Postpericardiotomy syndrome (PPS) is a medical syndrome referring to an immune phenomenon that occurs days to months (usually 1–6 weeks) after surgical incision of the

pericardium The pericardium, also called pericardial sac, is a double-walled sac containing the heart and the roots of the great vessels. It has two layers, an outer layer made of strong connective tissue (fibrous pericardium), and an inner layer made of ...

(membranes encapsulating the human heart). PPS can also be caused after a trauma, a puncture of the cardiac or

pleural The pleural cavity, pleural space, or interpleural space is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication bet ...

structures (such as a bullet or stab wound), after

percutaneous coronary intervention Percutaneous coronary intervention (PCI) is a non-surgical procedure used to treat narrowing of the coronary arteries of the heart found in coronary artery disease. The process involves combining coronary angioplasty with stenting, which is the ...

(such as

stent In medicine, a stent is a metal or plastic tube inserted into the lumen of an anatomic vessel or duct to keep the passageway open, and stenting is the placement of a stent. A wide variety of stents are used for different purposes, from expandab ...

placement after a

myocardial infarction A myocardial infarction (MI), commonly known as a heart attack, occurs when blood flow decreases or stops to the coronary artery of the heart, causing damage to the heart muscle. The most common symptom is chest pain or discomfort which may ...

or heart attack), or due to pacemaker or pacemaker wire placement.

Signs and symptoms

The typical signs of post-pericardiotomy syndrome include

fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

pleuritis Pleurisy, also known as pleuritis, is inflammation of the membranes that surround the lungs and line the chest cavity (pleurae). This can result in a sharp chest pain while breathing. Occasionally the pain may be a constant dull ache. Other sym ...

(with possible

pleural effusion A pleural effusion is accumulation of excessive fluid in the pleural space, the potential space that surrounds each lung. Under normal conditions, pleural fluid is secreted by the parietal pleural capillaries at a rate of 0.6 millilitre per kilog ...

pericarditis Pericarditis is inflammation of the pericardium, the fibrous sac surrounding the heart. Symptoms typically include sudden onset of sharp chest pain, which may also be felt in the shoulders, neck, or back. The pain is typically less severe when sit ...

(with possible

pericardial effusion A pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. The pericardium is a two-part membrane surrounding the heart: the outer fibrous connective membrane and an inner two-layered serous membrane. The two layers of t ...

), occasional but rare pulmonary infiltrates, and fatigue. Cough,

pleuritic Pleurisy, also known as pleuritis, is inflammation of the membranes that surround the lungs and line the chest cavity (pleurae). This can result in a sharp chest pain while breathing. Occasionally the pain may be a constant dull ache. Other sym ...

or retrosternal chest pain, joint pain and decreased oxygen saturation can also be seen in some cases. Other signs include

arthritis Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In som ...

, together with

petechiae A petechia () is a small red or purple spot (≤4 mm in diameter) that can appear on the skin, conjunctiva, retina, and mucous membranes which is caused by haemorrhage of capillaries. The word is derived from Italian , 'freckle,' of obscure origin ...

on the skin and palate.

Complications

Complications include

, pericardial effusion,

, pulmonary infiltration, and very rarely

pericardial tamponade Cardiac tamponade, also known as pericardial tamponade (), is the buildup of fluid in the pericardium (the sac around the heart), resulting in compression of the heart. Onset may be rapid or gradual. Symptoms typically include those of obstruct ...

. Of these cardiac tamponade is the most life-threatening complication. The pericardial fluid increases intra-pericardial pressure therefore preventing complete expansion of the atria and the ventricles upon the diastole. This causes equilibration of the pressure in all four heart chambers, and results in the common findings of the tamponade which are

pulsus paradoxus Pulsus paradoxus, also paradoxic pulse or paradoxical pulse, is an abnormally large decrease in stroke volume, systolic blood pressure and pulse wave amplitude during inspiration. The normal fall in pressure is less than 10 mmHg. When the drop ...

, Beck's triad of hypotension, muffled heart sounds, and raised

jugular venous pressure The jugular venous pressure (JVP, sometimes referred to as ''jugular venous pulse'') is the indirectly observed pressure over the venous system via visualization of the internal jugular vein. It can be useful in the differentiation of different for ...

, as well as EKG or Holter monitor findings such as

electrical alternans Electrical alternans is an electrocardiographic phenomenon of alternation of QRS complex amplitude or axis between beats and a possible wandering base-line. It is seen in cardiac tamponade and severe pericardial effusion and is thought to be relat ...

. Physically the patients who progress to severe pericardial tamponade obtundate, become mentally altered, and lethargic. If left untreated, severe decrease in cardiac output, vascular collapse, and hypoperfusion of body including the brain results in death.

