Congenital Heart Diseases:

When do most Congenital Heart Diseases arise?o Week 3-8: Organogenesis

What Congenital Disorder is caused by Rubella?o Patent Ductus Arteriosus (PDA)

What Congenital Disorder does Down’s Syndrome cause?o Endocardial Cushion Defecto Ostium Primum

What Congenital Disorder does Turner Syndrome cause?o Coarctation of the Aorta

What Congenital Disorder does Edward’s Syndrome cause?o Ventricular Septal Defect (VSD)

What Congenital Disorder does Cri du Chat Syndrome cause?o Ventricular Septal Defect (VSD)

Which type of shunt (RL or LR) causes Early Cyanosis?o Right Left

Which type of shunt (RL or LR) causes Late Cyanosis?o Left Right

What is the Eisenmenger Complex?o Late Cyanosis with the reversal of the LR Shunto Happens when the pressure in the Pulmonary Trunk increases higher

than the pressure in the Aorta due to the Pulmonary Hypertension caused by the LR Shunt

What is the most common Congenital Heart Defect?o Ventricular Septal Defect (VSD)

What is the most common Congenital Heart Defect discovered in adults?o Atrial Septal Defect (ASD)o Less blood flow less pressure presents later

What is the most common Cyanotic Congenital Heart Defect?o Teralogy of Fallot - usually not cyanotic at birtho Transposition of the Great Vessels is the most common cause of

Cyanosis immediately at birth.

What are the 3 causes of Left Right Shunts?o Ventricular Septal Defect (VSD)o Atrial Septal Defect (ASD)o Patent Ductus Arteriosis (PDA)

Ventricular Septal Defects:

What are the two types of VSD?

o Membranouso Muscular

How do you tell the difference between a Membranous VSD and a Muscular VSD?

o A Membranous VSD is by the valve. It is usually a single hole, and large.

o 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?o Shunt starts out LRo The blood keeps going back into the Right Heart, causing the

Pulmonary Artery Blood Flow to increase.o This causes Pulmonary Hypertension with Right Ventricular

Hypertrophyo Eventually, the Pulmonary Hypertension causes the pressure in the

Pulmonary Trunk to be greater than the pressure in the Aorta.o This causes the shift from LR to RLo (Eisenmenger Complex)

What kind of murmur would you hear in a patient with VSD?o Holosystolic Murmur

Atrial Septal Defects:

What are the three types of Atrial Septal Defects?o Ostium Primumo Ostium Secundumo Sinus Venosum

What is the most common type of ASD?o Ostium Secundum

What is the most common type of ASD in Down’s Syndrome patients?o Osterium Primum

Where in the heart is the Ostium Secundum usually found?o Foramen Ovale

What kind of murmur would you hear in a patient with ASD?o Diastolic Murmur with fixed splitting.

Patent Ductus Arteriosus:

What are some causes of a Patent Ductus Arteriosus?o Hypoxemiao Acidosiso Prematurity

What kind of murmur would you hear in a patient with PDA?

o Machinery Murmur What does Prostaglandins do to the time of closure?

o Delay Closure What does Indomethacin do to the time of closure?

o Promotes Closure

What are the 5 causes of RightLeft Shunts?o 5 T’s

Tetralogy of Fallot Transposition of the Great Vessels Truncus Arteriosus Tricuspid Atresia Total Anomalous Pulmonary Venous Return

What are the 4 Components of Tetralogy of Fallot?o VSDo Pulmonary Valvular Stenosiso Right Ventricular Hypertrophyo Dextraposition of the Aorta (Aorta straddles the septum)

What are some clinical features of Tetraology of Fallot?o Boot-Shaped Heart on X-Rayo Pulmonary Hypotension

What are some complications of having Tetraology of Fallot?o Endocarditiso Cerebral Abscesses (S. Aureus Septic Embolus)o Secondary Polycythemia Thrombosiso Clubbing of Fingers

What is characteristic of the mothers who have babies with Transposition of the Great Vessels?

o Diabetic Mothers What do babies with Transposition of the Great Vessels need to survive?

o 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?o Egg-Shaped Hearto Aorta is Anterior to the Pulmonary Trunk (should be Posterior)o 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?o The Embryologic Truncus Arteriosus did not separate into the Aorta

and the Pulmonary Artery like it should have. o 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?o Complete occlusion of the tricuspid valve orifice..

