Cardiology Flashcards

1
Q

List 4 infectious causes of myocarditis

A
Enterovirus
Coxsackie virus
Adenovirus
Parvovirus
EBV/CMV
Rickettsia
Diptheria
Hep C
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List 3 cardiac defects associated with DiGeorge

A

Conotruncal defects

  • TOF-usually PA, MAPCAs***
  • TA**
  • DORV
  • Subarterial VSD

Branchial arch defects

  • CoA
  • Interrupted aortic arch (type 2B!)***
  • Right aortic arch
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

List steps in transitional circulation

A
  1. UA closes, UV remains more patent
  2. Cord clamped–>removal of placenta–>ductus venosus closes–>↑SVR
  3. ↓PVR from (1) mechanical expansion of lungs and (2) ↑ in arterial PAO2 (causing ↑ in vasodilation)
  4. PVRoutput from right heart flows through lungs
  5. PDA closure (due to o ↑ arterial PAO2 )
  6. PFO closure (due to ↑ volume of flow returning to left atrium)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

In what % of adults does PFO persist?

A

1/3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the incidence of CHD i?

A

0.8% of live births

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the common CHD ?

A

VSD (1/3)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What cardiac lesions cause in utero heart failure (hydrops)?

A

Severe RVOTO
Severe AVVR
Ebstein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

List 3 genetic counselling points for CHD

A
  1. 1 affected sib risk increased to 2-6%, 2 sibs 10% (vs. 0.8% in general population)
  2. If 2 1st degree relatives, risk 20-30% in subsequent
  3. Tend to be similar class (conotruncal, atrioventricular septal, Rt obstruction, Lt obstruction)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How do you perform a hyperoxia and what do the results mean?

A

Apply 100% O2 x 15 min, then ABG taken from right radial artery

If Pao2 rises > 150 mm Hg: intracardiac right–left shunt essentially excluded

If PaO2 remains < 50 mmHg, cyanotic CHD vs severe PPHN/lung disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

CXR findings in TGA

A

Egg on a string

Thin mediastinum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

CXR findings in TA

A

Wide mediastinum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

CXR findings in Ebstein

A

Wall to wall heart

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the primary defect in TOF?

A

Abnormal infundibular septum, that separates aortic/pulmonary outflow tracts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

4 components of TOF

A

(1) RVOTO (pulmonary stenosis + subpulmonic area)
(2) VSD
(3) aorta that overrides ventricular septum
(4) RVH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which component of TOF determines degree of cyanosis?

A

RVOTO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

List 4 treatments for TOF spell and how they work

A

(1) Prone in knee-chest position (increases afterload thus decreasing R to L shunting)
(2) Supplemental O2
(3) Calming and holding
(4) Morphine (treat hyperpnea and decrease systemic catecholamines)
(5) Phenylephrine (increases afterload)
(6) β blockers (to block beta receptors in infundibulum therefore lessening RVOTO)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Describe the murmur in TOF

A

Loud harsh SEM at LUSB, sounding more holosytolic at LLSB

S2 single or soft P2 component

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

List CXR findings in TOF

A

Normal heart size
“Boot shaped heart” (elevation of apex, concavity of main PA area)
Right-sided AoA
↓ pulmonary vascularity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Repair and treatment of TOF

A

Repair in infancy

Severe RVOTO requires PGE infusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

List 2 long term complications patients with repaired TOF

A

Pulmonary insufficiency

RV dilatation (QRS duration increases)

Ventricular arrhythmias

Increased risk of SCD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Describe the physiology of tricuspid atresia

A

Single ventricle physiology!

All systemic venous return moves from RA to Lt side of heart by ASD

Blood then enters LV and is ejected to systemic circulation (through aorta) and to pulmonary circulation (by Lt–Rt shunt either by a PDA or a VSD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the classic ECG finding in tricuspid atresia

A

Leftward superior axis on ECG, LVH (similar to AVSD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Murmur in tricuspid atresia

A

Holosystoic murmur LSB

Prominent LV impulse (in most cyanotic CHD there is ↑d RV impulse)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe the pathophysiology of Ebstein’s

A

Downward displacement of an abnormal tricuspid valve into the RV

Leaflets adherent to wall

TV is insufficient resulting in TR

Extra volume of desaturated right atrial blood shunted right–left across an ASD or PFO

Due to combo of TR, poorly functioning small RV, and RVOTO from large sail-like TV leaflet, the effective output from Rt heart ↓s; if patent, PDA provides additional pulmonary flow

Due to TR, RA becomes enlarged

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What determines the severity of cyanosis in Ebstein?
1. Extent of TV displacement | 2. Severity of RVOTO
26
What murmur is characteristic of Ebsteins?
Holosystolic murmur from TR Gallop rhythm Multiple clicks at LLSB
27
What ECG findings are typical of Ebstein's?
RBBB without signs RVH | Associated with WPW
28
What is the classic CXR finding in Ebstein's?
"Wall to wall heart"
29
List the cyanotic heart lesions that have decreased pulmonary blood flow
(1) Tricuspic atresia (2) Tricuspid malformation (Ebstein) (3) TOF (4) Pulmonary atresia with intact septum
30
List the cyanotic heart lesions that have increased or normal pulmonary blood flow
NOTE: Can results from either i) Abnormal ventricular-arterial connection (i.e. TGA) ii) Mixing (1) TGA (2) Truncus arteriosus (3) Single ventricle without pulmonary obstruction (4) TAPVR
31
Which lesion classically results in post ductal sats > preductal sats?
TGA
32
What murmur is classic in TGA?
``` Usually none Can have holosystolic murmur from VSD Single S2 (because PV very posterior and can't hear) ```
33
What ECG finding is classic in TGA?
None | Usually normal :)
34
What are the CXR findings in TGA?
Egg on a string Narrow mediastinum Normal to increased pulmonary blood flow
35
What emergency treatment is required in TGA if PGEs fail?
BAS
36
Where do you pulmonary veins drain in supracardiac and infracardiac TAPVD?
Supracardiac-RA, coronary sinus, or SVC Infracardiac-Below the diaphgram into IVC, often via ductus venosus
37
Why are infracardiac TAPVDs more likely to be obstructed?
Ductus venosus closes after birth and can result in complete obstruction oxygenated pulmonary blood flow back to heart
38
Why is obstructed TAPVD a surgical emergency?
PGEs don't work! | May make things worse by increasing pulmonary blood flow
39
What is the classic CXR finding in obstructed TAPVD?
Dramatic perihilar pattern of pulmonary edema and a small heart
40
What is the classic CXR finding in unobstructed/mild TAPVD?
Snowman-in supracardiac Cardiomegaly PA and RV are prominent Increased pulmonary venous blood
41
What is the murmur of truncus arteriosus?
Single loud S2 Early systolic ejection click (from truncal valve) SEM over LSB
42
How does truncus arteriosus typically present?
Usually minimally cyanotic +murmur at birth | Signs of heart failure as PVR drops (few months of age)
43
What is the ECG finding in truncus arteriosus?
BVH
44
Describe the major hemodynamic abnormalities in HLHS
1. Inadequate systemic circulation 2. Depending on the size of the atrial-level communication, either pulmonary venous hypertension (restrictive foramen ovale) or pulmonary overcirculation (moderate or large ASD)
45
What surgery is used for HLHS?
Norwood-->Glenn-->Fontan
46
Which cyanotic heart lesions have a single S2?
TGA Truncus PA/IVS TOF HLHS Tricuspid atresia
47
Which cyanotic heart lesions have no murmur?
TGA
48
Which cyanotic heart lesion has diastolic murmur?
Truncus
49
List 4 side effects of PGEs
``` Apnea HypoTN Brady Fever ↓ plt ```
50
What is the most common type of ASD?
Secundum* (75%) Primum (15%) Sinus venosus (10%)
51
What associated defect should you always rule out in sinus venosus ASD?
Partial anomalous pulmonary venous drainage
52
What is the murmur of ASD?
SEM Fixed split S2 RV systolic lift Can have mid-diastolic rumble with increased flow across TV
53
List indications for ASD repair
1. Symptomatic 2. RV dilatation 3. Qp:Qs>=2:1
54
What type of shunt does PAPVD cause?
Left to right
55
Describe the pathophysiology of AVSD
Contiguous atrial and ventricular septal defects with Abnormal AV valves May be associated with hypoplasia of one ventricle Combine AVVR + left to right shunt cause Rt sided volume overload
56
Murmur of AVSD
Similar to ASD (SEM at LSB, wide fixed-split S2) | Additional apical holosytolic murmur from MV insufficiency
57
What is the classic ECG finding in AVSD***
(1) Leftward-superior axis** classic (2) Q wave leads I and aVL (3) Biventricular hypertrophy, (4) Tall P waves (rt atrial enlargement) (5) Rt conduction delay rSR' (especially after repair)
58
When should AVSD be repaired and why?
``` 4-6 months Avoid PHTN (can develop as early as 6-12 months) ```
59
When do VSDs become symptomatic typically and why?
4-8 weeks
60
CXR findings in VSD
Cardiomegaly Prominent PA ↑ pulmonary vasculature
61
List 3 physical exam findings consistent with VSD
``` Holosytolic murmur over LLSB (loud, harsh, blowing) Thrill Prominence of left precordium Parasternal lift Laterally displaced apex ```
62
What percentage of small VSDs close spontaneously and by what age?
(30-50%) close spontaneously Usually by 4yo More likely if muscular than membranous
63
When are large VSDs typically repaired?
4-6 months
64
In term infant, after how many days is a PDA unlikely to spontaneously close?
