Noncompaction Cardiomyopathy, NGUYEN TUAN VU, NGUYEN THI KIM SANG, DUONG PHI SON, PHAN THANH HAI
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A 33-year-old female patient presented with severe heart failure for one year and was previously diagnosed with dilated cardiomyopathy. She underwent cardiac imaging tests including echocardiography, ECG, and MSCT to rule out coronary artery disease. The echocardiography and MSCT revealed prominent trabeculations and a spongiform appearance of the left ventricle consistent with noncompaction cardiomyopathy. Noncompaction cardiomyopathy is a rare genetic heart condition characterized by excessive trabeculations and deep intratrabecular recesses in the left ventricle.
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A case of LV Non Compaction
The patient presented with altered sensorium, decreased movement in their right side, and deviation of their mouth to the left. Imaging showed a non-hemorrhagic infarct in the left fronto-parietal region consistent with a stroke. An echocardiogram found left ventricular non-compaction (LVNC), a mobile clot attached to the left ventricle, and normal systolic function. The final diagnosis was LVNC, stroke, hyperhomocysteinemia, and hyperlipidemia. LVNC is a rare heart condition where the left ventricle fails to compact during development, leaving prominent trabeculations. It can cause heart failure, arrhythmias, and thromboemb
Left ventricular noncompaction
This document summarizes the history and criteria for diagnosing left ventricular noncompaction (LVNC) via echocardiography. It describes the original 1990 criteria using X/Y ratios between trabeculations and recesses. Second 1999 criteria used a noncompacted to compacted myocardium ratio over 2.3. Later studies refined understanding. The latest 2014 criteria require all 4 criteria be met regarding prominent trabeculations, synchronous movement, two-layer structure, and perfusion of intertrabecular spaces. Echocardiography looks for a noncompaction to compaction ratio over 2 and more than 3 recesses communicating to the left ventricle in diastole. MRI diagnoses if noncompaction mass is
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Left ventricular non compaction is rare congenital cardiomyopathy with gaining interest due to advancement in imaging modalities for diagnosis and assessment of undulating phenotype
LEFT VENTRICULAR NON COMPACTION (LVNC)
Noncompaction cardiomyopathy is a rare congenital heart condition caused by the failure of the heart muscle to develop and thicken normally during fetal development. It results in a thickened heart muscle with prominent trabeculations and deep recesses.
The diagnosis is challenging due to a lack of consensus on diagnostic criteria and the inability to clearly differentiate between normal trabeculations and pathological noncompaction using imaging modalities like echocardiography and MRI. Current echocardiographic criteria may be too sensitive, leading to overdiagnosis.
The condition can cause heart failure, arrhythmias and blood clots. Treatment involves medications and lifestyle changes to manage symptoms. The long term prognosis depends on the extent of involvement
Left ventricular non compaction
LVNC is a rare genetic cardiomyopathy characterized by a spongy appearance of the myocardium due to incomplete compaction of the embryonic myocardium. It can present with heart failure, arrhythmias, or thromboembolism. Echocardiography and cardiac MRI are used to diagnose LVNC based on identifying a two-layered myocardium. Management involves treating heart failure and preventing thromboembolism with anticoagulation in high-risk patients. While the cause of LVNC is thought to be due to an arrest in normal myocardial compaction during embryonic development, the pathophysiology is not fully understood.
Noncompaction cardiomyopathy
This document discusses left ventricular noncompaction cardiomyopathy (LVNC), a rare genetic heart condition where the heart muscle does not compact properly during fetal development. It begins by defining LVNC and describing its increasing recognition due to advances in diagnostic imaging. The document then discusses the embryological development of the heart and how a disruption in the normal compaction process can lead to LVNC. It outlines the characteristic features of LVNC seen on imaging studies and potential genetic associations. The document concludes by discussing the challenges in diagnosing and managing LVNC.
Mitral regurgitation
Mitral regurgitation is the abnormal reversal of blood from the left ventricle to the left atrium, caused by disruption of the mitral valve apparatus. It can be divided into acute, chronic compensated, and chronic decompensated stages. The etiology includes primary causes where the valve itself is abnormal, and secondary causes where the heart problem leads to valve dysfunction. Diagnosis is typically made using 2D echocardiography for diastolic murmurs or systolic murmurs grade 3-6. Treatment options include medical management or surgical repair or replacement of the valve.
