bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2018‒12‒02
23 papers selected by
Richard James
University of Pennsylvania
  1. The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.
  2. Adults with congenital heart disease overestimate their physical activity level.
  3. The role of illness uncertainty in the relationship between disease knowledge and patient-reported outcomes among adolescents and adults with congenital heart disease.
  4. Postpartum anticoagulation in women with mechanical heart valves.
  5. First-in-man pulmonary artery stenting in children using the Bentley® BeGrow™ stent system for newborns and infants.
  6. Metformin intervention prevents cardiac dysfunction in a murine model of adult congenital heart disease.
  7. Bleeding and thrombotic events occur early in children on durable ventricular assist devices.
  8. First real-world experience with mobile health telemonitoring in adult patients with congenital heart disease.
  9. Predicting acquired cardiovascular disease in adults with congenital heart disease is risky business.
  10. Dysrhythmias in patients with a complete atrioventricular septal defect: From surgery to early adulthood.
  11. Success in a Fontan pregnancy: how important is ventricular function?
  12. Global outreach to improve the provision of cardiopulmonary bypass for patients with congenital heart disease.
  13. Struggling and Overcoming Daily Life Barriers Among Children With Congenital Heart Disease and Their Parents in the West Bank, Palestine.
  14. A Novel Nesting Protocol to Decrease Readmission and Increase Patient Satisfaction Following Congenital Heart Surgery.
  15. Patient and physician view on patient information and decision-making in congenital aortic and pulmonary valve surgery.
  16. Long Term Outcome Of Infants With Nec.
  17. A Novel Model Demonstrates Variation in Risk-Adjusted Mortality Across Pediatric Cardiac ICUs After Surgery.
  18. When Parents Have Misunderstandings About the Risks and Benefits of Palliative Surgery.
  19. Risk Factors and Biomarkers of Poor Outcomes.
  20. Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study.
  21. Augmentation of the pulmonary arteries at or prior to the Fontan procedure is not associated with worse long-term outcomes: a propensity-matched analysis from the Australia-New Zealand Fontan Registry.
  22. The current practice and care of paediatric patients post cardiac catheterisation.
  23. Post-Operative Pain and Comfort in Children After Heart Surgery: A Comparison of Nurses and Families Pre-operative Expectations.
  1. Int J Cardiol. 2018 Nov 07. pii: S0167-5273(18)32296-4. [Epub ahead of print]
    The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.
    Montanaro C, Merola A, Kempny A, Alvarez-Alvarez B, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan SV, Gatzoulis MA, Dimopoulos K.
      OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia.BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging.
    METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3).
    RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias.
    CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.
    Keywords:  Adult; Congenital heart disease; Cyanosis; Outcome; Pulmonary atresia
    DOI:  https://doi.org/10.1016/j.ijcard.2018.11.011
  2. Int J Cardiol Heart Vasc. 2019 Mar;22 13-17
    Adults with congenital heart disease overestimate their physical activity level.
    Larsson L, Johansson B, Wadell K, Thilén U, Sandberg C.
      Background: Physical activity reduces the risk of acquired cardiovascular disease, which is of great importance in patients with congenital heart disease (CHD). There are diverging data whether physical activity level (PAL) differs between patients with CHD and controls. Furthermore, it is unknown if PAL can be reliably assessed in patients with CHD using self-reported instruments.Methods: Seventy-five patients with CHD (mean age 37.5 ± 15.5 years, women n = 29 [38.7%]) and 42 age and sex matched controls completed the International Physical Activity Questionnaire (IPAQ) and carried the activity monitor Actiheart over 4 days. Time spent at ≥3 METS ≥21.4 min/day, i.e. reaching the WHO recommendation for PAL to promote health, was used as the outcome measure. Data on PAL obtained from IPAQ were compared with Actiheart.
    Results: The proportion of individuals reaching target PAL according to IPAQ was similar in patients with CHD and controls (70.7%vs.76.2%, p = 0.52) as well as between patients with simple and complex lesions. There was an overall difference between IPAQ and Actiheart in detecting recommended PAL (72.6%vs.51.3%, p < 0.001). In a subgroup analysis, this difference was also detected in patients but was borderline for controls. The negative predictive value for IPAQ in detecting insufficient PAL was higher in patients than in controls (73%vs.40%).
