Paeds - Cardiothoracic and Vascular Flashcards
What are duct dependent lesions?
Where patient would deteriorate rapidly upon closure of ductus arteriosus
Duct dependent lesions:
Hypoplastic left heart syndrome
Severe coarctation
Interrupted arch
Pulmonary atresia
Severe Ebsteins anomaly
TGA (if no VSD)
Cyanotic vs non-cyanotic heart disease
Give examples of each
Cyanotic - all the T’s
What happens in total anomalous pulmonary venous return?
What is most common type?
What happends in type 3 and how does it present?
Cyanotic heart disease where all the pulmonary veins drain into the right side of the heart
- Require a LARGE PFO or ASD for survival
- WILL PRESENT IN EARLY NEONATAL PERIOD/DAY 4/5 WHEN DUCT CLOSES*
- Can be total or partial (where only some pulm veins drain to right side)*
3 Types of total:
1. Supracardiac (most common)
Veins drain above heart
2. Cardiac
3. Infracardiac (most likely to get asked)
Veins drain below diaphragm (heptic veins or IVC)
- Obstruction on way through diaphragm is common and can cause pulmonary oedema in newborn*
- Asplenia - 50% of asplenia patients have congenital heart disease. Of those nearly 100% have TAPVR, (85% have additional endocardial cushion defects).*
What is transposition of great arteries?
Most common cause of cyanosis during first 24 hours
Seen in the infants of diabetic mothers
Aorta arises from right ventricle
Pulmonary trunk arises in left ventricle
–survival depends on ASD, VSD or PDA (usually VSD)–
2 types:
D type is as above - requires shunt and surgery
L type - where ventricles invert leading to a congenital correction. RA - LV. LA - RV. No shunt needed
What are the features of Tetralogy of fallot?
What is most common surgical complication?
- VSD
- Pulmonary stenosis
- Overriding aorta
- RV hypertrophy
Most common surg complication is pulmonary regurg
What is truncus arteriorus?
What rib sign will be present?
Where there is just one trunk supplying pulmonary and systemic circulation
Cyanotic heart disease
FORKED RIBS are present
Almost always has VSD
Assc with right sided aortic arch and Di-george syndrome
What are differences between infantile and adult coarctation?
What are the features of hypoplastic left heart?
What are its associations?
Left ventricle and aorta are hypoplastic
Must have an ASD or large PFO
Typcally have large PDA to fill aorta
Give pulmonary oedema
Assc with aortic coarctation and endocardial fibroelastosis
What are features of PDA?
When should it close?
What are its associations?
Normally closes 24 hours after birth
Can be kept open or close it with medications
Associated with:
1. Prematurity
2. Maternal rubella
3. Cyanotic heart disease
What is most common cause of neonatal pneumonia?
Group B Strep
Causes pleural effusions unlike other causes
Associations:
- prematurity
- long labour
- prolonged rupture of membranes
What are features of meconium aspiration?
- Patchy consolidation
- Air bronchograms are ABSENT
POST TERM Baby
-Globules of meconium can cause air trapping and therefore increased lung volumes
Transient tachypnoea of the newborn
What are the associations?
When does it usually resolve?
Classically: diabetic mother with baby born via C section at TERM
Essentially retained fluid in the lungs
(In normal delivery, vagina squeezes fluid out of lungs)
- Bilateral symmetric air space opacification with air bronchograms
- Pleural effusions
Peaks at 24 hours and resolves at 3 days
Primary ciliary dyskinesia vs Cystic fibrosis
Where is distributuion of bronchiectasis?
Primary ciliary dyskinesia
- Affects middle and lower lobes
- 50% have kartageners (situs inversus, sinusitis, bronchiectasis)
- Affected men are infertile
- Affected women are subfertile
Cystic Fibrosis
Affects upper lobes
What is Heterotaxy?
