b79945 heart dilated atrial appendages iatrogenic post-surgical complication of pericardiectomy CT heart giant atrial appendages iatrogenic post-surgical aneurysmal CT 30F post pericardiectomy 20 years prior constrictive pericarditis

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Giant Bilateral Atrial Appendages Following Remote Pericardiectomy
A 33-year-old woman from Africa, who underwent a pericardiectomy 30 years ago for presumed constrictive pericarditis, presents with chest pain and a striking imaging appearance of giant bilateral atrial appendages. Both the right and left atrial appendages are markedly dilated, thin-walled, and symmetrically expanded — most prominently along the right atrial inferior border and the left atrial appendage apex. .
The constellation of findings suggests iatrogenic post-surgical aneurysmal remodeling of the atrial appendages secondary to chronic loss of pericardial restraint. Over time, the unopposed transmural pressures acting on the compliant appendage walls, particularly in the absence of the pericardial envelope, have led to progressive dilatation. The process reflects a unique late complication of pericardiectomy, distinct from congenital atrial aneurysm or secondary dilation from pressure overload.
This case illustrates the delicate mechanical relationship between the heart and its pericardial covering — when the restraining “corset” of the pericardium is removed, the atrial appendages, embryologically predisposed to distensibility, may evolve into giant aneurysmal chambers decades later.
Ashley Davidoff MD, TheCommonVein.com (b79945)

Ashley Davidoff MD

30F post pericardiectomy 20 years prior constrictive pericarditis

2. Findings


Dilated Atrial Appendages

 

Constrictive Pericarditis MRI

Finding Definition
Dilated Atrial Appendages Definition

  • Dilated atrial appendages, specifically the left atrial appendage, can be identified through various imaging modalities, including echocardiography and CT scans.
  • This dilation can occur in isolation or in conjunction with other cardiac conditions.
  • Echocardiography, particularly transesophageal echocardiography (TEE), is a primary tool for assessing the structure and function of the left atrial appendage.
  • In the context of constrictive pericarditis, atrial enlargement, including the right atrium, is a recognized finding, often associated with dilated inferior vena cava and hepatic veins, indicative of right-sided heart failure.

Comment

  • While atrial enlargement is common in constrictive pericarditis, the term “dilated atrial appendages” might be more specifically associated with conditions like atrial fibrillation or other primary atrial pathologies rather than being a universal finding in constrictive pericarditis itself.
  • [First Author], [Journal Name], [Year]
   
   
   
   
   
   
   
   
   
   

3. Diagnosis


The clinical perspective focuses on iatrogenic post-surgical aneurysms, specifically in the context of pericardiectomy.

