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118 Cards in this Set
- Front
- Back
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What are two vascular pathologies in hypertension?
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Hyaline arteriolosclerosis
Hyperplastic arteriolosclerosis |
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What are some morphological features of hyaline arteriolosclerosis?
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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 |
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Who gets Hyaline arterolosclerosis?
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Elderly
Hypertension Microangiopathy in diabetes |
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What are some morphological features of hyperplastic arteriolosclerosis?
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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. |
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Who gets Hyperplastic arteriolosclerosis?
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It occurs in malignant hypertension - acute/severe elevation of blood pressure
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Describe vasculitis
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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. |
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What vessels are primarily affected in arteriolosclerosis?
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Arterioles
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What are the clinical manifestations of vasculitis?
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Myalgia
Fever Arthralgia Malaise |
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What are the two most common mechanisms behind vasculitis?
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Immune-mediated inflammation
Direct invasion of vascular walls by infectious pathogens |
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What is role of immune complex deposition in noninfectious vasculitis?
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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)
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How do antineutrophil cytoplasmic antibodies (ANCAs) effect noninfectious vasculitis?
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Circulating antibodies react with neutrophil cytoplasmic antigens.
Cytoplasmic localization (c-ANCA) - Wegener granulomatosis Perinuclear localization (p-ANCA_ microscopic polyanginitis and Churg-Strauss |
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What vascular problem may be caused by anti-endothelial cell antibodies?
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Noninfectious vasculitis
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What is polyarteritis nodosa?
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Small and medium-vessel vasculitis - main visceral arteries and their branches.
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What is the distribution of Polyarteritis nodosa?
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Systemic vasculitis
Medium sized and smaller muscular arteries Isolated forms: affect only one organ Generalized forms: Multiple organs involved - Kidney, heart, skin, skeletal muscle... |
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What are the microscopic features of Polyarteritis nodosa?
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Necrotizing inflammation - typically involves renal arteries and other viscera
Pulmonary vessels are spared Typically occur at branch points Inflammation is transmural Fibrinoid necrosis |
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What is associated with Polyarteritis nodosa (diseases?)
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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 |
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What is Kawasaki disease?
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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 |
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What are the signs/symptoms of Kawasaki disease?
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Fever
Red eyes Red-fissured lips Strawberry tongue Rash Redness Edema of palms and soles Cervical lymphadenopathy |
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What is giant-cell arteritis?
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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 |
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What vascular disease is associated with HLA-DR4?
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Giant-cell arteritis
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What is the morphology of the vessels in giant-cell arteritis?
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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) |
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What are some histological characteristics of giant-cell arteritis?
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Giant cells
Focal destruction of internal elastic Segmental involvement Granulomas |
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How would one treat giant-cell arteritis?
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Steroids - suppress inflammation/immune response
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If there are signs of giant-cell arteritis, what procedure/tests are performed?
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Biopsy
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What is a major risk of giant-cell arteritis, especially involving the temporal artery?
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Blindness
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What is a typical presentation (with physical exam findings) for a patient with giant-cell arteritis?
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An older patient (>50)
Headache, scalp tenderness, visual symptoms, jaw stiffness Artery is nodular and tender |
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What is Takayasu arteritis?
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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 |
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What is the triad of presentation for Wegener granulomatosis?
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Vasculitis affecting the RESPIRATORY tract
GRANULOMAS in the nose, sinuses, and lungs RENAL disease - glomerulonephritis *Associated with c-ANCA* |
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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 |
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What is Thromboangitis obliterans (Buerger disease)?
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A peripheral vascular disease of smokers - inflammation of small and medium-sized arteries (tibial, radial) - segmental occlusion of lumen, distal arm and leg.
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In what people does Thromboangitis obliterans/Buerger disease occur?
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Smokers, typically smaller people
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What is an arterial aneurysm?
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Localized dilation of blood vessels - acquired or congenital abnormality of medial layer.
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What are the classifications (4) for arterial aneurysms?
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Sacular
Dissecting Fusiform Arteriovenous |
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What are some main locations of arterial aneurysms?
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Abdominal (aortic)
Thoracic (aortic) Cerebral |
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What are characteristics of a true aneurysm?
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When the aneurysm involves all 3 layers of the arterial wall or the attenuated wall of the heart
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What are characteristics of a false aneurysm?
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Breach in the vascular wall
Extravascular hematoma Freely communicates with the intravascular space ("pulsating hematoma") |
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What is the difference between a saccular and a fusiform aneurysm?
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Saccular - a bulge on one side
Fusiform - equal bulging |
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What are the two most important causes of aortic aneurysms?
