Natural History and Clinical Outcome of ¡°Uncorrected¡± Scimitar Syndrome Patients: a Multicenter Study of the Italian Society of Pediatric Cardiology

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• 作者：Vladimiro L. Vida ; Maddalena Padrini ; Giovanna Boccuzzo ; Gabriella Agnoletti ; Sara Bondanza ; Gianfranco Butera ; Enrico Chiappa ; Maurizio Marasini ; Mara Pilati ; Giacomo Pongiglione ; Daniela Pr ; straller ; Maria Giovanna Russo ; Biagio Castaldi ; Giuseppe Santoro ; Isabella Spadoni ; Giovanni Stellin ; Ornella Milanesi ; on behalf of the Italian Society of Pediatric Cardiology
• 关键词：CHD ; congenital heart disease ; CHF ; congestive heart failure ; PAH ; pulmonary arterial hypertension
• 刊名：Revista Española de Cardiología (English Edition)
• 出版年：2013
• 出版时间：July, 2013
• 年：2013
• 卷：66
• 期：7
• 页码：556-560
• 全文大小：241 K

文摘

Introduction and objectives

To analyze the clinical status of patients with ¡°uncorrected¡± scimitar syndrome in a multicenter Italian study.

Methods

The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers).

Results

The median age at diagnosis was 1.05 years (range, 1 day-41 years). Thirty-three patients (75 % ) had an isolated form; 11 patients (25 % ) had associated congenital heart diseases. Twenty-two patients (50 % ) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4 % ] vs 2 of 33 [6.1 % ]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6 % ] vs 2 of 33 [6.1 % ]; P=.027) than patients with isolated forms. Ten patients (22.7 % ) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8 % ) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease.

Conclusions

In most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming ¡°associated¡± into ¡°isolated¡± forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms.