Nir V; Bentur L; Zucker-Toledano M; Gur M; Adler Z; Hanna M; Toukan Y; Masarweh K; Hakim F; Bar-Yoseph R
Pediatric pulmonology [Pediatr Pulmonol] 2022 Aug 15.
Date of Electronic Publication: 2022 Aug 15.
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 eight did not (NO). Pulmonary function tests were within normal range in both groups (FEV 1 % predicted O=87.6±16.5, NO=83±10.8%). However, 11/27 had abnormal FEV 1 , five 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 (V̇O 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.