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Abstract
Background: Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by abnormal communication between the ascending aorta and the main pulmonary artery. Without early surgical correction, this condition has a poor prognosis. However, few case reports have described adult survival of patients with untreated APW.
Case presentation: We report the case of a 15-year-old girl who developed irreversible pulmonary hypertension due to untreated APW. Initially, we suspected the presence of an extracardiac shunt using a simple calculation of the echocardiography-derived pulmonary-to-systemic flow (Qp/Qs) ratio during a routine echocardiography study, which provided us with a clue to proceed with further multimodal diagnostic evaluation. In this report, we describe a comprehensive diagnostic workup, including right heart catheterization and computed tomography imaging, which confirmed the diagnosis of severe irreversible pulmonary hypertension secondary to a large untreated APW.
Conclusion: This case report highlights the clinical utility of the echocardiography-derived Qp/Qs ratio as a valuable, noninvasive tool for diagnosing APW, which can lead to severe irreversible pulmonary hypertension. The multidisciplinary approach demonstrated in this case serves as a valuable example for clinicians evaluating similar cases. Therefore, APW should be considered in the differential diagnosis of severe pulmonary hypertension, even in adult patients.
Supplementary Files
- Video 1A - Transthoracic echocardiography showed normal left ventricular cavity size and function
- Video 1B - Transthoracic echocardiography showed normal left ventricular cavity size and function
- Video 2 - Parasternal short-axis view showed a dilated right-side heart with septal flattening during systole and diastole, consistent with right ventricular pressure and volume overload
- Video 3 - Trans-thoracic echocardiography with color Doppler showed moderate tricuspid regurgitation
- Video 4A - Parasternal short-axis pulmonary artery-focused view with color Doppler showed no evidence of shunts
- Video 4B - Suprasternal aortic arch view with color Doppler showed no evidence of shunts
- Video 5 - Apical-4 chamber was viewed with injected agitated saline contrast and showed suspected saline bubbles in the descending aorta (arrow) with no visible saline contrast seen in the left chambers of the heart
- Video 6 - Right heart catheterization showed passage of the catheter from the pulmonary artery to the aorta through the aortopulmonary window
- Video 7 - Pigtail catheter in the aorta showed the reversal of contrast from the pulmonary artery to the aorta, consistent with Eisenmenger physiology
- Video 8 - Computed tomography angiography 3D cine view showed the connection between the aorta and the pulmonary artery
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Article Type: Case Report
ELECTRON J GEN MED, Volume 21, Issue 6, December 2024, Article No: em613
https://doi.org/10.29333/ejgm/15646
Publication date: 26 Nov 2024
Article Views: 119
Article Downloads: 117
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