Constrictive Pericarditis


Article Author:
Niraj Yadav


Article Editor:
Momin Siddique


Editors In Chief:
Tonya Dawson


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
11/11/2018 9:19:46 AM

Introduction

The pericardium is the fibroelastic sac that covers the heart. Besides acting as a protective barrier, it also affects cardiac hemodynamics. Constrictive pericarditis is a condition in which granulation tissue formation in the pericardium results in loss of pericardial elasticity leading to restriction in the ventricular filling. It is usually a chronic condition however subacute, transient and occult variants have been described.[1][2]

Etiology

Worldwide, the leading cause of constrictive pericarditis is tuberculosis, and the incidence is about 50% of patients with tuberculous pericardial effusion despite antitubercular therapy. In developed nations, the leading cause of this condition is idiopathic or post-viral infection with incidence being 40% to 60% of total cases. It is also a known complication of any cardiac surgery and is a fairly common complication of mediastinal radiation therapy with an incidence ranging from 2% to 30% in patients treated with radiation. It has also been associated with connective tissue disorders such as rheumatoid arthritis and systemic lupus erythematosus (SLE). The diagnosis of constrictive pericarditis may be fairly simple however most of the time it is much more difficult to establish a cause. In many cases, a cause cannot be identified and is attributed to be an asymptomatic episode of viral pericarditis. [3][4][5] 

Epidemiology

Approximately 9% of patients with acute pericarditis develop constrictive physiology. In the developing world, infectious etiologies  (tuberculosis) remain the most common cause. The condition is rare in adults and very rare children. It is more common in those who have undergone cardiac surgery. There is a 3:1 male predominance. There is no known racial predilection.[6][7]

Pathophysiology

Pathophysiology of chronic constrictive pericarditis involves obliteration of pericardial cavity by granulation tissue during healing of an acute episode of fibrinous or serofibrinous pericarditis or resorption of chronic pericardial effusion. The granulation tissue gradually contracts over time and encases the heart and may get calcified. This rigid thickened pericardium limits the ventricular filling as the elastic limit of the diseased pericardium is much lesser than that of a normal pericardium. Ventricular filling in early diastole is not affected and is only impeded when the elastic limit of the pericardium is reached in contrast to cardiac tamponade where the ventricular filling is impeded throughout the diastole. This results in decreased end diastolic volume and decreased stroke volume and cardiac output. The thickened and scarred pericardium prevents the normal inspiratory decrease in intrathoracic pressure from being transferred to the heart chambers.

There are dissociation intrathoracic and intracardiac pressures. This leads to decreased venous return with inspiration as pulmonary venous pressure decreases. However, the left atrium pressure does not, and pulmonary veins to left atrial (LA) flow decreases on inspiration. This intrathoracic and intracardiac pressure dissociation is a distinguishing feature from cardiac tamponade as in cardiac tamponade the changes in intrathoracic pressure are still conducted to the heart and there is an increase in systemic venous return with inspiration.

In both disorders, there is equalization of the right atrial (RA), right ventricular (RV), left ventricular (LV), and pulmonary wedge pressure however cardiac tamponade the pressure decreases with inspiration whereas in constrictive pericarditis the RA pressure remains constant while the pulmonary wedge pressure decreases.[8][9][10]

History and Physical

Patients will often present with chronic symptoms. Their symptoms may be related to volume overload like weight gain and swelling or may be related to decreased cardiac output like progressive fatigue and dyspnea on exertion. They may also complain of increasing abdominal girth or abdominal discomfort. Abdominal complaints are secondary to either ascites or congestive hepatomegaly.

On physical examination, the jugular venous pressure (JVP) is usually elevated, however, may be normal in early constrictive pericarditis. JVP does not decrease with inspiration, and this is known as Kussmaul's sign. Kussmaul's sign is also present in tricuspid valve disease, and right-sided heart failure.Pulsus paradoxus (more than 10 mm Hg drop in systolic blood pressure during inspiration) can be seen however this is more common in patients with cardiac tamponade. An accentuated heart sound heard earlier than third heart sound called pericardial knock can be heard in almost half of the patients. Abdominal examination may reveal ascites or hepatomegaly. Other signs of chronic illness like muscle wasting may be present based upon the etiology. Peripheral edema may be present as well.

Evaluation

ECHO Cardiography

The American College of Cardiology and the European Society of Cardiology guidelines recommend the use of echocardiography for diagnosis of constrictive pericarditis and any other pericardial disease.  Two-dimensional echocardiography may show increased pericardial thickness without or without calcification. Two-dimensional echocardiography may also reveal dilatation of the inferior vena cava with the absence of inspiratory collapse. A sharp halt in diastolic filling may be seen along with the abrupt transient movement of intraventricular septum towards the right side which is also known as septal bounce.[2][3][11]

The M mode is very important to rule out constrictive pericarditis. The following features are frequently seen in constrictive pericarditis and An absence these features on M mode makes the diagnosis of constrictive pericarditis very unlikely.

