Paediatric Cardiology Flashcards
What are the three fetal shunts?
Ductus venosus (umbilical vein to inferior vena cava)
Foramen ovale (right atrium to left atrium)
Ductus arteriosus (Pulmonary artery to aorta)
How can you keep the ductus arteriosus open and when might you want to keep it open?
Prostaglandin infusion
When there is a cardiac abnormality causing right->left shunt.
What are features of an innocent murmur? Egs:
Soft
Short
Systolic
Sounds (S1, S2 normal)
Symptom-less
Situation dependent (standing/sitting; vary with position)
What murmur is associated with an atrial septal defect?
Ejection systolic murmur, crescendo decrescendo
what conditions are associated with PDA?
- hyaline membrane disease
- concurrent cyanotic congenital cardiac diseases
(because the initial stimulus for physiological closure of ductus arteriosus is high blood oxygen tension which happens after establishment of breathing in those with normal circulatory and respiratory systems)
What murmur is associated with a PDA?
Machinery murmur of 2nd heart sound
What murmur is associated with Tetralogy of Fallot?
Ejection systolic murmur heard best at LUSE (pulmonary stenosis)
Which congenital heart conditions cause CYANOTIC heart disease?
Tetralogy of Fallot
Transposition of the great arteries
What murmur is associated with aortic stenosis? What other features are there?
Ejection systolic murmur, crescendo-decrescendo
Narrow pulse pressure
Slow rising pulse
Soft or absent S2
S4
How is aortic stenosis managed?
If asymptomatic -> observe
If symptomatic -> valve replacement
What is the most common complication of aortic stenosis?
Left ventricular outflow tract obstruction
What conditions are associated with pulmonary stenosis?
Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome
How does pulmonary stenosis present?
Ejection systolic murmur - louder on inspiration
Often found incidentally
Symptoms can be fatigue, SOB, failure to thrive
What murmur is associated with pulmonary stenosis? What other signs are there?
Ejection systolic murmur loudest in the pulmonary area
Palpable thrill
Right ventricular heave due to RV Hypertrophy
Raised JVP
What are causes of heart failure in: Neonates, infants, and older children?
Neonates = due to obstructed circulation (Hypoplastic left heart syndrome, aortic stenosis, coaractation of the aorta, interruption of aortic arch)
Infants = due to high pulmonary blood flow (ventricular septal defect, atrioventricular septal defect, large PDA_
Older children (Eisenmenger syndrome, Rheumatic heart Disease, Cardiomyopathy)
Which heart conditions cause a left to right shunt?
ASD
VSD
PDA
Which heart conditions cause a right to left shunt?
Tetralogy of Fallot
Transposition of the great arteries
Which type of shunt causes cyanosis?
Right to left
What are signs of heart failure in neonates/infants?
Breathlessness
Sweating
Poor feeding
Recurrent chest infections
Failure to thrive
What are symptoms and signs of atrial septal defect?
Symptoms = wheeze, may be often asymptomatic
Signs =
Ejection systolic murmur
Fixed, widely split S2 (due to increased pulmonary flow–> delayed closure of pulmonary valve
What is seen on CXR in atrial septal defect?
Cardiomegaly
Enlarged pulmonary arteries
Increased pulmonary vascular markings
How does a ventricular septal defect present?
presents with heart failure, recurrent chest infections, SOFT pan systolic murmur, LOUD pulmonary S2.
How is a ventricular septal defect managed?
Smaller asymptomatic VSD = close spontaneously
Larger symptomatic VSD = Surgical repair required
What are signs of a patent ductus arteriosus?
Continuous crescendo-decrescendo machinery murmur (may continue during 2nd heart sound, making it difficult to hear)
Left subclavicular thrill
large volume, Bounding collapsing pulse (arises from the increased pressure gradients and a continuous murmur from turbulent flow across the ductus arteriosus)
Wide pulse pressure
Heaving apex beat
How is a patent ductus arteriosus managed?
In preterm infants can give Indomethacin/Ibuprofen to help close the PDA
If asymptomatic - wait to see if it closes by 1 year
If symptomatic - needs closing
What is Eisenmenger syndrome and which conditions typically can cause it? treatment?
When a heart condition causing a L to R shunt (later: acyanosis) , eventually causes pulmonary HTN and therefore it becomes a R to L shunt (early, cyanosis: reversal of direction of cardiac shunting) ; ie going from acyanotic to cyanotic
- Typically occurs in late teens
- Cyanosis
- May develop right heart failure
This leads to cyanosis
Causes= ASD, VSD, PDA,TOF
Mx: Eisenmenger syndrome is best treated by prevention - i.e. identifying and promptly treating causes of left-to-right shunts
Otherwise, the treatment for Eisenmenger syndrome would be a heart-lung transplant, or palliation if this is not an option
How is Eisenmenger syndrome managed?
