A rare coexistence: Hammock mitral valve and aortopulmonary window.

Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.

Transcatheter closure of aortopulmonary window with Amplatzer duct occluder II: additional size.

Aortopulmonary window is a rare cardiac defect, and early management with surgery or transcatheter closure is lifesaving. Here, a 9-month-old patient, who underwent a successful device closure with additional size-Amplatzer duct occlude, is presented to make emphasis that it may be considered as the device of choice for defects in close proximity to aortic valve and/or coronary ostium.

Prenatal Diagnosis of Aortopulmonary Window by 2-Dimensional Echocardiography: Summary of 8 Cases.

Aortopulmonary window (APW) is a rare congenital heart anomaly. A total of 8 cases with APW confirmed by echocardiography and surgery were retrospectively reviewed and the echocardiographic features analyzed. Among the 8 APW cases, 5 were type II and 3 were type III, the latter of which includes 2 cases complicated with Berry syndrome. Prenatal echocardiography can provide accurate information for the diagnosis of fetal APW. The prognosis depends on the timing of surgery and the nature of the associated cardiac anomalies.

Transposition of the great arteries: When echocardiography does not match the clinical picture.

Aortopulmonary window (APW) is a rare form of congenital heart disease seen in isolation or with complex cardiac lesions. APW has been associated with other cardiac defects such as interrupted aortic arch and Tetralogy of Fallot, but few cases have been reported of APW associated with transposition of the great arteries (TGA). In a newborn with TGA and intact ventricular septum, diagnosis of APW requires a high index of suspicion. This article reviews the literature on TGA with APW and illustrates the importance of additional evaluation in neonates with TGA when oxygen saturation and PaO2 do not match predicted clinical values.

Prenatal Diagnosis of an Aortopulmonary Window With an Interrupted Aortic Arch.

A prenatal aortopulmonary window with an interrupted aortic arch was detected in a 22-week-old fetus. The 3-vessel and trachea view showed a communication between the ascending aorta and the pulmonary artery. Early postnatal surgery was successful. A PubMed-based search identified all cases of prenatal aortopulmonary windows between 2002 and 2015. Nine articles were identified. The average gestational age at diagnosis was 28 weeks (range, 22-33 weeks). The most frequent aortopulmonary window was type I (40%). All cases were associated with congenital heart defects, mainly an interrupted aortic arch (50%). No chromosomal or extracardiac abnormalities were seen. Prenatal echocardiography is useful for early diagnosis of an aortopulmonary window. The prognosis depends on the time of surgery and the nature of the associated anomalies.

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.

Common Arterial Trunk Repair at the Red Cross War Memorial Hospital, Cape Town: A 20-Year Review of Surgical Practice and Outcomes.

BACKGROUND: This study describes the 20-year experience of managing common arterial trunk (CAT) in a low-and-middle-income country and compares the early and medium-term outcomes following the transition from conduit to nonconduit repair at the Red Cross War Memorial Children's Hospital. METHODS: Single-center retrospective study of consecutive patients aged less than 18 years who underwent repair of CAT from January 1999 to December 2018 at the Red Cross War Memorial Children's Hospital. Patients with interrupted aortic arch or previous pulmonary artery banding were excluded. RESULTS: Fifty-four patients had CAT repair during the study period. Thirty-four (63.0%) patients had a conduit repair, and 20 (37.0%) patients had a nonconduit repair. There were two intraoperative deaths. Thirty-day in-hospital mortality was 22.2% (12/54). Overall, in-hospital mortality was 29.6% (16/54). Eight (21.1%) late mortalities were observed. The actuarial survival for the conduit group was 77.5%, 53.4%, and 44.5% at 6, 12, and 27 months, respectively, and the nonconduit group was 58.6% at six months. The overall freedom from reoperation between the conduit group and nonconduit group was 66.2% versus 86.5%, 66.2% versus 76.9%, and 29.8% versus 64.1% at 1, 2, and 8 years, respectively. CONCLUSIONS: The outcomes following the transition to nonconduit repair for CAT in a low- and middle-income setting appear to be encouraging. There was no difference in mortality between conduit and nonconduit repairs, and importantly the results suggest a trend toward lower reintervention rates.

