Obstructive hypertrophic cardiomyopathy

Cardiovascular

Clinicals - History

Fact Explanation
Asymptomatic , Patients with obstructive hypertrophic cardiomyopathy can be asymptomatic as the disease has not yet developed up to the level to produce any symptoms, but in echocardiography there would be hypertrophied but non dilated left ventricular chamber., Asymptomatic ,
Patients with obstructive hypertrophic cardiomyopathy can be asymptomatic as the disease has not yet developed up to the level to produce any symptoms, but in echocardiography there would be hypertrophied but non dilated left ventricular chamber.,
Shortness of breath In obstructive hypertrophic cardiomyopathy the septum wall in between ventricles become hypertrophied and cause left ventricular outflow tract obstruction. Other than that the anterior displacement of the papillary muscles and mitral leaflets also lead to the left ventricular outflow tract obstruction and gradually cause heart failure. Shortness of breath
In obstructive hypertrophic cardiomyopathy the septum wall in between ventricles become hypertrophied and cause left ventricular outflow tract obstruction. Other than that the anterior displacement of the papillary muscles and mitral leaflets also lead to the left ventricular outflow tract obstruction and gradually cause heart failure.
Fatigue In obstructive hypertrophic cardiomyopathy the septum wall in between ventricles become hypertrophied and cause left ventricular outflow tract obstruction. Other than that the anterior displacement of the papillary muscles and mitral leaflets also lead to the left ventricular outflow tract obstruction and gradually cause heart failure. Fatigue
In obstructive hypertrophic cardiomyopathy the septum wall in between ventricles become hypertrophied and cause left ventricular outflow tract obstruction. Other than that the anterior displacement of the papillary muscles and mitral leaflets also lead to the left ventricular outflow tract obstruction and gradually cause heart failure.
Chest discomfort This can be occur due to myocardial ischemia. This may be due to small-vessel disease with decreased vasodilator capacity. Other factors as septal perforator artery compression, myocardial bridging, decreased coronary perfusion pressure, obstruction to LV outflow, and decreased capillary myocardial fiber ratio also may contribute. Chest discomfort
This can be occur due to myocardial ischemia. This may be due to small-vessel disease with decreased vasodilator capacity. Other factors as septal perforator artery compression, myocardial bridging, decreased coronary perfusion pressure, obstruction to LV outflow, and decreased capillary myocardial fiber ratio also may contribute.
Dizziness, faintishness During the hypertrophy process, the normal pattern of muscle cells is disturbed and can lead to problems with conducting system of the heart, resulting in arrhythmias. Dizziness, faintishness
During the hypertrophy process, the normal pattern of muscle cells is disturbed and can lead to problems with conducting system of the heart, resulting in arrhythmias.
Palpitations The normal pattern of muscle cells is disturbed and can lead to problems with conducting system of the heart, resulting in arrhythmias. Palpitations
The normal pattern of muscle cells is disturbed and can lead to problems with conducting system of the heart, resulting in arrhythmias.
Sudden death , In hypertrophic cardiomyopathy there are extensive myocardial disarray in which numerous
myocytes are arranged at a disorganized architecture
Other than that there is small-vessel disease which is
responsible for bursts of silent microvascular ischemia and myocyte death and ultimately repair as replacement fibrosis. These disorganization and scarring cause unstable electrophysiologicat state which lead to ventricular fibrilations.,
Sudden death ,
In hypertrophic cardiomyopathy there are extensive myocardial disarray in which numerous
myocytes are arranged at a disorganized architecture
Other than that there is small-vessel disease which is
responsible for bursts of silent microvascular ischemia and myocyte death and ultimately repair as replacement fibrosis. These disorganization and scarring cause unstable electrophysiologicat state which lead to ventricular fibrilations.,
Family history, Hypertrophic cardiomyopathy inherited as a Mendelian autosomal dominant trait. Therefore family history of sudden cardiac death is an important factor., Family history,
Hypertrophic cardiomyopathy inherited as a Mendelian autosomal dominant trait. Therefore family history of sudden cardiac death is an important factor.,

