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    Anomalies of The Pulmonary Veins

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    luulamthangtai0105
    This document discusses anomalies of the pulmonary veins, including their embryology and classifications. It describes partial anomalous pulmonary venous connection (PAPVC), total anomalous pulmonary venous connection (TAPVC), and Cor triatriatum. For each condition, it covers physiology, clinical features, diagnostic evaluation including echocardiography, and treatment approaches. Echocardiography is an important tool for preoperative evaluation to identify venous connections and assess hemodynamics. Surgical correction is usually indicated for significant shunting or obstruction.

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    Anomalies of The Pulmonary Veins

    Uploaded by

    luulamthangtai0105
    6 views30 pages
    This document discusses anomalies of the pulmonary veins, including their embryology and classifications. It describes partial anomalous pulmonary venous connection (PAPVC), total anomalous pulmonary venous connection (TAPVC), and Cor triatriatum. For each condition, it covers physiology, clinical features, diagnostic evaluation including echocardiography, and treatment approaches. Echocardiography is an important tool for preoperative evaluation to identify venous connections and assess hemodynamics. Surgical correction is usually indicated for significant shunting or obstruction.

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    Anomalies of the Pulmonary Veins

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    This document discusses anomalies of the pulmonary veins, including their embryology and classifications. It describes partial anomalous pulmonary venous connection (PAPVC), total anomalous pulmonary venous connection (TAPVC), and Cor triatriatum. For each condition, it covers physiology, clinical features, diagnostic evaluation including echocardiography, and treatment approaches. Echocardiography is an important tool for preoperative evaluation to identify venous connections and assess hemodynamics. Surgical correction is usually indicated for significant shunting or obstruction.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    6 views30 pages

    Anomalies of The Pulmonary Veins

    Uploaded by

    luulamthangtai0105
    This document discusses anomalies of the pulmonary veins, including their embryology and classifications. It describes partial anomalous pulmonary venous connection (PAPVC), total anomalous pulmonary venous connection (TAPVC), and Cor triatriatum. For each condition, it covers physiology, clinical features, diagnostic evaluation including echocardiography, and treatment approaches. Echocardiography is an important tool for preoperative evaluation to identify venous connections and assess hemodynamics. Surgical correction is usually indicated for significant shunting or obstruction.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 30

    Anomalies of the Pulmonary Veins

    Dr. Luu Lam Thang Tai


    Deparment of pediatrics,
    City children’s hospital
    Orcid: 000-0003-1062-2463
    References:
    • Moss and Adams' Heart Disease in Infants, Children, and Adolescents:
    Including the Fetus and Young Adult
    • Kirklin/Barratt-Boyes Cardiac Surgery
    • Braunwald’s Heart Disease - A Textbook of Cardiovascular Medicine
    • Echocardiography in Pediatric and Congenital Heart Disease From
    Fetus to Adult
    Contents:

    • Embryology of pulmonary veins


    • For each PAPVC, TAPVC, Cor triatriatum
    - Classification
    - Physiology
    - Clinical and diagnostic features
    - Treatment
    • Echocardiography
    Embryology of pulmonary veins
    Embryology of pulmonary veins
    Embryology of pulmonary veins

    Sinus venosus defect Malposition of septum primum


    Embryology of pulmonary veins

    PAPVC

    TAPVC
    Embryology of pulmonary veins

    Common pulmonary
    vein atresia

    Cor triatriatum
    Partial Anomalous Pulmonary Venous Connection

    • The embryologic splanchnic plexus is a midline structure


    • Left-sided pulmonary veins usually connect anomalously to
    derivatives of the left cardinal system (i.e., the coronary sinus and the
    left innominate vein).
    • Anomalous connections of the right pulmonary veins are usually to
    derivatives of the right cardinal system (i.e., the SVC or inferior vena
    cava [IVC]).
    • The most common type of PAPVC is of the left pulmonary veins to the
    left innominate vein.
    • The second most common types are anomalous connections of
    pulmonary veins from the right lung to the IVC.
    Partial Anomalous Pulmonary Venous Connection

    Moss & Adams classification:


    • Right Pulmonary Veins to Superior Vena Cava
    • Right Pulmonary Veins to Inferior Vena Cava
    • Left Pulmonary Veins to Inferior Vena Cava
    • Left Pulmonary Veins to Left Innominate Vein
    • Other Sites of Partial Anomalous Pulmonary Venous Connection
    Partial Anomalous Pulmonary Venous Connection

    Kirklin classification:
    • Sinus venosus malformation
    • Right superior pulmonary vein to Superior Vena Cava
    • Right pulmonary veins to Right Atrium
    • Right pulmonary veins to Inferior Vena Cava
    • Scimitar Syndrome (Right pulmonary veins to Inferior Vena Cava,
    Scimitar pulmonary veins to left atrium, Left pulmonary veins to
    Inferior Vena Cava)
    • Rare connections of right pulmonary veins (azygos vein, coronary sinus)
    • Left pulmonary venous connection
    • Bilateral partial pulmonary venous connection
    Partial Anomalous Pulmonary Venous Connection

    Physiology
    • Factors affects hemodynamic state:
    - Number of anomalously connected veins
    - The site of the anomalous connections
    - The presence and size (if present) of an ASD
    • Flow of anomalously connected veins compared with normal veins ?
    - Lung hypoplasia
    - Compliance of atrium
    - Pulmonary vascular resistance
    Partial Anomalous Pulmonary Venous Connection

    Clinical features
    • Similar to ASD and TAPVC, depend on the flow of anomalous
    pulmonary veins and Qp/Qs
    Electrocardiographic Features
    • Similar to uncomplicated ASD
    Radiologic features
    • Snowman sign
    • Azygos vein enlargement
    • Scimitar sign
    Partial Anomalous Pulmonary Venous Connection
    Partial Anomalous Pulmonary Venous Connection

