© 2014 Wiley Periodicals, Inc.
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CASE REPORT ______________________________________________________________________
Acute Type A Aortic Dissection with Cardiac Tamponade: Unexpected Late Survival without Aortic Surgery Katie Jerzewski, B.Sc.,*,y and Alexander Kulik, M.D., M.P.H.*,y *Lynn Heart and Vascular Institute, Boca Raton Regional Hospital, Boca Raton, Florida; and yCharles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida ABSTRACT Acute type A aortic dissection complicated by cardiac tamponade portends a lethal prognosis. We report the unusual case of an older female who unexpectedly survived for more than one year after presentation with acute dissection and tamponade. She was treated with a pericardial window and remains alive now four years after presentation. doi: 10.1111/jocs.12369 (J Card
Surg 2014;29:529–530) Approximately 10% of patients with acute type A aortic dissection present with cardiac tamponade. The existence of cardiac tamponade dramatically increases the risk of early mortality,1–3 and the majority of dissection patients with tamponade do not survive to hospital discharge.1 Surgical repair of the dissected aorta is believed to be the only effective treatment to prevent early death.4–5 We report the case of a patient who unexpectedly survived for more than one year after presentation with acute dissection and tamponade before undergoing palliative pericardial window surgery. CASE REPORT An 87-year-old female with a past medical history of hypertension and permanent pacemaker presented with acute chest pain radiating to her back. An electrocardiogram noted inverted T waves, but myocardial enzymes were negative, ruling out acute coronary syndrome. A chest X-ray demonstrated an enlarged cardiac silhouette. A transthoracic echocardiogram identified an aortic dissection with a dilated aortic root (5 cm) and significant aortic valve insufficiency. There
Conflict of interest: The authors acknowledge no conflict of interest in the submission. Address for correspondence: Alexander Kulik, M.D., M.P.H., Lynn Heart and Vascular Institute, Boca Raton Regional Hospital, 801 Meadows Road, Suite 104, Boca Raton, FL 33486. Fax: þ1-561-9556310; e-mail: [email protected]
was a large circumferential pericardial effusion and tamponade physiology, with diastolic compression of the right ventricle, systolic compression of the right atrium, and dilatation of the inferior vena cava. Computed tomography (CT) imaging confirmed the presence of an aortic dissection isolated to the ascending aorta (Type A, Debakey type II), with a 6.2 cm dissected ascending aortic aneurysm and a large pericardial effusion 3 cm in transverse dimension. Despite the echocardiographic evidence of tamponade, the patient remained hemodynamically stable (blood pressure 110/50). A lengthy discussion was held with the patient and her family regarding the option of aortic dissection repair. Surgery was predicted to be high risk, and would be associated with a long recovery given the patient’s older age and frailty. The patient declined surgical intervention and requested palliative medical therapy instead. Given the presence of cardiac tamponade, it was anticipated that the patient would only survive a matter of a few days. The patient was discharged home with hospice care. Surprisingly, the patient survived for more than one year, despite the presence of a large pericardial effusion with tamponade physiology and a dissected aortic aneurysm. Over that one-year period, the patient continued with her usual activities, including vacation and air travel, with the help of a full-time caregiver. Thirteen months after the initial diagnosis of acute dissection, the patient presented once again to the emergency department complaining of worsening shortness of breath and fatigue. A chest X-ray demonstrated a cardiac silhouette that occupied the entire width of the patient’s chest (Fig. 1). A transthoracic echocardiogram revealed an extremely large pericardial effusion with persistent tamponade physiology. A CT showed severe aortic root dilatation (7 cm), a massive pericardial effusion, and reflux of contrast into inferior vena cava consistent with cardiac tamponade (Fig. 2). Compared to the CT scan from 13 months earlier, the pericardial cavity had enlarged and was compressing the right and left lungs. The patient continued to refuse conventional aortic surgical repair. However, with worsening shortness of symptoms, she agreed to undergo a pericardial window. Via a left mini anterior thoracotomy, a pericardial window was performed and 1000 mL of serous fluid was evacuated under tension. Postoperatively, the patient had an uncomplicated recovery and was discharged home six days later. Now four years after the presentation, she continues to be active and traveling at age 91. DISCUSSION Patients who do not undergo surgery for acute type A aortic dissection have significantly higher early mortality rates, and should hospital discharge be possible, longterm survival is poor.6–7 Factors that are strong
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JERZEWSKI AND KULIK AORTIC DISSECTION AND TAMPONADE WITHOUT SURGERY
Figure 1. Chest X-ray documenting a large pericardial effusion spanning the entire width of the patient’s chest.