    Conclusions: The proportion of persons reaching sufficient PAL to promote health was similar in patients and controls. The self-reported instrument overestimated PAL in relation to objective measurements. However, with a high negative predictive value, IPAQ is a potentially useful tool for detecting patients with insufficient PAL.
    Keywords:  Actiheart; Adult congenital heart disease; IPAQ; Physical activity level
    DOI:  https://doi.org/10.1016/j.ijcha.2018.11.005
  3. Heart Lung. 2018 Nov 21. pii: S0147-9563(18)30214-0. [Epub ahead of print]
    The role of illness uncertainty in the relationship between disease knowledge and patient-reported outcomes among adolescents and adults with congenital heart disease.
    Schiele SE, Emery CF, Jackson JL.
      BACKGROUND: Greater general disease knowledge predicts better patient-reported outcomes (PROs) among congenital heart disease (CHD) survivors, but higher illness uncertainty is associated with elevated emotional distress and poorer well-being among patients with chronic disease.OBJECTIVES: This study explored the relationship of illness uncertainty and disease knowledge with emotional distress and health-related quality of life (HRQoL) among patients with CHD.
    METHODS: Individuals with CHD (N = 169, ages 15-39) completed self-report measures of disease knowledge (general and risk-related), illness uncertainty, depressive and anxiety symptoms, and HRQoL. Pearson correlations and regressions analyses were utilized.
    RESULTS: Greater risk-related knowledge was associated with greater anxiety (b = .41, p = .03, 95% CI = [.04, .77]) and poorer emotional HRQoL (b = -.53, p = .03, 95% CI = [-1.02, -.05]) when illness uncertainty was higher.
    CONCLUSION: When individuals with CHD feel uncertain about their disease course and outcomes, knowledge about future cardiovascular risks may result in higher levels of distress.
    Keywords:  Anxiety; Congenital heart disease; Depression; Disease knowledge; Health-related quality of life; Illness uncertainty
    DOI:  https://doi.org/10.1016/j.hrtlng.2018.10.026
  4. Int J Womens Health. 2018 ;10 663-670
    Postpartum anticoagulation in women with mechanical heart valves.
    Irani RA, Santa-Ines A, Elder RW, Lipkind HS, Paidas MJ, Campbell KH.
      Background: Women with mechanical heart valves (MHV) requiring anticoagulation (AC) are at high risk for hemorrhagic complications. Despite guidelines to manage antenatal and peripartum AC, there are few evidence-based recommendations to guide the initiation of postpartum AC. We reviewed our institutional experience of pregnant women with MHV to lay the groundwork for recommendations of immediate postpartum AC therapy.Study design: This descriptive retrospective cohort used ICD-9 and -10 codes to identify pregnant women with MHV on AC at the Yale-New Haven Hospital from 2007 to 2018. All identified patients were confirmed by chart review. Delivery hospitalization and the immediate postpartum AC management were reviewed. Maternal complications recorded were postpartum hemorrhage, transfusion, wound hematoma, intra-abdominal bleeding, stroke, valve thrombosis, and death. Further, immediate neonatal outcomes were detailed.
    Results: Forty-two pregnant women with nonnative heart valves were identified during the study period. From those pregnant women, nine had an MHV and were anticoagulated throughout gestation. Of 19 total pregnancies, 14 met the inclusion criteria. The median gestational age of the delivered pregnancies was early term (37w2d). Nine deliveries were via cesarean (64%). The median time to restart AC after birth was 6 hours. After six deliveries (43%), AC was initiated ≤6 hours postpartum. Hemorrhagic complications occurred in six cases (43%), including wound and intra-abdominal hematomas. Four cases (29%) required blood transfusion. No maternal strokes, thrombotic events, or deaths were recorded. Five (38.5%) neonates required admission to the neonatal intensive care unit.
    Conclusion: MHV in pregnancy was rare but was associated with significant maternal morbidity, particularly postpartum hemorrhagic complications. We noted significant variability in the timing of restarting postpartum AC and in the selected agents. Pooled institutional data and an interdisciplinary approach are recommended to minimize competing risks and sequelae of valve thrombosis and obstetrical hemorrhage and, thereby, to optimize maternal outcomes and develop evidence-based guidelines for postpartum AC management.