1 week | In a term infant, usually due to structurally abnormal PDA
65
List physical findings associated with PDA
Continuous murmur LUSB/LLSB (machinery-quality) Ventricular heave Prominent apical pulse Wide pulse pressure
66
List 3 reasons all PDAs in term infants should be surgically closed
Prevent endocarditis Prevent CHF Prevent PHTN
67
What is the most common CHD in Noonan's?
Dysplastic pulmonary valve (leading to stenosis)
68
List 3 clinical manifestations of severe pulmonary stenosis
Right sided HF - Hepatomegaly - Peripheral edema Cyanosis (from PFO shunt)
69
Murmur of pulmonary stenosis
SEM at LUSB radiating to lung fields Split S2 with delayed/soft P2 Pulmonic ejection click (stiff valve) RV lift
70
ECG findings in pulmonary stenosis
RVH | Right atrial enlargement
71
CXR findings in pulmonary stenosis
Cardiomegaly Prominent RV, RA Prominent PA due to poststenotic dilatation ↓ pulmonary vascularity
72
List 2 conditions associated with peripheral pulmonary stenosis
Williams Syndrome | Allagille syndrome
73
What are the 3 types of aortic stenosis and what conditions are associated with them?
Valvar AS-most common! Subvalvar AS-HOCM Supravalvar AS-Williams syndrome
74
Murmur in aortic stenosis
SEM at RUSB Radiating to neck Early systolic ejection click S4 with decreased LV compliance
75
ECG findings in aortic stenosis
LVH LAD LV strain (inverted t-waves in left leads)
76
Should patients with moderate/severe aortic stenosis participate in sports?
NO | At risk of SCD
77
Why do you have differential cyanosis with CoA?
Blood shunts R-->L through PDA to supply lower extremities, resulting in differential cyanosis (blue LEs)
78
List physical exam findings consistent with CoA
UE>LE pulses Radial-femoral delay Lower BP in LE Systolic murmur LSB
79
What finding on CXR is pathognomic of CoA?
Rib notching
80
ECG findings in CoA
Infants-RVH/BVH | Older children-LVH
81
What the physiology of ALCAPA?
Anomalous Lt Coronary Artery from Pulmonary Artery As PVR ↓s, perfusion pressure to LCA becomes inadequate Blood supply to LV myocardium severely compromised LV dilatation, dysfunction
82
How does ALCAPA typically present?
First few months of life | Recurrent attacks of discomfort, restlessness, irritability, sweating, dyspnea, and pallor
83
ECG in ALCAPA
Q waves and inverted T waves leads I and aVL; left precordial leads (V5 and V6) may have deep Q waves, elevated ST segments, inverted T waves
84
Explain physiologic split S2
On inspiration-->decreased intrathoracic pressure, fills right heart and increases RV ejection time and delayed closure of pulmonary valve
85
What lesions have fixed split S2
RV overload!: ASD TAPVD PS Ebstein anomaly
86
What is the murmur of mitral valve prolapse?
Mid systolic click | Late systolic murmur at apex
87
List 5 functional murmurs
Still's murmur Venous hum Innocent pulmonic murmur
88
Describe the features of Still's murmur
``` Medium-pitched Vibratory or musical Short SEM LLSB/MSB Age 3-7 years Decreases with sitting Changes with respiration/position DOES not radiate ```
89
Describe the features of innocent pulmonic murmur
Heard in supine position 2nd parasternal space High pitched, blowing
90
Describe features of venous hum
``` Early childhood Turbulence in jugular/venous system Heard in anterior neck/upper chest Subclavicular area Soft humming sound in systole and diastole – exaggerated or disappears with changing head position or compressing jugular venous system ```
91
In a patient with a murmur, list 5 features that suggestive of CHD
``` Diastolic Pansystolic Late systolic Grade III or higher Harsh Loudest at Left upper sternal border Associated with early or midsystolic click Abnormal second heart sound Absent or diminished femoral pulses ```
92
List 5 characteristics of innocent murmurs
``` Murmur intensity grade II or less Heard at left sternal border Varies with position (decreases when upright) Normal second heart sound No audible clicks Normal pulses Normal growth, development, no cyanosis ```
93
Which lesions cause ejection clicks
Stenosis (Ao/Pulm) MVP Dilated aorta/PA (TOF/TA)
94
How do you diagnose vascular rings?
CT and MRA | Old study-barium esophagram
95
What conditions predispose children to myocardial ischemia?
Hyperlipidemia Prior Kawasaki disease with coronary artery aneurysms or stenoses Substance use ALCAPA
96
List a differential for chest pain
Cardiac disease*** - LVOTO: Hypertrophic cardiomyopathy, aortic stenosis, coarctation of the aorta - Coronary artery anomalies (KD, ALCAPA) - Pericarditis - Myocarditis - Dilated cardiomyopathy - Tachyarrhythmias - Aortic root dissection - Drug induced angina (cocaine, amphetamines, bath salts, marijuana) Musculoskeletal: - Costochondritis - Muscle strain - Trauma - Pleurodynia - Slipping rib - Precordial catch Psychogenic conditions: - Panic attack - Anxiety - Somatization Respiratory conditions: - PTX*** - Pulmonary hypertension *** - Acute chest syndrome **** - Foreign body*** - Asthma - Pneumonia - Pleuritis - Pulmonary embolism Gastrointestinal: - Esophageal rupture (Boerhaave syndrome) *** - Gastroesophageal reflux - Esophagitis - Esophageal spasm - Gastritis
