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AP Window

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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Hugo González
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306 views

AP Window

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.

Uploaded by

Hugo González
Copyright
© Attribution Non-Commercial (BY-NC)
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd
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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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