Nonreferral Centers Research Articles

Temporal implementation of a regional referral pathway in transthyretin cardiac amyloidosis: Emilia-Romagna experience.

Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis. We aimed to examine the development of a regional network for the diagnosis and management of ATTR-CA and describe a cohort of patients with ATTR-CA, investigate diagnostic pathways and assess clinical outcomes according to diagnosis periods. We performed a survey study analyzing answers from 11 cardiology centers and we conducted a retrospective study including patients with ATTR-CA attending a referral center between 1 January 2012 and 31 December 2022, and categorized by the period of diagnosis (2012-2016 and 2017-2022). Over the years, a growing number of patients reached a diagnosis and were treated in the surveyed nonreferral centers of the region. The retrospective study showed a more significant diagnostic delay in the earlier period rather than the later one [13.4 (5-30.2) vs. 10.6 (5.0-17.9) months, P = 0.04]. Patients diagnosed after 2017 showed a greater survival rate than those diagnosed earlier ( P = 0.02). In the multivariate analysis, the year of diagnosis from 2017 remained independently associated with mortality [hazard ratio (HR) 0.46, 95% confidence interval (CI) 0.28-0.79; P = 0.005]. This study emphasized the shift toward noninvasive diagnostic criteria. It revealed a positive impact on patient survival and disease management with the use of disease-modifying therapies and diagnostic developments in more recent years. The findings underscore the importance of disease awareness and networking to reduce diagnostic delays and enhance patient journeys for ATTR-CA.

The specialized pediatric palliative care service in Italy: how is it working? Results of the nationwide PalliPed study

BackgroundAccurate estimation of the specialized pediatric palliative care (PPC) burden and the definition of the extent and quality of PPC service in Italy represent urgent needs to enable the proper allocation of PPC resources and the definition of prevention and educational plans. The PalliPed project aimed to provide the first comprehensive assessment of the characteristics of Italian patients requiring PPC, the quality and extent of regional PPC networks/facilities, and the number of dedicated resources. In this paper, we present the results of the second part of the project, regarding the implementation and quality of PPC services in Italy.MethodsThe PalliPed study had an observational cross-sectional design. All Italian specialized PPC centers/facilities were invited to participate in the project and complete a survey on the characteristics of PPC centers/facilities in different care settings, reporting data as of 24 October 2022. Data were collected online.Results19 PPC specialized centers/facilities from 12 Italian regions and two autonomous provinces responded to the survey. Among them, 11 are regional referral centers. Seven Italian regions out of 20 reported no PPC centers/facilities, mainly in central-southern Italy. Less than half (45%) of the regional referral centers cover the entire regional territory, and three offer 24/7 service. Ten centers have a dedicated team. Half of the eight non-referral centers offer 24/7 service and have a dedicated team. A total of 1,092 patients were reported by 18 centers as of 24 October 2022. Over the years, an increasing number of patients has been reported, rising from 1,202 (2019) to 1,544 (2021). The dedicated staff is inadequate, and most healthcare providers are not recognized at an institutional level. A shortage of ‘young’ staff and a lack of specific training was reported, particularly among nurses (77% had no training in PPC).ConclusionsThe results obtained show how training, information, and research interventions are still necessary for the reorganization of the available resources and definition of proper strategies to respond dynamically to the new emerging needs of these populations. At the same time, our study represents a first step in defining a national registry of PPC models, useful for monitoring evolutions, and critical issues and planning any new or corrective strategy.

Imaging of paraduodenal pancreatitis: A systematic review.

