Figure 1

High parasternal short-axis echocardiogram is showing the aortopulmonary window between the ascending
aorta (Ao) and main pulmonary artery (PA)

At cardiac catheterization we found a large APW with pulmonary to aortic flow ratio above 3 and elevated pulmonary
pressure (70% of systemic arterial pressure) [Figure 2].

Figure 2
Aortic angiography is showing filling of the main pulmonary artery through the aortopulmonary window
(arrow)

The patient was operated after diagnosis by transaortic approach using a median sternotomy and cardiopulmonary
bypass. The patient had good recovery from operation. His respiratory symptoms decreased after surgery.
Echocardiography was performed and showed normal left ventricular function, and no residual aortopulmonary defect.
No evidence of narrowing or stenosis of aorta and pulmonary arteries and no distortion of semilunar valve were found by
echocardiographic study. Our patient had trivial to mild MR after operation. He is active and asymptomatic at follow-up
period (one year). His mild mitral regurgitation is decreased to trivial regurgitation and cardiac function is normal.

DISCUSSION
At the fetal life, abnormal growth of aorticopulmonary septum, due to one of the rarest congenital heart defects,
APW[17,18] APW is a defect between two main great arteries in the presence of two separate aortic and pulmonary
semilunar valves.[11] Truncus Arteriosus can be differentiated from this anomaly by absence of two separated normally
aortic and pulmonary valves. In large defects this lesion often mimics as a large intra or extracardic shut such as
ventricular septal defect, atrioventricular septal defect or patent ductus arteriosus (PDA) and resulting in congestive heart
failure, pulmonary hypertension and progressive of pulmonary vascular disease.[2,12,13]

Nearly 50-70% of all patients with APW were associated with other cardiac malformations.[19–21] Several cardiac
malformation could be seen include coarctation of the aorta, type A interruption of aortic arch, ventricular septal defect,
atrial septal defect (ASD), PDA, transposition of the great arteries, and tetralogy of Fallot.[2,3,13,22]

Several classifications of APW have been presented. One of these classifications separates APW into 3 types, proximal or
type A, distal or type B, and total or type C.[10] Proximal defect which is the most common type is a small defect located
between the semilunar valves and the pulmonary bifurcation. Distal defects are located in the upper portion of the
ascending aorta before aortic branches near the pulmonary biforcation. The rarest type or total defects consist of the
entire aortopulmonary septum.

In the past, the diagnostic approach to APW was with cardiac catheterization.[21] By progressing non-invasive approach,
techniques and 2-dimensional echocardiography diagnosis of APW and associated anomalies is facilitated.[2,13,19,22] The
defect can be best visualized from a different views by echocardiographic study, including parasternal short and long-axis
views, high parasternal short-axis view and subcostal coronal view. Echocardiographic study helps us to identify the
morphological features and typing of the defect, exact location, sizing of defect, and associated cardiac anomalies. At
Doppler echocardiography, abnormal continuous forward flow in the pulmonary arteries indicates the presence of an
aortopulmonary communication.[23] Cardiac catheterization is indicated for estimating pulmonary vascular resistance
and pulmonary artery pressure.

Closure of APW is indicated in all patients and it should be performed as soon as possible after diagnosis. Successful repair
of an APW was reported from several years ago and many authors have reported their results.[14] First successful repair
of an APW using cardiopulmonary bypass was reported in 1957.[24] Putnam and Gross described the transpulmonary
approach in 1966.[25] Transaortic direct closure of an APW was first reported by Wright and coworkers in 1968.[15] In
1969, Deverall et al. described the first use of a patch to close an APW.[16] Currently, several patients have had successful
closure using various catheter-delivered devices.[26–29] Transaortic approach using a median sternotomy and
cardiopulmonary bypass was performed for our patient. Neither pulmonary stenosis or distortion nor coronary artery
injury was found. During the follow-up period (one year), no reintervention was needed and he had trivial MR.
CONCLUSION
Most patients with APW develop severe clinical symptoms such as congestive heart failure or pulmonary hypertension
during early stages of life. Early surgical closure or device employment is indicated as soon as the diagnosis is established
to prevent congestive heart failure and pulmonary hypertension. Other cardiovascular defects should be repaired. Repair
of APW have good results and indicated as soon as possible, even in early infancy. The outcome of these patients is
excellent if correction is performed before complication of disease such as pulmonary hypertension.

ACKNOWLEDGMENT
This work was supported by Cardiovascular Reaserch Center of Ahvaz Jundishapur University of Medical Sciences.

Footnotes
Source of Support: Nil

Conflict of Interest: None declared.

Article information
Heart Views. 2012 Jul-Sep; 13(3): 103–106.
doi:  10.4103/1995-705X.102153
PMCID: PMC3503352

Mehdi Ghaderian

Department of Paediatric Cardiology, Golestan Medical, Educational and Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
Address for correspondence: Dr. Mehdi Ghaderian, Department of Paediatric Cardiology, Golestan Medical, Educational, and Research Center, Ahvaz Jundishapur
University of Medical Sciences, # 90, Golestan Blvd., P.O. Box 6135733118, Ahvaz, Iran. E-mail: ghader_45@yahoo.co.uk

Copyright : © Heart Views

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use,
distribution, and reproduction in any medium, provided the original work is properly cited.

Articles from Heart Views : The Official Journal of the Gulf Heart Association are provided here courtesy of Wolters Kluwer -- Medknow Publications

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