CARDIOMYOPATHY cardiac mri carduiac ct imaging

Cardiomyopathy refers to a group of heart muscle diseases that affect the structure and function of the heart, leading to impaired heart function. There are several types of cardiomyopathy, including:

1. Dilated cardiomyopathy: The heart muscle becomes weakened and stretched, leading to a enlarged heart.
2. Hypertrophic cardiomyopathy: The heart muscle becomes thickened, leading to obstruction of blood flow.
3. Restrictive cardiomyopathy: The heart muscle becomes stiff, making it harder for the heart to fill with blood.
4. Left ventricular non-compaction: A condition where the heart muscle does not properly compact, leading to decreased heart function.

Cardiomyopathy can be caused by a variety of factors, including:

- Genetics
- High blood pressure
- Heart valve problems
- Heart attack or coronary artery disease
- Viral infections
- Alcohol or drug abuse
- Certain medications

Symptoms of cardiomyopathy may include:

- Shortness of breath
- Fatigue
- Swelling in the legs and feet
- Palpitations
- Chest pain
- Dizziness or lightheadedness

If you suspect you or someone else may have cardiomyopathy, it's important to seek medical attention right away. A healthcare provider can diagnose cardiomyopathy using a physical exam, imaging tests, and other procedures. Treatment options vary depending on the type and severity of the condition, but may include medications, lifestyle changes, or surgery.Cardiomyopathy refers to a group of heart muscle diseases that affect the structure and function of the heart, leading to impaired heart function. There are several types of cardiomyopathy, including:

1. Dilated cardiomyopathy: The heart muscle becomes weakened and stretched, leading to a enlarged heart.
2. Hypertrophic cardiomyopathy: The heart muscle becomes thickened, leading to obstruction of blood flow.
3. Restrictive cardiomyopathy: The heart muscle becomes stiff, making it harder for the heart to fill with blood.
4. Left ventricular non-compaction: A condition where the heart muscle does not properly compact, leading to decreased heart function.

Cardiomyopathy can be caused by a variety of factors, including:

- Genetics
- High blood pressure
- Heart valve problems
- Heart attack or coronary artery disease
- Viral infections
- Alcohol or drug abuse
- Certain medications

Symptoms of cardiomyopathy may include:

- Shortness of breath
- Fatigue
- Swelling in the legs and feet
- Palpitations
- Chest pain
- Dizziness or lightheadedness

If you suspect you or someone else may have cardiomyopathy, it's important to seek medical attention right away. A healthcare provider can diagnose cardiomyopathy using a physical exam, imaging tests, and other procedures. Treatment options vary depending on the type and severity of the condition, but may include medications, lifestyle changes, or surgery.Cardiomyopathy refers to a group of heart muscle diseases that affect the structure and function of the heart, leading to impaired heart function. There are several typ

1. CARDIOMYOPATHY

2. CLASSIFICATION PRIMARY Predominantly/solely confined to heart muscles SECONDARY Myocardial involvement occurs as part of systemic/multiorgan disorder

3. HYPERTROPHIC CARDIOMYOPATHY heterogeneous group of diseases related to sarcomere gene mutations autosomal dominant diverse phenotypic expressions variable natural progression, characterized by left ventricular (LV) hypertrophy in the absence of another systemic or cardiac disease

4. The hypertrophy in HCM most commonly involves the interventricular septum to varying degrees concentric, seen in the apex or free wall of the LV, at the midventricular level, or may extend into the right ventricle (RV). asymmetric, concentric, apical, midventricular, and preclinical HCM

5. ASYMMETRIC HCM the most common -60%–70% of cases Ventricular septum is disproportionately enlarged- anteroseptal myocardium most commonly involved The septal hypertrophy can be limited to the subaortic, midventricular, or apical regions In some patients, the hypertrophy can be focal and may involve only one or two LV segment At times, it may involve the entire length of the septum from base to apex or may extend and involve anterior or inferior walls of the LV

