Scimitar Syndrome

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Selen Bayraktaroglu - One of the best experts on this subject based on the ideXlab platform.

  • eponym Scimitar Syndrome
    European Journal of Pediatrics, 2010
    Co-Authors: Levent Midyat, Esen Demir, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The Syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the Syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of Scimitar Syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of Scimitar Syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of Scimitar Syndrome.

  • Scimitar Syndrome associated with partial anomalous pulmonary venous draining into superior vena cava
    European Journal of Pediatrics, 2010
    Co-Authors: Esen Demir, Levent Midyat, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.

Ida Jovanovic - One of the best experts on this subject based on the ideXlab platform.

  • anomalous origin of the left coronary artery from the pulmonary artery Scimitar Syndrome and aortic coarctation
    The Annals of Thoracic Surgery, 2014
    Co-Authors: Slobodan Ilic, Djordje Hercog, Milan Vucicevic, Irena Vulicevic, Branko Mimic, Milan Djukic, Ida Jovanovic
    Abstract:

    Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with Scimitar Syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome.

Esen Demir - One of the best experts on this subject based on the ideXlab platform.

  • eponym Scimitar Syndrome
    European Journal of Pediatrics, 2010
    Co-Authors: Levent Midyat, Esen Demir, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The Syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the Syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of Scimitar Syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of Scimitar Syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of Scimitar Syndrome.

  • Scimitar Syndrome associated with partial anomalous pulmonary venous draining into superior vena cava
    European Journal of Pediatrics, 2010
    Co-Authors: Esen Demir, Levent Midyat, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.

Azmeraw Gissila Aboye - One of the best experts on this subject based on the ideXlab platform.

  • imperforate anus associated with anomalous pulmonary venous return in Scimitar Syndrome case report from a tertiary hospital in ethiopia
    BMC Pediatrics, 2019
    Co-Authors: Tamirat Moges Aklilu, Messele Chanie Adhana, Azmeraw Gissila Aboye
    Abstract:

    Scimitar Syndrome is a rare form of partial anomalous pulmonary venous drainage associated with pulmonary hypertension and congestive heart failure that may lead to death in the newborn infant. Although it is described with anomalies of the lung, heart and their vascular structure, extremely rare association with imperforate anus had been reported. The third case of Scimitar Syndrome and imperforate anus will be reported in this case report. A 3 days old male neonate with imperforate anus presented with abdominal distention. Loop colostomy was done to relieve abdominal distension. The chest x-ray revealed a curved shadow on the right mid lung zone extending to the diaphragm abutting and indenting the inferior vena cava (Scimitar sign). Abdominal ultrasound, transthoracic echocardiography and computerized tomographic angiography confirmed the presence of Scimitar vein and associated dextro-position of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, secundum atrial septal defect with bidirectional shunt, patent ductus arteriosus, pulmonary hypertension, left superior vena cava, and systemic collateral arteries feeding the lower lobe of the right lung. The rare association of Scimitar Syndrome with imperforate anus is discussed. Scimitar Syndrome associated with imperforate anus with and without VACTERL association has been reported previously only in four cases. The knowledge of association between imperforate anus and Scimitar Syndrome helps for early detection and management of cases. It is recommended to have high index of suspicion in every newborn with imperforate anus to check for symptoms of dextro-position of the heart, right lung hypoplasia which may be indicate Scimitar Syndrome.

Levent Midyat - One of the best experts on this subject based on the ideXlab platform.

  • eponym Scimitar Syndrome
    European Journal of Pediatrics, 2010
    Co-Authors: Levent Midyat, Esen Demir, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The Syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the Syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of Scimitar Syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of Scimitar Syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of Scimitar Syndrome.

  • Scimitar Syndrome associated with partial anomalous pulmonary venous draining into superior vena cava
    European Journal of Pediatrics, 2010
    Co-Authors: Esen Demir, Levent Midyat, Memnune Askin, Figen Gulen, Zulal Ulger, Remziye Tanac, Selen Bayraktaroglu
    Abstract:

    Scimitar Syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.