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 Table of Contents  
INTERESTING CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 2  |  Page : 101-103

Pericardial tamponade due to main pulmonary artery dissection in a young patient with eisenmenger's syndrome


Department of Cardiology, Hero DMC Heart Institute, Dayanand Medical College, Ludhiana, Punjab, India

Date of Web Publication29-Aug-2019

Correspondence Address:
Rohit Tandon
Hero DMC Heart Institute, Dayanand Medical College, Ludhiana, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_8_19

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  Abstract 

Spontaneous pulmonary artery dissection is a rare catastrophic emergency. Its clinical course and natural history is not well studied due to paucity of available cases in the literature, so optimal management guidelines are lacking. Easy availability of bedside echocardiography has made early diagnosis of these near-miss cases possible. We describe a case of a 36-year-old male, a known case of Eisenmenger's syndrome (secondary to unoperated large ventricular septal defect), who presented to us in cardiogenic shock due to spontaneous main pulmonary artery dissection readily diagnosed and confirmed by bedside three-dimensional echocardiography.

Keywords: Echocardiography, Eisenmenger's syndrome, pulmonary artery dissection


How to cite this article:
Tandon R, Gupta H, Wander GS, Goyal A. Pericardial tamponade due to main pulmonary artery dissection in a young patient with eisenmenger's syndrome. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:101-3

How to cite this URL:
Tandon R, Gupta H, Wander GS, Goyal A. Pericardial tamponade due to main pulmonary artery dissection in a young patient with eisenmenger's syndrome. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2019 Sep 15];3:101-3. Available from: http://www.jiaecho.org/text.asp?2019/3/2/101/265762


  Introduction Top


Spontaneous pulmonary artery dissection is a rare catastrophic emergency. Its clinical course and natural history is not well studied due to paucity of available cases in the literature, so optimal management guidelines are lacking. Easy availability of bedside echocardiography has made early diagnosis of these near-miss cases possible.


  Clinical Presentation Top


A 36-year-old male, a known case of unoperated ventricular septal defect with Eisenmenger's physiology for the past 14 years, presented to the emergency room in cardiogenic shock after complaining of sudden-onset retrosternal chest pain followed by shortness of breath 2 h before admission. On examination, the patient was conscious, well oriented, systolic blood pressure was 80 mmHg, pulse rate 124 bpm, and SPO2 - 90% with engorged neck veins. Electrocardiogram showed sinus rhythm, right axis deviation with right ventricular pressure overload pattern in anterior leads. Transthoracic echocardiogram showed large pericardial effusion with a large, floating thrombus in pericardial fluid. 450 ml hemorrhagic fluid was immediately aspirated, which led to improvement in patient's hemodynamic status. Postpericardiocentesis echocardiogram showed moderately dilated right atrium and right ventricle with globally reduced right ventricular functions (tricuspid annular plane systolic excursion =12 mm and right ventricular fractional area change = 22%); grossly dilated main pulmonary artery (size: 55 mm) [Figure 1] with an oblique mobile flap originating just below the pulmonary valve dividing the main pulmonary artery into true and false lumens with exit point confirmed on color flow. Computed tomography (CT) scan was deferred considering the patient's labile hemodynamics [Figure 2]. Bedside three-dimensional (3D) echocardiography could easily define the flap margins and delineate false and true lumens adequately, confirming that it was not an echocardiography artifact and excluded any thrombus in the pulmonary artery [Videos 1 and 2], thereby confirming the diagnosis of acute rupture of pulmonary artery. Cardiothoracic and vascular surgery team decided for early surgery, but the patient had a sudden hemodynamic collapse and could not be resuscitated. Keeping in mind the rarity of this case, necropsy was planned, but his relatives refused to give consent.
Figure 1: Three-dimensional multiplanar reconstruction view clearly differentiates between true and false lumen excluding presence of thrombus

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Figure 2: Two-dimensional left parasternal upper short axis view showing an exit point in dissection flap through which false and true lumens are communicating by color Doppler

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  Discussion Top


Spontaneous dissection of the pulmonary artery is extremely rare.[1],[2],[3] It manifests as cardiogenic shock or sudden death; till now, only 59 cases of pulmonary artery dissection have been reported (including autopsy reports) [Table 1].
Table 1: Pulmonary artery dissection

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In these reports, there was equal sex distribution, and peak age of presentation was in third–sixth decades with equal sex distribution. Most common presenting symptoms were chest pain and dyspnea.[4],[5],[6],[7]

Pathophysiologically, the effects of chronic pulmonary hypertension as seen in pulmonary vasculature manifests are medial degeneration, elastic fiber fragmentation, and generalized dilatation of the pulmonary arterial tree,[1],[4],[7] which results in weakened dilated thin wall vulnerable to intimal tear by shear stress from raised intrapulmonary lumen pressure. In approximately 80% of the patients, the site of dissection is in the main pulmonary artery, usually without the involvement of its branches. Occasionally, an isolated dissection of the right or left pulmonary artery branches has also been reported. Most of the above-described cases had dissection usually at the site of aneurysm or marked dilatation (fragile points), except in one case where it occurred by pulmonary artery catheter. Dissection occurs in accordance with the Laplace's law, which states that wall tension is dependent on the pressure inside the vessel and vessel radius. This holds true for the parietal pulmonary wall which is maximally dilated in chronic pulmonary arterial hypertension (PAH) patients, and a break in intima occurs when there is a sudden rise in intrapulmonary pressures following undue physical exertion (as it occurred in our patient who had been cycling before collapse).