Paraduodenal pancreatitis (PP) represents a diagnostic challenge, especially in non-referral centers, given its potential imaging overlap with pancreatic cancer. There are two main histological variants of PP, the cystic and the solid, with slightly different imaging appearances. Moreover, imaging findings in PP may change over time because of disease progression and/or as an effect of its risk factors exposition, namely alcohol intake and smoking. To describe multimodality imaging findings in patients affected by PP to help clinicians in the differential diagnosis with pancreatic cancer. The systematic review was conducted according to the Preferred Reporting Items for Systematic reviews and Meta-analyses 2009 guidelines. A Literature search was performed on PubMed, Embase and Cochrane Library using (groove pancreatitis [Title/Abstract]) OR (PP [Title/Abstract]) as key words. A total of 593 articles were considered for inclusion. After eliminating duplicates, and title and abstract screening, 53 full-text articles were assessed for eligibility. Eligibility criteria were: Original studies including 8 or more patients, fully written in English, describing imaging findings in PP, with pathological confirmation or clinical-radiological follow-up as the gold standard. Finally, 14 studies were included in our systematic review. Computed tomography (CT) findings were described in 292 patients, magnetic resonance imaging (MRI) findings in 231 and endoscopic ultrasound (EUS) findings in 115. Duodenal wall thickening was observed in 88.8% of the cases: Detection rate was 96.5% at EUS, 91.0% at MRI and 84.1% at CT. Second duodenal portion increased enhancement was recognizable in 76.3% of the cases: Detection rate was 84.4% at MRI and 72.1% at CT. Cysts within the duodenal wall were detected in 82.6% of the cases: Detection rate was 94.4% at EUS, 81.9% at MRI and 75.7% at CT. A solid mass in the groove region was described in 40.9% of the cases; in 78.3% of the cases, it showed patchy enhancement in the portal venous phase, and in 100% appeared iso/hyperintense during delayed phase imaging. Only 3.6% of the lesions showed restricted diffusion. The prevalence of radiological signs of chronic obstructive pancreatitis, namely main pancreatic duct dilatation, pancreatic calcifications, and pancreatic cysts, was extremely variable in the different articles. PP has peculiar imaging findings. MRI is the best radiological imaging modality for diagnosing PP, but EUS is more accurate than MRI in depicting duodenal wall alterations.

Misdiagnosis of Thoracic Aortic Emergencies Occurs Frequently Among Transfers to Aortic Referral Centers: An Analysis of Over 3700 Patients.

BackgroundAcute aortic syndromes may be prone to misdiagnosis by nonreferral aortic centers with less diagnostic experience. We evaluated regional variability in these misdiagnosis trends among patients transferred to different regional quaternary care centers with presumed acute aortic syndromes.Methods and ResultsTwo institutional aortic center databases were retrospectively reviewed for emergency transfers in patients diagnosed with acute aortic dissection, intramural hematoma, penetrating aortic ulcer, thoracic aortic aneurysm, or aortic pseudoaneurysm between 2008 and 2020. Transferring diagnoses versus actual diagnoses were reviewed using physician notes and radiology reports. Misdiagnoses were confirmed by a board‐certified cardiothoracic surgeon. A total of 3772 inpatient transfers were identified, of which 1762 patients were classified as emergency transfers. The mean age was 64 years (58% male). Patients were transferred from 203 medical centers by ground (51%) or air (49%). Differences in transfer diagnosis and actual diagnosis were identified in 188 (10.7%) patients. Of those, incorrect classification of Type A versus B dissections was identified among 23%, and 30% of patients with a referring diagnosis of an acute aortic dissection did not have one. In addition, 14% transferred for contained/impending rupture did not have signs of rupture. All misdiagnoses were secondary to misinterpretation of imaging, with motion artifacts (n=32, 17%) and postsurgical changes (n=44, 23%) being common sources of diagnostic error.ConclusionsMisdiagnosis of acute aortic syndromes commonly occurred in patients transferred to 2 separate large aortic referral centers. Although diagnostic accuracy may be improving, there are opportunities for improved physician awareness through standardized web‐based imaging education.

Influence of the health-care pathway on the outcome of patients with infective endocarditis: Should all patients be treated in referral centers?

Mortality in infective endocarditis (IE) remains high. Few studies have compared the prognosis and management of patients with IE according to their health-care pathway (HCP) To determine the prognosis of patients treated for IE according to their HCP. To assess how the ESC guidelines are implemented concerning the performance of transesophageal echocardiography, the use of antibiotic therapy, and the performance of valve surgery; and to compare the epidemiological profile of IE according to the type of centers in which patients are hospitalized. In a prospective multicentric study including 22 hospitals in the South-East of France, patients with a diagnosis of IE were classified in 3 groups according to their HCP: patients diagnosed and entirely managed in a reference center or hospital with cardiac surgery (group 1), patients initially taken care in a non-referral center, then referred in a center including cardiac surgery (group 2) and patients totally managed in non-referral centers (group 3). Primary endpoint was death from all cause at one-year follow-up. Between 2014 and 2017, 342 patients were consecutively included: 119 in group 1, 111 in group 2 and 112 in group 3. One-year mortality was 26% (88 deaths) and was not significantly different between groups 1 and 2 (20% vs. 21%, P = 0.83). Patients in group 3 had a higher mortality (37%) compared to patients in groups 1 and 2 ( P < 0.001). Belonging to group 3 was a predicitive factor of one-year mortality in multivariate analysis (HR 2.56; IC95% 1.44–4.55, P = 0.001). ESC guidelines were not implemented similarly depending on the HCP ( P = 0.04). Patients in group 3 were significantly older ( P < 0.001) and had more comorbidities ( P < 0.001) than patients treated in referral centers ( Fig. 1 ). Prognosis of patients with IE is influenced by their HCP. Patients treated in non-referral centers have a worse prognosis than patients treated in referral or surgical centers.

