Scimitar syndrome
Scimitar syndrome is a very rare congenital heart disease. It is also referred to as phylogenetic lung syndrome, pulmonary venolobar, mirror-image lung syndrome, vena cava bronchovascular syndrome, Halasz’s syndrome and, epibronchial right pulmonary artery syndrome .This disease is characterized by anomalous right pulmonary venous return into the inferior vena cava.
Normally, the pulmonary vein carries oxygenated blood to the left chamber of the heart that is then pumped to the systemic circulation. However in this case, there is shunting of blood back to the inferior vena cava through an anomalous vein. This vein connects the pulmonary venous circulation and the systemic circulation. This vein assumes the shape of a scimitar, hence its name. This is visible under the X-ray. These cases are rare. They are approximated to at least 2 in 100,000 births. Scimitar syndrome is also more prevalent in females than in males. The probability of a female having this disorder is twice as much as in males.
It is characterized by, abnormal pulmonary drainage, hyperplastic right lung and a small or absent pulmonary artery, accessory diaphragm, bronchopulmonary sequestration, absence of the inferior vena cava and the displacement of some cardiac features. This condition affects not only the heart but also the lungs; especially the right lung. Several abnormalities, congenital thoracic defects are associated with the scimitar syndrome. Some of these abnormalities include lung hypoplasia, atrial septal defect, Swyer-James syndrome, ventricular septal defect, abnormal relationship of the pulmonary arteries and bronchi, coarctation of the aorta, abnormalities of the aortic arch, and
Some people who suffer from this condition are totally unaware of it. This is because its severity varies. To some it is a because of massive health issues, but others come to learn about it either by chance or through diagnosis. To some it causes major complications, respiratory insufficiency and heart failure, especially in infants whereas others are able to live with Scimitar syndrome for many years without complications. For this reason, some may need to undergo surgical repair whereas others do not. However, majority of the people affected are asymptomatic or only bear mild symptoms. Adults and older children are mostly diagnosed with it during chest radiography for different reasons.
Some of the common symptoms of these diseases are recurrent respiratory diseases, heart murmur, rapid breathing, and shortness of breath, chest pain, cyanosis and the retraction of the ribcage muscle. When one is suffering from this disease, they produce an abnormal chest radiograph. When conducting a check up on a patient who has recurrent respiratory illnesses, one of the pointers to this condition is reduced lung sounds on the right lung. This is because one of the effects of its characteristics is a hypoplastic right lung. Cyanosis is a blue or purple coloration of the skin or tissues that are close to the skin due to lack of sufficient blood supply. In this case, it is caused by the malformation and malfunctioning of some features of the heart and those of the lungs. A heart murmurs on the other hand is an unusual sound produced when the heart beats. There are two types of a heart murmur. One is normal and harmless and bound to occur once in a while in a person’s lifetime. The other is the abnormal heart murmur. This one should because alarm. It may be a symptom of this condition or any other type of heart problems. It calls for reduced intense body activity.
In the case where the patient actually has this condition, the chest radiograph is sure to shoe a small right lung as compared to the left one and an anomalous pulmonary vein. This vein is on the right side. A computed tomography is also bound to reflect loss of volume of the right lung parenchyma, shifting of the heart to the right and dextroposition of the heart. In children, it may lead to slower growth or failure to thrive. It will be found that such children will be found to weigh less or be shorter than they are expected at their age and when other factors are considered. Those who exhibit more severe symptoms are more likely to have more anomalies. At the same time, those who have more anomalies are more likely to have a worse prognosis. The age at which one is diagnosed with the scimitar syndrome is also very important in indicating the most probable outcome in a patient.
The cause of this condition is not known. Patients are majorly born with it. This condition is hereditary. For this reason, a child who has been born into a family where another person has it or where there is a family history of scimitar syndrome is at a higher risk of having it.
Patients suspected to be suffering from this condition should have cardiac catheterization to confirm these suspicions, monitor the course of the anomalous pulmonary venous drainage, assess the degree of shunting and monitor all the other cardiac defects that come with the condition. To detect other anomalies, ultrasonography and echocardiography may be performed. These details tell whether a corrective procedure is at all needed. Ventilation scans are also necessary for following up the condition. Magnetic resonance imaging produces clear images during check up with details that are sufficient to tell the extent of severity and number of anomalies within the heart and lungs. However, it is expensive and not recommended for routine checkups.
The scimitar syndrome is more likely to be fatal in infants as compared to their adult counterparts. Medical treatment in infants is recommended before surgery. This is to allow for growth of the features and reduce the risk of this operation. However, in a case where there is pulmonary hypertension or the body does not respond to the medical treatment, it is crucial to intervene using the surgical repair. At the time of angiography, the abnormal artery should be embolized. The reason for this is that it is likely to improve the deteriorating case of the patient. It is also safe and therefore unlikely to cause any negative effects of the patient.
Treatment of this disease is majorly carried out by surgery. There is created a baffle to redirect the anomalous return to the left atrium. The process of surgery may differ according to the opinion of the doctor. The surgical repair hardly results to normal flow of blood to the right lung. However, it stops the shunt from left to right. In infants, however, there is prevalent pulmonary venous obstruction after operation. The truth is that not all cases require surgery, especially in adults. Some are able to live on normally without it. This is mostly where the patient is asymptomatic.
References
Copel, J. (2012). Obstetric Imaging. New York: Elsevier Health Sciences.
Vida, V. (2012). The Scimitar Syndrome. New York: libreriauniversitaria.
