Tricuspid Atresia is a congenital heart defect in which the tricuspid valve does not form, preventing normal blood flow from the right atrium to the right ventricle. The right ventricle is often underdeveloped, and babies usually have an opening between the atria, such as an atrial septal defect, to allow blood to reach the left side of the heart. Other defects, including a ventricular septal defect or patent ductus arteriosus, may also help maintain blood flow to the lungs; symptoms and treatment depend on the amount of pulmonary blood flow and associated heart abnormalities.
Timely diagnosis and treatment are essential because tricuspid atresia can cause low oxygen levels, inadequate blood flow to the lungs, or excessive pulmonary blood flow that may damage the lung blood vessels. Early treatment helps support oxygen delivery, maintain appropriate blood flow, and reduce the risk of serious complications. Lifelong follow-up with a congenital heart specialist is necessary because complications may develop even after successful surgical treatment.
Causes
Risk Factors
Recent advances in treating tricuspid atresia in Malaysia include improved fetal and postnatal echocardiography, specialised paediatric cardiac intensive care, and staged surgical procedures tailored to the child's heart anatomy. Treatment may involve an initial shunt or other procedure to manage blood flow, followed by the bidirectional Glenn procedure and Fontan operation to improve circulation in children with single-ventricle physiology. Long-term monitoring helps detect complications such as abnormal heart rhythms, reduced heart function, or problems with the Fontan circulation. The treatment plan depends on the child's anatomy, oxygen levels, and overall condition.
Ductal Stenting: In selected babies, a stent may be placed in the ductus arteriosus to maintain pulmonary blood flow and reduce the need for an early surgical shunt.
Systemic-to-Pulmonary Artery Shunt: A surgeon may create a shunt to increase blood flow to the lungs when the baby's anatomy requires more pulmonary blood flow.
Balloon Atrial Septostomy (BAS) : A cardiologist performs a BAS (also known as a Rashkind procedure). They insert a thin, flexible tube called a catheter into a blood vessel, usually in the groin, and thread it up to the heart.
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Pulmonary Artery Banding: If too much blood reaches the lungs, doctors may use pulmonary artery banding in selected patients to control pulmonary blood flow and protect the pulmonary circulation.
Bidirectional Glenn Operation : In this staged procedure, the superior vena cava is connected directly to the pulmonary arteries, allowing blood from the upper body to flow to the lungs without passing through the right ventricle.
Fontan Procedure : The final major stage of palliation connects venous blood from the lower body to the pulmonary arteries, allowing blood to reach the lungs without ventricular pumping. Selected patients may undergo a fenestration.
Heart Transplantation : Rarely, transplantation may be considered when the heart becomes severely weakened, the Fontan circulation fails, and other treatments are unsuitable.
MediRehab (a chain of Rehab centres, part of MediGence) provides comprehensive tele-rehabilitation services designed to support Tricuspid Atresia patients in Malaysia. These services include:









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Interventional Cardiologist
29 Years of Experience
With a rich experience of more than 29 years, Dr. Nityanand has gained a vast spectrum of skills and expertise in performing all types of complex cardiac procedures. He is an expert in clinical & interventional cardiology and electrophysiology. He studied his medicine (MBBS) and MD from the Institute of Medical Sciences of Banaras Hindu University, Varanasi, and later completed his DM in Cardiology from the prestigious King George Medical University, Lucknow. View More
Last Reviewed - January 2026