Gaps in Evidence for Risk Stratification for Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

Gaps in Evidence for Risk Stratification for Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

Iranian Urology Association Coronavirus Disease 2019 (COVID-19) Taskforce Pamphlet (IUA-CTP) Recommended Practice based on National Epidemiologic Analysis.

Since the emergence of Covid19 epidemics different guidelines and protocols have been published by Urology associations. Most of these recommendations have focused on the aptitude of any disease or condition for postponement. With the evolution of the outbreak, it is clear that postponement of procedures is not the policy we can rely on exclusively. We must know where do we stand? Where are we going in our country? How useful our recommendations have been for urology practitioners? We try to draw a clearer although-to some extent- conjectural picture and to adjust our protocols to this picture of outbreak evolution. Assuming that anything in this predicament is subject to unexpected changes. For these goals, we raise these arguments in three sections. First, where do we stand and where are we going? Explaining the present situation and best available statistics of the disease, the velocity the disease is spreading and our approximate predicted date its subsidence or partial remission. In a web form survey, we tried to evaluate that in the absence of a clear picture of outbreak progress in a specific area, how useful experts' points of view will be for the urologists working in non-referral centers especially in relevance to equivocal and challenging cases. Will there be any significant difference at all? In the third section, we try to give the plot to guide scheduling or postponing procedures in any given are according to the level of involvement. Here we considered both the characteristics of the special urology condition and also the situation and progress of the outbreak in that area.

Noninvasive Prediction of Elevated Wedge Pressure in Pulmonary Hypertension Patients Without Clear Signs of Left-Sided Heart Disease: External Validation of the OPTICS Risk Score.

BackgroundAlthough most newly presenting patients with pulmonary hypertension (PH) have elevated pulmonary artery wedge pressure, identification of so‐called postcapillary PH can be challenging. A noninvasive tool predicting elevated pulmonary artery wedge pressure in patients with incident PH may help avoid unnecessary invasive diagnostic procedures.Methods and ResultsA combination of clinical data, ECG, and echocardiographic parameters was used to refine a previously developed left heart failure risk score in a retrospective cohort of pre‐ and postcapillary PH patients. This updated score (renamed the OPTICS risk score) was externally validated in a prospective cohort of patients from 12 Dutch nonreferral centers the OPTICS network. Using the updated OPTICS risk score, the presence of postcapillary PH could be predicted on the basis of body mass index ≥30, diabetes mellitus, atrial fibrillation, dyslipidemia, history of valvular surgery, sum of SV1 (deflection in V1 in millimeters) and RV6 (deflection in V6 in millimeters) on ECG, and left atrial dilation. The external validation cohort included 81 postcapillary PH patients and 66 precapillary PH patients. Using a predefined cutoff of >104, the OPTICS score had 100% specificity for postcapillary PH (sensitivity, 22%). In addition, we investigated whether a high probability of heart failure with preserved ejection fraction, assessed by the H2 FPEF score (obesity, atrial fibrillation, age >60 yrs, ≥2 antihypertensives, E/e' >9, and pulmonary artery systolic pressure by echo >35 mmHg), similarly predicted the presence of elevated pulmonary artery wedge pressure. High probability of heart failure with preserved ejection fraction (H2 FPEF score ≥6) was less specific for postcapillary PH.ConclusionsIn a community setting, the OPTICS risk score can predict elevated pulmonary artery wedge pressure in PH patients without clear signs of left‐sided heart disease. The OPTICS risk score may be used to tailor the decision to perform invasive diagnostic testing.

Surgical repair of an isolated left-sided partial anomalous pulmonary venous connection in an 18-month-old child.

