Total anomalous pulmonary venous return

Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart anomaly with an abnormal drainage anatomy of the entire pulmonary venous system. This contrasts with partial anomalous pulmonary venous return (PAPVR) where only part of the pulmonary veins are abnormal.

In TAPVR there is nothing draining into the left atrium. Therefore in order to complete the circuit there is almost always an associated shunting defect which is most frequently a patent foramen ovale (PFO)


Embryological failure of pulmonary venous development resulting is persistent patency of primitive systemic veins.


Can be classified into 4 types depending on site of anomalous venous union1

* type I : supracardiac ( commonest > 50 % ) : drainage is to either left brachiocephalic, SVC or aygous vein
* type II : cardiac ( next commonest ~ 30% ) : drainage is to coronary sinus, right atrium
* type III : infracardiac : drainage usually below the diaphragm to ductus venosus, IVC, portal vein or hepatic veins
* type IV : mixed pattern

Radiographic features

Plain film

The supra cardiac variant can classically depict a "Snowman" type appearance on a frontal chest radiograph 2. The 1st two types give cardiomegaly.


Direct visualisation of anomalous venous return.

Echocardiography / Ultrasound

Blind ended left atrium with no connecting veins.


* asplenia
* heterotaxy syndrome
* thoracic lymphangiectasia : infra cardiac
* pulmonary congestion : infra cardiac


Article Source : Dr Yuranga Weerakkody, Radiopaedia


  1. Demos TC, Posniak HV, Pierce KL et-al. Venous anomalies of the thorax. AJR Am J Roentgenol. 2004;182 (5): 1139-50. AJR Am J Roentgenol (full text) - Pubmed citation
  2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 27 (5): 1323-34. doi:10.1148/rg.275065148 - Pubmed citation
Unless otherwise stated, the content of this page is licensed under Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License