6. The normal thickness of the LV is 11 mm or less, septal thickness is greater than or equal to 15 mm or ratio of the septal thickness to the thickness of the inferior wall at the midventricular level is greater than 1.5 . In children, septal hypertrophy is defined as wall thickness >2SD above the mean for age, sex, or body size anteroseptal myocardium sigmoidal contour of the septum narrow the LV cavity and encroach on the LVOT. This pattern is often associated with SAM of the mitral valve leaflets. subaortic obstruction

7. The combination of these structural changes, mitral valve abnormalities, and hyperdynamic systolic flow along the LVOT also causes posteriorly directed mitral valve regurgitation. However, asymmetric septal hypertrophy with a reversed S septum does not cause LVOT obstruction

8. MRI Still images at end-diastole are used to calculate septal wall thickness. Cine cardiac MR imaging including cardiac mass, LV ejection fraction, myocardial contractility, and presence or absence of papillary muscle abnor malities In asymptomatic patients, LV ejection fraction can be initially normal. In symptomatic patients, the LV is commonly hyperdynamic with effac ment of the LV cavity during systole cine SSFP sequence in long-axis three-chamber views SAM as well as mitral regurgi tation (Fig 6) and a high-velocity dephasing jet across the LVOT (LGE) in HCM is midwall patchy or punctate hyperenhancement in a thickened myocardium in a noncoronary distribution

9. LGE is most commonly located in both the ventricular septum and free wall, apex and at the superior and inferior insertion points of the RV into the ventricular septum. At times, LGE can be seen in papillary muscles or the RV wall, but never corresponds to a coronary vascular distribution. phase-contrast MR imaging can also effectively calculate velocity and peak gradients across the LVOT The peak pressure gradient is derived with a simplified Bernoulli equation,=(4V2). >30 mm Hg

10. LVOT Obstruction LVOT obstruction is defined as a gradient greater than 30 mm Hg They involve a reduced effective LVOT diameter due to a combination of basal septal hypertrophy and abnormal SAM of the mitral valve leaflets, The abnormal anterior position of the leaflets leads to changes in the diastolic inflow and systolic outflow hydrodynamics, with subsequent further displacement of the mitral leaflets into the LVOT in systole and increased subvalvular pressure gradient at the outflow tract

11. The presence of SAM is very specific (97%), particularly when asymmetric septal hypertrophy is present (specificity, 99%) The high contractile load >myocardial oxygen demand> worsens ventricular filling and relaxation, and decreases coronary perfusion pressure, I addition to subaortic obstruction, SAM of the mitral valve leaflets also leads to mitral regurgitation due to a mismatch of posterior to anterior leaflet length and mobility

12. CONCENTRIC HCM Concentric HCM is the second most common phenotype > diffuse thickening of the LV >decrease in the size of the LV cavity The diagnosis should be made in the absence of a secondary cause like hypertension, aortic stenosis, or the patient being an endurance athlete differentiate concentric hypertrophy in HCM from the hypertrophy seen in hypertension and in athletic heart. there is a 2–5-mm regression in LV wall thickness in athlete’s heart over a short deconditioning period of about 3 months, which is not seen with concentric HCM.

13. Cine cardiac MR imaging allows differentiation of LV hypertrophy caused by aortic stenosis from that caused by HCM. In aortic stenosis the jet of turbulent flow is seen exactly across the valve with associated decrease in aortic valve area in systole, whereas in HCM, the jet is seen in the subaortic region and results from thickening of the adjacent basal anteroseptal wall of the LV The majority of hypertension patients have concentric LV hypertrophy with normal ejection fraction and normal chamber size,. The LV wall thickness in hypertensive heart disease also rarely exceeds 16 mm

14. Other infiltrative and deposition diseases like amyloidosis, sarcoidosis, and Fabry disease can also manifest as diffuse myocardial hypertrophy., LGE plays an important role in differen tiating HCM from these entities Cardiac amyloidosis > concentric LV wall thickening, but may also manifest as nodular thickening of the right atrial free wall as well as interatrial septum,. At contrast-enhanced MR imaging, there is a characteristic pattern of diffuse subendocardial delayed hyperenhancement in a nonvascular distribution, This pattern is due to amyloid protein deposition rather than fibrosis