What 2 defects are associated with this?o Atrial Septal Defect (to get blood from RL Atrial)o Hypoplastic Right Ventricle (Its not being used)

What does it mean when a baby is born with Total Anomalous Pulmonary Venous Connection?

o The Pulmonary Veins fail to connect to the Left Atrium. Blood is returned to the Right Atrium.

o Left Heart never gets oxygenated blood.

Coarctation of the Aorta:

What is Coarctation of the Aorta?o Narrowing or Constriction of the Aorta

What are the two types?o Adult: Post-Ductalo Infantile: Pre-Ductal

What happens in Pre-Ductal Coarctation?o The constriction is proximal to the PDAo The Aortic Arch receives oxygenated blood.o PDA supplies deoxygenated blood to the lower extremities

What side of the heart hypertrophies in Pre-Ductal?o The Right Side

What happens in Post-Ductal Coarctation?o Ductus Arteriosus is closed (Ligamentum Arteriosum)o Hypertension in the upper extremities.o Diminished or absent femoral pulses. o Prominent circulation through intercostal and internal mammary

arteries NOTCHING OF RIBS ON X-RAY What side of the heart hypertrophies in Post-Ductal?

o The Left Side

Acquired Heart Diseases I:

What is the definition of Congestive Heart Failure?o 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?o Low

What are the two ways to classify a Congestive Heart Failure case?o Systolic Dysfunction (Forward Failure)o Diastolic Dysfunction (Backward Failure)

What are the three diseases that caused Systolic Dysfunction?o Myocardial Infarctiono Dilated Cardiomyopathyo Valvular Disease

What are the three diseases that cause Diastolic Dysfunction?o Myocardial Hypertrophyo Restrictive Cardiomyopathyo Obstruction to Left Ventricular Filling

What disease caused Heart Failure with a HIGH Cardiac Output?o Grave’s Diseaseo Beri Beri: Thyamine Deficiency

Wet: Heart Dry: Brain (Wernicke’s Korsakoff)

o Paget’s Disease of the Bone (AV Shunts)o Pregnancy (Triplets, Quadruplets)o Severe Anemia

What are the only two things that cause Right Heart Failure?o Left Heart Failureo Pulmonary Hypertension

Hypertension Heart Disease:

What part of the heart hypertrophies during HHD?o The Left Ventricular Hypertrophies in a Concentric fashion (the wall

and the septum both hypertrophy)o Concentric = Pressure Overload

Does HHD cause Systolic or Diastolic dysfunction?o Diastolic Dysfunctiono Atrial Enlargement

What would Hypertensive Heart Disease look like microscopically?o The Myocytes would be enlarged (hypertrophy)o Cigar-Shaped/Boxcar-Shaped Nuclei (Larger than normal)

Left Heart Failure:

What kind of clinical features would you see in a patient with LHF?o Dyspneao Orthopnea (How many pillow do they need propped with to feel

comfortable breathing??)o Cough, Wheezing, Basal Raleso Third Heart Sound

How does LHF affect the Heart? Lungs? Brain?o Heart: Left Ventricular Hypertrophy/Dilatation + Atrial Dilatationo Lung: Congestion/Edema (Pulmonary EDEMA)o Brain: Hypoxia

What would LHF look like microscopically?o Heart Failure Cellso Hemosiderin-Laden Macrophages

Right Heart Failure:

What are the two diseases that cause Right Heart Failure?o Left Heart Failureo Pulmonary Hypertension

What are some clinical features of Right Sided Heart Failure?o Dependent Edema, Asciteso Pleural Effusiono Jugular Venous Distentiono Enlarged, Tender Liver

How does RHF affect the Heart? Liver, Kidney, Spleen? Brain?o Heart: Right Ventricular Hypertrophy/Dilatation + Atrial Dilatationo Liver, Kidney, Spleen: Passive Congestion

Nutmeg Livero Brain: Hypoxia

Cor Pulmonale:

What is Cor Pulmonale?o Right Sided Heart Failure caused by Pulmonary Hypertension

What are the two types of Cor Pulmonale?o Acute Cor Pulmonale

o Chronic Cor Pulmonale What causes Acute Cor Pulmonale?

o Massive Pulmonary Embolizationo Dilatation of the Right Heart

What causes Chronic Cor Pulmonale?o Chronic Pulmonary Hypertensiono Hypertrophy of the Right Heart

Ischemic Heart Disease:

What is the main cause of Ischemic Heart Disease?o Atherosclerosis – 90% of Cases

What are some examples of Non-Atherosclerotic causes of IHD?o Aortic Dissection, Syphyilitic Aortitis, Thomboembolization, PAN,

