Congenital cardiac associatations prenatal lithium turner syndrome Williams syndrome 22q11 (DiGeorge syndrome)

prenatal lithium- Ebstein anomaly turner syndrome- Bicuspid aortic valve, coarctation of aorta Williams syndrome- Supraclavicular aortic stenosis 22q11 (DiGeorge syndrome)- Truncus arteriosus, TOF

_ventricular septal defect_ what colour are the babies example of a what kind of ASD symptoms epidemiology associated with what

_ventricular septal defect_ pink babies (acyanotic) either a non-restrictive or restrictive CHD depending on size most common CHD Down's syndrome

atrial septal defect what colour are the babies symptoms epidemiology time of presentation

_atrial septal defect_ what colour are the babies pink- acyanotic symptoms typically asymptomatic, sometimes more frequent infections epidemiology 2nd most common acyantic CHD time of presentation mean age of diagnosis is 4.5 years from incidental finding of murmur sympotmatic presentation before the age of 40 years with arrhythmias and dyspnoea

atrial septal defect clinical features investigations management

atrial septal defect clinical features may also have auscultatory findings in infants (asymptomatic) ejection systolic murmur left specific details out investigations pulse oximetry ECHO CXR ECG management most children asymptomatic and require CHF therapy spontaneous closures in small lesions large defects require catheter closure

Congenital heart defects Flashcards by Sam Bradley

The umbilical cord and vein travel within the umbilical cord
what does the umbilical vein transport?
what does the umbilical artery transport?

umbilical vein- nutrition and oxygen to the baby

umbilical artery- waste products and CO2 from the baby

How well did you know this?

Not at all

Perfectly

how many foetal shunts are there

name them and explain where they shunt blood from/ to

3 foetal shunts. they bypass the lungs and liver as these organs aren’t fully functional until after birth

foramen ovale

shunt that bypasses the lungs
moves blood from the R atrium to the L atrium

Ductus arteriosus 

moves blood from the pulmonary artery to the aorta

Ductus venosus

moves oxygenated blood in the umbilical vein (from placenta) to bypass the liver and empty into the vena cava

How well did you know this?

Not at all

Perfectly

transitions at birth due to 2 actions

cord is clamped

causes a cessation of blood flow from the placenta and a reduction in systemic vascular resistance in the baby
this causes an ↑LS heart BP and closure of ductus venosus

baby takes first breath

this causes a huge decrease in pulmonary vascular resistance (lung expansion, collateral tug, decreases in hypoxic pulmonary contriction)
↑ in pulmonary venous return to L atrium and ↑ in R atrial pressure
pressure in L atrium > R atrium
flap closure of foramen ovale in minutes
systemic vascular resistance >pul vasc resistance causes flow to reverse through ductus arteriosus
ductus arteriosus starts to close due to high ppO2 passing through it
over subsequent weeks r vent wall reduces and left vent wall thickens

How well did you know this?

Not at all

Perfectly

what are congenital heart defects

a variety of malformations of the heart and or its major blood vessels that are present at birth

How well did you know this?

Not at all

Perfectly

general symptoms of CHD

lagging physical development
cyanosis, pallor, SOB, anxiety in breast feeding, rejection of the breast
heart murmurs from first days of life
increase in heart size
ECG signs of overload and hypertrophy in different areas (p-pulmonale, p-mitrale, right type of EOS)
radiography- increase in heart size, depletion of enhancement of pulmonary hypertension

How well did you know this?

Not at all

Perfectly

what is the commonest CHD

the majority of cases are picked up

CHDs can be divided into (2)

ventricular septal defect

antenatally

cyanotic and acyanotic CHDs

How well did you know this?

Not at all

Perfectly

Congenital cardiac associatations

alcohol in utero

congenital rubella

Trisomy 21 (Downs)

Mother has DM

Marfan syndrome

alcohol in utero- VSD, ASD, PDA, TOF
congenital rubella- PDA, Pulmonary artery Stenosis, Septal defects
Trisomy 21 (Downs)- ASD, VSD, AVSD
Mother has DM- TGA
Marfan syndrome- MVP, thoracic aortic aneurysm and dissection, aortic regurgitation

How well did you know this?

Not at all

Perfectly

Congenital cardiac associatations

prenatal lithium
turner syndrome
Williams syndrome
22q11 (DiGeorge syndrome)

prenatal lithium- Ebstein anomaly
turner syndrome- Bicuspid aortic valve, coarctation of aorta
Williams syndrome- Supraclavicular aortic stenosis
22q11 (DiGeorge syndrome)- Truncus arteriosus, TOF

How well did you know this?