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.

Aortopulmonary septal defect with interrupted aortic arch in a monochorionic diamniotic twin pregnancy.

We report a monochorionic diamniotic twin pregnancy with prenatal diagnosis of aortopulmonary septal defect combined with type B interrupted aortic arch in one of the fetuses. The mother was referred for fetal echocardiography at 24 weeks' gestation due to suspected congenital heart disease. Prenatal echocardiography revealed a defect of 2.8 mm between the main pulmonary artery and the ascending aorta. The course of the ascending aorta was straight to the neck and head, and there was no continuity of the aortic arch after the origin of innominate and left common carotid arteries. Thus, aortopulmonary septal defect with type B interrupted aortic arch was suspected. Postnatal echocardiography confirmed the diagnosis, and surgical repair was performed on the 10th day after birth. The combination of aortopulmonary septal defect with type B interrupted aortic arch is a very rare condition that can be diagnosed by fetal echocardiographic examination in the second trimester of gestation. Prenatal diagnosis is important for the prognosis, since early surgical intervention is needed to prevent development of severe heart failure in the neonate.

Prenatal ultrasound diagnosis and management of fetal aortopulmonary septal defects: a case series.

BACKGROUND: To investigate the prenatal ultrasound diagnosis and management of patients with aortopulmonary septal defects (APSDs). METHODS: A total of 8 fetuses with APSDs who underwent fetal echocardiography at our hospital from January 2015 to January 2019 were retrospectively included in this study. RESULTS: Among the 8 fetuses, there were 4 cases of type I APSD, 3 cases were type II, and 1 case was type III. Among the 8 cases, there were 2 cases of simple APSD. There were echocardiographic characteristics that were common to all 3 types of APSD. This included defects between the ascending aorta and the trunk of the pulmonary artery in the short-axis section of the aorta, and in the three vessels and the three-vessel trachea section. Furthermore, the "V"-shaped structure confluence point of all APSD cases was positioned more forward than normal in the three-vessel trachea section. Type I APSD can be better characterized by the cross-section of the double outflow tract of the aorta and the pulmonary artery, which is close to the aortic valve and pulmonary valve. Type II APSD can be clearly diagnosed by the short-axis view of the aorta. Since the defect between the aorta and the pulmonary artery is distant from the aortic valve and pulmonary valve, the defect does not involve the bifurcation of the pulmonary artery and may be associated with an ectopic origin of the right pulmonary artery. Type III APSD is similar to a permanent arterial trunk, and the space between the ascending aorta and the trunk of the pulmonary artery is completely missing. Color and pulse Doppler showed shunt flow in the defects. CONCLUSIONS: APSD can be diagnosed and classified by fetal echocardiography. This, together with the presence or absence of fetal intracardiac and extracardiac deformities, can provide valuable prenatal information to pregnant women and their families, which may facilitate timely diagnosis and timely surgical treatment after birth.

Aortic Reconstruction Using a Main Pulmonary Artery Flap in an Isolated Aortopulmonary Window.

Aortopulmonary window (APW) is a rare cardiac anomaly that was reported to occur in only 43 cases over 33 years at a large-volume cardiac center. It can present as an isolated anomaly or in combination with another cardiac anomaly. The surgical technique for APW has evolved from simple ligation to separation of the 2 great arteries. However, because of the rarity of APW, there is no standard surgical treatment for this disease entity. Herein, we present successful aortic reconstruction using a main pulmonary artery flap after separation of the 2 great arteries in a neonate with isolated APW.

Type II aortopulmonary window with tetralogy of Fallot: successful repair.

Aortopulmonary window associated with tetralogy of Fallot is a rare cardiac anomaly. An 8-month-old boy presented with failure to thrive and recurrent chest infections. Echocardiography and imaging studies revealed a type II aortopulmonary window with tetralogy of Fallot. Corrective surgery in the form of patch closure of the aortopulmonary window and intracardiac repair of tetralogy of Fallot was carried out successfully.

Valved patch closure of aortopulmonary window.

The case of an 8-year-old boy with an aortopulmonary window who underwent unidirectional valved patch closure of the window is described. The advantages of unidirectional valved patch closure in this setting are discussed.

