Congenital Heart Disease (CHD)

Cyanotic congenital heart disease encompasses a group of heart defects present at birth, leading to decreased oxygen levels in the blood. As a result, a blueish tint, termed “cyanosis,” appears on the skin, lips and nails. These defects often involve abnormal mixing of oxygen-rich and oxygen-poor blood within the heart or reduced blood flow to the lungs.

This condition is characterized by the malformation of the tricuspid valve, the valve that separates the top and bottom right heart chambers. As a result, the valve does not seal as it should, causing blood to flow in reverse from the bottom to the top chamber, thereby overburdening the heart. Typically, people with Epstein’s anomaly have an enlarged heart, and when not treated adequately, it can progress to heart failure.

This is a severe congenital heart defect in which the left side of the heart is underdeveloped. This includes the left ventricle, mitral valve, aorta, and aortic valve. As a result, the left side of the heart cannot effectively pump oxygen-rich blood to the body. Infants with HLHS require multiple surgeries to reroute blood flow, and without early intervention, the condition is fatal.

IAA is a rare condition in which the aorta isn’t properly developed, preventing blood circulation throughout the infant’s body. The aorta is a large blood vessel that carries blood from the heart to the rest of the body. It’s normally shaped like a candy cane, but in this condition, that arch is not fully formed or disrupted.

This condition is characterized by the inability of the pulmonary valve, which enables blood to flow from the heart to the lungs, to develop abnormally. Rather than opening and closing to allow blood to travel from the heart to the lungs, it holds a solid tissue layer. As a result, blood cannot follow its usual route to obtain oxygen from the lungs. Instead, some blood reaches the lungs through alternative channels within the heart and its arteries.

A tetralogy of fallot (TOF) is a heart defect with four problems – a hole between the lower chambers of the heart, an obstruction from the heart to the lungs, the aorta (blood vessel) lies over the hole in the lower chambers, and the muscle surrounding the lower right chamber becomes overly thickened.

Total anomalous pulmonary venous return is a condition in which the pulmonary veins, which transport blood from the lungs, connect to an incorrect part of the heart, altering the usual vein connections. This misconnection results in the mixing of oxygen-depleted blood with oxygen-saturated blood.

This condition is when the two main arteries leaving the heart, the pulmonary artery and the aorta, are switched in position. This results in oxygen-rich blood being circulated back to the lungs while oxygen-poor blood flows to the rest of the body.

Similar to pulmonary atresia, this is a condition where the valve between the two right chambers fails to develop. Instead, a solid tissue barrier blocks the blood flow between the right chambers. Consequently, this anomaly results in limited blood flow through the heart, causing an underdeveloped right ventricle.

Truncus arteriosus is a rare, life-threatening congenital heart defect where a single large blood vessel, instead of the usual two separate vessels (aorta and pulmonary artery), emerges from the heart. This single vessel carries blood from the heart to both the lungs and the rest of the body. Additionally, there’s often a hole (ventricular septal defect) between the heart’s lower chambers. This combined abnormality means oxygen-rich and oxygen-poor blood mix, causing the heart to work harder and leading to potential health complications.

These conditions refer to a group of congenital heart defects where there is no significant mixing of oxygen-poor (blue) blood with oxygen-rich (red) blood within the heart. As a result, there isn’t a noticeable blue or purple tint to the skin, lips, or nails, a condition termed “cyanosis.” In these defects, blood flow is typically increased to the lungs, leading to symptoms like breathlessness or fatigue. Examples include atrial septal defect and ventricular septal defect.

Aortic valve stenosis is a condition where the heart’s aortic valve narrows, restricting blood flow from the heart to the aorta, and subsequently to the rest of the body. This narrowing can strain the heart, leading to thickening of the heart muscle, chest pain, and shortness of breath.

Atrial septal defect is characterized by a hole in the septum separating the heart’s atria. This allows oxygen-rich blood to mix with oxygen-poor blood, leading to an increased workload on the heart and potential complications if untreated.

This defect is marked by a large hole in the center of the heart, combined with issues in the heart’s valves. It results in the mixing of oxygen-rich and oxygen-poor blood, making the heart work harder and potentially causing enlargement or failure if not addressed.

Normally, the aortic valve has three leaflets or cusps. In the bicuspid aortic valve, there are only two. This can affect the valve’s functionality, leading to stenosis or leakage, which can strain the heart over time.

This is a congenital condition where a part of the aorta is narrower than usual, inhibiting proper blood flow. It can increase blood pressure and strain the heart, leading to potential complications if not treated.

Normally, the ductus arteriosus, a blood vessel in the fetus, closes after birth. If it remains open, it can lead to excessive blood flow to the lungs. This might result in heart failure if not corrected.

Before birth, the foramen ovale allows blood to bypass the lungs. After birth, it usually closes. If it remains open, it’s called a patent foramen ovale, allowing some blood to bypass the lungs, although many individuals remain symptom-free.

This condition involves the narrowing of the pulmonary artery, restricting blood flow from the heart to the lungs. It can lead to breathlessness, fatigue, and other complications if the blood flow becomes severely limited.

