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Congenital Heart Diseases:When do most Congenital Heart Diseases arise?Week 3-8: OrganogenesisWhat Congenital Disorder is caused by Rubella?Patent Ductus Arteriosus (PDA)What Congenital Disorder does Down’s Syndrome cause?Endocardial Cushion DefectOstium PrimumWhat Congenital Disorder does Turner Syndrome cause?Coarctation of the AortaWhat Congenital Disorder does Edward’s Syndrome cause?Ventricular Septal Defect (VSD)What Congenital Disorder does Cri du Chat Syndrome cause?Ventricular Septal Defect (VSD)Which type of shunt (RL or LR) causes Early Cyanosis?Right LeftWhich type of shunt (RL or LR) causes Late Cyanosis?Left RightWhat is the Eisenmenger Complex?Late Cyanosis with the reversal of the LR ShuntHappens when the pressure in the Pulmonary Trunk increases higher than the pressure in the Aorta due to the Pulmonary Hypertension caused by the LR ShuntWhat is the most common Congenital Heart Defect?Ventricular Septal Defect (VSD)What is the most common Congenital Heart Defect discovered in adults?Atrial Septal Defect (ASD)Less blood flow less pressure presents laterWhat is the most common Cyanotic Congenital Heart Defect?Teralogy of Fallot - usually not cyanotic at birthTransposition of the Great Vessels is the most common cause of Cyanosis immediately at birth.What are the 3 causes of Left Right Shunts?Ventricular Septal Defect (VSD)Atrial Septal Defect (ASD)Patent Ductus Arteriosis (PDA)Ventricular Septal Defects:What are the two types of VSD?MembranousMuscularHow do you tell the difference between a Membranous VSD and a Muscular VSD?A Membranous VSD is by the valve. It is usually a single hole, and large.A Muscular VSD is not by the valve. It is usually multiple, smaller holes.What are the steps that cause the shift from LR to RL Shunt in VSD?Shunt starts out LRThe blood keeps going back into the Right Heart, causing the Pulmonary Artery Blood Flow to increase.This causes Pulmonary Hypertension with Right Ventricular HypertrophyEventually, the Pulmonary Hypertension causes the pressure in the Pulmonary Trunk to be greater than the pressure in the Aorta.This causes the shift from LR to RL(Eisenmenger Complex)What kind of murmur would you hear in a patient with VSD?Holosystolic MurmurAtrial Septal Defects:What are the three types of Atrial Septal Defects?Ostium PrimumOstium SecundumSinus VenosumWhat is the most common type of ASD?Ostium SecundumWhat is the most common type of ASD in Down’s Syndrome patients?Osterium PrimumWhere in the heart is the Ostium Secundum usually found?Foramen OvaleWhat kind of murmur would you hear in a patient with ASD?Diastolic Murmur with fixed splitting.Patent Ductus Arteriosus:What are some causes of a Patent Ductus Arteriosus?HypoxemiaAcidosisPrematurityWhat kind of murmur would you hear in a patient with PDA?Machinery MurmurWhat does Prostaglandins do to the time of closure?Delay ClosureWhat does Indomethacin do to the time of closure?Promotes ClosureWhat are the 5 causes of RightLeft Shunts?5 T’sTetralogy of FallotTransposition of the Great VesselsTruncus ArteriosusTricuspid AtresiaTotal Anomalous Pulmonary Venous ReturnWhat are the 4 Components of Tetralogy of Fallot?VSDPulmonary Valvular StenosisRight Ventricular HypertrophyDextraposition of the Aorta (Aorta straddles the septum)What are some clinical features of Tetraology of Fallot?Boot-Shaped Heart on X-RayPulmonary HypotensionWhat are some complications of having Tetraology of Fallot?EndocarditisCerebral Abscesses (S. Aureus Septic Embolus)Secondary Polycythemia ThrombosisClubbing of FingersWhat is characteristic of the mothers who have babies with Transposition of the Great Vessels?Diabetic MothersWhat do babies with Transposition of the Great Vessels need to survive?A PDA, VSD, or ASD to mix up the oxygenated blood.What is characteristic on inspection of a heart of a patient with Transposition of the Great Vessels?Egg-Shaped HeartAorta is Anterior to the Pulmonary Trunk (should be Posterior)Right Ventricular Hypertrophy because now the Right Heart is pumping blood to the body, and the Left Heart is pumping blood only to the lungs. What does it mean when a baby is born with a Truncus Arteriosus?The Embryologic Truncus Arteriosus did not separate into the Aorta and the Pulmonary Artery like it should have. Blood is received from both the right and left ventricles to a single, great artery.What does it mean when a baby is born with Tricuspid Atresia?Complete occlusion of the tricuspid valve orifice..What 2 defects are associated with this?Atrial Septal Defect (to get blood from RL Atrial)Hypoplastic Right Ventricle (Its not being used)What does it mean when a baby is born with Total Anomalous Pulmonary Venous Connection?The Pulmonary Veins fail to connect to the Left Atrium. Blood is returned to the Right Atrium.Left Heart never gets oxygenated blood.Coarctation of the Aorta:What is Coarctation of the Aorta?Narrowing or Constriction of the AortaWhat are the two types?Adult: Post-DuctalInfantile: Pre-DuctalWhat happens in Pre-Ductal Coarctation?The constriction is proximal to the PDAThe Aortic Arch receives oxygenated blood.PDA supplies deoxygenated blood to the lower extremitiesWhat side of the heart hypertrophies in Pre-Ductal?The Right SideWhat happens in Post-Ductal Coarctation?Ductus Arteriosus is closed (Ligamentum Arteriosum)Hypertension in the upper extremities.Diminished or absent femoral pulses. Prominent circulation through intercostal and internal mammary arteries NOTCHING OF RIBS ON X-RAYWhat side of the heart hypertrophies in Post-Ductal?The Left Side Acquired Heart Diseases I:What is the definition of Congestive Heart Failure?Inability of Cardiac Output to meet the Metabolic Requirements of tissues or organs.What is the Cardiac Output usually like in patients with Congestive Heart Failure?LowWhat are the two ways to classify a Congestive Heart Failure case?Systolic Dysfunction (Forward Failure)Diastolic Dysfunction (Backward Failure)What are the three diseases that caused Systolic Dysfunction?Myocardial InfarctionDilated CardiomyopathyValvular DiseaseWhat are the three diseases that cause Diastolic Dysfunction?Myocardial HypertrophyRestrictive CardiomyopathyObstruction to Left Ventricular FillingWhat disease caused Heart Failure with a HIGH Cardiac Output?Grave’s DiseaseBeri Beri: Thyamine DeficiencyWet: HeartDry: Brain (Wernicke’s Korsakoff) Paget’s Disease of the Bone (AV Shunts)Pregnancy (Triplets, Quadruplets)Severe AnemiaWhat are the only two things that cause Right Heart Failure?Left Heart FailurePulmonary HypertensionHypertension Heart Disease:What part of the heart hypertrophies during HHD?The Left Ventricular Hypertrophies in a Concentric fashion (the wall and the septum both hypertrophy)Concentric = Pressure OverloadDoes HHD cause Systolic or Diastolic dysfunction?Diastolic DysfunctionAtrial EnlargementWhat would Hypertensive Heart Disease look like microscopically?The Myocytes would be enlarged (hypertrophy)Cigar-Shaped/Boxcar-Shaped Nuclei (Larger than normal)Left Heart Failure:What kind of clinical features would you see in a patient with LHF?DyspneaOrthopnea (How many pillow do they need propped with to feel comfortable breathing??)Cough, Wheezing, Basal RalesThird Heart SoundHow does LHF affect the Heart? Lungs? Brain?Heart: Left Ventricular Hypertrophy/Dilatation + Atrial DilatationLung: Congestion/Edema (Pulmonary EDEMA)Brain: HypoxiaWhat would LHF look like microscopically?Heart Failure CellsHemosiderin-Laden MacrophagesRight Heart Failure:What are the two diseases that cause Right Heart Failure?Left Heart FailurePulmonary HypertensionWhat are some clinical features of Right Sided Heart Failure?Dependent Edema, AscitesPleural EffusionJugular Venous DistentionEnlarged, Tender LiverHow does RHF affect the Heart? Liver, Kidney, Spleen? Brain?Heart: Right Ventricular Hypertrophy/Dilatation + Atrial DilatationLiver, Kidney, Spleen: Passive CongestionNutmeg LiverBrain: HypoxiaCor Pulmonale:What is Cor Pulmonale?Right Sided Heart Failure caused by Pulmonary HypertensionWhat are the two types of Cor Pulmonale?Acute Cor PulmonaleChronic Cor PulmonaleWhat causes Acute Cor Pulmonale?Massive Pulmonary EmbolizationDilatation of the Right HeartWhat causes Chronic Cor Pulmonale?Chronic Pulmonary HypertensionHypertrophy of the Right HeartIschemic Heart Disease:What