53-Year-Old Man With Progressive Dyspnea and Orthopnea

Abstract

A 53-year-old man with hypertension (HTN) presented to his physician with worsening dyspnea on exertion and orthopnea of 3 weeks' duration. His symptoms were originally noticed while he was performing farm work and worsened to the point that he could not walk short distances without being short of breath. Before this decline, the patient was physically active daily. Over this same period, the patient also began to experience orthopnea and nocturnal dyspnea. He did not have any chest pain, lower extremity edema, or syncope. Aside from the patient's acute symptoms, he was relatively healthy. His only medical problems included HTN, for which he took enalapril inconsistently. Over the previous year, his systolic blood pressure had averaged in the 140s to 150s mm Hg. His family history was negative for any sudden cardiac death. Social history was notable for secondhand smoke exposure and alcohol use 3 times a week. Physical examination revealed a blood pressure of 145/53 mm Hg, heart rate of 82 beats/min, respiratory rate of 22 breaths/min, temperature of 36.7°C, and oxygen saturation of 93%. He was noted to be dyspneic on examination with no peripheral edema. There was mildly increased venous distention. Cardiac examination revealed an enlarged laterally displaced apical impulse with a short diastolic murmur at the left sternal margin. Lung examination was notable for a few basilar crackles. Chest radiography revealed borderline cardiomegaly with pulmonary venous congestion and small bilateral pleural effusions with no infiltrate. Electrocardiography identified normal sinus rhythm with a single premature ventricular contraction. Laboratory studies revealed the following (reference ranges provided parenthetically): hemoglobin, 13.1 g/dL (13.5-17.5 g/dL); white blood cell count, 7.6 × 109/L (3.5-10.5 × 109/L); platelet count, 285 × 109/L (150-450 × 109/L); sodium, 143 mmol/L (135-145 mmol/L); potassium, 3.9 mmol/L (3.6-5.2 mmol/L); creatinine, 0.9 mg/dL (0.8-1.3 mg/dL); C-reactive protein, 19.1 mg/L (≤8.0 mg/L); and N-terminal pro-B-type natriuretic peptide, 2444 pg/mL (10-64 pg/mL).1.Based on the initial presentation, which one of the following is the most likely valve lesion?a.Aortic valve stenosisb.Aortic valve insufficiencyc.Mitral valve insufficiencyd.Mitral valve stenosise.Pulmonary valve insufficiency Overall classification of valvular disease is based on a combination of physical examination findings, symptoms, and imaging results and is helpful for determining possible surgical intervention.1Nishimura R.A. Otto C.M. Bonow R.O. et al.2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines.J Am Coll Cardiol. 2014; 63 ([published correction appears in J Am Coll Cardiol. 2014;63(22):2489]): 2438-2488Crossref PubMed Scopus (1400) Google Scholar Aortic valve stenosis usually presents with symptoms of chest pain, dyspnea, and decreased exercise ability. On examination, a crescendo-decrescendo systolic murmur with radiation to the carotids is often auscultated.2Linefsky J.P. Otto C.M. Aortic valve disease.Medicine. 2014; 42: 638-643Abstract Full Text Full Text PDF Scopus (1) Google Scholar Although our patient had dyspnea, his murmur was noted to be diastolic and not systolic. Furthermore, a displaced apical impulse would not be consistent with aortic valve stenosis. Regurgitation or insufficiency of the aortic valve occurs when blood rushes retrograde from the aorta into the left ventricle, leading to a widened pulse pressure and potentially to increased left ventricular filling pressures. Clinically, insufficiency presents with exercise intolerance and symptoms of heart failure. Auscultation over the left sternal border often reveals a high-pitched diastolic murmur heard loudest while the patient is sitting upright during expiration.2Linefsky J.P. Otto C.M. Aortic valve disease.Medicine. 2014; 42: 638-643Abstract Full Text Full Text PDF Scopus (1) Google Scholar Often, a displaced, enlarged apical impulse is present, and the patient may have the physical examination finding of bounding pulses. Given the findings of widened pulse pressure, left sternal border diastolic murmur, and new signs of heart failure, the patient most likely has a lesion involving the aortic valve leading to insufficiency. Insufficiency of the mitral valve typically presents as a pansystolic murmur at the heart apex with radiation to the axilla, findings inconsistent with our patient's presentation. Mitral stenosis is often the result of rheumatic heart disease and occurs progressively over time.3Reid A. Ray S. Mitral and tricuspid valve disease.Medicine. 