Keywords
constriction - CP - pathophysiology - pericardial disease - pericarditis
Introduction
The pericardium is a relatively avascular fibrous sac surrounding the heart, composed
of two layers: the visceral and parietal pericardium. The visceral layer consists
of a single layer of mesothelial cells adherent to the epicardium, while the parietal
layer is a fibrous structure less than 2 mm thick, composed primarily of collagen
with some elastin. Between these layers lies a potential space normally containing
15 to 35 mL of serous fluid. Functionally, the pericardium stabilizes the heart within
the mediastinum, prevents excessive cardiac dilation during sudden volume shifts,
and serves as a barrier to limit the spread of infection from adjacent structures.[1]
[2]
Constrictive pericarditis (CP) is a chronic condition marked by fibrotic thickening
and often calcification of the pericardium, leading to impaired diastolic filling,
reduced cardiac output, and causes systemic venous congestion. Once primarily attributed
to infections such as tuberculosis—especially in the preantibiotic era[3]
[4]—its epidemiology has shifted significantly in recent decades. While tuberculosis
remains a leading cause of CP in endemic regions, more contemporary etiologies in
developed countries include prior cardiac surgery, interventional cardiac procedures,
thoracic radiation therapy, viral pericarditis, and autoimmune disorders. These causes
have been increasingly reported in recent literature and now account for a significant
proportion of cases.[5]
[6]
[7]
[8]
[9]
[10]
[11]
[12]
This evolving clinical landscape necessitates a reappraisal of CP, not only regarding
its etiology but also its diagnostic and therapeutic approach.
This review aims to explore the contemporary understanding of CP by bridging historical
context with modern practice, highlighting advances in diagnosis, shifting etiologies,
and current management strategies to improve patient outcomes.
Historical Perspective on CP
Historical Perspective on CP
CP has been recognized for centuries.[13] In 1669, Richard Lower offered the first clinical insight into pericardial constriction
by describing cardiac tamponade and pulsus paradoxus in patients with dyspnea, laying
the foundation for the hemodynamic understanding of pericardial diseases.[14]
During the 18th century, Giovanni Battista Morgagni and Giovanni Maria Lancisi provided
key pathological descriptions linking pericardial effusion and thickening to circulatory
compromise and specific clinical signs, such as distended neck veins and abdominal
swelling.[15]
[16]
The 19th century brought further clarity: Norman Chevers differentiated CP from other
cardiac pathologies,[17] while Adolph Kussmaul and Friedrich Pick described hallmark signs of pericardial
constriction including paradoxical pulse and hepatic congestion, findings that would
later carry diagnostic importance.[18]
[19]
A major therapeutic milestone occurred in 1929, when Churchill performed the first
successful pericardiectomy, later reinforced by Paul Dudley White's clinical series
showing favorable outcomes with surgical intervention.[20]
[21] The physiologic understanding of the disease advanced significantly in the mid-20th
century with Bloomfield et al's documentation of equalization of diastolic pressures
and the characteristic dip-and-plateau waveform, hallmarks of constrictive physiology.[22]
By the latter half of the 20th century, the condition's iatrogenic nature came into
focus, with Kendall et al and others, highlighting postoperative cardiac surgery as
a significant cause.[23]
[24]
[25]
[26]
[27]
[28]
[29] These historical insights have paved the way for today's understanding of CP, informing
both diagnostic approaches and therapeutic strategies.
Pathophysiology
CP results from scarring, thickening, and often calcification of the pericardium,
forming a rigid, noncompliant shell that restricts diastolic filling and limits total
cardiac volume. The hallmark hemodynamic feature is the equalization of end-diastolic
pressures across all four cardiac chambers—reflecting external constraint by the pericardium
rather than intrinsic myocardial dysfunction.[1]
[30]
In early diastole, rapid ventricular filling occurs due to pressure gradients between
the atria and ventricles. However, once the fixed pericardial reserve volume is reached,
filling is abruptly halted. This results in the characteristic “dip and plateau” or
“square root” sign on ventricular pressure tracings, a key diagnostic clue[1]
[31]
[32]
[33] ([Fig. 1]).
