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Peds Cardiology > General Info > Flashcards

General Info Flashcards

1
Q

Most common anatomy of a bicuspid aortic valve?

A
  1. R/L Fusion
  2. R/N Fusion
  3. L/N Fusion
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2
Q

Most common coronary artery pattern in D-TGA?

A

Usual coronary arteries

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3
Q

Most common coronary artery abnormality seen in D-TGA?

A

Circumflex from RCA

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4
Q

Fibrous continuity of which valves seen in majority of ToF?

A

Tricuspid/Aortic

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5
Q

ToF in South American and Southeast Asian patients?

A
  1. Complete absence of infundibular septum
  2. Continuity between aortic/pulmonary valves
  3. Double committed sub-arterial VSD
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6
Q

Mitral arcade?

A

Shortened/absent chordae

Leads to direct insertion of leaflets to papillary muscles

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7
Q

What does kidney release in response to decreased renal perfusion?

A

Renin

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8
Q

What does angiotensin II do?

A

Vasoconstriction

ADH/Vasopressin secretion

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9
Q

Most common aortic arch malformation in asymptomatic patients?

A

Left arch with aberrant right subclavian

  • 0.5% general population
  • Common in trisomy 21
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10
Q

Surgery for DORV with subpulmonary VSD?

A

Arterial switch with VSD closure

-Physiology is like TGA

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11
Q

Most common additional defect seen in ToF?

A

ASD or PFO (80%)

Right aortic arch (25%)

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12
Q

% of ToF with right aortic arch?

A

25%

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13
Q

Most common type of AVSD in trisomy 21?

A

Complete AV canal defect

Rastelli Type A

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14
Q

Most common reason for reoperation following partial or complete AV canal repairs?

A

AVVR (usually left)

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15
Q

LVOTO occurs more commonly after which type of AVCD repair?

A

Partial

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16
Q

Whale tail?

A

TAPVR to CS

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17
Q

Primary heart field cells become what cardiac structures?

A

LV and bilateral atria

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18
Q

Secondary heart field cells become what cardiac structures?

A

Majority of RV and conotruncal outflow tracts

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19
Q

Migration of neural crest cells into developing heart causes what embryologic process?

A

Septation of conotruncal outflows into aorta and pulmonary arteries

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20
Q

What fetal remnant delivers blood from hepatic veins to RA?

A

Ductus venosus/Ligamentum venosus

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21
Q

What is position of sinus venosus ASD relative to fossa ovalis?

A

Superior/Posterior

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22
Q

Where is sinus node found in left sided juxtaposition of the atrial appendages?

A

Anterior or Inferior

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23
Q

Left juxtaposition of the atrial appendages is associated with that types of cardiac defects?

A

Abnormal ventriculo-arterial connections

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24
Q

Where is sinus node found in right juxtraposition of the atrial appendages?

A

Normal

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25
Q

What types of lesions are seen in right juxtaposition of the atrial appendages

A

ASD, VSD, PDA (simple lesions)

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26
Q

What structure most likely damaged in patient with stridor following coarctation repair or surgical PDA ligation?

A

Left recurrent laryngeal nerve

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27
Q

Most common additional defect seen in HLHS and what % of patients?

A

Coarctation

80%

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28
Q

What shifts Hgb-O2 dissociation curve left?

A

Increased pH (Decreased H+)
Decreased CO2
Decreased 2,3-DPG
Decreased temp

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29
Q

What shifts Hgb-O2 dissociation curve right?

A 
Exercise
Decreased pH (Increased H+)
Increased CO2
Increased temperature
Increased 2,3-DPG
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30
Q

Ca stored where?

A

Sarcoplasmic reticulum

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31
Q

Most common morphology of truncal valve

A

Tricuspid

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32
Q

Most common abnormal morphology of truncal valve

A

Quadricuspid (20%)

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33
Q

Truncal valve is most commonly in continuity with what other valve?

A

Mitral

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34
Q

Best anatomical hallmark of LA?

A

Valve of fossa ovalis (septum primum)

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35
Q

Triangle of Koch

A

Tricuspid valve annulus (septal leaflet)
CS ostium
Tendon of Todaro

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36
Q

Most reliable anatomical feature that distinguishes RV?

A

Level of insertion of AV valve

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37
Q

Which aortic arch do the PAs and ductus come from?

A

Left 6th

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38
Q

Majority of true aortic arch comes from which arch?

A

Left 4th

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39
Q

Which arch gives rise to proximal right subclavian?

A

Right 4th

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40
Q

Which coronary supplies posteromedial papillary muscle?

A

Right

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41
Q

Anterolateral papillary muscle is supplied by which coronary artery?

A

LAD and Circumflex

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42
Q

Fetal circulation, highest O2 content?

A

Umbilical vein (blood from Mom)

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43
Q

Fetal circulation, lowest O2 content?

A

Umbilical arteries

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44
Q

Ligament of Marshall

A

Remnant of left SVC

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45
Q

What are distinguishing features of RA?

A

Limbus of fossa ovalis
Connection of IVC
Large pyramidal appendage

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46
Q

What is Libman-Sacks endocarditis?

A

Non-infective endocarditis associated with SLE affecting mitral valve

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47
Q

Normal location of sinus node?