The isolated left-sided partial anomalous pulmonary venous connection is a rare congenital anomaly. Only 3% of cases have been reported with drainage from the whole left lung into the left innominate vein. This anomaly is often asymptomatic and may go undetected (particularly in non-referral centers) until adulthood when symptoms begin to manifest. Transthoracic echocardiography is the main diagnostic tool; however, computed tomography angiography, and magnetic resonance imaging are widely used nowadays. Herein, we present the case of an 18-month-old girl with the diagnosis of isolated left-sided partial anomalous pulmonary venous connection as the left pulmonary veins were draining into the left innominate vein via a vertical vein. The patient underwent surgical repair by anastomosing the vertical vein to the left atrial appendage using cardiopulmonary bypass and cardiac arrest. The patient was discharged on the fifth day without complications. The precise follow-up for 12months showed improved clinical symptoms, and the successively performed TTE confirmed the excellent result of the operation.

Current trends in patient enrollment for robotic‐assisted laparoscopic prostatectomy in Belgium

During the last decade, an inverse stage migration has been observed in radical prostatectomy series at tertiary centers. However, it remains unclear whether similar trends can also be observed in solely robotic practices, including nonreferral centers. The aim of this study was to investigate the clinical and pathological trends in robotic-assisted laparoscopic prostatectomy (RALP) enrollment in Belgium over a period of 6 years through an analysis of a prospective registry. A prospective, multicenter database was constructed: consecutive patients undergoing RALP in Belgium from 2010 to 2015 were enrolled, and 7366 men were analyzed. Variations in clinical and pathological variables were explored as a function of the enrollment year with proportional odds for categorical variables and with linear regressions for continuous variables. Net increases were observed in the prostate-specific antigen levels, cT stage, and biopsy Gleason scores across the study years (P < .001). The rate of low-risk prostate cancer (PCa) decreased from 36% in 2010 to 21% in 2015, whereas the rate of intermediate-risk PCa rose from 47% to 58%, and the rate of high-risk PCa rose from 17% to 21%. In parallel, the pT2 stage rate decreased from 76% to 64%, and the rate of Gleason 6 (3 + 3) cases was reduced from 45% to 23% (P < .001). Conversely, the pT3a stage rate rose from 16% to 24%, the pT3b stage rate rose from 7% to 11%, and the rate of Gleason 7 (4 + 3) cases rose from 7% to 21% (P < .0001). Finally, more patients underwent node dissection, and positive lymph nodes were increasingly diagnosed (from 3% in 2010 to 7% in 2015). During the last 6 years of RALP implementation in Belgium, there was a significant increase in the enrollment of intermediate- and high-risk PCa patients. This yielded a significant increase in adverse pathological characteristics. These results suggest a paradigm shift in PCa treatment, with radical robotic surgery increasing for intermediate- and high-risk patients. Cancer 2017;123:4139-4146. © 2017 American Cancer Society.

Comparison of Safety, Efficacy, and Long-term Outcomes of Radiofrequency Ablation (RFA) for Treatment of Dysplastic Barrettʼs in a Low-Volume Center Compared to Published Standards from High-Volume Centers

Introduction: Radiofrequency ablation (RFA) is an established treatment modality for Barrett's esophagus with dysplasia (BE-D), favored given its cost effectiveness, low recurrence and adverse event rates, and greater patient satisfaction. Although it is practiced universally, comparative data between low-volume versus high-volume centers is lacking. The aim of our study is to evaluate safety, efficacy and long term outcomes of RFA in a low-volume center, and compare it to existing published values from highvolume centers, in an attempt to improve quality and establish better practice standards. Methods: Our IRB approved retrospective study involved all patients enrolled in Barrett's RFA program at our institution between 2010 and 2014. Results: A total of 24 patients were identified, but data on 4 was partly unavailable due to change of endoscopy software during this period, and were hence excluded.Charts of 20 patients (M: F=18:2, Caucasian:Black=16:4) were finally reviewed. The common symptoms for EGD referral included heartburn (85%), water brash (15%), dysphagia (15%), nausea (10%) and GI bleeding (10%). The biopsies showed high-grade dysplasia (HGD) in 10, low-grade dysplasia (LGD) in 9 and one patient had mix of LGD-HGD. 4/20 patients had nodules, who underwent EMR but none had adenocarcinoma. No other treatment was attempted on any of these patients before RFA. The number of RFA sessions needed for complete resolution of BE was variable - 1 session (1 patient), 2 sessions (7 patients), 3 sessions (5 patients), 4 sessions (4 patients), 5 sessions (1 patient) and 6 sessions (1 patient). One patient was lost to follow-up after 5thsession. Circumferential ablation was used in 80% of the patients in first round, 14% in second round and only focal therapy was needed in patients after that. The RFA treatments were welltolerated, with transient nausea being the commonest post-procedure complaint (50%), followed by mild chest discomfort and dysphagia (16.7%). None of the patients had any serious complications including perforation or bleeding. None of the patients developed adenocarcinoma of the esophagus during the course of follow-up till date. Conclusion: RFA was found to be safe and equally efficacious in a low-volume center, as is in highvolume centers, and long term outcomes were reassuring. These results call for greater use of RFA in management of dysplastic BE, in non-referral centers also.