Aortopulmonary Window Associated with an Ascending Aorta Aneurysm in an Adult.

Aortopulmonary window (APW) is a rare congenital malformation. It results from an incomplete division between the ascending aorta and the pulmonary artery. We describe a 26-year-old male, who presented with a grade II exertional dyspnea and palpitations. Echocardiography revealed an APW with an ascending aorta aneurysm. He underwent surgery under cardiopulmonary bypass without aortic cross-clamping. The APW was closed via the pulmonary artery flap technique using an autologous pericardial patch, and the aneurysm was repaired through the reduction aortoplasty technique. The patient was discharged on the 4(th) postoperative day. At 2 years' follow-up, he had remained asymptomatic and echocardiography showed aortic valve competence, ascending aorta diameter of 38 mm, and no residual shunt.

One-Stage Repair of an Interrupted Aortic Arch with an Aortopulmonary Window in a Premature Neonate.

Interrupted aortic arch with an aortopulmonary window is a rare congenital entity that is associated with high morbidity and mortality, especially in premature low-birth-weight infants, and the proper timing of surgical correction remains a matter of debate. We present the case of a premature infant weighing 1.6 kg who successfully underwent one stage surgical repair to treat interrupted aortic arch with an aortopulmonary window. The therapeutic management of this patient is described below, and a review of the literature is presented.

Aortopulmonary window: a single institution surgical experience.

BACKGROUND: Aortopulmonary window is a rare cardiac anomaly. We report our experience with this rare lesion and highlight the criteria for treating this defect without the use of cardiopulmonary bypass. METHODS: From May 2007 to April 2012, 10 patients, aged 1 to 10 months and weighing 3.4 to 5.5 kg, were operated on by a single surgeon using both off-pump and standard techniques. All patients underwent preoperative and postoperative echocardiographic assessment, and were followed up with clinical examinations and echocardiography. RESULTS: There was no operative death and all patients were alive at the last follow-up. No major morbidities were noted. Two cases were operated on off-pump and they had shorter intensive care unit stays. All patients were in New York Heart Association class I on follow-up, with no residual shunt noted in follow-up echocardiograms. CONCLUSIONS: The surgical closure of aortopulmonary window carries a low surgical risk. Early surgical closure prevents the development of pulmonary vascular disease and achieves good immediate and long-term outcomes. Off-pump repair techniques, when used judiciously, have a place in the treatment of this defect.

Diagnosis, treatment and outcomes of patients with aortopulmonary window.

BACKGROUND: Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects. AIMS: To present our experience with the diagnosis and outcome of APW cases. STUDY DESIGN: Retrospective cohort study. METHODS: Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively. RESULTS: Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation. CONCLUSION: In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect.

Surgical repair of complex aortopulmonary window: a case study

Abstract Aortopulmonary septal defect, also known as the aortopulmonary window, is a rare congenital macrovascular malformation. This case involves a 9-year-old boy with aortopulmonary septal defect (type I combined with type IV). Before surgery, milrinone and alprostadil were used to counteract high lung pressure. Surgery was performed under cardiopulmonary bypass, following which the pulmonary pressure decreased. The aorta was cut, and the right pulmonary artery opening was connected with the main pulmonary artery septal defect using polyester patch. An internal tunnel was made, and the deformity correction was completed. The child exhibited normal postoperative recovery with no discomfort. A complex aortopulmonary window is a rare condition that can be treated successfully with appropriate preoperative and surgical management.

Aortopulmonary window in infants.

One of the rarest congenital heart diseases that results from a defect between the main pulmonary artery and the proximal aorta is named aortopulmonary window (APW). Such abnormality could be isolated, but in fifty percent of patients may be associated with other cardiac abnormalities, including arch abnormalities, specifically coarctation of the aorta, interrupted aortic arch, tetralogy of fallot, and atrial septal defect (ASD). Surgical closure or catheter-delivered devices is recommended in all patients with APW and should be performed after diagnosis as soon as possible to prevent irreversible pulmonary vascular disease. In the current era, early mortality following repair of simple APW is low and depends on the presence of associated lesions, especially interrupted aortic arch. We report an 8-month-old boy with APW who was referred to our center by respiratory symptoms and heart murmurs.