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Papillary muscle hypertrophy is a rare condition where the diameter of one or both of the papillary muscles is >1.1 cm. It is a variant of hypertrophic cardiomyopathy (HCM), characterised by predominant papillary muscle hypertrophy without the typical eccentric left ventricular hypertrophy. It is often associated with chronic rheumatic mitral valve disease and glycogen storage disorders. There is a scarcity of literature on this condition, with a reported incidence of 0.4% based on a single study of 6731 patient echos in 1990.
| Characteristics | Values |
|---|---|
| Definition | A diameter in the horizontal or vertical axes of >1.1 cm in either one or both of the papillary muscles |
| Incidence | 0.4% based on a single study of 6731 patient echos reported in 1990 |
| Type | A variant of hypertrophic cardiomyopathy (HCM) |
| Consequence | Chronic rheumatic mitral valve disease and glycogen storage disorders |
| Diagnosis | Presence of a hypertrophied and nondilated left ventricle in the absence of another cardiac or systemic disease |
| Symptoms | LV outflow tract obstruction, often associated with hypertrophic cardiomyopathy |
| Abnormalities | Congenital disorders, neoplasms, mitral regurgitation, and parachute mitral valve |
| Treatment | Conservative management with imaging surveillance of mitral valve stenosis/regurgitation and annual follow-up in cardiology |
| Risk | Associated with sudden cardiac arrest of unknown cause |
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What You'll Learn
Papillary muscle hypertrophy is a rare finding
The papillary muscles are small muscular structures located within the left ventricular cavity. Typically, there are two papillary muscles: the anterolateral (AL) and posteromedial (PM) groups. However, there is a wide variation in papillary muscle morphology, and these variations can be asymptomatic or associated with symptoms related to obstruction of the LV outflow tract.
Papillary muscle hypertrophy is often associated with hypertrophic cardiomyopathy (HCM). It has been described as a variant of HCM, characterised by predominant papillary muscle hypertrophy without the typical eccentric LV hypertrophy seen in most cases of HCM. In some cases, papillary muscle hypertrophy may be a consequence of chronic rheumatic mitral valve disease and glycogen storage disorders.
The pathophysiology of papillary muscle hypertrophy related to rheumatic valve disease is not yet fully understood. However, it is thought to be associated with the decreased prevalence of rheumatic heart disease in contemporary literature. In patients with rheumatic valve disease and no family history of HCM, papillary muscle hypertrophy may be a potential cause of the disease. Additionally, the absence of myocardial fibrosis and typical ECG features of HCM may suggest papillary muscle hypertrophy as a more likely diagnosis.
Solitary papillary muscle hypertrophy is an uncommon variant of HCM and may present with electrocardiographic changes. Cardiac MRI can be a useful tool for further evaluation and ruling out uncommon variants of HCM.
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It is a variant of hypertrophic cardiomyopathy
Papillary muscle hypertrophy is a rare condition that is characterised by a diameter of >1.1 cm in either one or both of the papillary muscles. It is a variant of hypertrophic cardiomyopathy (HCM), which is a common genetic heart disease with an estimated prevalence of 1:2001 in the general population. HCM is typically diagnosed based on the presence of a hypertrophied and nondilated left ventricle, in the absence of another cardiac or systemic disease.
The papillary muscles (PMs) are an anatomical part of the left ventricular (LV) chamber. PM hypertrophy is a rare echocardiographic finding, with very few cases reported in the literature. It is often an incidental finding, as patients are often asymptomatic. However, it can be associated with significant morbidity and may even cause sudden cardiac death. In some cases, it may be a consequence of chronic rheumatic mitral valve disease and glycogen storage disorders.
The clinical significance of PM hypertrophy is not yet fully understood, and there is a gap in our knowledge of HCM. The structural characteristics of PMs have received limited attention, and there is a scarcity of literature on this condition. PM hypertrophy is thought to be associated with the development of negative precordial T waves and may be a precursor of apical hypertrophic cardiomyopathy. It is also associated with increased left ventricular outflow tract obstruction in HCM.
PM abnormalities are common in patients with HCM and can include anterior and/or apical displacement, bifidity, the presence of accessory PM, and direct insertion into the anterior mitral valve leaflet. These abnormalities may contribute to left ventricular outflow tract obstruction and should be addressed when treating HCM patients.
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It can be caused by chronic rheumatic mitral valve disease
Papillary muscle hypertrophy is a rare condition, with a reported incidence of 0.4% based on a 1990 study. It is defined as a diameter of >1.1 cm in either or both of the papillary muscles. This condition is often asymptomatic, but it has been postulated as a potential cause of sudden cardiac death.
The condition can be caused by chronic rheumatic mitral valve disease, which is associated with pronounced fibrosis. The mitral valve structure is altered by the rheumatic process, leading to increased stress on the supporting elements, resulting in papillary muscle hypertrophy. This is evident in the case of a 43-year-old woman who presented with right lower limb swelling and was found to have rheumatic mitral valve disease and incidental papillary muscle hypertrophy.
