Long term respiratory morbidity in patients with vascular rings: a review

Abstract

Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. Respiratory morbidity related to external airways compression is a major concern in children affected by vascular rings. Clinical presentation depends on the severity of the tracheal lumen reduction and the presence of associated tracheomalacia. Recurrent respiratory infections, wheezing, atelectasis, and hyperinflation are mostly reported. As they are nonspecific and therefore difficult to recognize, attention should be given to all children with history of respiratory distress, extubation failure, noisy breathing, and recurrent respiratory infections. Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients.

Keywords: Airway compression; Children; Complete vascular rings; Incomplete vascular ring; Tracheomalacia.

Fig. 1

3D volume rendering reconstruction of a cardiac CT in patient with Right Aortic Arch (RAA) and Aberrant Left Subclavian Artery (ALSA). The vascular structures together with the left duct ligament (not visible on CT) completely encircle the trachea (in blue) and the oesophagus (in violet). Panel A: front view. Panel B: back view. Panel C: trachea with the narrowing (red arrow) at the level of the ring

Fig. 2

3D volume rendering reconstruction of a cardiac CT in patient with Double Aortic Arch, constituted by a dominant right aortic arch (RAA) and a diminutive Left Aortic Arch (LAA). The vascular structures together completely encircle the trachea (in blue) and the oesophagus (in orange). Pulmonary artery in violet. Panel A: front view. Panel B: back view

Fig. 3

3D volume rendering reconstruction of a cardiac CT in patient with Left Pulmonary arterial sling. The Left Pulmonary Artery (LPA) arises posteriorly and distally from the Right Pulmonary Artery (RPA), coursing posteriorly to the trachea (T). A tracheal bronchus (commonly associated to LPA sling) is also detected (red arrow)

Fig. 4

Effect of intrathoracic pressure (Ppl) variation on tracheal lumen during a) inspiration and b) expiration phases. Ppl overcomes intra-tracheal pressure (Ptr) during expiration, leading to the lumen collapse (black arrows)

Fig. 5

Maximal expiratory flow-volume curve in a patient with complete vascular ring (RAA with mirror image branching and LLA). Note the flattening of the proximal portion of the expiratory flow-curve due to the external compression/tracheomalacia that affects to expiratory flow rates in the large airways but not in the peripheral ones. FEV1 2.12 L (85%), FVC 3.00 L (102%), FEV1/FVC 83%, FEF25-75% 1.82 L/s (65%)

Fig. 6

Algorithm for the diagnosis, treatment and monitoring of children affected by VR