“Infantile” form of the scimitar syndrome with pulmonary hypertension

Dupuis, C; Charaf, Louis A.C.; Breviere, G. M.; Abou, Pierre

doi:10.1016/0002-9149(93)90549-r

Cited by 140 publications

(160 citation statements)

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“…Scimitar syndrome has wide spectrum of clinical presentation and can be divided into an infantile form and a paediatric/young adult form [2]. The infantile form generally presents within the first 2 months of life with tachypnoea, failure to thrive and signs of heart failure.…”

Section: Discussionmentioning

confidence: 99%

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

Sridhar

Tofeig²

2003

Eur Respir J

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A 4-yr-old female was referred by her general practitioner with history of recurrent cough of 18 months duration. The cough was predominantly nocturnal with no associated wheeze, fever or systemic symptoms and no symptoms of gastrooesophageal reflux. There were no exercise-induced symptoms. Asthma was suspected and she had been treated with inhaled salbutamol with no significant improvement in her symptoms. There was no history of recurrent chest infections, foreign body aspiration or previous hospital admissions.On examination her weight was on the 3rd centile and height on the 50th centile. There were no cyanosis, clubbing or ear, nose and throat abnormalities. She had a markedly asymmetrical chest with less prominent right hemithorax compared with the left and mediastinal shift to the right. Breath sounds were diminished on the right side with no wheeze or crepitations. The rest of the cardiovascular examination was normal. There were no other abnormalities on clinical examination.In view of her chronic cough and failure to thrive she had a mantoux test, which was strongly positive. She had bacille Calmette-Guerin vaccination at birth and there was no recent contact with tuberculosis (TB). The sputum was negative for Mycobacterium tuberculosis. She was given TB chemoprophylaxis with Isoniazid and Rifampicin for 6 months, during which she had a symptomatic improvement in her cough.A chest radiograph ( fig. 1) and computed tomography (CT) scan of the thorax ( fig. 2) revealed characteristic findings.Bronchoscopy revealed abnormalities of the bronchial tree. She had a transthoracic echocardiogram, which revealed characteristic findings. The rest of the cardiac anatomy was normal. She underwent diagnostic cardiac catheterisation, which showed normal right-sided pressures (right atrial mean pressure 4 mmHg, right ventricle (RV) pressure 26 over 3 mmHg, main pulmonary artery (MPA) 24 over 8 mmHg, the right pulmonary artery (RPA) 20 over 8 mmHg and left pulmonary artery (LPA) 20 over 8 mmHg).Oximetry showed pulmonary blood flow/systemic blood flow ratio of 1.3:1. The superior vena cava saturation was 83%, right atrial saturation 88%, lower inferior vena cava (IVC) saturation 83%, right atrium (RA)-IVC junction 99%, RV 91%, MPA 90%, RPA 90% and LPA 86%.The follow through of the RPA angiogram ( fig. 3) revealed the characteristic abnormality. There were no aortic collaterals supplying the lungs. There was no significant intracardiac leftto-right shunt.Currently she is being managed conservatively with regular clinical and echocardiograph assessment.

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Section: Discussionmentioning

confidence: 99%

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

Sridhar

Tofeig²

2003

Eur Respir J

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“…In these patients impressive symptoms are rarely found, and prognosis is generally good. 3 On the other hand, the variant encountered in infancy is associated with marked pulmonary hypertension 4 and is usually associated with severe symptoms of respiratory distress, heart failure, or even cyanosis. In combination with other cardiovascular malformations, the prognosis is poor even when surgical or interventional therapy is undertaken.…”

Section: Discussionmentioning

confidence: 99%

“…3 It has been hypothesized that the abnormal systemic flow to the sequestered area of the right lower lung, which is typically found in the infantile form of the syndrome, contributed significantly to the severity of symptoms and to pulmonary hypertension. 4 ' 6 Histologic examination of the pulmonary vascular structures in infants with scimitar syndrome revealed an abnormally increased muscularity of pre-and intraacinar pulmonary arteries in both lungs. decreasing pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

“…The contribution of surgical ligation of the duct is difficult to assess. Based on the experience of others, 4 it might well have been of minor importance. The favorable outcome shows that early interruption of systemic flow through pulmonary sequestrations is a promising therapeutic option to decrease the shunt volume and ameliorate pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung

1997

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Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.

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“…However, infants often have postoperative pulmonary venous obstruction. There have been variations in survival reports in the literature with mortality rates ranging from 16 to 64% (Dupuis, Charaf, Breviere, & Abou, 1993;Huddleston, Exil, Canter, & Mendeloff, 1999;.…”

Section: Anomalous Pulmonary Veinsmentioning

confidence: 99%