    Treatment
    • Medical management: heart failure, increased pulmonary blood flow,
    pulmonary vascular disease
    • Surgical management: is indicated when patients exhibit evidence of
    pulmonary overcirculation or respiratory insufficiency
    Total Anomalous Pulmonary Venous Connection
    Total Anomalous Pulmonary Venous Connection
    Total Anomalous Pulmonary Venous Connection

    Key physiologic factors:


    • Venous mixing – Right to left shunting (ASD)
    • Pulmonary venous obstruction
    • Left-to-right shunting
    Total Anomalous Pulmonary Venous Connection

    Clinical features
    • Obstructed TAPVC – Patients with severe obstruction generally present
    as critically ill newborns with profound cyanosis, respiratory failure, and
    shock. Obstructed TAPVC results in elevated pulmonary artery pressure,
    pulmonary edema, respiratory distress, diminished systemic output,
    and hypotension.
    • Unobstructed TAPVC – Patients with unobstructed lesions may only
    have subtle cyanosis immediately after birth, which may be detected
    with pulse oximetry screening. Although hypoxia is invariably present in
    unobstructed TAPVC, the degree can vary, and some patients may not
    appear to be cyanotic. Commonly, oxygen saturations (SaO2) measured
    by cardiac catheterization were 88 and 92 percent.
    Total Anomalous Pulmonary Venous Connection

    Clinical features (Unobstructed → Obstructed)


    • Fixed split second heart sound (S2) → S2 is prominent
    • Systolic ejection murmur: the pulmonary valve
    • Diastolic rumble: the tricuspid valve
    • Hepatomegaly
    • Tachypnea
    • Variable degrees of cyanosis → Cyanosis
    • A anomalous soft, continuous murmur
    • Hypotension, diminished pulses
    Total Anomalous Pulmonary Venous Connection

    Xray features (Unobstructed → Obstructed)


    Increased pulmonary blood flow, enlarged right atrium, enlarged right
    ventricle, heart size is mildly enlarged
    → Cardiac silhouette is not enlarged, pulmonary artery dilation,
    pulmonary vein congestion, interstitial edema → ground glass
    appearance
    Electrocardiographic features
    ECG shows RAD, RVH (rsR’ pattern in V1), and occasional RAH
    Total Anomalous Pulmonary Venous Connection
    Total Anomalous Pulmonary Venous Connection

    Treatment
    • Medical management
    - Unobstructed: heart failure, increased pulmonary blood flow,
    pulmonary vascular disease
    - Obstructed: Oxygen + Ventilation + Inotropic support ± PEG1 ± ECMO
    • Palliative cardiac catheterization procedures: atrial septostomy, stent
    placements into severely obstructed positions.
    • Surgical management
    Cor Triatriatum
    Cor Triatriatum

    Clinical features
    • cor triatriatum presents in infancy with signs and symptom of
    pulmonary hypertension and pulmonary venous obstruction. Due to
    low cardiac output, children can show poor growth and weight gain,
    feeding difficulties, respiratory distress, and tet spells.
    • Most common symptoms: Dyspnea and orthopnea, Easy fatigability,
    Hemoptysis, Exercise intolerance and shortness of breath, Palpitations
    Cor Triatriatum
    Xray features
    - Pulmonary congestion with haziness (Kerley B-lines)
    - Ground glass appearance of acute pulmonary edema
    - Prominent pulmonary vessels
    - Pleural effusion
    - Left atrial enlargement
    - Cardiomegaly
    Electrocardiographic features
    ECG shows RVH, atrial fibrillation
    Total Anomalous Pulmonary Venous Connection
    Cor Triatriatum

    Treatment
    • Medical management
    - Heart failure, increased pulmonary blood flow, pulmonary vascular
    disease, thromboembolic prophylaxis
    • Surgical management: Complete surgical resection of atrial
    appendage/accessory membrane through a midline sternotomy
    under cardiopulmonary bypass and closure of atrial septum with a
    pericardial patch
    Echocardiography evalution of Pulmonary Veins Anomalities
    Preoperative examination

    - Identify each pulmonary vein and its connection site by 2D and color Doppler. Do not assume two veins from
    each lung; the number of individual pulmonary veins varies, and additional vein(s) with separate
    connection(s) to a systemic vein (e.g., left innominate vein, azygous vein, IVC, SVC) should be excluded.
    - Follow each anomalously connecting pulmonary vein from its origin to its connection to the systemic vein
    from multiple views by continuous 2D and by color Doppler sweeps.
    - Interrogate each pulmonary vein by spectral Doppler and record mean gradients; interrogate by spectral
    Doppler and record mean gradient in every site where flow velocity accelerates and the lumen narrows by
    color Doppler.
    - In TAPVC: measure the diameters of each pulmonary vein and the smallest diameter of the pulmonary
    venous confluence.
    Echocardiography evalution of Pulmonary Veins Anomalities
    Preoperative examination

    - Image the anatomic relationship between the pulmonary venous confluence and the left atrium.
    - Rule out mixed drainage (make sure no additional pulmonary veins enter a systemic vein separately).
    - Rule out anomalous systemic venous connections.
    - Assess atrial septum, direction of atrial shunt, and evaluate for restriction to flow (color and spectral
    Doppler).
    - Assess presence and degree of right ventricular volume load (TV annulus diameter; diastolic septal
    flattening; qualitative assessment of RV size; RV volume by 3D, if feasible).
    - Assess RV pressure by tricuspid and pulmonary regurgitation jet velocities and by systolic septal
    configuration.
    - Measure LA and LV volumes.
    - Exclude associated cardiovascular anomalies.

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