QuestionSBA
QuestionChoice
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
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Blalock-Taussig shunt
QuestionChoice
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
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Balloon atrial septostomy
QuestionChoice
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
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Prostaglandin infusion
QuestionChoice
This neonate has transposition of the great arteries (TGA) and has presented in heart failure. He has not developed rapid cyanosis on day 2 of birth (with closure of the ductus arteriosus) but has presented later at a few weeks of age, as the presence of a ventricular septal defect (VSD) has allowed for improved inter-circulatory mixing. The classical finding on CXR would be an "egg on string" appearance. The definitive treatment would be an operation to translocate both the great arteries to the opposite root, establishing ventriculoarterial concordance
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Arterial switch operation
QuestionChoice
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
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Indomethacin
QuestionComment
Sounded like Tet spells to me
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Malignant Myotonia
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How can you tell he has both TGA and a VSD and not TOF?
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QuestionComment
TOF would be ejection systolic murmur at left upper edge- pulmonary stenosis.
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Monoclonal Polyps
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Recessive Malignant
QuestionComment
it honestly sounds like TOF >> TGA with a VSD? the only thing is that a BT shunt is a temp procedure so sure that's not 'definitive' treatment but the stem should really be changed to look more like TGA maybe? the only indication for this to be a tga for me was mom having diabetes but. idk. 
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I still dont 100% agree that this is great description of TGA BUT after doing this paper again, I suppose one can argue and say that in TOF, the murmur is usually of pulmonary stenosis rather than the VSD and the CXR findings of a narrow mediastinum + large heart are also more likely to be TGA because 

The heart appears globular due to an abnormal convexity of the right atrial border and left atrial enlargement and therefore appears like an egg.

The superior mediastinum appears narrow due to stress-induced thymic atrophy and hyperinflated lungs which give the picture of an egg suspended by a string on a chest radiograph, hence the name egg-on-a-string.
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DNA Tachycardia
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DNA Tachycardia
QuestionComment
can wait to perform my first blalock-taussig shunt as an F1
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Axillary Gas
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Anyone not think of ebsteins anamoly, pan-systolic murmur heard at lower left sternal border with signs of right sided heart failure. Any help would be appreciated 
61246
QuestionComment
This is exactly what I thought
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Defibrillator Embolism
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Fronk
Concept
Chapter
# Summary
 
Congenital cardiac disease refers to a variety of heart abnormalities present at birth, often influenced by maternal risk factors such as infectious disease during pregnancy (e.g. rubella), exposure to teratogenic drugs (e.g. thalidomide, isotretinoin, lithium), substance misuse (e.g., alcohol), and poorly controlled type 1 or 2 diabetes. Identification primarily relies on signs and symptoms observed in newborns, with murmurs often being present, with/without cyanosis, and confirmatory investigations such as echocardiography. Management is typically multidisciplinary and can involve medical therapy, interventional procedures, or surgery, depending on the severity and type of heart defect.
 
 
# Definition
 

Congenital cardiac disease is a general term referring to abnormalities in the heart's structure that arise before birth. These defects can involve the heart walls, heart valves, or the arteries and veins near the heart.
 

# Epidemiology
 

The global incidence of congenital heart disease is estimated to be around 1% of live births, making it the most common type of congenital disorder. 
 

# Aetiology
 

Several factors can contribute to the development of congenital heart disease:
 

-  Infectious causes: 
     -  Maternal rubella infection during pregnancy
-  Teratogenic drugs: 
     -  Exposure to certain medications during pregnancy, such as thalidomide, isotretinoin, and lithium, increases the likelihood of developing congenital heart disease.
-  Substance misuse: 
     -  Maternal misuse of substances such as alcohol can also lead to this condition.
-  Maternal diabetes: 
     -  Poorly controlled type 1 and type 2 diabetes during pregnancy
     -  Maternal gestational diabetes is not a known risk factor 
- Family history of congenital heart disease
    - Risk is also increased in consanguineous couples 

    
# Classification

Congenital heart disease can be divided into cyanotic or acynotic heart disease.

