causes, symptoms, diagnosis, treatment, prognosis

The result is therefore that the oxygenated blood returning from the lungs via the scimitar vein, instead of entering the left side of the heart and being reinjected into the systemic circulation via the aorta, ends up in the right side of the heart, which feeds it into the pulmonary circulation, with an obvious decrease in the efficiency of the cardiopulmonary system.

It is usually associated with right lung hypoplasia, bronchial abnormalities and dextrocardia.

It is a type of abnormal partial pulmonary venous return (PAPRV).

Scimitar syndrome owes its curious name to the fact that the abnormal pulmonary vein takes the shape of a scimitar.

Scimitar Syndrome is also referred to by the following names, all of which are synonyms:

  • pulmonary hypogenesis syndrome;
  • Halasz syndrome;
  • right pulmonary artery epibronchial syndrome;
  • congenital pulmonary venolobar syndrome.

Scimitar Syndrome has an estimated prevalence between 1/100,000 and 1/33,333 live births

Women seem to be more affected than men.

Scimitar syndrome is a congenital condition, that is to say, already present at birth.

Symptoms usually begin within the first few months of life.

The exact causes of scimitar syndrome have not yet been fully clarified.

In some patients, an abnormal gene mapped to chromosome 4q12 has been found.

One of the risk factors could be familiarity.

Signs and symptoms

During the neonatal period, scimitar syndrome manifests as congestive heart failure, primarily due to pulmonary hypertension and respiratory distress; the newborn presents with fatigability, dyspnoea, heart failure, lack of appetite, irritability and frequent lung infections.

Diagnosis of scimitar syndrome

Diagnosis is suspected on physical examination and confirmed by transesophageal or transthoracic echocardiography, chest x-ray, angiography, computed tomography, and magnetic resonance angiography.

On objective examination, a systolic murmur of hyperinflux, a diastolic roll of tricuspid stenosis and a duplication of tone II are auscultated.

The characteristic identifiable sign on the chest X-ray, which also gives the disease its name, is a scimitar-shaped lesion (see image below).

Random diagnosis

In rare cases, scimitar syndrome remains relatively paucisymptomatic in newborns and infants, and in this case it may be diagnosed by chance in older children and adults who undergo chest x-rays for other reasons. . In very rare cases, it is never diagnosed in life.

Differential diagnosis

Scimitar Syndrome should be differentiated from:

  • pseudocimitar syndrome (abnormal descending vein draining into the left atrium)
  • Kartagener syndrome.

Prenatal diagnosis

Prenatal diagnosis is possible with echocardiography of the fetus.

Laboratory tests

On the chest X-ray, one can see an enlarged right ventricle, dilated pulmonary artery, pulmonary overflow, scimitar shadow of the abnormal right pulmonary vein along the right side of the heart.

Conditions associated with scimitar syndrome

Variable hypoplasia and malformations of the pulmonary arteries of the right lung and abnormal arterial supply from the aorta, which may originate above or below the diaphragm, have been observed.

The heart is usually moved to the right. In rare cases, the disease is manifested by a small shunt, heart murmur and recurrent respiratory infections in children and adults.

About a quarter of patients have associated congenital heart disease (aortic coarctation, tetralogy of Fallot, ductus arteriosus, ventricular septal defect).

Bronchogenic cysts, horseshoe lung, accessory diaphragm and hernias may be associated.

Treatment

The treatment depends on the hemodynamic state: when the amount of blood circulating in the inferior vena cava is minimal, the treatment may not be applied, but the patient should undergo periodic checks.

In cases of left-to-right shunt and significant pulmonary hypertension, surgical correction should be offered, which may include correction of abnormal venous return, ligation of collateral arteries, and right pneumonectomy.

The surgical treatment is carried out in extracorporeal circulation (CEC).

When to operate?

Surgical treatment is usually performed at preschool age, that is, before the age of 5/6 years.

Mortality and prognosis

When the amount of blood circulating in the inferior vena cava is minimal, life expectancy is comparable to that of a healthy individual.

The quality of life of these patients is high, but some sports may be prohibited, especially competitive ones.

In severe cases and if diagnosed in childhood, the syndrome is associated with significant mortality.

The main causes of death in people with scimitar syndrome are:

  • respiratory arrest;
  • heart failure;
  • severe lung infections.

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