Valvular and Congenital Heart Disease - Nichols and Newman Flashcards
1
Q
What are the two forms of cardiac adaptation to valvular disease? How is this different in acute scenarios?
A
-
Concentric hypertrophy: pressure overload = STENOSIS (+ HTN)
1. INC thickness (thich walls), but normal volume -> LV hypertrophy -
Eccentric hypertrophy: volume overload = REGURGITATION
1. INC dilation of chamber (more volume + thin walls) -> INC output (Starling) -
Acute: no time to adapt, so pt presents severely ill
1. Murmur heard, but no concentric or eccentric hypertrophy - INC pressure change with DEC structural changes in acute OR DEC pressure change with INC structural changes in chronic
2
Q
Mitral valve stenosis
A
- Rheumatic fever
- INC pressure behind mitral (stenosed) valve: concentric hypertrophy + dilation of LA (THROMBI), pulmonary HTN (atherosclerosis), concentric hypertrophy of RV
- Presentation: shortness of breath, alveolar fluid w/fibrinous material, hemoptysis (if chronic), right heart failure, afib (stroke), dysphagia (atrium compresses back on esophagus)
- Murmur: diastolic
3
Q
Mitral valve regurgitation
A
-
Mitral valve prolapse, LV dilation (left sided heart failure dilates mitral valve ring), rheumatic fever
1. Prolapse: leaflets replaced by myxomatous (spongy) tissue; eccentric hypertrophy (dilation) of LA - Presentation: can be asymptomatic with murmur, sudden rise in atrial pressure, INC preload (from atrium) + DEC afterload (from venting) = INC stroke volume
- Acute = no change in RA volume, INC pulmonary capillary wedge pressure (lg pressure buildup)
- Murmur: pansystolic
4
Q
What are the epi, patho and sign of mitral valve prolapse?
A
- Epi: most common valvular disease (about 2% of US population); primary or secondary
- Pathogenesis: primary form a genetic defect in connective tissue; secondary form injury
- Gross: redundant, billowing, ballooning, hooding, floppy leaflets, +/- elongated chordae tendineae (that can rupture)
- Microscopic: sometimes degeneration of and attenuation of outer zona fibrosa, and expanded myxomatous inner zona spongiosa (often normal)
- Sign: mid-systolic click
5
Q
What are 1, 2, and 3?
A
- Normal mitral valve
1. Zona atrialis (sometimes also called zona fibrosa)
2. Zona spongiosa
3. Zona fibrosa
6
Q
What do you see?
A
Expanded, myxomatous zona spongiosa (e.g., in mitral valve prolapse)
7
Q
What are the epi and patho of Libman-Sacks endocarditis?
A
- Epi: lupus
- Gross: small-medium verrucous, berry-like or flat vegetations, commonly on multiple valves (on either or both sides)
- Microscopic: necrotic debris, fibrinoid material, degenerating leukocytes, fibroblasts, and hematoxylin bodies
- Clinically important fact: rarely embolizes
8
Q
How is endocarditis related to valve pressure?
A
- Incidence of infection, marantic endocarditis, and rheumatic disease on a valve correlate with resting pressure on the closed valve:
1. Mitral: 116 mm Hg
2. Aortic: 72 mm Hg
3. Tricuspid: 24 mm Hg
4. Pulmonic: 5 mm Hg
9
Q
Why is it really important to “catch” infective endocarditis?
A
100% fatal if undiagnosed/untreated, but only 20% fatal if diagnosed/treated appropriately (IV AB’s, sx)
10
Q
What is the pathogenesis of infective endocarditis (4)?
A
- Most commonly of the valves, w/vegetations -> friable masses of infecting organisms and blood clot
1. Valvular endo injury
2. Platelet + fibrin deposition
3. Microbial seeding
4. Microbial multiplication - NOTE: valves may be “grossly normal,” but steps 1 and 2 precede 3 and 4
11
Q
What valves, etiologic agents, and portals of entry are generally involved in infective endocarditis?
A
- Valves: mitral > mitral + aortic = aortic > tricuspid (only 1% pulmonic)
- Etiologic agents: staph aureus (acute) and strep viridans (sub-acute; followed by enterococcus)
12
Q
What do you see? Why is this important?
A
- Central venous catheterization
- Portal of entry of bugs in infective endocarditis
13
Q
What do you see? Why is this important?
A
- Mouth with gingivitis, brushing teeth, chewing, dental procedures -> all portals of entry for bugs in infective endocarditis
14
Q
What is the gross pathology of infective endocarditis?
