Abstract
BACKGROUND Berry syndrome is a rare congenital cardiovascular disorder characterized by typical malformations (eg, right pulmonary artery [RPA] origin from the aorta, aortic arch interruption, and atrial septal defect), most of which require surgical correction in infancy or early childhood. CASE REPORT A 1-week-oldinfant presented with postnatal dyspnea, irregular respiration, and poor weight gain. A diagnosis of Berry syndrome was confirmed via transthoracic echocardiography and cardiac great-vessel computed tomography angiography. The infant first underwent successful initial surgery to correct the core heart malformations. Follow-up transthoracic echocardiography at 5 months of age revealed progressive RPA stenosis requiring staged interventional treatment. During preparation for stent deployment, the infant developed acute decompensation: oxygen saturation dropped sharply, and hemorrhagic tracheal secretions were observed, consistent with acute massive RPA hemorrhage. A multidisciplinary team (cardiac surgeons, anesthesiologists, and respiratory endoscopists) performed emergency intervention. Under bronchoscopic guidance, a 5-Fr bronchial blocker was inserted into the right main bronchus to occlude the bleeding site; the endotracheal tube was replaced; and bloody thrombi were removed via lavage, suction, and basket extraction. Post-procedure bronchoscopy confirmed patent left bronchi and no active RPA bleeding. The infant was extubated on post-intervention day 5 with stable hemodynamics and was discharged 2 weeks later with normal respiratory function. CONCLUSIONS This case represents the first successful application of bronchoscopy-guided bronchial blocker at our institution for control of acute massive RPA hemorrhage. For resuscitation of pediatric massive pulmonary artery hemorrhage, in addition to routine rescue measures, bronchoscopy-guided bronchial blocker placement offers an effective emergency strategy.