ABSTRACT
BACKGROUND AND OBJECTIVES: The aim of this study is to document the long-term outcome of patients with a vascular ring. METHODS: A single-center retrospective review of clinical symptoms was conducted in all patients born between 1980 and 2013, diagnosed with a complete vascular ring and at least 2 years of follow-up. Data were extracted from patient files and clinical symptoms were assessed by questionnaires sent to the parents. Age and diagnostic tools, type of surgery, postoperative complications, and the prevalence of clinical symptoms (stridor at rest, stridor with exercise, cough, exercise intolerance, dysphagia, and frequent respiratory infections) were reviewed. RESULTS: Fifty-one patients were included. The diagnosis was made before the age of 2 in 35/51. Surgery was performed in 41/51 patients with a limited number of reversible complications. The median follow-up was 8 (IQR 5-12) years. After 2 years, 21/51 patients were asymptomatic: 2/7 asymptomatic patients at diagnosis became symptomatic and symptoms resolved in 16/44 initially symptomatic patients. Consequently, 30/51 patients still had symptoms after 2 years. Of these, 16 had partial improvement, 5 stable symptoms, and 9 aggravation of symptoms. After 10 years, 26/36 patients were free of complaints. The most frequent long-term symptom was stridor. CONCLUSION: Surgical treatment of a congenital vascular ring is safe and mostly performed in patients with a double aortic arch. Conservative treatment for patients with little symptoms seems to be justifiable. A considerable number of patients have residual long-term symptoms after surgical relief of the vascular ring.
Subject(s)
Vascular Ring/diagnosis , Aorta, Thoracic/abnormalities , Child , Child, Preschool , Conservative Treatment , Cough , Deglutition Disorders , Exercise , Female , Humans , Male , Parents , Postoperative Complications , Respiratory Sounds , Retrospective Studies , Surveys and Questionnaires , Vascular Ring/surgery , Vascular Ring/therapyABSTRACT
Los anillos vasculares tienen una incidencia del 1 % entre las enfermedades cardiovasculares congénitas. Constituyen una malformación embriológica en la que el arco aórtico, sus ramas o las arterias pulmonares provocan presión sobre la tráquea y/o el esófago. Anatómicamente, se dividen en dos grupos, dependiendo de cómo rodean la tráquea y el esófago: completo o incompleto. La sintomatología suele iniciarse en los primeros meses de vida con estridor bifásico o espiratorio que aumenta con el llanto y la alimentación, tos traqueal de tonalidad metálica, infecciones respiratorias recurrentes, episodios de apnea refleja y cianosis, sibilancias, hiperextensión cervical, retracción esternal e intercostal, y dificultad en la alimentación. Se realizó un análisis retrospectivo de 28 pacientes con diagnóstico de anillo vascular que concurrieron a la consulta en el Servicio de Endoscopía Respiratoria del Hospital Garrahan entre enero de 2015 y septiembre de 2017.
Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented.
