Pericarditis, constitutional I31.1

Constrictive pericarditis was probably the first to be described by Galen. As early as 190 AD, Galen observed a thickening of the pericardium in animals and considered it possible in humans as well (Gall 1992).

A persistent inflammation of the pericardium leads to fibrosis, thickening, stiffening and finally to a sticking together of the two pericardial sheets. In addition, calcifications can develop. This process may be regionally limited or may involve the entire pericardium. In the case of severe calcifications, the heart is literally constricted by the calcifications and is referred to as an "armoured heart". The myocardium can also be involved in this process. This then leads to a regional or global myocardial atrophy. Prognostically, this is considered an unfavourable sign (Kühl 2004). Fibrosis results in a haemodynamically increasing obstruction of the filling of all four heart cavities during diastole. This in turn leads to an increase in pressure in the right atrium and thus also in the venous system (Bob 2001).

In contrast to pericardial tamponade, however, the rapid filling of the ventricles is not hindered at the beginning of diastole. Instead, the increased early filling in the middle diastole ends abruptly, i.e. at a point in time when the intracavitary pressure exceeds the intrapericardial pressure (the so-called dip-plateau phenomenon in invasive pressure monitoring). As a result of this obstruction of the filling, the diastolic pressure values in the heart cavities adjust to an increased level - similar to pericardial tamponade (Kühl 2004).

Constrictive pericarditis is one of three forms of chronic pericarditis (Bob 2001). The other two forms, which include persistent (also known as recurrent fibrinous pericarditis) and pericarditis with chronic inflammatory effusion, can change to a constrictive form in the course of their disease (Bob 2001).

According to Kühl (Kühl 2004), the disease can progress at different rates:

• acute (days to weeks)
• subacute (months)
• chronic (years)

Constrictive pericarditis can be caused by the following pathogens:

• idiopathic / viral (affects about 15% - 49% of cases [Pinger 2019])
• bacterial pathogens, especially Mycobacterium tuberculosis (in developing countries, these cause constitutional pericarditis in approx. 38% to 83% of cases [Pinger 2019])
• Autoimmune diseases (especially lupus erythematosus and rheumatoid arthritis)
• Infiltration of the pericardium in the context of malignant diseases
• Hemopericardium
• Exposure of the mediastinum to radiation (in approx. 1% - 31% of cases [Pinger 2019])
• in the context of a mycotic pericarditis
• uraemic
• induced by medication (e.g. penicillin, hydralazine, minoxidil etc.)
• after heart surgery (in approx. 11% - 39% of cases [Pinger 2019])
• parasitic pathogens

Due to peripheral and central venous congestion, the patient increasingly shows signs of chronic right heart failure with the following symptoms:

• peripheral edema
• unexplained upper abdominal pain
• Liver Enlargement
• Ascites
• Stasis proteinemia
• Reduction in performance
• Tiredness
• Pleural effusions
• Pulsus paradoxus here, during inspiration, a pathological drop in systolic blood pressure of more than 10 mmHg occurs; according to Bob (2001), this phenomenon occurs in about 30% of cases; Pinger (2019), on the other hand, points out that a pulsus paradoxus is very rare, in contrast to tamponade; pulsus paradoxus only occurs in the context of constrictive pericardial inflammation if a pleural effusion or severe pulmonary disease is present at the same time)
• Arrhythmias (are an indication of myocardial involvement [Kühl 2004])
• Dyspnoea, especially under stress, but also at rest
• coughing (the latter two symptoms only occur when the pressure in the right atrium rises above 18-30 mmHg
• kissing mouth sign - deep inspiration leads to a paradoxical increase in pressure in the jugular veins - Pinger 2019)

Chest x-ray: The heart is usually of normal size. The superior vena cava is typically dilated. Calcifications are almost always visible in the radiographic overview (Bob 2001), often as a visible calcified crescent (Paumgartner 2015).