Cyanotic Heart Disease:

-  Transposition of the great arteries (TGA)
-  Pulmonary and tricuspid atresias
-  Tetralogy of Fallot (ToF)
-  Total anomalous pulmonary venous return 
-  Persistent truncus arteriosus
-  Hypoplastic left heart

Acyanotic Heart Disease:

- Ventricular septal defect (VSD)
- Atrial septal defect (ASD)
- Patent ductus arteriosus (PDA)
- Aortic stenosis 
- Pulmonic stenosis 
- Coarctation of the aorta 
- Endocardial fibroelastosis 
 

# Signs and Symptoms
 

Newborns with congenital cardiac disease may present with a variety of signs and symptoms, including:
 

 -  Cyanosis
 -  Fast or troubled breathing
 -  Fatigue
 -  Poor feeding
 -  Delayed growth
 -  Heart murmur on auscultation
 

These symptoms can vary widely depending on the type and severity of the heart defect.
 

# Differential Diagnosis
 

In an infant presenting with the signs and symptoms of congenital heart disease, several conditions should be considered:
 

 -  **Respiratory distress syndrome**: This condition can cause fast, troubled breathing, and fatigue. However, it is typically associated with prematurity.
 -  **Pneumonia**: Can also lead to breathing difficulties and fatigue, but it is often accompanied by fever and abnormal lung sounds on auscultation.
 -  **Gastrointestinal disorders**: These can cause poor feeding and delayed growth but are typically accompanied by vomiting, diarrhoea, or abdominal distension.
 

# Investigations
 

Whilst some cases are detected during prenatal scans, many cases are detected after birth. The following investigations are key to diagnosing congenital heart disease:
 

 -  Pulse oximetry: To detect low oxygen levels in the blood. This should be measured both pre and post-ductal, with > 3% difference being significant.  
 -  Chest X-ray: Can reveal an enlarged heart or abnormal heart shape.
 -  Electrocardiogram (ECG): Can detect abnormal heart rhythms.
 -  Echocardiogram: The gold standard in diagnosing congenital heart disease, providing a detailed image of the heart's structure and function.
 

# Management
 

The management of congenital heart disease is complex and depends on the type and severity of the heart defect.

Red flag features require more urgent management. These features include:

- Lower limb saturations < 96%
- More than 3% pre/post ductal difference 
- Signs of heart failure or shock


It typically involves a multidisciplinary team and can include:

-  Conservative management:
     -  For certain defects (i.e. mild atrial septal defects, a watch-and-wait approach may be adopted as many will close by age 1)

- Medical management:
    - Some patients will require medications including diuretics and ACE inhibitors depending on the type of congenital heart disease. 
- Interventional procedures: Catheter-based procedures to repair certain types of heart defects.
 
- Surgical intervention: For more complex or severe heart defects, surgery may be necessary.

# Complications

Depending on the type and severity of congenital heart disease, potential complications include:

- Learning difficulties
- Pneumonia 
- Infective Endocarditis 
- Pulmonary hypertension 
- Heart failure 

Sudden cardiac death is very rare. 

# Prognosis

Surgically corrected congenital heart disease, particularly when isolated, has a good prognosis, however, there is still a reduced life expectancy when compared to the general population. 

# NICE Guidelines

[NICE Guidelines on Structural Heart Defects](https://www.nice.org.uk/guidance/conditions-and-diseases/cardiovascular-conditions/structural-heart-defects) 

 
#  References
 
 [NHS Congenital Heart Disease](https://www.nhs.uk/conditions/congenital-heart-disease/#:~:text=Congenital%20heart%20disease%20is%20a,babies%20born%20in%20the%20UK.) 
 
 [British Heart Foundation Congenital Heart Disease](https://www.bhf.org.uk/informationsupport/conditions/congenital-heart-disease) 
 
 [Patient Info Congenital Heart Disease in Children](https://patient.info/doctor/congenital-heart-disease-in-children) 
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Maternal risk factors for congenital heart disease
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Maternal risk factors for congenital heart disease
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Maternal risk factors for congenital heart disease
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Maternal risk factors for congenital heart disease
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Quesmed Tutorial: Congenital Heart Defects
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The arterial switch operation is the standard surgical treatment for transposition of the great arteries (TGA), a congenital heart defect where the aorta and pulmonary artery are swapped. This procedure involves switching the positions of the aorta and pulmonary artery, reattaching the coronary arteries to the new aorta, and correcting the alignment to restore normal circulation.
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