    Keywords:  anticoagulation; mechanical heart valve; postpartum hemorrhage; pregnancy
    DOI:  https://doi.org/10.2147/IJWH.S177547
  5. Int J Cardiol. 2018 Nov 19. pii: S0167-5273(18)35156-8. [Epub ahead of print]
    First-in-man pulmonary artery stenting in children using the Bentley® BeGrow™ stent system for newborns and infants.
    Quandt D, Knirsch W, Michel-Behnke I, Kitzmüller E, Obradovic M, Uhlemann F, Kretschmar O.
      BACKGROUND: Stent implantation into growing vessels is a common treatment option in infants and children with congenital heart disease (CHD) and corresponding vessel lesions. After stent implantation in small children, repetitive stent redilations are frequently necessary to accommodate for somatic growth. Until now, all available stents have limited final expansion diameters.MATERIAL AND RESULTS: The new Bentley BeGrow™ stent system for newborns and infants is a L605 cobalt‑chromium, pre-mounted, balloon expandable stent, which is compatible with a 4 French sheath and 0.014 inch guide wire thus allowing implantation in small vessels (4-6 mm). It offers a new, unique stent design that allows post-dilation steps up to Ø11.5 mm. While re-dilating up to Ø11.5 mm this new stent maintains radial force and shows uniform expansion with only minimal foreshortening. Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5 mm. Residual radial force maintains even after stent opening due to spiral arrangement of the predetermined breaking points. The 2 first-in-man pulmonary artery stent implantations in a newborn with univentricular circulation and a toddler with biventricular circulation are reported as part of the currently performed licencing trial (ClinicalTrials.govNCT03287024).
    CONCLUSION: The low-profile BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. In our first experience, it can be effectively implanted. Longer follow-up will evaluate multiple, stepwise redilations and controlled stent strut breakage, which have the potential to accommodate for somatic vessel growth and/or subsequent implantation of larger stents.
    DOI:  https://doi.org/10.1016/j.ijcard.2018.11.029
  6. Mol Metab. 2018 Nov 15. pii: S2212-8778(18)30836-6. [Epub ahead of print]
    Metformin intervention prevents cardiac dysfunction in a murine model of adult congenital heart disease.
    Wilmanns JC, Pandey R, Hon O, Chandran A, Schilling JM, Forte E, Wu Q, Cagnone G, Bais P, Philip V, Coleman D, Kocalis H, Archer SK, Pearson JT, Ramialison M, Heineke J, Patel HH, Rosenthal NA, Furtado MB, Costa MW.
      OBJECTIVE: Congenital heart disease (CHD) is the most frequent birth defect worldwide. The number of adult patients with CHD, now referred to as ACHD, is increasing with improved surgical and treatment interventions. However the mechanisms whereby ACHD predisposes patients to heart dysfunction are still unclear. ACHD is strongly associated with metabolic syndrome, but how ACHD interacts with poor modern lifestyle choices and other comorbidities, such as hypertension, obesity, and diabetes, is mostly unknown.METHODS: We used a newly characterized mouse genetic model of ACHD to investigate the consequences and the mechanisms associated with combined obesity and ACHD predisposition. Metformin intervention was used to further evaluate potential therapeutic amelioration of cardiac dysfunction in this model.
    RESULTS: ACHD mice placed under metabolic stress (high fat diet) displayed decreased left ventricular ejection fraction. Comprehensive physiological, biochemical, and molecular analysis showed that ACHD hearts exhibited early changes in energy metabolism with increased glucose dependence as main cardiac energy source. These changes preceded cardiac dysfunction mediated by exposure to high fat diet and were associated with increased disease severity. Restoration of metabolic balance by metformin administration prevented the development of heart dysfunction in ACHD predisposed mice.
