Congenital Heart Diseases Overview

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Questions and Answers

What is the primary characteristic of congenital heart diseases?

They are always caused by a genetic abnormality.

They are acquired conditions that develop after birth.

They are abnormalities of the heart present at birth. (correct)

They are limited to structural issues in the arteries.

What contributes to the development of congenital heart diseases as suggested by the information?

Previous cardiac surgeries in the family.

Environmental factors and nutritional deficiencies only.

Genetics and specific maternal behaviors such as heavy drinking. (correct)

Only maternal infections during pregnancy.

Which group of congenital heart disease includes conditions that can lead to cyanosis over time?

Malpositions of the heart

Cyanotic CHD

Obstructive CHD

Left to Right shunts (correct)

What is a common clinical manifestation of large ventricular septal defects (VSDs)?

Hypertrophy of the right ventricle. Signup and view all the answers

Which of the following statements is true about left to right shunts?

They result in increased pressure in the pulmonary circulation. Signup and view all the answers

What is the incidence of congenital heart diseases in premature infants compared to full-term infants?

Higher in premature infants. Signup and view all the answers

Which congenital heart defect is characterized by an opening between the two ventricles?

Ventricular septal defect (VSD) Signup and view all the answers

What percentage of ventricular septal defects (VSDs) occur in the membranous septum according to the information provided?

90% Signup and view all the answers

What is the most common type of atrial septal defect (ASD)?

Secundum ASD Signup and view all the answers

What is the consequence of a right-to-left shunt in congenital heart defects?

Cyanosis due to poor oxygenation Signup and view all the answers

What typically causes the ductus arteriosus to close following birth?

Increase in oxygen levels Signup and view all the answers

At what age does spontaneous closure of an atrial septal defect commonly occur if left uncorrected?

By 1 year Signup and view all the answers

Which of the following is NOT a characteristic feature of Tetralogy of Fallot?

Left ventricular hypertrophy Signup and view all the answers

Which condition is associated with significant polycythemia due to functional anemia?

Tetralogy of Fallot Signup and view all the answers

What is a common consequence of persistent truncus arteriosus?

Single arterial trunk Signup and view all the answers

Which type of atrial septal defect occurs adjacent to the atrioventricular valves?

Primum ASD Signup and view all the answers

What type of right ventricular hypertrophy is characterized by a greater pulmonary stenosis, leading to cyanosis?

Cyanotic tetralogy Signup and view all the answers

In corrected transposition of the great arteries, which vessels arise from the corresponding ventricles?

Aorta from Right Ventricle, Pulmonary Artery from Left Ventricle Signup and view all the answers

Which of the following cardiac conditions is associated with both a single large vessel and a ventricular septal defect?

Persistent truncus arteriosus Signup and view all the answers

What is the characteristic heart shape observed in a patient with acyanotic tetralogy?

Boot-shaped heart Signup and view all the answers

What is the consequence of tricuspid stenosis in relation to the right atrium?

Enlargement of the right atrium Signup and view all the answers

What is a common complication of coarctation of the aorta in the post ductal or adult type?

Hypertension in the upper extremities Signup and view all the answers

Which of the following conditions commonly presents with cyanosis in the lower half of the body while the upper part remains unaffected?

Preductal or infantile coarctation of the aorta Signup and view all the answers

Which of the following is the most common anomaly associated with aortic stenosis?

Congenital bicuspid valve Signup and view all the answers

What is a common method used to correct many congenital heart defects?

Open-heart surgery Signup and view all the answers

What is indicated for a serious congenital heart defect that cannot be repaired?

Heart transplant Signup and view all the answers

Which medications are typically used to help treat mild congenital heart defects?

Angiotensin-converting enzyme (ACE) inhibitors Signup and view all the answers

What long-term care is necessary for children with congenital heart defects?

Continuous monitoring and follow-up with a pediatric cardiologist Signup and view all the answers

Which procedure may be done in steps over a period of years for congenital heart defects?

Catheter or surgical procedures Signup and view all the answers

At what point does care for a child with a congenital heart defect transition to an adult specialist?

As the child grows older Signup and view all the answers

What is often necessary even after corrective surgery for congenital heart defects?

Lifelong monitoring and care Signup and view all the answers

Which of the following is NOT a treatment method for congenital heart defects?

Regular check-ups with a dentist Signup and view all the answers

Which children are at a higher risk of infection related to congenital heart defects?

Children whose defects were repaired with prosthetic material or devices. Signup and view all the answers

What is a primary concern for parents of children with congenital heart defects regarding exercise?

Children may need to limit the amount or type of exercise allowed. Signup and view all the answers

What role does the child's doctor play in managing exercise for children with congenital heart defects?

