Cardiology questions
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1) The document discusses a rare cardiac tumor known as an angiosarcoma that arises in the right atrium and can invade surrounding structures like the vena cava or tricuspid valve. 2) It also discusses Marfan syndrome, a connective tissue disorder associated with floppy mitral valves and aortic dissection, caused by a genetic mutation that leads to excessive TGF-β and weakening of heart and lung tissues. 3) Several questions are presented regarding various cardiac conditions, complications, diagnoses and treatment options.
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Aortic dissection nikku ppt
An aortic dissection occurs when blood tears the inner layer of the aorta, separating it from the middle layer. It is classified by location and timing of symptoms. Risk factors include hypertension, connective tissue disorders, and family history. Treatment depends on location but may include surgery, endovascular stent grafting, or medical management of blood pressure. Prognosis depends on type and treatment, with mortality rates declining with advances in surgical and endovascular techniques.
ECHOCARDIOGRAPHY IN CARDIAC TAMPONADE
This document discusses cardiac tamponade, which occurs when fluid rapidly accumulates in the pericardial sac, putting pressure on the heart and reducing cardiac function. Key points include:
- Pericardial effusion puts pressure on the heart, causing symptoms like chest pain and shortness of breath.
- Cardiac tamponade occurs when a rapid accumulation of fluid in the pericardial sac severely compresses the heart.
- Echocardiography is useful for diagnosing tamponade by showing findings like pericardial effusion, right ventricular collapse, and reduced respiratory variation in blood flow velocities.
- Tamponade is a medical emergency treated initially with medications and peric
Mitral valve
The mitral valve lies between the left atrium and left ventricle. Mitral stenosis is usually caused by rheumatic fever which causes scarring of the mitral valve leaflets and commissures. In early mitral stenosis, the leaflets can open but have restricted motion. Over time, the leaflets become thickened and rigid, reducing valve opening. This causes symptoms like dyspnea and pulmonary hypertension. On examination, findings may include an irregular pulse from atrial fibrillation, elevated jugular venous pressure, accentuated S1, and a diastolic murmur. Severe mitral stenosis can lead to right heart failure and complications like hemoptysis.
Echo in restrictive cardiomyopathy
This document provides an overview of echocardiographic evaluation of restrictive cardiomyopathy. Key points include:
- Restrictive cardiomyopathy is characterized by a nondilated left ventricle with abnormal diastolic function and typically normal systolic function.
- Causes include infiltrative diseases like amyloidosis and storage diseases. Echocardiography can help diagnose but it is more difficult than other cardiomyopathies.
- Findings include low diastolic volume, normal ejection fraction, diastolic dysfunction with rapid early mitral inflow. Echocardiography helps differentiate restrictive cardiomyopathy from constrictive pericarditis.
Sinus of valsalva aneurysm
This document summarizes the history and classification of sinus of Valsalva aneurysm (SOVA). Some key points:
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- The majority originate from the right coronary cusp (77%) and most commonly rupture into the right ventricle (67.9%).
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Prosthetic valve thrombosis
Prosthetic valve thrombosis (PVT) occurs when a blood clot forms on an artificial heart valve. It is more common with mechanical valves compared to bioprosthetic valves. Risk factors include being under-anticoagulated, poor cardiac output, pregnancy, and hypercoagulable states. Echocardiography can diagnose PVT by detecting increased transvalvular gradients, thickened valve cusps, limited cusp motion, or visible thrombus. For severe PVT with heart failure symptoms, emergency surgery is recommended. For less severe cases, fibrinolytic therapy can restore normal valve function in 64% of patients and is a reasonable first-line treatment alternative to surgery.
Aortic valve disease
This document discusses aortic valve disease, including aortic stenosis and aortic regurgitation. It provides definitions and descriptions of aortic valve stenosis in terms of etiology, pathophysiology, clinical presentation, investigations, and treatment options including surgical aortic valve replacement and percutaneous approaches. For aortic regurgitation, it covers etiology, pathophysiology, symptoms, physical exam findings for assessing severity, investigations, and indications for surgical valve replacement. Key points are made about compensatory changes in the left ventricle and how these relate to severity and timing of intervention.
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Aortic dissection nikku ppt
An aortic dissection occurs when blood tears the inner layer of the aorta, separating it from the middle layer. It is classified by location and timing of symptoms. Risk factors include hypertension, connective tissue disorders, and family history. Treatment depends on location but may include surgery, endovascular stent grafting, or medical management of blood pressure. Prognosis depends on type and treatment, with mortality rates declining with advances in surgical and endovascular techniques.