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Atherosclerosis
Cystic medial degeneration of the arterial media Other causes: congenital defects, infections, syphilis, systemic diseases (vasculitis) |
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What specific arteries may develop an atherosclerotic aneurysm?
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Abdominal aorta
Common iliac arteries Aortic arch Descending parts of the thoracic aorta |
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What are some characteristics of abdominal aneurysms?
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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 |
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How does aortic dissection develop?
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Blood enters the arterial wall (tear?)
It separates the layers of the wall Dissects the path Creates a false lumen |
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In what groups does aortic dissection most often occur?
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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) |
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What is a berry aneurysm?
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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.
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Between what cerebral arteries do berry aneurysms often develop?
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Often between...
...anterior cerebral artery and anterior communicating artery ...internal carotid and posterior communicating artery |
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What is a syphilitic aneurysm?
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Inflammation of aorta's vasa vasorum
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What are Mycotic aneurysms?
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Occur in septicemia
Bacterial endocarditis Spleen, mesentery, and renal artery are most commonly affected |
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What are varicose veins?
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Abnormally dilated veins
Tortuous Increased intraluminal pressure Superficial veins Venous stasis and edema |
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What is the luminal complication with varicose veins?
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The valves are compromised - may close backwards
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What is thrombophlebitis, phlebothrombosis?
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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 |
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What are some factors that cause predisposition for DVT?
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CHF
Neoplasia Pregnancy Obesity Postoperative state Immobilization |
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What kind of cancers my cause thrombophlebitis? Why?
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Adenocarcinomas most often
Cause a hypercoagulable state Migratory thrombophlebitis - Trousseau sign - occurs with adenocarcinoma of pancreas |
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What are some features of malignant tumors of blood vessels?
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Solid
Anaplasia Do not form well organized blood vessels Endothelial cells do not form well defined membrane Marker is CD31 or von Willebrand factor |
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What is angiomatosis?
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Widespread hematuria
Superficial Rarely becomes malignant Capillary hemangioma regresses by age 7 |
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What is a cavernous hemangioma?
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Involves large dilated blood vessels
Deep structures Do not regress Not encapsulated Cavernous blood filled spaces |
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What is von Hippel-Lindau disease?
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A cavernous hemangioma within the cerebellum or brain - cystic neoplasm in pancreas. Renal neoplasm.
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What is the most common type of birth defect?
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Congenital heart defects
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What is the incidence of heart malformation in live births?
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9-12 per 1,000 live births
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What is the best proof that there is no god?
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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.
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List some congenital heart deformities (8)
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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 |
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At what point in the life of the fetus do most congenital heart deformities develop?
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The first 6-8 weeks of fetal life
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What are some known genetic causes for congenital heart defects?
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Trisomies: 21, 13, 18
Genetic point mutations, deletions, etc. as seen in... Velo-Cardio-Facial Syndrome Alagille Syndrome Noonan Syndrome |
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What are some known environmental factors that cause or increase the risk for congenital heart defects?
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Maternal infections (Rubella)
Drugs - alcohol, hydantoin, lithium, thalidomide Maternal illness - DM, PKU, SLE |
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What is an atrial septal defect? How common is it?
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Common (6-11%) and correctable
Pathophysiology is related to the magnitude and direction of shunting of blood across the interatrial communication (size, ventricular compliance) |
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What are the four variants of atrial septal defects?
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Primum
Secundum (at the fossa ovalis) Superior vena cava Inferior vena cava |
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What clinical findings are associated with an atrial septal defect?
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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 |
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What are the ECG findings associated with ASD?
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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) |
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What are the CXR findings associated with ASD?
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RV and RA enlargement
Prominent pulmonary artery segment Increased pulmonary vascularity |
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How serious is ASD? What repercussions may follow if untreated?
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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 |
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What is an Amplatzer implant?
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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 |
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What is a ventricular septal defect? How common is it?
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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 |
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What are the classifications for a ventricular septal defect?
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Perimembranous
Inlset Outlet (infundibular) Central Muscular Marginal Muscular Apical Muscular |
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Describe perimembranous VSD - how common? Where is it located?
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It is the most common form of VSD, 80% of surgical and autopsy series - usually extends into muscular, inlet, or outlet area.
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Describe outlet VSD - how common? location?
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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
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Describe inlet VSD - how common? location?
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5-8% - posterior and inferior to perimembranous defect
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Describe muscular VSD - how common? location?
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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 |
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How does VSD present in a clinical examination?
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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 |
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How does VSD present on a CXR?