A

Subepicardial

Junction of SVC and crista terminalis

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48
Q

Blood supply to sinus node?

A

RCA (60%)

Circumflex (40%)

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49
Q

AV node blood supple

A

Dominant coronary artery- 80% RCA

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50
Q

Where is conduction system in inlet VSD?

A

Anterior/superior to defect

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51
Q

Gradient indicative for pulmonary balloon valvulplasty?

A

> 40mmHg (cath or echo)

-No gradient criteria is significant symptoms

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52
Q

Balloon size for pulmonary valvuloplasty

A

120-140%

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53
Q

Gradient indicative for aortic valvuloplasty?

A
  • AS with decreased LV function regardless of gradient
  • Cath peak >50
  • Cath peak >40 with symptoms
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54
Q

Balloon size for aortic valvuloplasty?

A

80-90%

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55
Q

Goal gradient for aortic valvuloplasty?

A

<25-35mmHg by cath

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56
Q

Catheter based gradient indicative for recoarctation angioplasty?

A
  • > 20mmHg

- <20 mmHg is significant collaterals, single ventricle, ventricular dysfunction

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57
Q

Balloon size for recoarctation?

A

-2-3 times stenosis, but <1mm larger than smallest proximal/distal vessel

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58
Q

Gradient indicative of need for branch PA angioplasty?

A
  • > 20-30mmHg
  • RVP > 1/2 to 2/3 systemic
  • Flow discrepancy
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59
Q

Balloon size for branch PA angioplasty?

A
  • 3-4 times narrowest segment

- 15-20% restenosis rate

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60
Q

Normal location of AV node?

A

Subendocardial

Triangle of Koch

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61
Q

What population are sub-arterial VSDs most commonly seen in?

A

Asian

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62
Q

Which aortic cusp is most likely cause of AI in perimembranous and subarterial/supracristal VSDs?

A

Right

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63
Q

What type of VSD is a overriding AV always associated with?

A

Malalignment

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64
Q

Abdominal features of asplenia?

A
  1. Midline liver
  2. 2 Mirror image right lung lobes
  3. Patent biliary tree with single gallbladder
  4. Malrotation common
  5. Aorta/IVC on same side of vertebral column
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65
Q

Abdominal features of polysplenia?

A
  1. Visceral situs varies
  2. Single gallbladder, but biliary atresia common
  3. Interrupted IVC with azygous continuation
  4. Multiple spleens on same side
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66
Q

Most common aortic-pulmonary position in DORV?

A

Side by side

Aorta to right

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67
Q

Crescent shaped valve at ostium of CS?

A

Thebesian valve

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68
Q

What is Chiari network?

A

Weblike structure from enlarged/persistent Eustachian and/or Thebesian valve or remnant of the valve of the sinus venosis

Extends from crista terminalis to Eustacian/Thebesian valve

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69
Q

Coronary artery features seen in truncus?

A
  • Left coronary system arises from posterolateral suface
  • LAD usually small and displaced leftward
  • RCA from anterolateral surface
  • Conal branch of RCA usually large
  • Left dominance in 25-30%
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70
Q

What coronary branch determines dominance?

A

Posterior descending

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71
Q

What % of population is right dominant v. left dominant v. co dominant for coronary arteres?

A

Right: 70%
Left: 10%
Co: 20%

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72
Q

Most common coronary artery anomaly?

A
  • Left circumflex from right main (no clinical significance)

- Left main from right sinus can be problematic if tracks between aorta and RVOT

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73
Q

Vertical vein embryology?

A

Connection between splanchnic plexus of lung buds and cardinal veins
-NOT a left SVC… this is more posterior than left SVC

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74
Q

Anterior indentation on barium esophagram?

A

Pulmonary artery sling

  • Tracks between trachea and esophagus
  • Associated with tracheal stenosis
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75
Q

CHD in Williams Syndrome?

A
  • Supravalvar AS and PS
  • Coronary stenosis
  • Renal artery stenosis - Leads to HTN
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76
Q

Most common association with right aortic arch?

A

ToF

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77
Q

Gene in Alagille?

A

JAG-1

-Liver disease, right heart disease (pulmonary stenosis, both valvar and branches)

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78
Q

NOTCH 1 mutation?

A

Aortic valve pathology

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79
Q

PTPN11 mutation?

A

LEOPARD Syndrome

Noonan Syndrome

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80
Q

TBX1 mutation?

A

22q11 or DiGeorge

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81
Q

Features of DiGeorge?

A 
Hypocalcemia
Immunodeficiency
IAA type B
Truncus
ToF
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82
Q

TBX5 mutation?

A

Holt-Oram

  • Limb anomalies (missing thumb)
  • Heart defects (ASD)
  • 75% have heart defects
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83
Q

How many papillary muscles with a classic parachute mitral valve versus most common type of parachute mitral valve?

A

1 with classic

2 with most common (asymmetric chordal attachments to one of the papillary muscles)

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84
Q

Features of sinus of valsalva aneurysms?

A
  1. Mostly male
  2. 2/3 right sinus
  3. Associated with outlet VSD
  4. Right sinus to RV rupture most common
  5. Non-coronary to RA rupture second most common
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85
Q

Posterior indentation on esophagram?

A

Vascular ring

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86
Q

Restrictive cardiomyopathy?