Also known as Situs ambiguus
Can have LEFT or RIGHT isomerism
WHATS IN LEFT THORAX IS REPLICATED ON RIGHT AND VICE VERSA
Left sided:
- Polysplenia
- Interrupted IVS with azygous or hemiazygous continuation
- Two lung lobes on either side
- Central liver
- Bowel malrotation
- Truncated pancreas
- Congenital heart disease but not as bad as right sided
- ASD/VSD/PAPVR*
Right sided
Mortality of 80% by age 1
100% assc with congential heart disease (TAPVR etc)
-Asplenia
- Three lung lobes on either side
- Malrotation
- Left sided IVC
- Horseshow kidney
- Normal liver
What are main respiratory diseases in the PREMATURE newborn?
1. Hyaline Membrane Disease (Surfactant deficiency)
X-ray will show granular opacification throughout both lungs
Progresses to GGO with air bronchograms
Does NOT cause pleural effusions
2. Pulmonary interstitial emphysema
Prevalent in infants of low birth weight
Caused by alveolar rupture due to barotrauma of MECHANICAL VENTILATION OR POSITIVE AIRWAYS PRESSURE
Pneumothorax and pneumomediastinum are complications
Streaky lucencies arising from the hila
- Bronchopulmonary dysplasia
Occurs due to barotrauma
Longer history
Occurs when baby has received ventilation with 21% oxygen for longer than 28 days
CXR: hyperinflated lungs which becomes ground glass then complete opacification with linear luncencies projecting from hila
What is the appearances of Extramedullary haematopoeisis?
Appear as soft tissue masses usually in solid visceral organs
**Due to failure of erythropoeisis in bone marrow**
CT: lobulated soft tissue mass. hypervascular. should not biopsy unless just FNA. can have fat within also.
Most commonly seen as hepatomegaly and splenomegaly
Posterior mediastinal mass is most common intrathoracic manifestation
-can be unilateral or bilateral
What is differential for posterior mediastinal mass in kid?
1. Neuroblastoma is most common in under 10 years
-Heterogenous with calcification and areas of necrosis
2. Extramedullary haematopoesis
- Coarse bone trabeculations and adjacent posterior mediastinal mass
- NO calcifications
3. Neuroenteric cyst
Cystic lesion associated with spinal abnormalities and scoliosis
4. Neurofibroma
Usually in child above 10
Rounded mass
What is differential for large hemithoracic mass in a 1-2 year old child?
Either 2 things
1. Askin Tumour
Heterogenous tumours
Patients normally present in early teens
Areas of cystic change within
Solid components enhance
NO calcification
Displaces mediastinal structures
Rib destruction is common (differentiates from pleuropulmonary blastoma)
2. Pleuropulmonary Blastoma
Occurs in 1 - 2 years
Enhancing heterogenous mass
Usually right sided
No calcification
No skeletal or rib involvement
Have an association with multicystic nephroma
What is CPAM?
Congenital pulmonary airway malformation
Usually antenatal diagnosis but can present later
-Cystic mass or masses in the lungs
-Can have air fluid levels
-Can exert mass effect and cause mediastinal shift
-Can cause hypoplasia of the ipsilateral lung
Congenital Lobar Hyperinflation
Due to underdeveloped bronchus = collapse during expiration leading to air trapping
Can have similar story of hypoxia and mediastinal shift
-Hyperinflated left upper lobe (can be right middle or upper lobe either)
Will appear on CXR as hyperlucent area due to airtrapping with mediastinal shift
Granular Neonatal CXR
Streaky perihilar neonatal CXR
Ropy neonatal chest x-ray
Transient Tachypnea of the Newborn
What is classical scenario?
Due to residual fluid in the lungs that is normally squeezed out by vagina during normal delivery
Hence..
