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.

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.

Type A interrupted aortic arch and type III aortopulmonary window with anomalous origin of the right pulmonary artery from the aorta.

Interruption of the aortic arch, aortopulmonary window, and anomalous origin of the right pulmonary artery from the ascending aorta are very rare congenital anomalies. It is even rarer to have all three anomalies in the same setting. We present a case of a newborn who was diagnosed with these lesions and describe the primary repair of these anomalies.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas.

The association of tricuspid atresia with aortopulmonary window is exceptionally rare. We report a patient with tricuspid atresia, normally related great arteries, non-restrictive ventricular septal defect, and no pulmonary stenosis (type IC) with an aortopulmonary window who underwent successful initial surgical palliation. The unique anatomical feature of this case, other than the presence of the aortopulmonary window, was the absence of pulmonary stenosis at the level of either the ventricular septal defect or the pulmonary valve. All other reported cases have described some degree of restriction of anterograde pulmonary flow due to pulmonary stenosis or atresia.

Holt-Oram syndrome with aortopulmonary window--a rare association.

A 4-month-old male infant presented with recurrent cough for 2 months. He had a shortened right upper limb with absent right thumb and continuous murmur in the left parasternal area. The X-ray showed an absent radius and the first metacarpal and phalyngeal bones on the right side. Echocardiogram revealed aortopulmonary window and small secundum atrial septal defect. Aortopulmonary window was successfully treated by device closure. Holt-Oram syndrome with aortopulmonary window is an extremely rare association.

Visualizing the invisible: aortopulmonary window diagnosis enhanced by 3D computer graphics.

An aortopulmonary window is a rare disorder that occurs in 0.1­0.2% of all congenital disorders. Our patient was a 1-month-old boy weighing 4180 g. The patient had heart failure associated with an aortopulmonary window. We used 3-dimensional computer graphic software (Viewtify, SCIEMENT) for diagnosis based on DICOM data from contrast-enhanced computed tomography. This made it easy to identify anatomical landmarks and findings and select the most suitable approach. We avoided stenosis of the right pulmonary artery and aorta. We encountered a case of an aortopulmonary window in which 3-dimensional computer graphic software was helpful in selecting the surgical technique. We report this case using 3-dimensional computer graphic images and present a review of the literature.

Transpulmonary correction of an isolated aortopulmonary window in an adult.

We describe the operative management of a 65-year-old patient with a congenital type I aortopulmonary window. At surgery, heavy calcifications on the aortic side of the defect, and close proximity with the left coronary ostium, prevented patch repair from the transaortic access. Patch closure through a combined transpulmonary approach was therefore required. This case illustrates unique features of a late-presenting aortopulmonary window.

Transcatheter closure of a residual aortopulmonary window through internal jugular vein access.

The use of Amplatzer septal occluder for closing a residual aortopulmonary defect has been described. This is usually performed by femoral access. We report closure of a residual aortopulmonary defect using right internal jugular vein access in a patient who had no femoral access as a result of previous cannulation for surgical repair. The 1 cm defect was closed successfully using a 10 mm Amplatzer septal occluder. Technical difficulty anticipated was unfounded although it was more cumbersome than femoral access. Left to right cardiac defects including PDA and AP window are amenable to transcatheter closure through internal jugular vein access.

Traumatic Aortopulmonary Fistula: An Acquired Type 1 Aortopulmonary Window.

We report a patient with an acquired traumatic aortopulmonary window. The patient presented with an aortopulmonary fistula between the proximal ascending aorta and pulmonary trunk, which was missed on the initial hospital admission. The 26-year-old patient presented with high-output cardiac failure and examination features of a diastolic runoff. Patch closure of the defect using a sandwich technique was undertaken, with resolution of symptoms.

Aortopulmonary window.

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.

Primary repair of aortopulmonary window with an interrupted aortic arch in a very low-birth-weight premature neonate.

The aortopulmonary window concomitant with an interrupted aortic arch is a rare occurrence. We successfully performed an emergency one-stage surgical repair of the aortopulmonary window (type 1) concomitant with an interrupted aortic arch (type A) in the case of a very low-birth-weight (1230-g) premature 2-day-old neonate. We describe the diagnosis, surgery, and postoperative course of this rare occurrence.

