Tetralogy of Fallot with absent pulmonary valve syndrome and severe diffuse tracheobronchomalacia in an infant: the value of modified Lecompte manoeuver and reduction pulmonary arterioplasty

An 8-week-old, 3.4-kg infant who had a foetal diagnosis of TOF/APVS was intubated after birth due to severe tracheobronchomalacia. He was transferred to our institution while intubated, on vasopressor support. An initial evaluation confirmed the diagnosis. A computed tomography (CT) scan showed severely aneurysmal bilateral branch PAs and advanced/diffuse airway compression down to the lobar/lobular level. The majority of the left lung was occupied with a severely aneurysmal left PA with little lung parenchyma remaining. After the patient was stabilized, the decision was made to proceed with surgical repair.

The patient failed extubation due to severe tracheobronchomalacia and was deemed inoperable at that time prior to being transferred to our institution.

The chest X-ray shows increased bilateral pulmonary vascularity with a large mass occupying the majority of the left lung.

A preoperative CT scan shows severely dilated bilateral branch PAs with the left PA aneurysmal to the point of occupying the majority of the left lung.

The airway appears to be compressed down to the secondary and tertiary bronchial levels.

Bronchoscopic evaluation shows the severity and the extent of the tracheobronchomalacia, which was worse on the left side than on the right side. After a multidisciplinary discussion, the decision was made to proceed with surgical repair. His chest was noticeably hyperdynamic.

A median sternotomy is performed. The right lung is hyperinflated, an upper lobe lung abscess is drained and the lung is repaired. These steps are followed by a pericardiotomy. When the chest is entered, it is noted that the patient does not have a thymus gland, and he was proven later to have DiGeorge syndrome. The aneurysmally dilated branch PAs are evident.

Heparin is administered systemically. The ascending aorta is mobilized off the PA and its branches. The right PA is encircled with a vessel loop. We decide to cannulate the left common carotid artery indirectly with a 3.5-mm Gore-Tex graft, which is connected to an 8 Fr arterial cannula. Cardiopulmonary bypass (CPB) is initiated after both venae cavae are cannulated, and the patient is cooled to 28 °C. A left ventricular vent is placed through the enlarged left atrium. An ascending aorta cardioplegia needle is then placed, the aorta is cross-clamped and cardioplegic arrest is achieved with antegrade cardioplegia.

An oblique right atriotomy is then performed. Stay sutures are placed; the ventricular septal defect is visualized and is closed with an appropriately sized bovine pericardial patch, which is sewn using running 6-0 Prolene sutures and further reinforced with multiple interrupted 6-0 Prolene sutures that are placed in a horizonal mattress fashion and are supported by bovine pericardial pledgets. The tricuspid valve is tested and felt to be competent. The atrial septal defect is partially closed, leaving a 4-mm atrial level shunt. The right atriotomy is then closed using 2 layers of running 6-0 Prolene sutures.

We replace the bicaval cannulas with a single atrial cannula to facilitate the subsequent steps of the operation. An arteriotomy is made in the main PA, and the branches are evaluated. As is evident, they are massively aneurysmal prior to the origin of the lobar branches, which then arise abruptly as finger-like projections from the main branch PA (a typical view in TOF with APVS).

We continue our mobilization to thoroughly dissect and isolate the branch PAs. The goal is to reduce their size through a combination of reduction anterior pulmonary arterioplasty by resection of strips from the anterior wall of the dilated PAs and posterior wall plication.

The branch PAs are then repaired over a Hegar dilator to ensure adequate sizing and to avoid compromising any of the origins of the lobar branches. Once this process is complete, we prepare a segment of the valved femoral vein homograft to be utilized as a right ventricle (RV)-to-PA conduit. The heart is then de-aired, and the aortic cross-clamp is removed. The femoral vein with the valve is connected in an end-to-end fashion to the left branch PA. Its length is then tailored and is placed in an orthotopic position in the native pulmonary annulus. This procedure establishes the continuity between the RV and the left PA. Because of the significantly dilated branch PAs and the compression of the airways, we decide to translocate the right branch PA anterior to the ascending aorta to ensure relief of the compression on the airway caused by the dilated PA. A segment of the femoral vein is then used to bridge the gap between the anteriorly translocated right PA and the femoral vein homograft neo-pulmonary conduit. All of these parts are sewn in place using running 6-0 Prolene sutures.

The patient is then rewarmed and is weaned off CPB without difficulty. An epicardial echocardiogram confirms good biventricular function, a widely patent outflow tract and branch PAs with no significant gradient and no residual ventricular level shunt. The direct RV pressure measurement shows less than 50% of the systemic pressure with no significant right ventricular outflow tract gradient. The carotid artery graft is trimmed and left in situ. Temporary epicardial pacemaker wires are placed. The patient’s chest is closed 24 hours later.

The aortic cross-clamp and CPB times were 146 and 241 minutes, respectively. The remaining postoperative course was uneventful. A planned tracheostomy was performed a week later. Postoperative bronchoscopic evaluation showed significant improvement in his tracheobronchomalacia. A 7-month follow-up bronchoscopic evaluation revealed improvement in both his right and left lungs.