A
  • Reduced ventricular volumes
  • Preserved ventricular function
  • Normal wall thickness
  • Dilated atria
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87
Q

What anesthetic to avoid in muscular dystrophy?

A

Succinylcholine

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88
Q

What post-op RV pressure should a VD be reopened following RV-PA conduit in PA/VSD?

A

> 70%

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89
Q

Most common cause of vascular ring?

A

Double aortic arch

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90
Q

2nd most common cause of vascular ring?

A

Right arch with diverticulum of Kommerrell

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91
Q

Embryonic origin of right arch with left sided diverticulum of Kommerell?

A
  • Right dorsal aorta
  • No left 4th
  • Left 6th persists
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92
Q

Which aortic arches disappear?

A

1, 2, 5

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93
Q

Embryology of IAA?

A

Absence of both 4th arches

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94
Q

Most common arch anomaly?

A
  • Left arch with retroesophageal right subclavian (aberrant right subclavian)
  • 38% of Trisomy 21 patients
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95
Q

Embryonic origin of left aortic arch with retroesophageal right subclavian?

A

Dissolution of right 4th and right 6th arches

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96
Q

Embryonic origin of left aortic arch with diverticulum of Kommerell?

A

Dissolution of right 4th and persistence of right 6th arches

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97
Q

Embryologic origin of right arch with mirror image branching?

A
  • No left dorsal aorta or right 6th

- Persistence of left 6th and left 4th

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98
Q

Most common arrhythmia in restrictive cardiomyopathy?

A
  • Atrial flutter

- 2nd and 3rd degree heart block

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99
Q

Describe Barth syndrome?

A
  • X-linked
  • Hypotonia, neutropenia, dilated cardiomyopathy
  • Diagnosed by elevated 3-methylglutaconic acid in urine
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100
Q

CS is derived from what?

A

Left common cardinal vein

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101
Q

Left SVC is derived from?

A
  • Persistence of left sinus horn

- Left anterior cardinal and left common cardinal veins

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102
Q

Right SVC is derived from?

A
  • Right anterior cardinal vein

- Right common cardinal vein

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103
Q

IVC is derived from?

A

Subcardinal and supracardinal veins

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104
Q

Which end of the PDA are vegetation seen with in endocarditis?

A

Pulmonary end

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105
Q

RF for conotruncal defects?

A
  1. Diabetes
  2. Maternal PKU
  3. Retinoic acid
  4. Trimethadione
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106
Q

What is the primary management for mild and moderate MR?

A

Medical: Diuretics and afterload reduction

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107
Q

What echo and ECG findings are seen in cor triatriatum?

A

RA and RV enlargement

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108
Q

Describe Scimitar Syndrome

A
  • Anomalous connection of right pulmonary veins to IVC

- Usually have right lung hypoplasia

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109
Q

What screening is recommended for 1st degree relatives of HCM patients?

A
  • ECHO every 12-18 months starting at age 12

- ECHO every 5 years after 21 if no hypertrophy is seen

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110
Q

Best imaging modality for partial pericardial defect?

A

MRI

  • Absence of portion of pericardium
  • 80% left sided
  • Heart displaced to left
  • Bulge of great vessels
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111
Q

Which aortic valve cusps most commonly prolapses with VSDs

A

Right (followed by non)

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112
Q

Classic ECG findings with Duchenne muscular dystrophy?

A

Deep Q in I, aVL, V5, V6

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113
Q

What is mitral arcade?

A

Shortened or absent chordae which leads to stenosis and insufficiency

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114
Q

Uhl’s anomaly?

A
  • Complete absence of RV myocardium with marked RV dilation (parchment heart)
  • Large P waves with diminished RV voltages
  • Cyanosis
  • Normal endocardial potentials (differs from Ebstein’s)
  • Similar pressure wave contours form RA/RV/PA
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115
Q

Where is AV node in L-TGA?

A

Anterior aspect of atrioventricular ring neat atrial septum

-Normally located in posterior position in subendocardium within triangle of Koch

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116
Q

When is coarctation repair recommended in asymptomatic infants?

A

2-3 years

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117
Q

What is most likely cause of myocarditis/dilated cardiomyopathy in a patient from Latin or South America?

A

Chagas disease
-Trypanosoma Cruzi

-Otherwise viral or coxsackie B is most common

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118
Q

Most common cause of bacterial pericarditis?

A
  1. S. Aureus
  2. H. Influenzae
  3. S. Penumonia
  • Fluid will be predominantly neutrophils
  • Most likely due to hematogenous dissemination from lung
  • Very ill at presentation
  • Need Abx + Drainage
  • Abx: Nafcillin/Oxacillin or Vanc + 3rd gen cephalosporin
  • Constrictive pericarditis is a more common complication with bacterial compared to viral
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119
Q

Most common cause of pericarditis?

A

-Viral: Coxsackievirus

  • Fluid is predominantly lymphocytes
  • NSAIDs for treatment
  • Constrictive pericarditis is rare
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120
Q

S2 changes with varying degrees of AS?

A
  • Mild-Mod: Normal splitting

- Severe: Single S2 due to prolonged ejection time

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121
Q

Formula for wall stress?

A

Stress = (Pressure * Radius) / Wall Thickness

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122
Q

Poiseulle’s Law of Resistance?

