Abstract
The anomalous systemic arterial supply to the normal basal segment of the left lung, classically called Pryce’s type I intralobar pulmonary sequestration, rarely causes severe symptoms during early infancy. A male newborn with 21 trisomy developed tachypnea at 8 days of age and required management by nasal directional positive airway pressure (DPAP). A chest X-ray showed cardiomegaly, and echocardiography showed an atrial septum defect (ASD) accompanied by right heart failure. Contrast-enhanced computed tomography found an anomalous vessel arising from the descending thoracic aorta and returning to the left pulmonary vein through the left lower lung. These findings led to a diagnosis of anomalous systemic arterial supply to the normal basal segments (intralobar pulmonary sequestration Pryce type I). His pulmonary hypertension deteriorated owing to aortopulmonary shunting through the anomalous vessel and left-to-right intracardiac shunting through the ASD. The medical treatments for the right heart overload were ineffective and, as a result, nasal DPAP continued to be required. On postnatal day 43, the anomalous vessel was transected surgically, and a subsequent left lower lobectomy was avoided because the blood supply seemed to be maintained by the bronchial arteries. Soon after the surgery, the patient’s circulatory and respiratory systems were stabilized. The patient’s respiratory support was discontinued on postoperative day 3. The patient’s course remains good 7 months after surgery.
