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118 Cards in this Set

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What are two vascular pathologies in hypertension?
Hyaline arteriolosclerosis

Hyperplastic arteriolosclerosis
What are some morphological features of hyaline arteriolosclerosis?
Thickening of the arteriole wall
Narrowing of the lumen
Thickness from hyalinization - pink homogenous hyaline

Lesion is due to leakage of plasma components across vascular endothelium, excessive ECM production by SM cells secondary to the chronic hemodynamic stress of hypertension
Who gets Hyaline arterolosclerosis?
Elderly
Hypertension
Microangiopathy in diabetes
What are some morphological features of hyperplastic arteriolosclerosis?
Similar morphology as hyaline (fibrous tissue, thickening)

Hyperplasia of smooth muscle cells of tunica media - many nuclei visible in the arterial wall

"onion-skin", concentric, laminated thickening of the walls with luminal narrowing. Fibrinoid deposits.
Who gets Hyperplastic arteriolosclerosis?
It occurs in malignant hypertension - acute/severe elevation of blood pressure
Describe vasculitis
Inflammation and necrosis of blood vessels, which may affect arteries, veins, and capillaries - especially the kidneys and organs with small vessels (eyes as well).

Signs may include blindness, proteinuria, capillary hemorrhage.
What vessels are primarily affected in arteriolosclerosis?
Arterioles
What are the clinical manifestations of vasculitis?
Myalgia
Fever
Arthralgia
Malaise
What are the two most common mechanisms behind vasculitis?
Immune-mediated inflammation

Direct invasion of vascular walls by infectious pathogens
What is role of immune complex deposition in noninfectious vasculitis?
Antibody and complement are detected in lesions. The nature of the antigens is unknown, drug hypersensitivity may be a cause (eg penicillin) or from a viral infection (Ab against viral proteins attack self proteins that are similar - HBsAg)
How do antineutrophil cytoplasmic antibodies (ANCAs) effect noninfectious vasculitis?
Circulating antibodies react with neutrophil cytoplasmic antigens.

Cytoplasmic localization (c-ANCA) - Wegener granulomatosis
Perinuclear localization (p-ANCA_ microscopic polyanginitis and Churg-Strauss
What vascular problem may be caused by anti-endothelial cell antibodies?
Noninfectious vasculitis
What is polyarteritis nodosa?
Small and medium-vessel vasculitis - main visceral arteries and their branches.
What is the distribution of Polyarteritis nodosa?
Systemic vasculitis

Medium sized and smaller muscular arteries

Isolated forms: affect only one organ
Generalized forms: Multiple organs involved - Kidney, heart, skin, skeletal muscle...
What are the microscopic features of Polyarteritis nodosa?
Necrotizing inflammation - typically involves renal arteries and other viscera

Pulmonary vessels are spared

Typically occur at branch points

Inflammation is transmural

Fibrinoid necrosis
What is associated with Polyarteritis nodosa (diseases?)
Hepatitis B - clinical features depend on location (bowel, pancreas, kidney, brain), a chronic disease with periods of activity (as is polyarteritis nodosa)

Same causes as normal vasculitis
What is Kawasaki disease?
A mucocutaneous lymph node syndrome

Childhood vasculitis, most common in acquired heart disease in children
Uniquely affects internal elastic layer of blood vessels, but affects other layers as well
Coronary arteries
What are the signs/symptoms of Kawasaki disease?
Fever
Red eyes
Red-fissured lips
Strawberry tongue
Rash
Redness
Edema of palms and soles
Cervical lymphadenopathy
What is giant-cell arteritis?
The most common arteritis - temporal artery, aorta, ophthalmic artery - large and medium arteries. A focal, chronic granulomatous inflammation of the temporal artery (usually).

Not associated with ANCA

Associated with HLA-DR4
What vascular disease is associated with HLA-DR4?
Giant-cell arteritis
What is the morphology of the vessels in giant-cell arteritis?
Vessels become cord-like and thin
Have narrow lumen
Granulomatous inflammation
Segmental involvement of blood vessel
The disease is benign and self limited (headaches come and go)
What are some histological characteristics of giant-cell arteritis?
Giant cells
Focal destruction of internal elastic
Segmental involvement
Granulomas
How would one treat giant-cell arteritis?
Steroids - suppress inflammation/immune response
If there are signs of giant-cell arteritis, what procedure/tests are performed?
Biopsy
What is a major risk of giant-cell arteritis, especially involving the temporal artery?
Blindness
What is a typical presentation (with physical exam findings) for a patient with giant-cell arteritis?
An older patient (>50)
Headache, scalp tenderness, visual symptoms, jaw stiffness

Artery is nodular and tender
What is Takayasu arteritis?
An inflammatory disease affecting the aorta and its branches - there is a weak peripheral pulse.
Fibrosis in all layers
Granulomatous inflammation

Tends to affect early-middle aged women of Asian descent
What is the triad of presentation for Wegener granulomatosis?
Vasculitis affecting the RESPIRATORY tract

GRANULOMAS in the nose, sinuses, and lungs

RENAL disease - glomerulonephritis

*Associated with c-ANCA*
What is Churg-Strauss syndrome?

