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Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm
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Available online at www.sciencedirect.com
ScienceDirect Journal of Acute Medicine xx (2015) 1e3 www.e-jacme.com
Case Report
Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm Yi-Ming Weng a,b,e, Tai-Yi Hsu c,e, Cheng-Wei Chan b,d,*, Jih-Chang Chen a,b a
Department of Emergency Medicine, Chang Gung Memorial Hospital, Taoyuan, Taiwan, ROC b College of Medicine, Chang Gung University, Taoyuan, Taiwan, ROC c Department of Emergency Medicine, China Medical University Hospital, Taichung, Taiwan, ROC d Department of Emergency Medicine, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Keelung, Taiwan, ROC Received 18 April 2014; revised 12 May 2015; accepted 18 June 2015 Available online ▪ ▪ ▪
Abstract Cardiogenic shock is defined by tissue hypoperfusion due to heart failure. The manifestations of acute right ventricular (RV) failure include a distended jugular vein and hypotension, but not rales. The causes of isolated acute RV failure include RV infarction, pulmonary embolism, cardiac tamponade, congenital disease, and valvular heart disease. Rupture of a sinus of Valsalva aneurysm (SVA) is rarely seen in emergency departments (EDs). We report a 46 year-old male who suffered from SVA rupture triggering acute RV failure and cardiogenic shock. Copyright © 2015, Taiwan Society of Emergency Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: Cardiogenic shock; Acute right ventricular failure; Rupture of a sinus of Valsalva aneurysm
1. Introduction
2. Case report
Cardiogenic shock is defined by tissue hypoperfusion due to heart failure. Acute right ventricular (RV) failure triggers cardiogenic shock by decreasing both preload and septal shift. Patients with acute RV failure may present with distended jugular veins and hypotension, but not rales. The causes of isolated acute RV failure include RV infarction, pulmonary embolism, cardiac tamponade, congenital disease, and valvular heart disease. Rupture of a sinus of Valsalva aneurysm (SVA) is rarely seen in emergency department. We report a case of SVA rupture triggering acute RV failure and cardiogenic shock. After emergency surgical repair, our patient was discharged without sequelae.
A 46-year-old male presented with acute abdominal pain 3 hours in duration. He had a medical history of hypertension and hyperlipidemia, which were being controlled by medication. He complained of moderate pain in the epigastric area and described it as dull in character. He denied any radiation pain. We found no chest/abdominal trauma or any aggravating factor. His symptoms included cold sweating, chest tightness, shortness of breath, nausea, and vomiting. No bloody vomitus or tarry stool was mentioned. At the emergency department, the patient appeared acutely ill and pale in color. Initial vital signs included a body temperature of 34 C, a pulse rate of 65 beats/min, and a blood pressure of 81/39 mmHg. Physical examination was unremarkable, except for a distended external jugular vein in the sitting position, a loud continuous diastolic heart murmur over the lower sternal border, and cold and clammy skin. Breathing sounds were clear and symmetrical in both lungs. The abdomen was soft, with mild tenderness over the peri-umbilical area but no peritoneal signs. Bedside ultrasonography was negative for ascites and
* Corresponding author. Department of Emergency Medicine, Chang Gung Memorial Hospital, Keelung, Number 222, Maijin Road, Anle District, Keelung City 204, Taiwan, ROC. E-mail address: [email protected] (C.-W. Chan). e These authors contributed equally to this manuscript.
http://dx.doi.org/10.1016/j.jacme.2015.06.002 2211-5587/Copyright © 2015, Taiwan Society of Emergency Medicine. Published by Elsevier Taiwan LLC. All rights reserved. Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002
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Y.-M. Weng et al. / Journal of Acute Medicine xx (2015) 1e3
pericardiac fluid. Electrocardiography revealed junctional rhythm without an ST-T change. The plain film showed a poorly expanded lung and cardiomegaly (Figure 1). Our initial suspicion was acute RV failure. To rule out pulmonary embolism, mechanical obstruction of the RV outflow, and any other anomaly, we ordered a computed tomographic (CT) scan. This revealed contrast pooling over the RV and superior and inferior vena cava. Retrograde flow into the RV via an aortic root was evident (Figures 2 and 3). We found no pulmonary embolism, mechanical lesion, or anomaly. Further two-dimensional transthoracic echocardiography revealed an SVA with a left-to-right
Figure 3. Shunt from the aortic root to the right ventricle (arrow), which is possible evidence of sinus of Valsalva aneurysm rupture.