is the main cause of Ischemic Heart Disease?Atherosclerosis – 90% of CasesWhat are some examples of Non-Atherosclerotic causes of IHD?Aortic Dissection, Syphyilitic Aortitis, Thomboembolization, PAN, Takayasu Arteritis, Kawasaki DiseaseWhat are 4 Clinical Syndromes associated with IHD?Angina Pectoris (Stable, Printzmetal, Unstable)Myocardial InfarctionChronic IHD with Heart FailureSudden Cardiac DeathWhat is characteristic of Stable/Typical Angina?Most CommonFixed Atheromatous Narrowing Pain upon Exertion – Relieved by RestS-T Segment DepressionWhat is characteristic of Prinzmetal/Variant Angina?Coronary Artery SpasmsPain at RestS-T Segment ElevationWhat is characteristic of Unstable/Crescendo Angina?Progressive, Complicated AtheromasCaused by the disruption of an atherosclerotic plaque with a superimposed partial (mural) thrombus.Leads to MIPatient has a progressive increase in the frequency, duration, and severity of pain.Myocardial Infarction:What are some clinical presentations for patients with MI?Variable Substernal PainRapid, Weak Pulse and Profuse Sweating (Diaphoresis)DyspneaCan be AsymptomaticWhat are the two types of Myocardial Infarction?TransmuralSubendocardialWhat do you see on an EKG if the patient has a Transmural MI?Presence of Q-WaveS-T Segment ElevationWhat do you see on an EKG if the patient has a Subendocardial MI?Absence of a Q-WaveS-T Segment DepressionWhat two enzymes are used to measure MI?TroponinCK-MB/CK2When do Troponin and CK2 rise?2-6 hours after the MI for both.How long do Troponin and CK2 stay elevated?Troponin: 6-7 DaysCK-MB: 3 DaysWhat are the 4 consequences of a MI?Contractile DysfunctionArrhythmiasMyocardial RupturePericarditisWhat would you expect to see within the first hour of an MI?NothingWhat would you expect to see within the first 4-24 hours of an MI?Waviness of Fibers at Border – Contraction BandsCoagulative NecrosisWhat would you expect to see within the first 2-7 days of an MI?Large Neutrohillic Infiltrate (Come in 24 hours, Leave in 3 days)Yellow-Tan with Hyperemic Border (blood tries to seep back in)Beginning of Granulation Tissue at day 7What would you see 7-10 days after an MI?Well-Developed Granulation TissueMaxillary Yellow-Tan in appearanceWhat would you see 2 Weeks after an MI?Scar TissueCollagen DepositionWhat stain would you use to stain Granulation Tissue?Trichrome StainStains collagenWhat are 4 complications of MI that can lead to death?Cardiac ArrhythmiaCardiogenic ShockCongestive Heart FailureThromboembolizationWhat are some other complications of MI?Myocardial RupturePapillary Muscle RuptureMural ThrombosisVentricular AneurysmFibrinosis Pericarditis When would you see a Myocardial Rupture occur after an MI?Day 4-7When Granulation Tissue is first formingWhat are the consequences of the rupture?Blood flows out into the Pericardial SacCauses Cardiac Tamponade HypotensionBlood cannot move out of the heart because the leaked blood compresses the heart.Muffled Heart SoundsJVDAcquired Heart Diseases II:Aortic Stenosis:What causes Calcific Aortic Stenosis?Wear and Tear in ElderlyWhat type of Calcification is seen in Aortic Stenosis?Dystrophic CalcificationWhat do patients with Calcific Aortic Stenosis present with?SyncopeAngina on ExertionWhat two diseases is this associated with?Rheumatic Heart Disease (Mitral is more common)Congenital Bicuspid ValveIf you have a Calcific Aortic Valve, what organism can cause Bacterial Endocarditis secondary to the valve problem?Streptococcus ViridansMitral Valve Prolapse:What causes Mitral Valve Prolapse?Myxoid Degeneration – Floppy ValveWhat does the Prolapsed Mitral Valve look like?A ballooning or hooding valveWhat do you hear on auscultation?Mid-Systolic ClickWhat disease is this associated with?Marfan’s SyndromeWhat is a major complication of this disease?Most common valve associated with Streptococcus ViridansIs the heart hypertrophied?Yes, the Left Ventricle is hypertrophied due to the regurgitation of the Mitral Valve due to the ProlapseRheumatic FeverWhat organism causes Rheumatic Fever?Group A, Beta Hemolytic Streptococcal Pyogenes Pharyngitis What is the general age of those who get Rheumatic Fever?Children/TeensWhat