Respiratory Outcome in Children with Scimitar Syndrome

Chemin, Aude; Bonnet, Damien; Bourgeois, M. Le; Lévy, Marilyne; Delacourt, Christophe

doi:10.1016/j.jpeds.2012.07.066

Cited by 7 publications

(11 citation statements)

References 23 publications

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“…Unfortunately, there were no available data regarding pulmonary function tests. It has been reported that up to 43% of scimitar syndrome patients have wheezing episodes, which were found to be associated with right lung hypoplasia when performing pulmonary function tests 14,15 . As it is unknown whether scimitar vein surgery or pulmonary arterioplasty improves right lung size, it is relevant to differentiate mild symptoms associated with right lung hypoplasia from the more severe symptoms of respiratory distress 1,4 .…”

Section: Discussionmentioning

confidence: 99%

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair

et al. 2021

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Introduction The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair. Methods We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross‐sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair. Results Median follow‐up time was 6 years (Q1–Q3, 2–12), with 96% 5‐year survival. Twenty‐three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%–46%; p = .001) and systolic pulmonary artery pressure (median, 34–29 mmHg; p = .004). Twenty‐three patients underwent scimitar vein repair; 5‐year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5‐year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003). Conclusions APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.

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Section: Discussionmentioning

confidence: 99%

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair

et al. 2021

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“…Generally, no hemodynamic abnormality or clinical symptoms are noted unless 50% or more of the pulmonary blood flow is re-directed to the pulmonary vasculature (5). Thus, individuals with a single anomalous pulmonary vein usually are asymptomatic and have a normal life span.…”

Section: Discussionmentioning

confidence: 99%

Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

et al. 2018

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With the growing use of imaging to aid in both diagnosis and treatment of patients presenting with a myriad of clinical presentations, incidental findings on imaging have become commonplace. Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition that often goes undiagnosed due to lack of symptoms early in life. In adulthood, it can manifest clinically as right-sided heart failure or pulmonary hypertension. We present a case of PAPVC, which, like most cases, was discovered incidentally on chest X-ray.

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“…Adult patients present with fatigue, exertional dyspnea, and recurrent lower respiratory tract infections,[ 5 ] although children with this condition have a higher incidence of respiratory complications. [ 7 ] Diagnosis requires a careful history, physical examination, and appropriate imaging studies. Physical examination may demonstrate a prominent precordial bulge (in patients with large left to right shunt), a systolic murmur or diastolic rumble.…”

Section: Discussionmentioning

confidence: 99%

Breathless at the point of a sword

et al. 2016

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Context:Scimitar syndrome is a congenital anomaly of pulmonary venous return where right pulmonary artery drains into right side other heart, instead of the left side, causing pulmonary hypertension resulting in shortness of breath, recurrent lower respiratory tract infections, chest pain, and fatigue. Early diagnosis and surgical intervention would correct this congenital anomaly reducing morbidity and complications in otherwise healthy young patients.Case Report:We present a case of an 18-year-old female who presented with exertional shortness of breath, fatigue, and recurrent lower respiratory tract infections. She had unremarkable physical examination but chest x-ray showed an abnormal opacity next to right heart border. Computed tomography (CT) chest was performed that showed possible scimitar syndrome. Transesophageal echocardiogram (TEE) and right heart catheterization (RHC) confirmed the diagnosis.Conclusion:Scimitar syndrome is a very rare congenital anomaly of pulmonary venous return. It is usually diagnosed in early childhood but the diagnosis may be delayed until later in adulthood. The consequences are pulmonary hypertension, right-sided heart failure, and frequent pulmonary infections resulting in increased morbidity, mortality, and frequent doctor visits for otherwise healthy young patients.

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Respiratory Outcome in Children with Scimitar Syndrome

Cited by 7 publications

References 23 publications

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair

Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

Breathless at the point of a sword

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