Paediatric Cardiology

Pulmonary valve stenosis narrows the valve between the right ventricle and the lung arteries. This may occur due to stiffening or hardening of the valve that results in reduced flow of blood to the heart through the valve. It is a congenital condition affecting the heart. However, it may occur in adults as a complication of other medical ailments. The stenosis or narrowing may range from mild to severe, with mild form presenting no clinical symptoms and may be diagnosed on a routine check-up. However, moderate to severe forms of pulmonary valve stenosis may present clinical symptoms and require aggressive treatment options. These include valve repair or replacement procedures. Symptoms A mild form of pulmonary valve stenosis shows no symptoms. However, moderate and severe forms vary in clinical presentation depending on the extent of the block of blood flow to the heart. Some signs and symptoms of pulmonary valve stenosis are: Shortness of breath Murmurs heard with a stethoscope Rapid heart rate Chest pain Lowered exercise tolerance Fatigue Dizziness or fainting Heavy or rapid breathing Swelling in the feet, ankles, face, eyelids, and abdomen. Cyanosis or blue discolouration seen on the lips, fingers, and toes Younger children or infants may appear cyanotic or blue due to the reduced flow of blood in the body. Causes Pulmonary valve stenosis is a congenital heart valve disease usually diagnosed at birth or early childhood. However, it can also be diagnosed later in life, resulting from many factors related to heart disease. In addition, pulmonary valve stenosis occurs in 2-3% of people with other congenital heart defects, such as: Tetralogy of Fallot: It is a condition in which the normal blood flow to the heart is impaired. Pulmonary atresia: A defect in the pulmonary valve, due to which the valve does not form, prevents blood from flowing to the lungs. Truncus arteriosus: The arteries are combined instead of being split into two, allowing oxygen-rich blood to blend with oxygen-poor blood. Aortic valve stenosis Atrial septal defect Ventricular septal defect Transposition of the major vessels Patent ductus arteriosus Other causes of pulmonary artery stenosis include: Rubella syndrome Williams syndrome, Alagille syndrome Takayasu's arteritis Problems that compress or apply pressure on the child's pulmonary artery from the outside. Surgical causes of pulmonary artery stenosis Some people may get pulmonary artery stenosis as a complication of surgery, such as: Transplant of the lungs Surgery to fix the congenital heart issue or to enhance the flow of blood through the patient's heart. Pulmonary artery banding purposely narrows the child's artery to lower blood flow to the lungs. Diagnosis Pulmonary valve stenosis, which often occurs as a congenital disability, is diagnosed at birth or in early childhood. A physical exam and the recording of the medical history and symptoms are done to diagnose pulmonary valve stenosis. In addition, doctors use a stethoscope to listen to the patient's heartbeats and look for a whooshing sound or murmur that is caused by turbulent blood flow across the narrowed valve. Doctors may advise certain tests to confirm pulmonary valve stenosis and evaluate the heart condition in adults and children alike, which include: Echocardiogram: An echocardiogram can confirm a diagnosis of pulmonary valve stenosis. In addition, it can help determine the severity of the condition. It is a painless test that records the electrical activity of the heart. Patches or electrodes are attached to various spots on the chest, wrist, and ankles. Its wires are connected to a computer that displays results. Chest X-ray: This shows the condition of the heart and lungs and helps determine if it has enlarged in size or shows any other damage. Stress test: Some kinds of tachycardia are triggered by exercise or strenuous physical activity. The heart's activity is monitored during a stress test while walking on a treadmill or riding a stationary bicycle. Catheterisation of the heart: Cardiac catheterisation allows a catheter to be directed through a blood vessel to the heart. This procedure can help diagnose or treat certain heart conditions, such as embolisms or arrhythmias. In addition, cardiac catheterisation provides essential information about the components of the heart, such as heart valves, heart muscles, and blood vessels. Other tests include: Computed tomography (CT): The advanced 3D CT technology provides doctors with an accurate evaluation of any morphological changes in the valve. Magnetic resonance imaging (MRI): In recent years, MRIs have proven to help evaluate valve pathology owing to the ability of MRI to assess the dynamics of the flow. Event monitoring: When long-term heart functioning readings need recording, event monitoring is advisable. Holter monitoring: Continuous monitoring of the heart rhythm can be done using this portable device. It can be used for several weeks. Pulmonary angiography: This test uses X-rays to examine how the blood flows through the valve and requires injecting contrast material into a vein. Healthcare providers use angiography to locate any structural changes in the valve and the extent of narrowing of the valve. Perfusion scan: This scan involves injecting a small amount of radioactive material to assess how well blood is flowing through each lung. Treatment Mild pulmonary valve stenosis requires only regular check-ups if no symptoms occur. However, intervention may be required to repair or replace the valve for moderate and severe pulmonary valve stenosis. The type of surgery depends on the valve's condition and the patient's overall health. The heart surgery procedures to treat pulmonary valve stenosis are: Balloon valvuloplasty Valvuloplasty improves blood flow through the heart and reduces the symptoms of pulmonary valve stenosis. However, the chances of relapse are high, which means that the valve may narrow again. Hence, in the future, some people will require valve repair or replacement. Pulmonary valve replacement If balloon valvuloplasty is contraindicated or cannot be performed, open-heart surgery or a catheter procedure may be done in order to replace the pulmonary valve. If other congenital heart defects prevail, those can also be repaired during the surgery. People who have undergone pulmonary valve replacement need to take prophylactic antibiotics before any invasive or dental procedures. This prevents chances of infection. Complications Some of the possible complications of pulmonary stenosis include: Infective endocarditis: People with heart valve problems like pulmonary valve stenosis carry an increased risk of developing bacterial infections of the heart, affecting its inner lining. Irregular heartbeat or arrhythmia: Irregular heartbeats may pose a threat if the valve prolapses, leading to severe regurgitation, followed by swelling of the heart chamber. Thickening of the heart muscle: The increased strain on the heart causes the heart walls to thicken, leading to right ventricular hypertrophy. Heart failure: If the right ventricle pumps inadequate blood, the heart eventually fails to function. Some of its symptoms include shortness of breath and swelling of the legs and abdomen. Pregnancy complications: The risks of complications during parturition and delivery are increased for women with severe pulmonary valve stenosis. Risk Factors Many conditions or factors contribute to causing pulmonary valve stenosis. Some of these include: Rheumatic fever: It is a complication of strep throat that can cause permanent damage to the heart and its valves. Furthermore, it can increase the risk of developing pulmonary valve stenosis later in life. Noonan syndrome: It is a genetic disorder that damages the heart and its valves and is one of the causes of pulmonary valve stenosis. Carcinoid syndrome: Carcinoid syndrome is a rare cancerous tumour that releases chemicals into the bloodstream that cause shortness of breath, flushing, and other symptoms. Furthermore, it may damage the valves of the heart when the syndrome spreads and involves the heart. German measles (rubella): Having rubella during pregnancy adds to the risk of developing pulmonary valve stenosis in the baby since it can be vertically transmitted. History of radiation therapy to the chest Prognosis The prognosis of treating pulmonary stenosis is good. With the advancement in technology, it is possible to prolong a person's life depending on the patient's condition. However, the prognosis is compromised in patients with multiple medical conditions and pulmonary valve stenosis. Review Reviewed by Dr. Mily Ray, Consultant - Paediatrics Cardiology, Paediatric (Ped) Cardiology.

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A single large vessel known as truncus arteriosus exits the heart during the fetus's development. Truncus arteriosus is a rare condition of the heart that is congenital or occurs at birth. It occurs when a single blood vessel comes out of the ventricles instead of the usual two -known as the pulmonary artery from the right ventricle and the aorta from the left ventricle. In babies with truncus arteriosus, this single large vessel never divides into two separate vessels. As a result, the wall separating the blood vessels is not closed completely. Babies with truncus arteriosus often have ventricular septal defects (VSD), which is a hole in the heart wall. This congenital heart defect allows too much blood to go into the lungs, which forces the heart and lungs to work harder. Truncus arteriosus is a life-threatening condition and requires surgery shortly after birth. Symptoms Babies born with truncus arteriosus often show signs of distress soon after birth. This is primarily because the heart works too hard to pump blood to the body. Some of the symptoms of this congenital heart defect include: Poor feeding Shortness of breath Pounding heart Rapid breathing Blue colouring of the skin Excessive