Kenan Abdurrahman Kara, Ergin Arslanoğlu, Fatih Tomrukçu, Abdullah Arif Yılmaz, Fatih Yiğit, Hakan Ceyran

Department of Pediatric Cardiovascular Surgery, Kartal Koşuyolu High Specialization Training and Research Hospital, Istanbul, Turkey

Keywords: Abnormal pulmonary venous connection; congenital cardiopulmonary anomalies; increased pul- monary blood flow; pulmonary hypertension; scimitar syndrome.

Abstract

Introduction: Scimitar syndrome is a combination of rare congenital cardiopulmonary anomalies that can occur in 3% to 6% of patients with a partial abnormal venous connection. The presence of accompanying cardiac anomalies in these patients and in cases such as severe hypoplasia of the right lung or accompanying pulmonary artery hypertension necessitate early surgery in early infancy.

Patients and Methods: Nine patients with scimitar syndrome operated on in our pediatric cardiac surgery clinic from 2012 to 2020 were retrospectively examined in our study. The ages of the patients ranged from 1 to 47 years, with a mean of 18.1 ± 14.4. Patients’ weight values ranged from 9 to 88, with a mean of 47.0 ± 31.5. One patient died and mortality was 11.1%. Of the patients, 4 (44.4%) were male and 5 (55.6%) were female. Patients’ pulmonary arterial pressure ranged from 0.15 to 94 mmHg, with a mean of 39.2 ± 22.5.

Results: Close to 25% scimitar vein stenosis or scimitar vein drainage occlusion has been reported in the post- operative period, mostly in the newborn group in the literature. Two patients had non-critical stenosis during the third year follow-up despite the absence of stenosis or occlusion during the first two years of follow-up of nine patients we followed. Their surgical follow-up is still ongoing since they are asymptomatic.

Conclusion: The course of the disease depends on the follow-up of the patient, the timing of the surgery, and the quality of the anastomosis. The follow-up and treatment of these patients will be more accurate in advanced centers experienced in scimitar surgery.