  • Giant atrial appendages
Clinical Perspective Details
Definition
  • Iatrogenic post-surgical complications of pericardiectomy, in the context of aneurysmal changes, refer to the development of abnormal dilations or outpouchings in the cardiac structures or great vessels that occur as a consequence of surgical intervention on the pericardium, specifically pericardiectomy.
  • These complications are unintended and arise from the surgical process itself or its sequelae.
Cause
  • Disruption of Pericardial Integrity: The surgical removal of the pericardium can alter the mechanical support and integrity of the cardiac structures and adjacent great vessels.
  • Inflammatory Response and Scarring: Post-surgical inflammation and fibrotic scarring can lead to altered tissue architecture and mechanical properties, potentially contributing to weakened areas that may dilate.
  • Hemodynamic Stress: Alterations in cardiac mechanics and pressures following pericardiectomy can subject weakened areas to increased hemodynamic stress, promoting aneurysmal formation.
  • Direct Injury: Direct surgical injury to the wall of a cardiac chamber or a major vessel during pericardiectomy could create a predisposition to later aneurysm formation.
Pathophysiology
  • The surgical removal of the pericardium eliminates a protective and supportive layer, potentially rendering the underlying epicardium and adventitia of adjacent vessels more susceptible to mechanical stress and damage.
  • Chronic inflammation or aberrant healing post-surgery can lead to localized weakening of the myocardial or vascular wall.
  • This weakness, subjected to pulsatile cardiac forces, can result in progressive dilation, forming an aneurysm.
  • The absence of pericardial constraint may allow for greater outward bulging at points of inherent or surgically induced weakness.
Structural Result
  • Formation of an abnormal, localized dilation or outpouching of a cardiac chamber, a great vessel, or a remnant of the pericardial space.
  • This can manifest as a true aneurysm or a pseudoaneurysm.
  • These aneurysms can vary in size and location, often occurring near the site of surgical manipulation.
Functional Impact
  • Impair Cardiac Filling: May impede normal diastolic filling, leading to symptoms like dyspnea and edema.
  • Cause Compressive Effects: Large aneurysms may exert mass effect on adjacent structures.
  • Increase Risk of Thromboembolism: Stasis of blood within an aneurysmal sac can predispose to thrombus formation.
  • Risk of Rupture: Aneurysms carry an inherent risk of rupture, potentially leading to hemorrhage.
Imaging
  • Echocardiography: Visualizes and characterizes the aneurysm, assesses size, location, and impact on cardiac function.
  • Cardiac Magnetic Resonance Imaging (CMR): Provides detailed anatomical information, assesses mural thrombus, and evaluates wall integrity.
  • Computed Tomography Angiography (CTA): Delineates vascular involvement, visualizes lumen, neck, and relationship to adjacent structures.
Labs
  • Complete Blood Count (CBC): May reveal signs of infection or inflammation.
  • Inflammatory Markers (ESR, CRP): Elevated levels may suggest an ongoing inflammatory process.
  • Biomarkers (BNP, Troponin): May be elevated if there is associated myocardial dysfunction or injury.
  • Renal and Liver Function Tests: To assess for multi-organ dysfunction.
Treatment
  • Conservative Management: For small, asymptomatic aneurysms with low risk of rupture, with close surveillance imaging.
  • Surgical Intervention: For larger, symptomatic aneurysms, or those with concerning features, involving aneurysm excision and reconstruction.
  • Endovascular Repair: In select cases, particularly for aneurysms of the great vessels, using stent-grafts.
Prognosis
  • Variable, contingent upon size and location of aneurysm, presence of symptoms, and risk of rupture or embolism.
  • Timeliness and appropriateness of treatment influence outcomes.
  • Patient’s overall health status affects tolerance of intervention and long-term recovery.
  • Complexity of repair and potential for residual cardiac dysfunction can affect long-term survival.

4. Medical History and Culture


 

The Corset Removed Blues

The Corset Removed Reggae

A woman, thirty-three. From Africa, she comes to see. A pain is in her chest. Her history is put to test.

Twenty years ago, a fight. Presumed constrictive, tight. A pericardiectomy. To set the troubled heart wall free.

(Chorus) But the “corset” was unbound. No restraint was to be found. And decades started turning, A lesson we are learning. The loss of that restraint, Left a mechanical complaint. Giant Bilateral Atrial Appendages, The late, post-surgical damages.

The imaging is so clear. A striking finding does appear. The appendages, both left and right, Have grown into a giant sight.

Markedly dilated, thin, Symmetrically, they did begin To expand along the border, A new and strange disorder.

The ventricles are preserved. The valves and vessels, unperturbed. No constriction holds it fast, This echo from the surgical past.

(Chorus) But the “corset” was unbound. No restraint was to be found. And decades started turning, A lesson we are learning. The loss of that restraint, Left a mechanical complaint. Giant Bilateral Atrial Appendages, The late, post-surgical damages.

(Bridge) It’s iatrogenic, we suggest. Aneurysmal remodeling’s quest. The chronic, unopposed duress, The transmural pressure’s stress.

The walls were so compliant, The pressures, non-defiant. Not congenital, not from strain, But from the missing pericardial reign.

(Outro) It shows the delicate, tight relation, A mechanical situation. When the restraining sheath is gone, The chambers may grow on and on. Decades later, we now see, The price of that old surgery.

 

Etymology

  • The term “pericardiectomy” is derived from the Greek words “perikardion” (around the heart) and “ektomē” (excision or removal).
  • It was first documented in the early 1910s, with the earliest evidence appearing in Thomas Stedman’s dictionary in 1913.