A

(8 * Viscosity * Length) / Radius ^4

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123
Q

Delayed gadolinium is ideal to assess for?

A

-Myocardial perfusion including infarction (hyperenhancement on delayed imaging)

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124
Q

Dark blood imaging good for?

A

Anatomic diagnosis and structure

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125
Q

Bright blood imaging good for?

A

Qualitative and quantitative assessment of ventricles and valves

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126
Q

Most common defects seen in rubella?

A

PS
PDA
VSD

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127
Q

Which type of IAA is most commonly seen in AP septation defects like AP window?

A

A (1/3 of IAA

-Distal to left subclavian

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128
Q

Which type of IAA is most commonly seen in DiGeorge?

A

B

-Between left subclavian and left carotid

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129
Q

Heart defect associated with WPW?

A
  • Ebstein’s

- LAD and WPW suggests Ebstein’s

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130
Q

What BP is seen with supravalvar AS?

A

Higher BP in right arm compared to left (due to Coanda effect)

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131
Q

Which genetic syndromes are associated with PAPVR?

A

Turner

Noonan

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132
Q

What defects are most commonly seen in Noonan?

A
  • PS
  • Hypertrophic cardiomyopathy
  • PTPN11 gene mutation
  • 80% have CHD
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133
Q

Which defect is most commonly associated with cc-TGA?

A
  • VSD (80%)

- LVOTO (subpulmonary) (30-50%)

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134
Q

Most common cause of viral myocarditis?

A

Coxsackie

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135
Q

AR murmur heard best in what position?

A

Patient leaning forward with breath held after exhalation

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136
Q

What position is MR best heard from rheumatic fever?

A
  • Left lateral decubitus

- Holosystolic, apical, radiates to the left axilla

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137
Q

What genetic syndrome is associated with congenital polyvalvular dysplasia?

A

Trisomy 18

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138
Q

High pitched click immediately after S1 at the apex?

A

BAV

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139
Q

Down sloping ST segments in V1 and V2 are characteristic of what?

A

Brugada

  • Na channelopathy
  • Loss of function SCN5A mutation
  • Cove type ST elevation in V1-V3
  • Unmasked with class 1 Na channel blockers (flecainide, procainamide)
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140
Q

Auscultation findings with worsening PS?

A

Murmur peaks later in systole and split widens (P2 is delayed)

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141
Q

Most common cardiac tumor in children?

A

Rhabdomyomas

  • Well circumscribed, noncapsulated, ventricular
  • Benign, spontaneous resolution
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142
Q

What defects are highly associated with NEC?

A

HLHS, Truncus

143
Q

Most common major manifestation of RF?

A

Migratory polyarthritis
Carditis
Chorea

144
Q

Myxoma?

A
  • Pedunculated
  • Friable
  • Most commonly in LA
  • Classic Triad: Obstruction, embolism, systemic illness
145
Q

Systemic venous baffle obstruction more common after?

A

Mustard

146
Q

Most common CHD in Cri-Du-Chat?

A

VSD

5p Deletion

147
Q

Recoarctation rate for infants undergoing angioplasty for native coarctation

A

60-65%

148
Q

Ellis-can Creveld?

A
  • Post axial polydactyly
  • Dwarfism
  • Abnormal fingernails
  • Amish
  • Large ASD/common atrium
149
Q

Max total dose of non-ionic contrast during cath?

A

6mL/kg

150
Q

Lung segment with highest blood flow?

A

RLL

151
Q

Which systemic vein has the highest O2 saturation?

A

Renal

152
Q

Which systemic vein has the lowest O2 saturation?

A

CS

153
Q

Equation for FS?

A

(EDD-ESD)/EDD

-FS maximal during 1st month

154
Q

Causes of holodiastolic flow reversal in aorta?

A
  • Large PDA
  • Severe AI
  • Systemic to pulmonary shunt
  • Large AV fistual
155
Q

Anatomic hallmarks of complete AVSD?

A
  • Cleft in anterior leaflet of left AV valve
  • AV valves at same level
  • Left papillary muscles rotated laterally
156
Q

Anatomic hallmarks of RA?

A
  • Broad based appendage

- Connections of the IVC and CS

157
Q

Most diagnostic echo finding in pericardial tamponade?

A

Collapse of RV in diastole

158
Q

WOW associated with what CHD?

A

Ebsteins

159
Q

Morphologic features of LV?

A
  • Higher insertion of AV valve
  • Lack of AV valve chordal attachments to septum (septophobic)
  • Smooth walled
  • Elliptical shaped
160
Q

Most common fetal arrhythmia?

A

PAC

161
Q

Normal orientation of the aorta?

A

Right and posterior to PA

162
Q

Typical aorta location in d-TGA?

A

Anterior and right to PA

163
Q

Typical aorta location in L-TGA

A

Anterior and left to PA

164
Q

Typical aorta location in DILV?

A

Left and anterior or PA

165
Q

RIsk of fetal exposure to lithium?

A

Ebstein (1%)

166
Q

Indomethacin exposure?

A

Ductal constriction

167
Q

Heart defect associated with fetal alcohol snydrome?

A

VSD

168
Q

Heart defect associated with isretinoin?

A

d-TGA

-Conotruncal defects

169
Q

Heart defect in fetal hydantoin syndrome from phenytoin?