is detected in the patient’s serum?Anti-Streptolysin O Antibodies (ASO Titers)What does the immune system cross react with?M ProteinsWhat are the two major criteria to diagnose Rheumatic Fever?Migratory, Asymmetric PolyarthritisPancarditisWhat are the nodules seen on microscopic slides of patients with Rheumatic Fever? How are these characterized?Aschoff BodiesGranulomatousMultinucleated Giant CellsWhat are the cells called that are inside of the Ascoff Bodies?Anitschkow (Catapillar) CellsWhat are the 3 Morphological Patterns associated with Rheumatic Fever?MyocarditisAschoff BodiesEarly MortalityPericarditisFibrinous “Bread and Butter” Exudate = Friction RubEndocarditisInvolves Valvular EndocardiumWhich valve is affected by Rheumatic Fever the most?Mitral ValveHow do you differentiate between Acute and Chronic Rheumatic Fever?Acute: Children, Small Vegetation at the Line of ClosureChronic: 10-15 Years Later, Fibrotic Fish-Mouth Deformity (Fusion of the Commissures)Infective Endocarditis (IE):What does the valve look like if the patient has Infective Endocarditis?Large, Beefy Red Vegetative Masses.Friable Masses EmbolizationInvolves the Free Margin of the ValveWhat are the two types?AcuteSubacuteHow do you differentiate between Acute and Subacute?Acute: Previously normal valve, Staph. Aureus, More DestructiveSubacute: Previously damaged valve, Staph. Viridans, Less DestructiveWhat bug and what valve is involved with IV Drug Use?Staph. AureusTricuspid Valve (first valve the drug comes to after the injection)Which type (Acute or Subacute) has the high-grade fever?AcuteWhat do you auscultate in these patients?A changing character of murmurs as the vegetation grows.What is characteristic of the Chronic Infective Endocarditis?The Small Septic Thrombi can break off and block blood supply elsewhere.Petechial Hemorrhages (Retina Roth’s Spots)Osler Nodes (Fingers and Toes)Janeway Lesions (Painless, Palms/Soles)What complications can the Chronic Infective Endocarditis lead to?StrokeGlomerulonephritisInfarctionHeart FailureAnemiaNon-Bacterial Thrombic EndocarditisWhat is another name for Non-Bacterial Thrombotic Endocarditis?Marantic Endocarditis – found in debilitated patientsWhat does the valve look like if the patient has Non-Bacterial Thrombic Endocarditis?Bland Vegetation Along the Line of ClosureYou can tell this from Rheumatic Fever because Rheumatic Fever is in children, while this is in the elderly.What can this be mistaken for?Mucinous Adenocarcinomas Hypercoagulable StateCarcinoid Heart DiseaseWhat does a heart with Carcinoid Heart Disease look like?Subendocardial Fibrosis of the RIGHT HEARTThe lungs have MAO, which degrades the tumor, so the Left Heart doesn’t have manifestationsWhat would be in the serum?Elevated Serotonin (5HT) and Metabolite (5-HIAA) because it is secreted by the tumor.Causes flushed face.Dilated CardiomyopathyWhat are some types of Cardiomyopathy?IdiopathicPost-Viral (Coxsackie)AlcoholPregnancyCardiotoxicityHemochromatosisWhat characteristic must the heart have to be diagnosed as a Dilated Cardiomyopathy?Progressive cardiac dilation of all 4 chambers.Low Ejection Fraction <40%Hypertrophic CardiomyopathyWhat is the typical patient that presents with Hypertrophic Cardiomyopathy?Young, Male, AthleteSome sort of Sport/ExerciseWhat is the inheritance pattern?Autosomal DominantWhat is the manifestations of the disease?Myofiber DisarrayAsymmetrical Septal Thickening Abnormal Diastolic FillingMyocardial HypertrophyBanana-Shaped Left Ventricular ChamberOutflow ObstructionRestrictive CardiomyopathyWhat are some diseases that cause Restrictive Cardiomyopathy?AmyloidosisSarcoidosisGlycogen Storage Disease (Pompe)What happens to the compliance in patients with this disease?Decrease in Ventricular Compliance Impaired Ventricular Filling during DiastoleIf the Restrictive Cardiomyopathy is caused by Amyloidosis, what would you see on microscopy?Deposits of Amyloid between the muscle fibers that are stained with Congo Red (Polarized Light)MyocarditisWhat is the most common cause of Myocarditis?Coxsackie A and B (B is more common)What are the Bacterial causes of Mayocarditis?Corynebacterium