PW342 New scoring system for risk evaluation in Eisenmenger syndrome

PW342 New scoring system for risk evaluation in Eisenmenger syndrome

PW342 New scoring system for risk evaluation in Eisenmenger syndrome Iveta Simkova*1, Monika Kaldararova2, Tatiana Valkovicova1, Anna Remkova3 1 Depar...

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PW342 New scoring system for risk evaluation in Eisenmenger syndrome Iveta Simkova*1, Monika Kaldararova2, Tatiana Valkovicova1, Anna Remkova3 1 Department of Cardiology and Angiology, Slovak Medical University and National Institute of Cardiovascular Diseasess, 2Children’s Cardiac Centre, National Institute of Cardiovascular Diseases, 3Hemomedika, Centre of Hemostasis and Thrombosis, Bratislava, Slovakia Introduction: Eisenmenger syndrome (ES) means irreversible pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD); with right-to-left shunt leading to systemic desaturation. ES patients (pts) are beyond CHD+PAH suffering also from complications due to cyanosis and secondary cyanosis-related multisystemic dysfunction. These non-cardiac problems are very often the cause of significant clinical worsening or even death. Objectives: Based on risk factors analysis during prospective long-term follow-up to create a scoring system predicting high-risk pts. Methods: 40 ES pts (28F/12M; median age 41.5 years; simple/combined shunts/complex CHD 57.5%/17.5%/25%) underwent detailed analysis of subjective complaints and noncardiac diseases, clinical, laboratory, ECG analysis, 24-hour ECG monitoring, 6MWD, echocardiography. Results: Risk factors were considered: age >40y. (55%), complex CHD (25%), Down syndrome (15%), NYHA III.-IV. (27.5%), 6MWD <380m (27.5%), arrhythmias (40%), syncope (5%), right ventricular dysfunction (10%), increased NTpro-BNP (>1000ng/l) (35%), systemic desaturation <85% (42.5%), oxygen therapy (15%), erythrocytosis (60%), hematocrite 0.65 (7.5%), hyperviscose syndrome (2.5%), hyposideremia (55%), vitamin B12 or folic acid deficiency (20%), bleeding event (35%), hemoptysis (12.5%), clinical signs of thromboembolism (17.5%), laboratory parameters indicating increased bleeding risk (30%) and risk for thrombosis (57.5%). Multisystemic affection: respiratory disease (35%), renal dysfunction (25%), hepatobiliar dysfunction (27.5%), hyperuricemia (57.5%), hypoalbuminemia (0), presence of 2 non-cardiac organ affection (70%). Scoring was performed as follows: for each factor present was added 1 point. High-risk pts were considered 10 points (35%), medium risk 6-9 points (35%) and low-risk pts with <5 points (30%). Conclusion: In ES management crucial is the understanding of secondary affections due to cyanosis and multisystemic dysfunction. These are often even more important for survival and maintenance of clinical stability than PAH/CHD itself. Many of these problems were separately analyzed and identified to play an important role in ES; though no systematic approach was created so far. A unique complex scoring system is now presented that can significantly help to evaluate ES pts, especially to define the ones at highest risk; and to help to prevent complications and improve further management and treatment considerations. Disclosure of Interest: None Declared

PW343 Psychosocial Morbidity in Adolescents and Young Adults with Congenital Heart Disease: Psychosocial Adjustment, Psychiatric Morbidity, Quality of Life and School Performance may be affected, but Social Support and traits of Personality play an important role in resilience Maria Emília G. Areias*1, Liliana Gomes1,2, Daniela Cerqueira1,2, Catarina Pinto1,2, Patrícia Vieira1,2, Isabela Freitas1,2, Flávio Teixeira1,2, Rosália Coelho1,2, Cláudia Moura3,4, Victor Viana5,6, José Carlos Areias3,4,7 1 Department of Psychology, Instituto Superior de Ciências da Saúde (CESPU), 2UNIPSA/CICS (CESPU), Gandra-Paredes, 3Pediatric Cardiology, Hospital S. João, 4Unidade de Investigação Cardiovascular, Faculty of Medicine, University of Porto, 5Pediatrics, Hospital S. João, 6Faculty of Nutrition, University of Porto, 7Pediatrics, Faculty of Medicine, University of Porto, Porto, Portugal