ECHOCARDIOGRAPHY IN CARDIAC TAMPONADE
This document discusses cardiac tamponade, which occurs when fluid rapidly accumulates in the pericardial sac, putting pressure on the heart and reducing cardiac function. Key points include:
- Pericardial effusion puts pressure on the heart, causing symptoms like chest pain and shortness of breath.
- Cardiac tamponade occurs when a rapid accumulation of fluid in the pericardial sac severely compresses the heart.
- Echocardiography is useful for diagnosing tamponade by showing findings like pericardial effusion, right ventricular collapse, and reduced respiratory variation in blood flow velocities.
- Tamponade is a medical emergency treated initially with medications and peric
Mitral valve
The mitral valve lies between the left atrium and left ventricle. Mitral stenosis is usually caused by rheumatic fever which causes scarring of the mitral valve leaflets and commissures. In early mitral stenosis, the leaflets can open but have restricted motion. Over time, the leaflets become thickened and rigid, reducing valve opening. This causes symptoms like dyspnea and pulmonary hypertension. On examination, findings may include an irregular pulse from atrial fibrillation, elevated jugular venous pressure, accentuated S1, and a diastolic murmur. Severe mitral stenosis can lead to right heart failure and complications like hemoptysis.
Echo in restrictive cardiomyopathy
This document provides an overview of echocardiographic evaluation of restrictive cardiomyopathy. Key points include:
- Restrictive cardiomyopathy is characterized by a nondilated left ventricle with abnormal diastolic function and typically normal systolic function.
- Causes include infiltrative diseases like amyloidosis and storage diseases. Echocardiography can help diagnose but it is more difficult than other cardiomyopathies.
- Findings include low diastolic volume, normal ejection fraction, diastolic dysfunction with rapid early mitral inflow. Echocardiography helps differentiate restrictive cardiomyopathy from constrictive pericarditis.
Sinus of valsalva aneurysm
This document summarizes the history and classification of sinus of Valsalva aneurysm (SOVA). Some key points:
- SOVA was first described in 1839 and the first successful repair was in 1956 using cardiopulmonary bypass.
- SOVAs can be congenital or acquired due to various connective tissue/inflammatory disorders.
- The majority originate from the right coronary cusp (77%) and most commonly rupture into the right ventricle (67.9%).
- The classic Sakakibara classification categorizes SOVAs arising from the right coronary cusp into three types based on location of rupture/protrusion. A modified classification exists for non-cor
Prosthetic valve thrombosis
Prosthetic valve thrombosis (PVT) occurs when a blood clot forms on an artificial heart valve. It is more common with mechanical valves compared to bioprosthetic valves. Risk factors include being under-anticoagulated, poor cardiac output, pregnancy, and hypercoagulable states. Echocardiography can diagnose PVT by detecting increased transvalvular gradients, thickened valve cusps, limited cusp motion, or visible thrombus. For severe PVT with heart failure symptoms, emergency surgery is recommended. For less severe cases, fibrinolytic therapy can restore normal valve function in 64% of patients and is a reasonable first-line treatment alternative to surgery.
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Aortic Regurgitation - Rivin
Aortic regurgitation occurs when the aortic valve does not close properly, allowing blood to flow back into the left ventricle. It can be caused by conditions that damage the aortic valve such as rheumatic fever or a congenital heart defect. Symptoms may include breathlessness, fatigue, and chest pain. Diagnosis is made through echocardiogram which can assess the severity. Treatment depends on severity but may involve lifestyle changes, medications, or aortic valve replacement surgery if symptoms worsen or damage to the heart progresses. Prognosis depends on severity and treatment, with severe untreated cases having a high risk of heart failure or sudden cardiac death.
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2) The pathophysiology of constrictive pericarditis where the inelastic pericardium constrains cardiac filling and prevents adaptation to volume changes.
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- Eisenmenger syndrome is caused by defects like VSDs, ASDs, and PDA that allow high blood flow to the lungs and cause pulmonary hypertension over time.
- Common causes of death include hemoptysis from pulmonary artery ruptures, heart failure, and complications from attempted defect repair surgery.
- Pathological findings show thickened pulmonary arteries that resemble the fetal pattern and contribute to high pulmonary vascular resistance.
