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    AP Window

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    Hugo González
    This document provides information about aortopulmonary window, a rare congenital heart defect where there is an abnormal opening between the ascending aorta and pulmonary artery. It discusses the history, classification into three types based on location of defect, pathophysiology of high left-to-right shunting and pulmonary overcirculation, associated anomalies, clinical features and diagnosis, indications and techniques for surgical repair, and postoperative risks including pulmonary stenosis. It also summarizes anomalous origin of the right pulmonary artery from the ascending aorta as a related rare condition.

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    AP Window

    Uploaded by

    Hugo González
    247 views13 pages
    This document provides information about aortopulmonary window, a rare congenital heart defect where there is an abnormal opening between the ascending aorta and pulmonary artery. It discusses the history, classification into three types based on location of defect, pathophysiology of high left-to-right shunting and pulmonary overcirculation, associated anomalies, clinical features and diagnosis, indications and techniques for surgical repair, and postoperative risks including pulmonary stenosis. It also summarizes anomalous origin of the right pulmonary artery from the ascending aorta as a related rare condition.

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    This document provides information about aortopulmonary window, a rare congenital heart defect where there is an abnormal opening between the ascending aorta and pulmonary artery. It discusses the history, classification into three types based on location of defect, pathophysiology of high left-to-right shunting and pulmonary overcirculation, associated anomalies, clinical features and diagnosis, indications and techniques for surgical repair, and postoperative risks including pulmonary stenosis. It also summarizes anomalous origin of the right pulmonary artery from the ascending aorta as a related rare condition.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    247 views13 pages

    AP Window

    Uploaded by

    Hugo González
    This document provides information about aortopulmonary window, a rare congenital heart defect where there is an abnormal opening between the ascending aorta and pulmonary artery. It discusses the history, classification into three types based on location of defect, pathophysiology of high left-to-right shunting and pulmonary overcirculation, associated anomalies, clinical features and diagnosis, indications and techniques for surgical repair, and postoperative risks including pulmonary stenosis. It also summarizes anomalous origin of the right pulmonary artery from the ascending aorta as a related rare condition.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 13
    At a glance
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    An aortopulmonary window is a rare congenital heart defect caused by abnormal septation of the truncus arteriosus into the aorta and pulmonary artery, resulting in an opening between the ascending aorta and pulmonary artery. It can lead to a left-to-right shunt, pulmonary overcirculation, and heart failure if not repaired.

    There are three main types of aortopulmonary windows based on their location: type I is between the ascending aorta and main pulmonary artery, type II is between the ascending aorta and right pulmonary artery, and type III has an anomalous origin of the right pulmonary artery from the ascending aorta.

    An aortopulmonary window causes a large left-to-right shunt, leading to pulmonary overcirculation and congestive heart failure. It can also cause pulmonary hypertension if not repaired.

    Aortopulmonary Window

    Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

    Aortopulmonary Window
    Definition
    A rare congenital heart defect with round, oval, or sometimes spiral opening between ascending aorta and pulmonary artery resulting from abnormal septation of the truncus arteriosus into the aorta and pulmonary artery * Aortopulmonary fistula * Aortic septal defect * Aorticopulmonary septal defect * Aortopulmonary fenestration

    Aortopulmonary Window
    1. History
    . Elliotson : 1st report in 1830 . Dodds and Hoyle : 1st clinical Dx. in 1949 . Gross : Successful ligation in 1952

    2. Classification
    . Type I : Between posteromedial wall of ascending aorta and lateral wall of MPA . Type II : Between posterior wall of ascending aorta and origin of RPA . Type III : Anomalous origin of RPA from posterolateral wall of ascending aorta

    Pathophysiology of AP Window
    A large, nonrestrictive communication exists between the proximal ascending aorta and the main pulmonary artery, resulting in a high-flow arterial level left-to-right shunt This leads to pulmonary overcirculation, and rapidly progressive congestive heart failure, pulmonary hypertension.

    Types of AP Window

    Aortopulmonary Window

    Aortopulmonary Window

    Pathology of AP Window
    1. Embryology and anatomy
    . Failure of fusion or malalignment of conotruncal ridges . Single large defect, rarely multiple . Aortic origin of Rt. pulmonary artery . Anomalous origin of Rt. & rarely Lt. coronary artery from PA

    2. Associated anomalies
    . IAA, VSD, TOF, PDA, TGA, SubAS

    3. Pathophysiology
    . Large left to right shunt . CHF, pulmonary hypertension

    Clinical Features & Diagnostic Criteria


    1. Clinical features
    . . . . . . Incidence is 0.15% of CHD Defect is small in 10% No tendency to close spontaneously Symptoms and signs of CHD in early life Systolic or continuous murmur Early pulmonary vascular disease

    2. Diagnosis
    . Two dimensional echocardiography . Cardiac catheterization & aortography

    3. Differential diagnosis
    . Large PDA, Truncus arteriosus, VSD+AR Ruptured aneurysm of the sinus Valsalva

    Operative Indications & Techniques


    1. Indications
    . Elective repair is advised before 3 months of age.

    . Symptomatic infants may be operated on promptly.

    2. Techniques
    . Division by direct suture
    . Patch closure

    Operative Results of AP Window


    1. Mortality : low

    2. Time-related survivorship : good


    The probability of surgical cure will be dependent on the age at operation and level of pulmonary

    vascular resistance.
    3. Late result * Supravalvular pulmonary stenosis * Subvalvar PS or supravalvar AS rarely

    Anomalous Origin of Right Pulmonary Artery from Ascending Aorta


    Rare congenital lesion with a high mortality and morbidity The natural history of this lesion is that of progressive heart failure with a 70% mortality rate at 1 year compared with an 84% survival rate at 1 year with surgical repair Early surgical repair is now the standard of treatment to prevent the deleterious effects of pulmonary overcirculation and irreversible changes.

    Anomalous Origin of RPA from AA

    (A, B) Anomalous right pulmonary artery (RPA) is detached from


    aorta, and a double trapdoor is created on main pulmonary artery. (C, D) Anastomosis of RPA is performed. (E) Completed repair.

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