Vladimiro L Vida1, Maddalena Padrini2, Giovanna Boccuzzo3, Gabriella Agnoletti4, Sara Bondanza5, Gianfranco Butera6, Enrico Chiappa7, Maurizio Marasini5, Mara Pilati8, Giacomo Pongiglione8, Daniela Prandstraller9, Maria Giovanna Russo10, Biagio Castaldi10, Giuseppe Santoro10, Isabella Spadoni11, Giovanni Stellin12, Ornella Milanesi2. 1. Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy. Electronic address: vladimiro.vida@unipd.it. 2. Pediatric Cardiology Unit, Department of Pediatrics, University of Padua, Padua, Italy. 3. Department of Statistical Science, University of Padua, Padua, Italy. 4. Department of Pediatric Cardiology, Regina Margherita Children's Hospital, Turin, Italy. 5. Department of Pediatric Cardiology and Cardiac Surgery, Giannina Gaslini Institute, Genova, Italy. 6. Pediatric Cardiology and GUCH Unit, Policlinico San Donato, Milan, Italy. 7. Department of Pediatric Cardiology, Meyer Hospital, Florence, Italy. 8. Department of Pediatric Cardiology, Bambino Gesù Children's Hospital, Rome, Italy. 9. Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Bologna, Italy. 10. Pediatric Cardiology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy. 11. Department of Pediatric Cardiology, CNR G. Pasquinucci Hospital, Massa, Italy. 12. Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy.
Abstract
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.
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.
Authors: Kongkiat Chaikriangkrai; Juan C Lopez-Mattei; Jose Flores-Arredondo; Huie Lin; Michael Reardon; Su Min Chang; Dipan Shah Journal: Methodist Debakey Cardiovasc J Date: 2014 Oct-Dec