J CARD SURG 2014;29:529–530
the increasing pressure as the pericardial fluid slowly accumulated, preventing severe compression of her cardiac chambers. While it may be contraindicated in the acute setting,4 in the chronic state, the pericardial window provided therapeutic relief. In our experience, we have found the thoracotomy approach to be welltolerated and more likely to prevent a recurrent pericardial effusion compared to a sub-xyphoid incision. Previous studies have demonstrated acceptable outcomes associated with medical treatment for acute type A aortic dissection. For patients who were denied surgery or refused intervention, 42% were noted to be alive 30 days after presentation,8 with some surviving up to three years post-hospitalization.6–7,9 Medical treatment may be particularly appropriate for dissection patients presenting with strong risk factors for adverse outcome, such as age more than 80 years, coma, shock, acute renal failure, and a history of previous cardiac surgery.10 Now four years after initial presentation, we believe this patient may be one of the longest reported survivors following conservative management of acute type A aortic dissection. REFERENCES
Figure 2. Computed tomography (CT) scan illustrating a severely dilated aortic root (7 cm), large pericardial effusion, and bilateral lung compression.
predictors of early mortality with acute type A aortic dissection include older age, abrupt onset of pain, and the presence of tamponade.2 Cardiac tamponade is the most common cause of early death.1–3 Dissection patients with tamponade are treated promptly with surgical aortic repair.1,3 Pericardiocentesis had previously been considered as a temporizing measure in the acute setting, but it is no longer recommended. Many patients die during an emergency pericardial drainage procedure due to the rebound increase in intra-aortic pressure that can occur, leading to recurrent hemorrhage and ultimately lethal tamponade, suggesting that this therapeutic modality is contraindicated for use during acute dissection.4 With medical management alone and no surgical repair, nearly all dissection patients who present with tamponade die during the initial hospitalization.1 We hypothesize that this patient had a surprisingly compliant pericardium that was able to accommodate
1. Gilon D, Mehta RH, Oh JK, et al: Characteristics and inhospital outcomes of patients with cardiac tamponade complicating type A acute aortic dissection. Am J Cardiol 2009;103:1029–1031. 2. Mehta RH, Suzuki T, Hagan PG, et al: Predicting death in patients with acute type a aortic dissection. Circulation 2002;105:200–206. 3. Isselbacher EM, Cigarroa JE, Eagle KA: Cardiac tamponade complicating proximal aortic dissection. Is pericardiocentesis harmful? Circulation 1994;90:2375–2378. 4. Feldman M, Shah M, Elefteriades JA: Medical management of acute type A aortic dissection. Ann Thorac Cardiovasc Surg 2009;15:286–293. 5. Nienaber CA, Eagle KA: Aortic dissection: New frontiers in diagnosis and management: Part II: Therapeutic management and follow-up. Circulation 2003;108:772–778. 6. Tsai TT, Evangelista A, Nienaber CA, et al: Long-term survival in patients presenting with type A acute aortic dissection: Insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation 2006;114: I350–I356. 7. Davies RR, Coe MP, Mandapati D, et al: Thoracic Surgery Directors Association Award. What is the optimal management of late-presenting survivors of acute type A aortic dissection? Ann Thorac Surg 2007;83:1593–1601, discussion 1601–1602. 8. Hagan PG, Nienaber CA, Isselbacher EM, et al: The International Registry of Acute Aortic Dissection (IRAD): New insights into an old disease. JAMA 2000;283:897– 903. 9. Scholl FG, Coady MA, Davies R, et al: Interval or permanent nonoperative management of acute type A aortic dissection. Arch Surg 1999;134:402–405, discussion 5–6. 10. Centofanti P, Flocco R, Ceresa F, et al: Is surgery always mandatory for type A aortic dissection? Ann Thorac Surg 2006;82:1658–1663, discussion 1664.