- Medical treatments are generally ineffective once int
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1) Prosthetic heart valve thrombosis can occur with both mechanical and biological valves and is influenced by surface factors, hemodynamic factors, and hypercoagulability.
2) Clinical presentation may include heart failure symptoms or embolic events, and imaging with TEE is the standard for evaluation.
3) Treatment depends on severity of symptoms and includes anticoagulation, fibrinolytic therapy, or emergency surgery for severe cases.
ECHOCARDIOGRAPHIC EVALUATION OF AORTIC STENOSIS
This document discusses the evaluation of aortic stenosis using echocardiography. It begins by describing the normal anatomy of the aortic valve and sinus of Valsalva. It then discusses various types of aortic stenosis including calcific, rheumatic, bicuspid aortic valve, and supravalvular stenosis. The document outlines how to assess aortic stenosis using 2D echo, M-mode, Doppler, and measurements of peak jet velocity to calculate pressure gradients across the valve. It emphasizes the importance of Doppler assessment and discusses limitations and sources of error in quantifying aortic stenosis severity by echocardiography.
Av canal defect
This document discusses atrioventricular canal defects (AVSDs), including their embryogenesis and pathophysiology. It describes the anatomy and classification of partial and complete AVSDs. Partial AVSDs involve a primum atrial septal defect with two distinct but contiguous AV valves, while complete AVSDs have a single common AV valve. The embryogenesis of AVSDs involves faulty development of the endocardial cushions. The document provides detailed descriptions and images of the anatomy and features of partial and complete AVSDs. It discusses the clinical aspects of AVSDs including prevalence, association with Down syndrome, surgical repair outcomes, and lifelong surveillance needs.
Dextrocardia
This document discusses three types of dextrocardia:
1. Situs inversus totalis, where the organs are mirrored and the cardiac apex is located on the right side.
2. Dextroversion with situs solitus, where the ventricles can rotate to the right side and defects like VSD and pulmonary stenosis are common.
3. Dextroposition, where external factors like lung hypoplasia cause the heart to be displaced to the right side, and defects causing left-to-right shunts often occur.
Diagnosis involves inspection of the precordium, auscultation, chest X-ray, and echocardiogram. Situs
ARVD
ARVC is a heritable heart muscle disorder that predominantly affects the right ventricle. It is caused by genetic defects in cardiac desmosomes, which are important for cell-to-cell adhesion. This leads to progressive loss of right ventricular myocardium and replacement by fibrofatty tissue. ARVC can cause dangerous ventricular arrhythmias and is a leading cause of sudden cardiac death in young people. Diagnosis involves imaging tests and electrocardiography to detect right ventricular structural abnormalities and arrhythmias.
Aortic disection
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- Clinical presentation varies from neonatal congestive heart failure to later cyanosis, arrhythmias, and right heart failure in adults. Associated defects are common.
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Tetralogy of Fallot : Dr. Akif Baig
Tetralogy of Fallot (TOF) is a congenital heart defect, which has four anatomical components:
Anterior malalignment ventricular septal defect (VSD)
Aortic override over the muscular septum
Variable degrees of subvalvar, valvar, and supravalvar pulmonary stenosis
Right ventricular (RV) infundibular narrowing and RV hypertrophy
Aortic Regurgitation - Rivin
Aortic regurgitation occurs when the aortic valve does not close properly, allowing blood to flow back into the left ventricle. It can be caused by conditions that damage the aortic valve such as rheumatic fever or a congenital heart defect. Symptoms may include breathlessness, fatigue, and chest pain. Diagnosis is made through echocardiogram which can assess the severity. Treatment depends on severity but may involve lifestyle changes, medications, or aortic valve replacement surgery if symptoms worsen or damage to the heart progresses. Prognosis depends on severity and treatment, with severe untreated cases having a high risk of heart failure or sudden cardiac death.
Congenital heart diseases
Congenital heart diseases are abnormalities present at birth that are caused by defects in heart development during weeks 3-8 of gestation. They range from mild to severe. The most common types are atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), tetralogy of Fallot, transposition of the great arteries, and coarctation of the aorta. Left-to-right shunts cause increased pulmonary blood flow while right-to-left shunts allow deoxygenated blood to bypass the lungs, causing cyanosis. Most defects are now surgically corrected.
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The document discusses constrictive pericarditis, providing details on:
1) The pathology of constrictive pericarditis which involves thickening and scarring of the pericardium leading to loss of elasticity.
2) The pathophysiology of constrictive pericarditis where the inelastic pericardium constrains cardiac filling and prevents adaptation to volume changes.