Aortopulmonary window with subaortic fibrous stenosis and septal defect: surgery through a minimal right vertical infra-axillary thoracotomy.

Aortopulmonary window with subaortic stenosis and ventricular septal defect is an uncommon congenital cardiac malformation that is repaired using cardiopulmonary bypass. The authors describe a 3-year-old patient on whom we performed surgery through a minimal right vertical infra-axillary thoracotomy. This minimally invasive surgery is likely to be applicable in a few cases.

Large Unrepaired Aortopulmonary Window Presenting in Adulthood.

BACKGROUND: Aortopulmonary window is an uncommon congenital heart disease, with untreated cases not surviving beyond childhood. However, very rarely it can present in adult patients with features of pulmonary hypertension. Clinically these patients cannot be differentiated from other more common conditions with left to right shunt. Transthoracic echocardiography if performed meticulously, can depict the defect in aortopulmonary septum. RESULTS: We report a case of large unrepaired aortopulmonary window in a 23 years old patient, diagnosed on transthoracic echocardiography.

Remarkably Still Repairable Large Aortopulmonary Window in an Adult Patient.

Aortopulmonary window (APW) is an abnormal congenital connection between the main pulmonary artery (MPA) and the ascending aorta, with intact aortic and pulmonary valves, leading to heart failure or, if not repaired early, to pulmonary vascular obstructive disease. We report the rare case of an asymptomatic adult with an unrestrictive APW, whose pulmonary arterial hypertension was remarkably still reversible, permitting successful repair.

Double outlet right ventricle and aortopulmonary window in a patient with Cornelia de Lange syndrome: a novel association.

We report a patient with Cornelia de Lange syndrome with congenital cardiac malformation; double outlet right ventricle and aortopulmonary window. This is the first report describing this combination of findings for Cornelia de Lange syndrome. Behalf of this report, the pathogenesis of congenital heart disease in Cornelia de Lange syndrome was also revisited.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle-dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

Percutaneous closure of nonrestrictive aortopulmonary window in three infants.

Aortopulmonary (AP) window, a communication between ascending aorta and pulmonary artery, is usually nonrestrictive and causes severe pulmonary vascular obstructive disease early in life. Only in 10% of the cases it is restrictive. There are sporadic case reports [1-8] of device closure of AP window which are mostly confined to these restrictive AP windows, that too in adults or relatively older children. Till date there is a single case report of device closure of nonrestirctive AP window in an infant [8]. We report our single experience of device closure of large, nonrestrictive AP windows in 3 infants. Percutaneous closure of AP window in each of the three patients was done by a different type of device i.e. duct occluder, muscular VSD occluder and perimembranous VSD occluder.

Aortopulmonary window--a review of nine cases.

INTRODUCTION: Aortopulmonary window (APW) is a rare anomaly, accounting for 0.1% of congenital heart defects. It consists of a communication between the ascending aorta and the pulmonary artery in the presence of normal separate aortic and pulmonary valves. Early treatment is usually required in order to prevent the development of irreversible pulmonary hypertension. OBJECTIVE: To assess the results of treatment in all patients diagnosed at our institution between January 1994 and November 2007, based on a retrospective longitudinal study. RESULTS: Nine patients treated for APW were identified. Their ages at diagnosis ranged from two days to 23 years; eight were infants aged 2 +/- 2.9 months. In this group clinical presentation was congestive heart failure in all cases. Five patients had associated lesions (interrupted aortic arch: 2; coarctation of the aorta: 2; VSD: 1; ASD: 3). In seven cases the diagnosis was made on the basis of echocardiography only. In the other two it was by cardiac catheterization, one patient with coarctation of the aorta and the other an adult patient with a smaller lesion who was initially misdiagnosed as having ductus arteriosus. All patients had corrective surgery, via a transaortic approach, with implantation of an autologous pericardial patch. All patients are alive and only one case has a small residual shunt at the correction site. CONCLUSION: A diagnosis of APW should always be kept in mind in the differential diagnosis of a child with congestive heart failure. Associated congenital heart anomalies should be excluded. Surgical repair appears to provide good short- and long-term results.