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Table of Contents
Year : 2021  |  Volume : 24  |  Issue : 3  |  Page : 396-398
Bronchial decompression following repair of absent pulmonary valve: Fine-tuning by procedural fiberoptic bronchoscopy

Department of Cardiac Anesthesia and Cardiothoracic Surgery, Royal Hospital, Muscat, Oman

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Date of Submission13-Aug-2020
Date of Decision15-Aug-2020
Date of Acceptance27-Sep-2020
Date of Web Publication09-Jul-2021


Marked aneurysmal dilation of the central and branch pulmonary arteries in utero in patients with tetralogy of Fallot with absent pulmonary valve can often exhibit extrinsic compression of the trachea and bronchi. The major morbidity in these patients remains postoperative ventilation issues. This case report highlights the role of intraoperative bronchoscopy in providing guidance for obtaining optimal bronchial decompression that was achieved by an initial pulmonary arteriopexy followed by an aortopexy.

Keywords: Airway management, bronchoscopy, congenital heart disease, infant pulmonary valve insufficiency, tetralogy of Fallot

How to cite this article:
Maddali MM, Al-Kindi HN, Kandachar PS, Abolwafa A, Ahmed AE. Bronchial decompression following repair of absent pulmonary valve: Fine-tuning by procedural fiberoptic bronchoscopy. Ann Card Anaesth 2021;24:396-8

How to cite this URL:
Maddali MM, Al-Kindi HN, Kandachar PS, Abolwafa A, Ahmed AE. Bronchial decompression following repair of absent pulmonary valve: Fine-tuning by procedural fiberoptic bronchoscopy. Ann Card Anaesth [serial online] 2021 [cited 2022 Jan 22];24:396-8. Available from:

   Introduction Top

Tetralogy of Fallot is a relatively common congenital cardiac lesion and approximately 3% to 6% of these patients have an absent pulmonary valve [APV].[1],[2],[3] Patients with tetralogy of Fallot and an APV in utero, develop marked aneurysmal dilation of the main pulmonary artery and its branches resulting in the extrinsic compression of the trachea and bronchi leading to tracheomalacia and bronchomalacia.[4] These patients require in addition to an intracardiac repair of the tetralogy of Fallot, additional surgical strategies for reduction of bronchial obstruction like downsizing of pulmonary arteries, translocation of the pulmonary arteries anterior to the aorta and away from the tracheobronchial tree, excision of nearly all aneurysmal pulmonary arteries and placement of a pulmonary homograft/valved conduit.[3] In this case report we explore what other procedures may help to manage the problem of airway compromise persisting despite the standard methods already described. An infant with 1q21.1 chromosomal microdeletion presented with a tetralogy of Fallot complicated by absence of the pulmonary valve. The vital role played by real time intraoperative fiberoptic bronchoscopy in the successful achievement of airway decompression that otherwise would have contributed to postoperative respiratory morbidity is highlighted. Real time fiberoptic bronchoscopy initially identified the residual right bronchial compression after separation from cardiopulmonary bypass. Then bronchoscopy displayed the limited extent to which right bronchial decompression was achieved following the pulmonary arteriopexy. Subsequently when aortopexy was done, bronchoscopy confirmed the achievement of the optimal bronchial decompression. The institutional ethical committee approval [SRC#CR6#2020] as well as informed consent from the parents was obtained to publish the case report.

   Case History Top

A four-month-old boy [weight: 6 kgs; height: 64 cms; Body Surface Area: 0.3 m2], known to have tetralogy of Fallot with APV in combination with large branch pulmonary arteries obstructing the right and left bronchi was referred to the authors institution for urgent surgery. He had recurrent hospital admissions with shortness of breath and stridor. He was known to have 1q21.1 chromosomal microdeletion.

The preoperative transthoracic echocardiography showed an ostium secundum atrial septal defect [5 mm], a large malalignment-type ventricular septal defect [7 mm] with overriding of aorta and with a hypoplastic conal septum, dilated right ventricle with heavily trabeculated outflow tract and an APV. The peak pressure gradient across the right ventricular outflow tract was 80 mmHg and there was severe pulmonary regurgitation. The pulmonary arteries were grossly dilated [main pulmonary artery: 20 mm; right pulmonary artery: 15 mm and left pulmonary artery: 10 mm] with no patent ductus arteriosus. A computerized tomography of the chest showed significant dilatation of main pulmonary arteries, significant narrowing of bilateral main, lobar, and segmental bronchi at the level of the hilum [Figure 1]a. The child was posted for an urgent surgery.
Figure 1: (a and b). Computerized tomography of the chest at the level of 4th thoracic vertebra showing the compression of the right bronchus between the right pulmonary artery and the aorta preoperatively (a) and significant improvement postoperatively of the previously noted narrowing of the right bronchus (b)

