Ventana Aortopulmonar. reporte de caso

RESUMEN La ventana aortopulmonar es una malformación cardiaca congénita poco frecuente que se encuentra entre el 0,2% y 0,6% de todas las cardiopatías congénitas. Consiste en una comunicación entre la aorta ascendente y el tronco de la arteria pulmonar en presencia de dos arterias con dos válvulas semilunares. Se presenta el caso de un lactante de 6 meses con 5,860 kg remitido al servicio de cardiología infantil por dificultad respiratoria y escasa ganancia ponderal. Entre los métodos diagnósticos se realiza la ecocardiografía doppler color requiriendo posteriormente complementar dicho método con cateterismo cardiaco y posterior resolución quirúrgica.

ABSTRACT Aortopulmonary window is a rare congenital heart malformation that is found in between 0.2% and 0.6% of all congenital heart disease. It consists of a communication between the ascending aorta and the trunk of the pulmonary artery in the presence of two arteries with two semilunar valves. We present the case of a 6-month-old infant weighing 5,860 kg referred to the pediatric cardiology service due to respiratory distress and low weight gain. As part of the diagnostic workup, color Doppler echocardiography was performed, along with subsequent cardiac catheterization and surgical resolution.

Fatal outcome of congenital aortopulmonary window with patent ductus arteriosus complicating pregnancy

Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.

Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension / 中国胸心血管外科临床杂志

@#Objective    To study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension. Methods    The clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg. Results    Windows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal. Conclusion    APW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.

Ventana aortopulmonar: Reporte de caso / Aortopulmonary window: Case report

RESUMEN La ventana aortopulmonar constituye una infrecuente anomalía cardíaca congénita. En la literatura mundial se han publicado alrededor de 300 casos. Se ha asociado a defectos cardíacos complejos, lo cual dificulta su diagnóstico. Se presenta el caso de un lactante menor con sintomatologia de falla cardíaca congestiva; durante estancia en unidad de cuidados intensivos cursa con múltiples comorbilidades; posterior a lo cual es llevado a cirugía cardíaca correctiva con peso inferior a 2000 gramos, con evolución satisfactoria. Se resalta la importancia de la corrección temprana, con el fin de evitar la progresión a enfermedad vascular pulmonar irreversible.

ABSTRACT Aortopulmonary window is a rare congenital heart defect. About 300 cases have been published in the world literature. It has been associated with complex heart defects, which makes it difficult to diagnose. We present a case of a minor infant with symptomatology of congestive heart failure; during stay in intensive care unit presents multiple co-morbidities. Subsequent to that, it is taken to corrective cardiac surgery with weight inferior to 2000 grams, with satisfactory evolution. The importance of early correction is emphasized in order to avoid progression to irreversible pulmonary vascular disease.

A Large Aortopulmonary Window with a Ventricular Septal Defect: A Rare Combination Presenting at the Age of 16

Aortopulmonary window is a rare congenital anomaly with a communication between ascending aorta and main pulmonary artery. It may be associated with other cardiac malformations like aortic arch anomalies, ventricular septal defect, tetralogy of fallot etc. Survival beyond infancy is rare and early surgical intervention is important to prevent development of irreversible pulmonary hypertension. We report a rare case of larger Aortopulmonary window along with a large ventricular septal defect presenting at the age of 16 years.