ENDOMYOCARDIAL FIBROSIS
Endomyocardial fibrosis (EMF) is a disease that is characterized by fibrosis of the apical endocardium of the right ventricle (RV), left ventricle (LV), or both.
The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure.
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LEFT VENTRICULAR NON COMPACTION (LVNC)
Noncompaction cardiomyopathy is a rare congenital heart condition caused by the failure of the heart muscle to develop and thicken normally during fetal development. It results in a thickened heart muscle with prominent trabeculations and deep recesses.
The diagnosis is challenging due to a lack of consensus on diagnostic criteria and the inability to clearly differentiate between normal trabeculations and pathological noncompaction using imaging modalities like echocardiography and MRI. Current echocardiographic criteria may be too sensitive, leading to overdiagnosis.
The condition can cause heart failure, arrhythmias and blood clots. Treatment involves medications and lifestyle changes to manage symptoms. The long term prognosis depends on the extent of involvement
Left ventricular non compaction
LVNC is a rare genetic cardiomyopathy characterized by a spongy appearance of the myocardium due to incomplete compaction of the embryonic myocardium. It can present with heart failure, arrhythmias, or thromboembolism. Echocardiography and cardiac MRI are used to diagnose LVNC based on identifying a two-layered myocardium. Management involves treating heart failure and preventing thromboembolism with anticoagulation in high-risk patients. While the cause of LVNC is thought to be due to an arrest in normal myocardial compaction during embryonic development, the pathophysiology is not fully understood.
Noncompaction cardiomyopathy
This document discusses left ventricular noncompaction cardiomyopathy (LVNC), a rare genetic heart condition where the heart muscle does not compact properly during fetal development. It begins by defining LVNC and describing its increasing recognition due to advances in diagnostic imaging. The document then discusses the embryological development of the heart and how a disruption in the normal compaction process can lead to LVNC. It outlines the characteristic features of LVNC seen on imaging studies and potential genetic associations. The document concludes by discussing the challenges in diagnosing and managing LVNC.
Mitral regurgitation
Mitral regurgitation is the abnormal reversal of blood from the left ventricle to the left atrium, caused by disruption of the mitral valve apparatus. It can be divided into acute, chronic compensated, and chronic decompensated stages. The etiology includes primary causes where the valve itself is abnormal, and secondary causes where the heart problem leads to valve dysfunction. Diagnosis is typically made using 2D echocardiography for diastolic murmurs or systolic murmurs grade 3-6. Treatment options include medical management or surgical repair or replacement of the valve.
ENDOMYOCARDIAL FIBROSIS
Endomyocardial fibrosis (EMF) is a disease that is characterized by fibrosis of the apical endocardium of the right ventricle (RV), left ventricle (LV), or both.
The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure.
The heart failure is associated with atrioventricular-valve regurgitation.
Endomyocardial fibrosis is a major cause of illness and death in areas where it is endemic, and in its severest form carries a very poor prognosis, with an estimated survival of 2 years after diagnosis.
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Aortic coarctation is a narrowing of the aorta that commonly occurs near the insertion of the ductus arteriosus. It accounts for 6-8% of congenital heart defects. Diagnosis involves physical exam findings like hypertension and diminished pulses as well as imaging tests like echocardiogram and CT/MRI angiography. Treatment depends on age and severity, ranging from surgical repair for critical cases to balloon angioplasty or stenting for older children with non-critical coarctation. Long term follow up is needed due to risks of complications like recoarctation and hypertension.
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Coarctation of the aorta is a congenital narrowing of the aorta near the site where the ductus arteriosus attaches. It can range from a localized stenosis to tubular hypoplasia of the aorta. Left untreated, it causes increased blood pressure in the upper body and heart complications due to increased workload. Surgical repair techniques include subclavian flap aortoplasty, end-to-end anastomosis, and patch angioplasty. Postoperative risks include recoarctation, spinal cord injury, and persistent hypertension. Long term follow up is needed due to risks of aneurysm and cardiovascular complications.