Clinicals - Examination

Fact Explanation
Abnormal 'a' wave in jugular venous pulse Occurs due to the right ventricular involvement., Abnormal 'a' wave in jugular venous pulse
Occurs due to the right ventricular involvement.,
Double apical thrust This occurs due to the powerful contraction of left atrial against the non compliant left ventricle., Double apical thrust
This occurs due to the powerful contraction of left atrial against the non compliant left ventricle.,
Reverse splitting of second heart sound(s2) In this splitting of s2 occurs in expiration and pulmonary valve close before the aortic valve , Reverse splitting of second heart sound(s2)
In this splitting of s2 occurs in expiration and pulmonary valve close before the aortic valve ,
Third(s3) and fourth(s4) heart sounds s3 - Best heard at the apex, occurs due to the left ventricular dysfunction
s4 - Left sided s4 which is often palpable is due to impaired left ventricular relaxation. Rarely right side s4 occurs in right ventricular dysfunction.,
Third(s3) and fourth(s4) heart sounds
s3 - Best heard at the apex, occurs due to the left ventricular dysfunction
s4 - Left sided s4 which is often palpable is due to impaired left ventricular relaxation. Rarely right side s4 occurs in right ventricular dysfunction.,
Systolic ejection murmur In left ventricular dysfunction murmur best heard at the apex. Grade of the murmur vary with the degree of the left outflow tact obstruction. In right ventricular dysfunction murmur best heard at the left sternal edge. , Systolic ejection murmur
In left ventricular dysfunction murmur best heard at the apex. Grade of the murmur vary with the degree of the left outflow tact obstruction. In right ventricular dysfunction murmur best heard at the left sternal edge. ,

Investigations - Diagnosis

Fact Explanation
Echocardiogram (echo), Transthoracic echo or Doppler examination can determine the location and extent of hypertrophy, systolic and diastolic function, the presence and degree of systolic anterior motion, the severity of the subaortic and/or midventricular obstruction, the direction and degree of mitral regurgitation, the presence of additional mitral valve abnormalities,and size of the left atrium. These tests are used in diagnosing, planning of the treatment and assessing of the treatment., Echocardiogram (echo),
Transthoracic echo or Doppler examination can determine the location and extent of hypertrophy, systolic and diastolic function, the presence and degree of systolic anterior motion, the severity of the subaortic and/or midventricular obstruction, the direction and degree of mitral regurgitation, the presence of additional mitral valve abnormalities,and size of the left atrium. These tests are used in diagnosing, planning of the treatment and assessing of the treatment.,
Magnetic resonance imaging(MRI), To identify the site and extent of hypertrophy when 2D echo cannot detect them especially in apical hypertrophy. , Magnetic resonance imaging(MRI),
To identify the site and extent of hypertrophy when 2D echo cannot detect them especially in apical hypertrophy. ,
Electrocardiogram(ECG), Changes may vary form no changes, mild changes to extensive changes with the degree of hypertrophy.
Abnormal Q wave - septal hypertrophy, may mimic myocardial infarction.
Giant T-negativity syndrome - apical hypertrophy.
ECG changes can be seen in patients who does not show any echocardiographic changes.,
Electrocardiogram(ECG),
Changes may vary form no changes, mild changes to extensive changes with the degree of hypertrophy.
Abnormal Q wave - septal hypertrophy, may mimic myocardial infarction.
Giant T-negativity syndrome - apical hypertrophy.
ECG changes can be seen in patients who does not show any echocardiographic changes.,
Chest X-Ray May be normal or show left ventricle or left atrial and/or right atrial enlargement with or without vascular redistribution in the lungs. Chest X-Ray
May be normal or show left ventricle or left atrial and/or right atrial enlargement with or without vascular redistribution in the lungs.
Heart catheterization and angiography Reserved for diagnosis, but diagnostic accuracy of echo/Doppler studies has dramatically lessened the need for invasive investigation. Heart catheterization and angiography
Reserved for diagnosis, but diagnostic accuracy of echo/Doppler studies has dramatically lessened the need for invasive investigation.