2014; 42: 632-637Abstract Full Text Full Text PDF Scopus (2) Google Scholar Shortness of breath and fatigue are common. The apical impulse is not displaced, and on auscultation, there is typically a low-pitched diastolic murmur heard over the apex. Given the patient's subacute onset of symptoms, displaced apical impulse, murmur location, and widened pulse pressure, it is unlikely that his murmur is from mitral stenosis. Pulmonic valve regurgitation is best heard over the left sternal border and is most often a diastolic murmur. However, this patient has no signs of right-sided heart failure such as jugular vein distention or peripheral edema, making this lesion unlikely.4Karnath B. Thornton W. Auscultation of the heart.Hosp Physician. 2002; 38: 39-43Google Scholar Given the presentation of dyspnea, a diastolic murmur over the left sternal border, and an elevation in N-terminal pro-B-type natriuretic peptide, decompensated heart failure would be the diagnosis at this point. After the patient's appointment, he was given the diagnosis of heart failure, started on furosemide, 80 mg daily, and instructed to follow up after completing further work-up for his heart failure.2.Which one of the following is the most appropriate next step in the evaluation of this patient?a.Transthoracic echocardiography (TTE)b.Repeated radiography after management with diureticsc.Coronary angiographyd.Cardiac magnetic resonance imaging (MRI)e.Myocardial biopsy For patients presenting with signs of heart failure, assessment with echocardiography is recommended. Assessment of ventricular function, including wall motion, cardiac structure, and valve function, are key components of the echocardiographic examination and help establish the underlying cause of heart failure.5Metra M. Teerlink J.R. Heart failure.Lancet. 2017; 390: 1981-1995Abstract Full Text Full Text PDF PubMed Scopus (368) Google Scholar Although chest radiography can reveal differences in pulmonary congestion and cardiomegaly following diuresis, it would not be as powerful in terms of diagnostic yield as TTE. Coronary angiography may be required downstream to differentiate ischemic from nonischemic etiologies of left ventricular dysfunction but would not be the first step in evaluation. Magnetic resonance imaging should not be considered for the initial testing strategy in the evaluation of heart failure but may have a role downstream, when tissue characterization using this imaging modality may be helpful as a diagnostic and prognostic tool. Cardiac biopsy, similar to MRI, would not be an appropriate initial diagnostic test in a patient with new-onset heart failure that is otherwise not refractory to initial treatment strategies.5Metra M. Teerlink J.R. Heart failure.Lancet. 2017; 390: 1981-1995Abstract Full Text Full Text PDF PubMed Scopus (368) Google Scholar Two weeks after the patient initially presented to his physician, he underwent TTE. The study revealed severe aortic valve regurgitation, a tricuspid aortic valve, moderate-severe sinus of Valsalva dilatation (53 mm), moderate-severe ascending aorta dilatation (52 mm), and mild-moderate left ventricular enlargement with a calculated ejection fraction of 59%. Given the TTE finding of severe aortic regurgitation, he was urgently referred to the valve clinic. The patient was evaluated the following day. Compared to his presenting examination, the patient exhibited a transmitted cardiac murmur into the base of the right carotid artery, prominent carotid upstrokes, and a clear systolic and diastolic to-and-fro murmur at the base of the heart. Diagnostic considerations for severe subacute aortic regurgitation and ascending aortic dilatation included poorly controlled HTN vs aortopathy. Transesophageal echocardiography was scheduled to investigate the morphology of the aortic valve and a closer examination of the aorta. Computed tomographic angiography of the chest and abdomen was also ordered to assess the patient's aorta for possible surgical correction of the valve. Transesophageal echocardiography (TEE) that day unexpectedly revealed a DeBakey type II aortic dissection. The dissection extended from the ascending aorta to beyond the left subclavian artery with incomplete coaptation of the aortic valve due to interposition of the dissection flap into the left ventricular outflow tract and a small tear of the right coronary cusp. Severe aortic valve regurgitation, mild to moderate dilatation of the left ventricle (ejection fraction, 50%), tricuspid aortic valve, and dilatation of the aorta were also noted.3.Given the patient's TEE findings, which one of the following is the most likely cause of the patient's ascending aortic dissection?a.Genetically mediated aortopathyb.HTNc.Infectious aortitisd.Chest traumae.Cocaine abuse Genetic aortopathies imply underlying inheritance of an aortic defect that predisposes individuals to thoracic aortic aneurysms and as a result, dissections. Although abdominal aortic disease is usually related to age and coronary artery disease risk factors, thoracic aortic aneurysms are more often associated with an underlying familial aortopathy even if no syndromic condition is present.