    CONCLUSIONS: This study reveals that early metabolic impairment reinforces heart dysfunction in ACHD predisposed individuals and diet or pharmacological interventions can be used to modulate heart function and attenuate heart failure. Our study suggests that interactions between genetic and metabolic disturbances ultimately lead to the clinical presentation of heart failure in patients with ACHD. Early manipulation of energy metabolism may be an important avenue for intervention in ACHD patients to prevent or delay onset of heart failure and secondary comorbidities. These interactions raise the prospect for a translational reassessment of ACHD presentation in the clinic.
    Keywords:  Adult congenital heart disease; Metabolism; Metformin; Obesity
    DOI:  https://doi.org/10.1016/j.molmet.2018.11.002
  7. Thromb Res. 2018 Nov 22. pii: S0049-3848(18)30613-3. [Epub ahead of print]173 65-70
    Bleeding and thrombotic events occur early in children on durable ventricular assist devices.
    Huang JY, Ignjatovic V, Sheridan BJ, Mathew J, D'Udekem Y, Brink J, Barton R, Callea G, Morsman D, Donath S, Opat S, Monagle P.
      INTRODUCTION: Durable Ventricular Assist Devices (VADs) are increasingly used in children with end-stage heart failure. Major complications are bleeding and thromboembolism (TE). Our objective was to determine the timing, incidence and risk factors for bleeding and TE in children implanted with VADs.METHODS: This was a retrospective cohort of 8 years experience for children implanted with HeartWare HVAD and Berlin Heart EXCOR VADs at the Royal Children's Hospital, Melbourne.
    RESULTS: 44 patients were implanted with Berlin Heart EXCOR or HeartWare HVAD devices. Major bleeding occurred in 17 patients (39%), 7 (16%) experienced thromboembolic strokes, 13 (30%) required device exchange for TE, and 4 (9%) experienced arterial thromboembolism. Twenty-seven patients (61%) were transplanted, three (7%) recovered, and six (14%) remain on device when censored. Eight patients (18%) died on VAD, with leading causes being thromboembolic stroke and intracranial bleeding. The majority of bleeding events and thromboembolic events occurred while patients were on unfractionated heparin (bleeding 66%, TE 40.5%) or transitioning between heparin and warfarin (bleeding 22%, TE 38%). Majority of patients were on more than one antiplatelet agent at the time of a major bleeding (87%) or thromboembolic (89%) event.
    CONCLUSIONS: The majority of bleeding and TE events occurring in children supported with durable VADs occur when they are on unfractionated heparin or transitioning to warfarin. Modifications to anticoagulation and monitoring in the early post-operative periods should be a research focus.
    Keywords:  Bleeding; Heart failure; Mechanical circulatory support; Pediatrics; Thrombosis; Ventricular assist device
    DOI:  https://doi.org/10.1016/j.thromres.2018.11.019
  8. Neth Heart J. 2018 Nov 28.
    First real-world experience with mobile health telemonitoring in adult patients with congenital heart disease.
    Koole MAC, Kauw D, Winter MM, Dohmen DAJ, Tulevski II, de Haan R, Somsen GA, Schijven MP, Robbers-Visser D, Mulder BJM, Bouma BJ, Schuuring MJ.
      BACKGROUND: Arrhythmias and heart failure are common and invalidating sequelae in adult patients with congenital heart disease (CHD). Mobile health (m-Health) enables daily monitoring and a timely response that might prevent deterioration. We present an observational prospective registry to evaluate feasibility of an m‑Health telemonitoring program for managing arrhythmia, heart failure and blood pressure in symptomatic adults with CHD.METHODS: Symptomatic adult patients with CHD are enrolled in an m‑Health telemonitoring program, which evaluates single-lead ECG, blood pressure and weight measurements. In case of symptoms extra measurements could be performed. Data are collected by mobile apps, matched with individualised thresholds. Patients are contacted if thresholds were exceeded or if arrhythmias were found, for treatment adjustments or reassurance. Data on emergency care utilisation, hospitalisation and patient-reported outcome measures are used to assess quality of life and self-management.
    RESULTS: 129 symptomatic CHD patients were invited to participate, 55 participated. Reasons for refusing consent included too time consuming to participate in research (30) and to monitor vital signs (14). At baseline 22 patients were in New York Heart Association class ≥ II heart failure, 43 patients had palpitations or documented arrhythmias, and 8 had hypertension. Mean follow-up was 3.0 months, one patient dropped out, and adherence was 97%.