Echocardiography: Also in echocardiography both fibrosis of the pericardium and possible calcifications are well visible. The relaxation of the ventricles is limited (Bob 2001). The two ventricles are otherwise unremarkable, unlike the atria, which are both dilated, as are the hepatic veins and the vena cava. In most cases there is also an abnormal septal bounce. This is the abrupt movement of the septum in early diastole. During inspiration this movement is directed towards the left ventricle, during expiration towards the right ventricle. The sensitivity of this movement is about 62%, the specificity about 93% (Pinger 2019).

Note: If the echocardiogram is completely unremarkable, this practically rules out constrictive pericarditis (Kühl 2004).

Transesophageal echocardiography: Transesophageal echocardiography has a higher sensitivity (about 95%) than echocardiography in visualizing the thickened pericardium, especially ventral to the free wall of the right ventricle (Kühl 2004). The specificity for the detection of a pericardium thickened to at least 3 mm is about 86% (Pinger 2019).

Computed tomography: Computed tomography has a high sensitivity in detecting calcification (Kühl 2004). The EACVI (European Association of Cardiovascular Imaging) recommends CT for planning pericardiectomy as a reasonable alternative to MRI (Pinger 2019).

Magnetic resonance imaging: The thickness of the pericardium (pathological values are 3 mm or more) can be determined well, which is particularly important in cases of constrictive pericarditis with no evidence of calcifications in X-rays and computer tomography (Kühl 2004). On the other hand, a thickened pericardium is not a reliable indication of constrictive pericarditis. In surgically proven constrictive pericarditis, for example, there is no thickening of the pericardium in about 18%. The thickness of the pericardium in such cases is < 2mm (Pinger 2019). In 2015 the EACVI (European Association of Cardiovascular Imaging) recommended to use MRI for planning pericardiectomy, CT as an alternative (Pinger 2019).

Medical history: ask for a history of pericarditis (Dopp 2018).

Auscultation: a so-called galloping rhythm often occurs, accompanied by a protodiastolic tone. This tone is caused by the sudden limitation of filling by the pericardium (Bob 2001) and is similar in sound to the mitral opening tone (Pinger 2019).

Laboratory: a low BNP value combined with clear signs of heart failure indicates constrictive pericarditis (Dopp 2018).

ECG: According to Bob (Bob 2001), the ECG contains the following

• often a low voltage
• T-wave negativation
• Atrial fibrillation (in about 40 % of patients)
• tachycardia (only by increasing the heart rate can the cardiac output be increased)

Imaging techniques (see there)

Right heart catheter examination: A reliable diagnosis can be made with the help of a cardiac catheter examination.

However, this invasive examination method is only required preoperatively in about 50% of patients who show typical clinical, typical echo findings and evidence of pericardial thickening (Pinger 2019). In this case a characteristic pressure profile with increased pressure in both the right atrium and the right ventricle is found. The increased pressure shows an end-diastolic pressure equalization between the right atrium, right ventricle and the pulmonary artery. Also characteristic are a double peaked atrial and jugular vein pressure curve and an early systolic pressure drop followed by a late diastolic pressure level, a so-called dip-plateau (Bob 2001), also called dip-and-plateau configuration or square root sign. The dip-plateau also occurs at

• extensive right ventricular infarction
• pronounced (sub-) acute tricuspid insufficiency
• Bradycardia (Pinger 2019)

Talreja (2008) proposes a "Systolic area index" for catheter-assisted diagnosis of constrictive pericarditis, in which the ratio of areas under the RV and LV curve in inspiration versus in expiration is determined in systole. A value of > 1.1 indicates constrictive pericarditis with a sensitivity of 97% and a specificity of 100%. However, in patients previously treated with diuretics, these hemodynamic changes may be uncharacteristic. Therefore, the rapid administration of a 1,000 ml NaCl solution is recommended for patients pretreated with diuretics whose right atrial pressure shows values of < 15 mmHg.

Endomyocardial biopsy: By means of endomyocardial biopsy it is possible to differentiate between restrictive cardiomyopathy and constrictive pericarditis (Paumgartner 2015).

