Pulmonary Venous Connection, Total Anomalous

• • 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

Surgery

• • 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

Indications

• • 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

Prognosis

• • 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 ...