97
What is the characteristic ECG of Pompe disease?
Prominent P waves Short P-R interval Massive QRS voltages
98
List 4 causes of hypertrophic cardiomopathy
Familial or sporadic HCM IEM (Mitochondrial disorders, GSD, Pompe, MPS, hemochromatosis) Genetic syndromes (Noonan, Beckwith Widemann, swyer's syndrome, LEOPARD syndrome, friedrich's ataxia) Infant of a diabetic mother
99
Describe the pathophysiology of HOCM
Cardiac myofibrils and myofilaments demonstrate disarray and myocardial fibrosis Increased LV wall thickness Asymmetric septal hypertrophy Systolic function preserved, diastolic dysfunction Systolic anterior motion of MV Resting or provocable outflow tract gradient
100
Physical exam findings in HOCM
Forceful LV apical impulse Systolic ejection murmur Increased intensity with: i) Upright posture (from a squatting, sitting, or supine position) ii)Valsalva maneuver Decrease intensity with: - Standing to a sitting or squatting position - Handgrip - Passive elevation of the legs.
101
What ECG findings are consistent with HOCM?
LVH ST segment and T-wave abnormalities Intraventricular conduction delays
102
List 3 management strategies for HOCM
Avoidance of competitive sports Beta blockers (reduce outflow tract obstruction) ICD if risk factors for SCD Screening of first degree relatives + genetic testing
103
List etiologies of dilated cardiomyopathy
Familial or sporadic DCM Viral myocarditis Muscular dystrophies IEM (fatty acid oxidation disorders, carnitine abnormalities, mitochondrial disorders, organic acidemia) Genetic syndromes (alstrom syndrome, barth syndrome) Drugs (anthracyclines) Ischemic (rare)
104
What is the inheritance pattern of DCM?
Familial-Autosomal dominant DMD/Beckers-X-linked Mitochondrial myopathies-mitochondrial or AR
105
Workup for DCM
CBC, renal and LFT, CPK, cardiac troponin I, lactate, plasma amino acids,urine organic acids, and an acylcarnitine profile Genetic and enzymatic testing may be useful Consider screening of 1st-degree family members with echo and ECG
106
List cardiac findings in Marfan's
Dilation of aortic root (50% of children, 60-80% of adults Mitral valve prolapse (40-50%) VT/SVT Prolonged QT Aortic aneurysm/dissection Dilated cardiomyopathy
107
Management of hypertrophic cardiomyopathy in IDM?
Symptomatic infants typically recover after 2-3 weeks of supportive care Echocardiographic findings resolve within 6 to 12 months If symptomatic-can try propanolol
108
What is the most common cardiac lesion in IDM?
HCM
109
What is pulsus alternans and what is it associated with?
Beat to beat alternation in pulse size and intensity Associated with left sided heart failure
110
What is pulsus bisferiens and what is it associated with?
Double beating pulse with two systolic beats Associated with aortic insufficiency, HOCM, high cardiac output states
111
What is pulsus paradoxus and what is it associated with?
Exaggerated decline in BP during inspiration (>10 mmHg), resulting from increase in negative thoracic pressure On inspiration, RV EDV increases, septum bows out and decreases LV preload Associated with tamponade, constrictive pericarditis, severe lung disease
112
What is pulsus bigeminus and what is it associated with?
Two heartbeats close together followed by a longer pause. The second pulse is weaker than the first Alternating QRS Associated with digitalis toxicity, HOCM
113
List 3 physical findings consistent with pericarditis
``` Muffled heart sounds Narrow pulse pressure JVD Pulsus paradoxus Pericardial friction rub ```
114
List 10 causes of PHTN
``` Idiopathic Familial Connective tissue disorder PVOD Pulmonary capillary hemangiomatosis PPHN Left sided heart disease Lung disease (ILD, CLD, OSA,) Chronic thrombotic/embolic disease Sarcoidosis Histiocytosis X ```
115
What is the definitive diagnostic test for PHTN?
Catheterization | -Should show pulmonary arterial HTN and NORMAL pulmonary capillary wedge pressure
116
List 5 causes of CHF that present at birth
``` Anemia Acidosis Hypoxia Hypoglycemia Hypocalcemia Sepsis ```
117
List 5 causes of CHF that present in first month
``` HLHS Aortic stenosis Coarctation VSD (4-8 weeks depending on size and fall in PVR) Anomalous left coronary (ALCAPA) ```
118
List 5 causes of CHF that present as child
``` Myocarditis Cardiomyopathy (dilated or hypertrophic) Tachyarrhythmia Severe anemia Rheumatic fever ```
119
List 5 important aspects in management of CHF
1) Ensure adequate nutrition - NG feeds - Aggressive reflux management 2) Diuretics 3) Afterload reduction - Useful in HF secondary to cardiomyopathy, mitral, aortic insufficiency left-to-right shunts 4) Fluid/salt restriction
