Aortopulmonary Window Research Articles

Surgical repair of an aortopulmonary window and subaortic stenosis in an adolescent: case report

Surgical repair of an aortopulmonary window and subaortic stenosis in an adolescent: case report

Simple Aortopulmonary window: Outcomes of surgical repair at Vietnam National Children’s Hospital

Purpose: Evaluate the midterm outcomes of surgical repair for patients diagnosed with simple aortopulmonary window at Vietnam National Children’s Hospital. Methods: We retrospective review data of patients who underwent surgical repair for simple aortopulmonary window from May 2014 to December 2023 at Vietnam National Children’s Hospital. Patients who had ventricular septal defect, interrupted aortic arch, aortic arch hypoplasia, double outlet of the right ventricle, or Berry syndrome were excluded from this study. Results: There were 37 patients who underwent surgical repair in this study. The mean age at the time of surgery was 259.37 ± 702.59 days, and the mean weight was 5.04 ± 3.7 kg. According to the Society of Thoracic Surgeons Congenital Heart Database Committee classification, sixteen patients were type1, twelve were type2, and nine were type3. Two patients underwent ligated window, 3 had window division and sutures without bypass, and 32 had patch repair. There were 4 in-hospital deaths (10.8%), no late deaths, and no patients required cardiac reoperation during a mean follow-up of 62.09 ± 31.07 months. Conclusions: Midterm survival beyond discharge of simple aortopulmonary window is excellent.

Neonatal Diagnosis of Berry Syndrome: Interrupted Aortic Arch, Aortopulmonary Window, Intact Ventricular Septum, and Ascending Aortic Origin of the Right Pulmonary Artery.

Neonatal Diagnosis of Berry Syndrome: Interrupted Aortic Arch, Aortopulmonary Window, Intact Ventricular Septum, and Ascending Aortic Origin of the Right Pulmonary Artery.

A Rare Case of a Large Aortopulmonary Window with Anomalous Right Pulmonary Artery From Ascending Aorta in a Young Adult

Background: The aortopulmonary window, a rare congenital condition, is an abnormal communication between the ascending aorta and the major pulmonary artery. This anomaly often necessitates early surgical intervention because its prognosis is unfavorable. Cases of adult survival without treatment are exceedingly rare. This condition is typically fatal in childhood if left untreated. In rare instances, it may manifest in adults, presenting symptoms similar to those of pulmonary hypertension. These patients exhibit clinical presentations that are indistinguishable from those of more common conditions characterized by a left-to-right shunt. Through careful transthoracic echocardiography, it is possible to identify the defect in the aortopulmonary septum. Results: We present a unique case of a 23-year-old adult diagnosed with a large, unrepaired aortopulmonary window, illustrating the challenges in diagnosing and managing such rare adult presentations.

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.

Transcatheter sub-occlusion of an aortopulmonary window as a bridge to surgery.

Transcatheter sub-occlusion of an aortopulmonary window as a bridge to surgery.

Simularea ferestrei aortopulmonare anatomice folosind modelarea 3D în blender

The aortopulmonary window (APW) is a potential the­ra­peu­tic target for a variety of conditions, including con­ge­ni­tal heart disease and pulmonary hypertension. How­ever, traditional methods for simulating the APW are often invasive and carry significant risks. This paper ex­plores the potential of using 3D modelling in blender to de­velop a novel, noninvasive method for simulating the APW. We present a 3D model of the APW created in blen­der, which incorporates anatomical details such as the aorta, pul­mo­­­nary valve, and the surrounding tissues. The model is then used to simulate the effects of different sti­mu­la­tion tech­niques, such as electrical stimulation and pres­sure waves. Our results suggest that 3D modelling in blen­der can be a valuable tool for developing and testing new me­thods for simulating the APW. The proposed method has several potential advantages over traditional methods. It is noninvasive, which avoids the risks associated with sur­gery. It is also highly versatile, as the stimulation pa­ra­me­ters can be easily customized to the individual pa­tient. Ad­di­tio­nally, the 3D model can be used to visualize the effects of stimulation on the APW, which can provide va­lua­ble insights into the mechanisms of action. While further research is needed to validate the proposed method, our results suggest that 3D modelling in blender has the po­ten­tial to revolutionize the treatment of conditions affecting the APW.

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.

