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Seminars in Thoracic and Cardiovascular... 2011Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into... (Review)
Review
Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. Aortopulmonary window may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. The most common associated lesions are arch abnormalities, specifically interrupted aortic arch and coarctation of the aorta. Antenatal diagnosis is rare. In the current era, early mortality following repair of simple aortopulmonary window approaches zero and depends on the presence of associated lesions, especially interrupted aortic arch. Long-term outcome should be excellent. Early morbidity includes pulmonary artery stenosis and residual aortopulmonary septal defects. Long-term follow-up is indicated to look for recurrent lesions such as the development of branch pulmonary artery stenosis and arch obstruction.
Topics: Aortopulmonary Septal Defect; Cardiac Surgical Procedures; Cardiopulmonary Bypass; Cardiovascular Abnormalities; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Rare Diseases; Risk Assessment; Severity of Illness Index; Survival Analysis; Treatment Outcome; Vascular Surgical Procedures
PubMed: 21444051
DOI: 10.1053/j.pcsu.2011.01.017 -
Echocardiography (Mount Kisco, N.Y.) Jun 2019An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate... (Review)
Review
An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.
Topics: Adult; Aortopulmonary Septal Defect; Echocardiography; Eisenmenger Complex; Humans
PubMed: 31116466
DOI: 10.1111/echo.14368 -
European Heart Journal May 2012
Topics: Aortopulmonary Septal Defect; Echocardiography, Doppler, Color; Female; Humans; Infant
PubMed: 21937480
DOI: 10.1093/eurheartj/ehr355 -
Cardiology in the Young Jan 2020Aortopulmonary window is a rare congenital heart lesion. It might be associated with other CHDs, as well as with anomalous origin of the coronary arteries. Anomalous... (Review)
Review
Aortopulmonary window is a rare congenital heart lesion. It might be associated with other CHDs, as well as with anomalous origin of the coronary arteries. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is the most commonly described coronary artery anomaly in association with aortopulmonary window. We are describing a premature neonate who was diagnosed to have aortopulmonary window and ARCAPA immediately after birth, and had a successful operation at the age of 4 months. This report highlights the importance of very careful assessment of the coronary arteries in patients with aortopulmonary window.
Topics: Aortic Coarctation; Aortopulmonary Septal Defect; Bioprosthesis; Blood Vessel Prosthesis Implantation; Coronary Vessel Anomalies; Echocardiography; Humans; Infant; Infant, Newborn; Infant, Premature, Diseases; Intensive Care Units, Neonatal; Male; Pulmonary Artery
PubMed: 31854282
DOI: 10.1017/S1047951119002543 -
Archivos de Cardiologia de Mexico 2015The aortopulmonary window is a rare cause of heart failure in the neonate. It must be ruled out if there are signs of pulmonary edema without the most frequent...
The aortopulmonary window is a rare cause of heart failure in the neonate. It must be ruled out if there are signs of pulmonary edema without the most frequent left-right shunts. We report the echocardiographic images of a newborn who was admitted with symptoms of heart failure at our institution.
Topics: Aortopulmonary Septal Defect; Female; Heart Failure; Humans; Infant, Newborn; Ultrasonography
PubMed: 25698530
DOI: 10.1016/j.acmx.2014.11.004 -
World Journal For Pediatric &... May 2022The aim of this study was to review our institutional experience with patients who underwent surgical repair of aortopulmonary window, either as an isolated lesion or...
The aim of this study was to review our institutional experience with patients who underwent surgical repair of aortopulmonary window, either as an isolated lesion or in association with other cardiac anomalies. Between January 2006 and December 2020, 183 patients underwent surgical repair of aortopulmonary window at our institute. Sixty-three patients had associated lesions (Group 1); 120 patients had isolated aortopulmonary window (Group 2). Median age was 7 months. The early mortality in Group 1 was significantly higher (12.7%) compared to Group 2 (0.8%) ( = .001). The most common associated anomaly was ventricular septal defect (29 patients). On univariable analysis, cardiopulmonary bypass time ( < .001), aortic cross-clamp time ( < .001), delayed chest closure ( = .02), sepsis ( = .006), tracheostomy ( = .002), extracorporeal membrane oxygenation ( < .001), associated lesions ( = .001), pulmonary artery hypertensive crisis ( < .001) were predictors for early mortality. On multivariable analysis only pulmonary artery hypertensive crisis was identified as predictor for early mortality ( = .03; odds ratio = 24). Survival at both 5 years and 8 years was 77% ± 6.5 in Group 1 and 98.8% ± 1.2 in Group 2 (≤.001). Freedom from reintervention at both 5 years and 8 years was 92.4% ± 5.2 in Group 1 and 100% in Group 2 ( = .055). Early outcomes of aortopulmonary window repair are excellent among patients in which this is an isolated lesion, as compared to those with associated lesions. Long-term outcomes in terms of freedom from reoperation are excellent in both the groups.
