TY - JOUR
T1 - Natural history and clinical outcome of "uncorrected" scimitar syndrome patients
T2 - a multicenter study of the italian society of pediatric cardiology
AU - Vida, Vladimiro L.
AU - Padrini, Maddalena
AU - Boccuzzo, Giovanna
AU - Agnoletti, Gabriella
AU - Bondanza, Sara
AU - Butera, Gianfranco
AU - Chiappa, Enrico
AU - Marasini, Maurizio
AU - Pilati, Mara
AU - Pongiglione, Giacomo
AU - Prandstraller, Daniela
AU - Russo, Maria G iovanna
AU - Castaldi, Biagio
AU - Santoro, Giuseppe
AU - Spadoni, Isabella
AU - Stellin, Giovanni
AU - Milanesi, Ornella
PY - 2013/7/1
Y1 - 2013/7/1
N2 - 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.
AB - 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.
KW - CHD
KW - CHF
KW - congenital heart disease
KW - congestive heart failure
KW - Estudio multicéntrico
KW - Evolución natural
KW - Multicenter study
KW - Natural history
KW - PAH
KW - pulmonary arterial hypertension
KW - Scimitar syndrome
KW - Síndrome de la cimitarra
UR - http://www.scopus.com/inward/record.url?scp=84936769889&partnerID=8YFLogxK
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U2 - 10.1016/j.rec.2013.03.008
DO - 10.1016/j.rec.2013.03.008
M3 - Article
C2 - 24776205
VL - 66
SP - 556
EP - 560
JO - Revista Espanola de Cardiologia
JF - Revista Espanola de Cardiologia
SN - 0300-8932
IS - 7
ER -