Bronchoscopy-Guided Bronchial Blocker for Acute Right Pulmonary Artery Hemorrhage in an Infant With Berry Syndrome: A Case Report

Introduction

Berry syndrome is a rare, complex congenital cardiovascular anomaly requiring surgical correction in infancy [1]. A key feature of this condition is a narrow retro-aortic space, which predisposes patients to right pulmonary artery (RPA) stenosis after initial corrective surgery [2]. This stenosis often involves the RPA anastomotic site or the retro-aortic compressed segment, requiring close follow-up and possible reintervention [3]. These post-surgical vascular anomalies increase the risk of acute pulmonary hemorrhage during reinterventions, posing urgent airway management challenges.

Bronchial blockers (BBs) are useful for lung isolation and airway management, particularly among children, in whom double-lumen intubation is not feasible because of small airways [4,5]. When inserted into the main bronchus to enable 1-lung ventilation without tracheal catheter exchange, BBs offer advantages such as minimized airway manipulation and preservation of mucosal integrity [5,6]. Although BBs are used in minimally invasive thoracic surgery, their role in controlling acute pulmonary hemorrhage among infants with complex congenital heart disease is underreported. This scenario requires targeted airway intervention to rapidly isolate bleeding, protect contralateral lung function, and facilitate hemostasis, making BB-guided approaches highly relevant.

Case Report

INITIAL DIAGNOSIS:

An infant developed tachypneic, irregular breathing at 1 week of age, without grunting, coughing, nasal congestion, convulsions, or abdominal distension. The infant was admitted to a local hospital 12 days after birth and remained hospitalized until day 19, with intermittent fever peaking at 38°C and lasting 1 to 2 days. Initial management included nasal cannula oxygen from days 12 to 14 after birth; continuous positive airway pressure from days 14 to 18; intravenous cefotaxime for infection control; and intravenous immunoglobulin to enhance anti-infective activity. Refractory tachypnea prompted transfer to our hospital’s neonatal department 19 days after birth. Transthoracic echocardiography (TTE) performed 20 days after birth and cardiac great-vessel computed tomography angiography performed 21 days after birth confirmed Berry syndrome with complex congenital heart defects, as shown in Figures 1A and 2A. Key anomalies included an aortopulmonary window; ascending aortic origin of the RPA; aortic arch interruption; secundum atrial septal defect; patent ductus arteriosus; mild mitral and tricuspid regurgitation; and severe pulmonary hypertension, with an estimated right ventricular systolic pressure of at least 136 mmHg.

FIRST SURGERY:

Following multidisciplinary evaluation and exclusion of contraindications, the infant underwent RPA anterior repositioning and reconstruction 23 days after birth under general anesthesia with cardiopulmonary bypass. The procedure included aortopulmonary window repair, aortic arch interruption plasty, atrial septal defect repair, patent ductus arteriosus ligation, and RPA reimplantation. Post-discharge medications included hydrochlorothiazide 2.5 mg twice daily, spironolactone 2 mg twice daily, and captopril 0.625 mg every 8 hours for approximately 1 month. Anticoagulation therapy was temporarily withheld postoperatively; resumption before reintervention (described below) was not documented.

REINTERVENTION:

At 5 months of age, outpatient follow-up TTE showed post-Berry-syndrome repair findings, including RPA stenosis; clinically significant right ventricular enlargement with wall hypertrophy; smaller left heart chambers; severe tricuspid regurgitation; mild pulmonary regurgitation; and severe hypertension involving the right ventricle, main pulmonary artery, and left pulmonary artery. Right ventricular pressure was 96 to 136 mmHg, as shown in Figure 1B. Concurrent cardiac great-vessel computed tomography angiography confirmed post-surgical pulmonary arterial hypertension, RPA stenosis, right heart enlargement with right ventricular wall thickening, hypoplastic left heart, and narrowing at the aortic arch interruption site anastomosis, as shown in Figure 2B. At this stage, the patient displayed exertional dyspnea and lip cyanosis, exacerbated by crying and post-feeding vomiting, with slight growth lag relative to peers and decreased urine output.