A
-
Vegetations: large (up to 3cm), friable, single or multiple, tan, grey, or red brown
1. Usually on line of valve closure (atrial side of AV valves, and ventricular side of semilunar valves) - The larger a vegetation, the more likely it is infective
- Destructive, and may cause: perforation of valve, adjacent abscess, fibrotic scarring, or calcification
- Infected (septic) emboli from endocarditis commonly go to kidney, heart, spleen, brain
15
Q
What are the microscopic pathology and common symptoms/signs of infective endocarditis?
A
-
Microscopic pathology:
1. Fibrin, platelets, masses of organisms, +/- necrosis, +/- neutrophils
2. Later: +/- lymphos, macros, fibroblasts, fibrosis - Symptoms: fever, chills, weakness, dyspnea
- Signs: fever, heart murmur, splenomegaly, petechiae
16
Q
What do you see?
A
- Osler nodes in fingers: tender to the touch
- Sign of infective endocarditis
17
Q
What do you see?
A
- Splinter hemorrhage under fingernail (aka, subungual splinter hemorrhages)
- Sign for infective endocarditis, but not specific -> much more commonly due to trauma
18
Q
What do you see?
A
- Janeway lesions: hemorrhages on palms or soles (5%)
- Sign of infective endocarditis
19
Q
What do you see?
A
- Roth spots: retinal hemorrhages due to infective endocarditis
20
Q
What are the common lab findings and complications associated with infective endocarditis?
A
- Lab findings: high ESR, anemia, proteinuria (may also have Rheumatoid factor or hematuria)
- Complications: heart failure, septic emboli (to kidneys, heart, spleen, brain)
21
Q
What tests/procedures are helpful in diagnosing infective endocarditis? Which of these is essential?
A
- Transthoracic echocardiography: 60% sensitivity for vegetations
1. Transesophageal echocardiography: >90% sensitive for vegetations
2. Absence of vegetations on echo does not exclude endocarditis dx - Murmur + echo + vegetations does NOT = dx -> need BLOOD CULTURES (essential for making specific dx to guide AB therapy)
1. Continuous low-grade bacteremia characteristic of infective endocarditis: 3 sites cultures, 30-60min apart before starting AB’s
2. Alert lab endocarditis suspected b/c some bac that cause endocarditis fastidious, or slow-growing, or both, so lab will use special culture media, and hold the cultures longer
22
Q
What are the pluses and minuses of surgical valve replacement?
A
- Can be life-saving, esp. w/acute disease
- Immediate relief w/chronic disease, but replaces one chronic disease w/another -> mechanical valves require lifelong anti-coagulation
- Bioprostheses deteriorate, with structural failure in up to 50% by 10 years, accelerating after that
- Complications include: leak, thrombosis, embolism, bleeding, and endocarditis
- Critical to make sure patients understand these risks/complications from the outset
23
Q
What do you see?
A
- Infective endocarditis: remember, fever + heart murmur = infective endocarditis (until proven otherwise)
- Mitral valve identifiable by chordae tendineae going to both leaflets (not aortic valve)
- Very large, extensive vegetations involving about 80% of leaflets (bigger the vegetations, the more likely they are infective)
- NOT mitral valve prolapse b/c many cases have valves that are microscopically normal; it is just too big. Even those with myxomatous change tend to have a normal surface -> inner zona spongiosa expanded, while outer zona fibrosa may be thinned, but is otherwise normal
- Size and extent of these very large, very extensive vegetations goes against marantic endocarditis
24
Q
What is a probe-patent foramen ovale (often shortened to foramen ovale)?
A
- About 20% of ppl have membranous flap of tissue over foramen ovale that can be opened by pushing a probe on it
- Normally, higher pressures on left side of heart keep flap in place, maintaining separateness of left and right heart circulations
1. If pressures on right side ever rise to level higher than left, can act like probe and push open foraman ovale b/t right and left atria, letting deoxygenated blood skip passing through lungs
25
What are the 4 components of tetralogy of fallot?
- Ventricular septal defect (VSD)
- Overriding aorta
- Pulmonic stenosis
- RV hypertrophy
26
Which congenital heart diseases are cyanotic and non-cyanotic?
- _Cyanotic_ (all start with T, except hypoplastic left heart): tetralogy of fallot, transposition of the great vessels, truncus arteriosus, tricuspid atresia, total anomalous pulmonary venous return
- _Non-cyanotic_: ventricular septal defect, atrial septal defect, patent ductus arteriosus, pulmonic stenosis, aortic stenosis
27
What is the most specific diagnosis you can make from this image?