A

Coarctation and LVOTO

170
Q

Most common heart defect in diabetic pregnancies?

A

VSD
TGA
Truncus
ToF

171
Q

Heart defect with ACEi use?

A

Septal defects

172
Q

Heart defect with SSRI use?

A

Septal defects

PPHTN

173
Q

Tei index

A

(Isovolumetric contraction time + Isovolumetric relaxation time) / Ejection time

174
Q

Most common complication of subpulmonic VSD?

A

AR from prolapse of right cusp

175
Q

Most common type of VSD seen with coarctation?

A

Perimembranous

176
Q

Treatment of choice for fetal tachycardia with evidence of hydrops?

A

Sotalol 80mg PO BID

177
Q

Most common associated lesion in DORV?

A

PS or PA

178
Q

Valsalva does what to venous return?

A

Decreases

179
Q

Valsalva does what to a Stills murmur?

A

Softer (decreased venous return)

180
Q

AAIR pacing is indicated for?

A

Sinus node dysfunction

181
Q

Hyperthyroid patients are prone to which cardiac arrhythmia?

A

A-fib

182
Q

Chagas disease causes what rhythm disturbance?

A

AV block

183
Q

ECG changes in hyperkalemia?

A
  1. Peaked T waves
  2. Widened QRS
  3. Flat P waves
  4. VF or cardiac standstill is not treated
184
Q

Valsalva does what to HOCM murmur?

A

Louder (decreased venous return)

185
Q

Load independent method for assessing LV systolic function

A

Stress-velocity index

186
Q

Treatment for neonatal atrial flutter?

A

Cardioversion

187
Q

What HR is in an indication for pacemaker in absence of CHD?

A

< 50

188
Q

M band is composed of?

A

Myosin only

189
Q

I ban consists of

A

Actin
Troponin
Tropomyosin

190
Q

What 2 myocyte proteins prevent actin and myosin interaction?

A

Troponin I

Tropomyosin

191
Q

What HR is an indication for pacemaker in presence of CHD?

A

< 70

192
Q

Kearns Sayre Syndrome?

A
  • Chronic opthalmoplegia

- Heart block (pacemaker indicated with bifasicicular block)

193
Q

A band consists of?

A

Actin and myosin (all filaments = A band)

194
Q

Treatment for Torsades de Pointes

A

IV Mg Sulfate

195
Q

Pompe’s disease?

A
  • HCM
  • Short PR interval
  • Glycogen storage disease
  • Hepatomegaly
196
Q

Typical ECG pattern seen in CPVT? Hos is it treated?

A
  • Normal at rest
  • Bidirectional VT with exercise or emotion
  • Treat with beta blockers
197
Q

What does amiodarone do the effect of warfarn, digoxin, cyclosporine and phentyoin?

A

Increases effect (will need to decrease dose)

198
Q

Which immunotherapy is associated with the development of diabetes?

A

Tacrolimus

199
Q

Amiodarone can have what effect on fetus?

A

Neonatal hypothyroidism

200
Q

WPW most commonly seen what 2 forms of CHD?

A

Ebstein
L-TGA
(Also HOCM)

201
Q

Brugada classic ECD findings and treatment?

A
  • ST elevation in V1/V2
  • T waves deeply coved negative
  • Caused by congenital defect of Na Channels (SCN-5A)
  • Causes young healthy people to develop VF, potential for death
  • Procainamide challenge used for diagnosis
  • Treatment = Defibrillator
202
Q

Lyme carditis causes what conduction distrubance?

A

AV nodal conduction abnormalities (heart block)

-Treatment: Ceftriaxone, doxycycline, amoxicillin

203
Q

Position of displaced conduction system in AV canal?

A

Posterior and inferior to VSD

204
Q

What does a short HV interval (<25ms) indicate?

A

Accessory pathway to ventricles

205
Q

LQTS1 typically presents during what?

A

Exercise

206
Q

LQTS1 is caused by what mutation?

A

KCNQ1

207
Q

Most likely diagnosis for tachycardias with RP longer than PR

A
  • Atypical AVNRT
  • PJRT (AVRT via slowly conducting accessory pathway
  • Atrial tachycardia
  • Atrial flutter
208
Q

Short RP regular narrow complex tachycardia?

A
  1. Typical AVNRT
  2. Orthodromic SVT
  3. Atrial tachycardia with AV block
  4. Junctional tachycardia with 1:1 retrograde activation
209
Q

Contradictions to exercise testing?

A
  • Severe AS
  • Recent MI or RF
  • Severe pulmonary hypertension
210
Q

Chief determinant of symptoms in primum ASD?

A

Degree of left AVVR

211
Q

Risk of IE coincides with what abnormality of a valve?

A

Degree of regurgitation (not structure)

212
Q

Paradoxical emboli are most common with what type of ASD?

A

Secundum (preferential flow from IVC goes towards mid-septum

213
Q

What is the incidence of CHD in patients with Trisomy 21?

A

50% of patients with Down Syndrome have CHD (most commonly ACSD - 2/3)

214
Q

Lab criteria for Kawasaki?

A
  • Albumin < or equal to 3g/dL
  • Anemia for age
  • Plts > or equal to 450,000/mm
  • WBC > or equal to 15,000/mm
  • Elevation of ALT
  • Sterile pyuria > or equal to 10 WBCs/hpf
215
Q

AHA IE guidelines?