sleepiness Poor growth Fatigue Widening of the fingertips Sweating Heart murmur Unresponsiveness Causes The exact cause of the truncus arteriosus is not known at the moment. With truncus arteriosus, a single artery comes out of both the right and left ventricles. There is also a hole between the two ventricles that causes the oxygen-poor and oxygen-rich blood to mix up. Some of this mixed blood goes to the lungs, and some to the rest of the body. However, in some cases, the mixed blood goes to the lungs more often, which causes a build-up of extra fluid around the lungs. This makes it difficult for the heart to pump blood from the lungs. This may also cause pulmonary hypertension, which is a life-threatening condition. It can also become inoperable due to obstructive PAH. Diagnosis Truncus arteriosus is usually diagnosed at birth. The doctor performs a physical examination to check for any heart murmur or sees a blue tint on the lips or skin. The doctor may advise the following tests to confirm the severity of truncus arteriosus. These may include: Echocardiogram: This is the preliminary test done to diagnose truncus arteriosus. During the procedure, the surgeon uses sound waves to produce heart images on a monitor. The test shows the single large vessel that leads from the heart or any other anomalies. Chest X-ray: The chest X-ray helps see the heart and lungs to check whether there is an accumulation of fluid in the lungs. Cardiac Catheterization: In this test, the medical expert inserts a catheter into the blood vessel and guides it to the heart. A dye is then released to check for any hole in the wall, along with the presence of a single large vessel. Electrocardiogram: The test is done to measure the duration and timing of the heartbeats. Treatment In most cases, surgery is the only option to treat the truncus arteriosus condition. However, depending on the condition of the baby, the doctor may suggest the following treatment: Surgery Most infants suffering from truncus arteriosus need to have surgery in the first few weeks after birth. During the open-heart surgery, the surgeon makes incisions around the chest and places a patch to close the hole in the heart wall. The surgeon then separates the pulmonary arteries from the trunk and connects them to the heart's right ventricle using special kinds of conduits (tubes). The surgeon then reconstructs the single large vessel and creates a new aorta connected to the left ventricle. After the surgery, the child needs life-long follow-up care with a cardiologist. The child will be given antibiotics and other medications. A child may need two or three surgeries to replace the conduits as their heart and body grow. Medications Some of the medications that may be necessary for the child includes: Diuretics prevent fluid from building in the body by increasing the frequency and volume of urination. Angiotensin-converting enzyme inhibitors make it easier for the heart to pump blood. Decongestants to help strengthen the heart muscle. Risk Factors Although the exact reason for truncus arteriosus is unknown, several factors may increase the risk of babies born with a heart condition. These may include: Viral illness during pregnancy Poorly-controlled diabetes during pregnancy Smoking Alcohol abuse during pregnancy Obesity Chromosomal disorders Certain medications Prevention In most cases, truncus arteriosus defect cannot be prevented. However, one can take a few steps before and during the course of pregnancy to ensure the baby's health. These may include: Get vaccination before pregnancy. Certain viruses like German measles can be harmful to the baby during pregnancy. So, it is essential to get vaccinated before planning to get pregnant. Take folic acid. Taking 400 micrograms of folic acid daily helps prevent congenital disabilities. Control diabetes. Women with diabetes during pregnancy may result in heart defects in the baby. So, it is advisable to talk to the doctor and learn how to control diabetes during pregnancy. Complication Truncus arteriosus can cause some severe complications with blood circulation. This is primarily because the blood is mixed and exits from a single vessel, making it harder for the heart to pump it to the body. Babies suffering from truncus arteriosus have a higher risk of the following complications: Heart Failure Endocarditis Cardiomegaly Respiratory Problems Pulmonary Hypertension Heart Valve Problems Lung Problems Poor Growth and Development Outlook for Patients The outlook for babies that have gone through complete surgical repair of the heart often has good results. The child will have an active life, though he may be under continuous observation by a pediatric cardiologist. Therefore, the child will need to see a cardiologist throughout their life. That said, untreated cases of truncus arteriosus can be fatal, and the child will not be able to survive during the first year of life. Review Reviewed by Dr. Mily Ray, Consultant, Paediatric (Ped) Cardiology.

