From the Guidelines
Total Anomalous Pulmonary Venous Return (TAPVR) should be diagnosed using cross-sectional imaging with CMR or CTA, and treated with surgical correction, which usually involves intracaval baffling into the left atrium, Warden procedure, or direct reimplantation of the anomalous pulmonary vein directly into the left atrium, as this approach is associated with a reduction in RV size and PA pressure 1.
Diagnosis
The diagnosis of TAPVR involves the use of imaging techniques to delineate pulmonary venous connections. According to the 2018 AHA/ACC guideline for the management of adults with congenital heart disease, cross-sectional imaging with CMR or CTA is ideal for this purpose, as it provides detailed information about the extracardiac vascular anatomy 1. Echocardiography is also an important part of the evaluation, particularly in patients with excellent acoustic windows, but CMR and CTA are superior for evaluating extracardiac vascular anatomy.
Treatment
The treatment of TAPVR typically involves surgical correction, which is associated with a reduction in RV size and PA pressure 1. The surgical approach usually involves intracaval baffling into the left atrium, Warden procedure, or direct reimplantation of the anomalous pulmonary vein directly into the left atrium. In higher-risk patients, invasive hemodynamic assessment can be useful for direct measurement of pressures, quantification of shunt magnitude, and measurement of pulmonary arterial resistance and responsiveness to pulmonary vasodilator therapy 1.
Key Considerations
Some key considerations in the treatment of TAPVR include:
- Pulmonary hypertension is a risk for adverse outcomes with surgery 1.
- Surgical repair of a scimitar vein can be technically challenging with a greater risk of postoperative vein thrombosis than is associated with more common and simpler anomalous pulmonary vein abnormalities 1.
- It is unusual for a single anomalous pulmonary venous connection of only 1 pulmonary lobe to result in a sufficient volume load to justify surgical repair, but if a patient has symptoms referable to the shunt, there is >1 anomalous vein, and a moderate or large left-to-right shunt, then surgical repair is associated with a reduction in RV size and PA pressure 1.
From the Research
Diagnosis of Total Anomalous Pulmonary Venous Return (TAPVR)
- TAPVR is a congenital heart defect where the pulmonary veins fail to return to the left atrium and instead drain into a systemic vein or directly to the right atrium 2, 3
- The diagnosis of TAPVR can be made using echocardiography, which can also help identify the site of drainage and the presence of any obstruction 2
- The clinical presentation of TAPVR can vary depending on the presence or absence of obstruction, with obstructed TAPVC presenting as a surgical emergency 3
Treatment of TAPVR
- Surgical correction is the primary treatment for TAPVR, with the goal of redirecting the pulmonary veins to the left atrium 4, 5, 6
- The surgical approach depends on the anatomic type of TAPVR, with obstructed TAPVC requiring urgent surgical intervention and unobstructed TAPVC being dealt with electively 6
- Postoperative care is critical, with attention to pulmonary artery hypertension and the potential need for re-intervention in cases of recurrent venous obstruction 4, 5
- The use of techniques such as ultrafiltration and improved myocardial protection has been shown to improve outcomes in patients with TAPVR 5
Outcomes and Prognosis
- The outcomes for patients with TAPVR have improved significantly in recent years, with operative mortality consistently less than 10% 6
- Long-term survival and quality of life are generally good for patients with TAPVR, with most patients being asymptomatic and having normal psychomotor development 5
- However, patients with single ventricle physiology and TAPVC remain a challenging group, with high operative mortality and poor long-term survival 6
- The management of postoperative pulmonary venous obstruction is critical, with a combination of re-operation and repeated balloon dilation being advocated as the best approach 4