Investigations - Management

Fact Explanation
Echocardiogram(echo), Used to evaluate management by assessing systolic and diastolic function, Echocardiogram(echo),
Used to evaluate management by assessing systolic and diastolic function,
Electrophysiological investigation, used ambulatory monitoring for detection and assessment of treatment of all arrhythmias, particularly ventricular arrhythmias, Electrophysiological investigation,
used ambulatory monitoring for detection and assessment of treatment of all arrhythmias, particularly ventricular arrhythmias,
Echocardiogram(echo), Transthoracic echo/
Doppler examination can assess the systolic and diastolic function and planning of the treatment and assessing of the treatment.,
Echocardiogram(echo),
Transthoracic echo/
Doppler examination can assess the systolic and diastolic function and planning of the treatment and assessing of the treatment.,
Nuclear angiography, To assess the systolic and diastolic ventricular function. , Nuclear angiography,
To assess the systolic and diastolic ventricular function. ,
Electrophysiological investigation, Used ambulatory monitoring for detection and assessment of treatment of all arrhythmias, particularly ventricular arrhythmias, Electrophysiological investigation,
Used ambulatory monitoring for detection and assessment of treatment of all arrhythmias, particularly ventricular arrhythmias,
Ecocardiogram These tests done in screening of first degree relatives of obstructive hypertrophic cardiomyopathy along with the detail history and physical examination. Ecocardiogram
These tests done in screening of first degree relatives of obstructive hypertrophic cardiomyopathy along with the detail history and physical examination.
Electrocardiogram These tests done in screening of first degree relatives of obstructive hypertrophic cardiomyopathy along with the detail history and physical examination. Electrocardiogram
These tests done in screening of first degree relatives of obstructive hypertrophic cardiomyopathy along with the detail history and physical examination.
Genetic screening, Hypertrophic cardiomyopathy inherited as a Mendelian autosomal dominant trait.
,
Genetic screening,
Hypertrophic cardiomyopathy inherited as a Mendelian autosomal dominant trait.
,

Management - Supportive

Fact Explanation
Management of arrhythmias, Pharmacological rate control by sotalol, cardioversion and anticoagulation is used in initial management. Dual-chamber pacing/ myectomy / cardiac transplant should be used in further management. , Management of arrhythmias,
Pharmacological rate control by sotalol, cardioversion and anticoagulation is used in initial management. Dual-chamber pacing/ myectomy / cardiac transplant should be used in further management. ,

Management - Specific

Fact Explanation
Medical managment , Negative inotropic agents (β-blockers, calcium antagonists and disopyramide) have been used to decrease the degree of outflow obstruction., Medical managment ,
Negative inotropic agents (β-blockers, calcium antagonists and disopyramide) have been used to decrease the degree of outflow obstruction.,
Pacemaker therapy , Dual-chamber (DDD) pacing is used to decrease the subaortic pressure gradient The mechanism is uncertain but may be related to decreased (or paradoxical) septal motion, late activation at the base of the septum with right ventricle apical pacing, or decreased left ventricle contractility., Pacemaker therapy ,
Dual-chamber (DDD) pacing is used to decrease the subaortic pressure gradient The mechanism is uncertain but may be related to decreased (or paradoxical) septal motion, late activation at the base of the septum with right ventricle apical pacing, or decreased left ventricle contractility.,
Surgical therapy, When patient is unresponsive to the medical therapy Septal Myectomy surgery can be performed. , Surgical therapy,
When patient is unresponsive to the medical therapy Septal Myectomy surgery can be performed. ,
Alternative therapy to surdery Alcohol septal ablation technique - In this option 1-4 ml of absolute alcohol is introduce to the target septal perforator coronary branch to produce myocardial infarction. This causes a reduction in septal thickness. Alternative therapy to surdery
Alcohol septal ablation technique - In this option 1-4 ml of absolute alcohol is introduce to the target septal perforator coronary branch to produce myocardial infarction. This causes a reduction in septal thickness.

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