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This document provides a comprehensive review of coarctation of the aorta (COA). It discusses COA's heterogeneous clinical presentation and various treatment modalities. While several theories exist for its etiology, there is increasing evidence of a genetic influence. Long-term morbidity related to hypertension remains substantial despite available treatments. The summary discusses COA's diverse severity and presentation, theories for its development, genetic factors, associated lesions, pathophysiology, and highlights that long-term hypertension remains a problem despite treatments.
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This document discusses coarctation of the aorta, including its embryology, nomenclature, pathophysiology, natural history, and clinical features. Some key points include:
- Coarctation of the aorta is a congenital narrowing of the aorta near the ductus arteriosus. Left untreated, 50% of patients will die within 10 years primarily due to heart failure.
- Associated anomalies include ventricular septal defects (40% of cases) and bicuspid aortic valves (46% of cases).
- Long-term complications include hypertension, aneurysm formation, dissection, and rupture.
- Natural history studies show mortality rates increase significantly from 25% at age
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- The mechanisms of IMR involve increased tethering forces on the mitral valve leaflets from left ventricular dilation and papillary muscle displacement, as well as reduced closing forces from decreased left ventricular contractility and altered annular contraction. This imbalance results in insufficient coaptation of the mitral valve leaflets.
- IMR can be classified as either symmetric or asymmetric based on the pattern of mitral valve tether
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3. Types of coarctation including preductal and postductal, and surgical techniques for repair such as patch aortoplasty or bypass grafting.
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commonly used for medical students, and helpful to use this ppt to study for them, and also a common man can understand very easily what is coarctation of aorta.
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This document discusses coarctation of the aorta, a congenital heart defect where the aorta is narrowed or constricted. It accounts for 5-8% of congenital heart defects. Coarctation can occur isolated or with other defects like bicuspid aortic valve or ventricular septal defect. Clinical presentation depends on age - infants may present with heart failure while older children and adults often present with hypertension. Imaging like chest x-ray and echocardiogram are used for diagnosis. Treatment involves surgery to repair the coarctation.
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This document discusses mitral valve disease and treatment options such as surgical repair/replacement and the MitraClip procedure. Some key points:
- Mitral regurgitation (MR) is the most common valve problem and increases in prevalence with age. Left untreated, MR can lead to heart failure and death.
- Surgical treatment has traditionally been the only option to reliably reduce MR, but many patients are considered too high-risk for surgery.
- The MitraClip procedure is a minimally invasive treatment that fills this gap for inoperable patients by using a clip to repair the mitral valve and reduce MR without open heart surgery.
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- It is a narrowing of the aorta that is usually congenital.
- It was first described in the late 18th century and various classifications have been developed.
- Signs and symptoms often include differences in blood pressure between the arms and legs.
- Associated conditions include other heart defects and genetic syndromes.
- Diagnosis involves imaging like echocardiography, CT, MRI and angiography.
- Treatment is usually surgical repair in infancy or childhood to relieve obstruction and reduce long-term risks.
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Aortic coarctation is a narrowing of the aorta that commonly occurs near the insertion of the ductus arteriosus. It accounts for 6-8% of congenital heart defects. Diagnosis involves physical exam findings like hypertension and diminished pulses as well as imaging tests like echocardiogram and CT/MRI angiography. Treatment depends on age and severity, ranging from surgical repair for critical cases to balloon angioplasty or stenting for older children with non-critical coarctation. Long term follow up is needed due to risks of complications like recoarctation and hypertension.
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Coarctation of the aorta is a congenital narrowing of the aorta near the site where the ductus arteriosus attaches. It can range from a localized stenosis to tubular hypoplasia of the aorta. Left untreated, it causes increased blood pressure in the upper body and heart complications due to increased workload. Surgical repair techniques include subclavian flap aortoplasty, end-to-end anastomosis, and patch angioplasty. Postoperative risks include recoarctation, spinal cord injury, and persistent hypertension. Long term follow up is needed due to risks of aneurysm and cardiovascular complications.
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- Associated anomalies include ventricular septal defects (40% of cases) and bicuspid aortic valves (46% of cases).
- Long-term complications include hypertension, aneurysm formation, dissection, and rupture.