The doctor provides advice on which activities are safe for the child. Signup and view all the answers

Why might some children with congenital heart defects need to take antibiotics?

To prevent infections before additional surgeries or dental procedures. Signup and view all the answers

What should parents encourage their child to focus on regarding activities?

Participating in other safe activities instead of focusing on limitations. Signup and view all the answers

Study Notes

Definition

Congenital heart diseases are heart or great vessel abnormalities present at birth.

Incidence

Incidence is higher in premature infants.
Faulty embryogenesis during the 3rd to 8th week of intrauterine life can cause congenital heart defects.

Cause

The exact cause is unknown.
Predisposing factors include:
- Genetic factors
- Rubella infection
- Drugs
- Heavy drinking during pregnancy

Classification

Malpositions of Heart:
- Dextrocardia (1 in 12,000 newborns)
Shunts (Acyanotic and cyanotic CHD):
- Abnormal communications between two chambers or blood vessels.
- Blood flow is determined by the pressure gradient.
Obstructions (Obstructive CHD):
- Obstruction of blood flow in the heart.

Shunts (Acyanotic and cyanotic CHD)

Left to Right Shunts (Acyanotic or Late Cyanotic Group):
- Cyanosis presents several months or years after birth.
- Examples:
  - Ventricular septal defect (VSD): 25-30%
  - Atrial septal defect (ASD): 10-15%
  - Patent ductus arteriosus (PDA): 10-20%
Right to Left Shunts (Cyanotic Group):
- Cyanosis presents in early postnatal life.
- Examples:
  - Tetralogy of Fallot (TOF): 6-15%
  - Transposition of great arteries: 4-10%
  - Persistent truncus arteriosus: 2%
  - Tricuspid atresia and stenosis: 1%

Left to Right Shunts

Increased pulmonary blood flow leads to:
- Increased pulmonary pressure.
- Overwork of the right ventricle muscle potentially leading to cardiac failure.
- Medial hypertrophy and intimal proliferation to prevent pulmonary edema.
- Prolonged increased pulmonary pressure can reverse blood flow from right to left, resulting in unoxygenated blood in systemic circulation and late cyanosis.
Once significant pulmonary hypertension develops, surgical treatment of cardiac defects is not possible.

Ventricular Septal Defect (VSD)

Opening between the two ventricles due to incomplete closure of the ventricular septum.
Majority are left to right shunts.
Dilated right heart.
Too much blood to lungs.
Increase in pulmonary pressure.

Morphology of VSD

90% in the membranous septum.
10% below the pulmonary valve or within the muscular septum.
Mostly single, with multiple VSDs in the muscular septum being less common.

Clinical Features of VSD

Depend on the size of the lesion.
Small lesions may be recognized later or close spontaneously.
Large VSDs are recognized early in life, cause left to right shunts, and lead to hypertrophy of the right ventricle.
Pulmonary hypertension can develop from birth.
Ultimately, shunt reversal, cyanosis, and death can occur.
If not corrected by 1 year of age, spontaneous closure is likely.

Atrial Septal Defect (ASD)

Abnormal fixed opening in the atrial septum due to incomplete tissue formation.
Usually goes unnoticed in infancy and childhood.
Typically presents later in life (around age 30), late cyanotic heart disorder.
Left to right shunt at the atrial level (pulmonary vascular resistance is less than systemic, and compliance of the right ventricle is greater than the left).
Pulmonary blood flow is increased to 2-4 times, leading to hypertrophy of the right atrium and right ventricle.
Pulmonary hypertension and right heart failure are uncommon.

Morphology of ASD

Three types based on location:
- Secundum ASD (90%): deficient or fenestrated oval fossa near the center of the septum.
- Primum ASD: occurs adjacent to the AV valves.
- Sinus venosus ASD: near the entrance of the superior vena cava.
AVSD (Atrio ventricular septal defect)

Patent Ductus Arteriosus (PDA)

Normal connection between the aorta and the bifurcation of the pulmonary artery.
Normally closes within the first or second day of life.
Persistence for longer than 3 months is considered abnormal.

Causes of PDA

Increased levels of PGE2 after birth:
- Seen in children with respiratory distress syndrome.
- Pharmacologic closure with indomethacin (PGE2 inhibitor) is possible.
Most often does not produce functional difficulties at birth.

Effects of PDA

A narrow PDA has no effect on growth and development during childhood.

Right to Left Shunts

Decreased pulmonary blood flow leads to:
- Poor oxygenation of blood entering the left side of the heart.
- This oxygen-poor blood flows to the systemic circulation, resulting in dusky blueness of the mucous membranes and skin (cyanosis).

