Endocarditis, rheumatic I09.1

As early as 1798, the London physician Robert Willan described erythema nodosum in connection with rheumatism (Holzmann 2013). The first case of erythema anulare was described by Colcot Fox in 1895 (Dermatological Society 1921).

Rheumatic endocarditis represents a specific inflammatory response to infection with group A beta-hemolytic streptococci. It occurs approximately 2 weeks after an infection with streptococcus A (e.g., after angina tonsillaris, pharyngitis, scarlet fever, erysipelas , etc.) and a new flare-up of disease, also known as rheumatic fever, can affect the joints, heart, cutis, and CNS (Herold 2022 / Thomas 2010).

Rheumatic endocarditis is one of the abacterial endocarditis (Thomas 2004) and is the most common form of abacterial endocarditis (Thomas 2010). It is a subset of pancarditis and rheumatic fever (Herold 2022).

Abacterial forms of endocarditis, also referred to as "noninfectious endocarditis," also include:

- E. Libman- Sacks, which is caused by systemic lupus erythematosus (SLE) (Herold 2022).

- Endomyocarditis eosinophilica, also known as "Löffler- syndrome", which can occur in the context of various diseases and is always associated with an increase in eosinophilic granulocytes (Herold 2022).

Until the early 20th century, rheumatic endocarditis was common in all countries, then the incidence decreased due to improved living conditions, better hygiene, less cramped living conditions in industrialized countries. The introduction of antibiotics had an additional effect.

In developing countries, however, RHD is still the most common cause of heart disease and is a major cause of mortality in adults.

Worldwide, RHD affects approximately 15-19 million people and accounts for ¼ million deaths (Kasper 2015).

The peak age of disease is between 5 - 15 years (Herold 2022).

Endocarditis rheumatica, also known as E. verrucosa, is caused by an autoimmune reaction that occurs in response to disease triggered by beta-hemolytic streptococci . It is also referred to as a streptococcal allergic second disease (Herold 2022).

After infection with beta-hemolytic streptococci, antigenic substances appear in the membrane of the streptococci. These are responsible for the formation of immune complexes. These immune complexes deposit between myocardial fibers, smooth muscle fibers and endocardium. As a result, the M- protein of streptococci cross-reacts with sarcolemmal myosin and tropomyosin. The subthalamic nucleus and caudate nucleus may also be affected (Thomas 2010).

The endothelial surface of the endocardium shows small, delicate, glassy, slightly reddish deposits, typically localized at the free valve edge (Thomas 2010).

Rheumatic heart disease (RHD) develops in up to 60% of patients with acute rheumatic fever (ARF) and may involve the endocardium, pericardium, or myocardium (Kasper 2015). Decisive for the prognosis of pancarditis is exclusively rheumatic endocarditis (Herold 2022).

E. rheumatica is commonly found at the closure margins of the mitral and aortic valves (Herold 2022).

The course is rather subacute or sometimes chronic (Burk 2008). Cardiac symptoms are not obligatory.

Due to the pancreatitis may exist:

- quiet systolic and / or diastolic heart murmur

- by the simultaneously existing pericarditis:

- pericardial rubbing

- precordial pain

- by the concomitant myocarditis:

- occurrence of extrasystoles

- signs of cardiac insufficiency (in case of severe myocarditis)

(Herold 2022)

- Aschoff nodules (due to myocarditis)

(Thomas 2004)

- Chorea minor (rare)

- Erythema anulare

- Erythema nodosum (Braun 2019)

To confirm the diagnosis, a detailed history should be taken, especially with regard to recent beta-streptococcal A infections such as angina tonsillaris, pharyngitis, scarlet fever, erysipelas, etc. (Burk 2008 / Thomas 2010).

On auscultation, particular attention should be paid to a (quiet) systolic or diastolic heart murmur (Burk 2008).

Further diagnosis consists of imaging procedures such as ECG and echocardiography as well as laboratory findings (see below).

ECG

In the ECG can be detectable:

- extrasystoles

- prolonged PQ interval

- changes in ST / T (Herold 2022)

Echocardiography (if necessary as swallow echo [Burk 2008])

- pericardial effusion

- myogenic dilatation of the heart (Herold 2022)

- Thickening of the heart valves

- NO destruction of the valves (Braun 2019)

- ESR elevated

- CRP elevated

- Leukocytosis

- Increased antibodies against streptococci (ASL titers)

An increase in the ASL titer occurs in about 80%. It is not suitable as a progression parameter (Braun 2019) and is also not conclusive of rheumatic endocarditis (Burk 2008).

