Peds Patho - Cardio

Front 1

What’s the first system to develop in the embryo?

Back 1

Cardiovascular

Front 2

When does the heart develop?

Back 2

2nd week of gestation

Front 3

When does blood start circulating?

Back 3

3rd week of gestation

Front 4

What is the fetal circulation flow pattern?

Back 4

Placenta, umbilical vein, liver, ductus venosus, IVC, RA, FO, LA, LV, aorta, coronary circulation, brain

Front 5

What’s the O2 sat of the fetus?

Back 5

About 80%

Front 6

What happens with the first breath?

Back 6

PVR decreases, RA pressure decreases, LA pressure increases, clamping the cord will induce constriction of the ductus venosus with 10 mins, decreased PGE will start to constrict DA, increased LAP will close the FO

Front 7

What’s the most common type of birth defect?

Back 7

Congenital heart disease

Front 8

What are the causes of congenital heart defects?

Back 8

Multi-factorial, genetics, environmental, teratogens, maternal factors, chromosome abnormalities or unknown

Front 9

When are teratogens most harmful?

Back 9

1st semester

Front 10

What is Trisomy 13, 18 and 21 associated with?

Back 10

AVC and VSD defects

Front 11

What is DiGeorge Syndome associated with?

Back 11

Conotruncal abnormalities (TOF)

Front 12

What is Noonan Syndrome associated with?

Back 12

PS, HCM

Front 13

What is Marfan syndrome associated with?

Back 13

Aortic Aneurysm, AR, MR

Front 14

What is Turners Syndrome associated with?

Back 14

Coarctation, AS, ASD

Front 15

What can maternal Lupus cause?

Back 15

CHB

Front 16

What can maternal rubella cause?

Back 16

PDA, PPS

Front 17

What can maternal alcohol cause?

Back 17

ASD/VSD

Front 18

Describe systolic murmurs?

Back 18

Occurs between closing of S1 and S2, can be innocent of pathological, usually created by leaking valves during systole, regurgitation murmurs begin in S1 and last through systole, ejection murmurs begin after S1 and end before S2

Front 19

Describe diastolic murmurs?

Back 19

Occurs between S2 and S1, always pathologic, usually occur during ventricular relaxation and filling, may indicate aortic or pulmonary insufficiency, regurgitation, MV and TV stenosis

Front 20

Why does mid-diastolic rumble occur?

Back 20

Occurs from increased flow across non stenotic valve from volume overload (VSD, ASD)

Front 21

When do continuous murmurs occur?

Back 21

PDA, shunt murmurs and AV fistulas

Front 22

What does RVH show on ECG?

Back 22

Large R wave in V1 and V2

Front 23

What does LVH show on ECG?

Back 23

Tall R wave in V5 and maybe V6

Front 24

What does a Right Bundle Branch look like ECG?

Back 24

Delay in RV depolarization, seen in V1 and V2 “rabbit ears”, QRS greater than 0.12 seconds

Front 25

What does a Left Bundle Branch Block look like?

Back 25

Delay LV depolarization, seen in V5 and V6, QRS greater than 0.12 seconds

Front 26

What does Right Atrial enlargement look like?

Back 26

Initial component of a P wave in 2 and 3 AVF is the larger, usually exceeds 2.5mm

Front 27

What does Left Atrial enlargement look like on ECG?

Back 27

The terminal portion of a diphasic P wave in V1 in large and wide, longer p waves

Front 28

What are the general signs and symptoms of congenital heart disease in infants?

Back 28

Dyspnea, feeding difficulties, failure to thrive, recurrent respiratory infections, murmur, cyanosis, tachycardia, tachypnea, arrhythmia and enlarged liver

Front 29

What are the general signs and symptoms of congenital heart failure in the child?

Back 29

Dyspnea, decreased exercise tolerance, growth failure, syncope, chest pain, cyanosis, clubbing, enlarged liver, arrhythmia and recurrent infections

Front 30

What are the acyanotic lesions with increased pulmonary flow?

Back 30

Patent Ductus Arteriosis, atrial septal defect, artioventricular canal defect

Front 31

When does a PDA usually close?

Back 31

Within 12-24 hours of life

Front 32

What is the magnitude of the shunt in a PDA dependent upon?

Back 32

The diameter and length of the ductus and the level of PVR (low PVR will have more pulmonary flow)

Front 33

What do you see on PE with a PDA?

Back 33

1-4/6 continuous machinery like murmur, heard loudest at LUSB

Front 34

What do you see on CXR with a PDA?

Back 34

Prominent MPA and increased pulmonary vascular resistance

Front 35

What do you see on ECG with a PDA?

Back 35

Sm to med: normal or LVH, large: BVH, tall R waves

Front 36

What do you see on Echo with a PDA?