AKA / Terminology

  • Pericardiectomy is also referred to as “pericardial stripping”.
  • “Pericardectomy” is a related term, with the earliest known use also dating back to the 1910s.
  • A partial pericardiectomy involves the removal of only a portion of the pericardium, while a total pericardiectomy aims for complete removal.

Historical Notes

  • The surgical treatment of constrictive pericarditis has a long history, evolving from procedures like “cardiolysis”.
  • Wilhelm Rehn is credited with the first pericardiectomy in 1920.
  • Dr. Stuart W. Harrington was a significant pioneer in this field, contributing to the understanding and surgical approach to pericardiectomy in the early to mid-20th century.
  • His work in the 1930s and 1940s, including recognizing epicardial constriction and developing surgical techniques, was foundational.
  • Mayo Clinic has a substantial history with this procedure, having performed 1472 pericardiectomies for constrictive pericarditis since 1936.
  • Historically, procedures were performed with limited technology and without the aid of experienced cardiac anesthesiologists, contrasting with modern practices.

Cultural or Practice Insights

  • Pericardiectomy is primarily indicated for constrictive pericarditis, a condition where the pericardium becomes stiff and inflamed, restricting the heart’s ability to fill properly.
  • The procedure aims to alleviate symptoms of heart failure caused by this restriction.
  • While it offers a potential cure for constrictive pericarditis, it does not address the underlying cause of the inflammation.
  • In cases where a definitive diagnosis cannot be reached through non-invasive means, a “diagnostic pericardiectomy” may be considered.
  • Historically, the effectiveness of pericardiectomy for relapsing pericarditis has been noted, with some studies suggesting complete symptom relief in a high percentage of patients following complete pericardiectomy.

Notable Figures or Contributions

  • Wilhelm Rehn: Credited with performing the first pericardiectomy in 1920.
  • Dr. Stuart W. Harrington: A pioneer in pericardiectomy for constrictive pericarditis, known for his detailed understanding of the condition, surgical skills, and contributions to surgical techniques, including the concept of “epicardiolysis”.
  • Dr. R. Lee Clark: Founder of the Medical Arts Publishing Foundation, which published journals like “The Heart Bulletin,” featuring medical illustrations related to cardiology.

Examples

  • Paintings: While direct paintings titled “Pericardiectomy” are not readily found, the broader themes of cardiac surgery, anatomical illustration, and the relief of suffering could be represented in medical art.
  • Sculptures: Similar to paintings, specific sculptures depicting pericardiectomy are rare. However, the concept of liberation or release from constraint could be artistically interpreted.
  • Photography: Contemporary medical photography often documents surgical procedures. Images of the surgical field during a pericardiectomy, sterile instruments, the surgical team in action, and post-operative recovery scenes could be found in medical journals or hospital archives.
  • Literature: Medical case reports and surgical technique articles extensively document pericardiectomy. The scientific literature itself serves as a form of literature detailing the procedure, its indications, and outcomes.
  • Poetry: The poem above offers a poetic interpretation of pericardiectomy. Additionally, poetry often explores themes of illness, healing, and the human body, which can resonate with the experience of undergoing such a procedure.
  • Song/Music: There are no widely recognized songs or musical compositions specifically about pericardiectomy. However, instrumental music, particularly that used in therapeutic settings or associated with themes of healing and resilience, could be conceptually linked.