Topics: Aorta; Aortopulmonary Septal Defect; Follow-Up Studies; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Infant; Reoperation; Treatment Outcome
PubMed: 35446205
DOI: 10.1177/21501351221077888 -
Journal of Cardiac Surgery Dec 2022The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with aortopulmonary window on virtual dissection of multidetector...
AIM
The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with aortopulmonary window on virtual dissection of multidetector computed tomography (CT) angiography data sets.
MATERIAL AND METHODS
We conducted a retrospective search of our departmental database from January 2014 to September 2021 to identify patients with aortopulmonary window and relevant information was extracted from the electronic case records and from routine examination as well as virtual dissection of CT data sets.
RESULTS
An aortopulmonary window was observed in 26 patients (20 males; 6 females). Based on location of the defect, a distal aortopulmonary window was the most common subtype, seen in 13/26 (50%) patients followed by a proximal, complete and intermediate subtypes seen in 7/26 (27%), 5/26 (19%) and 1/26 (4%) patients respectively. Associated ventricular septal defect was observed in 9/26 (34.6%) patients while an interrupted aortic arch was present in 5/26 (19.2%) patients. Tetralogy of Fallot was seen in 5/26 (19.2%) patients. Anomalous origin of right pulmonary artery from ascending aorta and crossed pulmonary arteries were seen in 2/26 (7.6%) patients each. An isolated aortopulmonary window without any simple/complex congenital anomaly was seen in 10/26 (38.5%) patients.
CONCLUSION
Aortopulmonary window is associated with a wide gamut of cardiovascular lesions, with ventricular septal defect being the commonest associated anomaly followed by tetralogy of Fallot and interrupted aortic arch respectively. Virtual dissection of multidetector CT angiography allows detailed anatomical evaluation of aortopulmonary window, allowing a clear visualization of the defect and associated cardiovascular anomalies.
Topics: Male; Female; Humans; Multidetector Computed Tomography; Retrospective Studies; Tetralogy of Fallot; Aortopulmonary Septal Defect; Pulmonary Artery; Aortic Coarctation; Cardiovascular Abnormalities; Angiography; Heart Septal Defects, Ventricular
PubMed: 36321703
DOI: 10.1111/jocs.17075 -
Arquivos Brasileiros de Cardiologia Jul 1999Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We... (Review)
Review
OBJECTIVE
Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature.
METHODS
Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions.
RESULTS
Heart failure occurred in 14 patients, and cyanosis in 3:2 from GB (tetralogy of Fallot--TF, and double outlet right ventricle--DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients.
CONCLUSION
APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.
Topics: Adolescent; Adult; Aortopulmonary Septal Defect; Child; Child, Preschool; Echocardiography, Doppler, Color; Female; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Treatment Outcome
PubMed: 10684142
DOI: 10.1590/s0066-782x1999000700006 -
Interactive Cardiovascular and Thoracic... Nov 2017To study the anatomic and haemodynamic data and results of surgery in patients undergoing surgical repair of aortopulmonary window beyond infancy.
OBJECTIVES
To study the anatomic and haemodynamic data and results of surgery in patients undergoing surgical repair of aortopulmonary window beyond infancy.
METHODS
Between July 2005 and December 2015, 23 patients, older than 1 year undergoing surgery for aortopulmonary window were analysed retrospectively. Postoperative clinical and echocardiography follow-up were performed.
RESULTS
Median age and weight at repair was 4 years (range 14 months-12 years) and 12 kg (range 3.5-22 kg), respectively. Fifteen patients had Richardson's Type I, 6 patients had Type II and 2 patients had Type III aortopulmonary window. Six patients had associated defects. Baseline mean systolic pulmonary artery pressure was 101 ± 14.9 mmHg (range 80-130, median 100 mmHg) and pulmonary vascular resistance index was 9.6 ± 5.9 (median 7.7 Wood units/m2, range 3.7-23.5 Wood units/m2). Patch repair of aortopulmonary window was performed using the sandwich method (transwindow) (n = 15), transaortic (n = 3) and transpulmonary artery (n = 2) approaches; 2 patients underwent double ligation and 1 underwent division and suturing. Two patients underwent valved patch closure of aortopulmonary window and 1 patient underwent valved patch closure of associated ventricular septal defect. There were 2 in-hospital deaths: one due to intractable pulmonary hypertension and the other due to low cardiac output. Mean follow-up was 36 months (range 2-119 months). Eighteen patients were in NYHA Class I at last follow-up. There were no late deaths or reoperation.
CONCLUSIONS
Surgery can be safely undertaken beyond infancy in carefully selected patients of aortopulmonary window with acceptable early and mid-term outcomes.
Topics: Aortopulmonary Septal Defect; Cardiac Surgical Procedures; Child; Child, Preschool; Echocardiography; Female; Follow-Up Studies; Hemodynamics; Humans; Hypertension, Pulmonary; Infant; Ligation; Male; Postoperative Period; Reoperation; Retrospective Studies
PubMed: 28633352
DOI: 10.1093/icvts/ivx158