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CASE REPORT ______________________________________________________________________
Acute Type A Aortic Dissection with Cardiac Tamponade: Unexpected Late Survival without Aortic Surgery Katie Jerzewski, B.Sc.,*,y and Alexander Kulik, M.D., M.P.H.*,y *Lynn Heart and Vascular Institute, Boca Raton Regional Hospital, Boca Raton, Florida; and yCharles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida ABSTRACT Acute type A aortic dissection complicated by cardiac tamponade portends a lethal prognosis. We report the unusual case of an older female who unexpectedly survived for more than one year after presentation with acute dissection and tamponade. She was treated with a pericardial window and remains alive now four years after presentation. doi: 10.1111/jocs.12369 (J Card
Surg 2014;29:529–530) Approximately 10% of patients with acute type A aortic dissection present with cardiac tamponade. The existence of cardiac tamponade dramatically increases the risk of early mortality,1–3 and the majority of dissection patients with tamponade do not survive to hospital discharge.1 Surgical repair of the dissected aorta is believed to be the only effective treatment to prevent early death.4–5 We report the case of a patient who unexpectedly survived for more than one year after presentation with acute dissection and tamponade before undergoing palliative pericardial window surgery. CASE REPORT An 87-year-old female with a past medical history of hypertension and permanent pacemaker presented with acute chest pain radiating to her back. An electrocardiogram noted inverted T waves, but myocardial enzymes were negative, ruling out acute coronary syndrome. A chest X-ray demonstrated an enlarged cardiac silhouette. A transthoracic echocardiogram identified an aortic dissection with a dilated aortic root (5 cm) and significant aortic valve insufficiency. There
Conflict of interest: The authors acknowledge no conflict of interest in the submission. Address for correspondence: Alexander Kulik, M.D., M.P.H., Lynn Heart and Vascular Institute, Boca Raton Regional Hospital, 801 Meadows Road, Suite 104, Boca Raton, FL 33486. Fax: þ1-561-9556310; e-mail: [email protected]
was a large circumferential pericardial effusion and tamponade physiology, with diastolic compression of the right ventricle, systolic compression of the right atrium, and dilatation of the inferior vena cava. Computed tomography (CT) imaging confirmed the presence of an aortic dissection isolated to the ascending aorta (Type A, Debakey type II), with a 6.2 cm dissected ascending aortic aneurysm and a large pericardial effusion 3 cm in transverse dimension. Despite the echocardiographic evidence of tamponade, the patient remained hemodynamically stable (blood pressure 110/50). A lengthy discussion was held with the patient and her family regarding the option of aortic dissection repair. Surgery was predicted to be high risk, and would be associated with a long recovery given the patient’s older age and frailty. The patient declined surgical intervention and requested palliative medical therapy instead. Given the presence of cardiac tamponade, it was anticipated that the patient would only survive a matter of a few days. The patient was discharged home with hospice care. Surprisingly, the patient survived for more than one year, despite the presence of a large pericardial effusion with tamponade physiology and a dissected aortic aneurysm. Over that one-year period, the patient continued with her usual activities, including vacation and air travel, with the help of a full-time caregiver. Thirteen months after the initial diagnosis of acute dissection, the patient presented once again to the emergency department complaining of worsening shortness of breath and fatigue. A chest X-ray demonstrated a cardiac silhouette that occupied the entire width of the patient’s chest (Fig. 1). A transthoracic echocardiogram revealed an extremely large pericardial effusion with persistent tamponade physiology. A CT showed severe aortic root dilatation (7 cm), a massive pericardial effusion, and reflux of contrast into inferior vena cava consistent with cardiac tamponade (Fig. 2). Compared to the CT scan from 13 months earlier, the pericardial cavity had enlarged and was compressing the right and left lungs. The patient continued to refuse conventional aortic surgical repair. However, with worsening shortness of symptoms, she agreed to undergo a pericardial window. Via a left mini anterior thoracotomy, a pericardial window was performed and 1000 mL of serous fluid was evacuated under tension. Postoperatively, the patient had an uncomplicated recovery and was discharged home six days later. Now four years after the presentation, she continues to be active and traveling at age 91. DISCUSSION Patients who do not undergo surgery for acute type A aortic dissection have significantly higher early mortality rates, and should hospital discharge be possible, longterm survival is poor.6–7 Factors that are strong
530
JERZEWSKI AND KULIK AORTIC DISSECTION AND TAMPONADE WITHOUT SURGERY
Figure 1. Chest X-ray documenting a large pericardial effusion spanning the entire width of the patient’s chest.