Physical examination showed temperature 36.1°C; pulse 128 beats/min; respiratory rate 40 breaths/min; blood pressures 106/85 mmHg (right upper limb), 93/57 mmHg (left upper limb), 120/73 mmHg (left lower limb), and 124/78 mmHg (right lower limb); and oxygen saturation 95% on room air. Perioral cyanosis, tachypnea, and coarse bilateral breath sounds were noted, without pronounced crackles or wheezing. A grade 3/6 systolic precordial murmur was auscultated.

Upon completion of preoperative evaluations, the infant underwent right heart catheterization with angiography and percutaneous RPA balloon angioplasty at 5 months of age. Following supine positioning, routine sterile preparation and draping were performed over the groin, infraumbilical region, and upper third of the thighs. Right femoral vein puncture was performed, followed by percutaneous dilation and placement of a 6-Fr sheath. A 2-mg heparin bolus was administered. A pigtail catheter was inserted via the femoral vein, and right ventricular angiography showed main pulmonary artery dilation. Main pulmonary artery angiography demonstrated RPA stenosis distal to the bifurcation. A push catheter was advanced through the sheath to the distal RPA over a 0.014-inch (0.356-mm) guidewire. Hand-injected contrast demonstrated a 1.2×10 mm RPA stenosis with post-stenotic dilation to 4.9 mm, as shown in Figure 3A. Hemodynamic data included right ventricular pressure 64/6/31 mmHg, main pulmonary artery pressure 62/13/31 mmHg, RPA bifurcation pressure 75/29/47 mmHg, and distal RPA mean pressure 11 mmHg. The 23-mmHg proximal-to-distal RPA pressure gradient was mainly attributed to Berry syndrome’s inherent anatomical trait – namely, a narrow retro-aortic space compressing the RPA – and to intraoperative-manipulation-induced local vascular spasm, which further increased blood flow resistance.

A 4.5×15 mm balloon catheter was advanced over a long guidewire to the RPA; its midpoint was aligned with the stenosis. The balloon was inflated with iohexol until waist resolution and dilated twice at 2- to 3-minute intervals, as shown in Figure 3B. Subsequently, 6×40 mm and 8×20 mm balloon catheters were used for dilation via the same protocol, as shown in Figure 3C and 3D. After balloon removal, repeat pulmonary angiography demonstrated improved RPA visualization, reduced stenosis, and unobstructed flow through the previously narrowed segment, as shown in Figure 3E.

Echocardiography continued to demonstrate RPA stenosis and hypoplasia. Repeat angiography confirmed residual post-dilation stenosis, and implantation of a 6×15 mm stent was planned. During delivery sheath adjustment over the guidewire, oxygen saturation suddenly dropped to approximately 38%, heart rate was 120 to 140 beats/min, and blood pressure was 106/46 mmHg; bloody secretions were evident in the endotracheal tube (ETT). Immediate interventions included oxygen titration, ETT suctioning, epinephrine instillation, and bag-valve ventilation with positive end-expiratory pressure (PEEP). Oxygen saturation recovered to 100% within 9 minutes. Echocardiography revealed no clinically significant pericardial effusion and a normal ejection fraction. After communication with the family, stent implantation was temporarily deferred; catheters were removed, hemostasis was achieved via compression, and the site was dressed.

COMPLICATION AND MANAGEMENT:

The child showed persistent RPA hemorrhage. Post-resuscitation oxygen saturation was maintained above 90% on ventilatory support, and the patient was transferred to the Cardiac Intensive Care Unit for further management. Immediately after transfer, the infant developed progressive severe hemodynamic decompensation related to ongoing RPA hemorrhage. Chest compressions were initiated as an emergency resuscitative measure to maintain circulatory perfusion, and electrical suction was used to clear copious bloody secretions. Four sequential intravenous epinephrine boluses were administered during resuscitation. A continuous epinephrine infusion was subsequently initiated, together with pituitrin (an oxytocin-vasopressin combination), for ongoing vasoactive support. Sodium bicarbonate was given to correct acidosis, and normal saline was infused for volume expansion. The child regained spontaneous circulation. Ventilator-assisted ventilation and continuous infusions of vasoactive agents – epinephrine and pituitrin – were initiated. Pituitrin was selected because norepinephrine at 0.5 μg/kg/min failed to stabilize systolic blood pressure (55–65 mmHg); heart rate reached 160 beats/min, near the infant’s age-related upper limit. The vasopressin component of pituitrin increases systemic vascular tone and blood pressure without pronounced heart rate elevation, thereby preventing increased myocardial oxygen consumption. Pituitrin was initiated as a continuous infusion at 0.01 U/kg/min, which stabilized systolic blood pressure at 70 to 80 mmHg and heart rate at 140 to 150 beats/min.