• Restrictive cardiomyopathies (endomyocardial biopsy makes it possible to differentiate between restrictive cardiomyopathy and constrictive pericarditis [Paumgartner 2015])
• V.-cava syndrome in case of tumour infestation of the upper mediastinum (tumour history, X-ray thorax, MRT etc.)
• Pulmonary hypertension (chronic ventilation disorders etc.)
• ischemic heart failure
• veno-occlusive disease (rarely occurring)

(Cool 2004)

• Tricuspid stenosis (Kasper 2015)
• nephrotic syndrome
• Cirrhosis of the liver
• HOCM (Hypertrophic Obstructive CardioMyopathy)
• constrictive-effusive pericarditis (despite pericardiocentesis and drainage, persistent hemodynamic alterations - especially persistent elevated pressure in the right atrium - with normal intrapericardial pressure)
• Pericardial tamponade; in the case of a tamponade:
  • Pulsus paradoxus
  • Effusion
  • Atrial pressure curve X or Xy
• but NOT the signs of constrictive pericarditis:
  • Dip and Plateau
  • diastolic extraton
  • Calcification of the pericardium
  • Atrial pressure curve W/M=XY/xY

(ping 2019)

Conservative treatment:

Initial drug treatment may be useful under certain circumstances

• in patients with bacterial or especially tuberculous pericarditis to prevent constriction
• in patients with acute constrictive pericarditis (see below)
• with a transient constriction
• for the treatment of cardiac congestion
• in patients with too high a risk of surgery or a contraindication for surgery

We then speak of constrictive pericarditis, which is still in the inflammatory stage, when:

• C-reactive protein still increases
• Detection of oedema or an inflammatory reaction in the CT or CMR

In these cases an anti-inflammatory treatment of 2-3 months is recommended (Adler 2015).

Pinger (2019) also recommends an initially conservative approach for 3 months in patients with acute constrictive pericarditis (e.g. in post-cardiovascular conditions), as one study showed spontaneous improvement in 36 of 212 patients. During this period, the following therapeutic measures should be taken:

• Diuretics
• NSAID
• in case of failure of NSAID prednisolone

It is suspected that the above-mentioned improved courses were due to a still florid pericardial inflammation with a constriction capable of recovery (so-called transient constrictive pericarditis).

Patients with constrictive pericarditis should never be treated with beta-blockers or Ca-antagonists, as the existing tachycardia is a compensatory mechanism. Only by increasing the heart rate can the cardiac output be increased in patients with constrictive pericarditis (Pinger 2019). Constrictive pericarditis caused by Mycobacterium tuberculosis requires at least 2 months of treatment with antituberculostatics before surgical resection of the pericardium. Only after this period is surgical intervention possible (Maisch 2008). Strang (2004) describes a reduced mortality with tuberculous etiology (within 2 years 4% vs 11%) with additional prednisolone administration under antituberculostatic treatment.

He recommends the following dosage in adults (in children a dose adjustment is necessary):

• 60 mg/d for week 1-4
• 30 mg/d for week 5-8
• 15 mg/d for week 9-10
• 5 mg/d for week 11

Surgical treatment:

In constrictive pericarditis, surgical treatment is predominantly the therapy of choice. In this case, either a decortication or a pericardiocentesis of both the parietal and the visceral leaf is performed.

Surgical intervention should be performed in time, at any rate before myocardial atrophy occurs, as atrophy of the myocardium would have a prognostically unfavourable effect (Bob 2001).

Today, the mortality rate of surgery is between 5% - 15% (Pinger 2019). In 2008 it was still 15% - 20% (Maisch 2008).

A haemodynamically complete normalisation occurs postoperatively in only about 60% of patients (Pinger 2019).

Both early detection and targeted treatment are decisive for the prognosis of constrictive pericarditis (Bob 2001).

A myocardial atrophy occurring in the course of the disease has proven to be unfavourable (Kühl 2004).

However, the long-term prognosis after pericardiocentesis is limited compared to age- and gender-related subjects (Kühl 2004).

The 5 and 10 year postoperative survival rates are 78% and 57%, respectively. The postoperative survival rate after 7 years depends very much on the causative disease. In case of idiopathic etiology it is about 88%, after cardiotomy 66% and after radiotherapy only 27% (Pinger 2019).

The incidence of postoperative heart failure NYHA III to IV after 5 and after 10 years is 25% and 41% respectively (Pinger 2019).

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