What is the triad? What differentiates it from Wegener's?
An allergic granulomatous vasculitis that occurs in young people with asthma.

Patients have c-ANCA or p-ANCA
Involves lung, kidney, and spleen

Triad: Allergic rhinitis, bronchial asthma, peripheral EOSINOPHILIA
What is Thromboangitis obliterans (Buerger disease)?
A peripheral vascular disease of smokers - inflammation of small and medium-sized arteries (tibial, radial) - segmental occlusion of lumen, distal arm and leg.
In what people does Thromboangitis obliterans/Buerger disease occur?
Smokers, typically smaller people
What is an arterial aneurysm?
Localized dilation of blood vessels - acquired or congenital abnormality of medial layer.
What are the classifications (4) for arterial aneurysms?
Sacular
Dissecting
Fusiform
Arteriovenous
What are some main locations of arterial aneurysms?
Abdominal (aortic)
Thoracic (aortic)
Cerebral
What are characteristics of a true aneurysm?
When the aneurysm involves all 3 layers of the arterial wall or the attenuated wall of the heart
What are characteristics of a false aneurysm?
Breach in the vascular wall
Extravascular hematoma
Freely communicates with the intravascular space ("pulsating hematoma")
What is the difference between a saccular and a fusiform aneurysm?
Saccular - a bulge on one side
Fusiform - equal bulging
What are the two most important causes of aortic aneurysms?
Atherosclerosis
Cystic medial degeneration of the arterial media

Other causes: congenital defects, infections, syphilis, systemic diseases (vasculitis)
What specific arteries may develop an atherosclerotic aneurysm?
Abdominal aorta
Common iliac arteries
Aortic arch
Descending parts of the thoracic aorta
What are some characteristics of abdominal aneurysms?
AAA occurs most often in males >50
Occurs as a complication of atherosclerosis
Distal to the renal arteries, and proximal to the bifurcation
Fusiform in shape
How does aortic dissection develop?
Blood enters the arterial wall (tear?)
It separates the layers of the wall
Dissects the path
Creates a false lumen
In what groups does aortic dissection most often occur?
Men aged 40-60 with antecedent HTN (more than 90% of cases of dissection)

Younger patients with systemic or localized abnormalities of connective tissue affecting the aorta (eg Marfan syndrome)
What is a berry aneurysm?
The most common type of cerebral aneurysm. A congenital defect at a branching point is common. Occurs in branching points in the circle of Willis.
Between what cerebral arteries do berry aneurysms often develop?
Often between...
...anterior cerebral artery and anterior communicating artery
...internal carotid and posterior communicating artery
What is a syphilitic aneurysm?
Inflammation of aorta's vasa vasorum
What are Mycotic aneurysms?
Occur in septicemia
Bacterial endocarditis
Spleen, mesentery, and renal artery are most commonly affected
What are varicose veins?
Abnormally dilated veins
Tortuous
Increased intraluminal pressure
Superficial veins
Venous stasis and edema
What is the luminal complication with varicose veins?
The valves are compromised - may close backwards
What is thrombophlebitis, phlebothrombosis?
Phlebothrombosis - characterized by clot formation
Thrombophlebitis - inflammation
Most often occur in deep veins of the legs - other locations include portal vein, periprostatic veins, large veins of the skull
What are some factors that cause predisposition for DVT?
CHF
Neoplasia
Pregnancy
Obesity
Postoperative state
Immobilization
What kind of cancers my cause thrombophlebitis? Why?
Adenocarcinomas most often
Cause a hypercoagulable state