A
  • Prosthetic valve
  • Prosthetic material used for valve repair
  • History of IE
  • Completely repaired CHD with prosthetic material or device for 6 months
  • Residual defect at side of prosthetic device
  • Valvulopathy in transplanted heart
  • Unrepaired cyanotic CHD
  • NOT needed for GI/GU procedures
216
Q

Most common organism in endocarditis?

A

Alpha hemolytic strep (S. Viridans)

217
Q

Second most common cause of endocarditis?

A

S. Aureus

218
Q

PCN allergic IE prophylacis?

A

Cephalexin, clindamycin, azithromycin or clarithromycin

219
Q

Most common cause of sudden death in athletes?

A

HCM

220
Q

Marfan Gene?

A

FBN1

221
Q

Loeys-Dietz gene?

A
  • AD
  • TGF Beta 1 or 2
  • Aterial tortuosity, hypertelorism, bifid uvula, clef palate, craniosynostosis, aortic dilation/dissection, pectus excavatum/carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus

-LDS2: Velety smooth, translusent skin, overlaps of LDS1

222
Q

Ehlers Danlos Gene?

A

COL1A1/A2

-Loose joints, chronic join pain, early onset arthrtiis

223
Q

ECG of ALCAPA?

A

Deep, wide Q waves in I/aVL/V3-V6

224
Q

High pitched early diastolic murmur?

A

AI

225
Q

Early low pitched decrescendo diastolic murmur?

A

PI

226
Q

Diastolic, mid to late peaking, low-pitched murmur

A

MS or TS

227
Q

Gold standard for diagnosis and classification of myocarditis

A

Endomyocardial biopsy

228
Q

Jones Criteria?

A

Major:

  • Joints: Migratory polyarthritis
  • Carditis: New-onset murmur (pericarditis)
  • Nodules: Subcutaneous
  • Erythema marginatum (red ring on trunk and extremity)
  • Sydenham chorea

Minor: Prolonged PR, elevated ESR/CRP/Temp, arthralgias

229
Q

Most common symptoms in rheumatic heart disease?

A

Migratory polyarthritis

230
Q

BP does what with inspiration and expiration?

A
  • Decreases with inspiration
  • Increases with expiration

-Pulsus Paradoxus: Exaggerated change in BP with inspiration (tamponade)

231
Q

Describe MR seen in rheumatic heart disease?

A

Posterolateral direction of jet

Prolapse of anterior leaflet

232
Q

PVR is lowest at what lung volume?

A

Functional residual capacity

233
Q

Which VSD types are highest risk for post-op heart block?

A

Canal

Perimembranous

234
Q

MOA of heparin?

A

Potentiates effects of anti-thrombin III (inhibits thrombin formation and prevents conversion of fibrinogen to fibrin)

235
Q

MOA of streptokinase?

A

Converts plasminogen to plasmin (which then cleaves fibrin)

This is a “clot buster”

236
Q

MOA of tPA?

A

Binds to fibrin converting plasminogen to plasmin

237
Q

MOA for enoxaparin?

A

LMWH- Catalyzes ATIII formation and activation

238
Q

Drug of choice for suicidal RV after pulmonary valvotomy?

A

Beta blockers

239
Q

Cyclosporine and tacrolimus mechanism?

A

Inhibit calcineurin,

Decrease T-call activation

240
Q

Major side effect of calcineurin inhibitors?

A

Nephrotoxicity
HTN
Gingival hyperplasia
Hirsuitism

241
Q

Most common lipoprotein disorder in childhood?

A

Familial combined hyperlipidemia

242
Q

MOA of sirolimus?

A

Antiproliferative

Decrease T cell production

243
Q

Anti-coagulation following mechanical mitral valve?

A

Warfarin (2.5-3.5) and ASA

244
Q

Anticoagulation after AVR?

A
  • Mechanical: Coumadin for INR 2-3 + low dose ASA
  • If RF, increase INR to 2.5-3.5
  • Bioprosthetic: ASA 75-100mg
  • If RF, coumadin for INR 2-3
245
Q

Most likely rhythm with an irregularly irregular wide complex rhythm in an otherwise health person?

A
  • Pre-excited a-fib
  • Avoid AV blocking agents (digoxin, Ca channel blockers, adenosine)
  • Cardioversion is best option, amiodarone if medication used
246
Q

Treatment of choice for CPVT VT/VF storm?

A

IV beta blocker

247
Q

What type of AV block can be seen with long QT?

A

2:1 AV block

248
Q

Most common indication for reoperation in truncus arteriosus?

A

RV-PA conduit failure

249
Q

Where does the AV node course in membranous VSD?

A

Posterior-inferior rim

250
Q

Double chambered RV typically seen with what type of VSD?

A
  • Perimembranous

- High association with discrete subaortic stenosis

251
Q

Most common indication for re-operation in partial AVC defect?

A
  • Left AVVR

- LVOTO more common with partial AVCD

252
Q

Deficiency of which rim of the ASD is an indication for surgical repair?

A

Posterior-inferior

-Also other abnormalities which would benefit from intracardiac repair (TR)

253
Q

Which deficient ASD rim is most associated with erosions?