shunt from the right coronary sinus into the RV. Rupture of an SVA was suspected. An emergency operation using a patch repair was performed immediately. Our patient made an uneventful recovery and was discharged 9 days after the surgery. 3. Discussion Figure 1. Chest x-ray revealing cardiomegaly.
Figure 2. Contrast regurgitation to the superior and inferior vena cava, evidence of poor heart pump function.
Our case presentation emphasizes that differential diagnosis of shock is critical for appropriate management. First, the finding of cold and clammy skin rendered a diagnosis of distributive shock unlikely. Also, neither neurogenic nor anaphylactic shock was considered, based on the present illness and medical history. Second, septic shock was ruled out because of the rapidly progressive course, lack of fever, and focus of infection. Third, rather than hypovolemic shock, we classified our case as cardiogenic shock because of the presence of a distended jugular vein without obvious volume loss. Furthermore, clear breathing sounds upon chest auscultation indicated an isolated RV failure. Although a retrograde flow was suspected following CT scan examination, we ordered two-dimensional transthoracic echocardiography because we considered this tool appropriate to aid diagnosis. Additionally, the loud diastolic heart murmur over the lower sternal border suggested valvular heart disease or an intracardiac shunt. Our report confirms previous suggestions that transthoracic echocardiography1e3 is a useful and reliable tool when employed to confirm SVA rupture. Other useful imaging modalities include transesophageal echocardiography,2e4 CT angiography,5 and magnetic resonance imaging. SVA is a dilatation caused by separation of the aortic media and annulus fibrosus.6 Acute rupture of the SVA is fatal. Most
Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002
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MODEL
Y.-M. Weng et al. / Journal of Acute Medicine xx (2015) 1e3
ruptured SVAs occur in patients <40 years of age1,2,4,6 and predominate in males.4,6 The right coronary sinus2,6 is affected most frequently, followed by the noncoronary sinus.7 Ruptures of an SVA into the right ventricle are most common.2,6 When an SVA ruptures, the patient may complain of chest pain,7 dyspnea,1,6 palpitations,1 tinnitus,1 orthopnea,6 upper abdominal discomfort, and edema of the lower extremities.6,8 Abdominal pain as the initial presentation of SVA rupture is rare; only one similar case has been reported previously.9 The cause of abdominal pain may be nonocclusive ischemia of the small bowel attributed to hypoperfusion caused, in turn, by acute heart-pump dysfunction.10 Other findings on physical examination include tachycardia, wide variations in pulse pressure, bounding carotid and peripheral pulses, pulmonary crackles,4 a prominent continuous precordial murmur8 with a thrill,4,8 hepatomegaly,6 upper abdominal tenderness, severe kidney insufficiency,6 and congestive hepatic insufficiency.6,8 The standard management for a ruptured SVA is early surgical intervention.1,2,6 Nonsurgical transcatheter-mediated closure of the rupture is another option.11 This report reminds clinicians that rupture of an SVA is a rare etiology of acute RV failure. An understanding of the pathophysiology of the disease entity helps emergency department physicians recognize specific manifestations of the condition. Prompt specialist attention with early intervention saves lives. Conflicts of interest All authors have no conflicts of interest to declare.