  • Posterior motion of ventricular septum on early diastole in inspiration 
  • Absence of increase in systemic venous return with inspiration 
  • Premature opening of the pulmonic valve due to higher right ventricular diastolic pressure compared to pulmonary arterial pressure

Doppler ECHO is used to evaluate hemodynamics of the disease and may reveal the following:

  • Abnormal passive filling of the ventricles in early diastole 
  • Increase in diastolic flow velocity across the tricuspid valve during inspiration and decrease during expiration 
  • Exaggerated reduction in flow velocity in the pulmonary veins and across the mitral valve in inspiration and a leftward shift of ventricular septum 

Electrocardiogram 

There are no specific signs of constrictive pericarditis on ECG which may reveal nonspecific ST changes and low voltage. Advanced and long-standing cases may show atrial fibrillation secondary to elevated atrial pressures.

CT scan and cardiac MRI are also frequently done especially before surgical management of constrictive pericarditis. These can reveal thickened pericardium and presence of calcifications. CT scans can detect calcifications better compared to cardiac MRI. Cardiac MRI is better to differentiate small effusions from pericardial thickening. 

Myocardial fibrosis or atrophy seen on CT or MRI is associated with a poor surgical outcome.

Occasionally patients undergo right heart catheterization for hemodynamic studies which may reveal increased right atrial pressure, increased RV end-diastolic pressure, prominent x and y descent on venous and atrial pressure tracings and greater inspiratory fall in pulmonary capillary wedge pressure compared to the left ventricular diastolic pressure.

Treatment / Management

Pericardiectomy is the only definitive management of chronic constrictive pericarditis and effort should be made to remove as much of the pericardium as possible. Extensive penetration of the myocardium by fibrosis and calcification is associated with poor outcome. Operative mortality ranges from 55% to 10%. It should be considered very cautiously in patients with the mild disease with few symptoms or patients with advanced disease and other comorbidities due to the high mortality of the procedure. Diuretics can be used to reduce edema or elevated venous pressures before the surgery or for palliative control of symptoms in patients who are not surgical candidates.[1][12][13]

A subset of patients may have a spontaneous resolution or may respond to medical management, and they are said to have transient constrictive pericarditis. Patients with newly diagnosed constrictive pericarditis who are hemodynamically stable and do not have stigmas of chronic constriction may be treated with anti-inflammatory agents for up to three months with close monitoring. If these patients develop signs of chronic constriction and hemodynamic instability they should undergo prompt surgical treatment.

Complications

  • Pulmonary hypertension
  • Hepatomegaly
  • Renal failure
  • Metabolic acidosis
  • Hypoxia
  • Shock
  • Death

Enhancing Healthcare Team Outcomes

In the US, constrictive pericarditis is not common, and long-term data are scarce. Anecdotal reports suggest that when the diagnosis is made early, the outcomes are good. But if the disorder is misdiagnosed or untreated, mortality rates in excess of 90% are common. For patients who undergo pericardiectomy, the 10-year survival is about 50%. Medical therapy alone leads to a poor life expectancy. The long-term survival after constrictive pericarditis depends on the cause. The worst outcomes are in patients with radiation-induced constrictive pericarditis and the best outcomes are seen in patients following open heart surgery. Negative prognostic factors include advanced age, renal malfunction, low ejection fraction and elevated pulmonary artery pressures. Most patients who are untreated quickly develop multiorgan dysfunction, hypoxia and metabolic acidosis. Patients should be promptly referred to a tertiary care center which specializes in the management of constrictive pericarditis when treatment is not available at the presenting hospital.[14][15][16] (Level V)


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Constrictive Pericarditis - Questions

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A 17-year-old male immigrant presents with a 6-month history of dyspnea on exertion. He complains of swollen legs. He had tuberculosis as a child that was treated with four drugs. ECG is normal. CT of the chest shows pericardial calcifications. Which of the following would not be expected on an exam?



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The differential between restrictive cardiomyopathy and constrictive pericarditis would be least aided by which of the following?



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What is the most common cause of constrictive pericarditis worldwide?



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What id the most common primary cause of constrictive pericarditis in the United States?



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Which of following is not a complication of constrictive pericarditis?



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What is the most common cause of constrictive pericarditis worldwide?