120
Cardiac lesions associated with T21
VSD*** most common AVSD ASD
121
Cardiac lesions associated with T13/T18
T13-ASD, VSD, PDA, valvar disease, CoA | T18-VSD, polyvalvar disease
122
Cardiac lesions associated with Turner syndrome
Bicuspid AoV Coarct AS
123
Cardiac lesions associated with Fragile X
Mitral valve prolapse | Ao root dilatation
124
Cardiac lesions associated with DiGeorge
Interrupted aortic arch | Truncus arteriosus
125
Cardiac lesions associated with CHARGE
``` ASD VSD AVSD PDA TOF ```
126
Cardiac lesions associated with Alagille
Peripheral pulmonic stenosis
127
Cardiac lesions associated with Williams
Supravalvular aortic stenosis | PPBS
128
Cardiac lesions associated with FASD
VSD, ASD
129
Cardiac lesions associated with Holt Oram
ASD (60%), VSD (30%), arrhythmia
130
Cardiac lesions associated with Ellis-van Creveld
ASD
131
Cardiac lesions associated with VACTERL
ASD VSD PDA TOF
132
Cardiac lesions associated with PHACES
VSD PDA Coarctation Arterial aneurysms
133
Cardiac lesions associated with Noonans
Pulmonic stenosis ASD Cardiomyopathy
134
What are the 3 most common bacteria causing infective endocarditis
1. Alpha hemolytic Strep (Strep viridans) (after dental procedures) 2. S. aureus (more common in kids without CHD) 3. Enterococci (GI/GU manipulation) 4. Pseudomonas (IV drug users) 5. Serratia (IV drug users) 6. CONS (indwelling line) 7. Fungal (after open heart sx)
135
Physical exam features of IE
New murmur Splenomegaly Petechiae Osler nodes(tender pea-sized intradermal nodules in the pads of the fingers and toes) Janeway lesions(painless small erythematous or hemorrhagic lesions on the palms and soles) Splinter hemorrhages(linear lesions beneath the nails) Roth spots New clubbing
136
Duke Criteria for IE
2 major criteria, 1 major and 3 minor, or 5 minor criteria suggest definite endocarditis Major criteria: (1) Positive blood cultures (2 separate cultures for a usual pathogen, ≥2 for less typical pathogens) (2) Evidence of endocarditis on echo (cardiac mass on a valve or other site, regurgitant flow near a prosthesis, abscess, partial dehiscence of prosthetic valves, or new valve regurgitant flow) Minor criteria: (1) Predisposing conditions (2) Fever (3) Embolic-vascular signs (4) Immune complex phenomena (glomerulonephritis, arthritis, rheumatoid factor, Osler nodes, Roth spots) (5) Single positive blood culture or serologic evidence of infection (6) Echo signs not meeting the major criteria
137
List complications of endocarditis
CHF Myocardial abscesses Emboli (often with CNS manifestations) Pulmonary emboli may occur in children with VSD or TOF Mycotic aneurysms Obstruction of a valve secondary to large vegetations Acquired VSD Heart block ``` Others: Meningitis Osteomyelitis Arthritis Renal abscess Purulent pericarditis Immune complex-mediated glomerulonephritis ```
138
Empiric therapy for endocarditis
Vanco +gent
139
Duration of treatment for endocarditis
4-6 weeks
140
Which groups have the highest probability of adverse outcomes from IE (and thus should receive prophylaxis)? (CPS)
1. Prosthetic cardiac valve or prosthetic material used for valve repair 2. Previous IE 3. Congenital heart disease (CHD) a. Unrepaired cyanotic CHD, including palliative shunts and conduits b. Completely repaired CHD with prosthetic material or device (both surgical and cath) during the first six months after the procedure c. Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization) 4. Cardiac transplant recipients who develop cardiac valvulopathy 5. Rheumatic heart disease if prosthetic valves or prosthetic material used in valve repair
141
Which procedures require endocarditis prophylaxis? (CPS)
1. Dental procedures that involve the manipulation of gingival tissue, the periapical region of teeth or perforation of the oral mucosa-including CLEANING 2. Invasive procedures of the respiratory tract that involves incision or biopsy of the respiratory mucosa, e.g.T&A
142
What antibiotic should be used for endocarditis prophylaxis and how many doses?
Single dose Amoxicillin/Ampicillin Pen allergic-keflex, clinda, azithro
143
What was the reasons for revising guidelines on cardiac prophylaxis?
Infections were more likely from exposures from everyday life and not that much more from dental procedures
144
What are the most common patterns of valve involvement in rheumatic heart disease?
MR>AR>MS>AS
145
What is a bacterial cause of myocarditis that has been eliminated by vaccination?
Diptheria (AV block, bundle branch block, or ventricular ectopy)
146
ECG findings in pericarditis
Low QRS voltage Diffuse ST segment elevation PR segment depression T wave inversion (later)
147
List causes of pericarditis
``` Viral (Enteroviruses, influenza, adenovirus, RSV, parvovirus) Bacterial (pneumococcal, Hib) TB JIA SLE Hypothyroidism CKD Malignancy ```