Not at all

Perfectly

Pink babies have

caused by a shunt from xxx to xxx

pink babies have an acyanotic congenital heart defect
caused by a shunt from left to right (no mixing of oxygenated and deoxygenated blood)

How well did you know this?

Not at all

Perfectly

acyanotic pink baby CHDs can be firgher subdivided into (3)

Restrictive (allowing little flow from left to right)
1. small ASDs/ VSDs and PDA (patent ductus arteriosus)
2. may close spontaneously
Non- restrictive (large defects allow significant left to right flow)
1. moderate- large ASDs, VSDs, AVSDs
Obstructive (severity of lesion decdies age of presentation)
1. aortic stenosis
2. co-arctation of the heart
3. pulmonary stenosis

How well did you know this?

Not at all

Perfectly

ventricular septal defect

what colour are the babies
example of a what kind of ASD
symptoms
epidemiology
associated with what

ventricular septal defect

pink babies (acyanotic)
either a non-restrictive or restrictive CHD depending on size
most common CHD
Down’s syndrome

How well did you know this?

Not at all

Perfectly

ventricular septal defect

time of presentation
clinical findings
investigations

How well did you know this?

Not at all

Perfectly

ventricular septal defects

management

management of ventricular septal defect

small lesions <5mm usually close spontaneously with no repair required
moderate lesion
- diuretic therapy (furosemide and spironolactone)
large lesion
- as per moderate
- schedule for surgery

How well did you know this?

Not at all

Perfectly

atrial septal defect

what colour are the babies
symptoms
epidemiology
time of presentation

atrial septal defect

what colour are the babies
- pink- acyanotic
symptoms
- typically asymptomatic, sometimes more frequent infections
epidemiology
- 2nd most common acyantic CHD
time of presentation
- mean age of diagnosis is 4.5 years from incidental finding of murmur
- sympotmatic presentation before the age of 40 years with arrhythmias and dyspnoea

How well did you know this?

Not at all

Perfectly

atrial septal defect

clinical features
investigations
management

atrial septal defect

clinical features
- may also have auscultatory findings in infants (asymptomatic)
- ejection systolic murmur
- left specific details out
investigations
- pulse oximetry
- ECHO
- CXR
- ECG
management
- most children asymptomatic and require CHF therapy
- spontaneous closures in small lesions
- large defects require catheter closure

How well did you know this?

Not at all

Perfectly

patent ductus arteriosus

example of what kind of defect

colour of baby

symptoms

epidemiology

Study These Flashcards

patent ductus arteriosus

example of what kind of defect
- acyanotic non-restrictive CHD
colour of baby
- pink
symptoms
- depends on size of lesion
- small- asymptomatic
- moderate- CHF with poor feeding, FTT
epidemiology
- 5-10% of all CHD

patent ductus arteriosus

clinical features
investigations
management

Study These Flashcards

patent ductus arteriosus

clinical features
- palpate liver (enlarged in heart failure)
- bounding pulses and wide pulse pressure
- continuous machinery murmur typically in ULSB best heard below left clavicle
investigations
- ECHO and doppler
- CXR and ECG are less useful in daignosis
management
- if preterm- spontaneous closure
- medical- ibuprofen
- surgical- catheter closure or PDA ligation

coarctation of the aorta

colour of baby, type of CHD
pathophysiology
time of presentation
clinical features

Study These Flashcards

pink baby, type of osbtructive CHD

pathophysiology

obstruction to the LV outflow tract leads to an increase in LV afterload which causes LV hypertrophy

time of presentation

symptoms present 3-5 days after birth when the duct begins to close as the PDA and foramen ovale allows blood to bypass the outflow obstruction

clinical features

absent femoral pulses
cold extremities
hepatomegaly

coarctation of the aorta

investigations

management

Study These Flashcards

coarctation of the aorta

investigations
- ECHO and doppler
management
- medical
  - IV infusion of prostaglandin E1 to keep ductus arteriosus open
  - left the details out

Cyanotic CHD babies are what colour

this is because deoxygenated blood enters the?

