Flexible Fiber-optic Bronchoscopy-directed Interventions in Children with Congenital Heart Diseases

INTRODUCTION

Children with congenital heart diseases (CHD) often have coexisting airway anomalies leading to airway obstruction.^1,2 Flexible fiber-optic bronchoscopy (FFB) can be performed at the bedside and provide plenty of useful information for managing such patients, preoperatively and postoperatively. The objectives of our study were to investigate the utility of FFB in children suffering with CHD and to study the effect of bronchoscopic findings on management decisions in the pediatric cardiac intensive care unit (PCICU).

MATERIALS AND METHODS

RESULTS

A total of 71 FFB were done in 58 patients with CHD over a period of 6 years (2012–2017). Nine patients required FFB twice, while two patients underwent bronchoscopy three times. The median age of patients was 2.5 months (range 1 day–12 years) and median weight was 3.4 kg (range 2–24 kg) (Table 1). Out of 58 patients, 40 (69%) were males. While 44 (62%) procedures were done transnasally, 27 (38%) were done via an endotracheal tube while patient was being mechanically ventilated.

Thirty-three patients had cyanotic congenital heart diseases (CCHD) and 25 patients had acyanotic congenital heart disease (ACHD). Among these, ventricular septal defect (VSD) (8/51) and transposition of great arteries (TGA) (9/51) were most common lesions in acyanotic and cyanotic heart diseases, respectively. Detailed distribution of various CHD is shown in Table 2. While 38 (53.5%) and 30 (42.3%) procedures were performed precardiac surgery and postcardiac surgery, respectively, 3 bronchoscopies were performed intraoperatively (cardiac repair with slide tracheoplasty).

DISCUSSION

Congenital heart disease is often associated with airway anomalies due to close anatomic proximity of cardiac and respiratory structures.^1–3 Flexible bronchoscopy allows the anatomic and functional assessment of the airway in patients with CHD at the bedside, before or after cardiac repair. Robotin et al.⁴ employed FFB in all patients suspected to have airway obstruction as first-line tool and found it to be 100% accurate in establishing diagnosis. While preoperative detection of airway obstruction allows surgical corrective measures during cardiac repair, postoperative detection helps in improving outcome and prognosis. Knowledge of coexisting airway anomalies is vital for better management of these patients. This study adds information about preoperative and postoperative indications of FFB and its role during surgery and in the management postoperative complications in CHD patients.

A wide spectrum of cardiac lesions is reported in this study including both cyanotic and acyanotic CHDs. Although number of patients with complex and simple CHD were equal (29 cases each), patients with complex CHD required multiple bronchoscopies. Airway assessment was done prior to surgical repair in nearly half of the patients. Lee et al.¹ reported similar profile of CHDs in their study. These results were reciprocated by Cerda et al.⁵ in their study who found no correlation between pulmonary disorder and type of cardiac lesion.

Among six different indications for FFB, most common were persistent atelectasis and persistent oxygen requirement, which included persistent invasive or noninvasive ventilation (NIV). These indications have been reported in several other pediatric series, as well as previous reports on cardiac patients.^3,5–7 Airway anomalies could be detected successfully in 69% of procedures with airway malacias being most common in our study. Lee et al. reported a diagnostic yield of 92% in their series of 52 cardiac patients with airway obstruction while Chapotte et al. reported 71% diagnostic yield.^1,2 The exact magnitude of airway problems in cardiac patients is still unknown as not all children with CHD undergo FFB assessment. In our study, tracheobronchitis was most frequently encountered FFB finding, though most common airway anomaly was tracheobronchomalacia.

Airway obstruction due to tracheobronchomalacia, tracheobronchitis, as well as extrinsic compression of airway is often witnessed with CHD and these were the cause for persistent atelectasis in our study as well. While most of the cases with airway malacias and airway compression were diagnosed preoperatively, 1 case with extrinsic compression of airway and 11 cases with tracheobronchomalacia were diagnosed after cardiac repair. Airway obstructions may not always be clinically evident in the preoperative period. Children with coexisting airway anomalies may become symptomatic in postoperative period due to intraoperative handling of airway and vascular structures, mechanical ventilation, or postoperative sepsis.^8,9 Only endotracheal vision can help evaluate severity of airway edema and trapped secretions.¹⁰ In our study, 51 procedures found tracheobronchitis as the cause for obstruction or worsening of existing anatomical obstruction.

Bronchoscopy was useful in determining postoperative complications like vocal cord palsy (3), subglottic stenosis (3), tracheobronchitis (25), and tracheobronchomalacia causing persistent atelectasis leading to difficult weaning from the ventilation support. One case each of laryngomalacia, congenital subglottic stenosis, congenital vocal cord palsy, and tracheomalacia presented with stridor preoperatively was diagnosed by FFB.