148
What is the cause of constrictive pericarditis?
Recurrent or chronic pericarditis Cardiac surgery Radiation to the mediastinum
149
What is the definition of hypertension?
Adults : BP ≥140/90 mmHg Prehypertension: SBP or DBP between 90th – 95thpercentile Children: SBP and/or DBP ≥95th percentile for age, sex, and height on ≥3 occasions Stage 1 HTN: BP between 95th - 99th percentile plus 5 mmHg Stage 2 HTN: BP >99th percentile plus 5 mmHg
150
List risk factors for primary HTN
Overweight Male Black, Hispanic Family hx of HTN
151
List causes of secondary HTN
Renal - Renovascular (fibromuscular dysplasia, UAC) - Glomerulonephritis - PCKD - CKD Cardiac -Coarctation Endocrine - Pheochromocytoma - Neuroblastoma - Cushing's syndrome - Hypercalcemia - Thyroid disorder Drugs - Steroids - OCP - Tacrolimus - Cyclosporine
152
Definition of hypertensive emergency
Severe HTN + End organ damage (seizures/encephalopathy, heart failure, renal dysfunction, papilledema/retinal hemorrhage/exudates)
153
List 4 signs of target end organ damage in chronic HTN
LVH (40% with subclinical HTN) Retinopathy Microalbuminuria Carotid intima-media thickness
154
Workup of HTN
1. Confirm - If repeated measurements >90th percentile, confirm with 24H ambulatory blood pressure monitoring - If BP >95th%ile confirmed, investigate for secondary causes ``` 2. Etiology: 4-limp BP Echocardiogram CBC, retic Urinanalysis, lytes, BUN/Cr Ca Renal U/S + doppler Urine tox ``` 3 Screen for end organ damage - Echo - UA - Eye exam 4. Screen for comorbidities - Fasting lipids and BG - Sleep history
155
List the indications for pharmacologic management of HTN
``` Symptomatic HTN Secondary HTN Target organ damage (incl LVH!) Diabetes (types 1 and 2) Persistent HTN despite nonpharmacologic measures ```
156
What drug classes can be used for hypertension management in children?
ACE-I, ARBs, β-blockers, Ca-channel blockers, and diuretics
157
What is the goal of HTN management?
Goal should be to reduce BP to <95th percentile If pt withCKD, diabetes, or target organ damage, goal should be to reduce BP to <90th%le
158
What antihypertensives should be used in patients with migraines?
β-Blockers or Ca-channel blockers
159
What antihypertensives should be used in diabetics with proteinuria or proteinuric renal disease?
ACEi/ARB
160
What antihypertensives should be used in hypertensive emergency?
IV Labetalol IV Nicardipine IV SNP (can cause CN toxicity)
161
How quickly should you decrease BP in hypertensive emergency?
No more than 25% in first 8 hours
162
When should you restrict physical activity (high static sports) in a patient with HTN?
Stage 2 HTN LVH
163
Proper BP measurement
Right arm (pre ductal; left can be falsely low in coarctation) Appropriate width cuff (bladder cover 80% of upper arm) Bladder length should encircle arm completely Seated and resting quietly x 5 minutes
164
List the late complications of Fontan
PLE Plastic bronchitis Arryhtmias (sinus node dysfunction, aflutter, SVT) Thromboembolism Baffle obstruction causing superior or inferior vena cava syndrome Liver cirrhosis
165
Right atrial enlargement on ECG
Peaked p wave in lead II and V1(>2.5mm)
166
Left atrial enlargement on ECG
P wave in lead II is bifid and >120 ms OR negative p in V1 (>1mm wide and deep)
167
RVH on ECG
R in V1 and/or S in V6 more than upper limit of normal range for age RAD
168
LVH on ECG
S in V1 and/or R in V6 more than upper limit of normal range for age LAD
169
RBBB on ECG
RSR’ in V1 | Slurred S in V6
170
LBBB on ECG
RSR’ in V6 | Slurred S in V1
171
ECG findings in Hyperkalemia
Peaked T waves Flat P waves Widening of QRS
172
ECG findings in hypokalemia
Flat T wave ST depression Prominent U wave
173
What electrolyte abnormalities can cause prolonged QTc?
Hypocalcemia Hypomagnesemia Hypokalemia
174
What are the 4 requirements for sinus rhythm?
Regular rate and rhythm for age P wave before every QRS complex Normal axis Upright P in lead II
175
What features would be concerning in a patient with PVCs?
Multiform/multifocal Associated with underlying heart disease History of syncope or fhx of sudden death Precipitated by or increased with activity Multiform or couplets Runs of PVCs with symptoms
176
List 5 causes of sinus bradycardia
``` Athletes Increased vagal stimulation Increased ICP Hypothyroidism Electrolyte disturbance (low K) Drug effect ```
177
What rate is consistent with a junctional rhythm?
40-60 bpm
178
What type of second degree heart block is more likely to progress to complete heart block?
Mobitz Type 2 (PR Interval is constant with abrupt failure of conduction of P wave)
179
Cause of Second degree AV block Mobitz I (Wenkebach)
Advanced vagal tone | Seen often in athletes
180
Causes of second degree AV block Mobitz 2
Aging degeneration of conduction system | Medications that block AV node (digoxin, betablockers, CCBs)