Primary surgical cytoreduction in advanced ovarian cancer: An outcome analysis within the MITO (Multicentre Italian Trials in Ovarian Cancer and Gynecologic Malignancies) Group

ObjectiveTo draw a reliable picture of the surgical management of advanced ovarian cancer (AOC) within the MITO Group, trying to correlate the disease extent at presentation, the category of center, and surgical outcome. MethodsThree tertiary referral centers for gynecologic oncology and four non-oncologic referral gynecologic surgical centers, participated in the project. A questionnaire was adopted to register perioperative data on AOCs (FIGO Stage IIICIV) consecutively operated on for a period of 12months. ResultsA total of 205 patients were registered into the study: 140 and 65 were recruited in oncological referral centers and non-referral centers, respectively. Following a multivariate analysis, the Eisenkop score and the category of center resulted the most potent predictors of complete surgical cytoreduction followed by PCI, preoperative CA125, and ASA score. Complete surgical cytoreduction was associated with oncological referral centers (60% vs 24.6%, p<0.001). The proportion of patients undergoing additional surgical procedures was significantly different comparing the two categories of centers (at least one additional procedure was performed in 81.4% vs 50.8% in oncological referral centers compared to the others, p<0.001). Despite the more aggressive surgery performed in oncological referral centers, the perioperative outcome measures were not significantly different in the two groups. ConclusionsThe chance of obtaining a complete cytoreduction mainly depends on patient characteristics, tumor spread, and quality of treatment. The latter is amenable for direct influence, and therefore, seems to be of utmost importance when considering efforts aiming at improvement in the outcome of this disease.

Who Is Treating AL Amyloidosis and How Are They Doing It? Results of a Global Physician Survey

Background: In light chain (AL) amyloidosis, aggregates of misfolded light chain or fragments of a light chain produced by clonal plasma cells accumulate in various organs and tissues, leading to progressive organ failure. This aggregation is particularly catastrophic when the heart is affected and is the primary cause of death. Diagnosis is often delayed because symptoms at onset can mimic those of other common conditions. Current treatments are aimed at eliminating the clonal plasma cells that produce the toxic light chains; however, optimal treatment regimens are undefined, and no therapies have received regulatory approval. Physicians were surveyed to identify potential investigators for clinical trials of AL amyloidosis and to detect regional treatment patterns. Here we present characteristics of physicians who typically treat this rare disease, the treatments they use, and differences across countries and regions.Method: The survey was designed and conducted by Prothena Biosciences in association with a contract research organization. Physicians (n = 133) in 22 countries were supplied the survey by email, and data were evaluated using descriptive statistics and χ2 tests.Results: Respondents (N = 116) represented 21 countries and 5 continents. The primary therapeutic area was hematology (80.2%), followed by oncology (14.7%), with the remaining physicians specializing in cardiology (2.6%) and internal medicine (2.6%). Most physicians (90.5%) practiced in an academic or university setting. The majority (99; 85.3%) self-identified as practitioners at major referral centers. These physicians saw an average of 65 patients with confirmed diagnoses of AL amyloidosis each year; 23 reported seeing ≥50 such patients each year, and 6 reported seeing ≥100 such patients each year. With the exception of one institution, physicians at nonreferral centers saw ≤18 patients with confirmed AL amyloidosis each year. Patients with newly diagnosed AL amyloidosis and those who were treatment naive accounted for 53.9% of all patients seen; of these, 60.4% had cardiac involvement. Most institutions (75.4%) preferred to use proteasome-inhibiting (PI) agents (bortezomib or combination) or marrow ablation followed by autologous stem cell transplantation (ASCT) in select patients (21.1%); these values generally paralleled actual treatments provided (64.0% and 20.6%, respectively). The remaining patients received treatments that did not include PI agents or ASCT. Equal percentages of European and North American responding physicians (65.4% vs 63.7%) reported using PI agents as initial treatment. However, significantly fewer European physicians than North American physicians used marrow ablation followed by ASCT (16.7% vs 26.4%; P < 0.05). Respondents from Asia, Australia, and South America, though fewer, primarily used PI agents as initial therapy and least frequently used marrow ablation and ASCT. Among all respondents, ASCT was performed primarily after chemotherapy (30%) rather than as initial treatment (13%); however, average timing to proceed to ASCT varied broadly (weeks to months).Conclusions: AL amyloidosis is treated primarily by hematologists with the use of proteasome-inhibiting agents, either alone or in combination. There is marked regional variation in the use of marrow ablation with ASCT. AL amyloidosis is a rare disease that, despite these treatments, remains progressive and fatal. Optimal treatment regimens remain undefined, and no specific therapeutic approaches exist that reverse organ dysfunction. DisclosuresGuthrie:Prothena Biosciences Inc: Employment, Other: Stock. Chapman:Prothena Biosciences Inc: Employment, Other: Stock. Koller:Prothena Biosciences Inc: Employment, Other: Stock, Travel expenses. Walling:Prothena Biosciences Inc: Consultancy. Kinney:Prothena Biosciences Inc: Employment, Other: Stock.