In our study, FFB had contributed in management of children in (36/71) 50.7% of procedures. This is comparable with Wood’s general series of 1,000 procedures, where contribution of FFB was in 76% of procedures.¹¹ Another general series of 200 FFB by Godfrey et al.⁶ estimated this percentage to be 90.5%. Persistent atelectasis was managed by BAL and prolonged PPV. Based on BAL culture reports and degree of airway inflammation, antibiotic change was advised. Findings of FFB suggested change of management from conservative to surgical at an early stage in 10 cases. This included tracheostomy (5/71) and slide tracheoplasty (5/71). Cerda et al.⁵ reported 5.8% cases where FFB findings proposed surgery. Intraoperative bronchoscopy was done in three cases, during slide tracheoplasty to demonstrate to surgeon the extent of tracheal abnormality before incision of trachea and to confirm resolution of airway obstruction and visual assessment of the airway lumen at the end of the corrective procedure.

Although very safe, FFB may present few complications like hypoxemia, bleeding, hemodynamic alterations like bradycardia, or sometimes post-bronchoscopy fever.^7,12 In our study, complications were noted in only 19% patients. These complications are reported earlier mainly in ICU settings by Nussbaum and Bar-Zohar and Sivan.¹³

CONCLUSION

Bedside FFB in children with CHD is a safe and an efficient tool with various diagnostic and therapeutic implications especially in children with symptoms suggestive of airway obstruction.

REFERENCES

1. Lee SL, Cheung YF, Leung MP, Ng YK, Tsoi NS. Airway obstruction in children with congenital heart disease: assessment by flexible bronchoscopy. Pediatr Pulmonol 2002;34(4):304–311. DOI: 10.1002/ppul.10164.

2. Chapotte C, Monrigal JP, Pezard P, Jeudy C, Subayi JB, De Brux JL, et al. Airway compression in children due to congenital heart disease: value of flexible fiberoptic bronchoscopic assessment. J Cardiothorac Vasc Anesth 1998;12:145–152. DOI: 10.1016/S1053-0770(98)90321-4.

3. Kockar T, Gunduz M, Oktem S, Gundogdu S, Demirel FG, Tastekin A, et al. Bronchoscopic findings in children with congenital heart diseases. European Respirat J 2015;46(S59): PA1355. DOI: 10.1183/13993003.congress-2015.PA1355.

4. Robotin M, Bruniaux J, Serraf A, Uva MS, Roussin R, Lacour-Gayet F, et al. Unusual forms of tracheobronchial compression in infants with congenital heart disease. J Thorac Cardiovasc 1996;112(2):415–423. DOI: 10.1016/S0022-5223(96)70269-6.

5. Cerda J, Chacón J, Reichhard C, Bertrand P, Holmgren NL, Clavería C, et al. Flexible fiberoptic bronchoscopy in children with heart diseases: a twelve years experience. Pediatr Pulmonol 2007;42(4):319–324. DOI: 10.1002/ppul.20577.

6. Godfrey S, Avital A, Maayan C, Rotschild M, Springer C. Yield from flexible bronchoscopy in children. Pediatr Pulmonol 1997;23(4):261–269. DOI: 10.1002/(SICI)1099-0496(199704)23:4<261::AID-PPUL3>3.0.CO;2-P.

7. Nussbaum E. Pediatric fiberoptic bronchoscopy: clinical experience with 2,836 bronchoscopies. Pediatr Crit Care Med 2002;3:171–176. DOI: 10.1097/00130478-200204000-00015.

8. Corno A, Giamberti A, Giannico S, Marino B, Rossi E, Marcelletti C, et al. Airway obstructions associated with congenital heart disease in infancy. J Thorac Cardiovasc Surg 1990;99(6):1091–1098.

9. Corno A, Picardo S, Ballerini L, Gugliantini P, Marcellett C. Bronchial compression by dilated pulmonary artery. Surgical treatment. J Thorac Cardiovasc Surg 1985;90(5):706–710. DOI: 10.1016/S0022-5223(19)38538-1.

10. Yamaguchi D, Tanigami H, Suematsu Y, Murakami A, Kitsuta Y, Ishii T, et al. Airway obstruction in children due to congenital heart disease (our 15 years experience). Jpn J Trauma Emerg Med 2012;3(1):17–24.

11. Wood RE. The emerging role of flexible bronchoscopy in pediatrics. Clin Chest Med 2001;22(2):311–317. DOI: 10.1016/S0272-5231(05)70045-9.

12. Fonseca MT, Camargos PA, AbouTaam R, Le Bourgeois M, Scheinmann P, de Blic J. Incidence rate and factors related to post-bronchoalveolar lavage fever in children. Respiration 2007;74(6):653–658. DOI: 10.1159/000107737.

13. Bar-Zohar D, Sivan Y. The yield of flexible fiberoptic bronchoscopy in pediatric intensive care patients. Chest 2004;126(4):1353–1359. DOI: 10.1378/chest.126.4.1353.