Berry syndrome is a rare congenital cardiac malformation, herein we report an adult male patient who was successfully repaired by one-stage surgery. 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. For complex cardiovascular malformations, the ideal treatment strategy must be tailored to the characteristics of the patient to provide maximum efficacy and safety.

Percutaneous closure of aortopulmonary window in a preterm infant

ABSTRACT Aorto-pulmonary window (APW) is a rare congenital heart disease. The non-restrictive types of APWs present at an early age and require early surgical correction. The transcatheter device closure in young infants pose significant challenges that include hemodynamic instability during arteriovenous loop formation and due to potential encroachment of the occlusive device on vital adjacent structures, and sizing of introducer sheaths and delivery systems. We report a successful transcatheter closure of an AP window in a 1.35 Kg preterm infant with heart failure requiring mechanical ventilation. To the best of our knowledge this is the smallest and youngest baby , wherein transcatheter device closure has been attempted for a non-restrictive aorto-pulmonary window. The procedure was done through a 4 French right femoral venous access, thus avoiding an arterial access. A 0.025 Terumo wire was maneuvered through the pulmonary artery across the APW without formation of an arteriovenous loop. The APW was successfully occluded with a Konar multi-functional device delivered via a 4-French delivery system. The baby could be successfully weaned off the ventilator and could be discharge. On 1 month follow up, baby was in good health and gaining adequate weight. Thus we conclude that careful selection of patients allows successful closure of APW, even in the smallest baby.

Aortopulmonary Window Device Closure

Aortopulmonary window (APW) is an uncommon congenital cardiac defect. Most infants presenting with APW will undergo conventional surgical repair. Experience with transcatheter closure of APW is limited. We report the case of a 5-year-old boy with APW, in whom transcatheter closure was performed successfully using Amplatzer ductal occluder device.

Berry syndrome: First Successful neonatal single-stage repair in India

ABSTRACT Berry et al. (1982) described a rare syndrome associated with distal aortopulmonary window, aortic origin of the right pulmonary artery, intact ventricular septum, and interruption or coarctation of the aorta. Here, we present the first neonatal case of single-stage repair of Berry syndrome in India. Timely surgery and skilled postoperative care define the short-term and long-term outcomes. Single-staged repair is preferred wherever feasible.

Operative Repair of Aortopulmonary Window: A 25-Year Experience.

Background: Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Methods: Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch. Median operative age was 19 days (range 2 days-1.5 years) via single-patch (n = 12, 41%), double-patch (n = 15, 52%), or ligation and division (n = 2, 7%). Results: The only mortality occurred in-hospital 1.4 years postoperatively following remote myocardial infarction. Factors associated with longer postoperative length of stay were complex APW (P = .003), genetic syndrome (P = .003), noncardiovascular comorbidities (P = .002), lower birth weight (P = .03), and lower operative weight (P = .03). Six patients (21%) with complex APW underwent unplanned cardiothoracic reintervention(s), including two with arch reintervention following arch advancement for interruption. Reintervention-free survival was similar for simple versus complex APW, operative age categories, and repair techniques. At median follow-up 5.5 years postoperatively, no patients had residual APW or persistent pulmonary hypertension, 1 (3%) had greater than mild ventricular dysfunction, and 25 (89% survivors) had NYHA class I functional status. Conclusions: Operative APW repair has excellent mid-term survival, durability, and functional status, regardless of operative age, cardiovascular comorbidities, or repair technique. Cardiac and noncardiac comorbidities may be associated with prolonged length of stay.

Innovative cardiovascular casting technique features the complex malformation of berry syndrome

BackgroundPrenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease. The proposed method provides a comprehensive display of the structural deformities, offering valuable insights for clinical practitioners seeking to comprehend this condition.Case presentationIn this report, we present a case where fetal echocardiography aided in diagnosing Berry syndrome, which was later confirmed through postpartum cardiovascular casting. Our experience highlights the importance of using the three-vessel view to diagnose APW and aortic origin of the right pulmonary artery. Additionally, obtaining true cross-sectional and sagittal views by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch is necessary to image IAA or coarctation of the aortic arch.ConclusionsEarly and accurate prenatal diagnosis of Berry syndrome is feasible and our cardiovascular cast can perfectly display the microvascular morphology of the fetal heart, which may have great application prospects for postpartum diagnosis and teaching of complex cardiac abnormalities.

Congenital Heart Defects in Patients with Molecularly Confirmed Sotos Syndrome.

Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning difficulties, and characteristic facial features caused by heterozygous pathogenetic variants in the NSD1 gene located on chromosome 5q35. The prevalence of heart defects (HDs) in individuals with Sotos syndrome is estimated to be around 15-40%. Septal defects and patent ductus arteriosus are the most commonly diagnosed malformations, but complex defects have also been reported. The aim of our study was to analyze the prevalence of HD, the anatomic types, and the genetic characteristics of 45 patients with Sotos syndrome carrying pathogenetic variants of NSD1 or a 5q35 deletion encompassing NSD1, who were followed at Bambino Gesù Children's Hospital in Rome. Thirty-nine of the forty-five patients (86.7%) had a mutation in NSD1, while six of the forty-five (13.3%) had a deletion. Most of the patients (62.2%, 28/45) were male, with a mean age of 14 ± 7 years (range 0.2-37 years). A total of 27/45 (60.0%) of the patients had heart defects, isolated or combined with other defects, including septal defects (12 patients), aortic anomalies (9 patients), mitral valve and/or tricuspid valve dysplasia/insufficiency (1 patient), patent ductus arteriosus (3 patients), left ventricular non-compaction/hypertrabeculated left ventricle (LV) (4 patients), aortic coarctation (1 patient), aortopulmonary window (1 patient), and pulmonary valve anomalies (3 patients). The prevalences of HD in the two subgroups (deletion versus intragenic mutation) were similar (66.7% (4/6) in the deletion group versus 58.91% (23/39) in the intragenic variant group). Our results showed a higher prevalence of HD in patients with Sotos syndrome in comparison to that described in the literature, with similar distributions of patients with mutated and deleted genes. An accurate and detailed echocardiogram should be performed in patients with Sotos syndrome at diagnosis, and a specific cardiological follow-up program is needed.

Tetralogy of Fallot with an anomalous origin of right coronary artery from the pulmonary artery in a case of type i aortopulmonary window—A diagnostic challenge

<p>Aortopulmonary (AP) window is a conotruncal defect with an incidence of < 1% amongst congenital heart defects. It often occurs in association with other congenital cardiac defects. Coronary artery anomalies in association with the AP window are infrequent. An anomalous right coronary artery (RCA) from the pulmonary artery (ARCAPA) is an extremely rare entity with 0.002% incidence in the general population. To the best of our knowledge, the triad of Tetralogy of Fallot (TOF), ARCAPA and AP window is not yet reported in the literature.</p>

Successful Percutaneous Closure of Large Aorto-Pulmonary Window in a 3-Year-Old Girl Using Occluder A DO I 6/8: A Case Report

This case report details the diagnosis, management, and successful percutaneous closure of a large aorto-pulmonary window with associated pulmonary hypertension in a 3-year-old female patient. The intervention involved the use of an Occluder ADO I 6/8 device. The report provides a comprehensive overview of echocardiographic findings and aortoangiography results, culminating in the therapeutic strategy and outcome.

Percutaneous Transcatheter Closure of a Post-device Residual Aorta-pulmonary Window

Introduction The aortopulmonary window (APW) is a rare congenital cardiac anomaly in which there is incomplete separation of the pulmonary artery and aorta. Percutaneous transcatheter closure is possible in a specific subset of APWs only, and surgery remains the mainstay of therapy. Clinical summary We report a rare case of percutaneous device closure of post-device residual APW in a 32-month-old baby who had residual shunt post the device closure at two months of age. Discussion Transcatheter closure of an aortopulmonary window is an effective and safe procedure for specific anatomic defects.

Case Report: Surgical Correction of Transposition of the Great Arteries with Aortopulmonary Window

Case Report: Surgical Correction of Transposition of the Great Arteries with Aortopulmonary Window

Iatrogenic Anomalous Origin of the Right Pulmonary Artery From the Aorta: A Rare Complication of Ligation of Aortopulmonary Window.

An aortopulmonary window (APW) is a communication between the main pulmonary artery (MPA) and the ascending aorta in the presence of two separate semilunar valves. Surgical strategies described for the management of APW include ligation, division and suturing, patch closure of APW (transaortic or transpulmonary or transwindow), and device closure. Ligation of a type-2 APW can result in stenosis or complete closure of the right pulmonary artery (RPA) off the MPA, while leaving the aorta still opening into the RPA. We describe a delayed presentation of iatrogenic anomalous origin of the RPA from the aorta, post APW ligation, and its management.