Migratory thrombophlebitis - Trousseau sign - occurs with adenocarcinoma of pancreas
What are some features of malignant tumors of blood vessels?
Solid
Anaplasia
Do not form well organized blood vessels
Endothelial cells do not form well defined membrane
Marker is CD31 or von Willebrand factor
What is angiomatosis?
Widespread hematuria
Superficial
Rarely becomes malignant
Capillary hemangioma regresses by age 7
What is a cavernous hemangioma?
Involves large dilated blood vessels
Deep structures
Do not regress
Not encapsulated
Cavernous blood filled spaces
What is von Hippel-Lindau disease?
A cavernous hemangioma within the cerebellum or brain - cystic neoplasm in pancreas. Renal neoplasm.
What is the most common type of birth defect?
Congenital heart defects
What is the incidence of heart malformation in live births?
9-12 per 1,000 live births
What is the best proof that there is no god?
All the fucked up congenital malformations which either kill babies before they are birthed or doom them to a life of misery and rejection from society the moment they take their first breath.
List some congenital heart deformities (8)
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Coarctation of the aorta
Tetralogy of Fallot
D-transposition of the great arteries
Truncus arteriosus
Total anomalous pulmonary venous return
At what point in the life of the fetus do most congenital heart deformities develop?
The first 6-8 weeks of fetal life
What are some known genetic causes for congenital heart defects?
Trisomies: 21, 13, 18

Genetic point mutations, deletions, etc. as seen in...
Velo-Cardio-Facial Syndrome
Alagille Syndrome
Noonan Syndrome
What are some known environmental factors that cause or increase the risk for congenital heart defects?
Maternal infections (Rubella)
Drugs - alcohol, hydantoin, lithium, thalidomide
Maternal illness - DM, PKU, SLE
What is an atrial septal defect? How common is it?
Common (6-11%) and correctable
Pathophysiology is related to the magnitude and direction of shunting of blood across the interatrial communication (size, ventricular compliance)
What are the four variants of atrial septal defects?
Primum
Secundum (at the fossa ovalis)
Superior vena cava
Inferior vena cava
What clinical findings are associated with an atrial septal defect?
Clinical findings include a precordial lit, systolic pulmonary flow murmur and fixed splitting of the second heart sound (sometimes)

With large shunts, a diastolic rumble across the tricuspid valve is present
What are the ECG findings associated with ASD?
Right axis deviation
Right axis elevation
Incomplete right bundle branch block (secundum ASD)
Superior LAD (primum ASD)
Abnormal P-wave axis (superiorly located sinus venous ASD)
What are the CXR findings associated with ASD?
RV and RA enlargement
Prominent pulmonary artery segment
Increased pulmonary vascularity
How serious is ASD? What repercussions may follow if untreated?
Majority of ASD secundum defects close spontaneously in childhood

Most children with ASD remain active and asymptomatic

If untreated, CHF and pulmonary HTN develop in adulthood

May result in atrial fibrillation or atrial flutter
What is an Amplatzer implant?
It is webbed barrier that is inserted into the septum, and fits so that it is closed on each side of the defect, preventing shunting.

Delivered via transcatheter
What is a ventricular septal defect? How common is it?
This is the most common of all congenital heart lesions - often occur with other deformities as well. It is a failure of the ventricular septum to close.

Isolated defect in 20% of patients with CHD
Many VSDs close spontaneously within the first few years of life
What are the classifications for a ventricular septal defect?
Perimembranous
Inlset
Outlet (infundibular)
Central Muscular
Marginal Muscular
Apical Muscular
Describe perimembranous VSD - how common? Where is it located?
It is the most common form of VSD, 80% of surgical and autopsy series - usually extends into muscular, inlet, or outlet area.
Describe outlet VSD - how common? location?
5-7% of VSD - more common in the far east. Situated just below the pulmonary valve - it is also known as: supracristal, conal, infundibular, subpulmonary, doubly committed subarterial
Describe inlet VSD - how common? location?
5-8% - posterior and inferior to perimembranous defect
Describe muscular VSD - how common? location?
5-20%
Central: mid-muscular, may have multiple apparent channels on RV side and coalesce to single defect on the LV side
Apical: multiple apparent channels on RV side may be single defect on LV side with central defect
Marginal: along RV septal junction
"Swiss cheese" septum: large number of muscular defects
How does VSD present in a clinical examination?
It is characterized by a systolic murmur, usually maximal at the lower left sternal border
When RV pressure is low, the VSD murmur is blowing and pansystolic
Small, muscular VSDs are usually very high-pitched and occupy early systole only because muscular contraction closes the defect
How does VSD present on a CXR?
Patients with a small VSD will have a normal CXR
The presence of a left-to-right shunt will create the appearance of LA and LV enlargement and increased pulmonary vascular markings
What are some characteristics of VSD on an ECG?
Lead II: Peaked P waves
Lead V3: Biphasic P waves
Biventricular hypertrophy
Describe PDA - what is the normal progression for the DA? What are the risks? Are there predisposing factors?
It is vital in fetal life to allow for circulation without lung involvement.

