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Conditions We Treat Part I I: Congenital Heart Diseases

Heart disease is not exclusive to adults; it can also affect children, although in different ways. The difference between adult and pediatric cases is in the underlying cause. For adults, heart disease primarily stems from atherosclerosis, a process where plaque accumulates within the coronary arteries, potentially leading to diminished blood flow and, in severe cases, heart attacks. However, in children, heart disease takes a different form, rarely involving plaque buildup. Instead, it often manifests as defects in the heart's walls and valves, disrupting the normal flow of blood. Unlike adults, who may develop heart disease later in life, children with heart conditions are typically born with these issues, known as congenital heart disease.


Today, we will dive into the intricacies of congenital heart diseases, shedding light on various conditions that impact newborns. These conditions, present at birth, can significantly alter the heart's function, affecting crucial processes such as oxygen and nutrient transport, toxin elimination, and blood pressure regulation. 


Congenital heart diseases encompass a diverse range of conditions, so we’ll only focus on the main ones. By doing so, we can better comprehend the complexities of these conditions and appreciate the significance of the surgeries we sponsor. Join us as we explore the specifics of these conditions and their implications for the affected individuals!


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Atrial Septal Defect

What is it? 

An abnormal opening or hole in the atrial septum, which is the wall that separates the two upper chambers of the heart, the right and left atria. This opening allows blood to flow between these chambers, which can lead to certain physiological changes.


How does it change the physiology of the heart? 

An atrial septal defect disrupts the normal separation of the right and left atria. As a result, oxygen-rich blood from the left atrium can mix with oxygen-poor blood from the right atrium, which then gets pumped into the lungs and the body. This can increase the volume of blood that the right atrium and ventricle have to handle.


What are the manifestations? 

While many ASDs are not immediately life-threatening, they can lead to complications over time, including an increased risk of developing pulmonary hypertension (high blood pressure in the lungs). This, in turn, can strain the right side of the heart and potentially lead to heart failure.


What are the treatment options? 

The choice of treatment depends on factors such as the size and location of the defect, the patient's age, and overall health. Early intervention is often recommended to prevent potential complications from worsening. Procedures to correct ASD include: 


Catheter-based Closure: In this minimally invasive procedure, a catheter is inserted through a blood vessel and guided to the heart. An ASD closure device is then placed to cover the hole, allowing tissue to grow over it and effectively close the defect.


Surgical Repair: For larger or more complex ASDs, open-heart surgery might be necessary. During the surgery, the hole is closed using sutures or a patch.

Coarctation of the Aorta

What is it? 

A narrowing or constriction of the aorta, the main artery that carries oxygenated blood from the heart to the rest of the body. This narrowing restricts blood flow and can lead to increased pressure in the heart and blood vessels.


How does it change the physiology of the heart? 

Coarctation of the aorta affects the normal distribution of blood throughout the body. Blood flow leaving the heart is impeded by the narrowing in the aorta, leading to increased pressure upstream from the constriction and decreased pressure downstream. This can strain the heart and lead to changes in blood flow to various organs.


What are the manifestations? 

Untreated coarctation of the aorta can lead to serious complications such as heart failure, enlargement of the heart, aneurysms (bulges in the weakened aorta), and even aortic rupture.


What are the treatment options? 

The treatment approach for coarctation of the aorta depends on the severity of the narrowing. Mild cases might be managed with medications to control blood pressure. However, more significant narrowings usually require intervention. Procedures to correct coarctation of the aorta include:


Balloon Angioplasty: In this minimally invasive procedure, a catheter with a balloon on its tip is inserted through a blood vessel and guided to the site of the narrowing. The balloon is then inflated to widen the narrowed area.


Surgical Repair: For more complex cases, surgical repair might be necessary. This involves removing the narrowed segment of the aorta and reattaching the two healthy ends.

Pulmonary Atresia

What is it? 

A congenital heart defect in which the pulmonary valve, which is responsible for allowing blood to flow from the right ventricle to the pulmonary artery and then to the lungs, is either absent or abnormally developed. As a result, blood flow to the lungs is severely restricted, and the right ventricle must find alternative ways to pump blood to the lungs.