Histologically, there are:

- histiocytes with owl-like nucleoli, the so-called Anitschkow cells

- Aschoff nodules

- giant cells around fibrinoid necroses (Herold 2022)

- eosin red, Gieson- yellow fibrin deposits on the valve surface (Thomas 2004)

- up to 2 mm large thrombocyte deposits in the area of the valve surface

- in the late stage (after > 20 years) collagen fiber-rich scarring with adhesions of the valves and retractions (Thomas 2006)

Differential diagnostics are especially other forms of endocarditis such as infective endocarditis, endocarditis Libman- Sacks (Burk 2008).

In addition, all other diseases febrile and erythema nodosum (Braun 2019).

- Transition to bacterial endocarditis (Tauchnitz 2009).

- Damage to the heart valves (rheumatic endocarditis is the most common cause of acquired valvular heart disease [Kaltenbach 2000]).

Therapy is with anti-inflammatory drugs such as ASA and corticosteroids (Burk 2008).

Antibiotic therapy is used more to treat the preceding infection. Antibiotics have no effect on rheumatic endocarditis (Burk 2008).

Penicillin is the drug of choice for streptococcus A in this case, as resistance to other antibiotics often exists (Herold 2022).

- Dosage recommendation

- Children: 100.000 I. E. Penicillin V / kg KG / d

- Adults: 3 - 4 million penicillin V / d

Duration of therapy: 10 days

In the presence of penicillin allergy can be treated with macrolides. In this case, resistance is found in up to 15% (Herold 2022). Suitable are, for example, vancomycin or clindamycin (Braun 2019).

It is not uncommon for surgical treatment of the affected heart valves to be required.

- Aortic stenosis:

From stage III (symptoms on mild physical exertion), surgical therapy is indicated (Buchta 2004). For more details see aortic valve stenosis.

- Pulmonary stenosis:

The treatment options of pulmonary valve stenosis consist of

- I. balloon valvuloplasty / stent implantation

- II. surgery (Herold 2022) For details see pulmonary valve stenosis

- Mitral stenosis:

Rheumatic mitral stenosis becomes symptomatic only after about 20 years (Kaltenbach 2000). For more details, see mitral valve stenosis.

- Mitral regurgitation:

Surgical measures for mitral regurgitation consist of:

- percutaneous interventional catheter therapy with mitral leaflet clipping (Kasper 2015).

- surgical valve replacement therapy or valve reconstruction (Pinger 2019) For more details see mitral regurgitation.

- Tricuspid valve stenosis:

Tricuspid valve stenosis due to rheumatic disease is usually operated on only when rheumatic mitral and aortic valve disease require surgery (Mewis 2006). For more details see Tricuspid valve stenosis.

The course of rheumatic endocarditis is:

- 1. streptococcal infection

After a latency period of 1 - 3 weeks there is the

- 2. appearance of rheumatic fever (duration approx. 6 - 12 weeks) with appearance of rheumatic endocarditis

- 3. heart valve defect of the respective affected heart valve(s). This occurs after 1 - 3 years

(Herold 2022) or, in the case of mitral stenosis, becomes symptomatic only after up to 20 years (Kaltenbach 2000).

The prognosis depends on early penicillin therapy. Each recurrence increases the risk of valve failure (Herold 2022). In adolescents, recurrence occurs in approximately 70% of cases and in adults in 20% (Thomas 2010).

If the leaflet or pocket valves are affected, local adhesions may occur, ultimately resulting in valve stenosis. If the chordae are affected, the chordae thicken and coalesce. This usually leads to scarring retraction, which presents as mitral valve regurgitation (Thomas 2010).

Eventual scarring shrinkage of the valves is irreversible (Herold 2022).

For recurrence prophylaxis, affected individuals should receive recurrence prophylaxis with penicillin until 18 years of age or until 40 years of age if valve involvement is proven.

Dosage Recommendation:

Penicillin G 1.2 million I. E. i. m. every 3 - 4 weeks.

In addition, regular dental hygiene and focal sanitation are required (Braun 2019).

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