Fig. 1 Left ventricular (LV) and right ventricular (RV) pressures recorded simultaneously,
showing that both pressures are equal throughout diastole. A long diastole (thick
arrow) following a premature beat shows equalization of the LV and RV diastolic pressures
and a “dip and plateau” pattern (“square root” sign).
In CP, the thickened and noncompliant pericardium restricts the normal transmission
of intrathoracic pressure changes to the cardiac chambers. As a result, right heart
filling becomes impaired during inspiration, contributing to Kussmaul's sign—a paradoxical
rise or lack of decline in jugular venous pressure (JVP) with inspiration. This impaired
pressure transmission also leads to paradoxical interventricular septal motion during
the respiratory cycle, a phenomenon known as septal bounce.[31]
[32] Septal bounce is characterized by the interventricular septum shifting toward the
left ventricle during inspiration and toward the right ventricle during expiration,
reflecting exaggerated ventricular interdependence caused by the encasing, rigid pericardium
([Fig. 2]).
Fig. 2 Septal bounce (ventricular interdependence) during respiratory phases in constrictive
pericarditis—due to noncompliant pericardium. RV, right ventricle; LV, left ventricle.
Clinical Presentation
CP often presents insidiously, with nonspecific symptoms such as malaise, lack of
energy, and easy fatigability, which can delay diagnosis. The clinical picture is
typically dominated by signs and symptoms of right-sided heart failure. Patients commonly
report progressive peripheral edema, abdominal bloating with increasing abdominal
girth, and right upper quadrant discomfort or pain due to hepatic congestion.[1]
[34]
[35]
A hallmark clinical sign is elevated JVP, often accompanied by a positive Kussmaul's
sign, a paradoxical rise or failure of the JVP to fall during inspiration. While highly
suggestive of CP, Kussmaul's sign is not specific and may also be observed in restrictive
cardiomyopathy, pulmonary embolism, right ventricular infarction, and advanced right
heart failure.[1]
[35]
On cardiac auscultation, heart sounds may be muffled due to the dense, fibrotic pericardium.
A classic auscultatory finding is the pericardial knock—a high-pitched, early diastolic
sound—reflecting abrupt cessation of ventricular filling. Although distinctive, this
finding is not always present. Pulsus paradoxus is uncommon in classic CP but, if
present, may suggest an effusive-constrictive variant.[1]
[2]
[35]
Additional physical findings include hepatosplenomegaly, ascites, and right upper
quadrant tenderness from hepatic congestion. A characteristic clue is disproportionate
peripheral edema in the absence of significant pulmonary rales, reflecting predominant
systemic venous congestion.[1]
[35]
[36]
Etiology
Globally, tuberculosis remains the most common cause of CP, especially in low- and
middle-income countries where the disease remains endemic.[4]
[5] In contrast, the etiology of CP in developed nations has shifted markedly in recent
decades.
In high-income settings, the majority of cases are now attributed to idiopathic or
viral pericarditis, accounting for approximately 42 to 49% of cases. This is followed
by postcardiac surgery (11–37%) and chest radiation (9–31%) as leading contributors.
Other notable etiologies include autoimmune diseases, malignancy, and postinfectious
causes unrelated to tuberculosis.[33]
[34]
[35]
[36]
[37]
A retrospective review of 135 cases of surgically confirmed CP at the Mayo Clinic
highlighted this shift in causation. The three most common identifiable etiologies
were previous cardiac surgery (18%), idiopathic or viral pericarditis (16%), and mediastinal
irradiation (13%). The study also revealed a male predominance, with 76% of cases
occurring in men.[33]
[34]
Given this changing landscape, an understanding of contemporary risk factors is essential
for early recognition. The next sections of this review will focus on the modern causes
of CP in greater detail, including postcardiac interventions, postradiation, autoimmune,
and iatrogenic contributors.
