic dissection that required emergent surgery by the same cardiothoracic surgeon. The family later revealed that the patient's sister died of an aortic dissection a few years prior, raising the possibility of genetic predisposition to aortopathies. The patient remained in shock during a time of night when ancillary surgical services were not immediately available and so, contrary to what guidelines suggest, a decision was made to perform an urgent ultrasound-guided pericardiocentesis from an apical approach. Forty milliliters of serous fluid were removed, after which no more fluid could be removed. Serous rather than sanguinous fluid initially was reassuring for the absence of tamponade due to acute coronary or vascular pathology. However, the patient remained unstable, so a subxyphoid approach was performed with needle placement in the pericardial space confirmed by agitated saline injection. A total of 150 mL of bloody fluid was removed, and a pericardial drain was inserted. It was thought that the initial serous fluid was from the pleural cavity. She had immediate blood pressure improvement after pericardiocentesis. A transesophageal echocardiogram (TEE) was then performed and revealed a dilated ascending aorta with sub-intimal hematoma. Reviewing her previous studies, a TEE conducted 6 months before assess for intracardiac thrombus revealed a small ulcerated plaque in the aortic arch that extended proximally to the aortic valve. The patient tolerated TEE well and became hypertensive in the coronary care unit, which was controlled using oral labetalol, 300 mg three times daily. A few hours later the cardiothoracic surgery team evaluated the patient and determined she had a contained aortic rupture. A repeat chest CT study 36 hours after presentation showed an interval increase in ascending aortic aneurysm intramural hematoma, though she remained clinically stable. After holding her rivaroxiban for 48 hours, she was taken urgently to the operating room to surgically manage her expanding intramural hematoma. She was found to have a Stanford type A dissection from the aortic root extending to her right innominate artery and underwent replacement of the ascending aorta with Dacron graft. She recovered rapidly and was discharged to a skilled nursing facility. Interestingly, 24 hours before presentation, her son had presented with an acute Stanford type A aortic dissection that required emergent surgery by the same cardiothoracic surgeon. The family later revealed that the patient's sister died of an aortic dissection a few years prior, raising the possibility of genetic predisposition to aortopathies. DiscussionAcute aortic syndromes present as three clinical scenarios: aortic dissection, penetrating atherosclerotic ulcer (PAU), and intramural hematoma (IMH). Aortic dissections are classified as either ascending (Stanford type A) or descending (Stanford type B) dissections, with type A dissections necessitating urgent surgical repair. The incidence of thoracic aortic aneurysm rupture is 3 per 100,000 person years.1Clouse W.D. Hallett Jr., J.W. Schaff H.V. et al.Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture.Mayo Clin Proc. 2004; 79: 176-180Abstract Full Text Full Text PDF PubMed Scopus (420) Google ScholarThe etiology can be either heritable or sporadic. Because of her strong family history of aortopathy, it is thought that there could be a genetic component. There is an increasingly recognized familial syndrome in up to 20% of patients with aortic dissections, called familial thoracic aortic aneurysm and dissection, that is associated with α-actin smooth muscle mutations (ACTA2 gene). They display an autosomal dominant mode of inheritance, and hence all first-degree relatives should be screened. Our patient's physical appearance was not consistent with the phenotypic manifestation of Marfans, Turners, Ehler-Danlos, or Loeys-Dietz syndrome. She also did not have a bicuspid aortic valve.In our patient, it is thought that her known PAU at the aortic arch caused an entry tear leading to an ascending dissection, pericardial tamponade, and subsequently a contained rupture. That would explain her bloody layering pericardial effusion causing tamponade. It is interesting that neither the CT scan nor TEE showed an aortic dissection, but both showed a thickened enlarged aorta, suggestive of dissection. This is an unusual presentation because PAU is typically present in the mid or distal descending thoracic aorta with penetration of the internal elastic lamina, leading to hematoma and associated with extensive atherosclerosis. Intramural hematomas are not associated with intimal tears, and it is unclear how they arise, but they may be associated with cystic medial necrosis. They are thought to be precursors to, rather than the result of, a dissection. Both PAU and IMH were associated with worse 1-year prognosis than type A or type B aortic dissections,2Coady M.A. Ikonomidis J.S. Cheung A.T. et al.Surgical management of descending thoracic aortic disease: open and endovascular approaches: a scientific statement from the American Heart Association.Circulation. 2010; 121: 2780-2804Crossref PubMed Scopus (134) Google Scholar possibly because PAU and IMH are diseases of the elderly.Current recommendations argue against placement of a pericardial drain in a patient with concomitant Stanford type A dissection and pericardial tamponade because that can lead to exsanguination into the pericardial space. Surgery is the treatment of choice. A case review showed that pericardiocenteses in patients presenting with hemopericardium from aortic dissection lead to death in 3 of 4 patients treated with successful pericardiocentesis.3Isselbacher E.M. Cigarroa J.E. Eagle K.A. Cardiac tamponade complicating proximal aortic dissection. Is pericardiocentesis harmful?.Circulation. 