How does it change the physiology of the heart? 

Pulmonary atresia disrupts the normal flow of blood from the heart to the lungs. With the pulmonary valve blocked or underdeveloped, blood cannot flow through its usual pathway to reach the lungs for oxygenation. This forces the right ventricle to pump blood using alternate routes, such as through other blood vessels, to supply oxygen to the body.


What are the manifestations? 

Pulmonary atresia can be life-threatening because it reduces the amount of oxygenated blood that reaches the body. In some cases, the right ventricle may become severely strained or damaged, leading to heart failure.


What are the treatment options? 

The treatment approach for pulmonary atresia depends on the specific anatomy of the defect, the patient's overall health, and the presence of associated heart defects. Procedures to address pulmonary atresia include:


Surgical Shunt: In some cases, a surgical shunt might be placed to create a pathway for blood flow to reach the lungs. This is a temporary measure that allows for oxygenation of blood until more definitive treatment can be performed.


Cardiac Catheterization: Some forms of pulmonary atresia can be treated using catheter-based interventions. Balloon valvuloplasty might be performed to open the blocked valve or create a new pathway for blood flow.


Surgical Repair: In more complex cases, open-heart surgery might be necessary to reconstruct or replace the pulmonary valve, create pathways for blood flow, and improve circulation.

Tetralogy of Fallot

What is it? 

Tetralogy of Fallot is a complex congenital heart defect that involves a combination of four distinct heart abnormalities. These four abnormalities are:


Ventricular Septal Defect (VSD): A hole in the wall that separates the two lower chambers (ventricles) of the heart.


Pulmonary Stenosis: Narrowing of the pulmonary valve or the pulmonary artery, which restricts blood flow to the lungs.


Right Ventricular Hypertrophy: Thickening of the right ventricle's muscular wall due to the increased workload.


Overriding Aorta: The aorta, the main artery that carries oxygenated blood to the body, is positioned above the VSD, allowing it to receive blood from both ventricles.


How does it change the physiology of the heart? 

Tetralogy of Fallot significantly alters the normal blood flow and oxygenation processes. The combination of a ventricular septal defect and pulmonary stenosis means that blood can flow from the right ventricle to the left ventricle through the VSD and mix with oxygenated blood. The overriding aorta further contributes to this mixing. Additionally, the right ventricular hypertrophy occurs as the heart's right side works harder to pump blood against the obstruction.


What are the manifestations? 

Tetralogy of Fallot can be life-threatening, particularly in severe cases. The degree of pulmonary stenosis and the size of the ventricular septal defect can impact blood flow and oxygenation, leading to significant strain on the heart and increased risk of sudden cardiac events.


What are the treatment options? 

Definitive treatment for Tetralogy of Fallot typically involves surgical repair to correct the various abnormalities and improve blood flow. The most common surgical procedure is:


Complete Repair: This procedure involves closing the ventricular septal defect and relieving the pulmonary stenosis. Depending on the specific anatomy, the repair may involve widening the pulmonary valve or replacing it. The goal is to redirect oxygen-poor blood from the right ventricle to the lungs for oxygenation.

Transposition of the Great Arteries 

What is it? 

A congenital heart defect in which the two main arteries leaving the heart, the aorta and the pulmonary artery, are switched in position. This means that oxygen-poor blood from the body is pumped back to the body, and oxygen-rich blood from the lungs is sent back to the lungs, resulting in two separate and parallel circulatory systems.


How does it change the physiology of the heart? 

TGA disrupts the normal flow of blood between the heart and the body. Normally, oxygen-poor blood returns from the body to the right atrium and then gets pumped to the lungs for oxygenation before returning to the left atrium and being pumped to the body through the aorta. However, in TGA, the two circulatory systems operate independently, with one carrying oxygen-poor blood to the body and the other carrying oxygen-rich blood to the lungs.


What are the manifestations? 

