وصف
Total anomalous pulmonary venous connection (TAPVC) is a condition in which, with or without pulmonary venous blockage, all four pulmonary veins drain into systemic veins or the right atrium. In the right atrium, systemic and pulmonary venous blood are mixed. In both foetal and newborn circulation, the presence of an atrial defect or foramen ovale (a component of the complex) is significant for left ventricular output. TAPVR and PAPVR may be detected during pregnancy, however this is less common than discovering them immediately after a baby is delivered. TAPVR babies will require surgery to correct the flaw. The severity of the child's illness and the precise design of the atypical connections between the pulmonary veins and the right atrium determine the age at which the surgery is performed. Normal blood flow through the heart will be restored as a result of the surgical TAPVR correction. In order to correct this problem, clinicians typically join the pulmonary veins to the left atrium, block any unnatural blood vessel connections, and seal the atrial septal defect. Infants with surgically corrected abnormalities may experience lifelong consequences; they are not necessarily cured. A cardiologist (a physician who specialises in the heart) will need to see a kid or adult with TAPVR on a regular basis to check on their progress, prevent problems, and assess their heart.
ملخص
- Total Anomalous Pulmonary Venous Connection (TAPVC) is a congenital heart defect where pulmonary veins fail to connect to the left atrium, instead draining into the right atrium or systemic veins. An atrial septal defect (ASD) or patent foramen ovale (PFO) is essential for survival, providing a right-to-left shunt. Drainage can occur into the right atrium, superior vena cava (SVC), coronary sinus, or portal vein.
- Embryologically, TAPVC arises from the failure of the common pulmonary vein to connect to the left atrium during gestation. Normally, the pulmonary venous plexus establishes a connection to the left atrium around 32-33 days of gestation. If this connection fails and the systemic venous connections do not regress, TAPVC results.
- The Darling classification categorizes TAPVC into supracardiac, cardiac, infracardiac, and mixed types. Supracardiac is the most common, with pulmonary veins draining via a vertical vein to the innominate vein, SVC, and then the right atrium. Cardiac involves drainage into the coronary sinus. Infracardiac is considered most critical due to obstructed venous drainage in the abdomen, where the veins usually drain into the portal system.
- Pathophysiologically, oxygenated blood flows into the right atrium, causing right heart volume overload and pressure increase. An obligatory shunt is crucial for delivering oxygenated blood to the left atrium. Decreased pulmonary vascular resistance (PVR) after birth leads to increased pulmonary blood flow and potential QP:QS mismatch, increasing the risk of pulmonary hypertension. Obstruction exacerbates pulmonary venous hypertension and edema.
- Clinical features vary based on obstruction. Unobstructed TAPVC presents with tachypnea, feeding difficulties, sweating, and mild cyanosis in the early weeks of life. Obstructed TAPVC manifests early with severe tachypnea, tachycardia, cyanosis, and significant dyspnea soon after birth. Prompt diagnosis is essential as without treatment, death can occur very quickly.
- Diagnosis involves chest X-rays (e.g., snowman appearance in supracardiac TAPVC), CT imaging (demonstrates course of the pulmonary veins) and echocardiography, which is the gold standard. Echocardiography determines the size and drainage pattern of pulmonary veins, the presence of obstruction, and the size of the interatrial communication.
- Surgical repair aims to establish a non-obstructed connection between the pulmonary veins and the left atrium. It is also necessary to interrupt any connections to the systemic venous circulation (such as ligation of the vertical vein) and usually closure of the ASD. Techniques vary depending on the TAPVC type, including direct anastomosis between the common venous chamber and the left atrium. Sutureless techniques are sometimes used when there is an unfavourable anatomy.
- Surgical outcomes have improved significantly. With recent advances, modern surgical survival rates are exceeding 90%. Long-term prognosis is generally favorable, but pulmonary vein obstruction can occur in up to 10-15% of patients, requiring long-term surveillance.
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