Back 36

LA and LV dilation and elevated pulmonary artery pressures

Front 37

What are the 3 types of Atrial Septal defects?

Back 37

Primum (at lower septum), sinus venosus (at top of septum) and secundum (forarmen ovale doesn’t close so defect is in middle of septum)

Front 38

What’s the most common type of Atrial Septal defect?

Back 38

Secundum

Front 39

What do you see on PE with a ASD?

Back 39

SEM in pulmonic areas as a result of increased pulmonary blood flow. Widely split S2 as a result of delay in RV emptying, an early mid-diastolic rumble at the LSB

Front 40

What do you see on CXR with an ASD?

Back 40

Enlarged RA and RV with a prominent main PA with increased pulmonary vascularity

Front 41

What do you see on ECG with an ASD?

Back 41

RV hypertrophy, RBBB and RA enlargement

Front 42

What do you see on echo with an ASD?

Back 42

RV volume overload

Front 43

What meds can you give for an ASD?

Back 43

Digoxin, diuretics

Front 44

What are the indications for a surgical closure of an ASD?

Back 44

CHF, dilated RV, failure to thrive and pulmonary hypertension

Front 45

What are the types of VSDs?

Back 45

Perimembraneous (most common), Inlet (canal type near TV) and Muscular ( swiss cheese throughout whole septum)

Front 46

What are the chromosomal abns associated with VSD?

Back 46

Trisomy 13,18 and 21

Front 47

What happens in a VSD?

Back 47

L to R shunting at ventricles, LA dilation, LV volume overload, increased pulmonary flow

Front 48

T or F: Babies will not be pink with a VSD?

Back 48

False will be pink

Front 49

What will you see on PE with a VSD?

Back 49

2-5/6 systolic murmur, harsh, holosystolic at LSB, may have a thrill, mid-diastolic rumble at apex due to increased flow across MV, early diastolic murmur of AI

Front 50

The smaller the VSD the _________ the murmur.

Back 50

Louder

Front 51

What do you see on CXR with a VSD?

Back 51

Cardiomegaly, enlarged LA and LV, prominent MPA and increased pulmonary vascularity

Front 52

What do you see on EKG with a VSD?

Back 52

Sm – normal, med – LVH, LAE and lg – BVH and LAE

Front 53

What do you see on echo with a VSD?

Back 53

LA and LV enlargement

Front 54

What meds are used to tx VSD?

Back 54

Digoxin and diuretics

Front 55

That are the indications to surgically close a VSD?

Back 55

CHF, dilated RV, failure to thrive, pulmonary htn

Front 56

When is a VSD usually closed surgically?

Back 56

1-2 yrs

Front 57

When you essentially have an ASD and a VSD you have?

Back 57

Atrioventricular Septal Defects

Front 58

What do you see on PE with a Atrioventricular Canal?

Back 58

Hyperactive precordium, possible thrill, 3-4/6 holosystolic murmur at apex and mid-diastolic rumble

Front 59

What do you see on CXR with an Atrioventricular Canal?

Back 59

Cardiomegaly with increased PVMs

Front 60

What do you see on EKG with an Atrioventricular Canal?

Back 60

RVH and LVH

Front 61

What do you see on echo with an Atrioventricular Canal?

Back 61

Degree of regurg?

Front 62

T or F: pts with Atrioventricular Canals will not go into heart failure

Back 62

False the definitely will

Front 63

What meds can be used to tx Atrioventricular Canals?

Back 63

Digoxin and diuretics

Front 64

What are the acyanotic lesions that obstruct flow?

Back 64

Coarctation of the aorta, aortic stenosis and pulmonary stenosis

Front 65

What is a Coarct associated with?

Back 65

PDA, bicuspid aortic valve, membraneous VSD and Turners Syndrome

Front 66

What happens with a coarct in the neonatal period?

Back 66

Severe arch obstruction with closure of the PDA, can lead to respiratory distress and cardiogenic shock

Front 67

What can happen in childhood with a Coarct?

Back 67

Can be latent, progressive with growth, most children are asymptomatic with occasional c/o leg pain or weakness with exercise

Front 68

What do you see on PE with a coarct?

Back 68

2-3/6 SEM at LUSB with radiation to left axillary line, BP gradient, decreased in lower extremities, decreased pulses in lower extremities

Front 69

What do you see on CXR with coarct?

Back 69

Cardiomegaly, pulmonary venous congestion, 3 sign formed by aortic knob, rib notching between 4th and 8th

Front 70

How do you tx coarct?

Back 70

Surgical repair or transcatheter balloon angioplasty with stent placement

Front 71

T or F: the risk of re-coarct or aortic anyeurism mandates life long follow up?