1994; 90: 2375-2378Crossref PubMed Scopus (108) Google Scholar Another small study showed that patients with type A aortic dissection and signs of preoperative cardiac tamponade without palpable pulses had a 16-fold increased risk of poor outcome and especially death as compared with patients with type A dissection and impending cardiac tamponade with palpable pulses.4Bayegan K. Domanovits H. Schillinger M. et al.Acute type A aortic dissection: the prognostic impact of preoperative cardiac tamponade.Eur J Cardiothorac Surg. 2001; 20: 1194-1198Crossref PubMed Scopus (41) Google Scholar However, we hypothesized that she was clinically unstable from her tamponade and that bleeding was self-contained, arguing for the need for emergent pericardiocentesis.In summary, this is an unusual presentation of an acute aortic syndrome with complicated diagnosis and management. The exact etiology of the patient's IMH, tamponade, and eventual dissection is unclear, though we suspect both PAU and familial aortopathy are responsible. No one diagnostic test led to a definitive diagnosis, requiring us to utilize multiple modalities quickly in this unstable patient. After synthesis of the information, an informed decision that differed from the guidelines produced a successful outcome to stabilize the patient and allow for a positive surgical outcome. Acute aortic syndromes present as three clinical scenarios: aortic dissection, penetrating atherosclerotic ulcer (PAU), and intramural hematoma (IMH). Aortic dissections are classified as either ascending (Stanford type A) or descending (Stanford type B) dissections, with type A dissections necessitating urgent surgical repair. The incidence of thoracic aortic aneurysm rupture is 3 per 100,000 person years.1Clouse W.D. Hallett Jr., J.W. Schaff H.V. et al.Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture.Mayo Clin Proc. 2004; 79: 176-180Abstract Full Text Full Text PDF PubMed Scopus (420) Google Scholar The etiology can be either heritable or sporadic. Because of her strong family history of aortopathy, it is thought that there could be a genetic component. There is an increasingly recognized familial syndrome in up to 20% of patients with aortic dissections, called familial thoracic aortic aneurysm and dissection, that is associated with α-actin smooth muscle mutations (ACTA2 gene). They display an autosomal dominant mode of inheritance, and hence all first-degree relatives should be screened. Our patient's physical appearance was not consistent with the phenotypic manifestation of Marfans, Turners, Ehler-Danlos, or Loeys-Dietz syndrome. She also did not have a bicuspid aortic valve. In our patient, it is thought that her known PAU at the aortic arch caused an entry tear leading to an ascending dissection, pericardial tamponade, and subsequently a contained rupture. That would explain her bloody layering pericardial effusion causing tamponade. It is interesting that neither the CT scan nor TEE showed an aortic dissection, but both showed a thickened enlarged aorta, suggestive of dissection. This is an unusual presentation because PAU is typically present in the mid or distal descending thoracic aorta with penetration of the internal elastic lamina, leading to hematoma and associated with extensive atherosclerosis. Intramural hematomas are not associated with intimal tears, and it is unclear how they arise, but they may be associated with cystic medial necrosis. They are thought to be precursors to, rather than the result of, a dissection. Both PAU and IMH were associated with worse 1-year prognosis than type A or type B aortic dissections,2Coady M.A. Ikonomidis J.S. Cheung A.T. et al.Surgical management of descending thoracic aortic disease: open and endovascular approaches: a scientific statement from the American Heart Association.Circulation. 2010; 121: 2780-2804Crossref PubMed Scopus (134) Google Scholar possibly because PAU and IMH are diseases of the elderly. Current recommendations argue against placement of a pericardial drain in a patient with concomitant Stanford type A dissection and pericardial tamponade because that can lead to exsanguination into the pericardial space. Surgery is the treatment of choice. A case review showed that pericardiocenteses in patients presenting with hemopericardium from aortic dissection lead to death in 3 of 4 patients treated with successful pericardiocentesis.3Isselbacher E.M. Cigarroa J.E. Eagle K.A. Cardiac tamponade complicating proximal aortic dissection. Is pericardiocentesis harmful?.Circulation. 1994; 90: 2375-2378Crossref PubMed Scopus (108) Google Scholar Another small study showed that patients with type A aortic dissection and signs of preoperative cardiac tamponade without palpable pulses had a 16-fold increased risk of poor outcome and especially death as compared with patients with type A dissection and impending cardiac tamponade with palpable pulses.4Bayegan K. Domanovits H. Schillinger M. et al.Acute type A aortic dissection: the prognostic impact of preoperative cardiac tamponade.Eur J Cardiothorac Surg. 2001; 20: 1194-1198Crossref PubMed Scopus (41) Google Scholar However, we hypothesized that she was clinically unstable from her tamponade and that bleeding was self-contained, arguing for the need for emergent pericardiocentesis. In summary, this is an unusual presentation of an acute aortic syndrome with complicated diagnosis and management. The exact etiology of the patient's IMH, tamponade, and eventual dissection is unclear, though we suspect both PAU and familial aortopathy are responsible. No one diagnostic test led to a definitive diagnosis, requiring us to utilize multiple modalities quickly in this unstable patient. After synthesis of the information, an informed decision that differed from the guidelines produced a successful outcome to stabilize the patient and allow for a positive surgical outcome." @default.
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