J CARD SURG 2014;29:529–530
the increasing pressure as the pericardial fluid slowly accumulated, preventing severe compression of her cardiac chambers. While it may be contraindicated in the acute setting,4 in the chronic state, the pericardial window provided therapeutic relief. In our experience, we have found the thoracotomy approach to be welltolerated and more likely to prevent a recurrent pericardial effusion compared to a sub-xyphoid incision. Previous studies have demonstrated acceptable outcomes associated with medical treatment for acute type A aortic dissection. For patients who were denied surgery or refused intervention, 42% were noted to be alive 30 days after presentation,8 with some surviving up to three years post-hospitalization.6–7,9 Medical treatment may be particularly appropriate for dissection patients presenting with strong risk factors for adverse outcome, such as age more than 80 years, coma, shock, acute renal failure, and a history of previous cardiac surgery.10 Now four years after initial presentation, we believe this patient may be one of the longest reported survivors following conservative management of acute type A aortic dissection. REFERENCES
Figure 2. Computed tomography (CT) scan illustrating a severely dilated aortic root (7 cm), large pericardial effusion, and bilateral lung compression.
predictors of early mortality with acute type A aortic dissection include older age, abrupt onset of pain, and the presence of tamponade.2 Cardiac tamponade is the most common cause of early death.1–3 Dissection patients with tamponade are treated promptly with surgical aortic repair.1,3 Pericardiocentesis had previously been considered as a temporizing measure in the acute setting, but it is no longer recommended. Many patients die during an emergency pericardial drainage procedure due to the rebound increase in intra-aortic pressure that can occur, leading to recurrent hemorrhage and ultimately lethal tamponade, suggesting that this therapeutic modality is contraindicated for use during acute dissection.4 With medical management alone and no surgical repair, nearly all dissection patients who present with tamponade die during the initial hospitalization.1 We hypothesize that this patient had a surprisingly compliant pericardium that was able to accommodate
1. Gilon D, Mehta RH, Oh JK, et al: Characteristics and inhospital outcomes of patients with cardiac tamponade complicating type A acute aortic dissection. Am J Cardiol 2009;103:1029–1031. 2. Mehta RH, Suzuki T, Hagan PG, et al: Predicting death in patients with acute type a aortic dissection. Circulation 2002;105:200–206. 3. Isselbacher EM, Cigarroa JE, Eagle KA: Cardiac tamponade complicating proximal aortic dissection. Is pericardiocentesis harmful? Circulation 1994;90:2375–2378. 4. Feldman M, Shah M, Elefteriades JA: Medical management of acute type A aortic dissection. Ann Thorac Cardiovasc Surg 2009;15:286–293. 5. Nienaber CA, Eagle KA: Aortic dissection: New frontiers in diagnosis and management: Part II: Therapeutic management and follow-up. Circulation 2003;108:772–778. 6. Tsai TT, Evangelista A, Nienaber CA, et al: Long-term survival in patients presenting with type A acute aortic dissection: Insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation 2006;114: I350–I356. 7. Davies RR, Coe MP, Mandapati D, et al: Thoracic Surgery Directors Association Award. What is the optimal management of late-presenting survivors of acute type A aortic dissection? Ann Thorac Surg 2007;83:1593–1601, discussion 1601–1602. 8. Hagan PG, Nienaber CA, Isselbacher EM, et al: The International Registry of Acute Aortic Dissection (IRAD): New insights into an old disease. JAMA 2000;283:897– 903. 9. Scholl FG, Coady MA, Davies R, et al: Interval or permanent nonoperative management of acute type A aortic dissection. Arch Surg 1999;134:402–405, discussion 5–6. 10. Centofanti P, Flocco R, Ceresa F, et al: Is surgery always mandatory for type A aortic dissection? Ann Thorac Surg 2006;82:1658–1663, discussion 1664.
