Background
Vascular ring is rare congenital malformation, it accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem. With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. The primary symptomatology associated with vascular rings relates to the structures that are encircled by the ring, chiefly the trachea, and esophagus.
Case presentation
A 38-year-old man with diagnosed vascular ring. The patient has history of diabetes mellitus, hypertension and obesity. Since 2007 the patient has manifested the symptoms of dyspnea and bronchial astma was diagnosed. She has been several time hospitalized due to dysnea.The bronchial asthma was diagmosed. She had received inhalational bronchodilators, antibiotics, and several courses of corticosteroids over the past few years. On 2010 and 2011 her doughter and son were operated because of vascular ring. On 2011 unsuccesfool renal artery denervation procedure was performed. On admission the patient’s general condition was good. Physical examination revealed obesity and high blood pressure and mild inspiratory and expiratory wheezes over the upper anterior chest with stridor, though her pulse oximetry showed 100% oxygen saturation on room air. Laboratory studies have found: E – 4920000, Hg- 14mg%, Ht- 41%, Alb- 24,6 g/l, ASPAT- 16 U/L, ALAT- 31 U/L, SatO2 – 96%. Spirometry show a normal value of ventilatory parameters. A barium esophagram showed small compression of the thorasic esophagus. Bronchoscopy reveald reduction about 1/3 inside diameter of the trachea during a cough. In gastroscopy compression of the thorasic esophagus was present. Echocardiography showed normal both left and right ventricles size and function. Reconstructed 3-D images (right panel) show an aberrant right subclavian artery originating from the aortic arch distal to a common origin of the carotid arteries, the truncus bicaroticus, and the left subclavian artery.
Current guidelines
A symptomatic aberrant right subclavian artery may be encountered by vascular surgeons, and a right supraclavicular approach would appear the most appropriate method of repair. Knowledge of aortic arch and root vessel anomalies is important for any clinician dealing with cardiothoracic, head and neck, or intracranial pathology. Vascular surgeons may encounter these anomalies during routine practice and may need to deal with their clinical sequelae.
References
1. Atkin GK, Grieve PP, Vattipally VR, Ravikumar KH, Das SK The surgical management of aortic root vessel anomalies presenting in adults, Ann Vasc Surg. 2007 Jul;21(4):525-34.
2. Sitzman TJ, Mell MW, Acher CW Adult-onset dysphagia lusoria from an uncommon vascular ring: a case report and review of the literature Vasc Endovascular Surg. 2009 Feb-Mar;43(1):100-2
3. Phil A. Hart and Patrick S. Kamath Dysphagia Lusoria Mayo Clin Proc. 2012 March; 87(3): e17.
Expert’s comments:(Written authorization required from each expert)
1. J. Kołcz, MD, PhD
The surgery in adult patient is high risck, specialy because of neurological complication. The surgery should be performed by vascular surgen.
2. K. Bederski, MD
Vascular rings are rare congenital anomalies of the aortic arch and its branches that compress the trachea and/or esophagus. Symptoms include stridor, respiratory distress and dysphagia of different intensity. Surgery in children is effective treatment.
3. Prokop- Staszecka, MD, PhD
There is no cleare that patient s symptoms are connected with vascular ring. Further, particulary pulmonological diagnosis is needed.
Expert’s comments:(Written authorization required from each expert)
The patient was qualified for father pulmonary diagnosis and angiography.
Authors:
Prof. Piotr Podolec MD, PhD1, Lidia Tomkiewicz-Pająk MD1 , PhD, Leszek Drabik MD1 , Henryk Olechnowicz MD2 PhD, Prof. Maria Olszowska MD, PhD1
Experts:
J. Kołcz, MD, PhD, K. Bederski, MD, Prokop- Staszecka, MD, PhD3
1 Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow, Poland
2 Department of Thoracosurgery, John Paul II Hospital, Krakow, Poland
3 Department of Pulmonology, John Paul II Hospital, Krakow, Poland