TGA can be life-threatening due to the lack of adequate oxygenation of the body's tissues. Without treatment, it can lead to severe hypoxemia (low oxygen levels) and complications such as heart failure.


What are the treatment options? 

TGA requires surgical intervention shortly after birth to correct the abnormal positioning of the arteries and establish a normal circulation pattern. The specific surgical approach depends on the presence of other heart defects and the anatomy of the condition. Procedures to address TGA include:


Arterial Switch Operation: This is the most common surgical procedure for TGA. It involves repositioning the aorta and the pulmonary artery to their correct positions. The coronary arteries are also reattached to the aorta. This allows oxygen-rich blood to flow from the left ventricle to the body and oxygen-poor blood to flow from the right ventricle to the lungs.

Truncus Arteriosis

What is it? 

The failure of the normal separation of the aorta and pulmonary artery during fetal development. Instead of the two separate arteries, there is a single large vessel that arises from both ventricles and receives blood from both the left and right ventricles. This leads to mixing of oxygenated and deoxygenated blood.


How does it change the physiology of the heart? 

Truncus arteriosus disrupts the usual separation of blood flow. Oxygen-rich blood from the left ventricle and oxygen-poor blood from the right ventricle mix together in the single large vessel (the truncus arteriosus). This mixed blood is then sent to the body and the lungs, leading to inefficient oxygenation and circulation.


What are the manifestations? 

Truncus arteriosus can be life-threatening due to the mixing of oxygen-rich and oxygen-poor blood, which leads to insufficient oxygen supply to the body's tissues. Additionally, the heart's pumping efficiency is compromised, which can lead to heart failure and other complications.


What are the treatment options? 

Truncus arteriosus requires surgical intervention to correct the abnormal connection between the ventricles and to establish a more normal blood flow pattern. The specific surgical approach depends on the severity of the defect and the presence of other heart abnormalities. Procedures to address truncus arteriosus include:


Complete Repair: This surgical procedure involves creating separate pathways for blood flow from the left ventricle to the body and from the right ventricle to the lungs. A patch is used to close the large vessel (truncus arteriosus), and the pulmonary artery is connected to the right ventricle.

Ventricular Septal Defect

What is it? 

A common congenital heart defect characterized by a hole in the septum that separates the two lower chambers (ventricles) of the heart. This hole allows blood to flow between the ventricles, potentially leading to changes in blood circulation and oxygenation.


How does it change the physiology of the heart? 

A ventricular septal defect creates an abnormal communication between the two ventricles. Oxygen-rich blood from the left ventricle can mix with oxygen-poor blood from the right ventricle through the VSD. As a result, the heart pumps a mixture of oxygenated and deoxygenated blood, which can affect the overall efficiency of oxygen delivery to the body.


What are the manifestations? 

While many VSDs might not pose an immediate life-threatening risk, larger defects can lead to complications such as heart failure, pulmonary hypertension (high blood pressure in the lungs), and an increased risk of developing infections in the heart.


What are the treatment options? 

Treatment for ventricular septal defects depends on the size of the defect, its impact on blood circulation, and the presence of symptoms. Some small VSDs might close on their own as the child grows. For larger defects or those causing symptoms, procedures to fix VSDs include:


Surgical Repair: Open-heart surgery is often performed to close the hole using a patch or sutures. The patch or sutures cover the hole in the septum, preventing the abnormal blood flow between the ventricles.


Catheter-Based Closure: In certain cases, transcatheter techniques might be used to close the VSD using a catheter and a device that is placed to seal the hole.




Pictured above is Quynh Anh, a young girl whom Healing Hearts Vietnam recently had the pleasure of sponsoring for surgical repair of an atrial septal defect that caused right heart failure. Since the procedure, she has done very well and is feeling much better!


Hopefully, this article can act as a good resource for better understanding the intricacies of congenital heart conditions like Quynh Anh's. We will be updating you soon with the next part of our series!


It is your contributions that allow us to continue sponsoring economically disadvantaged families in need of life-saving cardiovascular surgery. Thank you for your continued support in helping us work towards our mission!



Best,

Aaron


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