- Natural history studies show mortality rates increase significantly from 25% at age
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ishemic mitral regurgitation gopan Amrita hospital
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- The mechanisms of IMR involve increased tethering forces on the mitral valve leaflets from left ventricular dilation and papillary muscle displacement, as well as reduced closing forces from decreased left ventricular contractility and altered annular contraction. This imbalance results in insufficient coaptation of the mitral valve leaflets.
- IMR can be classified as either symmetric or asymmetric based on the pattern of mitral valve tether
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Mitral valve prolapse (MVP) is a common condition where the mitral valve leaflets bulge into the left atrium during systole. While often asymptomatic, it can increase the risk of arrhythmias, endocarditis, stroke, and mitral regurgitation. The classic findings are a mid-to-late systolic click and murmur. Treatment involves monitoring for complications and addressing symptoms like with beta-blockers. For severe mitral regurgitation, early surgical repair is recommended to prevent left ventricular dysfunction.
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This document discusses coarctation of the aorta, including:
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2. Theories on the pathogenesis of coarctation related to reduced blood flow through the left side of the heart or abnormal ductal tissue.
3. Types of coarctation including preductal and postductal, and surgical techniques for repair such as patch aortoplasty or bypass grafting.
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Rib notching
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Echocardiography in cardiac emergency
This document discusses the use of echocardiography in emergency clinical situations. It provides examples of common clinical indications for emergency echocardiography including hemodynamic instability, aortic dissection, acute coronary syndromes, and critically ill patients. The document outlines echocardiography algorithms and describes how to use echocardiography to diagnose conditions like cardiac tamponade, pulmonary embolism, hypotension, and penetrating chest trauma. Key findings are highlighted for various emergency scenarios.
Mitral regurgitation
This document summarizes mitral regurgitation (MR), including its causes, classifications, effects on cardiac structures and function, clinical presentations, and evaluations. It discusses how abnormalities of the mitral valve leaflets, chordae tendineae, papillary muscles or annulus can cause MR. Chronic MR can lead to left ventricular dilation and hypertrophy due to volume overload, though acute MR initially increases cardiac output through the Frank-Starling mechanism. Clinical exams and imaging tests are used to assess the severity and chronicity of MR and its effects on heart function.
Coarctation of aorta
This document discusses coarctation of the aorta, a congenital heart defect where the aorta is narrowed or constricted. It accounts for 5-8% of congenital heart defects. Coarctation can occur isolated or with other defects like bicuspid aortic valve or ventricular septal defect. Clinical presentation depends on age - infants may present with heart failure while older children and adults often present with hypertension. Imaging like chest x-ray and echocardiogram are used for diagnosis. Treatment involves surgery to repair the coarctation.
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An aortic dissection is a tear within the layers of the aortic wall. It can cause severe chest or back pain and risks damage to organs if blood flow is blocked. Prompt diagnosis through imaging tests and treatment are critical to survival. While type A dissections involving the ascending aorta require emergency surgery, type B dissections of the descending aorta can often be treated through intensive blood pressure control and medication to prevent expansion of the tear. Risk factors include hypertension, genetics, trauma, and certain medical conditions.
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This document discusses mitral valve disease and treatment options such as surgical repair/replacement and the MitraClip procedure. Some key points:
- Mitral regurgitation (MR) is the most common valve problem and increases in prevalence with age. Left untreated, MR can lead to heart failure and death.
- Surgical treatment has traditionally been the only option to reliably reduce MR, but many patients are considered too high-risk for surgery.
- The MitraClip procedure is a minimally invasive treatment that fills this gap for inoperable patients by using a clip to repair the mitral valve and reduce MR without open heart surgery.
- Clinical trials show the MitraClip procedure reduces MR
Coarc2
This document provides information on coarctation of the aorta, including:
- It is a narrowing of the aorta that is usually congenital.
- It was first described in the late 18th century and various classifications have been developed.
- Signs and symptoms often include differences in blood pressure between the arms and legs.
- Associated conditions include other heart defects and genetic syndromes.
- Diagnosis involves imaging like echocardiography, CT, MRI and angiography.
- Treatment is usually surgical repair in infancy or childhood to relieve obstruction and reduce long-term risks.