3
References 1. Guenther F, von Zur Muhlen C, Lohrmann J, Bode C, Geibel A. Rupture of an aneurysm of the noncoronary sinus of Valsalva into the right atrium. Eur J Echocardiogr. 2008;9:186e187. 2. Vincelj J, Starcevic B, Sokol I, Sutlic Z. Rupture of a right sinus of valsalva aneurysm into the right ventricle during vaginal delivery: a case report. Echocardiography. 2005;22:844e846. 3. Bouvier E, Porte JM, Laperche T, et al. Rupture of a sinus of Valsalva aneurysm in the left atrium. Arch Mal Coeur Vaiss. 2001;94:1409e1412. 4. Sanchez ME, García-Palmieri MR, Quintana CS, Kareh J. Heart failure in rupture of a sinus of valsalva aneurysm. Am J Med Sci. 2006;331:100e102. 5. Kantarci M, Doganay S, Gundogdu F, Unlu Y. A case with noncoronary sinus of Valsalva aneurysm: multidetector computed tomography findings. Heart Surg Forum. 2008;11:E372e374. 6. Galicia-Tornell MM, Marín-Solís B, Mercado-Astorga O, EspinozaAnguiano S, Martínez-Martínez M, Villalpando-Mendoza E. Sinus of Valsalva aneurysm with rupture. Case report and literature review. Cir Cir. 2009;77:441e445. 7. Turgeon AF, Dagenais F, Poirier P, Bauset R, Mathieu P. Myocardial infarction in a young woman secondary to a rupture of a noncoronary sinus of Valsalva aneurysm without coronary artery disease. Can J Cardiol. 2003;19:723e724. 8. Amoah AG, Frimpong-Boateng K, Baddoo H. Rupture of sinus of Valsalva aneurysm: case report. East Afr Med J. 2000;77:627e628. 9. Sharkey A, Cooper G. An unusual and life-threatening cause of epigastric pain presenting to the general surgical team: a case report. Ann R Coll Surg Engl. 2010;92:W26e27. 10. Baumgartner F, Velez M, Zulim R, State D. Abdominal pain, colomegaly, and jaundice from heart failure related to mitral stenosis. J Natl Med Assoc. 1989;81:1091e1093. 11. Cui W, van Bergen AH, Patel D, Javois AJ, Roberson DA. Transcatheter closure of ruptured sinus of Valsalva aneurysm and secundum atrial septal defect with limited inferior rim. Echocardiography. 2008;25:208e213.
Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002
MODEL
Available online at www.sciencedirect.com
ScienceDirect Journal of Acute Medicine xx (2015) 1e3 www.e-jacme.com
Case Report
Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm Yi-Ming Weng a,b,e, Tai-Yi Hsu c,e, Cheng-Wei Chan b,d,*, Jih-Chang Chen a,b a
Department of Emergency Medicine, Chang Gung Memorial Hospital, Taoyuan, Taiwan, ROC b College of Medicine, Chang Gung University, Taoyuan, Taiwan, ROC c Department of Emergency Medicine, China Medical University Hospital, Taichung, Taiwan, ROC d Department of Emergency Medicine, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Keelung, Taiwan, ROC Received 18 April 2014; revised 12 May 2015; accepted 18 June 2015 Available online ▪ ▪ ▪
Abstract Cardiogenic shock is defined by tissue hypoperfusion due to heart failure. The manifestations of acute right ventricular (RV) failure include a distended jugular vein and hypotension, but not rales. The causes of isolated acute RV failure include RV infarction, pulmonary embolism, cardiac tamponade, congenital disease, and valvular heart disease. Rupture of a sinus of Valsalva aneurysm (SVA) is rarely seen in emergency departments (EDs). We report a 46 year-old male who suffered from SVA rupture triggering acute RV failure and cardiogenic shock. Copyright © 2015, Taiwan Society of Emergency Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: Cardiogenic shock; Acute right ventricular failure; Rupture of a sinus of Valsalva aneurysm
1. Introduction
2. Case report
Cardiogenic shock is defined by tissue hypoperfusion due to heart failure. Acute right ventricular (RV) failure triggers cardiogenic shock by decreasing both preload and septal shift. Patients with acute RV failure may present with distended jugular veins and hypotension, but not rales. The causes of isolated acute RV failure include RV infarction, pulmonary embolism, cardiac tamponade, congenital disease, and valvular heart disease. Rupture of a sinus of Valsalva aneurysm (SVA) is rarely seen in emergency department. We report a case of SVA rupture triggering acute RV failure and cardiogenic shock. After emergency surgical repair, our patient was discharged without sequelae.