In patients with rheumatic heart disease, mitral regurgitation tends to demonstrate a central jet. Operative repair is usually successful in improving symptoms and reducing regurgitation. Mitral annuloplasty and papillary muscle relocation are the surgical options.
Cardiac MRI is an important imaging modality for diagnosing papillary muscle hypertrophy due to its high resolution and soft tissue contrast. It helps differentiate between various causes of the condition. In patients with rheumatic valve disease, cardiac MRI can demonstrate the absence of myocardial fibrosis, which is frequently found in patients with hypertrophic cardiomyopathy.
Overall, papillary muscle hypertrophy due to chronic rheumatic mitral valve disease is a rare condition that can have serious consequences. It is characterised by increased stress on the supporting elements of the mitral valve, leading to pronounced fibrosis and potential sudden cardiac death. Early detection and treatment are crucial for managing this condition.
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It can be identified with the help of echocardiography
Papillary muscle hypertrophy is a rare condition, with very few cases reported in the literature. It is defined as a diameter in the horizontal or vertical axes of >1.1 cm in either one or both of the papillary muscles. This condition is identified as a variant of hypertrophic cardiomyopathy (HCM), which is characterised by increased thickness of the left ventricular walls.
Echocardiography is a crucial tool for identifying papillary muscle hypertrophy. It involves the use of ultrasound to create images of the heart, particularly the left ventricle and papillary muscles. The test can be performed at rest or under stress to evaluate the heart's function and structure.
During an echocardiogram, the parasternal short-axis view of the left ventricle at the level of the papillary muscles is often obtained. This view allows for the measurement of the papillary muscles, particularly the posteromedial and anterolateral muscles. In a reported case, a young woman with rheumatic mitral valve disease was found to have unusual hypertrophy of the postero-medial papillary muscle, measuring 1.45 cm x 1.98 cm, while the anterolateral muscle measured 0.82 cm x 0.81 cm.
Additionally, echocardiography can help visualise the impact of papillary muscle hypertrophy on the mitral valve and left ventricular function. For example, in some cases, there may be mitral regurgitation, thickening of the mitral valve leaflets, or obstruction of the left ventricular outflow tract.
Furthermore, stress echocardiography is an important tool to determine the presence of ventricular gradients and assess the heart's function under physiological stress. This can provide valuable information for the diagnosis and management of HCM.
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It is associated with sudden cardiac arrest
Papillary muscle hypertrophy is a rare condition that is characterised by an increased thickness of the left ventricular walls. It is considered a variant of hypertrophic cardiomyopathy (HCM) and is often associated with chronic rheumatic mitral valve disease. This condition can lead to dynamic mid-wall obstruction and symptomatic heart failure.
The papillary muscles are small muscular structures located within the left ventricular cavity. They play a crucial role in the functioning of the mitral valve and the left ventricle. Typically, there are two groups of papillary muscles: the anterolateral and the posteromedial. However, there is significant variation in their morphology, and these variations can be asymptomatic or cause symptoms related to obstruction of the left ventricular outflow tract.
Accessory papillary muscles and papillary muscle hypertrophy have been associated with sudden cardiac arrest (SCA) of unknown causes. A study reviewed the data of 190 patients with SCA and found that the prevalence of accessory papillary muscles and papillary muscle hypertrophy was significantly higher in the group with unknown causes of SCA compared to those with known causes or without SCA. This suggests that these structural abnormalities may play a role in triggering sudden cardiac arrest.
The exact mechanisms linking papillary muscle hypertrophy to sudden cardiac arrest are not yet fully understood. However, it is known that papillary muscle abnormalities can lead to mitral regurgitation, and if untreated, papillary muscle rupture can be fatal. Additionally, hypertrophic cardiomyopathy, which is associated with papillary muscle hypertrophy, can lead to severe cardiac dysfunction and the development of ventricular dysrhythmias. These factors may contribute to the increased risk of sudden cardiac arrest in individuals with papillary muscle hypertrophy.
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Frequently asked questions
Papillary muscle hypertrophy is a rare form of hypertrophic cardiomyopathy (HCM) characterised by predominant papillary muscle hypertrophy without any other left ventricular segment anomalies.
Common causes of papillary muscle hypertrophy include systemic hypertension and HCM.
Symptoms of papillary muscle hypertrophy range from asymptomatic to obstruction of the left ventricular outflow tract.
Papillary muscle hypertrophy is diagnosed by measuring the diameter of the papillary muscles. If the diameter in the horizontal or vertical axes is >1.1 cm in either one or both of the papillary muscles, it is considered abnormal.
The treatment for papillary muscle hypertrophy depends on the underlying cause and the severity of the condition. In some cases, conservative management and imaging surveillance may be recommended, while in other cases, surgical intervention may be necessary.