Surgery treatment of Berry syndrome / 中华胸心血管外科杂志

Objective The purpose of this manuscript was to compare the outcomes of different surgical methods of Berry syndrome and to figure out the occurrence of postoperative morbidity.Methods From January 2003 through December 2017,nineteen infants with Berry syndrome underwent one-stage repair at Shanghai Children's Medical Center.There were 12 male and 7 female.Among them,six patients were neonates.The IAA morphology was type A in 17 patients and type B in 2 patients.The APW morphology was type Ⅰ a in 6 patients,type Ⅱ b in 11 patients,and type Ⅲ in 2 patients.Preoperative mechanical ventilation was required in 4 patients.Three different surgical correction techniques were employed to repair the APW and AORPA,including intra-aortic baffle in 8 patients,right pulmonary artery (RPA) detachment in 6 patients,and RPA angioplasty with aortic cuff in 5 patients.The descending aorta was then anastomosed to the aortic arch by an end-to-side anastomosis with a patch augmentation in the anterior wall.Results The mean CPB and aortic cross-clamp time was (146.7 ± 63.5)minutes (range,79 to 260 minutes) and (74.3 ± 27.4)minutes(range,46 to 147 minutes),respectively.There were 3 deaths.One patient died of severe pneumonia and multiorgan dysfunction on postoperative day 26.One patient suffered severe low cardiac output syndrome after surgery and died on postoperative day 1.One patient died of congestive heart failure at 2 months after discharge.Reoperations were required in 3 patients during the follow-up period.RPA arterioplasty with bovine pericardial patch augmentation was performed in 2 patients for RPA restenosis.Lecompte maneuverer was performed in 1 patient to release the compression of the RPA from the ascending aorta.Conclusion The mortality of one-stage repair of Berry syndrome was high.Surgical correction should be performed as soon as diagnosed.An intra-aortic baffle patch is suitable for type Ⅱ a APW defect patients beyond the neonatal period.Pulmonary hypertension crisis is important after surgery.RPA restenosis is the main reason for reoperation.

Ventana aortopulmonar / Aortopulmonary window

La ventana aortopulmonar es una causa poco frecuente de insuficiencia cardiaca en el neonato. Debe ser descartada en caso de existir edema pulmonar sin los shunts izquierda-derecha más frecuentes. Presentamos las imágenes ecocardiográficas de un recién nacido que ingresó con clínica de insuficiencia cardiaca en nuestra institución.

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 frequents left-right shunts. We report the echocardiographic images of a newborn who was admitted with symptoms of heart failure at our institution.

One-Stage Correction of Tetralogy of Fallot, Pulmonary Atresia and Major Aortopulmonary Collateral Artery Associated with Aortopulmonary Window / 日本心臓血管外科学会雑誌

A 4-year-old boy was born with cyanosis and was given a diagnosis of tetralogy of Fallot and pulmonary atresia. Echocardiography showed membranous atresia of the pulmonary trunk that was connected to the left side of the ascending aorta via an aortopulmonary window 3 mm in diameter. Four major aortopulmonary collateral arteries (MAPCAs) were detected by cardiac catheterization and computed tomography angiography prior to undergoing surgery at 4 years of age. We performed one-stage complete unifocalization and definitive repair via a median sternotomy. The MAPCA supplying the left lower lobe was anastomosed to the true left pulmonary artery and the pulmonary artery trunk was augmented with an autologous pericardium patch. We then reconstructed the right ventricular outflow tract using a transannular patch and simultaneously patch-closed the VSD. The right/left ventricle pressure ratio after weaning from cardiopulmonary bypass was 0.8. The postoperative course was uneventful and the patient was discharged 26 days later. Seven months after the procedure, the right/left ventricle pressure ratio was decreased to 0.56 on cardiac catheterization.

Interrupted Aortic Arch and Aortopulmonary Window Associated with Complete Atrioventricular Septal Defect.

We report a rare case of a neonate with interrupted aortic arch, aortopulmonary window and complete atrioventricular septal defect. To the best of our knowledge, this unusual triad has not been previously described. The main question of the surgical strategy for CAVSD, in setting of associated defects, is to classify the CAVSD as balanced or unbalanced.