    CONCLUSION: The first results indicate that this program is feasible with high adherence.
    Keywords:  adult congenital heart disease; arrhythmia; e-Health; heart failure; m-Health
    DOI:  https://doi.org/10.1007/s12471-018-1201-6
  9. Int J Cardiol. 2018 Oct 22. pii: S0167-5273(18)35722-X. [Epub ahead of print]
    Predicting acquired cardiovascular disease in adults with congenital heart disease is risky business.
    Lui GK, Bhatt AB.
      
    DOI:  https://doi.org/10.1016/j.ijcard.2018.10.061
  10. Congenit Heart Dis. 2018 Nov 28.
    Dysrhythmias in patients with a complete atrioventricular septal defect: From surgery to early adulthood.
    Houck CA, Evertz R, Teuwen CP, Roos-Hesselink JW, Kammeraad JAE, Duijnhouwer AL, de Groot NMS, Bogers AJJC.
      OBJECTIVE: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD.STUDY DESIGN: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients' medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias.
    RESULTS: Median age at repair was 6 months (interquartile range 3-10) and median age at last follow-up was 24 years (interquartile range 21-28). SND occurred after a median of 17 years (interquartile range 11-19) after repair in 23% of patients, requiring pacemaker implantation in two patients (12%). Regular supraventricular tachycardia was observed in three patients (4%). Atrial fibrillation and ventricular tachyarrhythmias were not observed. Twenty-seven patients (36%) had first-degree AVB, which was self-limiting in 16 (59%) and persistent in 10 (37%) patients. One patient developed third-degree AVB 7 days after left atrioventricular valve replacement. Spontaneous type II second-degree AVB occurred in a 28-year-old patient. Both patients underwent pacemaker implantation.
    CONCLUSIONS: Clinically significant dysrhythmias were uncommon in young adult patients after cAVSD repair. However, three patients required pacemaker implantation for either progression of SND or spontaneous type II second-degree AVB. Longer follow-up should point out whether dysrhythmias will progress or become more prevalent with increasing age.
    Keywords:  adult congenital heart disease; atrial tachyarrhythmias; atrioventricular conduction block; complete atrioventricular septal defect; sinus node dysfunction
    DOI:  https://doi.org/10.1111/chd.12724
  11. Cardiol Young. 2018 Nov 28. 1-3
    Success in a Fontan pregnancy: how important is ventricular function?
    Ordonez MV, Trinder J, Curtis SL.
      The Fontan operation is a palliative surgical procedure for patients whose hearts cannot support a biventricular circulation. The haemodynamic changes that occur in pregnancy are particularly challenging for Fontan patients and the outcomes are variable. We present a case where fetal outcome was particularly poor despite a lack of high risk features pre-pregnancy.
    Keywords:  Fontan circulation; cardiac output; fetal outcomes; pregnancy
    DOI:  https://doi.org/10.1017/S1047951118001865
  12. Artif Organs. 2018 Nov 28.
    Global outreach to improve the provision of cardiopulmonary bypass for patients with congenital heart disease.
    Matte GS.
      
    DOI:  https://doi.org/10.1111/aor.13374
  13. J Fam Nurs. 2018 Nov 27. 1074840718809710
    Struggling and Overcoming Daily Life Barriers Among Children With Congenital Heart Disease and Their Parents in the West Bank, Palestine.
    Elissa K, Sparud-Lundin C, Axelsson ÅB, Khatib S, Bratt EL.
      Advances in early diagnosis, treatment, and postoperative care have resulted in increased survival rates among children with congenital heart disease (CHD). Research focus has shifted from survival to long-term follow-up, well-being, daily life experiences, and psychosocial consequences. This study explored the everyday experiences of children with CHD and of their parents living in the Palestinian West Bank. Interviews with nine children aged 8 to 18 years with CHD and nine parents were analyzed using content analysis. The overall theme that emerged was facing and managing challenges, consisting of four themes: sociocultural burden and finding comfort, physical and external limitations, self-perception and concerns about not standing out, and limitations in access to health care due to the political situation. To provide optimum care for children with CHD and their parents, health care providers and policy makers must understand the negative consequences associated with sociocultural conditions and beliefs about chronic illness.