Quotes and/or Teaching Lines

  • “Recognizing and treating epicardial constriction is essential in performing an adequate pericardiectomy.” (Attributed to Dr. Stuart W. Harrington’s work).
  • “Constrictive pericarditis remains an important cause of diastolic heart failure because, in contrast to other etiologies that are primarily myocardial abnormalities and difficult to manage, constrictive pericarditis can be cured in many patients by operation.”
  • “Radical resection of the pericardium, facilitated by CPB, helps minimize the risk of recurrent constrictions and the need for reinterventions.”
  • The term “pericardiectomy” is derived from the Greek words “perikardion” (around the heart) and “ektomē” (excision or removal).
  • It was first documented in the early 1910s, with the earliest evidence appearing in Thomas Stedman’s dictionary in 1913.
  • Pericardiectomy is also referred to as “pericardial stripping”.
  • “Pericardectomy” is a related term, with the earliest known use also dating back to the 1910s.
  • A partial pericardiectomy involves the removal of only a portion of the pericardium, while a total pericardiectomy aims for complete removal.
  • The surgical treatment of constrictive pericarditis has a long history, evolving from procedures like “cardiolysis”.
  • Wilhelm Rehn is credited with the first pericardiectomy in 1920.
  • Dr. Stuart W. Harrington was a significant pioneer in this field, contributing to the understanding and surgical approach to pericardiectomy in the early to mid-20th century.
  • His work in the 1930s and 1940s, including recognizing epicardial constriction and developing surgical techniques, was foundational.
  • Mayo Clinic has a substantial history with this procedure, having performed 1472 pericardiectomies for constrictive pericarditis since 1936.
  • Historically, procedures were performed with limited technology and without the aid of experienced cardiac anesthesiologists, contrasting with modern practices.
  • Pericardiectomy is primarily indicated for constrictive pericarditis, a condition where the pericardium becomes stiff and inflamed, restricting the heart’s ability to fill properly.
  • The procedure aims to alleviate symptoms of heart failure caused by this restriction.
  • While it offers a potential cure for constrictive pericarditis, it does not address the underlying cause of the inflammation.
  • In cases where a definitive diagnosis cannot be reached through non-invasive means, a “diagnostic pericardiectomy” may be considered.
  • Historically, the effectiveness of pericardiectomy for relapsing pericarditis has been noted, with some studies suggesting complete symptom relief in a high percentage of patients following complete pericardiectomy.
  • Wilhelm Rehn: Credited with performing the first pericardiectomy in 1920.
  • Dr. Stuart W. Harrington: A pioneer in pericardiectomy for constrictive pericarditis, known for his detailed understanding of the condition, surgical skills, and contributions to surgical techniques, including the concept of “epicardiolysis”.
  • Dr. R. Lee Clark: Founder of the Medical Arts Publishing Foundation, which published journals like “The Heart Bulletin,” featuring medical illustrations related to cardiology.
  • Paintings: While direct paintings titled “Pericardiectomy” are not readily found, the broader themes of cardiac surgery, anatomical illustration, and the relief of suffering could be represented in medical art.
  • Sculptures: Similar to paintings, specific sculptures depicting pericardiectomy are rare. However, the concept of liberation or release from constraint could be artistically interpreted.
  • Photography: Contemporary medical photography often documents surgical procedures. Images of the surgical field during a pericardiectomy, sterile instruments, the surgical team in action, and post-operative recovery scenes could be found in medical journals or hospital archives.
  • Literature: Medical case reports and surgical technique articles extensively document pericardiectomy. The scientific literature itself serves as a form of literature detailing the procedure, its indications, and outcomes.
  • Poetry: The poem above offers a poetic interpretation of pericardiectomy. Additionally, poetry often explores themes of illness, healing, and the human body, which can resonate with the experience of undergoing such a procedure.
  • Song/Music: There are no widely recognized songs or musical compositions specifically about pericardiectomy. However, instrumental music, particularly that used in therapeutic settings or associated with themes of healing and resilience, could be conceptually linked.
  • “Recognizing and treating epicardial constriction is essential in performing an adequate pericardiectomy.” (Attributed to Dr. Stuart W. Harrington’s work).
  • “Constrictive pericarditis remains an important cause of diastolic heart failure because, in contrast to other etiologies that are primarily myocardial abnormalities and difficult to manage, constrictive pericarditis can be cured in many patients by operation.”
  • “Radical resection of the pericardium, facilitated by CPB, helps minimize the risk of recurrent constrictions and the need for reinterventions.”

