A 4‐yr‐old presenting with chronic cough and asymmetrical chest

Sridhar, Arani; Tofeig, Magdi

doi:10.1183/09031936.03.00002603

Cited by 3 publications

(3 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The infantile form is a severe form of the disease which has a worse prognosis, with major associated cardiac lesions and pulmonary hypertension. The diagnosis of adult form is made incidentally, and indeed, some patients may be completely asymptomatic [24,41] (Tables 1 and 2). …”

mentioning

confidence: 99%

Eponym

et al. 2010

View full text Add to dashboard Cite

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.

show abstract

mentioning

confidence: 99%

Eponym

et al. 2010

View full text Add to dashboard Cite

show abstract

“…One of the challenges facing the diagnosis of scimitar syndrome is that it could be mistaken for asthma, as seen in a case report of a four-year-old girl who presented with a chronic cough. Asthma was the main differential, and she received salbutamol without significant improvement, only to be diagnosed later based on chest X-ray findings [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Bronchial Asthma With Scimitar Syndrome: A Case Report

Jamil,

Alsalahi,

Alamri

et al. 2024

Cureus

View full text Add to dashboard Cite

Scimitar syndrome is a rare congenital cardiopulmonary anomaly; it is also called venolobar syndrome, hypogenic lung syndrome, and Halasz syndrome. The syndrome is characterized by cardiac dextroposition, right lung and pulmonary artery hypoplasia as well as complete or partial anomalous pulmonary venous drainage of the right lung. We report a case of a 22-month-old full-term male child with a severe form of scimitar syndrome diagnosed at birth. The X-ray demonstrated dextrocardia and right lung hypoplasia, while the echocardiography clearly illustrated the scimitar vein. The patient had multiple ER visits and hospitalizations due to asthma exacerbation that was aggravated by recurrent respiratory tract infections; he responded well to asthma medications during his admissions yet compliance to his prophylactic asthma medications was poor at home.

show abstract

“…Those with the infantile type usually have a severe form of the disease with major associated cardiac lesions. The diagnosis of the adult form is generally made incidentally, and some patients may be completely asymptomatic [7,13]. We present here a patient whose venous return of middle and lower lobes of the right lung is into the superior vena cava, which is an extremely rare finding for the disease.…”

Section: Introductionmentioning

confidence: 97%

Scimitar Syndrome associated with partial anomalous pulmonary venous draining into superior vena cava

et al. 2010

View full text Add to dashboard Cite

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.

show abstract

A 4‐yr‐old presenting with chronic cough and asymmetrical chest

Cited by 3 publications

References 6 publications

Eponym

Eponym

Bronchial Asthma With Scimitar Syndrome: A Case Report

Scimitar Syndrome associated with partial anomalous pulmonary venous draining into superior vena cava

Contact Info

Product

Resources

About