    Keywords:  Palestinian; adolescent; child; congenital heart disease; parents; qualitative
    DOI:  https://doi.org/10.1177/1074840718809710
  14. J Pediatr Nurs. 2018 Nov - Dec;43:pii: S0882-5963(17)30630-9. [Epub ahead of print]43 1-8
    A Novel Nesting Protocol to Decrease Readmission and Increase Patient Satisfaction Following Congenital Heart Surgery.
    Wright TE.
      BACKGROUND: Pediatric patients post-cardiac surgery have complex care needs requiring extensive discharge education and skill competency by caregivers to transition from the hospital environment to a medical home. The purpose of this quality improvement project was to implement a nesting protocol in the cardiovascular intensive care unit (CVICU) to improve discharge teaching and care coordination, with a goal to reduce readmission rates and increase caregiver satisfaction and understanding.METHODS: A nesting protocol was created to provide clear and consistent guidelines to the multidisciplinary team. Pre- and post-intervention data was collected from caregiver satisfaction surveys, using a Likert scale, to determine understanding of nesting and feeling of preparedness upon discharge. In 2016 and 2017, retrospective chart reviews were performed to evaluate readmission data. SQUIRE 2.0 guidelines were utilized when writing this article (Ogrinc et al., 2015).
    RESULTS: Caregivers reported an increase in satisfaction and understanding of the nesting process post-intervention with an increase of 4.48%. Readmission rates did not improve from 2016 to 2017. However, only 6 months of 2017 were reviewed.
    CONCLUSIONS: Readmission is a significant problem for children with complex CHD. Post-discharge care requires caregivers to understand the medical care that their children require. Pre-intervention data revealed deficiencies in understanding regarding care regimens, infection control, and nutrition, which correlated with the most frequent causes for readmission among this population. The protocol developed addressed multiple issues concerning discharge readiness.
    Keywords:  Congenital heart disease; Discharge; Nesting; Rooming-in
    DOI:  https://doi.org/10.1016/j.pedn.2018.07.009
  15. Open Heart. 2018 ;5(2): e000872
    Patient and physician view on patient information and decision-making in congenital aortic and pulmonary valve surgery.
    Etnel JRG, Helbing WA, Roos-Hesselink JW, The R, Bogers AJJC, Takkenberg JJM.
      Background: To assess the current state of patient information and decision-making in congenital aortic and pulmonary valve disease, we conducted a survey among patients, parents and physicians.Methods: A questionnaire was sent by ground mail to 157 adults and 32 parents of children who previously underwent surgery for congenital aortic or pulmonary valve disease at 0-40 years of age between January 2005 and February 2014 at the Erasmus University Medical Center and to all paediatric and adult congenital cardiologists and congenital cardiac surgeons in the Netherlands (n=88).
    Results: 73 patients/parents (39% response rate, 62 adult patients, 11 parents of paediatric patients) and 35 physicians (40% response rate) responded. Median patient age at the time of surgery was 25.7 years. Basic disease-specific knowledge was adequate in 42% of patients/parents and numeracy was sufficient in 47%. Patients/parents reported that they rely heavily on their physicians for information and often experience difficulty in finding reliable information elsewhere. They lack information on psychosocial aspects of disease (29% of respondents) and risks and benefits of treatment options (26%). They feel less involved in decision-making than they would prefer to be (p=0.014). Decisional conflict at the time of surgery was experienced by 31% of patients/parents. If they had to do it again, 72% of patients/parents would want the same treatment. Quality of life is often impaired due to various valve-related anxieties and lifestyle changes. Physicians reported that they are unable to fully inform and sufficiently involve patients, due to limited patient/parent knowledge and understanding (56%) and limited time during consultations (32%). Patients/parents (98%) and physicians (97%) agree that they should have shared roles in decision-making.
    Conclusion: The substantial shortcomings in our current practice of patient information and decision-making underline the need for innovative solutions, such as careful implementation of patient information tools and shared decision-making in the care path.
