History and exam
Key diagnostic factors
common
history of acute rheumatic fever (ARF) during childhood
Most common cause of rheumatic valvular disease and tricuspid stenosis (TS). A positive history should increase index of suspicion.
dyspnea
elevated jugular venous pressure with prominent a-wave
Prominent a-waves are hallmarks of TS in patients in sinus rhythm. In patients with atrial fibrillation, a-wave is lost, but jugular venous pressures are still elevated.
low-frequency presystolic (diastolic) murmur at lower left sternal border
Can be confused or attributed to similar murmur heard with mitral stenosis. Must have high index of suspicion for TS if other signs of right heart involvement are present. Murmur increases in intensity with inspiration and squatting.
Other diagnostic factors
common
age: 20-39 years
This is the most common age for rheumatic heart disease (RHD) to present following childhood acute rheumatic fever (ARF).[6]
exercise intolerance
In severe forms, cardiac output may be limited leading to fatigue and exercise intolerance.
fatigue
In severe forms, cardiac output may be limited leading to fatigue and exercise intolerance.
jugular pulsations
Prominent venous pulsations in the neck may cause fluttering and patient discomfort.
abdominal swelling and discomfort
Due to hepatomegaly and ascites from chronic systemic venous congestion.
edema
Occurs especially in extremities.
cyanosis or hypoxemia
Due to right-to-left atrial level shunting.
atrial fibrillation
TS caused by rheumatic heart disease can present with atrial fibrillation in up to 40% to 70% of patients.
hepatomegaly
Can be pulsatile in severe forms of TS.
ascites, edema, anasarca
Related to degree of systemic venous hypertension.
uncommon
age: infancy or childhood
Congenital TS presents at an early age, and is often associated with cyanosis or other structural heart defects.
episodic facial flushing, watery diarrhea, or bronchoconstriction
Symptoms of patients with carcinoid heart disease. These patients will also present with the symptoms of TS.
absent right ventricular lift or heave
The absence of this sign suggests the right ventricle is protected from volume or pressure overload. In patients with mitral stenosis and pulmonary hypertension, right heart pressures are elevated and a right ventricular lift is usually present.
opening snap
Can often be confused with opening snap of rheumatic mitral stenosis. However, the opening snap of TS will intensify during inspiration.
endocarditis stigmata (e.g., splinter hemorrhages, Osler nodes, Janeway lesions)
Physical findings that relate to intracardiac vegetations embolizing to systemic peripheral arterial beds. Most commonly found in left-sided vegetations or if an intracardiac communication is present (e.g., patent foramen ovale).
Risk factors
strong
group A streptococcal (GAS) pharyngitis
Outbreaks of acute rheumatic fever (ARF) follow epidemics of GAS pharyngitis or scarlet fever. Antibiotic treatment programs for documented GAS pharyngitis have markedly reduced the incidence of ARF and rheumatic heart disease (RHD).[6] Most patients with ARF develop elevated antibody titers to anti-streptococcal antigens, including streptolysin O, hyaluronidase, and streptokinase. Human host antibodies directed against GAS antigens have been demonstrated to cross-react with antigens (e.g., myosin, tropomyosin, and laminin) of the human heart.[6][22][23][24]
metastatic carcinoid tumors
Carcinoid heart disease usually only occurs in patients whose primary intestinal tumor has metastasized to the liver.[19] Metastatic carcinoid tumors produce paraneoplastic substances (e.g., serotonin), which are thought to lead to the characteristic endocardial fibrous white plaque formation within the valve leaflets and tricuspid valve chordal apparatus, which lead to valve distortion, foreshortened leaflets, and the inability of the valve to coapt and open completely.
weak
artificial tricuspid valve
Tricuspid valve replacement leads to abnormal flow characteristics that predispose to bacterial superinfection in rare cases.
intravenous drug use
Intravenous drug use provides a direct route for introducing bacteria into the bloodstream with secondary seeding of the tricuspid valve. Bacterial endocarditis secondary to intravenous drug use may rarely lead to leaflet thickening and fibrosis even in the presence of a previously normal valve.
pacemaker/defibrillator leads crossing tricuspid valve orifice
Adhesions between lead and valve may lead to progressive fibrosis and restricted valve motion.[1]
genetic predisposition and environmental factors
Etiology of congenital tricuspid stenosis is presumed to be multifactorial with genetics and environmental factors both playing a role.
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