Functional capacity and quality of life in patients with vascular ring

Background Vascular rings are congenital anomalies of the aortic arch that compress the trachea and esophagus and may require corrective surgery. Data about the long-term effects of vascular rings are scarce. We aimed to evaluate the long-term cardiorespiratory, exercise capacity, and quality of life of vascular ring patients. Methods A single center prospective study evaluating spirometry, echocardiography, six-minute walk test (6MWT), cardiopulmonary exercise testing (CPET), and quality of life questionnaire (SF36) in patients with a diagnosis of vascular ring, with or without corrective surgery. Results Twenty-seven patients participated (11.9 +/- 6 years, 52% males). The most common diagnosis was double aortic arch (16 patients, 59%). Nineteen patients had corrective surgery (O) and 8 did not (NO). Pulmonary function tests were within normal range in both groups (FEV1% predicted O = 87.6 +/- 16.5, NO = 83 +/- 10.8%). However, 11/27 had abnormal FEV1, 5 had abnormal FVC, and 13 (48%) had flattening of the expiratory curve. 6MWD and oxygen uptake were similarly mildly reduced in both groups; (6MWD O = 80.1 +/- 10.7% predicted, NO = 74.1 +/- 10.9% and oxygen uptake O = 78.5 +/- 23.2% predicted, NO = 73.4 +/- 14.3%). Peak O-2 pulse (V?O-2/HR% predicted) was mildly reduced in the NO group (O = 88.4 +/- 17.3%, NO = 75.8 +/- 16.2%). Echocardiogram and SF36 scores were normal in all patients. Conclusions Long-term evaluation of patients born with vascular rings revealed mild pulmonary impairment, reduction in 6MWD, and oxygen uptake. The NO group had also mild reduced peak O-2 pulse. Larger, long-term studies assessing functional parameters in operated and non-operated patients are needed to assess disease/surgery limitation in patients with vascular rings. Clinical trial registration number: NCT04781738.