Introduction
Pulmonary atresia with ventricular septal defect (VSD) is a cyanotic congenital heart condition. It is caused due to under or maldeveloped right ventricular outflow tract with pulmonary valve and tract atresia. In this condition, the person affected will be missing a functional pulmonary valve that helps to move the blood to the pulmonary artery. The blood moves from the pulmonary artery to the lungs, where it takes in oxygen. In the absence of this valve, the blood will not get enough oxygen to the cells.
What Is Meant by Pulmonary Atresia?
Pulmonary atresia is a congenital heart disease that is present at birth. In this condition, the pulmonary valve does not develop properly or is blocked after birth. Without a proper pulmonary valve, blood cannot move through the pulmonary artery to reach the lungs, where it gets oxygen. In this condition, oxygen-poor blood gets circulated throughout the body.
The pulmonary artery is on the right side of the heart, between the right ventricle and the pulmonary artery (which carries blood to the lungs). The pulmonary artery helps blood flow from the right ventricle to the pulmonary artery.
How Does Pulmonary Atresia Affect the Newborn?
The newborn affected by this condition may have the following:
-
Due to lack of oxygen, the lips, fingers, and toes may look blue.
-
Improper development of right ventricle and tricuspid valve.
-
Foramen ovale, which is an opening situated between the right atrium and left atrium, should be closed; instead, it is left open. This causes oxygen-poor blood to move from the right to the left atrium, and then the blood flows through the left ventricle to the aorta.
Newborns with this condition will have issues with a dividing wall in the heart, and this wall helps the blood to flow where it is supposed to flow.
What Are the Types of Pulmonary Atresia?
-
Pulmonary Atresia With a Ventricular Septal Defect:
The wall opening separating the right and left ventricles causes a mix of oxygen-rich and oxygen-poor blood.
-
Pulmonary Atresia With a Ventricular Septum:
The wall which separates the right and left heart is intact.
What Are the Symptoms of This Condition?
Some common symptoms of pulmonary atresia are:
-
Rapid breathing.
-
A bluish tinge over the lips, fingers, and toe.
-
Cool and pale skin.
-
Breathing problems.
-
Irritability.
-
Poor feeding.
What Are the Cause of Pulmonary Atresia?
The exact cause of this condition is unknown. It occurs during the first eight weeks when the fetus develops in the uterus.
How Is Pulmonary Atresia With Ventricular Septal Defect Diagnosed?
This condition is diagnosed at birth or soon after when certain tests are performed, these are:
-
Pulse Oximeter:
A pulse oximeter helps monitor the amount of oxygen in the blood through a small sensor attached to the hand or foot. This procedure is simple and painless.
-
Chest X-Ray:
Chest X-ray reveals the size of the heart and lungs.
-
Echocardiogram:
In this procedure, sound waves are used to form the image of the heart. This procedure performed on the mother's belly before delivery helps diagnose pulmonary atresia of the fetus inside the mother's womb.
-
Electrocardiogram ((ECG or EKG):
This procedure is very simple and measures the electric activity of the heart. The heartbeat is monitored through sticky patches called electrodes. A monitor connected displays the result. This procedure reveals how fast and slow the heart beats and helps diagnose irregular heart rhythms.
-
Cardiac Catheterization:
The cardiologist places a small tube called the catheter in the blood vessels in the arm or groin and to an artery to the heart and injects a dye through the catheter. This helps in a more proper image of the heart arteries on an X-ray image.
-
Cardiac CT Scan (Computed Tomography):
This procedure is used to take pictures of the heart and blood vessels. It reveals the size and shape of the heart and lungs and helps to know about major aortopulmonary collateral arteries (MAPCAs) and plan for the treatment required.
How Can Pulmonary Atresia With Ventricular Septal Defect Be Treated?
The newborns affected with pulmonary atresia with ventricular septal defect require immediate treatment. Treatment procedures may include recommendations for medications, surgery, and other procedures.