6. MCQs


Part A

Questions Answers
1. What is the primary pathophysiological mechanism by which constrictive pericarditis leads to left atrial dilatation and subsequent atrial appendage abnormalities? The stiffening and restrictive nature of the pericardium in constrictive pericarditis impedes diastolic filling of the ventricles. This leads to elevated left atrial pressures and volumes, driving left atrial dilatation. The atrial appendage, as an extension of the left atrium, subsequently dilates or may become aneurysmal due to these sustained pressure and volume changes.
Atrial fibrillation leading to increased LA volume and pressure.
Direct inflammatory involvement of the atrial wall by the pericarditis.
Reduced cardiac output causing a reflex increase in atrial size.
2. Discuss the embryological development of the left atrial appendage and how congenital dysplasia of the musculi pectinati can predispose to its aneurysmal dilatation. The left atrial appendage develops from the embryonic left atrium. Congenital dysplasia of the musculi pectinati, which form the ridges within the appendage, can lead to weakened walls and abnormal folding, predisposing to aneurysmal dilatation.
The left atrial appendage forms from the primitive pulmonary veins. Abnormal development of the myocardium can result in thinning and subsequent aneurysmal changes.
The atrial appendage originates from the sinus venosus. Defects in its septation can cause dilatation.
It arises from the epicardium. Overgrowth of the epicardial tissue can lead to aneurysmal formation.
3. In a patient with a history of pericardiectomy for constrictive pericarditis, what are the potential mechanisms that could lead to iatrogenic, post-surgical aneurysmal dilatation of the atrial appendages? Iatrogenic aneurysmal dilatation of atrial appendages post-pericardiectomy can arise from surgical manipulation, particularly if there was an attempt at appendage ligation or plication that was incomplete or disrupted. Alterations in intracardiac hemodynamics due to pericardial removal, leading to altered stress distribution, or direct injury to the appendage during surgery could also contribute.
Residual inflammation from the original pericarditis.
Spontaneous development of cardiac fibrosis post-surgery.
Development of a new autoimmune response against the pericardium.
4. What are the significant clinical implications and potential complications associated with a dilated or aneurysmal atrial appendage in the context of a post-pericardiectomy state? The primary complication is an increased risk of thrombus formation within the appendage, leading to potential systemic embolization, particularly stroke. Arrhythmias like atrial fibrillation may also be more prevalent.
Increased risk of pulmonary embolism.
Development of constrictive physiology in the absence of pericardial disease.
Reduced cardiac output due to impaired atrial contractility.
5. What specific echocardiographic views and modalities (e.g., TTE, TEE) are most crucial for characterizing the morphology and function of a dilated atrial appendage, particularly in assessing for thrombus burden and potential embolic risk? Transesophageal echocardiography (TEE) is crucial for detailed morphological assessment of the left atrial appendage (LAA), including its size, shape, and the presence of thrombus. Specific views like the mid-esophageal two-chamber view and the dedicated LAA view allow for direct visualization. Assessment of contractile function and spontaneous echo contrast are also vital for embolic risk stratification.
Transthoracic echocardiography (TTE) alone, focusing on apical four-chamber views.
Intracardiac echocardiography (ICE) used during electrophysiology procedures.
Doppler echocardiography to assess blood flow velocity within the appendage.
6. Beyond echocardiography, what role does cardiac MRI or CT angiography play in the comprehensive evaluation of an aneurysmal atrial appendage, especially in delineating its relationship with adjacent structures and assessing for associated cardiac abnormalities? Cardiac MRI and CT angiography provide excellent anatomical detail, aiding in characterizing the appendage’s size, morphology, its spatial relationship to adjacent structures (e.g., pulmonary veins, aorta), and can help identify associated cardiac abnormalities or complications that may not be fully visualized by echocardiography.
They are primarily used for assessing atrial appendage function.
Their main role is in guiding percutaneous interventions on the appendage.
They are mainly used to evaluate the degree of pericardial calcification.
7. How can imaging differentiate between a true aneurysmal dilatation of the atrial appendage and other cystic or mass-like lesions in the vicinity of the left atrium post-operatively? Imaging modalities like echocardiography (especially TEE), cardiac MRI, and CT angiography can differentiate based on characteristic morphology, continuity with the left atrium, Wall enhancement patterns (in MRI/CT), and the presence or absence of internal vascularity or septations specific to the atrial appendage.
Differentiation is only possible with endomyocardial biopsy.
All cystic lesions near the left atrium post-operatively are assumed to be appendage dilatations.
Fluoroscopy is the most effective method for this differentiation.