- Zone of healing tissue with bluish ground substance and a few lymphs and macs
- Zone of basophilic debris (nuclear dust)
- Outer layer of condensed fibrin with scattered basophilic debris
- Central outer area of little blue dots way too small to be lymphocyte nuclei, just the right size for bacterial cocci (all bacteria are blue in H&E)
- **Infective endocarditis**, w/bacterial cocci: probably staph or strep, since staphy cause majority of acute bac endocarditis and strep cause majority of subacute bac endocarditis
28
This gross pathology most likely represents...?
- **Acute rheumatic valvulitis**: tiny (1-2mm) verrucous (wart-like) vegetations lined up on line of valve closure
- _Differential_:
1. Vegetations of infective endocarditis usually much larger
2. Vegetations of Libman-Sacks usually bigger than this (intermediate size) and rarely this numerous, nor are there features to suggest it (i.e., being on both sides of valve)
3. Marantic a strong consideration, but so many so tiny vegetations that acute rheumatic valvulitis is just slightly more likely
29
Briefly describe each of these.
- _Rheumatic heart disease (RHD)_: rheumatic fever phase marked by small, warty vegetations along lines of closure of leaflets
- _Infective endocarditis (IE)_: large, irregular masses on valve cusps that extend onto chordae
- _Nonbacterial thrombotic endocarditis (NBTE)_: typically exhibits small, bland vegetations, usually attached at line of closure (one or many may be present)
- _Libman-Sacks endocarditis (LSE)_: small, or medium-sized vegetations on either or both sides of valve leaflets
30
88-y/o hispanic male dies of a stroke, and his heart has the features in this image. The best diagnosis for this cardiac condition is...?
Infective endocarditis because it is destructive: can eat a hole in a heart valve
31
6 day-old boy presented to cardiology clinic with cyanosis. Born via spontaneous vaginal delivery at 37 weeks gestational age to a G1P0 mother following an uncomplicated pregnancy. Although he failed an “oxygen screen” in the hospital, he was otherwise well and was discharged to home on day 3 with instructions to follow up with the cardiology clinic. At his clinic visit, he was cyanotic w/O2 saturations in the 70s. After a chest x-ray and echocardiogram revealed congenital heart abnormalities, he was admitted to the pediatric intensive care unit.
Following surgical exploration of his heart and great vessels, a palliative procedure was performed. His chest x-ray showed a boot-shaped heart. He died on hospital day 81 and autopsy revealed the gross pathology shown. Ao = aorta, AV = aortic valve, RA = right atrium, TV = tricuspid valve, RV = right ventricle. The asterisk identifies...? What is the overall diagnosis?
- Ventricular septal defect with overriding aorta
- Tetralogy of fallot: 1) VSD, 2) overriding aorta, 3) pulmonic stenosis, 4) RV hypertrophy
32
A 22-year-old white female college student unaware that she was born with a probe-patent foramen ovale and with heterozygous factor V Leiden mutation has her leg mauled by a wolf, while on a camping trip in a remote area of Wyoming. Half a day later, she suddenly turns blue.
What happened?
- Her leg wounds caused a large DVT due to her hypercoagulable state, and large thromboembolus from leg obstructed right ventricular outflow so much so suddenly that the pressure in her right atrium rose to higher than in her left atrium, which caused the membranous tissue flap over the foramen ovale to open and let deoxygenated blood into the left atrium
- **Eisenmenger syndrome**, seen most often in children with congenital heart diseases with holes between the left and right heart (ASD, VSD, etc.) and chronic left-to-right shunt that causes progressive pulmonary hypertension until one day the shunt reverses
33
This 84-year-old white male with a history of (1) hypertension (2) smoking (remote) (3) dyslipidemia (4) abdominal aortic aneurysm (5) prostate cancer presented with dyspnea on exertion. His heart had the condition shown.
What is the best diagnosis for his cardiac condition?
Calcific aortic stenosis
34
Mitral valve prolapse (briefly)
- Common
- Benign
- Mid-systolic click
- +/- regurgitation
35
Marantic endocarditis (highlights)
- Common
- Thrombi on valves
- Precursor to infective endocarditis
36
Libman-Sacks endocarditis (briefly)
- Part of SLE
- Most common in young, black females
- Rarely embolizes
37
Infective endocarditis (briefly)
- Uncommon
- Fatal, if missed
- Heart murmurs
- Blood cultures
38
Calcific aortic stenosis (briefly)
- Common
- Male
- Systolic ejection murmur
- Onset of symptoms = time for valve replacement
39
47-year-old white male had a history of alcohol and cocaine abuse and mitral valve prolapse. Brought to the ER nearly unresponsive with flaccid right side, fever of 40.3, pulse 136, blood pressure 189/98, respirations 30, diffuse pulmonary rhonchi and “no murmurs” specifically dictated by the admitting physician. Admission blood cultures all yielded Staphylococcus aureus. He died 36 hours later, and his heart showed the attached image. The most likely reason the admitting physician heard no murmur is...?