-
Surgeries and Other Procedures:
The newborn affected with this condition requires one or more procedures and surgeries to improve the blood flow and heart function. This procedure is based on the structure of the arteries and the presence of any MAPCAs.
Some Common Surgeries Performed Are:
-
Catheter Method From the Pulmonary Artery:
In this procedure, the blood vessels coming out of the pulmonary artery are reconstructed. A balloon is placed at the catheter's tip, which helps widen any narrow region. A stent (a rigid tube) is placed between the aorta and pulmonary artery to open it, allowing the blood to flow to the lungs.
-
Systemic-To-Pulmonary Artery Shunt:
This procedure is required in the initial days of life to enhance blood flow to the lungs. This forms a connection for the blood to flow through the synthetic tube.
-
Neonatal Complete Repair:
If the newborn has properly developed pulmonary arteries and no MAPCAs, the doctor can perform a repair during the initial four weeks of life. During the surgery, the ventricular septal defect is closed, and an artificial valve with a tube is placed between the ventricle and the pulmonary artery.
-
One-Stage Complete Repair:
This procedure, also called uni focalization, is a procedure that is performed to connect every MAPCA to form a new pulmonary artery. The surgeon completes the repair procedure by closing the VSD. A graft forms a connection between the ventricles and the pulmonary artery. This procedure is performed within four to six months of age.
-
Staged Uni Focalization:
If MAPCs are small or have many narrowing regions, surgery is done stage-wise to correct them. This makes the arteries grow before complete repair.
When Should a Person See the Doctor?
The child should have regular follow-ups with the pediatric cardiologist. This starts after two to four weeks. Few children may need many heart catheterizations or may require special medication. Long-term complications may require:
-
Abnormal heart rhythm.
-
Narrowing of the pulmonary artery.
Conclusion
Pulmonary atresia is a congenital heart condition that may differ from person to person. This condition can be diagnosed at birth or after birth and can be treated through medications or surgical procedures. The proper medical situation of the child should be known, and regular follow-ups with the doctor are mandatory once the baby returns home. These visits can help the doctor determine any other complications that may develop.
Introduction
Pulmonary atresia with ventricular septal defect (VSD) is a cyanotic congenital heart condition. It is caused due to under or maldeveloped right ventricular outflow tract with pulmonary valve and tract atresia. In this condition, the person affected will be missing a functional pulmonary valve that helps to move the blood to the pulmonary artery. The blood moves from the pulmonary artery to the lungs, where it takes in oxygen. In the absence of this valve, the blood will not get enough oxygen to the cells.
What Is Meant by Pulmonary Atresia?
Pulmonary atresia is a congenital heart disease that is present at birth. In this condition, the pulmonary valve does not develop properly or is blocked after birth. Without a proper pulmonary valve, blood cannot move through the pulmonary artery to reach the lungs, where it gets oxygen. In this condition, oxygen-poor blood gets circulated throughout the body.
The pulmonary artery is on the right side of the heart, between the right ventricle and the pulmonary artery (which carries blood to the lungs). The pulmonary artery helps blood flow from the right ventricle to the pulmonary artery.
How Does Pulmonary Atresia Affect the Newborn?
The newborn affected by this condition may have the following:
-
Due to lack of oxygen, the lips, fingers, and toes may look blue.
-
Improper development of right ventricle and tricuspid valve.
-
Foramen ovale, which is an opening situated between the right atrium and left atrium, should be closed; instead, it is left open. This causes oxygen-poor blood to move from the right to the left atrium, and then the blood flows through the left ventricle to the aorta.
Newborns with this condition will have issues with a dividing wall in the heart, and this wall helps the blood to flow where it is supposed to flow.
What Are the Types of Pulmonary Atresia?
-
Pulmonary Atresia With a Ventricular Septal Defect:
The wall opening separating the right and left ventricles causes a mix of oxygen-rich and oxygen-poor blood.
-
Pulmonary Atresia With a Ventricular Septum:
The wall which separates the right and left heart is intact.
What Are the Symptoms of This Condition?
Some common symptoms of pulmonary atresia are:
-
Rapid breathing.