Takayasu Arteritis, Kawasaki Disease What are 4 Clinical Syndromes associated with IHD?

o Angina Pectoris (Stable, Printzmetal, Unstable)o Myocardial Infarctiono Chronic IHD with Heart Failureo Sudden Cardiac Death

What is characteristic of Stable/Typical Angina?o Most Commono Fixed Atheromatous Narrowing o Pain upon Exertion – Relieved by Resto S-T Segment Depression

What is characteristic of Prinzmetal/Variant Angina?o Coronary Artery Spasmso Pain at Resto S-T Segment Elevation

What is characteristic of Unstable/Crescendo Angina?o Progressive, Complicated Atheromaso Caused by the disruption of an atherosclerotic plaque with a

superimposed partial (mural) thrombus.o Leads to MIo Patient has a progressive increase in the frequency, duration, and

severity of pain.

Myocardial Infarction:

What are some clinical presentations for patients with MI?o Variable Substernal Paino Rapid, Weak Pulse and Profuse Sweating (Diaphoresis)o Dyspnea

o Can be Asymptomatic What are the two types of Myocardial Infarction?

o Transmuralo Subendocardial

What do you see on an EKG if the patient has a Transmural MI?o Presence of Q-Waveo S-T Segment Elevation

What do you see on an EKG if the patient has a Subendocardial MI?o Absence of a Q-Waveo S-T Segment Depression

What two enzymes are used to measure MI?o Troponino CK-MB/CK2

When do Troponin and CK2 rise?o 2-6 hours after the MI for both.

How long do Troponin and CK2 stay elevated?o Troponin: 6-7 Dayso CK-MB: 3 Days

What are the 4 consequences of a MI?o Contractile Dysfunctiono Arrhythmiaso Myocardial Ruptureo Pericarditis

What would you expect to see within the first hour of an MI?o Nothing

What would you expect to see within the first 4-24 hours of an MI?o Waviness of Fibers at Border – Contraction Bandso Coagulative Necrosis

What would you expect to see within the first 2-7 days of an MI?o Large Neutrohillic Infiltrate (Come in 24 hours, Leave in 3 days)o Yellow-Tan with Hyperemic Border (blood tries to seep back in)o Beginning of Granulation Tissue at day 7

What would you see 7-10 days after an MI?o Well-Developed Granulation Tissueo Maxillary Yellow-Tan in appearance

What would you see 2 Weeks after an MI?o Scar Tissueo Collagen Deposition

What stain would you use to stain Granulation Tissue?o Trichrome Staino Stains collagen

What are 4 complications of MI that can lead to death?o Cardiac Arrhythmiao Cardiogenic Shock

o Congestive Heart Failureo Thromboembolization

What are some other complications of MI?o Myocardial Ruptureo Papillary Muscle Ruptureo Mural Thrombosiso Ventricular Aneurysmo Fibrinosis Pericarditis

When would you see a Myocardial Rupture occur after an MI?o Day 4-7o When Granulation Tissue is first forming

What are the consequences of the rupture?o Blood flows out into the Pericardial Saco Causes Cardiac Tamponade Hypotension

Blood cannot move out of the heart because the leaked blood compresses the heart.

o Muffled Heart Soundso JVD

Acquired Heart Diseases II:

Aortic Stenosis:

What causes Calcific Aortic Stenosis?o Wear and Tear in Elderly

What type of Calcification is seen in Aortic Stenosis?o Dystrophic Calcification

What do patients with Calcific Aortic Stenosis present with?o Syncopeo Angina on Exertion

What two diseases is this associated with?o Rheumatic Heart Disease (Mitral is more common)o Congenital Bicuspid Valve

If you have a Calcific Aortic Valve, what organism can cause Bacterial Endocarditis secondary to the valve problem?

o Streptococcus Viridans

Mitral Valve Prolapse:

What causes Mitral Valve Prolapse?o Myxoid Degeneration – Floppy Valve

What does the Prolapsed Mitral Valve look like?o A ballooning or hooding valve

What do you hear on auscultation?o Mid-Systolic Click

What disease is this associated with?o Marfan’s Syndrome

What is a major complication of this disease?o Most common valve associated with Streptococcus Viridans

Is the heart hypertrophied?o Yes, the Left Ventricle is hypertrophied due to the regurgitation of the

Mitral Valve due to the Prolapse

Rheumatic Fever

What organism causes Rheumatic Fever?o Group A, Beta Hemolytic Streptococcal Pyogenes Pharyngitis