Seen in:
- C-section, Maternal Sedation, Maternal Diabetes
- -Onset at 6 hours, peaks in 1 day, resolves by day 3*
Fluid in the fissures and coarse interstitial markings
Lung Volumes - Normal to INcreased
Respiratory distress syndrome/Surfactant Deficient Disease/Hyaline membrane disease
Who gets it?
What are CXR appearances?
Disease of premature kids
Born without surfactant
-Most common cause of death in premature newborns
Low lung volumes
Bilateral granular opacities
A normal plain film at 6 hours excludes this
Summary of Lung issues in Neonates
Pulmonary interstitial emphysema
How does it occur and when?
Results from ventilation in ICU which forces air from alveoli into lymphatics and interstitium
- First weeks of life
- Linear lucencies on CXR
- Warning sign for impending pneumothorax
Intralobular vs Extralobular sequestration
What ages do they occur in??
• Intralobar: Much more common (75%). Presents in adolescence or adulthood as recurrent pneumonias (bacteria migrates in from pores of Kohn). Most commonly in the left lower lobe posterior segment (2/3s). Uncommon in the upper lobes.
In contradistinction from extralobar sequestration, it is rarely associated with other developmental abnormalities. Pathology books love to say “NO pleural cover” - but you can ’t see that shit on CT or MR.
• Extralobar: Less common of the two (25%). Presents in infancy with respiratory compromise (primarily because of the associated anomalies - Congenital cystic adenomatoid malformation (CCAM), congenital diaphragmatic hernia, vertebral anomalies, congenital heart disease, pulmonary hypoplasia). It rarely gets infected since it has its own pleural covering. These arc sometimes described as part of a bronchopulmonary foregut malformation, and may actually have (rarely) a patent channel to the stomach, or distal esophagus. Pathology books love to say “has a pleural cover” - but you can’t see that shit on CT or MR.
Swyer James
What is it caused by?
OLDER CHILD OR ADOLESCENT (NOT MONTHS OLD) (congential lobar inflation can give similar signs in neonate)
Unilateral lucent lung
Occurs post viral infection in childhood with resultant obliterative bronchiolitis
The size of the affected lobe(s) is smaller than normal
CT: hyperlucent lung with diminished vascularity
Child with multiple cavitatory cystic lung nodules
What is diagnosis?
Papillomatosis
Due to perinatal HPV infection
Can cause transformation to squamous cell cancer
Involvement of trachea differentiates from LCH
Primary ciliary dyskinesia vs CF
What are differences?
CF - upper lobes affected
Primary ciliary dyskinesia - lower lobes affected
Suspicious appearances of thymus
What are they?
Thymoma is more common in kids under 10
Lymphoma is more common in kids over 10
Anterior mediastinal masses
Different types of germ cell tumours?
- Teratoma - cystic with fat and calcium
- Seminoma - large and lobulated, straddles midline
- Non-seminomatous - very large, invasive with haemorrhage and necrosis (see picture)
Most common posterior mediastinal mass in child under 2 years?
Neuroblastoma
- Can involve ribs and vertebral bodies
- If there are mets in the lungs (think of Wilms, its more common to met to lung than neuroblastoma)
These other variations of neuroblastoma can also be found in posterior mediastinum:
What is an Askin tumour?
Essentially a Ewings Sarcoma of the chest wall/ribs
Displaces structures early on then invades as it gets bigger
Neuroenteric Cyst
What is it?
Intraspine cysts in an extramedullar location
- DO NOT make contact with CSF*
- -Associated with vertebral anomalies (scoliosis, hemivertebrae, split cord)*
How to approach posterior mediastinal masses?
Most common in under 10?
In under 10 years - think Neuroblastoma
What is Epsteins anomaly?
Box shaped heart***
Apical displacement of the posterior and septal tricuspid valve leaflefts leading to part of the right ventricle becoming part of right atrium
Associated with:
***Hypoplastic Aorta
***Hypoplastic pulmonary trunk
L transposition vs D transposition
D = switching of outlet vessels
L = switching of ventricles
