Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the italian society of pediatric cardiology.
OBJECTIVE
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.
Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy. Electronic address: vladimiro.vida@unipd.it.
Vladimiro L
VL
Vida
Pediatric Cardiology Unit, Department of Pediatrics, University of Padua, Padua, Italy.
Maddalena
M
Padrini
Department of Statistical Science, University of Padua, Padua, Italy.
Giovanna
G
Boccuzzo
Department of Pediatric Cardiology, Regina Margherita Children's Hospital, Turin, Italy.
Gabriella
G
Agnoletti
Department of Pediatric Cardiology and Cardiac Surgery, Giannina Gaslini Institute, Genova, Italy.
Sara
S
Bondanza
Pediatric Cardiology and GUCH Unit, Policlinico San Donato, Milan, Italy.
Gianfranco
G
Butera
Department of Pediatric Cardiology, Meyer Hospital, Florence, Italy.
Enrico
E
Chiappa
Department of Pediatric Cardiology and Cardiac Surgery, Giannina Gaslini Institute, Genova, Italy.
Maurizio
M
Marasini
Department of Pediatric Cardiology, Bambino Gesù Children's Hospital, Rome, Italy.
Mara
M
Pilati
Department of Pediatric Cardiology, Bambino Gesù Children's Hospital, Rome, Italy.
Giacomo
G
Pongiglione
Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Bologna, Italy.
Daniela
D
Prandstraller
Pediatric Cardiology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy.
Maria Giovanna
MG
Russo
Pediatric Cardiology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy.
Biagio
B
Castaldi
Pediatric Cardiology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy.
Giuseppe
G
Santoro
Department of Pediatric Cardiology, CNR G. Pasquinucci Hospital, Massa, Italy.
Isabella
I
Spadoni
Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy.
Giovanni
G
Stellin
Pediatric Cardiology Unit, Department of Pediatrics, University of Padua, Padua, Italy.
Ornella
O
Milanesi
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007558:Italy; D008297:Male; D012587:Scimitar Syndrome / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
CHD; CHF; Estudio multicéntrico; Evolución natural; Multicenter study; Natural history; PAH; Scimitar syndrome; Síndrome de la cimitarra; congenital heart disease; congestive heart failure; pulmonary arterial hypertension