3) Key diagnostic features of constrictive pericarditis seen on echocardiogram include septal bounce, rapid early diastolic mitral inflow, and increased mitral annular velocities that rise with inspiration.
Mitral valve scoring before BMV
This document discusses various echocardiographic scoring systems used to assess mitral valve anatomy and predict outcomes of percutaneous balloon mitral valvuloplasty (PBMV). The Wilkins score and Commissural Calcification score are described in detail. The Wilkins score grades leaflet thickening, mobility, calcification and subvalvular involvement on a scale of 4-16. A score ≤8 indicates favorable anatomy for PBMV. The Commissural Calcification score quantifies calcification at each commissure. Other discussed scores include the Cormier score, RT-3DE score, Chen score, Reid score and Nobuyoshi score. Limitations of the scoring systems and ideas for an ideal future scoring
L tga anatomy, management-
- L-TGA, also known as corrected transposition of the great arteries, is a rare congenital heart defect where the ventricles are transposed and the atrioventricular valves are discordant.
- The embryological cause is abnormal leftward looping of the heart during development, resulting in the morphologic right ventricle being on the left side and pumping blood to the lungs, while the morphologic left ventricle is on the right side and pumps blood to the body.
- Associated abnormalities are common, including ventricular septal defects, pulmonary stenosis, tricuspid valve anomalies, and conduction system abnormalities. Long term, the right ventricle is poorly suited to function as the systemic
EISENMENGER SYNDROME- PAUL WOOD
This document discusses Eisenmenger syndrome, a condition where pulmonary hypertension develops due to increased blood flow through defects between the systemic and pulmonary circulations. It provides details on causes, clinical features, pathology findings, and treatments. Key points include:
- Eisenmenger syndrome is caused by defects like VSDs, ASDs, and PDA that allow high blood flow to the lungs and cause pulmonary hypertension over time.
- Common causes of death include hemoptysis from pulmonary artery ruptures, heart failure, and complications from attempted defect repair surgery.
- Pathological findings show thickened pulmonary arteries that resemble the fetal pattern and contribute to high pulmonary vascular resistance.
- Medical treatments are generally ineffective once int
Prosthetic valve thrombosis
1) Prosthetic heart valve thrombosis can occur with both mechanical and biological valves and is influenced by surface factors, hemodynamic factors, and hypercoagulability.
2) Clinical presentation may include heart failure symptoms or embolic events, and imaging with TEE is the standard for evaluation.
3) Treatment depends on severity of symptoms and includes anticoagulation, fibrinolytic therapy, or emergency surgery for severe cases.
ECHOCARDIOGRAPHIC EVALUATION OF AORTIC STENOSIS
This document discusses the evaluation of aortic stenosis using echocardiography. It begins by describing the normal anatomy of the aortic valve and sinus of Valsalva. It then discusses various types of aortic stenosis including calcific, rheumatic, bicuspid aortic valve, and supravalvular stenosis. The document outlines how to assess aortic stenosis using 2D echo, M-mode, Doppler, and measurements of peak jet velocity to calculate pressure gradients across the valve. It emphasizes the importance of Doppler assessment and discusses limitations and sources of error in quantifying aortic stenosis severity by echocardiography.
Av canal defect
This document discusses atrioventricular canal defects (AVSDs), including their embryogenesis and pathophysiology. It describes the anatomy and classification of partial and complete AVSDs. Partial AVSDs involve a primum atrial septal defect with two distinct but contiguous AV valves, while complete AVSDs have a single common AV valve. The embryogenesis of AVSDs involves faulty development of the endocardial cushions. The document provides detailed descriptions and images of the anatomy and features of partial and complete AVSDs. It discusses the clinical aspects of AVSDs including prevalence, association with Down syndrome, surgical repair outcomes, and lifelong surveillance needs.
Dextrocardia
This document discusses three types of dextrocardia:
1. Situs inversus totalis, where the organs are mirrored and the cardiac apex is located on the right side.
2. Dextroversion with situs solitus, where the ventricles can rotate to the right side and defects like VSD and pulmonary stenosis are common.
3. Dextroposition, where external factors like lung hypoplasia cause the heart to be displaced to the right side, and defects causing left-to-right shunts often occur.