The respiratory endoscopy service was consulted for bedside bronchoscopy. Given the infant’s small airway, with an estimated right main bronchus diameter of 3.5 mm, a 5-Fr flexible BB was selected to avoid airway mucosal injury and ensure bronchoscopy compatibility. The right main bronchus was occluded using the 5-Fr BB, with the balloon positioned at a depth of 15 cm. After inflation with 2 mL of air, airway pressure was continuously monitored. Peak airway pressure reached 22 cmH2O, approaching the 25 cmH2O safety threshold and prompting a 0.3-mL reduction in balloon volume (from 2.0 to 1.7 mL of air). This 1.7-mL volume was maintained during the approximately 24-hour retention period, with balloon deflation every 3 hours for brief right lung ventilation and reinflation to the target volume thereafter. Pressure subsequently stabilized at 18 to 20 cmH2O, preventing atelectasis. The bronchoscope was used for serial saline lavage, suctioning, and extraction of bloody thrombi with a foreign body basket, as shown in Figure 4A–4C. The BB’s position in the right main bronchus was confirmed; both the ETT and BB were secured.

Morning bronchoscopy 1 day post-reintervention revealed minor fresh bleeding around the BB. Repeat bronchoscopy in the afternoon showed no clinically significant fresh bleeding; the device was removed bronchoscopically, without subsequent right lung bleeding, as shown in Figure 4D. BB retention was approximately 24 hours, with balloon deflation every 3 hours to allow brief ventilation. Subsequent bronchoscopy demonstrated no fresh bleeding on direct visualization but revealed copious thick white secretions. Saline lavage and serial suctioning confirmed patent bronchial lumens.

OUTCOME:

Five days post-reintervention, the child was weaned from mechanical ventilation and transitioned to nasal high-flow oxygen. Nasal cannula oxygen was initiated 11 days post-reintervention and discontinued 13 days later. Treatment included aspirin for antiplatelet therapy; diuretics and captopril for cardiac load reduction and functional improvement; and sildenafil for pulmonary arterial hypertension management. The child remained stable and was discharged 19 days post-reintervention. Figure 5 details the complete diagnostic and treatment course (from neonatal diagnosis and initial surgery through staged reintervention at 5 months of age and discharge 19 days later).

Discussion

Pulmonary hemorrhage, particularly pulmonary artery hemorrhage, is a major life-threatening complication of congenital heart disease surgery, especially after complex cardiac reconstruction. Its occurrence is closely linked to cardiac dysfunction, pulmonary hypertension, and pulmonary vascular dysplasia [7]. In the present case, an infant with Berry syndrome underwent primary corrective surgery in the neonatal period, and the initial recovery was uneventful. However, follow-up TTE revealed RPA stenosis and severe pulmonary hypertension, leading to worsening cardiac dysfunction and respiratory failure that required rehospitalization. Initial balloon dilation was performed, but persistent RPA stenosis prompted planned stent placement. Massive RPA hemorrhage occurred suddenly during guidewire repositioning; mechanical injury was considered the primary cause.

Childhood pulmonary hemorrhage presents with diverse clinical features. In addition to hemoptysis, children may have dyspnea, tachypnea, and clinically significant hypoxemia. Cough is common, often beginning as nonproductive and later becoming blood-tinged. Severe cases may involve cyanosis and altered mental status, indicating respiratory failure [8]. The infant in the present case exhibited tachypnea, cyanosis, mild growth retardation, and decreased urine output, consistent with cardiac dysfunction. Intraoperative massive bloody secretions from the ETT indicated acute RPA hemorrhage.