Back 71

True

Front 72

What are the 3 types of AS?

Back 72

Valvular (most common), Subvalvular and Supravalvular

Front 73

What is AS associated with?

Back 73

Other left sided cardiac anomalies, abn mital valve, ventricular cavity, subaortic region arch of isthmus area

Front 74

What do you see on PE with AS?

Back 74

Thrill at RUSB, harsh SEM 2-4/6 at RUSB with radiation to neck and apex, possible AI, diastolic murmur and narrow pulse pressure if severe

Front 75

What do you see on CXR with AS?

Back 75

Infants – cardiomegaly with pulmonary edema, older kids – possible cardiomegaly in severe AS left atrial enlargement

Front 76

What do you see on EKG with AS?

Back 76

Mild- normal, mod to severe – LVH

Front 77

What do you see on ECHO with AS?

Back 77

LVH, MR, diastolic function may be impaired

Front 78

How do you manage AS?

Back 78

Balloon valvuloplasty (at birth), aortic valve replacement, Ross procedure

Front 79

What is PS associated with?

Back 79

Hypertrophied RV, normal TV, normal distal pulmonary arteries

Front 80

What is the initial procedure of choice for PS?

Back 80

Balloon valvuloplasty

Front 81

What do you see on PE with PA?

Back 81

Ejection click with valvular PS, varying with respirations, SEM 2-5/6, thrill at LUSB with radiation to back and sides

Front 82

What do you see on CXR with PS?

Back 82

Nrm to decreased PVMs

Front 83

What do you see on EKG with PS?

Back 83

Mild-normal, mod – RAD-RVH and severe – RAE – RVH

Front 84

What do you see on Echo with PS?

Back 84

RVH

Front 85

What are the cyanotic defects with decreased pulmonary blood flow?

Back 85

Tricuspid atresia, tetralogy of fallot and pulmonary atresia

Front 86

What is tricuspid atresia?

Back 86

No opening from the RA into either ventricle

Front 87

In TA, great vessels can be ______ _______ or ________.

Back 87

Normally related or transposed

Front 88

There’s almost always a ______ with TA.

Back 88

ASD

Front 89

What do you see on PE with TA?

Back 89

Single S2, 2-3/6 systolic regurg murmur at LLSB, associated with a VSD

Front 90

What do you see on CXR with TA?

Back 90

Nrm or slightly enlarged heart, may have boot shaped heart

Front 91

What do you see on EKG with TA?

Back 91

Superior axis, RAE

Front 92

What do you see on ECHO with TA?

Back 92

RAE

Front 93

What 4 things will pts have with TOF?

Back 93

1. Pulmonary stenosis, 2. VSD (usually lg and perimembraneous) 3. Aorta which is righward relative to the interventricular septum, 4. Hypertrophy of the RV

Front 94

What do you see on PE with TOF?

Back 94

Loud SEM possibly a thrill at middle and LUSB, loud S2, clubbing and polycythemia in older kids

Front 95

What do you see on CXR with TOF?

Back 95

Boot shaped heart, with normal or decreased PVMs

Front 96

What do you see on EKG in TOF?

Back 96

RAD and RVH

Front 97

What do you see on ECHO in TOF?

Back 97

Size of VSD and degrees of PS

Front 98

What do you see in CBC in TOF?

Back 98

Elevated hematocrit

Front 99

What causes TET spells?

Back 99

Decreased pulmonary flow and increased right to left shunting

Front 100

How do TET spells manifest?

Back 100

Irritability, profound cyanosis, hyperapnea and syncope

Front 101

How to tx TET spells?

Back 101

Oxygen, knee to chest position, morphine, phenylephrine and squatting

Front 102

How do you manage TOF?

Back 102

Meds – Digoxin, enalapril and lasix, Surgery – may do shunting or full repair depending on severity

Front 103

What is pulmonary atresia?

Back 103

Spectrum of anatomic disorders involving the pulmonary valve, tricuspid valve, RV and coronary circulation, essentially there is a large ASD, the ductus arteriosis is the only pulmonary blood flow

Front 104

How to you tx pulmonary atresia?

Back 104

Surgery – Systemic PA shunt, Fontan procedure, heart transplant

Front 105

What are the cyanotic defects with variable pulmonary blood flow?

Back 105

Transposition of the great vessels, double outlet RV, truncus arteriosis and single ventricle

Front 106

What is a L-TGA?

Back 106

A corrected transposition, the TV is on the left and the LV is on the right

Front 107

Which type of TGA is more common?

Back 107

D-TGA

Front 108

What is a D-TGA?

Back 108

Aorta arises anteriorly from the RV, the pulmonary artery arises posteriorly from the LV and origin and distribution of the coronary artery are variable

Front 109

What do you need to survive with TGA?