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A 55-year old male presents with dyspnea, fatigue, and orthopnea of 4 weeks duration. Over the past two weeks, he has developed swelling of his abdomen and ankles. His past medical history reveals he had radiation therapy to the chest because of a mediastinal mass. Physical exam reveals an ill-looking male with cachexia and jugular venous distension. High-resolution CT scan reveals a thickened pericardium (greater than 6 mm) with diffuse calcification. Which of the following is not true about this patient's cardiac pathology?



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In a patient in whom you suspect constrictive pericarditis, which of the following tests may offer you a diagnosis?



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A 65-year old presents with a long history of progressive dyspnea, fatigue, and orthopnea. Last month he also noticed swelling of his extremities and vague abdominal discomfort. On physical, the patient appears ill and cachectic. He may have constrictive pericarditis. On physical exam, what one universal feature is almost always present in this disorder?



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A 65-year old presents with a long history of progressive dyspnea, fatigue, and orthopnea. Last month, he also noticed swelling of his extremities and vague abdominal discomfort. On physical, the patient appears ill and cachectic. His jugular venous pressure is elevated, but the apical impulse cannot be felt. On auscultation, if there is a diastolic knock, what diagnosis should be considered?



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A 65-year-old lady who underwent radiation therapy for breast cancer presented with complaints of progressive of swelling of her legs and gradually worsening shortness of breath over the past one year. Physical examination showed elevated jugular venous pressure and pulsus paradoxus. What is the definitive management for this condition



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A patient post coronary artery bypass graft (CABG) surgery presents with shortness of breath, fatigue, peripheral edema, and ascites. ECG reveals non-specific ST changes. The chest x-ray shows cardiomegaly with flecks of calcium around the pericardium. What is the best treatment for this patient?



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Constrictive Pericarditis - References

References

Maisch B, [Management of pericarditis and pericardial effusion, constrictive and effusive-constrictive pericarditis]. Herz. 2018 Oct 12     [PubMed]
Mukai-Yatagai N,Haruki N,Kinugasa Y,Ohta Y,Ishibashi-Ueda H,Akasaka T,Kato M,Ogawa T,Yamamoto K, Assessment of myocardial fibrosis using T1-mapping and extracellular volume measurement on cardiac magnetic resonance imaging for the diagnosis of radiation-induced cardiomyopathy. Journal of cardiology cases. 2018 Oct     [PubMed]
Pessinaba S,Sonhaye L,Agbétiafa M,Wossinu Quacoe M,Aloumon M,Yayehd K,Amadou L,d'Alméida R,Damorou F, [The chronic constrictive pericarditis, a real calcified gangue realizing a mid-ventricular bottleneck: A case report]. Annales de cardiologie et d'angeiologie. 2018 Aug 24     [PubMed]
Wang FF,Hsu J,Jia FW,Lin X,Miao Q,Chen W,Fang LG, Left ventricular strain is associated with acute postoperative refractory hypotension in patients with constrictive pericarditis and preserved ejection fraction. Journal of thoracic disease. 2018 Jul     [PubMed]
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Murashita T,Schaff HV,Daly RC,Oh JK,Dearani JA,Stulak JM,King KS,Greason KL, Experience With Pericardiectomy for Constrictive Pericarditis Over Eight Decades. The Annals of thoracic surgery. 2017 Sep     [PubMed]
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Imazio M, [Ten questions about constrictive pericarditis]. Giornale italiano di cardiologia (2006). 2018 Jul-Aug     [PubMed]
Rehman KA,Betancor J,Xu B,Kumar A,Rivas CG,Sato K,Wong LP,Asher CR,Klein AL, Uremic pericarditis, pericardial effusion, and constrictive pericarditis in end-stage renal disease: Insights and pathophysiology. Clinical cardiology. 2017 Oct     [PubMed]
Fardman A,Charron P,Imazio M,Adler Y, European Guidelines on Pericardial Diseases: a Focused Review of Novel Aspects. Current cardiology reports. 2016 May     [PubMed]
Armanious MA,Mohammadi H,Khodor S,Oliver DE,Johnstone PA,Fradley MG, Cardiovascular effects of radiation therapy. Current problems in cancer. 2018 Jul     [PubMed]
Nachum E,Sternik L,Kassif Y,Raanani E,Hay I,Shalabi A,Buber J, Surgical Pericardiectomy for Constrictive Pericarditis: A Single Tertiary Center Experience. The Thoracic and cardiovascular surgeon. 2018 May 27     [PubMed]
Nozohoor S,Johansson M,Koul B,Cunha-Goncalves D, Radical pericardiectomy for chronic constrictive pericarditis. Journal of cardiac surgery. 2018 Jun     [PubMed]

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