Answer for BIR CoW 12 Sep 2021
Hypertrophic Cardiomyopathy with Mitral regurgitation
Findings
• Dilatation of left atrium noted. • Mitral regurgitation noted. • Asymmetric hypertrophy of interventricular septum (basal region). • Systolic anterior motion of mitral value with signal drop noted DELAYED ENHANCEMENT IMAGING revealed no abnormal or delayed myocardial gadolinium enhancement.
Discussion
Hypertrophic cardiomyopathy (HCM) is a type of cardiomyopathy defined by left ventricular hypertrophy, that cannot be only explained by abnormal loading conditions another cardiac, metabolic or systemic disease. It is the leading cause of sudden cardiac death (from arrhythmias) in infants, teenagers, and young adults. Associations • LVOT obstruction is present in 70% of cases , it is defined as a gradient >30 mmHg o a gradient >50 mm Hg is often considered the threshold of haemodynamic significance o often 'obstructive hypertrophic cardiomyopathy' is used when LVOT obstruction is present most commonly occurs at the basal interventricular septum with systolic anterior motion (SAM) of the chordal apparatus, the dynamic obstruction may occur deeper within the left ventricle hypertrophic papillary muscles may cause obstruction in the mid-cavity • can be associated with systolic anterior motion (SAM) of the anterior mitral leaflet, which can increase LVOT obstruction and decreased coronary and systemic outflow o systolic anterior motion of the posterior leaflet, less commonly, may also occur • other secondary signs include: o mitral regurgitation leaflet accessory tissue and elongation may also be observed o left auricle dilation o papillary muscle abnormalities hypertrophy direct insertion to anterior leaflet of mitral valve antero-internal displacement Subtypes • asymmetric hypertrophic cardiomyopathy • symmetrical or concentric hypertrophic cardiomyopathy • apical hypertrophic cardiomyopathy (Yamaguchi syndrome) • midventricular hypertrophic cardiomyopathy • mass-like hypertrophic cardiomyopathy /tumefactive hypertrophic cardiomyopathy • burned-out phase: end-stage Another method is a four-pattern model : • septal hypertrophy alone ~45% • septal and other segments hypertrophy but sparing the apex ~ 16% • apical segments along with any other segment hypertrophy ~ 27% • apical hypertrophy alone ~ 13% FINDINGS: Cardiac MRI, with its capabilities in evaluating cardiac morphology and function, has emerged as a technique particularly well-suited to hypertrophic cardiomyopathy diagnosis and phenotypic characterisation. It is superior to echocardiography in identifying areas of segmental hypertrophy not reliably visualised or underestimated by echocardiography (i.e. anterolateral and apical segments). MRI is useful in evaluating hypertrophic cardiomyopathy patients with thin-walled scarred left ventricular apical aneurysms, end-stage systolic dysfunction, massive left ventricular wall hypertrophy, associated thickening of the right ventricular wall as well as substantial morphologic diversity with regard to papillary muscles and mitral valve. MRI can also demonstrate systolic anterior motion (SAM) of the mitral valve, which along with basal septal hypertrophy, results in LVOT obstruction. Mitral regurgitation may also be noted. Cardiac MRI plays a key role in risk stratification . Negative prognostic indicators in high-risk patients include: • left ventricular wall thickness ≥30 mm • gradient across LVOT ≥30 mmHg • delayed wall enhancement which represents fibrosis • decreased ejection fraction to <50% (burned-out phase) • presence of left ventricular apical aneurysms Cardiac MRI has a role in asymptomatic HCM mutation carriers by identifying phenotypic markers of HCM in the absence of left ventricular hypertrophy including: • myocardial crypts • elongated mitral valve leaflets • late gadolinium enhancement o patchy/streaky intramyocardial patterns at the right ventricular insertion sites within the hypertrophied myocardium suggest fibrosis o prognostic value with increased risk of sudden cardiac death (SCD)
REFERENCES: • 1. Abbara S, Walker TG, Imbesi SG. Diagnostic Imaging, Cardiovascular. Amirsys Inc. (2008) ISBN:1416033408. Read it at Google Books - Find it at Amazon • 2. Dähnert W. Radiology review manual. Lippincott Williams & Wilkins. (2007) ISBN:0781738954. Read it at Google Books - Find it at Amazon • 3. Belloni E, De cobelli F, Esposito A et-al. MRI of cardiomyopathy. AJR Am J Roentgenol. 2008;191 (6): 1702-10. doi:10.2214/AJR.07.3997 - Pubmed citation • 4. Hansen MW, Merchant N. MRI of hypertrophic cardiomyopathy: part I, MRI appearances. AJR Am J Roentgenol. 2007;189 (6): 1335-43. doi:10.2214/AJR.07.2286 - Pubmed citation • 5. Ibrahim T, Schwaiger M. Diagnosis of apical hypertrophic cardiomyopathy using magnetic resonance imaging. Heart. 2000;83 (1): E1. doi:10.1136/heart.83.1.e1 - Free text at pubmed - Pubmed citation • 6. Hansen MW, Merchant N. MRI of hypertrophic cardiomyopathy: part 2, Differential diagnosis, risk stratification, and posttreatment MRI appearances. AJR Am J Roentgenol. 2007;189 (6): 1344-52. doi:10.2214/AJR.07.2287 - Pubmed citation • 7. Chun EJ, Choi SI, Jin KN et-al. Hypertrophic cardiomyopathy: assessment with MR imaging and multidetector CT. Radiographics. 2010;30 (5): 1309-28. doi:10.1148/rg.305095074 - Pubmed citation
Findings
• Dilatation of left atrium noted. • Mitral regurgitation noted. • Asymmetric hypertrophy of interventricular septum (basal region). • Systolic anterior motion of mitral value with signal drop noted DELAYED ENHANCEMENT IMAGING revealed no abnormal or delayed myocardial gadolinium enhancement.
