Arterial tortuosity syndrome is a rare disorder that might affect pulmonary circulation and cause pulmonary arterial hypertension. Surgery is the first treatment of choice but for patients who are not surgical candidates, balloon pulmonary angioplasty (BPA) and pulmonary arterial stenting might be another option. During these interventions, intervention related complications may occur. One of the most important complication for this procedure is reperfusion related lung injury and pulmonary edema.
A 22-year-old male patient admitted to our hospital with the diagnosis of congenital pulmonary artery stenosis with pulmonary hypertension due to arterial tortuosity syndrome. The patient is consulted with thoracic surgery and considered as inoperable because of severe and diffuse involvement of pulmonary arterial circulation. BPA and pumonary stenting with fractional flow reserve was decided as treatment of choice for the patient. During the procedure, just after pulmonary stenting of involved artery, reperfusion lung injury (RLI) and edema was occured and patient was transferred to coronary intensive care unit for the supportive care. After 24 hours of intensive diuresis and non-invasive positive pressure ventilation the complication was successfully managed.
Vascular tortuosity syndrome is a rare connective tissue disorder, which is caused by mutations mostly transmitted by an autosomal recessive manner. The main pathology is excessive proliferation in the arterial walls leading to tortuosity together with stenosis. If stenosis is diffuse in the pulmonary arteries, it may lead to increased pulmonary arterial pressure together with increased right ventricular pressure. Pulmonary stenting is an alternative treatment for inoperable congenital pulmonary arterial stenosis. However, the intervention has some potential complications that can be caused by the technique and the lesion type during the procedure or lung injury after reperfusion. It is important to manage the complications with the right approach for the safett and the success of the treatment.