A 46-year-old male presented with acute abdominal pain 3 hours in duration. He had a medical history of hypertension and hyperlipidemia, which were being controlled by medication. He complained of moderate pain in the epigastric area and described it as dull in character. He denied any radiation pain. We found no chest/abdominal trauma or any aggravating factor. His symptoms included cold sweating, chest tightness, shortness of breath, nausea, and vomiting. No bloody vomitus or tarry stool was mentioned. At the emergency department, the patient appeared acutely ill and pale in color. Initial vital signs included a body temperature of 34 C, a pulse rate of 65 beats/min, and a blood pressure of 81/39 mmHg. Physical examination was unremarkable, except for a distended external jugular vein in the sitting position, a loud continuous diastolic heart murmur over the lower sternal border, and cold and clammy skin. Breathing sounds were clear and symmetrical in both lungs. The abdomen was soft, with mild tenderness over the peri-umbilical area but no peritoneal signs. Bedside ultrasonography was negative for ascites and
* Corresponding author. Department of Emergency Medicine, Chang Gung Memorial Hospital, Keelung, Number 222, Maijin Road, Anle District, Keelung City 204, Taiwan, ROC. E-mail address: [email protected] (C.-W. Chan). e These authors contributed equally to this manuscript.
http://dx.doi.org/10.1016/j.jacme.2015.06.002 2211-5587/Copyright © 2015, Taiwan Society of Emergency Medicine. Published by Elsevier Taiwan LLC. All rights reserved. Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002
+ 2
MODEL
Y.-M. Weng et al. / Journal of Acute Medicine xx (2015) 1e3
pericardiac fluid. Electrocardiography revealed junctional rhythm without an ST-T change. The plain film showed a poorly expanded lung and cardiomegaly (Figure 1). Our initial suspicion was acute RV failure. To rule out pulmonary embolism, mechanical obstruction of the RV outflow, and any other anomaly, we ordered a computed tomographic (CT) scan. This revealed contrast pooling over the RV and superior and inferior vena cava. Retrograde flow into the RV via an aortic root was evident (Figures 2 and 3). We found no pulmonary embolism, mechanical lesion, or anomaly. Further two-dimensional transthoracic echocardiography revealed an SVA with a left-to-right
Figure 3. Shunt from the aortic root to the right ventricle (arrow), which is possible evidence of sinus of Valsalva aneurysm rupture.
shunt from the right coronary sinus into the RV. Rupture of an SVA was suspected. An emergency operation using a patch repair was performed immediately. Our patient made an uneventful recovery and was discharged 9 days after the surgery. 3. Discussion Figure 1. Chest x-ray revealing cardiomegaly.
Figure 2. Contrast regurgitation to the superior and inferior vena cava, evidence of poor heart pump function.
Our case presentation emphasizes that differential diagnosis of shock is critical for appropriate management. First, the finding of cold and clammy skin rendered a diagnosis of distributive shock unlikely. Also, neither neurogenic nor anaphylactic shock was considered, based on the present illness and medical history. Second, septic shock was ruled out because of the rapidly progressive course, lack of fever, and focus of infection. Third, rather than hypovolemic shock, we classified our case as cardiogenic shock because of the presence of a distended jugular vein without obvious volume loss. Furthermore, clear breathing sounds upon chest auscultation indicated an isolated RV failure. Although a retrograde flow was suspected following CT scan examination, we ordered two-dimensional transthoracic echocardiography because we considered this tool appropriate to aid diagnosis. Additionally, the loud diastolic heart murmur over the lower sternal border suggested valvular heart disease or an intracardiac shunt. Our report confirms previous suggestions that transthoracic echocardiography1e3 is a useful and reliable tool when employed to confirm SVA rupture. Other useful imaging modalities include transesophageal echocardiography,2e4 CT angiography,5 and magnetic resonance imaging. SVA is a dilatation caused by separation of the aortic media and annulus fibrosus.6 Acute rupture of the SVA is fatal. Most
Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002
+
MODEL
Y.-M. Weng et al. / Journal of Acute Medicine xx (2015) 1e3
ruptured SVAs occur in patients <40 years of age1,2,4,6 and predominate in males.4,6 The right coronary sinus2,6 is affected most frequently, followed by the noncoronary sinus.7 Ruptures of an SVA into the right ventricle are most common.2,6 When an SVA ruptures, the patient may complain of chest pain,7 dyspnea,1,6 palpitations,1 tinnitus,1 orthopnea,6 upper abdominal discomfort, and edema of the lower extremities.6,8 Abdominal pain as the initial presentation of SVA rupture is rare; only one similar case has been reported previously.9 The cause of abdominal pain may be nonocclusive ischemia of the small bowel attributed to hypoperfusion caused, in turn, by acute heart-pump dysfunction.10 Other findings on physical examination include tachycardia, wide variations in pulse pressure, bounding carotid and peripheral pulses, pulmonary crackles,4 a prominent continuous precordial murmur8 with a thrill,4,8 hepatomegaly,6 upper abdominal tenderness, severe kidney insufficiency,6 and congestive hepatic insufficiency.6,8 The standard management for a ruptured SVA is early surgical intervention.1,2,6 Nonsurgical transcatheter-mediated closure of the rupture is another option.11 This report reminds clinicians that rupture of an SVA is a rare etiology of acute RV failure. An understanding of the pathophysiology of the disease entity helps emergency department physicians recognize specific manifestations of the condition. Prompt specialist attention with early intervention saves lives. Conflicts of interest All authors have no conflicts of interest to declare.
3
References 1. Guenther F, von Zur Muhlen C, Lohrmann J, Bode C, Geibel A. Rupture of an aneurysm of the noncoronary sinus of Valsalva into the right atrium. Eur J Echocardiogr. 2008;9:186e187. 2. Vincelj J, Starcevic B, Sokol I, Sutlic Z. Rupture of a right sinus of valsalva aneurysm into the right ventricle during vaginal delivery: a case report. Echocardiography. 2005;22:844e846. 3. Bouvier E, Porte JM, Laperche T, et al. Rupture of a sinus of Valsalva aneurysm in the left atrium. Arch Mal Coeur Vaiss. 2001;94:1409e1412. 4. Sanchez ME, García-Palmieri MR, Quintana CS, Kareh J. Heart failure in rupture of a sinus of valsalva aneurysm. Am J Med Sci. 2006;331:100e102. 5. Kantarci M, Doganay S, Gundogdu F, Unlu Y. A case with noncoronary sinus of Valsalva aneurysm: multidetector computed tomography findings. Heart Surg Forum. 2008;11:E372e374. 6. Galicia-Tornell MM, Marín-Solís B, Mercado-Astorga O, EspinozaAnguiano S, Martínez-Martínez M, Villalpando-Mendoza E. Sinus of Valsalva aneurysm with rupture. Case report and literature review. Cir Cir. 2009;77:441e445. 7. Turgeon AF, Dagenais F, Poirier P, Bauset R, Mathieu P. Myocardial infarction in a young woman secondary to a rupture of a noncoronary sinus of Valsalva aneurysm without coronary artery disease. Can J Cardiol. 2003;19:723e724. 8. Amoah AG, Frimpong-Boateng K, Baddoo H. Rupture of sinus of Valsalva aneurysm: case report. East Afr Med J. 2000;77:627e628. 9. Sharkey A, Cooper G. An unusual and life-threatening cause of epigastric pain presenting to the general surgical team: a case report. Ann R Coll Surg Engl. 2010;92:W26e27. 10. Baumgartner F, Velez M, Zulim R, State D. Abdominal pain, colomegaly, and jaundice from heart failure related to mitral stenosis. J Natl Med Assoc. 1989;81:1091e1093. 11. Cui W, van Bergen AH, Patel D, Javois AJ, Roberson DA. Transcatheter closure of ruptured sinus of Valsalva aneurysm and secundum atrial septal defect with limited inferior rim. Echocardiography. 2008;25:208e213.
Please cite this article in press as: Weng Y-M, et al., Rare cause of abdominal pain and shock: Rupture of a sinus of Valsalva aneurysm, Journal of Acute Medicine (2015), http://dx.doi.org/10.1016/j.jacme.2015.06.002