County-Level Effects of Prehospital Regionalization of Critically Ill Patients: A Simulation Study.

Regionalization may improve critical care delivery, yet stakeholders cite concerns about its feasibility. We sought to determine the operational effects of prehospital regionalization of nontrauma, nonarrest critical illness. King County, Washington. Discrete event simulation study. All 2006 hospital discharge data, linked to all adult, eligible patients transported by county emergency medical services agencies. We simulated active triage of high-risk patients to designated referral centers using a validated prehospital risk score; we studied three regionalization scenarios: 1) up triage, 2) up and down triage, and 3) up and down triage after reducing ICU beds by 25%. We determined the effect on patient routing, ICU occupancy at referral and nonreferral hospitals, and emergency medical services transport times. A total of 119,117 patients were hospitalized at 11 nonreferral centers and 76,817 patients were hospitalized at three referral centers. Among 20,835 emergency medical services patients, 7,817 patients (43%) were eligible for up triage and 10,242 patients (57%) were eligible for down triage. At baseline, mean daily ICU bed occupancy was 61% referral and 47% at nonreferral hospitals. Up triage increased referral ICU occupancy to 68%, up and down triage to 64%, and up and down triage with bed reduction to 74%. Mean daily nonreferral ICU occupancy did not exceed 60%. Total emergency medical services transport time increased by less than 3% with up and down triage. Regionalization based on prehospital triage of the critically ill can allocate high-risk patients to referral hospitals without adversely affecting ICU occupancy or prehospital travel time.

Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry.

There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. To describe the epidemiology, outcomes, and risk factors for mortality in pediatric pulmonary hypertension in Spain. We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, a total of 225 patients diagnosed with pulmonary hypertension in 1998 or after were collected from 21 referral and nonreferral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population, and analyzed risk factors for mortality (Nice etiologic group, clinical and hemodynamic variables). Patients were classified as follows: group I, pulmonary arterial hypertension (n = 142; 61%); group II, left heart disease (n = 31; 14%); group III, respiratory disease (n = 41; 18%); group IV, thromboembolic pulmonary hypertension (n = 2; 1%); or group V, mostly inherited metabolic diseases (n = 10; 4.5%). Of the patients studied, 31% had multifactorial pulmonary hypertension. Mean age at diagnosis was 4.3 ± 4.9 years (50% < 2 yr). Survival rates at 1 and 3 years were 80 and 74% for the whole cohort, and 89 and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (P < 0.001), age at diagnosis younger than 2 years old (P < 0.001), advanced functional class at diagnosis (P < 0.001), and high right atrial pressure at diagnosis (P = 0.002). In moderate to severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.

