Surgery for scimitar syndrome: the Melbourne experience

Abstract

Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the long-term follow-up.

Incidental detection of a curved radiopacity on the chest X-ray

“…[6] Surgical treatment is only indicated in scimitar syndrome if left to right shunt is significant or severe symptoms and right ventricular dilatation and concomitant cardiac lesions are seen. [7] The type of surgical management to be used is controversial and includes reimplantation of anomalous pulmonary vein into the left atrium, division of abnormal arterial supply of the lung, or partial/complete resection of the right lung. [8] In conclusion, with the increased usage of imaging modalities, scimitar syndrome may be detected incidentally and should be identified.…”

Oral squamous cell carcinoma in a patient with scimitar syndrome

“…General anesthesia and respiratory management through mechanical ventilation during surgery was deemed high risk because of the possibility of respiratory failure and impairment of pulmonary function. Furthermore, when patients with scimitar syndrome receive chemotherapy, there are several concerns regarding circulatory and respiratory damage during treatment because the syndrome is characterized by anomalous pulmonary venous drainage and pulmonary hypoplasia []. Cardiac failure caused by transfusion‐associated circulatory overload during systemic chemotherapy and the increased risk for interstitial pneumonia caused by chemotherapeutic agents associated with adverse side effects also need to be prevented [].…”

Transcatheter intervention in a child with scimitar syndrome

“…Recently, multiple reports have shown transcatheter intervention, including embolisation of the APC and closure of the cardiac defects, may improve symptoms, decrease pulmonary arterial pressure,11,12 and postpone or even eliminate the need for surgical correction 7,8,13. Instead of a surgical approach, patients with scimitar syndrome who have significant left-to-right shunt due to APC and other cardiac defects are largely suitable for transcatheter intervention.…”