Effects of Right to Left Shunts

Functional anemia leads to increased synthesis of hemoglobin and red blood cells, resulting in polycythemia (increased blood volume).
Clubbing of the tips of the fingers and toes due to reduced oxygen.

Tetralogy of Fallot (TOF)

Combination of shunts with obstruction leading to functional impairment.
One of the most common cyanotic heart diseases.
Involves four features:
- VSD
- Displacement of the aorta to the right side, overriding the septal defect.
- Sub-pulmonary stenosis (obstruction).
- Right ventricular hypertrophy.
Clinical manifestations depend on the extent of pulmonary stenosis and VSD.

Types of TOF

Cyanotic TOF: Pulmonary stenosis is greater, increasing resistance to blood flow in the right ventricle.
- Blood flows to the left ventricle, causing cyanosis.
Acyanotic TOF (Pink Tetralogy):
- VSD is larger, and pulmonary stenosis is mild, resulting in a left to right shunt.
- Characteristics similar to VSD.
- Boot-shaped heart.

Effects of TOF

Pressure hypertrophy of the right atrium and right ventricle.
Small tricuspid valve.
Small left atrium and left ventricle.
Enlarged aortic orifice.

Transposition of Great Arteries

Regular transposition:
- Aorta arises from the right ventricle.
- Pulmonary artery arises from the left ventricle.
- Cyanosis present from birth.
Corrected transposition:
- Aorta arises from the right ventricle.
- Pulmonary artery arises from the left ventricle.
- Pulmonary veins drain into the right atrium.
- Superior and inferior vena cava drain into the left atrium.
- Physiologically corrected circulation.

Persistent Truncus Arteriosus

The arch that separates the aorta from the pulmonary artery fails to develop.
A single large vessel receives blood from both ventricles.
Often associated with a ventricular septal defect.
Early systemic cyanosis.
Poor prognosis.

Tricuspid Atresia and Stenosis

Often associated with pulmonary stenosis or atresia.
- Atresia: absence of the tricuspid orifice, a dimple in the floor of the right atrium.
- Stenosis: the tricuspid ring is small and hardened.

Obstructions (Obstructive CHD)

Coarctation of the aorta: 5-7%
- Localized narrowing in any part of the aorta.
- More common in males and females with Turner syndrome.
Aortic stenosis and atresia: 4-6%
Pulmonary stenosis and atresia: 5-7%

Coarctation of the Aorta

Postductal or adult type:
- Obstruction is just distal to the ductus arteriosus, which is closed.
- Characterized by hypertension in the upper extremities, weak pulses and low blood pressure in the lower extremities, effects of arterial insufficiency such as coldness and claudication (muscle pain due to reduced oxygen).
Preductal or infantile type:
- Narrowing proximal to the ductus arteriosus, which remains patent.
- Lower half of the body is cyanosed while the upper part of the body receives blood from the aorta.

Aortic Stenosis and Atresia

The most common anomaly of the aorta is a congenital bicuspid valve.

Long-term Treatment of Congenital Heart Disease

Many congenital heart defects are corrected using open-heart surgery.
Minimally invasive heart surgery may be an option.
Heart transplant may be an option for severe heart defects that cannot be repaired.
Medications can be used to help the heart work more efficiently.
Some treatments may need to be done in steps over a period of years and may need to be repeated as the child grows.

Long-term Monitoring and Treatment of Congenital Heart Disease

Lifelong monitoring and treatment are necessary.
Even after surgery to correct a heart defect, ongoing care is required for life.
The child will be initially monitored by a pediatric cardiologist and later transitioned to an adult congenital cardiologist.
Adults with congenital heart defects may need additional treatments for their condition.

Exercise for Children with Congenital Heart Disease

Exercise may need to be restricted.
Most children with congenital heart defects can participate in normal or near-normal activity.
The child's doctor can recommend safe activities.
Encourage participation in alternative activities if some pose specific dangers.

Infection Prevention for Children with Congenital Heart Disease

Depending on the type of heart defect and the surgery used, additional steps may be needed to prevent infection.
Children at higher risk for infection include those with repaired defects involving prosthetic materials or devices, like artificial heart valves.
Congenital heart defects can increase the risk of infections in the lining of the heart or heart valves (infective endocarditis).
Antibiotics may be prescribed to prevent infection before surgeries or dental procedures.

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Description

This quiz covers the essential aspects of congenital heart diseases, including their incidence, causes, and classifications. Learn about various conditions such as dextrocardia and the differences between acyanotic and cyanotic shunts. Test your knowledge on this critical topic in pediatric health and cardiology.