Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Asymptomatic Tumor Thrombus in the Left Atrium from Squamous Cell Carcinoma

A 67-year-old female patient presented asymptomatically for further evaluation of a chest mass. Other than significant smoking history, the patient had been healthy with a recently treated case of uncomplicated pneumonia. The mass originated in the aortopulmonary window of the left mediastinum and invaded proximally into the left superior pulmonary vein and subsequently into the left atrium. The mass protrusion into the mitral valve occupied 50% of the left atrium space but showed no clinical symptoms of a valvular blockade. Poorly differentiated squamous cell carcinoma was identified upon biopsy. These findings of a primary lung tumor with atrial extension in an asymptomatic patient point to the importance of age-appropriate screening and standardization treatment modalities.

Surgical Management for Late Presentation of Interrupted Aortic Arch with Aortopulmonary Window

We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.

An Overview of Aortopulmonary Window

Aortopulmonary (AP) window is exactly a rare congenital anomaly that represents approximately 0.2 and 0.5% of all congenital heart abnormalities. It consists of communication between the aorta and the pulmonary artery or its branches. Although closely related to AP window, the pulmonary artery origin from the ascending aorta (also termed “hemitruncus”) is classified as a separate defect. AP window was first described by Elliotson in 1830 in an autopsy study. In 1948, Robert Gross successfully ligated an AP window in a patient undergoing a thoracotomy for closure of a patent ductus arteriosus (PDA). In 1957, Cooley and associates described the first successful repair of AP window using cardiopulmonary bypass. Aortopulmonary window occurs due to the abnormal development of the heart’s major blood vessels during early fetal growth. In most cases, this heart defect occurs by chance, with no clear reason. This condition can occur on its own or with other heart defects such as: 1. Tetralogy of Fallot, 2. Pulmonary atresia, 3. Truncus arteriosus, 4. Atrial septal defect, 5. Patent ductus arteriosus, 6. Interrupted aortic arch. Treatment for aortopulmonary window involves surgery to close the hole between the aorta and the pulmonary artery with a patch or device. This surgery is normally done as soon as possible after the diagnosis is made, usually when the child is a newborn. Associated lesions are usually repaired during the same surgery. Associated lesions are usually repaired during the same surgery. More complex repairs and myocardial protection strategies are required in patients with associated lesions, increasing the morbidity and mortality associated with the operation.