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar Given the patient's TEE findings of thoracic aneurysmal dilatation and his relatively young age, he most likely has a genetic aortopathy leading to his current presentation. Genetic aortopathies can manifest as syndromic conditions like Marfan syndrome or nonsyndromic conditions like bicuspid aortic valve with associated aortic disease.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar However, no bicuspid valve was seen on TEE, and the patient had no other findings of syndromic conditions such as lens dislocation or increased height.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar Although the patient did have a history of HTN, it was not severe, and given his relatively young age, a familial etiology should be a higher consideration, especially given the implications for screening.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar Infection of the aorta is a rare cause of dissection and typically presents with a recent history of an invasive procedure, immunocompromised status, or fever.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar Although the patient did have a remote history of fever, he was not immunocompromised and had had no recent procedures. Trauma to the chest wall can also lead to aortic dissection, accounting for about 5% of aortic dissections. However, this patient had experienced no trauma.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 8LeMaire S.A. Russell L. Epidemiology of thoracic aortic dissection.Nat Rev Cardiol. 2011; 8: 103-113Crossref PubMed Scopus (233) Google Scholar Cocaine usage has been reported to lead to aortic dissection but is a rare cause of dissection and unlikely in this case.8LeMaire S.A. Russell L. Epidemiology of thoracic aortic dissection.Nat Rev Cardiol. 2011; 8: 103-113Crossref PubMed Scopus (233) Google Scholar After the diagnosis of ascending aortic dissection was made on TEE, the patient was emergently taken to the coronary intensive care unit (ICU) and admitted for further management of a subacute Debakey II aortic dissection.4.Which one of the following is the most appropriate definitive management option for this patient?a.Management of the patient's HTN with a β-blockerb.Open repair with resection and replacement of the ascending aorta and aortic rootc.Aortic valve replacement without aorta interventiond.Endovascular stent grafting of the aortae.Wrapping of the ascending aorta with polyester mesh Definitive management of an aortic dissection centers around the location of the dissection. Continued medical management with a β-blocker once the patient's systolic blood pressure has decreased to less than 120 mm Hg is only appropriate when the dissection is involving the descending aorta.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar Open repair with resection and grafting of the aorta is the treatment of choice for ascending dissections given the high risk for rupture and development of cardiovascular and neurologic complications.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 9Nishida H. Tabata M. Fukui T. Sato Y. Kin H. Takanashi S. A systematic approach to improve the outcomes of type A aortic dissection.J Thorac Cardiovasc Surg. 2017; 154: 89-96.e1Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Aortic valve replacement alone would be inappropriate because not attending to the dissection exposes the patient to further risk of aortic rupture. Endovascular stent grafting should be reserved for patients who are not candidates for open repair and is not currently approved for dissections of the ascending aorta or the arch.