what are the 5 Ts of cyanotic disease

Study These Flashcards

blue
this is because deoxygenated blood enters the systemic circulation

5T’s of cyanotic disease

Tetralogy of fallot (TOT)
Transposition of great arteries (TGA)
Truncus arteriosus
Total anomalous pulmonary venous drainage
Tricuspid atresia (also HLHS)

quick explanations of the 5 Ts

Study These Flashcards

TOF
TGA- aorta comes off right ventricle, PA comes off left vent (may be associated with ASD or VSD- as they allow 2 new separate systems to mix blood
Truncus arteriosus- pulm artery and aorta haven’t divided- single common overlying blood vessel
Total Anomalous Pulmonary Venous Drainage- pulm veins do not connect to left atrium
1. 3 types described by where the PVs connect (All have ASDs or else not compatible with life)
2. -supracardiac= PVs drain into right atrium
3. -Cardiac= PVs drain into the right atrium through coronary sinus
4. -Infracardiac= PVs drain into RA through hepatic veins and IVC (has highest risk of obstruction)
Tricuspid atresia- tricuspid valve does not form (hypoplastic RV common)
1. -similar defect affects left heart- hypoplastic left heart syndrome (HLHS)
Ebsetin anomaly- displaced tricuspid leaflets, artificially atrializing the RV

tetralogy of fallot is comprised of which 4 anomalies

Study These Flashcards

tetralogy of fallot is comprised of:

Right ventricular outflow obstruction
Ventricular septal defect
Over-riding aorta
Right ventricular hypertrophy

tetralogy of fallot

symptoms

epidemiology

time of presentation

Study These Flashcards

tetralogy of fallot

symptoms
- cyanosis
- poor feeding, sweating during feeds
- severity is variable
epidemiology
- most common form of acyanosis
time of presentation
- during neonatal periods when PDA starts to close
- identification of “tet” spells due to ↑RV to LV shunt due to pul stenosis
- identification of crescendo-decrescendo murmur with a harsh ejection systolic quality

what are the investigations for TOF

ECHO and doppler- assess location and number of VSDs and severeity of RVOTO

ECG- right atrial enlargement and RV hypertrophy (R axis deviation, prominent R waves anteriorly and S waves posteriorly)

CXR

cardiac catheterisation

Study These Flashcards

ECHO and doppler- assess location and number of VSDs and severeity of RVOTO
ECG- right atrial enlargement and RV hypertrophy (R axis deviation, prominent R waves anteriorly and S waves posteriorly)
CXR
cardiac catheterisation

what is a tet spell

These occur due to a reduction in pulmonary blood flow. PBF affected by PVR and SVR due to 2 circulations mixing. * Often precipitated by crying, pooping, feeding, dehydration, fever. * Anything that inc PVR or dec SVR will reduce PBF * Now more deoxy blood enters * Baby goes hyper-cyanotic * Hypoxia inc PVR and makes situation worse! * Need to increase SVR – babies may lift knees to chest, kids may squat

***_Transposition of the great vessels_*** * what kind of defect * pathophysiology * symptoms * time of presentation * clinical findings * investigations * management

* what kind of defect * cyanotic defect * pathophysiology * aorta arises from the RV and the pul artery from the LV, results in deoxygenated blood blood from RV circulating around the body * symptoms * cyanosis, poor feeding, sweating during feeds * time of presentation * neonatal period when ductus arteriosus begins to close * clinical findings * cyanosis, tachypnoea, murmur * investigations * foetal ultrasound, ECHO, CXR, ECG * management * balloon atrial septostomy * arterial switch procedure ![]()

what is total anomalous pulmonary venous drainage to sustain life there must be; where do the pulmonary veins feed into the heart in * supracardiac * cardiac * infracardiac

none of the 4 pulmonary veins feed back into the left atrium but rather into the right side of the heart to sustain life there must be a patent ductus arteriosus * **supracardiac**= PVs drain into right atrium * **Cardiac**= PVs drain into the right atrium through coronary sinus * **Infracardiac**= PVs drain into RA through hepatic veins and IVC (has highest risk of obstruction) ![]()

what is truncus arteriosus

![]() a rare heart defect that present at birth (congenital) one large blood vessel leads out of the other

what is tricuspid atresia

cyanotic CHD * the valve that controls blood flow from the right upper chamber of the heart to the right lower chamber of the heart doesn't form at all. * which restricts blood flow and causes the right lower heart chamber (ventricle) to be underdeveloped ![]()

what is hypoplastic heart syndrome

* left side of the heart is critically underdeveloped. * If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body. * Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome ![]()

what is Ebstein anomaly

cyanotic CHD * Displacement of tricuspid valve leaflets downward into RV, artificially “atrialising” the ventricle. * Associated with * Tricuspid regurgitation and right HF. * Can be caused by lithium exposure in utero.

what are the duct dependant lesions

![]()

what is eisenmenger

* Associated with septal defects and PDA shunt * Over time, excess Pulmonary Blood Flow from Left to Right shunt causes Pulmonary Hypertension and Right Ventricular Hypertension * When Pulmonary Vascular Resistance\>Systemic Vascular Resistance, there is a functional reversal of flow from Right to Left * Cyanosis now occurs

Congenital heart defects Flashcards

(33 cards)