15. . Differentiating cardiac sarcoidosis and concentric HCM is important other thoracic manifestations of sarcoidosis like mediastinal and hilar adenopathy, . noncaseating gran ulomatous infiltration of the myocardium.>. the LV free wall is most commonly involved, followed by the interventricular septum, papillary muscles, RV, and atria in descending order At cardiac MR imaging, the granulomas appear as patchy areas of T2 hyperintensity. At contrast-enhanced MR imaging, cardiac sarcoidosis demonstrates symmetric subepicardial and midmyocardial delayed enhancement in a nonvascular distribution;

16. Infiltrative disorders like glycogen/lysosomal storage diseases including Fabry, Danon , and AMP kinase are the other most common nonsarcomeric diseases >can mimic that of Fabry disease >X-linked autosomal recessive >deficiency of lysosomal a-galactosidase A. It causes concentric LV hypertrophy, valvular disease, atrial/ventricular arrhythmias, and chest pain due to microvascular ischemia. Cardiac MR imaging shows a symmetrically thickened LV wall and delayed enhancement in the basal inferolateral LV midwall (16,40). In HCM, the fibrosis indicates end-stage intramyocardial ischemia, whereas in Fabry disease, the fibrosis is due to inflammation related tissue injury

17. APICAL HCM It is also known as Yamaguchi syndrome and is characterized by the presence of giant negative T waves on the electrocardiogram and a spade like configuration of the LV cavity, exertional angina or dyspnea and may have electrocardiographic findings similar to those in acute coronary syndromes . benign prognosis. However one third-myocardial infarction, atrial fibrillation, and stroke aims of imaging is to differentiate apical hypertrophy from unstable angina and apical thrombus.

18. The diagnosis of pure apical HCM LV thickening is predominantly confined to the apex measuring 15 mm or more, with a ratio of apical LV wall thicknesses to basal LV wall thicknesses of 1.3–1.5 “ace of spades” configuration of the LV cavity at end diastole,

19. complications of apical HCM include apical infarctions with resultant aneurysm formation, (burned-out apex) . An apical aneurysm further in creases the risk of thrombi formation and thromboembolic phenomena. The presence of apical aneurysms or a significant amount of delayed enhancement -Predictor cardiac MR imaging allows differentiation of apical HCM from LV noncompaction cardiomyopathy, which typically demonstrates prominent trabeculae and deep intertrabecular recesses.

20. MID VENTRICULAR HCM Isolated midventricular obstruction is a rare form of HCM characterized by LV hypertrophy predominantly localized in the midmyocardial segments This gives a characteristic hourglass or dumbbell shape to the LV at cardiac CT and MR imaging Midsystolic muscular apposition of the interventricular septum with the free wall of the LV > midcavity obstruction.( gradiant >30 mm Hg).SAM and an LVOT gradient are typically absent in this form of HCM. The pressure overload in the apical LV chamber increases myocardial stress and de creases perfusion, leading to fibrosis and development of an apical aneurysm >thrombus

21. apical aneurysm formation is rare in HCM as a whole (2%) but has a particular predilection for the apical and midventricular variants At cardiac MR imaging, an apical aneurysm is seen as a focal, dyskinetic, thin walled bulge often showing delayed transmural enhancement due to scar formation

22. MASS LIKE HCM In this variant, there is exuberant focal thickening of a segment of the LV, simulating a cardiac mass. Cardiac CT and especially MR imaging play a crucial role in differentiating this form of HCM from a cardiac tumor ( the presence of contractility at spin-echo MR tagging in mass like HCVM

23. PRECLINICAL HCM Genetic screening > first-degree relatives,. , patients may initially have electro cardiographic abnormalities, increased ejection fraction, and delayed myocardial relaxation even before myocardial hypertrophy is evident. In such genotype-positive and phenotype-negative individuals, a clinical assessment as well as screening using serial electrocardiograms, two-dimensional echocardiography, or cardiac MR imaging is recommended at periodic intervals depending on the patient’s age and clinical status: yearly in children and adolescents and about every 5 years in adults . The role of cardiac MR imaging is evolving in asymptomatic HCM mutation carriers. Some studies have pointed at the presence of myocardial crypts as a prephenotypic marker of HCM in the absence of LV hypertrophy ). At MR imaging or CT, crypts are seen as blind pits or V-shaped blood-filled fissure-like invaginations in the LV myocardium, at the RV insertion points