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ishemic mitral regurgitation gopan Amrita hospital
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Similar to Noncompaction Cardiomyopathy, NGUYEN TUAN VU, NGUYEN THI KIM SANG, DUONG PHI SON, PHAN THANH HAI
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rare case of cardioembolic stroke in a child
A 10-year-old girl presented with right-sided weakness. Imaging showed large infarcts in both cerebral hemispheres. Echocardiogram revealed left ventricular non-compaction (LVNC), a rare heart condition characterized by excessive trabeculations. This was determined to be the cause of her cardioembolic stroke. LVNC can cause thrombus formation due to slow blood flow in deep recesses, increasing embolic risk even without other structural heart defects. Cardiac MRI is the gold standard for LVNC diagnosis but echocardiogram remains important.
Aortic Dissection
The document describes a case of a 46-year-old male who presented with sudden onset chest and back pain that progressed to weakness in his lower extremities. Imaging revealed an aortic dissection involving the ascending aorta and descending aorta. He underwent surgery to replace the dissected ascending aorta but later developed multiple complications and died. The document also reviews the classification, presentation, risk factors, diagnosis and management of aortic dissections.
cardiomyopathy.ppt
Cardiomyopathies are diseases of the heart muscle that can cause cardiac dysfunction. The main types include dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. Dilated cardiomyopathy is characterized by ventricular dilation and impaired contraction, with the majority of cases being idiopathic. Etiologies of dilated cardiomyopathy include infectious, inflammatory, toxic, metabolic, inherited, and peripartum causes. Treatment aims to reduce preload and afterload through medications like diuretics, vasodilators, and ACE inhibitors. Prognosis depends on the underlying etiology.
cardiomyopathy2.ppt
Cardiomyopathies are diseases of the heart muscle that can cause cardiac dysfunction. The main types include dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. Dilated cardiomyopathy is characterized by ventricular dilation and impaired contraction, with the majority of cases being idiopathic. Etiologies of dilated cardiomyopathy include infectious, inflammatory, metabolic, inherited, and toxic causes. Treatment aims to reduce preload and neurohormonal activation through medications like diuretics, ACE inhibitors, and beta-blockers. Prognosis depends on the underlying etiology. Hypertrophic cardiomyopathy is defined by left ventricular hypertrophy not due to pressure overload. It is characterized by impaired di
cardiomyopathy2 (3).ppt
1) Dilated cardiomyopathy is characterized by dilation and impaired contraction of the ventricles, often leading to heart failure. The majority of cases are idiopathic, but it can result from various infectious, toxic, metabolic, inherited and other etiologies.
2) Peripartum cardiomyopathy presents in the last month of pregnancy or within 5 months postpartum, and has an unknown etiology but may involve inflammatory cytokines. It carries a risk of death and recurrence with future pregnancies.
3) Treatment of dilated cardiomyopathy focuses on reducing preload and afterload through medications like diuretics, ACE inhibitors, and beta-blockers to help compensate for impaired contractility and prevent ventricular
Chest pain
This document provides an overview of evaluating and managing chest pain. It discusses the many potential causes of chest pain including cardiac, pulmonary, gastrointestinal and psychiatric conditions. Key points include that up to 30% of cardiac catheterizations for suspected coronary artery disease are negative. The diagnostic workup involves considering the patient's history, symptoms, physical exam, ECG, cardiac biomarkers, imaging and ruling out life-threatening causes like aortic dissection and pulmonary embolism. For suspected acute coronary syndrome, treatment involves antiplatelet agents, anticoagulants, nitroglycerin, beta-blockers, oxygen and considering cardiac catheterization if within 90 minutes of symptoms.
cardiovascular system-diagnostic tools
This document provides an overview of various diagnostic tools used in cardiology, including electrocardiography (ECG), non-invasive cardiac imaging modalities like echocardiography and nuclear imaging, and invasive diagnostic procedures like cardiac catheterization and coronary angiography. It describes the basic principles, clinical applications, and abnormalities detected by each diagnostic modality.
CHD
The document discusses coronary artery disease (CAD) and its typical presentation and evaluation. It notes that CAD is usually caused by atherosclerosis and describes the pathophysiology of atherosclerosis. For evaluation, it describes that echocardiogram can show wall motion defects, stress testing can detect ischemia, and coronary angiography can identify blockages. Angina is a common initial manifestation of CAD and is typically relieved by rest. Diagnosis is made based on history, physical exam, and testing like ECG and cardiac biomarkers.