-
A bluish tinge over the lips, fingers, and toe.
-
Cool and pale skin.
-
Breathing problems.
-
Irritability.
-
Poor feeding.
What Are the Cause of Pulmonary Atresia?
The exact cause of this condition is unknown. It occurs during the first eight weeks when the fetus develops in the uterus.
How Is Pulmonary Atresia With Ventricular Septal Defect Diagnosed?
This condition is diagnosed at birth or soon after when certain tests are performed, these are:
-
Pulse Oximeter:
A pulse oximeter helps monitor the amount of oxygen in the blood through a small sensor attached to the hand or foot. This procedure is simple and painless.
-
Chest X-Ray:
Chest X-ray reveals the size of the heart and lungs.
-
Echocardiogram:
In this procedure, sound waves are used to form the image of the heart. This procedure performed on the mother's belly before delivery helps diagnose pulmonary atresia of the fetus inside the mother's womb.
-
Electrocardiogram ((ECG or EKG):
This procedure is very simple and measures the electric activity of the heart. The heartbeat is monitored through sticky patches called electrodes. A monitor connected displays the result. This procedure reveals how fast and slow the heart beats and helps diagnose irregular heart rhythms.
-
Cardiac Catheterization:
The cardiologist places a small tube called the catheter in the blood vessels in the arm or groin and to an artery to the heart and injects a dye through the catheter. This helps in a more proper image of the heart arteries on an X-ray image.
-
Cardiac CT Scan (Computed Tomography):
This procedure is used to take pictures of the heart and blood vessels. It reveals the size and shape of the heart and lungs and helps to know about major aortopulmonary collateral arteries (MAPCAs) and plan for the treatment required.
How Can Pulmonary Atresia With Ventricular Septal Defect Be Treated?
The newborns affected with pulmonary atresia with ventricular septal defect require immediate treatment. Treatment procedures may include recommendations for medications, surgery, and other procedures.
-
Surgeries and Other Procedures:
The newborn affected with this condition requires one or more procedures and surgeries to improve the blood flow and heart function. This procedure is based on the structure of the arteries and the presence of any MAPCAs.
Some Common Surgeries Performed Are:
-
Catheter Method From the Pulmonary Artery:
In this procedure, the blood vessels coming out of the pulmonary artery are reconstructed. A balloon is placed at the catheter's tip, which helps widen any narrow region. A stent (a rigid tube) is placed between the aorta and pulmonary artery to open it, allowing the blood to flow to the lungs.
-
Systemic-To-Pulmonary Artery Shunt:
This procedure is required in the initial days of life to enhance blood flow to the lungs. This forms a connection for the blood to flow through the synthetic tube.
-
Neonatal Complete Repair:
If the newborn has properly developed pulmonary arteries and no MAPCAs, the doctor can perform a repair during the initial four weeks of life. During the surgery, the ventricular septal defect is closed, and an artificial valve with a tube is placed between the ventricle and the pulmonary artery.
-
One-Stage Complete Repair:
This procedure, also called uni focalization, is a procedure that is performed to connect every MAPCA to form a new pulmonary artery. The surgeon completes the repair procedure by closing the VSD. A graft forms a connection between the ventricles and the pulmonary artery. This procedure is performed within four to six months of age.
-
Staged Uni Focalization:
If MAPCs are small or have many narrowing regions, surgery is done stage-wise to correct them. This makes the arteries grow before complete repair.
When Should a Person See the Doctor?
The child should have regular follow-ups with the pediatric cardiologist. This starts after two to four weeks. Few children may need many heart catheterizations or may require special medication. Long-term complications may require:
-
Abnormal heart rhythm.
-
Narrowing of the pulmonary artery.
Conclusion
Pulmonary atresia is a congenital heart condition that may differ from person to person. This condition can be diagnosed at birth or after birth and can be treated through medications or surgical procedures. The proper medical situation of the child should be known, and regular follow-ups with the doctor are mandatory once the baby returns home. These visits can help the doctor determine any other complications that may develop.