What is the general age of those who get Rheumatic Fever?o Children/Teens

What is detected in the patient’s serum?o Anti-Streptolysin O Antibodies (ASO Titers)

What does the immune system cross react with?o M Proteins

What are the two major criteria to diagnose Rheumatic Fever?o Migratory, Asymmetric Polyarthritiso Pancarditis

What are the nodules seen on microscopic slides of patients with Rheumatic Fever? How are these characterized?

o Aschoff Bodies Granulomatous Multinucleated Giant Cells

What are the cells called that are inside of the Ascoff Bodies?o Anitschkow (Catapillar) Cells

What are the 3 Morphological Patterns associated with Rheumatic Fever?o Myocarditis

Aschoff Bodies Early Mortality

o Pericarditis Fibrinous “Bread and Butter” Exudate = Friction Rub

o Endocarditis Involves Valvular Endocardium

Which valve is affected by Rheumatic Fever the most?o Mitral Valve

How do you differentiate between Acute and Chronic Rheumatic Fever?o Acute: Children, Small Vegetation at the Line of Closureo Chronic: 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?o Large, Beefy Red Vegetative Masses.o Friable Masses Embolizationo Involves the Free Margin of the Valve

What are the two types?o Acuteo Subacute

How do you differentiate between Acute and Subacute?o Acute: Previously normal valve, Staph. Aureus, More Destructiveo Subacute: Previously damaged valve, Staph. Viridans, Less Destructive

What bug and what valve is involved with IV Drug Use?o Staph. Aureuso Tricuspid Valve (first valve the drug comes to after the injection)

Which type (Acute or Subacute) has the high-grade fever?o Acute

What do you auscultate in these patients?o A changing character of murmurs as the vegetation grows.

What is characteristic of the Chronic Infective Endocarditis?o The Small Septic Thrombi can break off and block blood supply

elsewhere.o Petechial Hemorrhages (Retina Roth’s Spots)o Osler Nodes (Fingers and Toes)o Janeway Lesions (Painless, Palms/Soles)

What complications can the Chronic Infective Endocarditis lead to?o Strokeo Glomerulonephritiso Infarctiono Heart Failureo Anemia

Non-Bacterial Thrombic Endocarditis

What is another name for Non-Bacterial Thrombotic Endocarditis?o Marantic Endocarditis – found in debilitated patients

What does the valve look like if the patient has Non-Bacterial Thrombic Endocarditis?

o Bland Vegetation Along the Line of Closureo You can tell this from Rheumatic Fever because Rheumatic Fever is in

children, while this is in the elderly. What can this be mistaken for?

o Mucinous Adenocarcinomas Hypercoagulable State

Carcinoid Heart Disease

What does a heart with Carcinoid Heart Disease look like?o Subendocardial Fibrosis of the RIGHT HEART

The lungs have MAO, which degrades the tumor, so the Left Heart doesn’t have manifestations

What would be in the serum?

o Elevated Serotonin (5HT) and Metabolite (5-HIAA) because it is secreted by the tumor.

o Causes flushed face.

Dilated Cardiomyopathy

What are some types of Cardiomyopathy?o Idiopathico Post-Viral (Coxsackie)o Alcoholo Pregnancyo Cardiotoxicityo Hemochromatosis

What characteristic must the heart have to be diagnosed as a Dilated Cardiomyopathy?

o Progressive cardiac dilation of all 4 chambers.o Low Ejection Fraction <40%

Hypertrophic Cardiomyopathy

What is the typical patient that presents with Hypertrophic Cardiomyopathy?o Young, Male, Athleteo Some sort of Sport/Exercise

What is the inheritance pattern?o Autosomal Dominant

What is the manifestations of the disease?o Myofiber Disarrayo Asymmetrical Septal Thickening o Abnormal Diastolic Fillingo Myocardial Hypertrophyo Banana-Shaped Left Ventricular Chambero Outflow Obstruction

Restrictive Cardiomyopathy

What are some diseases that cause Restrictive Cardiomyopathy?o Amyloidosiso Sarcoidosiso Glycogen Storage Disease (Pompe)

What happens to the compliance in patients with this disease?o Decrease in Ventricular Compliance Impaired Ventricular Filling

during Diastole If the Restrictive Cardiomyopathy is caused by Amyloidosis, what would you

see on microscopy?

o Deposits of Amyloid between the muscle fibers that are stained with Congo Red (Polarized Light)

Myocarditis

What is the most common cause of Myocarditis?o Coxsackie A and B (B is more common)