A

Retro-aortic (anterior/superior)

254
Q

What is recommended following ASD device closure?

A

ASA + SBE ppx x6 months

255
Q

What is recommended following PDA device closure?

A

SBE ppx x6 months

256
Q

Conduit size needed for Melody valve?

A

16mm or greater

257
Q

What is the AV node supplied by?

A

RCA

258
Q

Embryological origin of the CS?

A
  1. Left horn of sinus venosus

2. Proximal left common cardinal vein

259
Q

What course of left main from right sinus is associated with sudden death?

A

If it passes between aorta and PA

260
Q

LAD from right sinus is commonly seen in what heart defect?

A

-ToF (usually passes in front of RVOT)

261
Q

Most frequently seen post-operative complications after atrial switch?

A
  • Baffle leaks (10-20%)
  • SVC obstruction (5-10%)
  • IVC obstruction (1%)
262
Q

Most common reason for re-operation following Ross procedure?

A

Pulmonary homograft failure

263
Q

Most common indication for re-operation in complete AVCD?

A

MR

264
Q

Strongest predictor for development of post-op MR following CAVC repair?

A

Post-op severe MR

265
Q

What lab test need regularly checked while taking amiodarone?

A

LFTs, PFTs, TFTs

266
Q

Most common short term complication following arterial switch procedure?

A

Supravalvar PS (LeCompte maneuver reduces incidence)

267
Q

Most common long term complication in repaired Scimitar?

A

PV obstruction

268
Q

Strongest predictor of CVA in Eisenmenger?

A

Microcytosis from Fe deficiency

269
Q

Which syndrome is associated with hypertelorism and bifid uvula?

A

Loeys-DIetz

-Treat with ARBs

270
Q

IPAH is associated with what gene abnormality?

A

BMPR2

271
Q

What lab monitoring should be performed with endothelin antagonist?

A

Liver function

272
Q

Which side is PA absent in ToF v. Truncus?

A 
ToF = Opposite arch
Truncus = Same as arch
273
Q

Most common associated lesions with Ebsteins?

A
  1. ASD
  2. PFO
  3. VSD
274
Q

1st aortic arch gives rise to?

A

Part of maxillary artery (branch of the external carotid)

275
Q

2nd aortic arch gives rise to?

A

Stapedial and hyoid artery (facial arteries)

276
Q

3rd aortic arch gives rise to?

A

Common carotid and proximal part of internal carotid

277
Q

5th aortic arch gives rise to?

A

Nothing- it regresses

278
Q

Phase 0 of AP?

A

Rapid depolarization, Na rapidly into cell, Ca slowly into cell

279
Q

Phase 1 of AP?

A

Repolarization, inactivation of Na channels and opening of few K channels (K out)

280
Q

Phase 2 of AP?

A

Ca in… Repolarization is slowed by influx of Ca via L-type Ca (slow) channels balancing theoutward K current (some Na involved in creating plateau)

281
Q

Phase 3 of AP?

A

K out… Rapid repolarization

282
Q

Phase 4 of AP?

A

Resting potential, K in

283
Q

Where is I-band in sarcomere?

A

Light band, made up of actin and thin filaments

Z: Line in the center

284
Q

Where is A-band in sarcomere?

A

Dark band, made of myosin and thick filaments which may overlap with actin filaments (has both myosin and actin)

285
Q

What is H-zone in sarcomere?

A

Zone of myosin filaments only (no overlap with actin filaments) within the A-band
-The lighter stripe in the center of the A-band

286
Q

Where is the M line in a sarcomere?

A

Middle of the H-band, myosin only

287
Q

Describe T-tubules of sarcomere?

A
  • Envelope the myofibril at the level of the Z disk forming couplings with the SR
  • Allows transmission of teh AP to cellular interior
288
Q

What embryological structure does the SVC arise from

A

Right common cardinal vein and anterior cardinal vein

289
Q

What embryological structure does the outflow tract of the LV arise from?

A

Bulbus cordis

290
Q

What fetal structures have the lowest O2 saturation?

A

CS and SVC

291
Q

Where is the AV node in L-TGA?

A
  • Anterior aspect of AV ring near atrial septum

- Anterior to VSD if present

292
Q

Where is AV node located in CAVC?

A
  • Posterior to VSD

- Inferior to CS

293
Q

Where is AV node located in tricuspid atresia?

A
  • Posterior to VSD

- Floor of RA

294
Q

Where is AV node located in DILV?

A

Anterior to AV ring

295
Q

Hypokalemia changes?

A
  • U wave

- Prolonged QTc

296
Q

Hyperkalemia changes?

A
  • Peaked T-waves
  • Prolonged QRS
  • AV block
297
Q

Characteristics of manhaim tachycardia?

A

Delta wave with normal PR interval

298
Q

Hypokalemia increases arrhythmogenic effects?

A

Digoxin

299
Q

Broad based T waves seen in long QT type?

A

LQT1

300
Q

Low amplitude or notched T waves seen in long QT type?

A

LQT2

301
Q

Long ST segment seen in long QT type?

A

LQT3

302
Q

Exertion or swimming related sudden death?

A

LQT1

303
Q

Auditory stimulation or postpartum sudden death seen in?

A

LQT2

304
Q

Sleep related sudden death seen in?

