Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome): Report of three cases and review of the literature

Abstract

Among the different anatomical forms of anomalous pulmonary venous return, that of the inferior vena cava is of particular interest for the following reasons: the special radiological pattern which is referred to as the scimitar sign and the associated anomalies which often occur in the lungs. We have successfully operated on three' patients who are reported in this paper, and we have included a review of the literature. CASE REPORTS CASE 1 Liliane D., aged 9, presented because of repeated episodes of 'bronchitis' since infancy. At the age of 2 years a diagnosis of atrial septal defect (A.S.D.) had been made. On examination she was noted to have retarded development and some bulging of the left hemithorax. On auscultation she had a systolic murmur of moderate degree in the second and third intercostal spaces on the left, radiating towards both axillae and the back and of maximum intensity in the right axilla. On radiography (Fig. 1) the right border of the heart was predominantly enlarged, showing increased vascularization and also an opacity along the right cardiac border. The electrocardiogram showed right axis deviation and hypertrophy of the right ventricle.Catheterization confirmed the existence of an A.S.D. of ostium secundum type. There was a leftto-right shunt of 3 volumes, and moderate elevation of the pulmonary artery pressure. The right ventricular pressure was 48 mm. Hg. Stenosis of the right pulmonary artery with a gradient of 20 mm. Hg across the obstruction was also found. Right anomalous pulmonary venous drainage was found, but the exact site was not known.Operation was performed on 18 September 1963, using a mid-sternal incision and extracorporeal circulation with a moderate degree of hypothermia. Findings at operation included a huge right atrial chamber, and a small aorta and superior vena cava.On opening the right pleural space it became evident 'Recently, a fourth patient has been successfully operated on.

The importance of chest radiograph in the diagnosis of scimitar syndrome

“…Em outras situações, poderemos ter A importância da radiografia de tórax no diagnóstico ... -Lucas E, et alii Figura 2 -TC de tórax demonstra a veia pulmonar direita anômala (seta) conectada na veia cava inferior, que se encontra bastante dilatada mais de um vaso de drenagem venosa, isto é, um dirigindose para o átrio esquerdo, e o outro, anormalmente, para a VCI. O suprimento arterial pulmonar também poderá variar amplamente 1,3 , podendo se originar da artéria pul-monar, das artérias brônquicas ou de vasos sistêmicos anômalos originários da aorta, isto é, como o caso descrito, produzindo um seqüestro pulmonar direito. Em cerca de metade dos casos, o tamanho do pulmão direito é discretamente reduzido, porém existem situações com hipoplasia pulmonar severa e conseqüente dextroposição cardíaca 2,4 .…”

“…Tem como elemento principal a drenagem anômala venosa pulmonar direita para a veia cava inferior, que se associa freqüentemente a alterações como dextrocardia sem situs inversus, hipoplasia do pulmão direito e da artéria pulmonar, anomalias do suprimento vascular do pulmão direito e malformações da segmentação brônqui-ca 1,10 . Os autores apresentam um caso de cujo diagnóstico suspeitou-se através da radiografia de tórax.…”

The scimitar syndrome: clinical spectrum and surgical treatment

“…4, the vein was deviated through the atrial septum into the left atrium, a technique which has been described in several other cases. In some of these cases, if the length of the scimitar was limited a patch sutured around an opening in the atrial septum or an interposition graft was used to make the connection with the left atrium [3,5,14,19,23,26,27]. In patient No.…”

“…PAPVC is found in 0.4-0.7% of adults at autopsy [13]. The scimitar syndrome forms about 3-5% of all PAPVC [14,15].…”

Scimitar Syndrome With Unilobar Right Lung– A Case Report

“…2 Scimitar syndrome will have a varied presentation from asymptomatic to severe symptomatic (pulmonary hypertension, heart failure). 3 Infants and neonates present with severe symptoms due to the underlying congenital cardiac defects like ASD, whereas adults present with recurrent respiratory tract infections or haemoptysis. 4 The diagnosis of scimitar syndrome can be made by chest x-ray, 2D echo, and other imaging techniques like contrast CT of lung, CT angiography and bronchoscopy.…”