Introduction

Congenital surgery for congenital heart defects repairs or treats a heart defect that is present in a child. Congenital heart defects are often diagnosed during prenatal screenings or shortly after birth. Surgical interventions become necessary when an abnormality poses a risk to the child’s sustained health or overall welfare. 

Surgical interventions may be required to:

• Repair a hole in the heart
•  To repair complex defects, such as problems with the location of blood vessels near the heart or how they are formed.
• To substitute or repair a valve.
• To widen narrowed blood vessels.¹   

Congenital heart surgery aims to repair or reconstruct the affected structures, allowing the heart to function more effectively and reducing the risk of associated complications. The specific surgical approach depends on the nature and severity of the defect, and it may involve techniques such as open-heart surgery or minimally invasive procedures.

Overview of congenital heart defects

Congenital Heart Defects (CHDs) manifest at birth and have the potential to impact both the structure and the function of the infant’s heart. They can affect how the blood flows through the heart and out to the rest of the body. CHDs can range in severity from mild to severe. 

Critical Congenital Heart Defect affects approximately one in every four infants born with a heart defect. One year after birth, infants with critical CHD require surgery or other procedures. Congenital heart defects (CHDs) constitute the most prevalent form of congenital defect.²   

Types of congenital heart defects

Aortic Valve Stenosis (AVS) 

A valve that connects the heart to the rest of the body will not properly open and close and may cause blood leakage. When an inadequately functioning valve obstructs the blood flowing out from the heart, pressure may build up inside the heart and cause damage. 

Atrial Septal Defect (ASD)

This defect permits the leakage of oxygen-rich into the heart’s oxygen-depleted blood chambers. ASD is a septal defect separating the heart's two upper chambers (atria). The septum is a partition that divides the left and right sides of the heart.

Coarctation of the Aorta (CoA)

A constriction of the aorta, the principal artery supplying blood to the body. This constriction disrupts blood flow to the upper and lower regions of the body through distinct vessels, where the arteries branch out. Increased blood pressure and cardiac injury may result from CoA. 

Complete Atrioventricular Canal Defect (CAVC)

A large hole in the center of the heart will affect the four chambers where they would normally be divided. The oxygen-poor blood from the body and the oxygen-rich blood from the lungs do not mix when the heart is appropriately divided. A CAVC permits the blood to mingle while the chambers and the valves fail to deliver the blood to each station of circulation in an efficient manner.  

D-transposition of the great arteries

Blood is carried in a cycle by a normal blood pattern: body-heart-lungs-heart-body. When a d-transposition occurs, the blood pathway would be impaired as the two arteries would be connected to the wrong chambers of the heart. This indicates that the blood flow cycle is stuck in either : 

Body-heart-body (in the absence of oxygen delivery to the lungs) or lungs-heart-lungs( in the absence of oxygen delivery to the body). 

I-Transposition of the great arteries

The lower section would be reversed in the heart. This abnormality of the heart causes a reversal of the typical blood flow pattern as the right and left lower chambers of the heart are reversed.

Ebstein’s Anomaly

A heart valve that does not close properly to direct the blood flow in the correct direction. There may be a leakage of blood from the lower to upper chambers of the heart on the right side. 

Pulmonary Valve Stenosis

A fused or thickened cardiac valve that does not open fully. Blood may exit the heart via the pulmonary valve, enter the pulmonary artery, and travel to the lungs. 

Single Ventricle Defects

This rare disorder affects one lower chamber of the heart.

• Hypoplastic Left Heart Syndrome (HLHS)

The left side of the heart would be underdeveloped. The aorta and left ventricle are small in size, and the arterial septal fissures have not matured properly.

• Pulmonary Atresia

The pulmonary valve would be absent, oxygen is supplied exclusively to the blood that is redirected to the lungs through openings that typically close during development. 

• Tricuspis Artesia

In the absence of a tricuspid valve, blood cannot normally travel from the body into the heart. If the oxygen is not adequately replenished into the circulation, the body’s normal cycle of body-heart-lungs-heart cannot be completed. 

Tetralogy of Fallot

Four complications characterise a heart defect:

• A hole between the lower chambers of the heart.
• There would be an obstruction from the heart to the lungs
• The blood vessel aorta is positioned above the hole in the lower chambers
• The muscle that envelops the lower right chamber undergoes excessive thickening.

Total Anomalous Pulmonary Venous Connection (TAPVC)

A defect in the veins. TAPVC is characterised by an abnormal blood flow path from the lungs to the heart and out of the body. Instead, the veins originating from the lungs connect to the heart at abnormal positions, resulting in the leakage and entrance of oxygenated blood into the wrong chamber. 