Part B

1. What is the primary pathophysiological mechanism by which constrictive pericarditis leads to left atrial dilatation and subsequent atrial appendage abnormalities?
A.
The stiffening and restrictive nature of the pericardium in constrictive pericarditis impedes diastolic filling of the ventricles. This leads to elevated left atrial pressures and volumes, driving left atrial dilatation. The atrial appendage, as an extension of the left atrium, subsequently dilates or may become aneurysmal due to these sustained pressure and volume changes.
B. Atrial fibrillation leading to increased LA volume and pressure.
C. Direct inflammatory involvement of the atrial wall by the pericarditis.
D. Reduced cardiac output causing a reflex increase in atrial size.
A.
B. x
C. x
D. x
  • This answer correctly identifies the primary hemodynamic consequence of constrictive pericarditis: impaired ventricular filling leading to elevated left atrial pressure and volume, which subsequently causes left atrial and appendage dilatation.
  • Citation: Janardhanan, P. et al. (2010). Constrictive pericarditis. Heart.
  • Options B, C, and D describe associated conditions or consequences, not the primary pathophysiological driver initiated by constrictive pericarditis itself.
2. In a patient with a history of pericardiectomy for constrictive pericarditis, what are the potential mechanisms that could lead to iatrogenic, post-surgical aneurysmal dilatation of the atrial appendages?
A.
Iatrogenic aneurysmal dilatation of atrial appendages post-pericardiectomy can arise from surgical manipulation, particularly if there was an attempt at appendage ligation or plication that was incomplete or disrupted. Alterations in intracardiac hemodynamics due to pericardial removal, leading to altered stress distribution, or direct injury to the appendage during surgery could also contribute.
B. Residual inflammation from the original pericarditis.
C. Spontaneous development of cardiac fibrosis post-surgery.
D. Development of a new autoimmune response against the pericardium.
A.
B. x
C. x
D. x
  • This answer accurately points to direct surgical effects such as incomplete ligation, disruption of surgical repairs, or direct injury as causes of iatrogenic appendage dilatation post-pericardiectomy. Changes in hemodynamics after pericardial release are also relevant.
  • Citation: Murphy, J. et al. (2005). Atrial appendage closure for stroke prevention in patients with atrial fibrillation. Circulation.
  • Options B, C, and D describe inflammatory or fibrotic processes, which are related to the underlying disease or general post-surgical healing, but not specifically to the iatrogenic mechanisms introduced by the surgery itself.
3. What specific echocardiographic views and modalities (e.g., TTE, TEE) are most crucial for characterizing the morphology and function of a dilated atrial appendage, particularly in assessing for thrombus burden and potential embolic risk?
A.
Transesophageal echocardiography (TEE) is crucial for detailed morphological assessment of the left atrial appendage (LAA), including its size, shape, and the presence of thrombus. Specific views like the mid-esophageal two-chamber view and the dedicated LAA view allow for direct visualization. Assessment of contractile function and spontaneous echo contrast are also vital for embolic risk stratification.
B. Transthoracic echocardiography (TTE) alone, focusing on apical four-chamber views.
C. Intracardiac echocardiography (ICE) used during electrophysiology procedures.
D. Doppler echocardiography to assess blood flow velocity within the appendage.
A.
B. x
C. x
D. x
  • TEE provides superior visualization of the LAA, enabling detailed assessment of morphology, thrombus, and function, which is critical for embolic risk stratification.
  • Citation: Srivathsan, K. et al. (2013). Left atrial appendage thrombus in patients with atrial fibrillation undergoing electrophysiology procedures. Journal of Cardiovascular Electrophysiology.
  • While TTE provides general cardiac information, it is often suboptimal for detailed LAA evaluation. ICE and Doppler are useful adjuncts but not the primary modality for comprehensive assessment of thrombus and morphology.
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