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

PURPOSE: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases. MATERIALS AND METHODS: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed. RESULTS: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology. CONCLUSION: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Aortopulmonary Window / 대한흉부외과학회지

BACKGROUND: Aortopulmonary window (APW) is a very rare congenital heart anomaly, often associated with other cardiac anomalies. It causes a significant systemic to pulmonary artery shunt, which requires early surgical correction. Accurate diagnosis and surgical correction will bring good outcomes. The purpose of this study was to describe our 20-year experience of aortopulmonary window. MATERIAL AND METHOD: Between March 1985 and January 2005, 16 patients with APW underwent surgical repair. Mean age at operation was 157.8+/-245.3 (15.0~994.0) days and mean weight was 4.8+/-2.5 (1.7~10.7) kg. Patent ductus arteriosus (8), atrial septal defect (7), interrupted aortic arch (5), ventricular septal defect (4), patent foramen ovale (3), tricuspid valve regurgitation (3), mitral valve regurgitation (2), aortic valve regurgitation (1), coarctation of aorta (1), left superior vena cavae (1), and dextrocardia (1) were associated. Repair methods included 1) division of the APW with primary closure or patch closure of aorta and pulmonary artery primary closure or patch closure (11) and 2) intra-arterial patch closure (3). 3) Division of the window and descending aorta to APW anastomosis (2) in the patients with interrupted aortic arch or coarctation. RESULT: There was one death. The patient had 2.5 cm long severe tracheal stenosis from carina with tracheal bronchus supplying right upper lobe. The patient died at 5th post operative day due to massive tracheal bleeding. Patients with complex aortopulmonary window had longer intensive care unit and hospital stay and showed more morbidities and higher reoperation rates. 5 patients had reoperations due to left pulmonary artery stenosis (4), right pulmonary artery stenosis (2), and main pulmonary artery stenosis (1). The mean follow-up period was 6.8+/-5.6 (57.0 days~16.7 years)years and all patients belonged to NYHA class I. CONCLUSION: With early and prompt correction of APW, excellent surgical outcome can be expected. However, optimal surgical method needs to be established to decrease the rate of stenosis of pulmonary arteries.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

One-Stage Repair of Interrupted Aortic Arch and Aortopulmonary Window in a Neonate / 대한흉부외과학회지

The combination of interrupted aortic arch and aortopulmonary window is a rare presentation of congenital heart disease, which requires early diagnosis and surgical treatment. We describe a successful one-stage repair of the anomaly through median sternotomy in a 10-day-old neonate weighing 2.46 kg.

A Case of Transcatheter Occlusion of Aortopulmonary Window(APW) after PAW Banding

Aortopulrnonary window is an uncommon cardiac anomaly accounting for approximately 0.2-0.6% of all congenital cardiac anomalies in which there is a connection between the ascending aorta and pulmonary trunk. Since the first report of successful surgical repair, many investigators have advocated surgical closure of all types of aortopulmonary windows using different technique. The majority of aortopulmonary windows require surgical therapy due to extensive size or location. A few cases of interventional closure of APW with double umbrella and buttoned device are reported, but Gianturco coil has not been used previously. A case is described in an infant with a small aortopulmonary window which was closed by Gianturco coil after APW banding. He did not receive corrective operation because his parents who are Jehovahs Witnesses refused transfusion. Complete occlusion of the defect was achieved without complications. Transcatheter coil closure of a small aortopulmonary window is feasible in infancy and the technique is likely to be applicable in a few cases.

Transcatheter closure of congenital vascular malformations with cook coils and aGA amplatzer duct occluder in children / 中国介入心脏病学杂志

Objective To explore the method and therapeutic principle of transcatheter closure of coronary artery fistula, aortopulmonary collaterals and aortopulmonary window. Methods Interventional occlusion of 4 cases of coronary artery fistulas and 1 case of aortopulmonary collaterals with Cook coils, 1 case of coronary artery fistulas and 1 case of aortopulmonary window with AGA Amplatzer duct occluder. The mean age of 7 children were 8.5 years. Results Transcatheter closure of 7 cases of vascular malformations were successful. During the follow-up period of 1 month to 5 years, no residual shunt and other complications appeared. Conclusion The curative effect of transcatheter closure of vascular malformations was good. But for the occlusions′ success and prevention from complications occurrence, the indications of closure should be obeyed, the time of X-ray exposure should be less than 60 minutes.