Truncus Arteriosus

When a person has a single major artery that carries blood to the lungs and body as opposed to two distinct ones, the circulation of blood in a healthy heart is as follows: body-heart-lungs-heart-body. With the Truncus Arteriosus, the blood that exits the heart does not follow this path, it has a single vessel, as opposed to two distinct vessels for the lungs and the body. With only one artery, there is no specific path for oxygen to the lungs before it returns to the heart to deliver oxygen to the body.

Ventricular Septal Defect (VSD)

A VSD is a hole in the wall that divides the two lower chambers of the heart.³ 

Diagnostic tools and assessment

Fetal diagnosis 

Ongoing advancements in parental imaging include progress in echocardiography, which has contributed to an increase in the success rate of foetal Congenital Heart Surgery diagnosis.⁴A foetal echocardiography is done from the late first trimester onward, employing a systematic and standardized methodology to identify foetuses afflicted with congenital heart disease (CHD) or other types of primary or secondary cardiac abnormalities. 

Parental diagnosis 

All pregnancies must undergo routine obstetric anatomic ultrasound examination in the second trimester to detect cardiac anomalies. Certain pregnancies are influenced by maternal and/or foetal factors that elevate the likelihood of foetal cardiac disease, thus foetal echocardiography is recommended in such cases. The majority of congenital heart disease occurs in low-risk pregnancies.⁵  

Preparation for surgery

• The stomach of your child must be empty before the operation. The nurse will inform you when your son/daughter should stop eating and drinking. Please consult the medication section for information regarding which ones you are required to discontinue for your child before your surgery.
• It is necessary to bathe your child in the evening for five minutes, ensuring that the child is thoroughly cleaned. 
• It is necessary to ensure that your child is as comfortable as possible during his/her hospital stay.⁶ 

Surgical management

The surgical management of congenital heart defects involves various procedures, each tailored to address specific anatomical abnormalities and functional issues. The type of surgery depends on the specific congenital heart defect and its severity. Here are some common surgical interventions for congenital heart defects:

• Open-Heart Surgery:

Many congenital heart surgeries involve opening the chest to access the heart. This is typically done through a median sternotomy or a thoracotomy, depending on the specific defect.

• Cardiopulmonary Bypass:

During open-heart surgery, the patient is connected to a heart-lung machine, which takes over the pumping action of the heart and allows surgeons to temporarily stop the heart. This facilitates the repair of structural abnormalities.

• Repair or Reconstruction:

Surgeons may repair or reconstruct heart structures to restore normal blood flow and function. This can involve closing holes, repairing or replacing valves, and reconstructing vessels.

• Septal Defect Closure:

Atrial septal defects (ASD) and ventricular septal defects (VSD) are common congenital heart defects. Surgery involves closing the abnormal openings in the septum.

• Valve Repair or Replacement:

Abnormalities in heart valves may require repair or replacement. Valve repair is preferred when possible to preserve the patient's tissue.

FAQs

Can you live a normal life with congenital heart disease?

Yes, you can live a normal life with congenital heart disease.

Can congenital heart defects be fixed?

Congenital heart defects are often treated in childhood.

What is the most common treatment for congenital heart defects?

Open heart surgery is the most common treatment for congenital heart defects.

What is the recovery time for a congenital heart defect?

The majority of congenital heart defects improve with time and do not require any form of treatment.

Summary

Congenital surgery for congenital heart defects repairs or treats a heart defect that is present in a child. Congenital heart defects are often diagnosed during prenatal screenings or shortly after birth. Surgical interventions become necessary when an abnormality poses a risk to the child’s sustained health or overall welfare. 

Types of Congenital Heart Defects include Aortic Valve Stenosis, Atrial Septal Defect, Coarctation of Aorta, Complete Atrioventricular Canal Defect, d-transposition of the great arteries, I-transposition of the great arteries, Ebsteins Anomaly, Pulmonary Valve Stenosis, Single Ventricle Defect, Tetralogy of Fallot, Total Anomalous Pulmonary connection, Truncus Arteriosus, Ventricular Septal Defect. Fetal Diagnosis and Parental Diagnosis are done for the assessment of congenital Heart Defects. 

The surgical management of Congenital Heart Surgery includes Open Heart Surgery, Cardiopulmonary Bypass, Repair and Reconstruction, Septal Defect Closure, Valve Replacement or Repair.