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What is scimitar syndrome?

What is scimitar syndrome?

Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases with poor outcome in infants. It is characterized by anomalous drainage of the right pulmonary veins, lung sequestration with aberrant vascular supply, and lung hypoplasia.

Are patients with Scimitar drainage stenosis asymptomatic?

Three patients with instrumental diagnosis of scimitar drainage stenosis (1 patient in group 1 and 2 patients in group 2) are completely asymptomatic. So far, they have not been treated. At multivariate analysis (logistic regression), no variables were significantly associated with outcomes.

What does a scimitar vein on a CT scan indicate?

The scimitar vein is better appreciated with computed tomography (performed at age 4 days; white arrowheads), which also confirmed right lung hypoplasia and mild compression of the right lower lobe.

Scimitar, or pulmonary venolobar, syndrome is a rare but well-known congenital cardiovascular defect that includes a hypoplastic right pulmonary artery and right lung, which leads to displacement of cardiac structures into the right hemithorax, anomalous systemic arterial supply to the right lung, and a …

Why is it called scimitar syndrome?

The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a scimitar, a type of curved sword.

Can scimitar syndrome be cured?

Less commonly, scimitar syndrome is discovered in adults, many of whom have mild or no symptoms at the time of diagnosis (3). Conventionally the syndrome is corrected surgically by redirecting central venous return toward the left atrium.

Is scimitar syndrome genetic?

Although pathogenic variants in specific genes can clearly cause CHDs, the genetic factors contributing to most cases of scimitar syndrome remain unidentified.

Is scimitar syndrome life threatening?

In its infant form, the scimitar syndrome is diagnosed within the first 2 months after birth, with symptoms of failure to thrive, tachypnea, heart failure and cyanosis. There is an associated mortality of about 45%.

How common is scimitar syndrome?

Scimitar syndrome (SS) is a rare congenital heart malformation occurring in one to three per 100,000 live births with a 2:1 female predominance and accounts for the 3%–6% of partial anomalous pulmonary venous connection (PAPVR) [1,2]. The true incidence may be higher because many patients are asymptomatic.

Is scimitar syndrome fatal?

Results: No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction.

How is scimitar syndrome diagnosed?

Disease in older children and adults commonly presents with recurrent pulmonary infections and/or exertional dyspnea. This group of the patients usually has a benign course. Diagnosis of this syndrome is straightforward in presence of characteristic radiological sign (scimitar sign) on conventional chest radiography.

Is scimitar syndrome rare?

Scimitar syndrome is a rare congenital cardiovascular anomaly in which a part or the entire right lung is drained by pulmonary veins connecting to the vena cava, in combination with hypoplasia of the right lung, pulmonary hypertension and potential other cardiac defects [1–4].

What is scimitar lung syndrome?

Scimitar syndrome, also known as hypogenetic lung syndrome, is characterized by a hypoplastic lung that is drained by an anomalous pulmonary vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.

What is the prevalence of Scimitar syndrome?

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies with incidence of approximately 1–3 per 100,000 live births, consisting of a partial anomalous pulmonary venous connection of the right lung to the IVC, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung.

Is multipatch repair of Scimitar syndrome associated with pulmonary vein obstruction?

A new multipatch technique is described for repair of scimitar syndrome that in the short to intermediate term has not been associated with postoperative pulmonary vein obstruction. Surgical repair of scimitar syndrome using reimplantation or standard baffles is often associated with postoperative pulmonary vein obstruction.

Which CT findings are characteristic of Scimitar syndrome?

The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return. Radiology. 1986;159 (1): 25-6. Radiology (citation) – Pubmed citation

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