P-024 YI The Incidence of Crohn’s Disease and Rate of Early Surgery in the U.S. Military Health Care System

Surgery is often required to treat complications of Crohn's Disease (CD) such as medically refractory inflammation, fistulas or strictures. Surgical intervention, especially early in the disease course, may be decreasing with the advent of more effective medical therapy for CD as gastroenterologists rely more readily on immunosuppressive strategies before referring for surgery. The US Military Health System (MHS) has a diverse population of patients with equal access, and the direct care military treatment facilities (MTF) utilize the same electronic medical record (EMR) nationwide. The aim of this study was to calculate the incidence of CD in a nationwide military population and to further characterize the CD population, while also assessing outcomes such as surgery, hospitalization and emergency room visits during the first 2 years of disease. Using the EMR and healthcare claims databases under the MHS Data Repository (MDR), the total number of new diagnoses of CD were examined from 2009 to 2012. The data collected were derived from a nationwide network of military treatment facilities and included a total study population of 3.2 million. For each new diagnosis of CD, demographic variables, basic laboratory data, other chronic comorbidities, and serious associated diseases or complications were analyzed. Outcomes such as ER visits, admissions, surgeries and deaths were also recorded up to 2 years from the time of diagnosis. Seven hundred and three new diagnoses of Crohn’s disease were identified. The average annual incidence of CD was 7.3 per 100,000 patients per year. Fifty-two percent of patients were male. Fifty-three percent of patients were under the age of 35. Within two years of diagnosis, 9% patients underwent surgery for CD with an average annual rate of 1 surgery for every 11 new diagnoses per year. Within two years of diagnosis, 38% of patients were admitted to the hospital (71% of these admissions were directly related to CD). Seven percent of patients were admitted to the ICU and 64% were seen in the ER. Two percent required total parenteral nutrition. No patients died. Three percent of patients were diagnosed with Clostridium difficile colitis. Two percent of patients had a pulmonary embolism or deep vein thrombosis. Our study shows a similar incidence rate of CD compared to an earlier large population study in the US (7.3/100,000 compared to 7.9/100,000), which suggests a stable incidence over time. The early surgery rate in our study is lower than that found in other published studies. This may correlate with a trend of decreasing reliance on surgery in the era of biologic medications. Our hospitalization rate is slightly higher than that reported in an earlier study in Olmsted County (32% at 1 year), but lower than that reported in an earlier study from Denmark (83% at 1 year) which indicates that treatments are more effective, but the disease still portends significant morbidity and utility of healthcare resources. Further research may help further elucidate the effects that the biologic agents have had in improving outcomes and reducing surgery in non-referral centers.

Implantable Cardioverter-defibrillator Therapy for Hypertrophic Cardiomyopathy: Usefulness in Primary and Secondary Prevention

Introduction and objectivesHypertrophic cardiomyopathy is a frequent cause of sudden death. Clinical practice guidelines indicate defibrillator implantation for primary prevention in patients with 1 or more risk factors and for secondary prevention in patients with a history of aborted sudden death or sustained ventricular arrhythmias. The aim of the present study was to analyze the follow-up of patients who received an implantable defibrillator following the current guidelines in nonreferral centers for this disease. MethodsThis retrospective observational study included all patients who underwent defibrillator implantation between January 1996 and December 2012 in 3 centers in the province of Barcelona. ResultsThe study included 69 patients (mean age [standard deviation], 44.8 [17] years; 79.3% men), 48 in primary prevention and 21 in secondary prevention. The mean number of risk factors per patient was 1.8 in the primary prevention group and 0.5 in the secondary prevention group (P=.029). The median follow-up duration was 40.5 months. The appropriate therapy rate was 32.7/100 patient-years in secondary prevention and 1.7/100 patient-years in primary prevention (P<.001). Overall mortality was 10.1%. Implant-related complications were experienced by 8.7% of patients, and 13% had inappropriate defibrillator discharges. ConclusionsIn patients with a defibrillator for primary prevention, the appropriate therapy rate is extremely low, indicating the low predictive power of the current risk stratification criteria.

