4. CLASSIFICATION
1 1primary
Primary
a) Lofeller's endocarditis
b) Endomyocardial fibrosis
Secondary
a) Infiltrative disease
b) storage disease
c) Post - radiation disease
13. defintion
Constrictive cardiomyopathy (CCM) is a rare
heart condition characterized by the stiffening or
hardening of the heart muscles. This condition
makes it difficult for the heart to properly relax
and fill with blood between heartbeats, leading
to impaired pumping of blood throughout the
body. As a result, individuals with constrictive
cardiomyopathy may experience symptoms like
fatigue, shortness of breath, fluid retention, and
swelling in the legs and abdomen. Treatment
may involve medications to manage symptoms
or, in severe cases, surgical interventions to
alleviate the constriction of the heart.
14. CAUSE
The etiology, or causes, of constrictive cardiomyopathy can vary. Some common causes include:
1.Infiltrative diseases: Conditions like amyloidosis or sarcoidosis where abnormal substances build up in the
heart muscle, making it stiff.
2. Previous cardiac surgery or radiation therapy: Surgical procedures on the heart or chest area, or
radiation therapy for cancer in the chest, can lead to scarring and stiffening of the heart muscle.
3. Infections:Certain infections, such as tuberculosis or viral infections affecting the heart, can cause
inflammation and scarring, leading to constriction.
4. Idiopathic causes: In some cases, the cause of constrictive cardiomyopathy might not be identified
(idiopathic CCM).
5. Genetic factors: In rare instances, genetic mutations can lead to abnormal changes in heart muscle
structure and function, contributing to the development of constrictive cardiomyopathy.
6. Metabolic disorders: Conditions like hemochromatosis (excess iron in the body) or glycogen storage
diseases can affect the heart's function and structure.
7. Chemotherapy or certain medications: Some chemotherapy drugs and medications can have cardiotoxic
effects, leading to heart muscle damage and stiffness.
15. • Shortness of breath (dyspnea): Difficulty
breathing, especially during physical activity or
when lying flat, due to fluid buildup in the
lungs.
• Fatigue: Feeling tired or exhausted, even with
minimal physical exertion.
• Swelling (edema): Accumulation of fluid in the
legs, ankles, and abdomen, causing swelling
and discomfort.
• Decreased exercise tolerance: Difficulty in
engaging in physical activities that were once
manageable.
• Abdominal discomfort: Swelling or pain in the
abdomen due to fluid accumulation (ascites).
• Fluid retention: Weight gain and bloating due
to fluid buildup in the body.
• Elevated jugular venous pressure: Visible
bulging of the neck veins due to increased
pressure in the veins leading to the heart.
• Fatigue and weakness: Feeling generally weak
or tired due to reduced cardiac output.
• Irregular heartbeat: Palpitations or a sensation
of irregular heartbeats.
• Cough: Persistent coughing, especially when
lying down, due to fluid accumulation in the
lungs (pulmonary congestion).
16. DIAGNOSIS
Diagnosing constrictive cardiomyopathy involves a combination of clinical assessment, imaging studies, and
sometimes invasive procedures to confirm the diagnosis. Specific diagnostic findings include:
1. Echocardiography:This imaging test can show signs of thickened heart walls, abnormal relaxation of the
heart, and changes in the movement of the heart chambers. A key finding is septal bounce, where the
septum (the wall between the heart's chambers) moves abnormally during the cardiac cycle.
2. Cardiac MRI (Magnetic Resonance Imaging):** MRI can provide detailed images of the heart's structure
and function, helping to identify signs of thickening or stiffening of the heart muscle.
3. CT (Computed Tomography) scan:** CT imaging can reveal thickening of the pericardium (the sac around
the heart) and help in assessing the structure of the heart chambers.
4. Cardiac catheterization:Invasive testing involving the insertion of a catheter into the heart can measure
pressures within the heart chambers. A characteristic finding in constrictive cardiomyopathy is equalization of
pressures in the heart chambers during diastole.
5. EKG (Electrocardiogram): While not specific to CCM, an EKG can sometimes show changes such as low-
voltage QRS complexes or signs of atrial enlargement.
6. Laboratory tests:Blood tests might be done to check for specific biomarkers or underlying causes, such as
checking for abnormal protein levels in conditions like amyloidosis.
17. TREATMENT
o The treatment for constrictive cardiomyopathy (CCM) aims to manage symptoms, improve heart function, and address any
underlying causes if possible. Treatment options may include:
o 1. Medications: Diuretics are commonly prescribed to reduce fluid buildup and relieve symptoms of swelling and shortness of
breath. Other medications may include those to manage heart failure, control blood pressure, or address specific underlying
causes if identified (such as anti-inflammatory drugs for conditions causing inflammation).
o 2. Surgery: In some cases, surgical intervention might be necessary to remove the thickened or scarred pericardium (the sac
around the heart). This procedure, known as a pericardiectomy, can help alleviate the constriction and improve heart function.
However, surgery is typically considered for cases where symptoms are severe and significantly impacting the patient's quality
of life.
o 3. Lifestyle modifications: lifestyle changes such as limiting salt intake, maintaining a healthy weight, regular exercise (as
recommended by the doctor), and avoiding alcohol and smoking can help manage symptoms and improve overall heart health.
o 4. Monitoring and follow-up: Regular monitoring and follow-up with a cardiologist are essential to track the progression of the
condition, adjust medications, and address any new symptoms or complications promptly.
o 5. Treatment of underlying causes:*If the CCM is secondary to another condition (like amyloidosis or sarcoidosis), managing
that underlying condition becomes a crucial part of the treatment strategy.
