CT Assessment of Aortopulmonary Septal Defect: How to Approach It?

An aortopulmonary septal defect or aortopulmonary window (APW) is a rare cardiovascular anomaly with direct communication between the ascending aorta and the main pulmonary artery leading to a left-to-right shunt. It is accompanied by other cardiovascular anomalies in approximately half of patients. In order to avoid irreversible sequelae, interventional or surgical treatment should be performed as soon as possible. Cardiovascular CT, as a fast, non-invasive technique with excellent spatial resolution, has an increasing role in the evaluation of patients with APW, enabling precise and detailed planning of surgical treatment of APW and associated anomalies if present. This article aims to review the anatomical and clinical features of aortopulmonary septal defect with special emphasis on its detection and characterization by a CT examination.

Device closure of residual aortopulmonary window using Konar-MF occluder device: an attractive new option.

We report a case of a residual aortopulmonary window where a new Konar-MF occluder device was used to close the defect. This device has a low profile and conforms to the anatomy of aortopulmonary window very nicely without unnecessary protrusion on either side. This report highlights the advantage of Konar-MF occluder device for closure of such defects.

Successful one-stage surgical repair in a rare adult patient with berry syndrome.

BACKGROUND: Berry syndrome is a rare congenital cardiac malformation, herein we report an adult male patient who was successfully repaired by one-stage surgery. CASE DESCRIPTION: 18-year-old male patient presenting symptoms of chest tightness and shortness for over a year presented to outpatient clinic in our department to have corrective procedure heart. CTA revealed an Aortopulmonary Window (APW) type III, measuring 4.6 cm in maximum diameter. The right pulmonary artery originated from the ascending aorta, and the pulmonary trunk exhibited dilation with dimensions of 8.3 cm × 5.7 cm × 5.9 cm. Additional findings included Interrupted Aortic Arch (IAA) type A, intact ventricular septum, and Patent Ductus Arteriosus (PDA). Echocardiography showed bidirectional shunt at the level of APW in severe pulmonary hypertension. The right heart catheterization indicated a mean pulmonary artery pressure of 70mmHg and a pulmonary artery resistance of 5 Wood units. We evaluated after two weeks of treatment with epoprostenol at a rate of 20 ng/(kg. min) and found a significant improvement in pulmonary-artery pressure. Finally, we communicated with the patient's family and decided to proceed with the procedure. CONCLUSIONS: For complex cardiovascular malformations, the ideal treatment strategy must be tailored to the characteristics of the patient to provide maximum efficacy and safety.

3D Echocardiographic and Computed Tomographic Angiography Guidance for Percutaneous Closure of a Type II (Intermediate) Aortopulmonary Window.

A 4-year-old female was diagnosed with Type II Aortopulmonary window after being seen for a murmur. By utilizing multimodality advanced imaging, including 2D and 3D echo, computed tomography (CTA) with semi-transparent overlay as a road map, we were able to preoperatively plan camera angles and device selection as well as close the defect percutaneously under echocardiography guidance. This report highlights the importance of a multimodality imaging approach to interventional procedures.

Prognostic significance of mediastinal standardized uptake value on positron emission tomography/computed tomography in patients with left upper lobe non-small cell lung cancer: Is invasive staging of aortopulmonary window lymph nodes necessary?

Background: This study aims to investigate whether the invasive staging of aortopulmonary window lymph nodes could be omitted in the presence of a suspected isolated metastasis in the aortopulmonary window lymph node on positron emission tomography/computed tomography. Methods: Between January 2010 and January 2016, a total of 67 patients (54 males, 13 females; mean age: 59.9±8.7 years; range, 44 to 76 years) with metastatic left upper lobe tumors to aortopulmonary window lymph nodes were retrospectively analyzed. According to positron emission tomography/computed tomography findings in clinical staging, the patients were classified as positive (+) (n=33) and negative (-) (n=34) groups. Results: There was a statistically significant difference between the two groups in terms of sex distribution, lymph node diameter on computed tomography, maximum standardized uptake value of aortopulmonary window lymph nodes, and tumor diameter (p<0.001 for all). A trend toward significance was found to be in pT status, LN #6 metastases, and pathological stage between the two groups (p=0.067). The five-year overall survival rate for all patients was 42.4% and there was no significant difference between the groups (p=0.896). The maximum standardized uptake value of the aortopulmonary window lymph nodes was a poor prognostic factor for survival (area under the curve=0.533, 95% confidence interval: 0.407-0.675, p=0.648). Conclusion: Invasive staging of aortopulmonary window lymph nodes can be omitted in patients with isolated suspected metastasis to aortopulmonary window lymph nodes in non-small cell lung cancer of the left upper lobe.

