Lightning Learning: TAPVD
STOP!
Total Anomalous Pulmonary Venous Drainage (TAPVD) is a congenital heart defect (CHD) where “the four pulmonary veins do not drain into the left atrium but instead drain via unique pathways into the right atrium or systemic veins” (n.b. atrial septal defect are present in all cases to allow shunting of oxygenated blood).
Whilst rare (8 per 100,000 live births) it is an important cause of cyanotic congenital heart disease. In A&E consider it in cases of…
Collapsed cyanotic neonates
Infants with mild cyanosis
BEWARE
Presentation varies with degree of pulmonary obstruction to pulmonary blood flow (see below). The subtypes of TAPVD & associated cardiac defects (~30%) are also influential.
LOOK
Pre/post ductal sats screening, hyperoxia test & CXR may help, but may be normal.
CXR may include engorged pulmonary vasculature, diffuse interstitial infiltrate, signs of heart failure (e.g. enlarged heart size) or altered cardiac silhouette, i.e. the snowman sign of supracardiac TAPVD.
Definitive Imaging includes…
1st: Echocardiography (non-invasive)
2nd: CT/MRI imaging (as required)
3rd: Angiography (gold standard)
LEARN
Management: (if acutely unwell for severe obstruction)
Intubate, ventilate, sedate, inotropes, Rx for pulmonary hypertension, +/- ECMO. Prostaglandin E1 helps systemic perfusion but may worsen pulmonary blood flow!
BEWARE: Get early help and prepare to transfer for surgery. Survival can be as high as 85-87% at 18 years of age.
Further Reading
- Total Anomalous Pulmonary Venous Return (Don't Forget The Bubbles)
Many thanks to Dr Lisa Keillor for peer reviewing this topic!