A

LQT3

305
Q

Beta blockers most effective in LQT…?

A

LQT1

306
Q

Hyperthyroidism can cause what arrhythmia?

A

A-fib

307
Q

What AH jump is associated with dual AV node physiology?

A

> 50msec

308
Q

Short HV interval seen in?

A

Accessory pathways <25-35msec

309
Q

What type of VSD must be there for straddling tricuspid valve and straddling mitral valve?

A

Tricuspid: Inlet

Mitral: Malalinged

310
Q

Short PR interval seen with?

A
  1. WPW
  2. Glycogen storage disease
  3. Normal
311
Q

WPW seen commonly in waht CHD?

A

Ebsteins
L-TGA
HCM

312
Q

Describe absence of a pulmonary artery in relationship to arch sidedness in truncus v. ToF

A
  • Truncus: Same side as arch

- ToF: Opposite side of arch

313
Q

Ductus commonly absent in what CHD?

A
  1. Truncus

2. ToF-Absent pulmonary valve

314
Q

Main reason for reoperation in truncus?

A

Conduit replacement

315
Q

What gradient and RV pressure is considered severe conduit stenosis?

A

> 50mmHg

RVSP >75% systemic

316
Q

ToF with AVSD is seen in what syndrome?

A

Trisomy 21

317
Q

Most common cardiac tumor in children?

A

Rhabdomyoma

318
Q

What cardiac tumor shows delayed enhancement on CMR?

A

FIbroma

319
Q

Single, friable, pedunculated mass within LA? Treatment?

A

Myxoma, Resection

320
Q

Multiple, well circumscribed, echo bright lesions seen within the ventricular myocardium are most likely?

A

Rhabdomyomas

321
Q

What genetic syndrome should be ruled out in the presence of multiple ventricular tumors?

A

Tuberous sclerosis

322
Q

“Spider cells” are found with which cardiac tumor?

A

Rhabdomyomas

323
Q

Single, firm, intramural tumor seen within LV free wall or interventricular septum?

A

Fibroma (usually don’t regress)

324
Q

Cardia myxomas are associated with what?

A

Multiple lentigines syndrome

325
Q

Most frequent malignant primary cardiac tumor?

A

Angiosarcoma - Hemorrhagic effusion seen

326
Q

Coronary artery fistulas are most likely to empty where?

A

RV

327
Q

What septal thickness is associated with sudden death in HOCM

A

> 30mm

328
Q

Cyanosis and dyspnea on exertion in a Fontan when standing upright is concerning for?

A

Pulmonary AVM

329
Q

LAD in newborn raises suspicion for?

A
  • Tricuspid atresia

- AVCD (superior axis)

330
Q

Treatment for mild-mod and severe carditis in acute rhaumatic fever?

A
  • High dose ASA for 4-6 weeks

- Steroids for 2 weeks then taper and add ASA is severe

331
Q

Norepinephrine MOA is predominantly?

A
  • Alpha agonist - Vasoconstriction

- Very little beta

332
Q

Dobutamine MOA?

A
  • Mostly beta 1

- CO increases, SVR decreases

333
Q

Left pressures > Right pressures?

A

Restrictive cardiomyopathy

334
Q

RVSP > 50mmHg

A

Restrictive cardiomyopathy

335
Q

LV pressure = RV pressure?

A

Constrictive cardiomyopathy

336
Q

RVSP < 50mmHg

A

Constrictive cardiomyopathy

337
Q

Pulmonary venous baffle obstruction more common after?

A

Senning

338
Q

Systemic baffle obstruction more common after?

A

Mustard

339
Q

Cyanosis following hybrid procedure most likely caused by?

A

Restrictive atrial septum

340
Q

Most common associated defect with congenital absence of pericardium?

A

ASD

341
Q

Most common VSD in DORV?

A

Subaortic

342
Q

Estimated rate of erosion of ASD devices?

A

1/1000

343
Q

Type 1/Alpha error?

A
  • Incorrectly rejecting the null hypothesis

- Saying there is a difference when there isn’t

344
Q

Type 2/Beta error?

A
  • Incorrectly accepting the null hypothesis

- Saying there is no difference when there is

345
Q

First line therapy for idiopathic pulmonary HTN?

A

Ca channel blockers

346
Q

Doppler gradients for PS?

A

Mild <40
Moderate 40-60
Severe > 60

347
Q

Cath based gradient for coarctation intervention?

A

> 20mmHg

348
Q

Most common associated defect with Ebstein?

A

ASD

349
Q

Second most common associated defect with Ebstein’s

A

PS

350
Q

Clefts in mitral valve are in what leaflet?

A

Anterior

351
Q

Embryology of normal left arch

A
  • Left dorsal, left 4th, left 6th, right 4th

- No right 6th or right dorsal

352
Q

Embryology of right aortic arch with mirror image branching?

A
  • Right dorsal, left 4th, left 6th, right 4th

- No right 6th or left dorsal

353
Q

Embryology of left arch with aberrant right subclavian?

A
  • Left dorsal, left 4th, left 6th

- No right dorsal, right 4th or right 6th

354
Q

Embryology of right aortic arch with aberrant left subclavian/diverticulum of Komerell?

A
  • Right dorsal, right 4th, left 6th

- No left dorsal, left 4th

Peds Cardiology (20 decks)

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