181
List causes of complete heart block
Congenital: - Anti-ro/la antibodies - cc-TGA, atrial isomerism - Long QT Acquired: • Cardiac surgery • Infectious: Rheumatic fever, myocarditis, Chagas disease
182
When is ventricular pacing indicated in CHB?
Symptomatic Ventricular pauses ≥3 seconds Resting HR <40 beats/min
183
What monitoring and treatment is required for mother's with anti-Ro/anti-La antibodies
Regular fetal echos from 16 weeks GA until delivery Can give IVIg or steroids to mom's (mixed evidence)
184
What is the ventricular rate in complete heart block?
40-60 bpm
185
What are two things newborns with CHB are at risk for in the future?
Arrhythmia | Sudden Death
186
With atrial fibrillation and atrial flutter, how high can the atrial rate get?
300 bpm
187
What are the two main mechanisms of SVT?
AVNRT - More common after 2 years of age - 2 conducting pathways in the AV Node (fast and slow) AVRT - More common in infants - Extra-nodal accessory pathway (e.g. WPW)
188
List clues that tachycardia is due to SVT
``` Absent/retrograde P wave No HR variation QRS narrow HR>180 in children HR>220 in infants ```
189
Management of SVT
1. Vagal maneuvers (ice to face, valsava, blow into straw, bear down) 2. Adenosine - 0.1 mg/kg, then 0.2 mg/kg 3. Synchronized cardioversion (if unstable) 4. Consider amiodarone
190
ECG findings in WPW
Delta wave Wide QRS Short PR
191
What two structural abnormalities of heart can be associated with WPW?
Ebstein anomaly | Congenital corrected TGA
192
List causes of ventricular tachycardia
``` Myocarditis Cardiomyopathy Myocardial Infarction Cardiac Tumours Long QT Syndrome WPW Syndrome Congenital Heart Disease Mitral Valve Prolapse Trauma Metabolic: hyoxia, acidosis, hypocalcemia, hypoglycemia, hypo/hyperkalemia Toxins ```
193
Treatment for torsades
IV MgSO4
194
Definitive treatment of WPW
Catheter ablation of accessory pathway
195
Causes of syncope
``` Vasovagal Breathholding spell Orthostatic hypotension Hypoglycemia Cardiac • Long QT Syndrome • SVT • Pre-excitation Syndrome (WPW) • V Tach • Hypertrophic and Dilated Cardiomyopathy • Vavular defects • Pulmonary HTN • Myocarditis ```
196
List syncope mimics
``` Seizures Migraine syndromes Hyperventilation Conversion Disorders Narcolepsy ```
197
What features of syncope on history would be suggestive of a cardiac etiology?
During exertion Triggered by startle or loud noise-long QT No prodrome Palpitations Chest pain Family history of SCD, arrythmia Hx of CHD/acquired heart disease or arrythmia
198
What features should you look for on ECG in a patient with syncope?
``` QT interval Delta wave/pre-excitation Epsilon wave Brugada Heart block ```
199
What are the two clinical phenotypes of congenital long QT syndrome and how are they inherited?
1. Romano Ward Syndrome – AD, purely cardiac phenotype 2. Jervell and Lange-Nielsen Syndrome – AR, associated with congenital sensorineural deafness and a more severe clinical course
200
Components of Long QT syndrome score
``` 1. ECG findings (in the absence of medications or disorders known to affect these features) o QTc (= QT/√RR) ≥480 msec: 3 points 460 to 470 msec: 2 points >450 to 460 msec (in males): 1 point o QTc at fourth minute of recovery from exercise stress test ≥480 ms: 1 point o Torsades de pointes: 2 points o T-wave alternans: 1 point o Notched T wave in three leads: 1 point o Resting heart rate below second percentile for age (restricted to children): 0.5 point ``` 2. Clinical findings o Syncope With stress: 2 points Without stress: 1 point 3. Family history (The same family member cannot be counted in both of these criteria) o Family members with LQTS: 1 point o Unexplained sudden cardiac death in immediate family members <30 years of age: 0.5 point
201
Diagnostic work up for long QT syndrome
ECG Exercise test Genetic testing
202
Treatment for Long QT syndrome
Beta blockers | In those that don't respond, ICD
203
Which type of Long QT syndrome does not respond to beta blockers?
LQT3 | Needs ICD!
204
QT prolonging drugs
Antibiotics—macrolides (erythromycin, clarithromycin, telithromycin, azithromycin), clindamycin, septra Antifungal agents—fluconazole, itraconazole, ketoconazole Antiprotozoal agents—pentamidine isethionate Antihistamines—Diphenhydramine Antidepressants—tricyclics such as imipramine (Tofranil), amitriptyline (Elavil),desipramine (Norpramin), and doxepin (Sinequan) Antipsychotics—haloperidol, risperidone, phenothiazines such as thioridazine(Mellaril) and chlorpromazine (Thorazine), carbamezipine Antiarrhythmic agents – Procainamide, amiodarone, sotalol, flecainide Lipid-lowering agents—probucol Antianginals—bepridil Diuretics (through K+ loss)—furosemide (Lasix), ethacrynic acid (bumetanide[Bumex]) Oral hypoglycemic agents—glibenclamide, glyburide Organophosphate insecticides Promotility agents—cisapride Vasodilators—prenylamine Caffeine
