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Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

IntroductionPatients with complex health care needs (PCHCN) are individuals who require numerous, costly care services and have been shown to place a heavy burden on health care resources. It has been argued that an important issue in providing value-based primary care concerns how to identify groups of patients with similar needs (who pose similar challenges) so that care teams and care delivery processes can be tailored to each patient subgroup. Our study aims to describe the most common chronic conditions and their combinations in a cohort of elderly PCHCN.MethodsWe focused on a cohort of PCHCN residing in an area served by a local public health unit (the “Azienda ULSS4-Veneto”) and belonging to Resource Utilization Bands 4 and 5 according to the ACG System. For each patient we extracted Expanded Diagnosis Clusters, and combined them with information available from Rx-MGs diagnoses. For the present work we focused on 15 diseases/disorders, analyzing their combinations as dyads and triads. Latent class analysis was used to elucidate the patterns of the morbidities considered in the PCHCN.ResultsFive disease clusters were identified: one concerned metabolic-ischemic heart diseases; one was labelled as neurological and mental disorders; one mainly comprised cardiac diseases such as congestive heart failure and atrial fibrillation; one was largely associated with respiratory conditions; and one involved neoplasms.ConclusionsOur study showed specific common associations between certain chronic diseases, shedding light on the patterns of multimorbidity often seen in PCHCN. Studying these patterns in more depth may help to better organize the intervention needed to deal with these patients.