The Modern Landscape of CP
The Modern Landscape of CP
Postcardiac Interventions
-
(1) Cardiac surgeries, including coronary artery bypass grafting (CABG), valve replacement, and other open-heart
procedures, are well-recognized contributors to the development of CP. The primary
mechanism involves trauma and subsequent scarring of the pericardium incurred during
or following surgical intervention.[23]
[24]
[25]
[26]
[27]
[28]
[29]
[33]
[37]
During cardiac surgery, the pericardium is typically opened to access the heart. This
manipulation can result in direct injury to the pericardial layers, triggering acute
inflammation. The disruption of the pericardial structure initiates a cascade of inflammatory
responses that may persist beyond the perioperative period. Notably, post-pericardiotomy
syndrome (PPS)—a known postoperative complication—is characterized by pericardial
inflammation that, if severe or unresolved, can evolve into chronic fibrosis and pericardial
thickening.[5]
[29]
[30]
Intraoperative or postoperative bleeding into the pericardial space, whether due to
incomplete hemostasis or coagulopathy, exacerbates the inflammatory process. Blood
accumulation in the pericardial sac stimulates a fibrinous inflammatory response that
may ultimately result in fibrotic scarring. Animal models have shown that pericardial
adhesions form when spilled blood contacts injured serosal surfaces.[27]
[28] The extent of inflammation and fibrosis depends not only on the volume of blood
but also on the severity of pericardial trauma.
Risk factors for developing postsurgical CP include longer operative times and extensive
manipulation of the heart and pericardium. The timing of disease onset can vary widely—from
as early as 3 weeks to decades after surgery—making high index of suspicion essential.[25]
[26]
Early-onset constriction is often driven by acute inflammation and unresolved surgical
complications, while late-onset disease tends to reflect chronic scarring, fibrosis,
and calcification. This temporal variability requires clinicians to maintain a high
index of suspicion, especially in patients with compatible symptoms and a history
of cardiac surgery.[22]
[25]
[26]
The true incidence of postsurgical CP is likely underestimated. Mild or subclinical
cases may resemble low cardiac output syndrome—particularly in patients following
valve surgery or CABG with underlying left ventricular dysfunction. These patients
may respond to standard heart failure therapies, such as diuretics, and thus evade
further diagnostic evaluation.[29]
[30]
Although PPS has been implicated as a risk factor, occurring in up to 30% of patients
after cardiac surgery, its role remains uncertain. The presence of PPS does not reliably
predict progression to constriction, and conversely, CP can occur in patients without
any signs of PPS.[23]
[25]
[29]
-
(2) Percutaneous coronary interventions (PCIs), while a common and vital procedure, can occasionally result in CP, though
this is a rare complication. A notable case reported in 2006 described a patient who
developed CP 6 months following PCI. The patient presented with exertional dyspnea,
excessive fatigue, progressive lower extremity edema, and increasing abdominal girth
due to ascites. Transthoracic echocardiography and hemodynamic assessment via cardiac
catheterization confirmed the diagnosis of pericardial constriction, underscoring
the potential role of PCI in triggering this condition.[6]
While the exact pathophysiological mechanisms remain somewhat unclear, coronary artery
perforation during catheter or wire manipulation has been identified as a potential
cause of CP following PCI.[9]
[10] However, this is generally not directly attributed to coronary perforation during
the procedure itself. Rather, CP following PCI is typically a consequence of inflammation
and subsequent fibrosis of the pericardium. This inflammatory response is often triggered
by mechanical irritation, the use of contrast agents, or other procedural factors.
During PCI, catheters and guidewires are maneuvered within the coronary arteries and
heart chambers. This manipulation can lead to minor trauma to the epicardial surface
or pericardium, which in turn causes localized inflammation. Additionally, the use
of iodinated contrast media during PCI can provoke an inflammatory reaction, particularly
in susceptible individuals. In more severe cases, coronary artery perforation, although
rare, can occur as a complication of PCI. If blood and contrast material leak into
the pericardial space due to perforation, it can trigger acute pericarditis.[28] Chronic inflammation and the healing response may then lead to fibrosis and thickening
of the pericardium, ultimately resulting in CP.[9]
[10]
-
(3) Catheter-based ablation procedures, particularly those used to treat atrial fibrillation (AF), have revolutionized the
management of cardiac arrhythmias. However, while these procedures are generally effective,
they can occasionally lead to complications involving the pericardium, including acute
pericarditis and, in rare cases, CP.[38]
[39]
[40]
[41]
During catheter-based ablation, energy sources such as radiofrequency (RF) or cryoablation
are used to create lesions in the myocardium to disrupt abnormal electrical circuits.