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar Wrapping with polyester mesh is only appropriate for moderate-sized aortic aneurysms, not dissected aortas.10González-Santos J.M. Arnáiz-García M.E. Wrapping of the ascending aorta revisited—is there any role left for conservative treatment of ascending aortic aneurysm?.J Thorac Dis. 2017; 9: S488-S497Crossref PubMed Scopus (20) Google Scholar Intravenous esmolol and nitroglycerin were administered in the ICU for heart rate and blood pressure control, and the patient underwent cardiovascular surgery consultation. He was subsequently treated with an aortic valve resuspension, ascending aortic replacement with a 28-mm Dacron graft, proximal descending thoracic aorta stenting, total arch replacement with 26-mm Dacron graft, implantation of the arch vessels off the aortic arch graft with grafting of the left common carotid, left subclavian, and innominate arteries. Following surgical replacement and repair, intraoperative TEE revealed a functioning tricuspid aortic valve with trace aortic insufficiency and preserved ventricular function. The postoperative course was otherwise unremarkable. Transthoracic echocardiography and computed tomography on day of discharge revealed no aortic insufficiency with a stable ejection fraction of 51%. The patient was discharged home with a follow-up appointment in 1 week and scheduled computed tomographic angiography 6 months and 1 year following his operation. He was discharged with a regimen of losartan, metoprolol, furosemide, and aspirin.5.Which one of the following goals is most important for the long-term reduction of complications and improved prognosis in this patient?a.Body mass index less than 30 kg/m2b.Resting heart rate less than 80 beats/minc.Blood pressure less than 120/80 mm Hgd.Avoidance of secondhand smokee.Low-density lipoprotein cholesterol less than 100 mg/dL The most important causes of morbidity and mortality following an aortic dissection include aneurysm formation, dissection around the repaired aorta, and aortic rupture.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar Although maintaining healthy body mass index is important for overall cardiovascular health, it is not the most important long-term goal in managing an aortic dissection. Additionally, an elevated heart rate is not the most important risk factor for the development of long-term complications following repair. Blood pressure control with β-blockade is currently the first-line therapy for preventing long-term complications following a dissection.11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar Patients with a history of a dissection should have their blood pressures monitored closely because blood pressure readings above 120/80 mm Hg have been linked to worse overall prognosis. Patients with Marfan syndrome in particular require tight control.11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar Avoidance of cigarette smoke and control of atherosclerosis risk factors are integral parts of postdissection management, but the most emphasized risk factor to be controlled is the blood pressure.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar Patient was informed that he should monitor his blood pressure at home, with a goal of less than 120/80 mm Hg. He was instructed to continue losartan and increased-dosage metoprolol and to call his cardiologist's office if his blood pressure became consistently elevated. Aortic dissection is a vascular disease that can affect any area of the aorta and is associated with a high risk of mortality. The primary mechanism of an aortic dissection revolves around damage to the aortic intima leading to an opening or tear. This damage allows blood to enter the defect and separate the intimal and medial layers of the aorta, creating a false lumen.12Nienaber C.A. Clough R.E. Management of acute aortic dissection.Lancet. 2015; 385: 800-811Abstract Full Text Full Text PDF PubMed Scopus (349) Google Scholar The 2 major classification schemes for dissections are the DeBakey system (which uses the location of the entry tear and how far it travels) and the Stanford system (which uses the location of the entry tear only).7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 12Nienaber C.A. Clough R.E. Management of acute aortic dissection.Lancet. 2015; 385: 800-811Abstract Full Text Full Text PDF PubMed Scopus (349) Google Scholar Currently, the average incidence of aortic dissection is 2.9 to 3.5 per 100,000 person-years with an older male predominance (average age of 60-80 years).7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 12Nienaber C.A. Clough R.E. Management of acute aortic dissection.Lancet. 2015; 385: 800-811Abstract Full Text Full Text PDF PubMed Scopus (349) Google Scholar Major risk factors include HTN, familial defects involving transforming growth factor β–mediated changes to extracellular matrix proteins and smooth muscle myosin proteins, Marfan syndrome, bicuspid valve–associated aortopathy, cocaine use, and atherosclerosis.