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  • • 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

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Symptoms and Signs

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  • 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

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

    • Presence of other associated cardiac anomalies uncommon but high mortality

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  • • Echocardiography

    • Cardiac catheterization if diagnosis unclear or if balloon septostomy necessary

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  • • All should be surgically repaired

    • Timing dictated by degree of obstruction

    • Intubation, resuscitation

    • No medical therapy

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Surgery

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  • • 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

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Indications

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  • • All should be surgically repaired

    • Obstructed total anomalous pulmonary venous connection: Surgical emergency

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Treatment Monitoring

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

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Prognosis

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  • • 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

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