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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 |
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What are some characteristics of VSD on an ECG?
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Lead II: Peaked P waves
Lead V3: Biphasic P waves Biventricular hypertrophy |
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Describe PDA - what is the normal progression for the DA? What are the risks? Are there predisposing factors?
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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 |
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What are some ECG findings associated with PDA?
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V1: biphasic P wave
Ventricular hypertrophy |
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What can be done to close a PDA?
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Insert a coil via a catheter - the coil fits into the PDA
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What is coarctation of the aorta? When is it diagnosed? Is it an emergency?
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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 |
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What is a classic presentation for ductal-dependent coarctation of the aorta?
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Cardiovascular collapse
Decreased systemic pulses and perfusion Hypotension Metabolic acidosis |
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What signs are useful in diagnosing ductal-dependent coarctation of the aorta?
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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 |
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What is seen in the ECG and CXR for coarctation of the aorta?
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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 |
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What is seen in the echocardiogram in a patient with coarctation of the aorta?
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Echo establishes the diagnosis with demonstration of a critical narrowing of the isthmus, sometimes extending into the transverse arch.
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In coarctation of the aorta, what heart sounds may be present?
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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 |
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What role do collateral vessels have in relation to coarctation of the aorta?
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Collateral vessels tend to develop in late-presenting coarctation - they aid in blood supply, but may mask the severity of the obstruction
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How is critical coarctation approached?
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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 |
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What changes have occurred in a heart with Tetralogy of Fallot compared to a normal heart?
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There is partial obstruction of the right ventricular outflow and pulmonary valve
Thickened right ventricle (hypertrophy) Usually ventricular septal defect Increased outflow in aorta |
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If an infant or child displays cyanosis, which congenital heart defect is likely the cause?
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Tetralogy of Fallot
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What is simple Tetralogy of Fallot?
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Malalignment VSD, aortic to mitral fibrous continuity, adequate confluent PA's, various degrees of RVOTO, anterograde RV flow with normal AV connections
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What may be noted during auscultation of a patient with Tetralogy of Fallot?
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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 |
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What are some ECG signs of Tetralogy of Fallot?
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In unrepaired Tetralogy of Fallot, one might see Right Axis Deviation and Right Ventricular Hypertrophy
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What are some CXR signs of Tetralogy of Fallot?
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Boot-shaped heart silhouette
Decreased pulmonary vascularity |
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What is transposition of the great arteries?
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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 |
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What other congenital heart defects are associated with transposition of the great arteries?
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VSD
LVOT obstruction (PS) |
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Describe simple d-TGA - what is the typical presentation?
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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 |
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What are the physical findings in a patient with d-TGA?
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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 |
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What are the signs for d-TGA seen in an ECG, CXR, and echocardiogram?
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ECG - normal at birth, but right ventricular hypertrophy after a few days
CXR - narrow mediastinal shadow - because of the parallel relationship of the arteries |
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What is a Jatene Arterial switch?
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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. |
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What is Truncus Arteriosus Communis?
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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 |
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What is Truncus Arteriosus?
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Pulmonary arteries arise from the aorta
Truncal valve, occasionally quadracuspid, stenotic, or insufficient - overrides the ventricular septal defect Ventricular septal defect, large |
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What are some signs of Truncus Arteriosus?
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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 |
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How would Truncus Arteriosus manifest in an ECG, CXR, and Echo?
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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 |
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How would a cardiac catheter help diagnose Truncus Arteriosus?
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It will help clarify the anatomy of the branch pulmonary arteries, coronary arteries, ventricular septum, or aortic arch
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What is Total Anomalous Pulmonary Venous Return?
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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 |
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How do infants with TAPVR present?
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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 |
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How do ECG, CXR, or Echo indicate TAPVR?
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ECG - unremarkable
CXR - very characteristic features of normal cardiac silhouette and progressive pulmonary venous congestion Echo - confirms diagnosis |
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What are the three varieties of TAPVR?
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Supracardiac
Intracardiac Infradiaphragmatic |
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What is tricuspid atresia?
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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 |
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In tricuspid atresia, these defects may be present (~5)
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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 |
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How would tricuspid atresia present clinically?
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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 |
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What are the ECG, CXR, Echo findings for tricuspid atresia?
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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 |
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What steps are taken to correct tricuspid atresia.
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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 |
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What is a Blalock-Taussig Shunt?
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Connects the brachiocephalic trunk/right subclavian artery with the right branch of the pulmonary trunk
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What is a Bidirectional Glenn Shunt?
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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 |
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What is the Fontan procedure?
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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. |