Modern imaging tools (echocardiography/CT/cardiac magnetic resonance imaging) are able to diagnose both the flap and adequately quantify its extent and exit to entry points. Chest radiography is not specific, but it may definitely alert the physicians regarding the presence of chronic PAH. Except for echocardiography, all modalities are not universally available in emergency setting. In this patient, echocardiography showed a linear mobile flap-like structure within the main pulmonary artery, which was easily confirmed by 3D echocardiography. We decided for surgical repair in this case, as it has been performed in three cases previously with a successful result; however, this patient succumbed to this catastrophic event. We believe that it occurred as his dissection was complete involving the entire thickness of the main pulmonary artery as in these cases the false lumen expands rapidly in size rather than developing a reentry site (as is seen in aortic dissection), resulting in pericardial tamponade, major bleed, and subsequently death.[4],[8],[9],[10],[11],[12],[13],[14]


  Conclusion Top


Spontaneous pulmonary artery dissection/rupture is a rare mechanical complication of chronic PAH having a dismal outcome. Screening with the help of available imaging modalities for risk factors of dissection, i.e., pulmonary artery dilatation and pulmonary aneurysm, may help prevent a catastrophic complication and expeditiously consider transplantation for patients at risk.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Mohammad K, Sahlol M, Egiebor O, Sadikot RT. Idiopathic pulmonary artery dissection: A case report. J Med Case Rep 2009;3:7426.  Back to cited text no. 1
    
2.
Walley VM, Virmani R, Silver MD. Pulmonary arterial dissections and ruptures: To be considered in patients with pulmonary arterial hypertension presenting with cardiogenic shock or sudden death. Pathology 1990;22:1-4.  Back to cited text no. 2
    
3.
Andrews R, Colloby P, Hubner PJ. Pulmonary artery dissection in a patient with idiopathic dilatation of the pulmonary artery: A rare cause of sudden cardiac death. Br Heart J 1993;69:268-9.  Back to cited text no. 3
    
4.
Lopez-Candales A, Kleiger RE, Aleman-Gomez J, Kouchoukos NT, Botney MD. Pulmonary artery aneurysm: Review and case report. Clin Cardiol 1995;18:738-40.  Back to cited text no. 4
    
5.
Steurer J, Jenni R, Medici TC, Vollrath T, Hess OM, Siegenthaler W, et al. Dissecting aneurysm of the pulmonary artery with pulmonary hypertension. Am Rev Respir Dis 1990;142:1219-21.  Back to cited text no. 5
    
6.
Sakamaki Y, Minami M, Ohta M, Takahashi T, Matsumiya G, Miyoshi S. Pulmonary artery dissection complicating lung transplantation for primary pulmonary hypertension. Ann Thorac Surg 2006;81:360-2.  Back to cited text no. 6
    
7.
Wuyts WA, Herijgers P, Budts W, De Wever W, Delcroix M. Extensive dissection of the pulmonary artery treated with combined heart-lung transplantation. J Thorac Cardiovasc Surg 2006;132:205-6.  Back to cited text no. 7
    
8.
Wunderbaldinger P, Bernhard C, Uffmann M, Kürkciyan I, Senbaklavaci O, Herold CJ, et al. Acute pulmonary trunk dissection in a patient with primary pulmonary hypertension. J Comput Assist Tomogr 2000;24:92-5.  Back to cited text no. 8
    
9.
Inayama Y, Nakatani Y, Kitamura H. Pulmonary artery dissection in patients without underlying pulmonary hypertension. Histopathology 2001;38:435-42.  Back to cited text no. 9
    
10.
Senbaklavaci O, Kaneko Y, Bartunek A, Brunner C, Kurkciyan E, Wunderbaldinger P, et al. Rupture and dissection in pulmonary artery aneurysms: Incidence, cause, and treatment – Review and case report. J Thorac Cardiovasc Surg 2001;121:1006-8.  Back to cited text no. 10
    
11.
Song EK, Kolecki P. A case of pulmonary artery dissection diagnosed in the emergency department. J Emerg Med 2002;23:155-9.  Back to cited text no. 11
    
12.
Khatchatourian G, Vala D. Images in cardiovascular medicine. Acute type I aortic dissection with concomitant pulmonary artery dissection. Circulation 2005;112:e313-4.  Back to cited text no. 12
    
13.
Hsu HH, Tzao C, Tsai CS, Sun GH, Chen CY. Acute concomitant pulmonary artery and aortic dissection with rupture. Int J Cardiovasc Imaging 2007;23:411-4.  Back to cited text no. 13
    
14.
Navas Lobato MA, Martín Reyes R, Lurueña Lobo P, Maté Benito I, Guzmán Hernández G, Martí de Gracia M, et al. Pulmonary artery dissection and conservative medical management. Int J Cardiol 2007;119:e25-6.  Back to cited text no. 14
    


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