While the primary target is myocardial tissue, the energy can inadvertently affect
adjacent structures, including the epicardium and pericardium. Excessive heat from
RF ablation or extreme cold from cryoablation can cause thermal injury to the pericardial
layers.[39] This injury triggers an inflammatory response in the pericardium, which can lead
to acute pericarditis. In rare instances, this inflammation may evolve into CP.
CP following catheter-based ablation for AF is primarily caused by thermal injury
to the pericardium, microscopic perforations, or an exaggerated immune response. These
insults induce acute inflammation, which, if unresolved, can progress to chronic fibrosis
and calcification of the pericardium. The resulting rigidity impairs cardiac filling,
leading to the clinical syndrome of CP.[9]
[39]
[41]
Radiation Therapy
Radiation-induced CP is a late complication of thoracic radiation therapy, often manifesting
years to decades after exposure, particularly in patients treated for malignancies
involving the chest, such as Hodgkin's lymphoma, breast cancer, and other mediastinal
tumors.[11]
[12] Heart disease occurring after irradiation of the mediastinum was first recognized
in the 1960s, when long-term survival after radiotherapy for Hodgkin's disease became
frequent.[12] In recent years, with improvements in the techniques of radiotherapy and with greater
use of chemotherapy, cases of heart disease after radiation have been fewer and less
severe. However, since pericarditis may develop or become clinically manifest many
years after the radiotherapy, new cases do continue to appear.[42]
Radiation therapy damages the pericardium through a combination of acute inflammatory
responses and chronic fibrotic processes. Radiation causes direct injury to the pericardial
cells, leading to an acute inflammatory response. This phase is characterized by pericardial
effusion, which may be asymptomatic or present as acute pericarditis. Inflammation
leads to increased vascular permeability and fibrin deposition in the pericardial
space. Persistent inflammation triggers fibroblast activation and collagen deposition,
resulting in thickening of the pericardium.[42]
[43]
[44]
Over time, chronic inflammation and oxidative stress lead to progressive fibrosis
and calcification of the pericardium. The normally elastic pericardial tissue becomes
rigid, impairing diastolic filling of the heart.
Contributing factors include higher doses of radiation (> 30 Gy) and larger treatment
fields, which increase the risk of pericardial injury.[45]
[46] Preexisting cardiovascular disease, autoimmune conditions, or genetic predispositions
may exacerbate the effects of radiation. Patients who undergo cardiac surgery after
radiation therapy are at higher risk due to the cumulative trauma to the pericardium
along with the myocardium.[45]
[47]
The latency period between radiation exposure and the development of CP can range
from months to decades, with most cases occurring 5 to 20 years after treatment. Calcification
is a hallmark of advanced disease and contributes to the restrictive physiology seen
in CP.[12]
Autoimmune Diseases
Several autoimmune diseases have been associated with CP, though this manifestation
is relatively rare. The progression from pericardial inflammation to constriction
can occur if the initial inflammation is recurrent or inadequately treated.[48]
[49]
[50]
[51]
Systemic lupus erythematosus (SLE): SLE is one of the most common autoimmune diseases associated with pericardial
involvement. Pericarditis is a frequent cardiac manifestation in SLE patients, and
it can present as the first manifestation of SLE.[50] While CP is less common, it can develop as a complication of recurrent or chronic
pericardial inflammation.[50]
Rheumatoid
arthritis (RA): RA can involve the pericardium, leading to pericarditis. Although CP is rare
in RA, it has been reported, particularly in cases with prolonged disease duration
or inadequate control of systemic inflammation.