Usually closes after birth

More common in premature babies, those with in utero rubella exposure

Pulmonary vascular disease if not noted
Infective Endocarditis risk

If repaired, cured
What are some ECG findings associated with PDA?
V1: biphasic P wave
Ventricular hypertrophy
What can be done to close a PDA?
Insert a coil via a catheter - the coil fits into the PDA
What is coarctation of the aorta? When is it diagnosed? Is it an emergency?
The aorta is constricted before/near the ductus arteriosus

Diagnosis is made during infancy or childhood 80% of the time

It is common to survive into adulthood
Severe coarctation is dependent on ductus arteriosus remaining patent, the only source of significant oxygenated blood. If the DA closes, results in shock

Drugs are used to maintain PDA while surgical options are chosen
What is a classic presentation for ductal-dependent coarctation of the aorta?
Cardiovascular collapse
Decreased systemic pulses and perfusion
Hypotension
Metabolic acidosis
What signs are useful in diagnosing ductal-dependent coarctation of the aorta?
Reduced lower-extremity pulses compared to normal upper-extremity pulses
Lower lower-extremity BP, at least 20 mmHg lower than the BP in the upper-extremities

Hypertension in right arm (relative), unless there is anomalous origin of the right subclavian artery
Carotid pulsations may be hyperdynamic
Pulse delay between the right arm and the femoral or popliteal arteries

*Left subclavian artery may be close to the aortic narrowing and thus not be hypertensive
What is seen in the ECG and CXR for coarctation of the aorta?
ECG - LVH, RVH, or both

CXR - cardiomegaly, pulmonary edema - prominent curvilinear shadow along the mid-right sternal border that represents a dilated ascending aorta.
An indentation at the coarctation site may produce a "3 sign" adjacent to the area beneath the transverse arch and above the main pulmonary artery silhouette
Notching on the underside of the ribs (usually 3-9) from collateral vessels may be apparent
What is seen in the echocardiogram in a patient with coarctation of the aorta?
Echo establishes the diagnosis with demonstration of a critical narrowing of the isthmus, sometimes extending into the transverse arch.
In coarctation of the aorta, what heart sounds may be present?
Murmur or a bruit may be heard in the left interscapular position, either due to the coarctation or the collaterals?

If collaterals are present, continuous murmurs may be heard over the parasternal areas and around the left scapula
What role do collateral vessels have in relation to coarctation of the aorta?
Collateral vessels tend to develop in late-presenting coarctation - they aid in blood supply, but may mask the severity of the obstruction
How is critical coarctation approached?
Initial management strategies focus on maintaining ductal patency, supporting systemic perfusion
PGE1 initiated
Surgical correction - resection of the discrete region of narrowing and primary repair with end-to-end anastamosis
What changes have occurred in a heart with Tetralogy of Fallot compared to a normal heart?
There is partial obstruction of the right ventricular outflow and pulmonary valve

Thickened right ventricle (hypertrophy)

Usually ventricular septal defect

Increased outflow in aorta
If an infant or child displays cyanosis, which congenital heart defect is likely the cause?
Tetralogy of Fallot
What is simple Tetralogy of Fallot?
Malalignment VSD, aortic to mitral fibrous continuity, adequate confluent PA's, various degrees of RVOTO, anterograde RV flow with normal AV connections
What may be noted during auscultation of a patient with Tetralogy of Fallot?
Normal to increased precordial impulse and a single S2
A systolic murmur of outflow obstruction at the base of the heart when the pulmonary outflow is patent
What are some ECG signs of Tetralogy of Fallot?
In unrepaired Tetralogy of Fallot, one might see Right Axis Deviation and Right Ventricular Hypertrophy
What are some CXR signs of Tetralogy of Fallot?
Boot-shaped heart silhouette
Decreased pulmonary vascularity
What is transposition of the great arteries?
A congenital heart lesion with an abnormal origin of the aorta and pulmonary artery from the ventricular complex. About 5% of all congenital heart defects

Men : Women = 3 : 1
What other congenital heart defects are associated with transposition of the great arteries?
VSD
LVOT obstruction (PS)
Describe simple d-TGA - what is the typical presentation?
It is the most common form of cyanotic heart disease and one of the most common forms of symptomatic heart disease in the newborn

The newborn who presents early with profound cyanosis but without respiratory distress
What are the physical findings in a patient with d-TGA?
Cyanosis in all four extremities
Tachypneic, but not in respiratory distress
Pulses and perfusion are normal
Precordium is active but not increased compared to that of a normal newborn
Normal S1, S2 single and loud
Murmurs are rarely present
What are the signs for d-TGA seen in an ECG, CXR, and echocardiogram?
ECG - normal at birth, but right ventricular hypertrophy after a few days
CXR - narrow mediastinal shadow - because of the parallel relationship of the arteries
What is a Jatene Arterial switch?
Sever the two arteries above the valves and switch them - sew them back in the right places.

