Skip to Main Content


  • • A congenital heart lesion that increases pulmonary arterial blood flow

    • Results in left-to-right shunt, results in lung infection, pulmonary vascular congestion, pulmonary artery (PA) hypertension, right heart failure, pulmonary vasoconstriction, pulmonary vascular obstructive disease

    Eisenmenger syndrome: Increased pulmonary hypertension such that left-to-right shunt ceases and shunt becomes right-to-left, requiring heart-lung transplant

    • Inhaled nitric oxide, oxygen, or IV tolazoline reverses PA vasoconstriction

    • PA band is palliative and can reduce PA flow to alleviate RV failure and progression of pulmonary hypertension

    • Pulmonary veins do not make direct connection with LA, instead confluence connects to central systemic veins, draining into RA

    • Blood gets to LA atrium via atrial septal defect (ASD) or patent ductus arteriosus (PDA)

    • Similar oxygen saturation in PA and aorta

    • 3 types (depends on site of connection):

    • -Type I, Supracardiac: Left-sided vertical vein drains into innominate vein (45%)

      -Type II, cardiac: Connection to RA or coronary sinus (25%)

      -Type III, infracardiac: Connection to infradiaphgragmatic inferior vena cava (IVC) or portal vein (25%)

      5% have mixed venous drainage

    • Pulmonary venous obstruction occurs in nearly all with infracardiac connection, < 25% with supracardiac connection

    • Obstruction leads to increased pulmonary vascular resistance

    • Associated anomalies rare


Symptoms and Signs


  • No obstruction: Symptoms relate to pulmonary overcirculation and hypertension (poor feeding, failure to thrive, tachypnea, diaphoresis)

    Obstruction: Profound cyanosis, respiratory failure, hypotension within first few hours of life

    Infants: Severe heart failure, cyanosis poor pulses, acidosis

    • Pulmonary midsystolic murmur, some with fixed split, loud S2

    • Oxygen saturation similar in aorta and PA

    • PA and wedge pressures elevated

    • Degree of obstruction determines clinical presentation

    • Enlarged RA and RV with severe pulmonary vascular congestion


  • • Must distinguish type (I, II, III) in order to plan repair

    • Presence of other associated cardiac anomalies uncommon but high mortality


  • • Echocardiography

    • Cardiac catheterization if diagnosis unclear or if balloon septostomy necessary


  • • All should be surgically repaired

    • Timing dictated by degree of obstruction

    • Intubation, resuscitation

    • No medical therapy




  • • Supracardiac and infracardiac: Anastomosis of pulmonary venous confluence to LA, legate anomalous connection

    • Cardiac drainage: Unroofing coronary sinus and patch closure of atrial septum (coronary sinus drains into LA)

    • Critically ill neonates with obstruction may need hypothermic circulatory arrest to repair lesion




  • • All should be surgically repaired

    • Obstructed total anomalous pulmonary venous connection: Surgical emergency


Treatment Monitoring


  • • Postoperatively after cardiac connection repair: Alkalosis, increased oxygen tension, sedation, deep analgesia to prevent PA hypertensive crisis




  • • Mortality limited to patients with severe obstruction (10-15%) due to pulmonary hypertension

    • Patients with associated anomalies have high mortality (> 30%)

    • Recurrence of pulmonary obstruction: 5-10%

    • Good long-term prognosis for patients who survive without recurrence

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.