GHEART Vol 9/1S/2014


March, 2014


POSTER/2014 WCC Posters

Introduction: Remarkable progresses in medical and surgical care in congenital heart disease (CHD) allow patients to survive until a later time in adulthood and they are facing challenges for adjustment throughout their life cycle. Objectives: To study Quality of Life (QOL), Psychiatric Morbidity (PM), Psychosocial Adjustment (PSA), School Performance (SP), Physical Limitations (PL) of patients with Congenital Heart Disease (CHD) and the role of Social Support (SS) and Personality (P) on resilience. Methods: 196 CHD patients, 111 male, 12-26 years (M¼16.973.30), 109 cyanotic. Clinical and demographic history was collected. Participants were interviewed once on topics as SS, family educational style, self-image and PL, were administered a standardized psychiatric interview (SADS-L) and completed self-report questionnaires on P (NEO-PI-R), QOL (WHOQOL-BREF) PSA (YSR,ASR). One of their relatives filled the observational versions of the questionnaires (CBCL,ABCL). Results: We found a 15.3% lifetime prevalence of psychopathology (12.6%:males;18.8%:females) and 54.9% retentions in school (M¼ 1.55 year  0.50). Comparing our patients to the population as a whole, they have better QOL in environmental (t¼7.946;p¼0.000), social relationships:t¼4.296;p¼0.000), and overall (t¼2.489; p¼0.014). Cyanotic showed worse QOL (physical:t¼-4.407;p¼0.000; psychological:t¼2.805; p¼0.006; environmental:t¼-4.233;p¼0.000) and overall. Complex CHD report worse QOL (physical: t¼-3.357; p¼0.001; environmental: t¼-2.796; p¼0.006) and more somatic complaints (u¼1644.000; p¼0.033); female patients refer more somatic complaints (u¼4400.000; p¼0.005), feelings of anxiety/depression (u¼3803.500; p¼0.020), thought problems (u¼3489.000; p¼0.001) and internalization (u¼2655.500; p¼0.000); Patients submitted to surgery report worse QOL (physical: t¼-3.268; p¼0.001; psychological: t¼-2.934; p¼0.004; environmental: t¼-2.236; p¼0.027) and more attention problems (u¼2979.000; p¼0.005). Patients with better SS have better QOL (physical:t¼2.128;p¼0.035; psychological:t¼2.161;p¼0.032;social relationships: t¼4.130; p¼0.000; environmental:t¼2.618;p¼ 0.010; overall:u¼2001.000; p¼0.002), and less withdrawn behaviour (u¼2102.500;p¼0.011). Neurotic traits correlate to worse QOL and more social problems in cyanotic patients, and more social problems in the whole group. Conclusion: CHD patients seemed to be more prone to PM, worse PSA and SP, although SS plays a crucial role in resilience. P seem to provide a key explanation for a better or worse QOL and PSA. Disclosure of Interest: None Declared

PW344 Associations between sense of coherence, self-perceived health and physical health status in Australian adults with congenital heart disease Sarah Eaton*1, QiFeng Wang2, Samuel Menahem3 Melbourne Medical School, University of Melbourne, 2Monash University, Melbourne, 3 Wheatbelt GP Network, Perth, Australia


Introduction: Continuous advances in treatment of congenital heart disease (CHD) have resulted in an increasing number of infants and children progressing to adulthood. A strong sense of coherence has been found to have a positive impact on the health and well-being of adults with chronic health conditions. Objectives: To determine how sense of coherence and self-perceived health relate to the physical health status of adults with CHD. Methods: As part of the APPROACH - International Study, adults with CHD were recruited from a consultant practice. Self reported questionnaires were completed including a 12 item health status survey, EuroQOL 5D, Brief Illness Perception Questionnaire, Orientation to Life Questionnaire and Hospital Anxiety and Depression Scale. Clinical information was provided by the participants’ current cardiologist. Results: To date, 40 participants have been recruited with complete data available on 34. Ages range from 17-49, with a mean age of 27. Currently 97% of participants are in NYHA class 1. More than three quarters have had surgery for their cardiac condition. A higher sense of coherence was found to positively correlate with a better physical health status (Pearson correlation r¼0.45, p<0.05), while negatively correlating with symptoms of anxiety and depression (r¼-0.48, p<0.01) and impact on life (r¼-0.39, p<0.05). It was found that a worse perception of illness positively correlated with greater impact on participants lives (r¼0.46, p<0.05) and negatively correlated with their physical health status (r¼-0.42, p<0.05). Conclusion: Participants with a strong sense of coherence experienced less impact on their life, less symptoms of anxiety and depression and better physical health. Participants who perceived their illness as more threatening experienced a greater impact on their life and worse physical health. These findings highlight the importance for clinicians of adults with CHD to focus on the positive aspects of their health despite the impact of their congenital anomaly. Disclosure of Interest: None Declared

PW345 Satisfaction with life and quality of life in adults with congenital heart disease and their correlation with anxiety and depression – An Australian sample Sarah Eaton*1, QiFeng Wang2, Samuel Menahem3 Melbourne Medical School, University of Melbourne, 2Monash University, Melbourne, 3 Wheatbelt GP Network, Perth, Australia


Introduction: Following medical and surgical advances almost 85% of children born with congenital heart disease (CHD) reach adulthood. Many report a worse quality of life and



Objectives: We sought to document the frequency and causes of death for those with a diagnosis of PAH complicating CHD, which has so far not been well described. Methods: We have established a Bi-National Registry of PAH-CHD cases from Australia and New Zealand, comprising 360 adults with a confirmed diagnosis of PAH and CHD and who have been seen at least once since Jan 1, 2000, at age over 16 years. Results: 70 patients are known to have died (31 males, age 37  13 years). The underlying causes of PAH were ASD (21%), PDA (23%), VSD (30%) and AVSD (16%); the others had more complex CHD lesions (12%). 73% had Eisenmenger Syndrome and 24% had Down’s Syndrome. Compared to survivors, the decedents were more likely to have a single ventricle (13% vs 4%; p<0.001) and were less likely to have Tetralogy of Fallot (1% vs 10%; p<0.02). There was no significant difference in the proportion of males, smokers or those with Eisenmenger Syndrome, Down’s, ASD, PDA, AVSD or associated lung disease in survivors compared with decedents. A similar proportion of patients in both groups (27%) had undergone an intervention for their congenital heart disease. The causes of death were varied and predominantly include cardiac arrest, right heart failure, renal failure and sepsis. Conclusion: Death is common in adults with PAH-CHD, often at a young age. The commonest causes are progressive heart failure and sudden cardiac deaths however sepsis and other causes have been documented. The effect of therapy on the risk and causes of death in this group requires further prospective study. Disclosure of Interest: None Declared