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.

Type ii aortopulmonary window with isolated left subclavian artery from left pulmonary artery

Abstract Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

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

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.

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.

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 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.

Surgical repair of D-TGA with an aortopulmonary window and ventricular septal defects / Correção cirúrgica da transposição das grandes artérias com janela aortopulmonar e defeitos do septo ventricular

D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.

A transposição das grandes artérias com uma janela aortopulmonar é uma anomalia congênita rara. Descrevemos um caso de transposição das grandes artérias com janela aortopulmonar e múltiplos defeitos do septo ventricular em um menino de 5 meses submetido a tratamento cirúrgico com sucesso.

Percutaneous device closure of nonrestrictive aortopulmonary window in a 4 months' infant.

Aortopulmonary window is a rare congenital heart disease occurring in 0.2-0.6% of all patients with congenital heart disease. It is usually nonrestrictive and is conventionally treated surgically at an early age to prevent the development of pulmonary vascular obstructive disease. In 10% of the patients, it is restrictive and case reports of its percutaneous closure are mostly limited to these patients. We report percutaneous device closure of nonrestrictive AP window in a 4 months old, 4 kg child. To the best of our knowledge this is the youngest child to have undergone percutaneous device closure of an AP window.

Janela aortopulmonar: impacto das lesões associadas no resultado cirúrgico / Aortopulmonary window: impact of associated lesions on surgical results

OBJETIVOS: A janela aortopulmonar (JAP) é uma comunicação entre a artéria pulmonar (AP) e a aorta ascendente na presença de duas valvas semilunares separadas. Nesta revisão, descrevemos nossa experiência na história natural da JAP e do impacto de lesões associadas nos resultados cirúrgicos de pacientes tratados em nosso serviço. MÉTODOS: Estudo longitudinal retrospectivo, com revisão dos prontuários dos pacientes diagnosticados entre 1995 e 2005. RESULTADOS: Dos 9 pacientes diagnosticados como portadores de JAP, 6 apresentavam cardiopatia associada. Sete pacientes foram submetidos a tratamento cirúrgico, ocorrendo dois óbitos. Um paciente teve a cirurgia contra-indicada pela presença de hipertensão pulmonar, e outro faleceu antes do procedimento cirúrgico por complicação infecciosa respiratória. CONCLUSÃO: Os resultados cirúrgicos são satisfatórios quando a JAP se apresenta como defeito isolado e quando a cirurgia é realizada precocemente, evitando-se o desenvolvimento de hipertensão arterial pulmonar (HAP) irreversível. A presença de cardiopatia congênita complexa associada é fator de pior prognóstico em nossa série de casos.

OBJECTIVES: The aortopulmonary window (APW) is a communication between the pulmonary artery (PA) and the ascending aorta in the presence of two separate semilunar valves. This review describes the natural history of the APW and the impact of associated defects on surgical results in patients treated at our institution. METHODS: Retrospective longitudinal study, based on the review of medical files of patients diagnosed between 1995 and 2005. RESULTS: Of nine patients diagnosed as having APW, six had associated lesions. Seven patients were submitted to surgical treatment with two deaths. One patient was not submitted to surgery due to pulmonary hypertension and another one died before the surgery due to a respiratory infection complication. CONCLUSION: The surgical results are satisfactory when the APW presents as an isolated defect and when surgery is performed early, preventing the development of irreversible arterial pulmonary hypertension (APH). The presence of associated complex congenital heart disease is a bad prognostic factor in our series.

Transposition of great arteries with aortopulmonary window: an unusual cause of prepared left ventricle at 11 months.

In patients with transposition of great arteries, presence of aortopulmonary window is very uncommon and associated with high morbidity and mortality. This report describes the case of an 11-month-old female patient in which aortopulmonary window was restrictive, and protected the patient from developing pulmonary vascular disease. The patient underwent successful arterial switch and repair of aortopulmonary window.

Transcatheter closure of aortopulmonary window.

Aortopulmonary window is an uncommon congenital cardiac defect. Most infants presenting with aortopulmonary window will require conventional surgical repair. Experience with transcatheter closure of aortopulmonary window is limited. We report the case of a 9-year-old girl with aortopulmonary window, in whom transcatheter closure was performed successfully using Amplatzer ductal occluder device.

Ventana aortopulmonar asociada a interrupción del arco aórtico. Descripción de tres casos clínicos / Aorto-pulmonary window associated to interrupted aortic arch. Description of three clinical cases

La asociación de ventana aortopulmonar (VAP) con interrupción del arco aórtico (IAA) es un hallazgo excepcional dentro del grupo de cardiopatías congénitas ductus dependientes. Presentamos nuestra experiencia reciente de tres casos clínicos (edades: tres días, cuatro días y cuatro meses) que ilustran distintas modalidades de presentación clínica y anatómica, que a su vez condicionan diferentes tácticas de tratamiento quirúrgico e influyen en la evolución postoperatoria. Todos los pacientes recibieron el tratamiento médico convencional, incluyendo la infusión de prostaglandina. El diagnóstico ecocardiográfico se confirmó en todos los casos con aortografía de contracorriente, la cual demostró en todos los pacientes IAA tipo A y VAP tipo I en los dos primeros, tipo II en el tercero. En un caso se procedió a interponer un segmento de vena cava superior izquierda para corregir la IAA, cerrando la VAP mediante ligadura, sin circulación extracorpórea (CEC). En el segundo caso, también sin CEC, se utilizó la arteria carótida izquierda para restituir la continuidad del arco aórtico, cerrando la VAP por ligadura simple. En el tercer caso, se reparó la IAA mediante anastomosis término-terminal ampliada y luego bajo CEC se interrumpió la VAP con cierre directo de la aorta y parche de pericardio autólogo en la pulmonar. El caso 1 tuvo una evolución tormentosa, requiriendo plicatura frénica y reparación de úlcera gástrica. En controles posteriores se demostró una obstrucción significativa del arco aórtico, que requirió angioplastia con buen resultado en el corto término. El caso 2 evolucionó sin complicaciones. El caso 3 falleció en postoperatorio inmediato. Se trataba de un paciente de diagnóstico tardío, con resistencia vascular pulmonar elevada.

Tetralogy of fallot coexisting with aortopulmonary window: a case report

Teralogy of Fallot and aortopulmonary window are rarely associated. One-day-old male infant diagnosed preoperatively in the Department of Pediatric Cardiothoracic Surgery in Children's Hospital Medical Center at Cincinnati, Ohio [United States], with truncus arteriosus, was discovered intraoperatively to have tetralogy of Fallot coexisting with aortopulmonary window. The child underwent a successful intraoperative repair. The hypothesis concerning the embryological pathogenesis of this association is discussed. The difficulty of preoperative diagnosis is emphasized