Also, close the other patent areas of the heart while you're at it, since those aren't necessary anymore.
What is Truncus Arteriosus Communis?
The complex is characterized by a single semilunar valve and annulus with a single great vessel arising from the ventricles - accounts for 2% of congenital cardiac anomalies.
Important cause of death in infancy
What is Truncus Arteriosus?
Pulmonary arteries arise from the aorta

Truncal valve, occasionally quadracuspid, stenotic, or insufficient - overrides the ventricular septal defect

Ventricular septal defect, large
What are some signs of Truncus Arteriosus?
Cyanosis is usually mild
Signs of CHF appear early
Pulmonary blood flow can be torrential, particularly as PVR progressively falls
Tachypnea present with HF, pulse oximetry may demonstrate desaturation
Precordium is hyperdynamic due to volume overload, pulses are bounding due to diastolic runoff into the pulmonary arteries
S2 is single and an ejection click is common - systolic murmur heard at the left sternal border, diastolic murmur if truncal valve regurgitation
How would Truncus Arteriosus manifest in an ECG, CXR, and Echo?
ECG - usually biventricular hypertrophy although may be normal in the first few days - prominent R waves in V1 (RVH) and inferior, lateral leads (LVH).
CXR - cardiomegaly, increased PVM
Echo - can establish the diagnosis
How would a cardiac catheter help diagnose Truncus Arteriosus?
It will help clarify the anatomy of the branch pulmonary arteries, coronary arteries, ventricular septum, or aortic arch
What is Total Anomalous Pulmonary Venous Return?
TAPVR - has abnormal return of the pulmonary venous flow to the right atrium or some tributary
Right-to-left transatrial shunt
Obstruction may occur - infradiaphragmatic

Repair involves connecting the pulmonary venous confluence to the left atrium and closing the intra-atrial connection
How do infants with TAPVR present?
TAPVR with obstruction present within the first few hours to days, and the greater the obstruction, the earlier the manifestations

Typically demonstrate cyanosis and evidence of respiratory distress due to pulmonary venous congestion

PE - ill appearing, cyanotic, tachypneic, minimal cardiovascular findings, possibly accentuated pulmonary component of S2
How do ECG, CXR, or Echo indicate TAPVR?
ECG - unremarkable
CXR - very characteristic features of normal cardiac silhouette and progressive pulmonary venous congestion
Echo - confirms diagnosis
What are the three varieties of TAPVR?
Supracardiac
Intracardiac
Infradiaphragmatic
What is tricuspid atresia?
The complete absence of an opening between the right atrium and and right ventricle, resulting in lack of development of the right ventricle. A cyanotic abnormality. Aorta and pulmonary artery may be normally related, or may be transposed.

In absence of ASD, fetus dies
In tricuspid atresia, these defects may be present (~5)
ASD - right to left shunting
RV - hypoplastic
RA - enlarged, hypertrophic
LV - enlarged, decreased ventricular function from volume overload
VSD - if this is sufficient and there are no associated pulmonary obstructions, there will be minimal cyanosis and increased pulmonary circulation
How would tricuspid atresia present clinically?
Infant is cyanotic and O2 saturation is similar in all extremities
Tachypnea, no respiratory distress
Peripheral pulses and perfusion are normal
Right ventricular impulse is decreased, a thrill from a ventricular VSD in a cyanotic newborn strongly suggests tricuspid atresia. Normal S1, S2 is variable depending on associated defects
What are the ECG, CXR, Echo findings for tricuspid atresia?
ECG - RAE and decreased right ventricular forces
CXR - findings depend on associated defects
Echo - shows no tricuspid valve tissue in the AV groove, obligatory flow across the atrial septum into the left atrium
What steps are taken to correct tricuspid atresia.
Infants have a single functional ventricle since the other will never be sufficiently large

Systemic to pulmonary artery shunt usually required soon after birth.
Bidirectional Glenn shunt and a modified Fontan operation
What is a Blalock-Taussig Shunt?
Connects the brachiocephalic trunk/right subclavian artery with the right branch of the pulmonary trunk
What is a Bidirectional Glenn Shunt?
Separate the superior vena cava from the RA, and the pulmonary trunk from its left and right branches.

The superior portion of the SVC is then connected to the right branch of the pulmonary trunk
What is the Fontan procedure?
This is used when the child has a single effective ventricle.

It diverts the venous blood from the right atrium to the pulmonary arteries without passing through the morphologic right ventricle.