Cardiac Us
The document discusses the use of emergency cardiac ultrasound as a rapid assessment tool in the emergency department. It provides an overview of the technique, including probe selection and standard image planes. Key applications described are detection of pericardial effusions and tamponade, assessment of cardiac activity and wall motion in cardiac arrest and hypotension, and identification of injuries in trauma. Accuracy is high for identifying pericardial effusions but sensitivity varies for other applications such as pulmonary embolism and myocardial infarction.
Radiation Associated Cardiac Disease
CVD in cancer survivors.Screening of cancer survivors.Chest Radiotherapy .JACC Scientific Expert Panel
( J Am Coll Cardiol 2019;74:905–27 )manifestations of chest and mediastinal radiotherapy .
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Cardiac CT Angiography to detect Myocardial Bridging
CTCA is a reliable non-invasive tool for detecting myocardial bridging in coronary artery disease. [The study] found an 8.2% frequency of myocardial bridging in 219 patients with coronary artery disease who underwent CTCA. CTCA allows for visualization of the length and depth of the bridging artery and measurement of stenosis. While myocardial bridging can be clinically significant when associated with hemodynamic changes, in most cases it remains asymptomatic. CTCA is an emerging alternative to other invasive tests for diagnosing myocardial bridging.
Heart failure
treatment of Heart Failure, CCF,
Powerpoint presentation of Heart Failure
LVF
management of Heart failure
TRANS-CRANIAL DOPPLER
Trans-Cranial Doppler (TCD) is a non-invasive ultrasound technique used to evaluate cerebral blood flow velocities. There are two main types of TCD devices - non-duplex devices which identify arteries "blindly" based on Doppler shift and duplex devices which combine Doppler with B-mode imaging to directly visualize arteries. TCD allows evaluation of intracranial steno-occlusive disease, vasospasm, aneurysms, and other conditions. It can detect elevated velocities indicative of stenosis but has limitations including operator dependence and inability to image distal arteries. TCD is useful for monitoring conditions like sickle cell disease where elevated velocities increase stroke risk.
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Noncompaction Cardiomyopathy, NGUYEN TUAN VU, NGUYEN THI KIM SANG, DUONG PHI SON, PHAN THANH HAI
- 1. Dr Nguyen Thi Kim Sang PhD Dr Nguyen Tuan Vu MEDIC HCM
- 2. HISTORY Pt TP, 33 Y.O, F, presented by severe heart failure for 1 year Previous diagnosis : dilated cardiomyopathy She was sent to MEDIC for cardiac MSCT to rule out Coronary artery disease. Decreased S1. audible S3, 2/6 apical systolic murmur.
- 3. ECG Short PR, delta wavs, QS in V2-V6, D1 aVL
- 4. Echocardiography Decreased LV systolic function
- 5. Echocardiography LV diastolic dysfunction Decreased RV function
- 7. Echocardiography Decreased LV systolic function Diagnostic criteria NC/C leyer > 2
- 8. Echocardiography TDI and 3 D Echocardiography
- 10. Noncompaction Cardiomyopathy Intertrabecular Recesses Multiple Trabeculae Predominant location at Apex, mid lateral, mid inferior
- 11. Diagnostic Criteria NC layer/ C layer > 2,2 Involving >2 segments Sens. 100%, Spec. 95% ( Tomography, volume 6, Issue 5, Sept.-Oct. 2012, pp346-354 )
- 12. MSCT 640: 3D Imaging Trabeculated and spongiformed LV
- 13. Coronary MSCT-angiography LM, LAD and CX are normal
- 14. Noncompacted cardiomyopathy Echocardiography Apical 4 C view and Parasternal short axis view Prominent trabeculations and spongiformed myocardium of LV
- 15. Summary Reported by Engberding and Benber in 1984 Mutation in LDB3, genetic cardiomyopathy Myocardial sinusoids Severe heart failure, Arrhythmias, Thrombus formation, sudden death Diagnosis: Echocardiography, MRI, MSCT Medical treatment ( ACEI, Betabloker, Aspirin, Anticoagulant ), ICD, heart transplant. Long term prognosis is unknown Value of cardiac MSCT in Pt with heart failure.
- 16. Thank you so much!