Scimitar syndrome presenting in infancy

Huddleston, Charles B.; Exil, Vernat; Canter, Charles E.; Mendeloff, Eric N.

doi:10.1016/s0003-4975(98)01227-2

Cited by 115 publications

(124 citation statements)

References 13 publications

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“…Özellikle konjestif kalp yetersizliği olan infantlarda aortopulmoner besleme arterinin kateterizasyon yöntemiyle tıkanması öne-rilen en iyi ve en basit tedavi şekli olup sıklıkla semptomların ve pulmoner hipertansiyonun gerilemesine katkıda bulunur. [1,2,18,19] Özellikle ilave kardiyak anomalisi olan olgularda aortopulmoner besleme arterinin tıkanması cerrahi girişimin geciktirilerek daha uygun şartlarda yapılmasına yardımcı olabilir. [1,2] Bizim çalış-mamamızda da infantil SS'li hastaların üçünde farklı cihazlar ile aortopulmoner besleme arterinde tıkanma sağlandı.…”

Section: Discussionunclassified

Scimitar syndrome: different clinical presentations and results

Demir¹

2014

Turk Gogus Kalp Dama

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Amaç: Bu çalışmada Scimitar sendromu tanısı konulmuş hastalar değerlendirildi.Ça lış mapla nı:Çalışmaya Ocak 2003 -Aralık 2011 tarihleri arasın-da kliniğimizde Scimitar sendromu tanısı konulmuş ortalama ağır-lıkları 16.7±21.8 kg olan, 12 hasta (8 kız, 4 erkek; ort. yaş 4.0±4.8 yıl; dağılım 1 ay -16 yıl), alındı. Hastaların klinik bulguları, fizik muayene, telekardiyografi, ekokardiyografi, kalp kateterizasyonu ve cerahi bulguları ve izlem verileri geriye dönük olarak gözden geçirildi. Bul gu lar:On iki hastadan beşi (%42) infantil tip ve yedisi (%58) çocuk/erişkin tip Scimitar sendromu olarak değerlendirildi. İnfantil tipteki hastaların tümünde taşipne ve kalp yetersizliği bulguları varken, çocuk/erişkin tipteki hastaların birinde tekrarlayan alt solunum yolu enfeksiyonu öyküsü vardı. Ek kardiyak defektler hastaların yedisinde atriyal septal defekt, birinde aort koarktasyonu, ikisinde patent duktus arteriyozus ve birinde ventriküler septal defekt ile pulmoner atrezi idi. Bir hastada iki taraflı Scimitar sendromu vardı. İnfantil Scimitar sendromlu hastaların tümü şiddetli pulmoner hipertansiyonlu iken, çocuk/erişkin tipteki hastalardan biri hafif pulmoner hipertansiyonlu idi. Aortopulmoner kollateral arter saptanan dokuz hastadan altısında arter transkateter yolla kapatıldı. On hastaya (%83) cerrahi girişim uygulandı. İnfantil Scimitar sendromlu bir hasta ameliyat sonrası erken dönemde, ameliyat edilmeyen iki taraflı Scimitar sendromlu diğer bir hasta ise takipte akciğer enfeksiyonu nedeniyle kaybedildi.So nuç: Scimitar sendromu farklı klinik tablolarla seyredebilen nadir bir sendromdur. Çocuk/erişkin tip hastalığı olan hastalar sıklıkla asemptomatiktir; ancak tekrarlayan akciğer enfeksiyonları da izlenebilir. Aortopulmoner kollateral oklüz-yon semptomatik iyileşme sağlayabilse de özellikle Scimitar veni tıkanmış ve ilave kardiyak anomalisi bulunan hastalarda cerrahi gerekir.Anah tar söz cük ler: Ekokardiyografi; pulmoner hipertansiyon; Scimitar sendromu.Background: This study aims to evaluate patients diagnosed with Scimitar syndrome.

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Section: Discussionunclassified

Scimitar syndrome: different clinical presentations and results

Demir¹

2014

Turk Gogus Kalp Dama

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“…Not all patients with scimitar syndrome have all the above abnormalities, but the defining trait is the anomalous pulmonary venous return of all or most of the right lung to the IVC [1].…”

Section: Discussionmentioning

confidence: 99%

“…The infantile form is a severe form of the disease with major associated cardiac lesions, including atrial-septal defect, ventricular-septal defect, coarctation of aorta, patent ductus arteriosus, and hypoplastic right lung. Pulmonary hypertension is quite common in infants presenting with scimitar syndrome [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…In children with the infantile form of Scimitar syndrome, although repair of the anomalous pulmonary venous return and ligation of collaterals is recommended, right pneumonectomy, either as a primary therapy or if repair fails, have shown similar early and late results [1]. In the adult form of Scimitar syndrome repair is indicated if the shunt is large (pulmonary blood flow/systemic blood flow ratio w2:1) [3,4].…”

Section: Discussionmentioning

confidence: 99%

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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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“…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%