Discussion
Hypertrophic cardiomyopathy (HCM) is a type of cardiomyopathy defined by left ventricular hypertrophy, that cannot be only explained by abnormal loading conditions another cardiac, metabolic or systemic disease. It is the leading cause of sudden cardiac death (from arrhythmias) in infants, teenagers, and young adults. Associations • LVOT obstruction is present in 70% of cases , it is defined as a gradient >30 mmHg o a gradient >50 mm Hg is often considered the threshold of haemodynamic significance o often 'obstructive hypertrophic cardiomyopathy' is used when LVOT obstruction is present most commonly occurs at the basal interventricular septum with systolic anterior motion (SAM) of the chordal apparatus, the dynamic obstruction may occur deeper within the left ventricle hypertrophic papillary muscles may cause obstruction in the mid-cavity • can be associated with systolic anterior motion (SAM) of the anterior mitral leaflet, which can increase LVOT obstruction and decreased coronary and systemic outflow o systolic anterior motion of the posterior leaflet, less commonly, may also occur • other secondary signs include: o mitral regurgitation leaflet accessory tissue and elongation may also be observed o left auricle dilation o papillary muscle abnormalities hypertrophy direct insertion to anterior leaflet of mitral valve antero-internal displacement Subtypes • asymmetric hypertrophic cardiomyopathy • symmetrical or concentric hypertrophic cardiomyopathy • apical hypertrophic cardiomyopathy (Yamaguchi syndrome) • midventricular hypertrophic cardiomyopathy • mass-like hypertrophic cardiomyopathy /tumefactive hypertrophic cardiomyopathy • burned-out phase: end-stage Another method is a four-pattern model : • septal hypertrophy alone ~45% • septal and other segments hypertrophy but sparing the apex ~ 16% • apical segments along with any other segment hypertrophy ~ 27% • apical hypertrophy alone ~ 13% FINDINGS: Cardiac MRI, with its capabilities in evaluating cardiac morphology and function, has emerged as a technique particularly well-suited to hypertrophic cardiomyopathy diagnosis and phenotypic characterisation. It is superior to echocardiography in identifying areas of segmental hypertrophy not reliably visualised or underestimated by echocardiography (i.e. anterolateral and apical segments). MRI is useful in evaluating hypertrophic cardiomyopathy patients with thin-walled scarred left ventricular apical aneurysms, end-stage systolic dysfunction, massive left ventricular wall hypertrophy, associated thickening of the right ventricular wall as well as substantial morphologic diversity with regard to papillary muscles and mitral valve. MRI can also demonstrate systolic anterior motion (SAM) of the mitral valve, which along with basal septal hypertrophy, results in LVOT obstruction. Mitral regurgitation may also be noted. Cardiac MRI plays a key role in risk stratification . Negative prognostic indicators in high-risk patients include: • left ventricular wall thickness ≥30 mm • gradient across LVOT ≥30 mmHg • delayed wall enhancement which represents fibrosis • decreased ejection fraction to <50% (burned-out phase) • presence of left ventricular apical aneurysms Cardiac MRI has a role in asymptomatic HCM mutation carriers by identifying phenotypic markers of HCM in the absence of left ventricular hypertrophy including: • myocardial crypts • elongated mitral valve leaflets • late gadolinium enhancement o patchy/streaky intramyocardial patterns at the right ventricular insertion sites within the hypertrophied myocardium suggest fibrosis o prognostic value with increased risk of sudden cardiac death (SCD)
REFERENCES: • 1. Abbara S, Walker TG, Imbesi SG. Diagnostic Imaging, Cardiovascular. Amirsys Inc. (2008) ISBN:1416033408. Read it at Google Books - Find it at Amazon • 2. Dähnert W. Radiology review manual. Lippincott Williams & Wilkins. (2007) ISBN:0781738954. Read it at Google Books - Find it at Amazon • 3. Belloni E, De cobelli F, Esposito A et-al. MRI of cardiomyopathy. AJR Am J Roentgenol. 2008;191 (6): 1702-10. doi:10.2214/AJR.07.3997 - Pubmed citation • 4. Hansen MW, Merchant N. MRI of hypertrophic cardiomyopathy: part I, MRI appearances. AJR Am J Roentgenol. 2007;189 (6): 1335-43. doi:10.2214/AJR.07.2286 - Pubmed citation • 5. Ibrahim T, Schwaiger M. Diagnosis of apical hypertrophic cardiomyopathy using magnetic resonance imaging. Heart. 2000;83 (1): E1. doi:10.1136/heart.83.1.e1 - Free text at pubmed - Pubmed citation • 6. Hansen MW, Merchant N. MRI of hypertrophic cardiomyopathy: part 2, Differential diagnosis, risk stratification, and posttreatment MRI appearances. AJR Am J Roentgenol. 2007;189 (6): 1344-52. doi:10.2214/AJR.07.2287 - Pubmed citation • 7. Chun EJ, Choi SI, Jin KN et-al. Hypertrophic cardiomyopathy: assessment with MR imaging and multidetector CT. Radiographics. 2010;30 (5): 1309-28. doi:10.1148/rg.305095074 - Pubmed citation
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!