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Total Anomalous Pulmonary Venous Return (TAPVR) is a rare heart problem present at birth. Normally, the pulmonary veins carry oxygen-rich blood from the lungs to the left side of the heart. In TAPVR, these veins connect to the wrong place, sending the blood back to the right side instead. This causes the oxygen-rich blood to mix with oxygen-poor blood. As a result, the body doesn’t get enough oxygen, leading to breathing problems and a blue skin color. TAPVR is serious and needs surgery soon after birth to fix the blood flow and help the baby survive. Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare (Okereke & Sharma, 2018).
There are four main types of TAPVR, based on where the lung veins connect: supracardiac, cardiac, infracardiac, and mixed. In supracardiac TAPVR, the pulmonary veins drain into a vertical vein that connects to the superior vena cava, a large vein that brings blood from the upper body to the heart. In the cardiac type, the veins drain straight into the right atrium or a nearby vein called the coronary sinus. In infracardiac TAPVR, the veins connect to lower body veins like the portal vein, hepatic veins, or inferior vena cava. The mixed type happens when different lung veins connect in different wrong ways. In all of these types, oxygen-rich blood from the lungs goes to the right side of the heart instead of the left atrium, which means the body does not get the full amount of oxygen it needs to work properly. TAPVR is a cardiac malformation in which all pulmonary veins on either side are not connected to the left atrium, but directly to the somatic vein or the right atrium. Almost all patients with TAPVR present with symptoms of heart failure in the neonatal period, which require early surgical correction. It is unusual to encounter a adult patient with TAPVR due to low survival rate in untreated cases (Wang et al., 2022).
TAPVR affects how the blood flows through the body by mixing oxygen-rich blood with oxygen-poor blood. This lowers the amount of oxygen the body gets. Because of this, babies with TAPVR may have blue skin or lips (cyanosis), trouble breathing, and difficulty feeding. The body tries to fix the problem by breathing faster and making the heart beat faster, but this only helps for a short time. If TAPVR is not treated quickly, it can lead to serious problems like heart failure or even death.
It’s important to diagnose TAPVR early, usually with heart imaging like echocardiography. Surgery is the only fix and should be done soon after birth. The goal is to connect the lung veins to the left side of the heart. In severe cases, machines like ECMO can support the heart and lungs while the body heals.
In conclusion, TAPVR is a heart problem that affects how blood flows and mixes in the body. It is a serious condition that needs to be found and treated with surgery as early as possible to avoid long-term harm or death. Knowing how TAPVR works helps doctors treat it better. Learning from other serious heart and lung cases also helps improve care and save lives in complex situations like TAPVR.
References
Okereke, M., & Sharma, M. (2018). Massive pulmonary embolism treated with catheter-directed thrombolysis. BMJ Case Reports, 2018, bcr-2017-221074. https://doi.org/10.1136/bcr-2017-221074
Wang, X., Liu, D., Yang, X., Zhang, Y., Yan, M., & Xu, Y. (2022). Clinical outcomes of extracorporeal membrane oxygenation in acute high-risk pulmonary embolism: a retrospective cohort study. Journal of Cardiothoracic Surgery, 17(1), 300. https://doi.org/10.1186/s13019-022-01990-9
A heart disease called total anomalous pulmonary venous return is a rare heart disease that occurs at birth. The pulmonary veins generally transport oxygen-rich blood from the lungs to the left side of the heart but with TAPVR these veins attach to the wrong side, which causes blood to go back to the right side instead. This causes the oxygen-rich blood to mix with the oxygen-poor blood, resulting in the body not getting enough oxygen, which leads to breathing problems or even the skin turning blue and if not treated immediately, it can lead to heart failure and, in severe cases, death. TAPVR is not only a rare congenital heart defect but it is a serious condition that requires surgery immediately after birth for the infant to survive.
The four main types of TAPVR are the supracardiac, cardiac, infracardiac, and mixed, depending on where the lungs and veins connect. With supracardiac TAPVR, the pulmonary veins drain into a vertical vein, which joins the superior vena cava. The veins in the cardiac TAPVR empty into the right atrium or the coronary sinus. The veins in the infracardiac attach to the lower body veins, which are the inferior vena cava, hepatic veins, or portal veins. When several lung veins connect incorrectly, the mixed type occurs, which means oxygen-rich blood from the lungs goes onto the right side of the heart instead of the left atrium, resulting in less oxygen. TAPVR usually has symptoms like heart failure during the neonatal period but does not usually occur in adults due to low survival rates.
To diagnose this condition, it is usually done with imaging like echocardiography. After surgery there should be no problems but in severe cases a machine called ECMO is needed to support the heart and lungs while the body is healing. Being aware of this condition not only helps doctors treat it but also saves lives.