Comprehensive evaluation is required for diagnosis. Essential tests include complete blood count, coagulation profiles, and blood gas analysis. Imaging studies, such as chest radiography and computed tomography, can detect abnormal pulmonary opacities suggestive of bleeding [9]. Flexible bronchoscopy – serving both diagnostic and therapeutic functions – is widely used clinically. It provides unique value in pulmonary hemorrhage management by enabling direct visualization of bronchial lesions, precise localization of the bleeding site, and targeted hemostatic intervention. Compared with conventional intratracheal instillation of hemostatic agents, this targeted approach – based on clear identification of the bleeding site – offers greater therapeutic specificity and more effective hemostasis [10]. In the present case, intraoperative massive RPA hemorrhage prompted bronchoscopic airway inspection and suctioning of bloody secretions. In consultation with the anesthesiologist, a 5-Fr BB was placed in the right main bronchus to achieve left lung 1-lung ventilation. The procedure proceeded smoothly; postoperative serial bronchoscopies were performed to examine the airways and suction secretions, then assist with BB removal.

The decision to use bronchoscopy-guided BB for this infant’s massive intraoperative pulmonary hemorrhage was informed by our prior experience concerning ultrasound-guided bronchoscopy and cryobiopsy among children with interstitial lung diseases. Prior literature has described the broader clinical utility and safety considerations of bronchoscopic biopsy techniques, including cryobiopsy, in pediatric pulmonary diagnostics [11]. Unlike adults, infants have unique airway characteristics that pose technical challenges for BB use, including a narrow airway (eg, right main bronchus measuring 3.5 mm), fragile mucosa prone to injury, high airway compliance, rapid respiratory rate, and small tidal volume. These factors make infants more sensitive to ventilation interruption and airway stimulation. To address these challenges, we selected a 5-Fr flexible BB, rather than larger adult sizes. Its slender design ensured compatibility with narrow infant airways while minimizing the risk of mucosal injury. Considering infants’ low airway pressure tolerance, balloon pressure was adjusted from 22 cmH2O to the range of 18 to 20 cmH2O, a value below adult thresholds. This adjustment achieved effective hemostasis while preventing atelectasis, thus reflecting individualized refinement of standard techniques for infants.

The successful rescue of our patient supports the efficacy of BB placement in preventing and managing pulmonary hemorrhage. Increasing evidence indicates that BB-guided 1-lung ventilation benefits thoracic surgery patients by reducing intraoperative complications and improving safety [12,13]. Bronchoscopy-guided BB placement also minimizes airway manipulation and improves positioning success. For children with sudden massive pulmonary hemorrhage, interventions such as pulmonary artery embolization, lobectomy, or extracorporeal membrane oxygenation are rarely feasible immediately. Prompt hemostasis is critical, creating a window for further treatment. In the present case, vascular spasm resolved after BB-mediated hemostasis, and 1-week follow-up ultrasound showed that the RPA transstenotic pressure gradient had decreased to 12 mmHg, reflecting the indirect benefit of hemorrhage control in reducing RPA resistance.

Treatment strategies are tailored to the etiology and severity of hemorrhage. Supportive care is fundamental, and mechanical ventilation is often required to maintain adequate oxygenation. In our department, acute pediatric pulmonary hemorrhage is primarily managed via synchronized intermittent mandatory ventilation combined with PEEP of 4 to 10 cmH2O. This strategy expands alveoli, reduces alveolar exudation, and compresses pulmonary capillaries to decrease blood flow, thereby exerting a pressure-mediated hemostatic effect. PEEP levels are adjusted individually based on hemorrhage severity and patient age, with appropriate prolongation of inspiratory time to improve gas exchange. Mean airway pressure is maintained at 15 to 20 cmH2O to ensure optimal ventilation efficiency [14]. Blood oxygen saturation must be closely monitored throughout treatment to assess ventilation efficacy and guide parameter adjustments. Other supportive measures include hemostatic agents, antibiotics, and blood product transfusions [15].

The prognosis of childhood pulmonary hemorrhage depends on timely diagnosis, prompt treatment, and the underlying disease. Early intervention improves outcomes and reduces complications such as acute respiratory distress syndrome, pulmonary fibrosis, and multiorgan failure. Close pediatric intensive care unit monitoring, supported by a multidisciplinary team including pulmonologists, cardiologists, and surgeons, is essential.

Conclusions

For resuscitation of pediatric massive pulmonary artery hemorrhage, in addition to routine rescue measures, collaboration between bronchoscopists – who provide airway visualization and secretion clearance – and anesthesiologists – who place BBs and implement 1-lung ventilation – offers an effective emergency strategy.