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar, 8LeMaire S.A. Russell L. Epidemiology of thoracic aortic dissection.Nat Rev Cardiol. 2011; 8: 103-113Crossref PubMed Scopus (233) Google Scholar The major symptoms of aortic dissections can vary widely based on the size and acuity of the injury. However, the most common signs include severe back or chest pain that is often described as tearing/sharp, new-onset heart failure, and syncope. Severe pain is seen in over 90% of dissections.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar Dyspnea and new aortic regurgitation are also reported.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 8LeMaire S.A. Russell L. Epidemiology of thoracic aortic dissection.Nat Rev Cardiol. 2011; 8: 103-113Crossref PubMed Scopus (233) Google Scholar Uncommonly, individuals may have a painless dissection. On physical examination, a new aortic insufficiency murmur may be appreciated as well as asymmetric blood pressure readings.11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar However, given the wide variety of dissection types, physical examination alone is not sufficient for diagnosis. Definitive diagnosis of an aortic dissection requires rapid imaging, typically using computed tomography or TEE (MRI has been used uncommonly in the diagnostic strategy). These imaging studies have been 100% sensitive and are all reliable tests for diagnosis, with the choice of testing dependent on local institutional availability and expertise.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 11Weigang E. Nienaber C.A. Rehders T.C. Ince H. Vahl C.F. Beyersdorf F. Management of patients with aortic dissection.Dtsch Arztebl Int. 2008; 105: 639-645PubMed Google Scholar Initial management for an aortic dissection involves initiating an intravenous β-blocker and a vasodilator to reduce the amount of shearing force along the false lumen and to reduce the chance of rupture. Commonly, the goal is for the blood pressure to be below 120/80 mm Hg. Patients should then be transferred to the ICU for continuous blood pressure monitoring.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar, 12Nienaber C.A. Clough R.E. Management of acute aortic dissection.Lancet. 2015; 385: 800-811Abstract Full Text Full Text PDF PubMed Scopus (349) Google Scholar Definitive management depends on whether the dissection involves the ascending or descending aorta. For an ascending dissection, the treatment involves expeditious open repair and replacement of the dissected aorta. Descending dissections can be managed initially with close blood pressure management in an ICU; if there are signs of progressive disease, including refractory pain, neurologic signs, or imaging signs of impending rupture, a surgical solution may be considered. Endovascular techniques may be considered in selected cases of descending thoracic dissections.7Hiratzka L.F. Bakris G.L. Beckman J.A. et al.2010 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.Circulation. 2010; 121 ([published correction appears in Circulation. 2010;122(4):e410]): e266-e369Crossref PubMed Scopus (1917) Google Scholar Ascending dissections involving the thoracic aorta are imperfectly understood. Although HTN and other risk factors can cause damage to the thoracic aorta, it is now believed that over 90% of idiopathic thoracic aneurysms and dissections of the ascending aorta are due to a genetic susceptibility.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar Currently, it is believed that overactivation of the transforming growth factor β pathway causes this susceptibility because an increase in this pathway leads to smooth muscle and extracellular matrix dysfunction and disarray.6Isselbacher E.M. Lino Cardenas C.L. Lindsay M.E. Hereditary influence in thoracic aortic aneurysm and dissection.Circulation. 2016; 133: 2516-2528Crossref PubMed Scopus (129) Google Scholar Awareness of these genetic predispositions are important for general management of ascending aortic disease and should be considered in patients presenting with what appears to be a straightforward ascending dissection caused by cardiovascular risk factors. Although not the most common cause, chronic ascending aortic dissection with resultant regurgitation should be part of the differential diagnosis for patients presenting with new-onset heart failure in the clinical setting because rapid recognition is critical for management and prognosis for the patient.