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Wolff-Parkinson-White syndrome is an extra signalling pathway between the upper and lower chambers of the heart that causes a rapid heartbeat (tachycardia). It is also known as preexcitation syndrome. This condition is a congenital heart defect, which means that it has been present since birth. However, the syndrome is relatively rare. WPW syndrome is not life-threatening but poses a risk of a serious heart problem. Rarely, it may cause sudden cardiac death in children and young adults. Symptoms of Wolff-Parkinson-White Syndrome Symptoms of Wolff-Parkinson-White syndrome may include one or more of the following: Racing feeling in the chest. Shortness of breath (dyspnea) during fast heartbeats. Heart palpitations – a sudden pounding, fluttering. Dizziness – feeling lightheaded or faint. Anxiety Fatigue Rarely, cardiac arrest (sudden death). Signs and symptoms seen in infants include: Pale or faded skin colour (pallor). Restlessness or irritability. Rapid breathing. Blue or grey discolouration of the skin, lips, and nails (cyanosis). Poor nutrition Some people have WPW without any symptoms, and the condition is known as WPW pattern. This pattern is detected on routine ECG. Diagnosis of Wolff-Parkinson-White Syndrome Cardiac tests allow the detection of unusually rapid heartbeats and help assess conditions that may cause arrhythmias. The tests include: Electrocardiogram: This quick and painless test measures the heart's electrical activity. During an ECG, sensors are placed in different positions on the chest, arms and legs. It helps assess the signal patterns of the heart. This helps determine the type of tachycardia and what may be causing the rapid heart rate. Some of the latest personal devices, like smartwatches, also monitor electrocardiograms. Event monitoring: Event monitoring is advisable when long-term heart functioning readings need recording. Holter monitoring: Continuous monitoring of the heart rhythm for 24 hours can be done using this portable device. It can be used for several days if needed. Echocardiogram: This uses sound waves to form images of the heart in motion. It helps assess issues with the flow of blood, heart valves, and heart muscle. Echo will define cardiac anatomy to rule out structural defects and assess ventricular function. Chest X-ray: It helps evaluate the status of the lungs and heart. Cardiac magnetic resonance imaging (MRI): This test provides still or moving pictures of blood flow through the heart and helps determine the cause of ventricular tachycardia or ventricular fibrillation. MRI is needed in selective cases of arrhythmia. It provides information about the cardiac structure and function to look for causes of arrhythmia. Electrophysiological (EP) testing and mapping: Also called an EP study, helps confirm a tachycardia diagnosis or determine where the faulty signalling occurs in the heart. An EP study is mainly used to diagnose isolated arrhythmias. Treatment for Wolff-Parkinson-White Syndrome The treatment for Wolff-Parkinson-White (WPW) syndrome depends upon the severity and frequency of symptoms. Additionally, factors such as the type of heart rhythm problem (such as arrhythmia) causing the fast heart rate also contribute to deciding on a treatment plan. The treatment goals are to slow a fast heart rate when it occurs and prevent future episodes. Treatment options for a rapid heart rate include: Vagal manoeuvres: Vagal manoeuvres such as coughing, sitting down, and having a bowel movement all affect the vagal nerve, which lowers heart rate. During these positions, specific actions must be performed when a rapid heartbeat episode occurs. Cardioversion is a procedure that can help rectify irregular heartbeats. It is performed when medicines fail to regularize heart rhythm or the child is sick. This method uses electrical impulses to normalize heart rhythm. Medications: If vagal manoeuvres fail to control a rapidly beating heart, medicines can help improve the condition and restore heart rhythm. Intravenous medicines act faster and are the chosen route of drug administration. Catheter ablation: Radiofrequency waves are used to destroy the abnormal pathway (accessory pathway) after defining by electrophysiology study. Lifestyle and Home Remedies Doctors recommend following a heart-healthy lifestyle for patients with Wolff-Parkinson-White (WPW) syndrome or episodes of a fast heart rate. Take these steps: Avoid caffeine or other stimulants Don't smoke Limit or avoid alcohol Maintain a healthy weight Eat a healthy diet Get regular exercise Manage emotional stress Complications of Wolff-Parkinson-White Syndrome Some complications of Wolff Parkinson's White syndrome include the following: Tachyarrhythmia Dizziness or syncope Complications of drug therapy (e.g., arrhythmia) Palpitations Sudden cardiac death Complications associated with invasive procedures and surgery. Recurrence Risk Factors of Wolff-Parkinson-White Syndrome Risk factors that place a patient at higher risk for sudden cardiac death include: Male gender Septal location of the accessory pathway. History of atrial fibrillation or AVRT. Age less than 35 years. Multiple accessory pathways. Rapid anterograde conduction of the accessory pathway. Prognosis of Wolff-Parkinson-White Syndrome Once identified and treated, Wolff Parkinson's White syndrome has an excellent prognosis. Furthermore, it has the potential for a permanent cure with radiofrequency (RF) catheter ablation therapy. Asymptomatic patients with only preexcitation on ECG also have a very good prognosis. However, many develop symptomatic arrhythmias over time, which may be prevented with prophylactic EPS and RF catheter ablation. People who have a history of a sudden heart attack in their family or symptoms of tachyarrhythmias or cardiac arrest have the worst prognosis. However, the prognosis is excellent if timely treatment is provided, including curative ablation. Review Reviewed by Dr. Munesh Tomar - Director, Paediatric (Ped) Cardiology, Cardiac Sciences, Cardiology.

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