Diagnosis involves inspection of the precordium, auscultation, chest X-ray, and echocardiogram. Situs
ARVD
ARVC is a heritable heart muscle disorder that predominantly affects the right ventricle. It is caused by genetic defects in cardiac desmosomes, which are important for cell-to-cell adhesion. This leads to progressive loss of right ventricular myocardium and replacement by fibrofatty tissue. ARVC can cause dangerous ventricular arrhythmias and is a leading cause of sudden cardiac death in young people. Diagnosis involves imaging tests and electrocardiography to detect right ventricular structural abnormalities and arrhythmias.
Aortic disection
This document provides information about a seminar on aortic dissection presented by Monika Devi. It discusses the introduction, types, causes, symptoms, risk factors, diagnosis, treatment, complications and prevention of aortic dissection. The two main types are Type A, which involves the ascending aorta, and Type B, which only involves the descending aorta. Causes include high blood pressure, genetic conditions like Marfan syndrome, and traumatic injury. Symptoms can include chest pain and symptoms of a stroke. Treatment depends on the type but may involve medications to lower blood pressure or surgery to repair the tear in the aorta. Complications can include death, organ damage or stroke if not properly treated.
Strain and strain rate
This document discusses strain and strain rate imaging techniques used to quantify regional myocardial function. It describes various methods to measure strain, including tissue Doppler, 2D speckle tracking, and cardiac MRI. It outlines normal values and patterns of strain in healthy individuals and how strain is altered in various cardiac diseases, such as coronary artery disease, heart failure, cardiomyopathies, and congenital heart disease. Strain imaging can identify myocardial scar, viability, dysfunction, and response to treatments.
Mitral regurgitation by dr kalpana chetia
Mitral regurgitation - rheumatic heart disease is the commonest cause of MR. It coexists with MS and AR, particularly Ar
Aortic dissection 2015
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.
Constrictive pericarditis
Constrictive pericarditis is caused by scarring and thickening of the pericardium, restricting cardiac filling. It is diagnosed using echocardiography which shows septal bounce, exaggerated mitral inflow, and hepatic vein reversal. While similar to restrictive cardiomyopathy, constrictive pericarditis shows increased ventricular interaction and respiratory effects on cardiac physiology. Surgical pericardiectomy is usually required for treatment but is high risk, with post-op complications common. Long-term survival depends on the underlying cause of constriction.
EBSTEIN ANOMALY
This document provides information on Ebstein's anomaly, including its anatomy, embryology, clinical presentation, diagnosis, and natural history. Some key points:
- Ebstein's anomaly is a congenital defect involving downward displacement of the tricuspid valve into the right ventricle. This can cause dilation of the right atrium and dysfunction of the right ventricle.
- Clinical presentation varies from neonatal congestive heart failure to later cyanosis, arrhythmias, and right heart failure in adults. Associated defects are common.
- Diagnosis is made through echocardiogram demonstrating displacement of the tricuspid valve leaflets. Other tests like ECG, chest x-ray, and
Left ventricular diastolic dysfunction in echocardiography
Left ventricular diastolic dysfunction refers to the heart's inability to properly relax and fill during diastole. It can be caused by primary myocardial diseases like cardiomyopathy, hypertension, or secondary issues like aortic stenosis. Diagnosis involves evaluating left ventricular mass, dimensions, and function using 2D echocardiography, Doppler ultrasound to assess mitral inflow and pulmonary vein patterns, and tissue Doppler imaging of mitral annular motion. Diastolic dysfunction is graded from mild to severe based on these evaluation findings.
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Cmp
This document provides information on various types of cardiomyopathy and myocarditis. It discusses the causes, clinical features, diagnostic evaluation and management of conditions such as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, left ventricular noncompaction, arrhythmogenic right ventricular cardiomyopathy, endocardial fibroelastosis, and myocarditis. The document contains detailed information on the pathogenesis, clinical presentation, investigations including echocardiography, MRI and endomyocardial biopsy, and treatment of these conditions.