Pathogenesis

The cause is believed to be an autoimmune response against damaged cardiac tissue. This is supported by excellent response to immunosuppressive (steroid) therapy. This condition is a febrile illness caused by immune attack of the

pleura The pulmonary pleurae (''sing.'' pleura) are the two opposing layers of serous membrane overlying the lungs and the inside of the surrounding chest walls. The inner pleura, called the visceral pleura, covers the surface of each lung and dips bet ...

and the

. Possible cell mediated immunity led by Helper T-cells and Cytotoxic T-cells is postulated to be important in the pathogenesis of this condition. There is also possibility of anti-cardiac antibodies created idiopathically, or due to concurrent cross-reactivity of the antibodies produced against viral antigens, however the latter assumption is not fool-proof or completely reliable due to conflicting studies.

Diagnosis

A chest X-ray might depict pleural effusion, pulmonary infiltration, or pericardial effusion. During medical doctor examination, a

pericardial friction rub A pericardial friction rub, also pericardial rub, is an audible medical sign used in the diagnosis of pericarditis. Upon auscultation, this sign is an extra heart sound of to-and-fro character, typically with three components, two systolic and ...

can be auscultated indicating pericarditis. Auscultation of the lungs can show

crackles Crackles are the clicking, rattling, or crackling noises that may be made by one or both lungs of a human with a respiratory disease during inhalation. They are usually heard only with a stethoscope ("on auscultation"). Pulmonary crackles a ...

indicating pulmonary infiltration, and there can be retrosternal/pleuritic chest pain worse on inspiration (breathing in). Patient can also depict sweating (

diaphoresis Perspiration, also known as sweating, is the production of fluids secreted by the sweat glands in the skin of mammals. Two types of sweat glands can be found in humans: eccrine glands and apocrine glands. The eccrine sweat glands are distribut ...

) and agitation or anxiety.

Treatment

Colchicine

Colchicine Colchicine is a medication used to treat gout and Behçet's disease. In gout, it is less preferred to NSAIDs or steroids. Other uses for colchicine include the management of pericarditis and familial Mediterranean fever. Colchicine is taken ...

has been used effectively to prevent

, and inflammation that follows surgery of the pericardium.Eur Heart, J. (2009).

for pericarditis: hype or hope? Oxford Journal. Vol 30. 532-539. Although no current drug on the market prevents post-pericardiotomy syndrome, colchicine seems to provide an effective and safe way to treat pericarditis by reducing inflammation. Colchicine is a natural product extracted from plants, and is a secondary metabolite (an organic compound not directly related to growth and development in an organism). Colchicine interferes with the inflammatory process by altering several important steps in the pathway. Microtubules are structural components of the cytoskeleton that lengthen and shrink for important cell functions. Colchicine binds to β- tubulin and forms tubulin-colchicine complexes.,Eur Heart, J. (2010) Colchicine for the Prevention of the Post-pericardiotomy Syndrome (COPPS): a multiculture, randomized, double-blind, placebo controlled trial. Oxford Journal. Vol 31. 2749-2754. These complexes interfere with microtubule formation microtubules. Low doses of colchicine can inhibit the formation of microtubules, while high doses depolymerize or break down a polymer to a monomer.Spyridon Deftereos, S., Giannopoulos, G., Papoutsidakis, N., Panagopoulou, V., Kossyvakis, C., Raisakis, K., Stefanadis, C. (2013). Colchicine and the heart. Journal of the American College of Cardiology. Vol 62(20), 1817-1825. Therefore, any process involving cytoskeleton change, including mitosis and motility of white blood cells, are highly impacted. Microtubule disruption decrease neutrophil adhesion, an important step for inflammation. Neutrophils are recruited to the target location of inflammation via signals from the endothelium where they adhere and play a role in the inflammatory response. Colchicine diminishes neutrophil adhesion by decreasing expression of selectins, a family of cell adhesion molecules. In addition, colchicine prevents the movement and secretion of intercellular granules, substances, proinflammatory enzymes from neutrophils, thus making a significant impact on inflammatory processes within the body. The high concentration of colchicine in neutrophils, sixteen times greater compared the plasma levels, can account for the positive therapeutic effects. Many mediators are altered to assist neutrophils during inflammation, including the monokine tumor necrosis factor-alpha (TNFα).Molad, Y. (2002). Update on Colchicine and Its Mechanism of Action. Current Rheumatology Reports. Vol 4. 252-256. Cytokines help stimulate the acute phase reaction in response to inflammation. Colchicine inhibits macrophage production of TNFα, leading to the interference between TNFα and neutrophil interaction. There are many more effects of colchicine that are currently under research, and some aspects of this metabolite are not fully understood. There was great hope that Colchicine could be a primary preventive measure in treating Post-Pericardiotomy Syndrome due to its anti-inflammatory effects. In the COPPS-2 trial, however, perioperative use of colchicine compared with placebo reduced the incidence of postpericardiotomy syndrome but not of postoperative AF or postoperative pericardial/pleural effusion. The increased risk of gastrointestinal adverse effects reduced the potential benefits of colchicine in this setting. Thus colchicine is not likely going to be the ideal way to prevent this problem.

Epidemiology

More common in children and often common in patients receiving cardiac operations that involves opening the pericardium. CABG surgery is a common culprit.

References

External links

{{Heart diseases Heart diseases Vascular-related cutaneous conditions Syndromes