DiphtheriaExotoxin Fatty ChangeBorrelia BurgdorferiLyme DiseaseWhat would you see on a microscopic slide of Viral Myocarditis?Lymphocytic InfiltratePericarditis:What are the 4 diseases that cause Fibrous Pericarditis?RUMSR: Rheumatic FeverU: UremiaM: Myocardial InfarctionS: Systemic Lupus ErythematosisPericarditis can be Acute Suppurative or Acute Sanguinous. What causes the Acute Sanguinous Type?TuberculosisTumor InfiltrationThere are two types of Chronic Pericarditis – what are they?Adhesive MediastinopericarditisConstrictive PericarditisTumors:What are the three most common Benign Tumors found in the heart?Myxoma (Most Common)FibromaRhabdomyomaWhere in the heart is a Myxoma usually found?Left AtriumWhat are the two types of Myxoma?Sessile TypePedunculated Type Describe the Sessile TypeBroad-Based (Large Mass)Describe the Pedunculated TypePolypCan have Ball Valve/Wrecking Ball Effect on the Mitral Valve, causing damage.What is a Rhabdomyoma?Most frequent primary tumor of the heart in infants and children.Tumor of the Striated Muscle – Spider Cells seen MicroscopicallyTutoring Questions:Describe the Eisenmenger Complex:Found in Left Right ShuntsLR Shunt Increased Pulmonary Artery Blood Flow Pulmonary Hypertension Right Ventricular Hypertrophy Reversal of shunt with CyanosisHow do you treat PDA?Indomethacin: Promotes the closureWhat is the most common cause of PDA?Prematurity (Neonatal RDS) HypoxemiaHow can you determine the prognosis of Tetralogy of Fallot?The degree of Pulmonary StenosisWhat disease causes Pulmonary HYPOtension?Tetralogy of FallotWhat gives you a Boot-Shaped Appearance on X-Ray?Tetralogy of FallotWhat is required to survive Transposition of Great Vessels?A PDAKeep it open with ProstaglandinsWhich disease causes an egg-shaped heart?Transposition of Great VesselsWhich disease causes a Hypoplastic Right Ventricle?Tricuspid AtresiaPre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Cyanosis of the Lower Extremities?Pre-DuctalPre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes hypertension in the Upper Extremities?Post-DuctalPre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Notching of the Ribs?Post-DuctalPre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Right Ventricular Hypertrophy?Pre-DuctalPre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Diminished or absent femoral pulses?Post-Ductal What is a complication of MI after the white scar forms (>3 weeks)?Left Ventricular Aneurysm Mural Thrombus Left Sided EmbolismIf the MI is Subendocardial and less than 1 week old, what complication could the patient have?Rupture of the Papillary MuscleChange/New MurmurValvular InsufficiencyIf the MI is Transmural and less than 1 week old, what complication could the patient have?Myocardial Rupture Cardiac TamponadeMuffled Heart SoundsJVDWhat happens during Reperfusion Injury?The patient has an MI CK-MB rises in 2-6 hours CK-MB stays elevated for 3 days Patient was given tPA CK-MB started going down, but reperfusion injury occurred O2 produces free radicals Another increase in CK-MBWhat disorder does Myxoid Degeneration cause?Mitral Valve ProlapseWhat are the three components of an Aschoff Body? What disease is this associated with?GranulomatousAnitschkow CellsMultinucleated Giant CellsWhat are the two major jones criteria of Rheumatic Fever?Migratory, Asymmetrical PolyathritisPancarditisWhich heart disease causes Osler Nodes and Janeway Lesions?Infective EndocarditisWhat heart disease do IV Drug Users get?Infective EndocarditisStaph Aureus Tricuspid ValveWhat heart disease can be caused by Hemochromatosis?Dilated CardiomyopathyDIE HAPPIWhat heart disease can be caused by Pregnancy?Dilated CardiomyopathyDIE HAPPIWhat heart disease can be caused by Alcohol?Dilated CardiomyopathyDIE HAPPIWhat are 2 complications of Dilated Cardiomyopathy?Mural ThrombusCHFIf one patient comes in with dysphagia, and another comes in with MV Insufficiency Murmur), what disease do they BOTH have?MyxomaIf a cancer patient, being treated with radiation, comes into the office with a heart disease, which heart disease do they have?Chronic Pericarditis ................
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