Complete heart-lung transplantation required
Which congenital heart condition is associated with Turner syndrome?
Coarctation of the aorta Aortic stenosis (due to bicuspid aortic valve)
How does coarctation of the aorta present? PMH?
Weak femoral pulses
Mid systolic murmur
Tachypnoea
Radio (Brachio)-femoral delay (leg pain, tiredness eg playing football): increased BP in upper, decreased BP in lower extremities: . In the presence of a tight coarctation, the blood supply to the legs will come through the ductus arteriosus from the pulmonary arteries, hence the saturation in the legs is lower when compared to the right arm saturation.
headaches, dizziness
epistaxis
Apical click
PMH: Turner;’s syndrome (bicuspid aortic valve)
How is coarctation of the aorta managed?
If critical, need to keep ductus arteriosus open with prostaglandin infusion whilst awaiting surgery
What are the 4 features of Tetralogy of Fallot?
- Ventricular septal defect
- Right ventricular hypertrophy
- Pulmonary stenosis (right ventricular outflow obstruction, determines severity of TOF)
- Overriding aorta
How does Tetralogy of Fallot cause cyanosis?
Pulmonary stenosis means that there is higher pressure on R side of heart, this means that there is a R to L shunt through the VSD
How does Tetralogy of Fallot present?
Ejection systolic murmur (due to pulmonary stenosis)
Cyanosis
“Tet spells” - cyanotic episodes, infant will typically squat
What is seen on CXR in Tetralogy of Fallot?
Boot shaped heart
How is Tetralogy of Fallot managed?
Surgical repair
ebstein’s anomaly exam findings:
- pansystolic murmur (tricuspid regurgitation)
- mid-diastolic murmur (tricuspid stenosis
Ebstein’s anomaly associations
- Lithium
- Wolff-Parkinson-White syndrome
What is Ebstein’s Anomaly?
A congenital heart defect where the tricuspid valve is set lower than usual, causing the RA to be larger than usual and the RV to be smaller (& hypocontractile) than usual
What are signs of transposition of the great arteries?
Loud single S2 (There may or may not be a murmur depending on the presence of a ventricular septal defect)
Prominent right ventricular impulse
-TGA is usually diagnosed with 24 to 48 hours of birth due to closure of patent ductus arteriosus
What does a continuous machinery murmur indicate?
Patent ductus arteriosus
What can an ejection systolic murmur indicate?
Aortic stenosis - LLSE (crescendo-decrescendo)
Pulmonary stenosis - Pulmonary area
Atrial septal defect (crescendo-decrescendo)
Aortic stenosis vs. ASD?
Aortic stenosis = narrow pulse pressure, slow rising pulse, soft S2
ASD = fixed splitting of S2
What might be seen on an ECG in an ASD?
RBBB
Which condition can cause patients with a DVT to develop a stroke?
Atrial septal defect
severity=degree of pulmonary stenosis
2 main physiological complications of PDA:
This creates a left to right shunt where blood from the left side of the heart crosses to the circulation from the right side.
- This increases the pressure in the pulmonary vessels causing pulmonary hypertension )–> pulmonary oedema)
- leading to right sided heart strain as the right ventricle struggles to contract against the increased resistance (hypoperfusion to tissues)
presentation of PDA:
-Shortness of breath
-Difficulty feeding
-Poor weight gain
-Lower respiratory tract infections
how is PDA diagnosed?
- echocardiogram.
- The use of doppler flow studies during the echo can assess the size and characteristics of the left to right shunt
An echo is also useful for assessing the effects of the PDA on the heart, for example demonstrating hypertrophy of the right ventricle, left ventricle or both.
Acyanotic vs cyanotic general symptoms:
Spot diagnosis
Boot-shaped heart
transposition of great vessels pathophysiology/RFs::
anatomical reversal of aorta & pulmonary artery –> 2 closed loop systems
-fatal unless intracardiac (PFO, VSD, ASD) or extracardiac shunt (PDA)
-no murmur
-prominent right ventricular impulse
-RF: maternal diabetes
pathophysiology of tricuspid atresia:
The tricuspid valve is absent and the right ventricle is hypoplastic due to absence of the inflow into the right ventricle. In the vast majority of cases there will be a ventricular septal defect (VSD) present and the size of this will affect the size of the right ventricular cavity.
timeline of cyanosis
how to differentiate between cardiac & non-cardiac causes of cyanosis:
Hyperoxia (nitrogen washout) test
-infant given 100% oxygen for 10 mins
-ABG is taken
-A pO2 of <15kPa =congenital cyanotic heart disease
order of acyanotic heart disease (common–> least common).
