Case report: Additional Imaging Supports TAPVR Care

August 12, 2026

Advanced imaging – either preoperatively or postoperatively – may improve surgical planning or reintervention for infradiaphragmatic total anomalous pulmonary venous return (TAPVR). A recent case report published in JACC: Case Reports from Phoenix Children’s suggests that advanced diagnostic studies, including cardiac CT or MRI, can identify anatomical differences in patients that may benefit from a different operative approach.

“The key lesson is that echocardiography may not show the full anatomy in complex TAPVR, even when the study appears complete,” said Sebastián Quiñones-Carrasquillo, MD, a cardiac surgeon at Phoenix Children’s. a cardiac surgeon at Phoenix Children’s. “The practical takeaway is this: If the echocardiogram, the patient’s condition or the operative findings do not match, consider the possibility of another pulmonary venous pathway. This is especially important in infradiaphragmatic, mixed or unusual forms of TAPVR.”

In this case report, the Phoenix Children’s team examined a 2-day-old newborn with hypoxemic respiratory failure and pulmonary congestion with suspected infradiaphragmatic TAPVR. Echocardiography and surgery revealed a right-sided confluence draining via the vertical vein. However, postoperative CT showed a separate left-sided confluence into the splenic vein. This case informs how providers should approach surgical intervention with these patients.

When to Consider Supplemental Advanced Imaging

While helpful, echocardiography does not always fully explain the patient’s physiology. A degree of uncertainty always exists around the total anomalous pulmonary venous return anatomy, Dr. Quiñones-Carrasquillo said. According to existing research, diagnoses made on initial echocardiography are incorrect in roughly 9% of patients.

“Clinical experience and published studies show that echocardiography can sometimes miss or misclassify complex TAPVR anatomy, especially when the drainage is infradiaphragmatic, mixed, posterior or outside standard echo view,” he said.

Providers should watch for the possibility that these concerns are present:

Drainage into the portal, hepatic, splenic or systemic venous system
Dual infradiaphragmatic vertical veins
Mixed-type TAPVR with veins draining to more than one location
Nonconfluent pulmonary veins where the right and left veins don’t join together
Pulmonary vein atresia
Pulmonary vein stenosis or narrowing
Separate pulmonary venous confluence

To identify these potential problems, Dr. Quiñones-Carrasquillo and his colleagues, cardiologist Todd Nowlen, MD and Daniel Velez, MD, recommend cardiac CT or MRI.

“Cardiac CT is often the most useful next test when the patient is stable enough. It can show the full path of the pulmonary veins, the exact drainage site and any areas of narrowing or obstruction. It also provides 3D images that help surgeons plan the repair or reoperation,” Dr. Quiñones-Carrasquillo said.

Cardiac CT imaging can also reveal:

Anatomy needed for repair or reoperation
How veins relate to the diaphragm, portal system, splenic vein and left atrium
If separate right- and left-sided confluences exist
Whether all pulmonary veins connect to one confluence

Dr. Quiñones-Carrasquillo said cardiac MRI can also help to delineate anatomy. However, obtaining images takes longer, making it a less practical option for these patients.

When to Perform Cardiac CT or MRI Imaging

When questions arise about a patient’s anatomy and TAPVR, Dr. Quiñones-Carrasquillo recommended providers base any imaging decisions on the baby’s stability.

“If the newborn is unstable, surgery should not be delayed for CT or MRI. Obstructed TAPVR is a surgical emergency, and you should not postpone lifesaving surgery. These babies can quickly develop severe low oxygen levels, pulmonary hypertension, acidosis and circulatory collapse,” he said. “However, if the baby can be stabilized, even briefly, cardiac CT should be considered before surgery to help define anatomy and improve surgical planning.”

For infants who can be stabilized, providers should pursue preoperative imaging when:

Anatomy is difficult to define by echocardiography
Echocardiography suggests missed or unusual drainage
Heterotaxy syndrome or other complex anatomy is suspected
One or more of the four pulmonary veins can’t be clearly seen
Patient seems sicker than echocardiography findings suggest

Findings that Warrant Repeat Imaging or Reintervention

In some instances, patients continue to experience minor blood flow problems after surgery. Depending on the severity or if other symptoms appear over time, providers may choose to monitor the drainage or pursue additional treatment, Dr. Quiñones-Carrasquillo said.

“Residual anomalous drainage may be safe to watch at first if it is unobstructed,” he said. “But the team should move toward repeat imaging or reintervention if there are signs that the pathway is causing strain on the heart.”

He advised providers to watch for these warning signs:

Evidence of pulmonary venous obstruction
Increasing oxygen requirement
Narrowing seen on echocardiography, CT or MRI
New or worsening respiratory distress
Rising Doppler gradients across the residual pathway
Rising right-sided heart pressures
Worsening right ventricular dilation

The Need for Advanced Support

Obstructed infradiaphragmatic TAPVR is a life-threatening condition. Timely intervention is vital to an infant’s best outcome, Dr. Quiñones-Carrasquillo said. Phoenix Children’s Center for Heart Care offers advanced, multidisciplinary care to support these patients.

“Referring physicians should consider involving Phoenix Children’s as early as possible,” he said. “Early involvement allows the team to help with stabilization, imaging decisions, transport planning, timing of surgery and long-term follow-up.”

A More Comprehensive Approach for TAPVR Care

This case report highlights the need for a more robust approach to TAPVR care and surgical planning, Dr. Quiñones-Carrasquillo said. Providers should never postpone surgery for unstable patients who need immediate intervention. However, if echocardiography is incomplete or inconclusive – or if additional physiologic concerns are present – they should capture additional imaging as soon as possible.

“This case report expands on the known spectrum of TAPVR and is a reminder that in complex congenital heart disease, what appears complete on imaging may not always be complete in reality,” he said. “Dual nonconfluent infradiaphragmatic TAPVR with splenic venous drainage is extremely rare. Still, the care team should make a deliberate effort to account for all pulmonary veins for every TAPVR repair.”

Refer a Patient or Request a Consult

Phoenix Children's Center for Heart Care provides comprehensive, multidisciplinary care for infants and children with complex congenital heart disease, including infradiaphragmatic and mixed-type TAPVR – with access to advanced diagnostic imaging and surgical expertise for the highest-complexity cases. To refer a patient or request a consultation, call 602-933-KIDS (5437) or submit a referral online.

Source:

Quiñones-Carrasquillo, S., Nowlen, T., Alaeddine, M., Kisamori, E., & Velez, D. A. (2026). Dual-Drainage Infradiaphragmatic TAPVR With Splenic Venous Connection: What You See Is Not What You Get. JACC. Case reports, 31(22), 107932. https://doi.org/10.1016/j.jaccas.2026.107932