205
ECG findings in Brugada syndrome
Coved-ST elevation in right precordial leads and RBBB
206
What is the pathophysiology of Catecholaminergic Polymorphic Ventricular Tachycardia ?
- Type 1 is autosomal dominant associated with a mutation in the ryanodine receptor gene RYR2 at 1q42.1 - Type 2 is autosomal recessive and related to a mutation in the calsequestrin 2 gene CASQ2 at 1p13.3
207
How do you diagnose CPVT?
Polymorphic VT on exercise test
208
What situations trigger VT in CPVT?
Usually after arousal/emotional experiences (catecholamine release)
209
What is the most common type of long QT syndrome?
Romano Ward
210
Criteria for classic Kawasaki Disease
5 days of fever+ 4/5 of: - B/L non-exudative bulbar conjunctival injection - Oral/Mucosal Changes: strawberry tongue, dry/cracked lips, injection of mucosa/pharynx - Polymorphous exanthema (anything but vesicular) - Cervical LAD with nod >1.5cm: nonsuppurative, usually unilateral - Extremity Changes: palmar/plantar erythema or edema
211
List cardiac manifestations of KD other than coronary aneurysms
Myocarditis Pericarditis Mitral regurgitation
212
What percentage of untreated patients with KD develop coronary aneurysms and when?
Coronary artery aneurysms develop in up to 25% of untreated patients in the second to third week of illness Rarely before day 10
213
When should screening echos be done in KD?
``` At diagnosis 2-3 weeks If normal: Repeat at 6-8 weeks Repeat at 1 year with lipid profile ```
214
List risk factors for aneurysms in KD
Age younger than one year or older than nine years Male sex Fever ≥14 days Not treated (with IVIG) Serum sodium concentration <135 mEq/L Hematocrit <35 percent White blood cell count >12,000/mm3
215
With timely treatment, what is the risk of coronary aneurysms in KD?
<5%
216
What is the prognosis of coronary aneurysms in KD?
50% of coronary aneurysms regress in 1 to 2 years | Giant aneurysms unlikely to resolve
217
What is the prognosis of mitral insufficiency in rheumatic heart disease?
Spontaneous improvement usually occurs with time
218
When does mitral stenosis typically appear in rheumatic heart disease?
Usually takes 10 yr or more for the lesion to become fully established
219
What is the prognosis of aortic insufficiency in rheumatic heart disease?
Unlike MI, AI does not regress
220
Cardiac lesions associated with trisomy 22p (cat eye)
TAPVD TOF VSD
221
What constitutes a positive hyperoxic test?
PaO2 <150 after 10 minutes of 100% O2
222
What are the 3 types of duct dependent lesions?
Duct dependent pulmonary circulation Duct dependent systemic circulation Duct dependent mixing (TGA)
223
List acyanotic heart lesions with normal pulmonary blood flow
Pulmonary stenosis CoA AS Mitral stenosis/regurg
224
ECG findings in ASD
RAD | Primum-LAD (like AVSD)
225
Complications of ASD
``` PHTN CVA/emboli Atrial fibrillation Dyspnea Recurrent infections ```
226
Do patients with T21 have increased risk of developing PHTN with same cardiac lesion as a health kid?
YES
227
Treatment of TOF spell
Quiet, calm environment Knee to chest/squatting (increase venous return) O2 (pulmonary vasodilation) Fluid Morphine (treat hyperpnea and systemic catecholamines) Phenylephrine (increase SVR) Propanolol
228
3 unique things about TAPVD
1. Presents with cyanosis severe respiratory distress 2. PGEs don't work 3. No medical therapy works; need surgery right away!
229
Treatment of rheumatic fever
1. PenG x 10 days OR IM penicillin x 1 dose 2. ASA 3. Prednisone for severe carditis
230
What is the classic presentation of Brugada syndrome?
Unexplained nocturnal death
231
ECG changes in digitalis toxicity
``` PVCs Bradycardia Atrial tachyarrhythmias with AV block Ventricular bigeminy, junctional rhythms Bidirectional ventricular tachycardia ``` Chronic digoxin use: - T wave changes (flattening or inversion) - QT interval shortening - Scooped ST segments with ST depression in the lateral leads - Increased amplitude of the U waves
232
Surgical indications for endocarditis
Increase in vegegatation size after 4 weeks of antibiotics Persistent vegetation after systemic embolization Valvular dysfunction (perforation, rupture, AI/MI causing heart failure) New heart block
233
What are the guidelines for secondary prophylaxis in rheumatic fever for the following groups: i) Without carditis ii) With carditis, no valvular disease iii) With carditis, persistent valvular disease
Without Carditis→ 5 years or until 21 years of age, whichever is longer With Carditis, but no valvular disease → 10 years or until 21 years of age, whichever is longer With Carditis, persistent valvular disease → 10 years or until 40 years of age, whichever is longer, sometimes lifelong prophalaxis
234
List 3 options for antiobiotic prophylaxis for ARF
1. IM Benzathine PenG every 4 weeks 2. Pen V oral daily 3. Sulfadiazine daily