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Keeping in mind the possibility that anesthesia introduction could result in airway compromise, the heart lung machine was primed and kept ready to institute emergency cardiopulmonary bypass support in case a life-threatening situation due to inability to ventilate/oxygenate the patient arose. After administration of general anesthesia without any airway compromise, a fiberoptic bronchoscopy was done that showed bilateral bronchial narrowing [80% pulsatile narrowing in the right and 50% in the left bronchus during systole]. The child underwent intracardiac repair on hypothermic cardiopulmonary bypass. The ventricular septal defect was closed with a bovine pericardial patch [Edwards Lifesciences Corp. Irvine, CA, USA]. A 14 mm glutaraldehyde-preserved valve-containing bovine jugular vein graft [Contegra, Medtronic Inc., Minneapolis MN, USA] was used to reconstruct the right ventricular outflow tract along with pulmonary artery reduction arterioplasty. The child was separated from cardiopulmonary bypass and a fiberoptic bronchoscopy was done using a 2.8 mm intubation fiberscope [11301 AA1, Karl Storz, Tuttlingen, Germany]. The lower trachea and the left main bronchus appeared wide with no external compression. The right bronchus appeared narrowed due to a pulsatile external mass [Figure 2]a. A right pulmonary arteriopexy was done by suturing the anterior aspect of the right pulmonary artery to the aorta to lift the pulmonary artery anteriorly reducing the external compression on the right bronchus [Figure 2]b and Videoclip 1]. As it was felt that the bronchial decompression was not optimal, an aortopexy was done under bronchoscopy guidance by suturing the aorta to the undersurface of the sternum. This helped further open up the right bronchus [Figure 2]c. The patient was shifted to the intensive care unit for further hemodynamic and ventilatory management. The child's trachea was extubated on the 5th postoperative day successfully and he was discharged home on the 12th postoperative day. A computerized tomography done during the 6th week following surgery revealed a complete relief of the previously noted narrowing of the distal trachea as well as narrowing of the proximal right and left mainstem bronchi [Figure 1]b. The child was doing well with no airway compromise at the 6 months follow up.
Figure 2: (a-c). Real time intraoperative bronchoscopy after separation from cardiopulmonary bypass displaying the right bronchial compression by an external mass (a), opening up of the right bronchus following pulmonary arteriopexy (b) and further opening up of the right bronchus after aortopexy (c)

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   Discussion Top

The successful management of an infant with tetralogy of Fallot and an APV is described. The child had repeated hospitalizations due to airway problems and resolving the airway-related issues was one of the top priorities in this child in addition to the surgical repair of the lesions. Real time intraoperative fiberoptic bronchoscopy played a major role in achieving this goal by providing the surgeon constant guidance. Initially the bronchoscopy could display the extent of right bronchial compression after separation of the child from cardiopulmonary bypass.

Bronchoscopy readily displayed the bronchial lumen opening up after the first stage pulmonary arteriopexy. Subsequently it was felt that the bronchial lumen could further be decompressed and hence the aorta was attached to the undersurface of the sternum in the form of an aortopexy. Bronchoscopy guided the surgeon to achieve the best possible lumen with the optimal tension to be exerted on the sutures used for arteriopexy and the aortopexy thereby avoiding hemodynamic compromise. Computerized tomography that was done a month post-operatively demonstrated that the technique is effective and the right bronchus showed adequate patency thereby avoiding any surgical or cardiological re-intervention.

Tracheobronchial decompression by suspension of the pulmonary artery to the retrosternal fascia in addition to an aneurysmorrhaphy in a patient with tetralogy of Fallot with APV was described earlier.[5] But pulmonary arteriopexy with aortopexy based on intraoperative bronchoscopy findings as was done in this current child was not reported to the best of the authors' knowledge.

In conclusion, in patients with tetralogy of Fallot with APV, real time intraoperative bronchoscopy can be a valuable tool in gaining an optimal airway patency during the primary repair of this complex cardiac lesion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.  Back to cited text no. 1
Kirshbom PM, Kogon BE. Tetralogy of Fallot with absent pulmonary valve syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004;7:65-71.  Back to cited text no. 2
Hraska V, Photiadis J, Schindler E, Sinzobahamvya N, Fink C, Haun C, et al. A novel approach to the repair of tetralogy of Fallot with absent pulmonary valve and the reduction of airway compression by the pulmonary artery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009;12:59-62.  Back to cited text no. 3
Jochman JD, Atkinson DB, Quinonez LG, Brown ML. Twenty years of anesthetic and perioperative management of patients with tetralogy of Fallot with absent pulmonary valve. J Cardiothorac Vasc Anesth 2017;31:918-21.  Back to cited text no. 4
Bove EL, Shaher RM, Alley R, McKneally M. Tetralogy of Fallot with absent pulmonary valve and aneurysm of the pulmonary artery: Report of two cases presenting as obstructive lung disease. J Pediatr 1972;81:339-43.  Back to cited text no. 5

Correspondence Address:
Madan M Maddali
Royal Hospital, P.B.No: 1331, P.C: 111, Muscat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aca.ACA_203_20

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  [Figure 1], [Figure 2]