Inadequate physical examination
40
45-year-old south Asian female manages a motel in the South. She presents with intermittent fever, chills, weakness and progressive dyspnea on exertion for 3 months. Her temperature is 38.2, pulse 102 and blood pressure 100/70. On cardiac auscultation she has a grade II/VI holosytolic murmur.
What test is most likely to yield a specific diagnosis of the cause of her condition?
Blood culture (essential for making a specific diagnosis to guide AB therapy for infective endocarditis)
41
A 45-year-old south Asian female manages a motel in the South. She presents with intermittent fever, chills, weakness and progressive dyspnea on exertion for 3 months. Her temperature is 38.2, pulse 102 and blood pressure 100/70. She has a grade II/VI holosytolic murmur.
What organism would blood cultures most likely yield?
- **Strep viridans**
- Remember:
1. Acute bacterial endocarditis: usually sudden, due to highly virulent orgs (i.e., S aureus)
2. Subacute bac endocarditis: insidious onset over weeks or months, due to less virulent org (i.e., strep viridans)
- _NOTE_: endocarditis occurs on atrial side of mitral and ventricular side of aortic valve b/c these areas are continuously hit by being abruptly brought together to close heart chambers for the cardiac cycle and suffer microscopic trauma all the time (all organisms require endocardial cellular injury, then clot deposition to infect)
42
What is this?
- **Mitral valve prolapse** at open heart surgery
- Note the uniform color of the valve; it looks normal except for being redundant and folded up into mountains and valleys
- No vegetations are evident.
43
Why can pregnancy cause mitral regurgitation from prolapse to go into remission?
- Pregnancy causes a 45% increase in blood volume (average 45%)
- Can cause the left ventricle to dilate enough to allow redundant mitral valve leaflets to coapt without regurgitation
44
A 25-year-old East Asian male medical student has an episode of syncope at bedtime. EKG is most likely to show a diagnostic...?
- **ST segment elevation** -\> Brugada syndrome: persistently elevated ST descending w/upward convexity to inverted T-wave (coved type; V1-V3); mostly YOUNG ASIAN MALES that present at avg age of 40 w/syncope or sudden death due to vtach, esp. DURING SLEEP
- NOT congenital QT syndrome (channelopathies) b/c present in CHILDHOOD w/syncope or sudden death due to TORSADE, triggered by EXERCISE
- NOT arrhythmogenic right ventricular cardiomyopathy (ARVC) b/c clinical presentation typically non-specific (epsilon wave notch in terminal part of QRS, QRS prolongation, T-wave inversion, etc.); most prevalent in N ITALY, and sometimes triggered by exercise
45
30-year-old black female engineer presents with the insidious onset of dyspnea on exertion. Her pulse is 80 and blood pressure 130/90. On cardiac auscultation, she has a high pitched sound after S2 and a diastolic rumble. Her heart has the attached image.
The complication of her cardiac condition shown by the arrow is most likely?
- **Thrombus formation**: any condition causing stasis of blood in the left atrium predisposes to clot formation (in this case, Rheumatic mitral stenosis is causing impaired outflow of blood from left atrium)
- _Virchow's triad_: 1) endothelial injury, 2) abnormal blood flow, 3) hypercoagulability
46
24-year-old white male medical student in Memphis, TN, presents in February with the abrupt onset of fever, chills, weakness, dyspnea, cough, nasal congestion, sore throat, myalgias and malaise.
He most likely has?
Influenza
47
24-year-old white male medical student in Memphis, TN, presents in February with the abrupt onset of fever, chills, weakness, dyspnea, cough, nasal congestion, sore throat, myalgias and malaise.
He appears “toxic” (ill). His BMI is 25, temperature 39.5, pulse 120, blood pressure 110/70, respirations 22 and saturation 97%. He has a grade III/VI pansystolic high pitched “blowing” murmur and a grade I/IV mid-late diastolic low pitched rumbling murmur. His lungs are clear, abdomen normal and extremities without edema, but he has a few petechiae on his soles.
What is the most likely diagnosis?
Infective endocarditis. Most likely cause is: mitral valve prolapse.
48
What is carcinoid heart disease?
- _Carcinoid syndrome_ results from bioactive compounds like serotonin released by carcinoid tumors
- Systemic manifestations include flushing, diarrhea, dermatitis and bronchoconstriction
- **Carcinoid heart disease**: cardiac manifestations caused by the bioactive compounds and occurs in half of the patients in whom the systemic syndrome develops -\> endocardium and valves of the right heart are primarily affected
