Scimitar Syndrome: Experience with 6 Patients

Abstract

Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous drainage to the inferior vena cava, causing a left-to-right shunt. Six patients with scimitar syndrome were diagnosed in our hospital between 2002 and 2008. There were 4 girls and 2 boys; 4 < 5 kg in weight, 2 < 8 kg in weight. Scimitar syndrome was suspected in 5 cases because of dextroversion, and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Computed tomography confirmed the diagnosis in all patients. Two patients had horseshoe lung, 2 had a unilobar right lung, 1 had a hypoplastic right lung, and 1 had a hypoplastic right lower lobe. Three patients had severe pulmonary arterial hypertension, 2 had moderate pulmonary arterial hypertension, and one had normal pulmonary arterial pressure. All patients had lower respiratory tract infections, volume loss of the right lung, a normal or hyperinflated left lung, dextroversion of the heart, and scimitar arteries from the descending aorta. Pneumonectomy was performed in 3 patients, lobectomy in 1, ligation of anomalous vessels in 1, and 1 died before surgery.

Evaluation of pulmonary vein variations and anomalies with 64 slice multi detector computed tomography

“…Also in our study, the prevalence of PAPVR was 0.25%. Scimitar syndrome is a form of par- tial anomalous venous drainage with an unknown exact incidence [16]. However, it is estimated to occur in two out of 100,000 births, with a 2:1 female preponderance [17].…”

Unique technique of surgery in an unusual variety of Scimitar syndrome: A Case Report

“…Our patient presented with heart murmur and was diagnosed at the age of seven years, so this case would be classified to the patients group of "adult" Scimitar syndrome [3,4]. In this patient we found an unknown variety with drainage of the right lower lobe to the inferior vena cava, from the right upper lobe to the superior vena cava and to the azygos vein and additionally an ASD.…”

“…Usually, there is a single vein that runs from the middle of the right lung to the cardiophrenic angle [3]. Another established variety is a doubled-arched vein in the upper and lower lung with drainage into the inferior vena cava [12].…”

“…Since the syndrome may be undetected in asymptomatic patients, the true incidence is difficult to determine [2,3]. Two different types of Scimitar syndrome can be identified.…”

Scimitar Syndrome and Azygos Continuation of the Inferior Vena Cava Diagnosed in an Adult-An Unusual Association

“…Although the first case report of anomalous pulmonary venous drainage into the inferior vena cava was published in 1836, 3 the pathogenesis of scimitar syndrome remains unclear. It seems to originate from a basic developmental disorder of the entire lung bud early in the embryogenesis.…”