Clinical features and pregnancy outcomes in women with aortopulmonary window defect: case series.

Background: Aortopulmonary window is a rare congenital heart defect that results in severe pulmonary arterial hypertension (PAH), Eisenmenger syndrome, and congestive heart failure in the first months of life. Pregnancy is absolutely contraindicated in the patients with this condition. Case summary: This paper describes two clinical cases of pregnancy in patients (28 and 20 years old) with aortopulmonary window defect, severe PAH, and Eisenmenger syndrome that ended in preterm delivery by caesarean section. One patient died in the postpartum period due to progression of right ventricular heart failure. The younger patient survived childbirth and the postpartum period; later, she continued therapy at the PAH centre. Discussion: We describe unusual cases of clinical features in pregnant women with aortopulmonary window defect. Due to the rare occurrence and low survival rate of patients with uncorrected aortopulmonary window defect, descriptions of clinical cases of this defect in adults are very rare. It is very important to note the necessity of observation of these patients in specialized centres by a multidisciplinary team of healthcare professionals, due to the high risk of cardiovascular, obstetric complications, and death.

Late Identification of a Stealthy Aorto-Pulmonary Window: A Case Report.

The term "aortopulmonary window" (APW), often referred to as "aortopulmonary septal defect," refers to a rare congenital medical disorder where there is an improper direct link between the main pulmonary artery and the ascending aorta. It can be combined with other cardiac congenital conditions or be an isolated lesion. Herein, we report the incidental discovery of a minor, restrictive aortopulmonary septal defect in a 60-year-old male who denied having any clinical symptoms. Incidentally detected APW in adulthood is uncommon and, hence, can be readily overlooked, a fortiori, in asymptomatic patients.

A rare disease detected in a school-age child aortopulmonary window with anomalous right coronary artery from the pulmonary artery.

Aortopulmonary window is a condition characterized by a communication between the pulmonary artery and the ascending aorta. The coexistence of aortopulmonary window and an anomalous right coronary artery originating from the pulmonary artery is rarely observed together, as mentioned in previous studies. In this report, we aim to describe our diagnostic and treatment experiences with a 6-year-old patient diagnosed with aortopulmonary window associated with an abnormal origin of the right coronary artery from the pulmonary artery.

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report.

BACKGROUND: Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation. CASE PRESENTATIONS: A 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful. CONCLUSIONS: We have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient's background requiring lung transplantation, and in these cases close postoperative follow-up is required.

Successful repair of interrupted aortic arch with aortopulmonary window associated with long gap oesophageal atresia and Type C tracheoesophageal fistula: challenging and rare case report.

Congenital heart diseases are most common anomalies associated with tracheoesophageal fistula (TEF) and oesophageal atresia (EA). The physiology and anatomy of heart disease is major determinant factor influencing outcome in patients with TEF/EA. We present a successfully treated case of complex congenital heart anomaly of interrupted aortic arch (IAA) with aortopulmonary window (APW) with TEF/EA.

Intermediate outcome for the single-stage surgical repair of aortopulmonary window associated with interrupted aortic arch.

OBJECTIVES: Aortopulmonary window (APW) associated with an interrupted aortic arch (IAA) is a rare cardiac malformation with significant mortality and morbidity. The goal of this study was to report the intermediate outcomes of single-stage repair concentrating on the surgical techniques and postoperative reintervention for this rare cardiac lesion. METHODS: Eleven patients were diagnosed with IAA-associated APW and underwent single-stage surgical repair at Vietnam National Children's Hospital. RESULTS: The APW anatomy types were types I, II and III in 1, 4 and 6 patients, respectively. The IAA morphology was type A in 9 patients and type B in 2 patients. The median age was 27 [interquartile range (IQR) 6-79] days, and the median weight was 3.5 (IQR 2.8-4.5) kg. The aortic arch was repaired using direct end-to-side tissue anastomosis in 7 patients, and patch aortoplasty was performed in 4 patients. Six patients underwent APW closure with an intra-aortic baffle, and 5 patients required right pulmonary artery detachment and reimplantation. One early death occurred. Four patients required reinterventions: 1 patient required reoperation due to aortic stenosis and 3 required balloon angioplasty for either recurrent aortic arch stenosis (n = 1) or right pulmonary stenosis (n = 2) with a mean follow-up time of 3.1 years (IQR 0.5-4.3 years). CONCLUSIONS: Single-stage repair of IAA-associated APW can be achieved with good survival outcomes in children. However, the need for repeat reintervention or reoperation remains high, and the growth of both the aorta and pulmonary arteries should follow carefully as the patient grows. CLINICAL REGISTRATION NUMBER: VNCH-RICH-18-96.