Terapia con desfibrilador automático implantable en la miocardiopatía hipertrófica: utilidad en prevención primaria y secundaria

Introduction and objectivesHypertrophic cardiomyopathy is a frequent cause of sudden death. Clinical practice guidelines indicate defibrillator implantation for primary prevention in patients with 1 or more risk factors and for secondary prevention in patients with a history of aborted sudden death or sustained ventricular arrhythmias. The aim of the present study was to analyze the follow-up of patients who received an implantable defibrillator following the current guidelines in nonreferral centers for this disease. MethodsThis retrospective observational study included all patients who underwent defibrillator implantation between January 1996 and December 2012 in 3 centers in the province of Barcelona. ResultsThe study included 69 patients (mean age [standard deviation], 44.8 [17] years; 79.3% men), 48 in primary prevention and 21 in secondary prevention. The mean number of risk factors per patient was 1.8 in the primary prevention group and 0.5 in the secondary prevention group (P=.029). The median follow-up duration was 40.5 months. The appropriate therapy rate was 32.7/100 patient-years in secondary prevention and 1.7/100 patient-years in primary prevention (P<.001). Overall mortality was 10.1%. Implant-related complications were experienced by 8.7% of patients, and 13% had inappropriate defibrillator discharges. ConclusionsIn patients with a defibrillator for primary prevention, the appropriate therapy rate is extremely low, indicating the low predictive power of the current risk stratification criteria.Full English text available from: www.revespcardiol.org/en

Extended Lymphadenectomy in Gastric Cancer Is Debatable

Much debate still exists regarding the appropriate extent of lymphadenectomy for gastric adenocarcinoma. In high incidence countries in Eastern Asia, more extensive (e.g. D2) lymphadenectomies are standard, and these surgeries are generally done by experienced surgeons with low morbidity (<20 %) and mortality (<1 %). In United States and Western Europe, where the incidence of gastric adenocarcinoma is much lower, the majority of patients are treated at non-referral centers with less extensive (e.g. D1 or D0) lymphadenectomy. This symposium article first reviews early studies that led to recommendations for less extensive lymphadenectomy. Two large prospective, randomized trials performed in the United Kingdom and the Netherlands in the 1990s failed to demonstrate a survival benefit of D2 over D1 lymphadenectomy, but these trials have been criticized for inadequate surgical training and high surgical morbidity (43-46 %) and high mortality rates (10-13 %) in the D2 group. We then discuss more contemporary studies that support more extensive lymphadenectomy with a minimum of 16 lymph nodes for adequate staging. The 15-year follow-up of the Netherlands trial now demonstrates an improved disease-specific survival and locoregional recurrence in the D2 group. A prospective, randomized trial from Taiwan found a survival benefit of more extensive lymphadenectomies, and another randomized trial from Japan found adding dissection of para-aortic nodes to a D2 lymphadenectomy did not improve survival. Western surgeons have increasingly accepted the importance of performing more than a D1 node dissection, and Eastern surgeons are accepting that more than a D2 node dissection does not improve survival and increases morbidity. Thus both Eastern and Western approaches are favoring D2 lymphadenectomy as a standard, and on this topic we appear to be harmonizing.

Varying Lymphadenectomies for Gastric Adenocarcinoma in the East Compared with the West: Effect on Outcomes

There are notable differences in surgical approaches to gastric adenocarcinoma throughout the world, particularly in terms of the extent of lymphadenectomy (LAD). In high-incidence countries such as Japan and South Korea, more extensive (e.g., D2) lymphadenectomies are standard, and these surgeries are generally done by experienced surgeons with low morbidity and mortality. In countries such as the United States, where the incidence of gastric adenocarcinoma is 10-fold lower, the majority of patients are treated at nonreferral centers with less extensive (e.g., D1 or D0) lymphadenectomy. There is little disagreement among gastric cancer (GC) experts that the minimum lymphadenectomy that should be performed for gastric adenocarcinoma should be at least a D1 lymphadenectomy, and many of these experts recommend a D2 lymphadenectomy. More extensive lymphadenectomies provide better staging of patient disease and likely reduce locoregional recurrence rates. Two large, prospective randomized trials performed in the United Kingdom and the Netherlands in the 1990s failed to demonstrate a survival benefit of D2 over D1 lymphadenectomy, but these trials have been criticized for inadequate surgical training and high surgical morbidity and mortality rates (10% to 13%) in the D2 group. More recent studies have demonstrated that Western surgeons can be trained to perform D2 lymphadenectomies on Western patients with low morbidity and mortality. The 15-year follow-up of the Netherlands trial now demonstrates an improved disease-specific survival and locoregional recurrence in the D2 group. Retrospective analyses and one prospective, randomized trial suggest that there may be a survival benefit to more extensive lymphadenectomies when performed safely, but this assertion requires further validation.