Pericardial involvement is a common occurrence in RA, affecting about one-third of
patients with a prevalence from 30 to 50% and is frequently asymptomatic.[48]
[49]
[51]
Systemic
sclerosis (scleroderma): Systemic sclerosis is known to affect the heart, including the pericardium.
Pericardial involvement can lead to effusions and, in rare cases, progress to CP due
to fibrosis and calcification of the pericardial layers.[51]
Sjögren's
syndrome: While pericardial involvement is uncommon in primary Sjögren's syndrome, cases of
pericarditis and even CP have been documented. The pathogenesis is thought to involve
immune-mediated inflammation leading to fibrosis.[52] In all these conditions, early recognition and treatment of pericardial inflammation
are crucial to prevent progression to CP. Management typically involves immunosuppressive
therapy tailored to the underlying autoimmune disease.[51]
Viral Pericarditis
Viral pericarditis is the most common cause of acute pericarditis in developed countries.
Common causative agents include coxsackievirus B, echovirus, adenovirus,[30]
[36]
[53]
[54] and more recently COVID-19.[55]
[56]
[57] The typical course of viral pericarditis is self-limited; however, in rare cases,
persistent or recurrent inflammation can lead to chronic fibrotic changes of the pericardium,
ultimately resulting in CP.[30]
[36]
The mechanism is believed to involve an exaggerated or unresolved immune response
following viral infection, leading to pericardial thickening, fibrosis, calcification,
and loss of pericardial elasticity. These structural changes impair diastolic filling
of the heart, causing the classic hemodynamic findings of CP. Clinical signs may appear
weeks to months after the initial viral illness, and the diagnosis is often supported
by imaging findings (e.g., pericardial thickening on computed tomography [CT] or magnetic
resonance imaging [MRI]) and history of preceding viral symptoms.[30]
[36]
[53]
[54]
End-Stage Renal Disease Pericarditis
Pericarditis in end-stage renal disease (ESRD) can occur either before dialysis initiation
(uremic pericarditis) or during maintenance dialysis (dialysis-associated pericarditis).[58]
[59] Uremic pericarditis has been arbitrarily defined as pericarditis that develops before
or within 8 weeks of initiation of dialysis, while dialysis-associated pericarditis
is used to define pericarditis in patients on dialysis for more than 8 weeks. Both
forms result from the accumulation of uremic toxins, chronic inflammation, and fluid
overload. Inadequate dialysis, persistent systemic inflammation, or secondary infections
may exacerbate the condition.[58]
Patients with ESRD are particularly prone to developing pericardial calcifications,
a hallmark of advanced constrictive physiology.[49]
[60] Risk factors for progression to CP include delayed or suboptimal dialysis, chronic
fluid overload, and recurrent pericardial effusions.[58]
[59]
[60]
[61]
Clinical suspicion should be raised when dialysis patients present with signs of right
heart failure (e.g., peripheral edema, ascites, jugular venous distention) that are
not explained by volume overload alone. Diagnosis is confirmed through echocardiography,
CT, or cardiac MRI, and management often requires both aggressive dialysis and, in
severe cases, pericardiectomy.[58]
[59]
[60]
[61]
Diagnosis
The diagnosis of CP is often challenging and must be individualized based on clinical
context. In some patients, a diagnosis may be suggested by the medical history, physical
examination, and basic imaging such as chest radiography. In others, more advanced
investigations, including echocardiography, cross-sectional imaging, and invasive
hemodynamic studies, may be required.[35]
[37]
Timely diagnosis requires a high index of suspicion, informed by clinical presentation,
imaging findings, and hemodynamic data. Fortunately, advancements in multimodality
imaging, including echocardiography, cardiac CT, and cardiac MRI, have improved early
recognition and accurate classification. Importantly, CP is a potentially curable
condition; some patients respond to anti-inflammatory therapy, while others benefit
from surgical pericardiectomy.[35]
A high index of suspicion remains essential, particularly in patients presenting with
signs of right-sided heart failure that seem disproportionate to pulmonary congestion
or left-sided cardiac disease. A comprehensive understanding of the disease's pathophysiology,
along with judicious use of diagnostic tools, greatly enhances diagnostic accuracy,
especially in cases where pericardial and myocardial involvement coexist.[33]
[34]
[35]
[36]
[37] Differentiating CP from restrictive cardiomyopathy is of critical clinical importance,
as CP is a potentially reversible condition with surgical treatment, whereas restrictive
cardiomyopathy typically carries a more progressive course with limited curative options.