24. DCM Presence of a left or biventricular dilatation with severely impaired systolic function in the absence of abnormal loading conditions Primary or secondary Ischemic/non ischemic cardiac magnetic resonance (CMR) allows identifing and characterizing the presence and location of myocardial damage in most of the cases combining its unique tissue characterization capabilities with the assessment of biventricular regional and global function

25. Clinical manifestations are obviously more often related to signs and symptoms of congestive heart failure such as dyspnoea and effort-related fatigue [6, 76]. Major arrhythmias leading to syncope, embolic events, and even sudden cardiac death may occur at any stage of the disease [1, 14]. Poor contractile function and stasis can also lead to the formation of mural thrombi with symptoms related to distal embolism

26. MRI

27. evaluation and quantification of LV dilatation and systolic dysfunction and detection of possible underlying tissue abnormalities partic ularly myocardial fibrosis a standard imaging protocol in DCM a four-chamber horizontal long-axis, two-chamber vertical long-axis, and short-axis views using breath-hold steady-state free precession (SSFP) cinesequences with ful lcoverage of both ventricles to provide assessment of biventricular volumes and global and regional functions

28. RV mass is preserved in DCM patients as compared to normal subjects, whereas LV mass is significantly greater with an end diastolic diameter greater than 55 mm In advancedcases,LV dysfunction may be associated with diffuse myocardial wall thinning (diastolic wall thickness < 5.5 mm) LV dilatation is a representative macroscopic finding of DCM and is sometimes associated with RV dilatation and hypertrabeculation of both ventricles. The thickness of the LV wall increases or decreases to compensate for the dilatation. Occasionally, mural thrombi are present in the ventricles.

29. LGE Three types of DCM-related LGE are reported: midwall LGE; subendocardial or transmural LGE, which suggests a prior myocardial in farction ; and no LGE Linear or bandlike midwall LGE is seen in 28%–35% of patients with DCM (40,41) and corresponds histopathologically to replacement fibrosis and fibrofatty change The images obtained in most patients show no LGE , but this absence can correspond to diffuse interstitial fibrosis when the null point method is used to acquire LGE images. Thus, the absence of LGE does not exclude disease of the heart muscle.

30. ARVC Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy fibrofatty replacement of the RV myocardium. The left ventricle is also involved in at least 15% of patients. The patients develop progressive RV failure ventricular arrhythmias

31.  right ventricle can have regional wall thinning, hypertrophy, dilatation and microaneurysms.   cine images RV dysfunction, microaneurysm formation, and focal areas of RV dyskinesia. There are two variants of ARVC: fatty and fibro-fatty. The fatty form is characterized by fatty replacement of the myocardium without thinning of the ventricular wall. sites subtricuspid area, the right ventricular apex, and the infundibulum, the 'triangle of dysplasia

32. MRI can show segmental hypokinesis, dilatation, fatty infiltration in the right ventricular myocardium, small aneurysms and late enhancement of the myocardium Fat infiltration is seldom the only abnormality seen on MRI in ARVC, it should coincide with right ventricular regional dysfunction 

33. RESTRICTIVE The most common cause of restrictive cardiomyopathy is amyloidosis [20]. Amyloid deposits in the myocardium cause abnormal diastolic function with biatrial enlargement, concentric thickening of the left ventricle and reduced systolic function of usually both ventricles. Cardiac involvement in systemic amyloidosis occurs in up to 50% and has a poor prognosis with a median survival of 6 months

34. Diffuse hypokinesia of ventricles late enhancement image shows enhancement over the entire subendocardial circumference, variably extending into the neighboring myocardium [21]. Sometimes it is difficult to find the optimal inversion time for nulling the normal myocardium The most important differential diagnosis of restrictive cardiomyopathy is constrictive cardiomyopathy. MRI can differentiate between those two diagnoses: Pericardium is usually thickened in constrictive cardiomyopathy Diastolic septal bounce is seen in constrictive, but not in restrictive cardiomyopathy 