Risk factors:
VSD>ASD>PDA.
PMH: Downs (AVSD: endocardial cushion defect)
-Maternal: diabetes, alcohol
small VSDs vs medium-large VSDs symptoms
small: usually asymptomatic until eisenmenger syndrome
Medium-large: FTT, recurrent bronchopulmonary, infections, exertional dyspnoea
VSD murmur:
pansystolic (intensifies with hand clenching; louder in small defects)
-mid-diastolic murmur
ASD RFs:
PMH: Down’s
Maternal: alcohol consumption
PDA RFs:
-child: prematurity
-mother: TORCH infections (1st trimester=RUBELLA), alcohol consumption
Transposition of great vessels CXR findings:
egg on a string appearance
spot diagnosis
CXR: rib notching
complications of coarctation of aorta:
- secondary HTN
- endocarditis
- berry aneurysm–> subarachnoid haemorrhage (also associated with polycystic kidney disease)
complications of pulmonary stenosis:
RVOTO–> RVH
Eisenmenger syndrome pathophysiology & what happens in ASD/VSD vs PDA:
postnatal cardio-pulmonary changes:
causes of conjunctival haemorrhages
Whooping cough, leptospirosis
A new mother presents to the Emergency Department with her four-day-old child, concerned about his breathing and colour. Initial observations are as follows:
Temperature 36.8°C
Heart rate 200 bpm
Blood pressure 80/50 mmHg
Respiratory rate 80 bpm
Oxygen saturations 80% (on room air), which improves to 85% on 100% oxygen.
The neonate is a mottled blue colour, but lung fields are clear on auscultation, and heart sounds are normal. Alongside your initial A-E assessment, what other treatment is important to initiate in this case?
A. Nebulised corticosteroids
B. Intravenous antibiotics
C. Prostaglandin E infusion
D. Nebulised salbutamol
E. Start continuous positive airway pressure (CPAP)
C. PGE2 infusion
This baby is likely to have a cyanotic congenital heart abnormality such as transposition of great arteries or tetralogy of Fallot. There is not always a clinically audible murmur present. This typically presents as an increasingly cyanotic newborn in the first few days of life, as the previously patent ductus arteriosus closes and the newborn can no longer rely on it to allow oxygenated blood into the systemic circulation. These babies don’t appear to work so hard to breathe compared with babies with lung disease. Another clue that this is less likely to be a respiratory condition is the minimal improvement with high flow oxygen. A prostaglandin infusion will help keep the ductus arteriosus open whilst awaiting specialist input
Not CPAP:
The use of CPAP may be indicated in those children with impending respiratory failure, for example, due to bronchiolitis. However, this clinical presentation is more in keeping with a cardiac cause for cyanosis as opposed to a respiratory cause. Although there is minimal improvement with high flow oxygen, and there is a temptation to start a more intense form of respiratory support, it is unlikely to improve the neonate’s clinical picture
Maternal RFs for congenital cardiac disease:
- Infectious causes of congenital cardiac disease include maternal rubella infection during pregnancy
- Drugs that increase the risk of congenital heart disease include teratogenic medicines (e.g. thalidomide, isotretinoin, lithium) and substance misuse (e.g., alcohol).
- Maternal diabetes increases the risk of congenital cardiac disease, although this only applies to poorly controlled type 1 and 2 diabetes, and not gestational diabetes. This is likely because by the time gestational diabetes has developed, the heart is mostly formed.
when does ductus ateriosus close?