Laboratory Evaluation
Inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein
may be elevated in transient or subacute constriction and can help identify patients
who might benefit from anti-inflammatory therapy.[35]
Liver function tests may show elevated enzymes and hyperbilirubinemia due to hepatic
congestion from chronic right heart failure. Hypoalbuminemia can occur secondary to
protein-losing enteropathy or proteinuria has been reported, which completely resolved
following pericardiectomy.[62]
Natriuretic peptides (B-type natriuretic peptide [BNP], N-terminal-pro-BNP) are typically
only mildly elevated, which can aid in differentiating CP from restrictive cardiomyopathy.[63]
Imaging
Chest X-ray may reveal pericardial calcification, which is pathognomonic when present
in the appropriate clinical setting. Echocardiography is the first-line imaging modality
for evaluating suspected CP. It is essential to consider CP in patients with prior
pericarditis, pericardial effusion, cardiac surgery, interventional procedures, or
chest radiation.
Two-dimensional and Doppler echocardiography can demonstrate the hallmark physiologic
features of CP, such as: respiration-related ventricular septal shift (septal bounce)—the
most sensitive marker (93%); preserved or increased medial mitral annular e' velocity
along with reduced lateral mitral annular e' velocity, the so-called “annular reversus”;
and prominent expiratory diastolic flow reversal in hepatic veins.[36]
[64]
[65] These findings reflect the core pathophysiologic mechanisms of CP: dissociation
of intrathoracic and intracardiac pressures and interventricular dependence within
a fixed volume.
Cardiac MRI: Accurately assesses pericardial thickening (> 4 mm) with sensitivity of 93%.[66] Cardiac MRI pericardial late gadolinium enhancement (LGE) can identify active pericardial
inflammation, guide anti-inflammatory therapy, and could predict the reversibility
of CP after anti-inflammatory therapy. It can be performed serially in CP patients
to follow disease progression and resolution with medical management, thus avoiding
unwanted referrals to surgery.[67]
[68] It also helps differentiate CP from restrictive cardiomyopathy with high sensitivity
and specificity, particularly by detecting myocardial delayed enhancement (present
in restrictive cardiomyopathy but typically absent in isolated CP). Assesses myocardial
pathology when cardiomyopathy is suspected.[66]
Cardiac CT: Provides excellent visualization of pericardial calcification, fibrosis, and thickening
([Fig. 3]). Not influenced by body habitus and useful when echocardiography is suboptimal.
Clearly demonstrates respirophasic septal motion and alternative causes of dyspnea
(e.g., lung disease, diaphragmatic paralysis, etc.).[66]
[69]
[70]
Fig. 3 Computed tomography (CT) of the heart (H) showing a thick pericardium (P; arrow).
It must be noted that pericardial constriction could happen in patients with normal
pericardial thickness. Talreja et al[71] reported up to 18% of surgically confirmed CP cases may show normal pericardial
thickness, underscoring that normal imaging does not exclude the diagnosis. Pericardial
thickness was not increased in 18% of patients with surgically proven CP, although
the histopathological appearance was focally abnormal in all cases. When clinical,
echocardiographic, or invasive hemodynamic features indicate constriction in patients
with heart failure, pericardiectomy should not be denied on the basis of normal thickness
as demonstrated by noninvasive imaging.[71]
Invasive Hemodynamic Assessment
Cardiac catheterization remains the gold standard when noninvasive tests are inconclusive. Typical findings
include equalization of diastolic pressures across all four chambers, dip-and-plateau
(square root) sign in ventricular pressure tracings, and discordance between intrathoracic
and intracardiac pressures, confirming the pathophysiology of constriction[36]
[64]
[65]
[70] ([Fig. 1]).