Congenital unilateral absence of right pulmonary artery with VSD and wide aortopulmonary window in an adult patient: a case report.

Unilateral absence of pulmonary artery (UAPA) is a very rare condition, with an estimated prevalence of 1 in 200,000 population, which is commonly associated with various cardiovascular anomalies or can occur in an isolated manner. Isolated cases survive to adulthood and remain asymptomatic, but they may frequently experience hemoptysis, repeated infections, or symptoms like dyspnea and chest pain. Due to the rarity of the disorder and its ambiguous appearance, diagnosis can be very challenging. Case presentation: We present a case of a 28-year-old male who visited our center with the diagnosis of ventricular septal defect with Eisenmenger syndrome elsewhere for further evaluation and was found to have right-sided UAPA with ipsilateral pulmonary hypoplasia and some associated cardiac anomalies. Clinical discussion: Discussions are held regarding typical chest radiograph findings, diagnostic methods, and possible therapies. Conclusion: Physicians should be aware of UAPA, which might go undiagnosed for several years despite regular medical care and can show up later in life, causing chronic respiratory symptoms along with Eisenmenger syndrome and ventricular septal defect like in our case.

Two-staged surgical repair of Berry syndrome type 2B.

Berry syndrome is a rare congenital heart disease that requires complete corrective surgery. In certain extreme cases, such as ours, a two-stage as opposed to single-stage repair is a possibility. In doing so, we also used annotated and segmented three-dimensional models for the first time in Berry syndrome, adding to growing evidence that such models enhance the understanding of complex anatomy for surgical planning.

Heart failure in a newborn with tetralogy of Fallot: uncommon association of a common anomaly.

Heart failure in tetralogy of Fallot in the newborn period is rare and is usually due to either large aortopulmonary collaterals or absent pulmonary valve syndrome with severe pulmonary regurgitation. Pink tetralogy of Fallot and tetralogy of Fallot with disconnected pulmonary arteries from the aorta may present beyond the newborn period with heart failure when Pulmonary vascular resistance (PVR) falls. We describe the diagnostic and therapeutic pathway in a rare case of heart failure in newborn with tetralogy of Fallot.

Computed Tomography Measurements in Assessment of Idiopathic Vocal Fold Paralysis.

OBJECTIVE: Our study aimed to both identify the incidence and clinical presentation of patients presenting with idiopathic vocal fold paralysis (IVFP). Secondarily we aimed to assess the radiographic findings of computed tomography (CT) studies along the course of the vagus and recurrent laryngeal nerves (RLN), specifically measuring for potential areas of compression at the skull base and mediastinum. We hypothesized that a proportion of patients diagnosed with IVFP would have significant differences in measurements when compared to age-gender matched controls on CT imaging. STUDY DESIGN: Institutional review board approved retrospective cohort study METHODS: We assessed patients presenting to a tertiary voice care center between 2003 and 2019 with diagnosis of vocal fold paralysis. Data collected includes demographics, clinical exam findings, and CT measurements including dimensions at bilateral jugular foramen, aortopulmonary (AP) window and surrounding vascular structures. Statistical analysis was then performed using Pearson χ2, and Mann Whitney U tests to determine differences variables of interest. RESULTS: A total of 606 patients were reviewed. Of these patients 60 (9.9%) patients were determined to have IVFP. Left-sided VFP was seen more commonly in 78.3% (N = 47) of idiopathic patients. Our IVFP group had an overall higher incidence of cardiovascular disease [OR = 3.378, 95%CI 1.907-5.941, P < 0.001] compared to those with identified causes for paralysis. Specifically, IVFP patients showed a higher incidence of combined cardiovascular co-morbidities (P < 0.001), mitral valve stenosis (P = 0.007), pulmonary hypertension (P < 0.0001) and congestive heart failure (P < 0.001). When evaluating CT imaging, the AP window was mentioned in only 2 (3.3%) of our idiopathic patients CT reports. The IVFP cohort had a lower median AP window volume (P = 0.020) when compared to age-gender matched controls. This significantly smaller AP window volume was also seen when only left sided IVFP compared to both right-sided paralysis (P < 0.001) and age matched control patients (P < 0.001). CONCLUSION: The significance of cardiovascular comorbidities combined with findings of statistically narrowed AP window may help provide an explanation of a subset of patients diagnosed with IVFP.

Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies.

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.

Evaluation of aortopulmonary window using virtual dissection of multidetector computed tomography angiography data sets.

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.

Tetralogy of Fallot with double aortic arch and aortopulmonary window: a very rare trifecta.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.