In this defect, the pulmonary valve, which allows blood to go to the lungs, is narrowed or obstructed. This restricts blood flow, making the heart pump harder, potentially leading to an enlarged heart or other issues.

This involves a hole in the wall separating the heart’s lower chambers (ventricles). This defect can cause oxygen-rich blood to mix with oxygen-poor blood, leading to potential heart strain and other complications if not treated.

During a physical examination, doctors check for signs of congenital heart disease, such as a heart murmur, cyanosis, or unusual pulses. An exercise stress test measures the heart’s response to physical activity. The patient exercises on a treadmill or stationary bike while their heart rate, breathing, and blood pressure are monitored to detect potential abnormalities.

Cardiac catheterization involves inserting a thin tube (catheter) into a blood vessel and guiding it to the heart. This provides valuable information about the heart’s chambers, blood flow, and pressures, allowing doctors to diagnose and sometimes treat heart defects.

A chest X-ray provides images of the heart and lungs. It can be used to check whether there’s an excess amount of blood in the lungs, or whether the heart is larger than normal, as both may be signs of heart disease. It can reveal signs of heart failure, lung issues, and structural abnormalities of the heart, aiding in the diagnosis of congenital heart disease.

An echocardiogram, also known as an echo, uses sound waves to produce detailed images of the heart’s structure and function. This painless and noninvasive test can show the size, shape and movement of heart valves and chambers, helping to identify any congenital anomalies or malfunctions.

There is no need for special preparation, except in the case of a child who needs a sedative. A sedative will only be given to the child who has difficulty staying calm and quiet during the test.

The technician will apply a small amount of transparent gel on the child’s chest and then scan their chest with the help of a 15 cm long probe. The image of the heart is obtained on the screen of the echocardiogram machine.

When the machine records the blood flow passing through the heart, you will be able to hear the heartbeat. During the test, your children may feel slight pressure caused by the probe passed over the chest by the technician.

The test takes about 30 minutes to complete.

This noninvasive test records the electrical activity of the heart. Sticky patches with sensors (electrodes) are placed on the skin of the chest. Wires connect the patches to a device, which displays results, detecting abnormal heart rhythms, indicating areas of damage, and providing insights into heart muscle function.

A test used to measure the oxygen level (oxygen saturation) in the blood. It is an easy, painless measure of how well oxygen is being sent to parts of your body furthest from your heart, such as the arms and legs. A small sensor is placed on the fingertip, and using light, it determines the percentage of oxygen in the blood. Too little oxygen may be a sign of a heart or lung problem.

A type of echo test that uses sound waves to create pictures of your heart. Unlike other types of echo tests, a TEE creates pictures from inside your body. This procedure involves passing a thin, flexible tube down the esophagus to get a more detailed image of the heart. A TEE can diagnose many problems, including blood clots and infections in your heart. It offers a closer view than a standard echocardiogram, especially useful for certain conditions or when assessing the heart’s back structures.

A cardiac CT scan provides detailed cross-sectional images of the heart. By using X-rays, it can highlight structural abnormalities, vessels, and surrounding tissues, offering a comprehensive view of the heart’s anatomy. This quick but detailed and high resolution scan shows your physician problems with your heart structure, valves, arteries, aorta and more.

An MRI of the heart uses magnetic fields and radio waves to create detailed 3D images of the heart and surrounding structures, allowing for more accurate measurements of the heart and its cavities. It can show congenital heart defects, measure the heart’s function and provide insights into blood flow, making it a valuable tool for diagnosis and treatment planning.

During the procedure, a thin flexible tube (catheter) is threaded through the blood vessels to the heart.

Diagnostically, it helps doctors understand the heart’s anatomy, measure pressures within the heart chambers, and observe blood flow patterns. Therapeutically, it allows for interventions such as widening narrowed blood vessels, closing off blood vessels or heart defects, and replacing faulty heart valves.

This minimally invasive approach often offers a shorter recovery time and less discomfort than open-heart surgery, making it a preferred choice for certain heart conditions.

Heart surgery remains a cornerstone in treating many congenital heart defects, especially those that can’t be addressed with catheterization alone. Surgeons can repair anomalies like holes in the heart, valve defects, and more complex structural abnormalities.

The type and approach of surgery depend on the nature of the defect. Open-heart surgery might involve using a heart-lung machine, which takes over the heart’s pumping action while surgeons work on the heart.

Sheba’s team has pioneered a distinctive, minimally invasive approach for specific congenital heart defects. With this method, our surgeons make a cosmetic incision below the breast, ensuring no visible scars in the future. Regardless of the method, the goal is to restore normal heart function and improve the patient’s quality of life.

While surgeries and procedures can rectify structural anomalies in the heart, medications play an essential role in managing symptoms and preventing complications in patients with congenital heart disease.

These drugs can regulate heart rhythms, lower blood pressure, reduce fluid accumulation, and prevent blood clots. For instance, diuretics can help eliminate excess fluid from the body, aiding patients with heart failure. Beta-blockers and ACE inhibitors can improve heart function and alleviate symptoms.

Additionally, anticoagulants might be prescribed to prevent blood clots, especially in conditions where blood flow is abnormal.