The Mayo Clinic diagnostic criteria integrate echocardiographic and catheterization data, emphasizing ventricular interdependence
and pressure dissociation unique to CP[72] ([Table 1]).
Table 1
Mayo Clinic diagnostic criteria for constrictive pericarditis
|
Category
|
Findings/Criteria
|
|
Clinical findings
|
- Elevated jugular venous pressure (JVP) with Kussmaul's sign
- Peripheral edema, ascites
|
|
Echocardiographic features
|
- Septal bounce (paradoxical septal motion)
- Respiratory variation in mitral/tricuspid inflow (> 25%)
- Annulus reversus (medial E′ > lateral E′)
- Annulus paradoxus (E′ preserved despite elevated filling pressures)
|
|
Cardiac catheterization
|
- Equalization of end-diastolic pressures in all chambers
- Dip-and-plateau (square root) sign
- Ventricular discordance with respiration
|
|
Imaging (CT or MRI)
|
- Pericardial thickening (> 2 mm)
- Pericardial calcification
- Abnormal interventricular dependence
|
|
Additional findings
|
- Improvement with anti-inflammatory therapy (in subacute/inflammatory cases)
- Biopsy/histology may show chronic inflammation or fibrosis
|
Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging.
Distinguishing CP from restrictive cardiomyopathy is critical, as pericardiectomy
can cure CP, while treatment for restrictive cardiomyopathy is supportive.[69]
[70]
Treatment
The management of CP depends on the activity of the inflammatory process, the chronicity
of the disease, the severity of symptoms, and the underlying etiology. An individualized
approach is essential, with treatment goals centered on alleviating symptoms, reducing
inflammation when present, and, when necessary, performing surgical intervention to
relieve pericardial constriction.[30]
[34]
[37]
In patients with active pericardial inflammation, a subset of them experiences reversibility
of pericardial inflammation, a condition referred to as “transient constriction” may
respond to anti-inflammatory therapy without surgical intervention.[34]
[73] Predictors of favorable response include chest pain, elevated inflammatory markers,
and moderate to severe LGE of the pericardium on cardiac MRI. These features suggest
ongoing inflammation that may still be reversible.[34]
[67]
[68] Thus, detection of pericardial inflammation may be useful, as it may identify patients
with transient CP who are good candidates for anti-inflammatory therapy.
When CP becomes chronic and the pericardium progresses to fibrosis and calcification,
medical therapy alone is insufficient. In these cases, the definitive treatment is
radical pericardiectomy, which involves surgical excision of the thickened, fibrotic
pericardium ([Fig. 4]). This procedure restores normal cardiac filling by removing the physical constraint
around the heart. Early surgical intervention, before the development of significant
myocardial dysfunction, is associated with better hemodynamic recovery and long-term
survival. However, pericardiectomy carries substantial perioperative risk, especially
in patients with advanced disease, comorbidities such as chronic kidney disease, or
a history of prior cardiac surgery.[33]
[65]
[74]
Fig. 4 Intraoperative photograph taken during surgical stripping of the pericardium (P),
illustrating the markedly thickened and adherent pericardial tissue. H, heart.
Conclusion
CP is a complex condition with shifting etiologies—from tuberculosis to modern causes
like cardiac interventions and radiation therapy. Advances in imaging (MRI, CT) and
hemodynamic assessment have refined diagnosis, while treatment hinges on distinguishing
reversible inflammation (managed medically) from chronic fibrosis (requiring pericardiectomy).
Perspective
Looking ahead, the evolving landscape of CP demands heightened vigilance, particularly
among oncologists, cardiologists, and rheumatologists managing at-risk patients. Future
research should explore biomarkers for early detection and refine pericardiectomy
techniques in high-risk groups. Bridging historical insights with contemporary practice
will be key to improving outcomes in this potentially curable disease.