Large vessel disease
Large vessel disease, specifically aortic aneurysms and dissections, can affect the ascending aorta, aortic arch, descending thoracic aorta, and abdominal aorta. Risk factors include conditions that increase wall stress like hypertension, as well as genetic syndromes involving connective tissue abnormalities. Clinical presentation of abdominal aortic aneurysms may include pain, palpable masses, or hypotension from rupture. Diagnosis involves imaging like CT to measure aneurysm size. Surgical or endovascular repair is indicated for ruptured aneurysms or those over 5.5cm in size. Thoracic aortic aneurysms and dissections also involve medial degeneration and increased wall stress, with acute presentations including pain and pulse deficits. Gen
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Rheuumatic heart disese
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Sva
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This document discusses various types of arrhythmias and heart conditions including their causes, characteristics, and clinical presentations. It covers topics such as:
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Acute aortic dissection is a potentially life-threatening condition where blood flows into the layers of the aortic wall, splitting it apart. It occurs in approximately 2.9 per 100,000 people per year in the US. There are several classification systems, but the most important distinguishes between type A dissections, which involve the ascending aorta and require surgery, and type B dissections, which do not involve the ascending aorta and can be treated medically. Risk factors include hypertension, connective tissue disorders like Marfan syndrome, bicuspid aortic valves, pregnancy, and trauma. Prompt diagnosis and treatment are crucial as the risk of death is approximately 1% per hour without treatment.
valvularheartdisease-180524125726 (1).pptx
This document discusses various types of valvular heart disease, including their causes, pathophysiology, clinical manifestations, investigations, and management. It covers mitral stenosis, mitral regurgitation, mitral valve prolapse, aortic stenosis, aortic regurgitation, tricuspid stenosis, and tricuspid regurgitation. For each condition, it provides details on etiology, effects on heart function, common symptoms, diagnostic tests, medical and surgical treatment options, and patient education points.
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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.
Coarctation Of Aorta
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Cardiology questions
- 1. Cardiology Questions University of UTAH Dr Ihab Suliman
- 3. • (D) This is a rare neoplasm (all cardiac neoplasms are rare) but the size alone suggests a malignancy.
- 4. • Nearly 80% of cardiac angiosarcomas arise as mural masses in the right atrium. Typically, they completely replace the atrial wall and fill the entire cardiac chamber. • They may invade adjacent structures (eg, vena cava, tricuspid valve).
- 6. • C) After about 4 days following infarction, the muscle will still be necrotic and many neutrophils will persist, while macrophage infiltration will be beginning
- 8. • C). Mitral valve stenosis leads to left atrial enlargement, but the left ventricle is usually small. • THere is typically a 'fishmouth' shaped mitral valve that has stenosis as well as insufficiency, since it does not close completely. Most mitral valvular disease in adults results from rheumatic valvulitis.
- 10. • C) CORRECT. A stab wound into heart can lead to hemopericardium with tamponade
- 12. • (D) Marfan syndrome is a connective tissue disorder that is associated with floppy mitral valve and also with cystic medial necrosis that predisposes to aortic dissection.
- 13. • Marfan syndrome (It is inherited as a dominant trait. It is carried by a gene called FBN1, which encodes a connective protein called fibrillin-1. • People have a pair of FBN1 genes. Because it is dominant, people who have inherited one affected FBN1 gene from either parent will have Marfan's.
- 14. • The normal fibrillin-1 protein binds to another protein, transforming growth factor beta (TGF-β). TGF-β has deleterious effects on vascular smooth muscle development and the integrity of the extracellular matrix. Researchers now believe that secondary to mutated fibrillin there is excessive TGF-β at the lungs, heart valves, and aorta, and this weakens the tissues and causes the features of Marfan Syndrome.
- 15. • Since angiotensin II receptor blockers (ARBs) also reduce TGF-β, they have tested this by giving ARBs (losartan, etc.) to a small sample of young, severely affected Marfan syndrome patients. In some patients, the growth of the aorta was indeed reduced.
- 17. • (B) She has coarctation of the aorta, and the constriction is postductal, allowing prolonged survival. Her physical characteristics also suggest Turner syndrome (monosomy X).
- 19. • D) The small arteries of the kidney are affected by hyperplastic arteriolosclerosis. Malignant hypertension is often preceded by chronic hypertension that leads to left ventricular hypertrophy. Hypertension is a risk for CNS hemorrhage
- 21. • D) The bioprosthesis has the advantage of not requiring anticoagulation, but it does not wear well with time, and typically must be replaced within 5 to 10 years when its leaflets undergo progressive calcification leading to stenosis.
- 23. • A) The most common cause for a primary myocarditis is a virus (such as Coxsackie virus). • Viral myocarditis can be a cause for sudden death in a young person.
- 25. • (B) The history points to infectious endocarditis and acute congestive heart failure. Staphylococcus aureus and Pseudomonas aeruginosa are the most likely organisms to be found with a history of injection drug use.
- 27. • Biatrial enlargement • Left anterior fascicular block • Left ventricular hypertrophy
- 29. • AF