Back 109

PDA, PFO

Front 110

How do you manage a TGA?

Back 110

PDA patency with PGE, balloon atrial septostomy and atrial switch (at 1 wk of life)

Front 111

What is a truncus arteriosis?

Back 111

Single great vessel gives off the coronary arteries and pulmonary arteries, complete mixing of blood at levels of VSD and single semilunar valve (essentially aorta and pulmonary artery are one)

Front 112

What are the 3 types of total anomalous pulmonary venous connections?

Back 112

1. Supracardiac (PV to vertical vein and then to innominate), 2. Cardiac (PV to RA or coronary sinus), 3. Infracardiac (PV to intraabdominal veins)

Front 113

T or F: In total anomalous pulmonary vein connection bother pulmonary and systemic venous blood returns to RA so there must be right to left shunt?

Back 113

True

Front 114

When does a total anomalous pulmonary vein connection need to be repaired?

Back 114

Immediately

Front 115

What is HLH?

Back 115

Continuum of congenital heart anomalies characterized by underdevelopment of the left heart structures

Front 116

T or F: HLH pts don’t usually get heart transplants?

Back 116

False, very common cause of transplantation

Front 117

What are the clinical features of HLH?

Back 117

Extracardiac malformations, congestive heart failure, cyanosis, cardiovascular collapse, shock, usually dx in utero

Front 118

What are the 3 stages of repairing HLH?

Back 118

Norwood, Bidirectional Glenn and Fontan but will likely need transplant

Front 119

What is a vascular ring?

Back 119

Anomalies of the aortic arch or pulmonary artery and trachea

Front 120

How does a vascular ring present?

Back 120

Respiratory, wheezing, pneumonia, feeding difficulties and GI issues

Front 121

A vascular ring may compress the _______ and _______.

Back 121

Trachea and esophagus

Front 122

What is anomalous Left Coronary Artery?

Back 122

LCA arises from PA when should come from aorta

Front 123

How does ALCAPA present?

Back 123

Asymptomatic until childhood, then chest pain, difficulty breathing, syncope and sudden death

Front 124

How to tx ALCAPA?

Back 124

Reimplant artery surgically

Front 125

What is dilated cardiomyopathy?

Back 125

Dilation of one or both of the ventricles with varied degree of systolic function

Front 126

What’s the most common cause of heart transplant?

Back 126

Dilated cardiomyopathy

Front 127

What do you see on PE with dilated cardiomyopathy?

Back 127

Signs and symptoms of heart failure, JVD, narrowed pulse pressure, SEM from TR/MR and enlarged liver

Front 128

What do you see on EKG with dilated cardiomyopathy?

Back 128

RVH, LVH, BVH and sinus tachycardia

Front 129

What do you see on CXR with dilated cardiomyopathy?

Back 129

Enlarged heart, pulmonary edema

Front 130

What is hypertrophic cardiomyopathy?

Back 130

Genetic disease that leads to asymmetric enlargement of the LV, disease of cardiac sarcomere at contractile apparatus

Front 131

What’s the most common cause of sudden death in children (esp. athletes)?

Back 131

HCM

Front 132

What do you see in the history of HCM?

Back 132

Dyspnea with exertion, chest pain, presyncope, syncope and palpitations

Front 133

What you see on PE with HCM?

Back 133

May be normal, S4, harsh systolic murmur in late systole and holosystolic murmur at apex

Front 134

What do you see on EKG with HCM?

Back 134

LVH, atrial enlargement, ST-T changes including T waves inversions

Front 135

What do you see on ECHO with HCM?

Back 135

Confirms dx

Front 136

What are the primary cardiomyopathy causes of heart failure with structurally nrm heart?

Back 136

Dilated, hypertrophic, restrictive, non-compaction, arrhythmogenic right ventricular dysplasia

Front 137

What are the secondary cardiomyopathies that cause heart failure in structurally normal hearts?

Back 137

Myocarditis, MI, ischemia, arrhythmia, drug and toxin exposure

Front 138

What are non cardiac issues that can cause heart failure?

Back 138

Sepsis and renal failure

Front 139

What are the causes of CHF in kids?

Back 139

Congenital heart defects, cardiomyopathies, cardiovascular surgery, severe anemia, acidosis, myocarditis, tachy arrythmias and brady arrhythmias

Front 140

What are the subtle signs of CHF in infants and children?

Back 140

Irritability, lethargy, poor suck, prolonged feeding, falls asleep with feeds and vomiting

Front 141

What are the clinical signs of CHF?

Back 141

Tachycardia, peripheral vasoconstriction, pale/mottled color, cool extremities, oliguria and hepatosplenomegaly

Front 142

What Meds are used for CHF?

Back 142

Digoxin, Lasix and aldactone