What are the Bacterial causes of Mayocarditis?o Corynebacterium Diphtheria

Exotoxin Fatty Changeo Borrelia Burgdorferi

Lyme Disease What would you see on a microscopic slide of Viral Myocarditis?

o Lymphocytic Infiltrate

Pericarditis:

What are the 4 diseases that cause Fibrous Pericarditis?o RUMSo R: Rheumatic Fevero U: Uremiao M: Myocardial Infarctiono S: Systemic Lupus Erythematosis

Pericarditis can be Acute Suppurative or Acute Sanguinous. What causes the Acute Sanguinous Type?

o Tuberculosiso Tumor Infiltration

There are two types of Chronic Pericarditis – what are they?o Adhesive Mediastinopericarditiso Constrictive Pericarditis

Tumors:

What are the three most common Benign Tumors found in the heart?o Myxoma (Most Common)o Fibromao Rhabdomyoma

Where in the heart is a Myxoma usually found?o Left Atrium

What are the two types of Myxoma?

o Sessile Typeo Pedunculated Type

Describe the Sessile Typeo Broad-Based (Large Mass)

Describe the Pedunculated Typeo Polypo Can have Ball Valve/Wrecking Ball Effect on the Mitral Valve, causing

damage. What is a Rhabdomyoma?

o Most frequent primary tumor of the heart in infants and children.o Tumor of the Striated Muscle – Spider Cells seen Microscopically

Tutoring Questions:

Describe the Eisenmenger Complex:o Found in Left Right Shuntso LR Shunt Increased Pulmonary Artery Blood Flow

Pulmonary Hypertension Right Ventricular Hypertrophy Reversal of shunt with Cyanosis

How do you treat PDA?o Indomethacin: Promotes the closure

What is the most common cause of PDA?o Prematurity (Neonatal RDS) Hypoxemia

How can you determine the prognosis of Tetralogy of Fallot?o The degree of Pulmonary Stenosis

What disease causes Pulmonary HYPOtension?o Tetralogy of Fallot

What gives you a Boot-Shaped Appearance on X-Ray?o Tetralogy of Fallot

What is required to survive Transposition of Great Vessels?o A PDAo Keep it open with Prostaglandins

Which disease causes an egg-shaped heart?o Transposition of Great Vessels

Which disease causes a Hypoplastic Right Ventricle?o Tricuspid Atresia

Pre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Cyanosis of the Lower Extremities?

o Pre-Ductal Pre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes

hypertension in the Upper Extremities?o Post-Ductal

Pre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Notching of the Ribs?

o Post-Ductal Pre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes

Right Ventricular Hypertrophy?o Pre-Ductal

Pre-Ductal or Post-Ductal Coarctation of the Aorta: Which causes Diminished or absent femoral pulses?

o Post-Ductal What is a complication of MI after the white scar forms (>3 weeks)?

o Left Ventricular Aneurysm Mural Thrombus Left Sided Embolism

If the MI is Subendocardial and less than 1 week old, what complication could the patient have?

o Rupture of the Papillary Muscle Change/New Murmur Valvular Insufficiency

If the MI is Transmural and less than 1 week old, what complication could the patient have?

o Myocardial Rupture Cardiac Tamponade Muffled Heart Sounds JVD

What happens during Reperfusion Injury?o 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-MB

What disorder does Myxoid Degeneration cause?o Mitral Valve Prolapse

What are the three components of an Aschoff Body? What disease is this associated with?

o Granulomatouso Anitschkow Cellso Multinucleated Giant Cells

What are the two major jones criteria of Rheumatic Fever?o Migratory, Asymmetrical Polyathritiso Pancarditis

Which heart disease causes Osler Nodes and Janeway Lesions?o Infective Endocarditis

What heart disease do IV Drug Users get?o Infective Endocarditis

Staph Aureus Tricuspid Valve What heart disease can be caused by Hemochromatosis?

o Dilated Cardiomyopathyo DIE HAPPI

What heart disease can be caused by Pregnancy?

o Dilated Cardiomyopathyo DIE HAPPI

What heart disease can be caused by Alcohol?o Dilated Cardiomyopathyo DIE HAPPI

What are 2 complications of Dilated Cardiomyopathy?o Mural Thrombuso CHF

If one patient comes in with dysphagia, and another comes in with MV Insufficiency Murmur), what disease do they BOTH have?

o Myxoma If a cancer patient, being treated with radiation, comes into the office

with a heart disease, which heart disease do they have?o Chronic Pericarditis