Ductus arteriosus closes on day 4/5 of life ); if collapse then, duct-dependent problem
A. Coarctation of aorta
B. congenital pneumonia
C. hypoplastic left heart syndrome
D. tetralogy of fallot
E. transposition of great arteries
E. transposition of great arteries
E: cyanosed (decreased sats), coarctation of aorta doesn’t cause cyanosis, HLHS is possible, TOF possible but there’s a machinery murmur vs ejection systolic VSD from TOF, congenital pneumonia: o2 will increase after hyperoxia test. No antenatal history; raises probability of significant cardiac lesion
what is truncus arteriosus:
Truncus arteriosus is when the left and right ventricles share a common outlet. It arises due to the failure of the aorta and pulmonary trunk to separate during foetal development. This common vessel is called the truncus arteriosus. While this is a cyanotic condition, it would be apparent upon birth and not be unmasked by the closing the ductus arteriosus
what is total anomalous pulmonary venous drainage:
While this is a cyanotic condition, it would present as cyanotic upon birth. Here the pulmonary veins (carrying oxygenated blood) do not drain into the left atrium as expected. It goes to either the right atrium or the systemic circulation, both of which result in a mixed supply. If draining to the right atrium, there is usually an atrial septal defect to allow for a right-to-left shunt and enabling systemic circulation
Transposition of great vessels CXR findings:
egg on a string appearance
which investigations are done for cyanotic heart disease?
- Pulse oximetry (usually shows hypoxia)
- Hyperoxia test
- CXR
- ECG
- Echocardiography for murmus (graded from 1-6)
what is the name for Prostaglandin E1 analogue?
Alprostadil
A 21-day old baby boy is brought into the Emergency Department by his worried mother as he has been having shortness of breath and irritability during feeds. His weight is on the 5th percentile for his age.
His mother has diabetes mellitus, but otherwise had an uncomplicated pregnancy and spontaneous vaginal delivery at 38 weeks.
He is tachypnoeic with accessory muscle use. On examination, there is a pansystolic murmur at the lower left sternal border and hepatomegaly.
An electrocardiogram shows right axis deviation. Chest X-ray (CXR) reveals a narrow mediastinum and an enlarged heart.
Which is the most definitive management of his condition?
A. Blalock-Taussig shunt
B. Balloon atrial septostomy
C. Prostaglandin infusion
D. Arterial switch operation
E. Indomethacin
Not A: Blalock-Taussig shunt
This would be an initial surgical procedure done in Tetralogy of Fallot to create a pathway between the subclavian and pulmonary arteries, thus relieving cyanosis whilst awaiting definitive corrective surgery
Not B: Balloon atrial septostomy
This is a surgical procedure done to stabilise patients with severe hypoxaemia due to inadequate mixing between the two parallel circuits in transposition of the great arteries (TGA). A balloon is placed across the atrial septum and used to enlarge the foramen ovale or existing atrial septal defect. However, it is only a temporary measure before corrective surgery for TGA
Not C: PG infusion
This would be part of initial management to maintain patency of the ductus arteriosus, allowing blood to travel from the left to the right side of the heart. However, in this case, the presence of a ventricular septal defect would already allow for left to right shunting of blood
Not E: indomethacin
This is a prostaglandin antagonist that is used in the treatment of patent ductus arteriosus. Anatomical closure would likely already have occurred at this age. Furthermore, it should not be used in cyanotic heart disease as it would worsen hypoxaemia by closing off the left to right shunt
associations and congenital heart defects:
list of cyanotic vs acyanotic conditions:
murmur summary
A 6-month-old female infant is found to have a heart murmur at a routine 6-month baby check. She has previously been healthy, apart from infantile colic. Her weight is on the 25th percentile and height is on the 10th percentile.
She is not cyanosed, has no chest deformity, and her peripheral pulses are normal. There is a palpable thrill at the left sternal edge, and the apex beat is palpable in the fifth intercostal space on the midclavicular line. A grade 4/6 systolic murmur is audible at the left sternal edge, which is radiating to the left axilla and to the back. There is no variation with respiration or posture.
What is the most likely diagnosis of her presentation?
A. Benign flow murmur
B. Ventricular Septal Defect
C. Atrial Septal Defect
D. Mitral Stenosis
E. Tetralogy of Fallot
B. Ventricular Septal Defect
The presence of a thrill with a murmur always indicates a pathological lesion and in combination with the nature, quality and radiation of the murmur as described negates a benign flow murmur as the cause. A thrill is usually not felt with lesions less than grade 4 out of 6 intensity. A ventricular septal defect is a congenital hole in the septum between the two ventricles. It is commonly associated with an underlying genetic condition, Down syndrome or Turner syndrome. VSDs are initially symptomless, and patients can present as late as adulthood. Initially, the shunt is from the left to the right ventricle due to high pressure on the left side. Over a while, after the onset of pulmonary hypertension, increased right-sided pressure causes a right-to-left shunt, known as Eisenmenger syndrome. Large VSDs require surgical correction with transvenous catheter closure via the femoral vein or open-heart surgery.
Not E: TOF
This infant is not cyanosed which makes tetralogy of Fallot unlikely, although there can be complicated forms which may not have obvious cyanosis.