35. Cardiac sarcoidosis atrioventricular block, serious ventricular arrhythmia, or LV dysfunction Manifestations of cardiac involve ment by sarcoidosis can range from no symptoms, to advanced heart failure, to sudden cardiac death diagnosis of cardiac sardoidosis is sometimes challenging because sarcoidosis often involves small areas of the myocardium without abnormally affecting LV function, and commonly used tests such as electrocardiography and echocardiography have limited utility in the diagnosis of this disease

36. Granulomas and/or fibrous scars tend to be distributed in the subepicardial layer. The four distribution patterns are disseminated, massive, zonal, and dendritic The thickness of the LV varies from thick to thin. Sarcoid lesions ultimately heal by means of fibrosis, which can lead to a ventricular aneurysm.

37. LGE are frequently seen on the RV side of the basal interventricular septum and in the subepicardial layer of the lateral wall of the LV in patients with cardiac sarcoidosis, they can be present anywhere. appearance of LGE ranges from patchy to sharp and band like, and the myocardial thickness fluctuates according to disease activity, with the myocardium being thicker during the active phase and thinner during the healed phase Areas of LGE correspond to both epithelioid granuloma (active phase) and fibrosis (healed stage)

38. (a, b) Short-axis (a) and four-chamber (b) LGE MR images obtained after the injection of 0.1 mmol/kg of gadolinium-based contrast material show areas of high signal intensity (arrows) on the RV side of the interventricular septum, in the subepicardial layer of the inferior wall, and in the papillary muscle.

39. Tako -tsubo cardiomyopathy Tako -Tsubo cardiomyopathy or apical ballooning syndrome > transient cardiomyopathy > postmenopausal women after physical or emotional stress. Patients present with symptoms mimicking an acute myocardial infarction. The ECG changes and abnormal laboratory findings may also mimic an infarction , coronary angiography is usually normal, left ventricle angiogram > marked hypokinesia of the apical cardiac segments LGE -nil

40. The modified Mayo Clinic criteria for diagnosis of takotsubo cardiomyopathy: Transient hypokinesis, dyskinesis or akinesis of the left ventricular mid-segments with or without apical involvement The regional wall motion abnormalities extend beyond a single epicardial vascular distribution A stressful trigger is often present. Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture. New electrocardiographic abnormalities either ST-segment elevation and/or T-wave inversion or modest elevation in cardiac troponin level. Absence of pheochromocytoma or myocarditis.

41. PERIPARTUM CARDIOMYOPATHY Peripartum/postpartum cardiomyopathy  is a  dilated cardiomyopathy that may occur in the last trimester of pregnancy through the first several months postpartum. cine SSFP  sequences can demonstrate systolic dysfunction, similarly to echocardiography delayed enhancement in the left ventricular mid-myocardium has been described in the anterior and anterolateral wall

42. LV non compaction Non-compaction of the left ventricle , also known as  spongiform cardiomyopathy  or  left ventricular non-compaction (LVNC)  is a phenotype of hypertrophic ventricular trabeculations and deep interventricular recesses Cardiac MRI (CMR) has a better contrast resolution than echocardiography and it is the modality of choice for the diagnosis of spongiform cardiomyopathy.

43. A good diagnostic clue   is a ratio of non-compacted telediastolic myocardium to compacted telediastolic myocardium of more than   2.3:1 (sensitivity: 86%, specificity: 99%)  10,11 . For improved discrimination of left ventricular non-compaction versus other cardiomyopathies with hypertrabeculated myocardium the following MRI criteria were proposed  11,12 : percentage left ventricular myocardial mass (non-compacted) >25 % total left ventricular myocardial mass index (non-compacted) >15 g/m 2 non-compacted/compacted myocardium ratio of ≥3:1 in at least one of the following segments (1–3, 7–16) – the apical segment 17 is excluded trabeculation (non-compacted/compacted) in segments 4–6 of ≥2:1