    Keywords:  cardiac surgery; congenital heart disease; surgery-valve; valvular disease
    DOI:  https://doi.org/10.1136/openhrt-2018-000872
  16. Curr Pediatr Rev. 2018 Nov 30.
    Long Term Outcome Of Infants With Nec.
    Federici S, De Biagi L.
      Necrotizing enterocolitis (NEC) is a significant complication for premature newborns. Infants who survive NEC are at increased risk of having poor long-term physiological and neurodevelopmental growth. The objective of this paper is to give a comprehensive description of the long-term consequences of NEC. Despite the rise in incidence of NEC there is a paucity of data regarding long-term outcomes of these infants that can be divided into two groups. The first group includes gastrointestinal complications that could occur in relation to the bowel disease, the surgical treatment and quality of the residual bowel. These complications are strictures and short bowel syndrome (SBS). Intestinal strictures are a common occurance after recovery from NEC that should be investigated with a contrast study in case of suspicious clinical findings or before reversal ostomy. If the study demonstrates a stricture in a symptomatic patient, resection with anastomosis is needed. SBS is the result of a massive intestinal resection or of a dysfunctional residual bowel and it can occur in a fourth of patients affected by NEC. The second group includes neurodevelopmental impairment and growth. Neurodevelopmental outcomes in patients with NEC have not been widely reported. Infants with NEC are at high risk for adverse neurodevelopmental outcomes whose cause can be multifactorial and linked to perinatal events, severity of disease, surgical treatment and its complications and hospitalization. Understanding the morbidity of NEC with a long-term follow-up would aid neonatologists and pediatric surgeons to make informed decisions in providing care for these patients. Further research on this topic is needed.
    Keywords:  Necrotizing enterocolitis; Strictures; neurodevelopmental impairment ; outcome; short bowel syndrome
    DOI:  https://doi.org/10.2174/1573396315666181130144925
  17. Pediatr Crit Care Med. 2018 Nov 26.
    A Novel Model Demonstrates Variation in Risk-Adjusted Mortality Across Pediatric Cardiac ICUs After Surgery.
    Tabbutt S, Schuette J, Zhang W, Alten J, Donohue J, Gaynor JW, Ghanayem N, Jacobs J, Pasquali SK, Thiagarajan R, Dimick JB, Banerjee M, Cooper D, Gaies M.
      OBJECTIVE: To develop a postoperative mortality case-mix adjustment model to facilitate assessment of cardiac ICU quality of care, and to describe variation in adjusted cardiac ICU mortality across hospitals within the Pediatric Cardiac Critical Care Consortium.DESIGN: Observational analysis.
    SETTING: Multicenter Pediatric Cardiac Critical Care Consortium clinical registry.
    PARTICIPANTS: All surgical cardiac ICU admissions between August 2014 and May 2016. The analysis included 8,543 admissions from 23 dedicated cardiac ICUs.
    INTERVENTIONS: None.
    MEASUREMENTS AND MAIN RESULTS: We developed a novel case-mix adjustment model to measure postoperative cardiac ICU mortality after congenital heart surgery. Multivariable logistic regression was performed to assess preoperative, intraoperative, and immediate postoperative severity of illness variables as candidate predictors. We used generalized estimating equations to account for clustering of patients within hospital and obtain robust SEs. Bootstrap resampling (1,000 samples) was used to derive bias-corrected 95% CIs around each predictor and validate the model. The final model was used to calculate expected mortality at each hospital. We calculated a standardized mortality ratio (observed-to-expected mortality) for each hospital and derived 95% CIs around the standardized mortality ratio estimate. Hospital standardized mortality ratio was considered a statistically significant outlier if the 95% CI did not include 1. Significant preoperative predictors of mortality in the final model included age, chromosomal abnormality/syndrome, previous cardiac surgeries, preoperative mechanical ventilation, and surgical complexity. Significant early postoperative risk factors included open sternum, mechanical ventilation, maximum vasoactive inotropic score, and extracorporeal membrane oxygenation. The model demonstrated excellent discrimination (C statistic, 0.92) and adequate calibration. Comparison across Pediatric Cardiac Critical Care Consortium hospitals revealed five-fold difference in standardized mortality ratio (0.4-1.9). Two hospitals had significantly better-than-expected and two had significantly worse-than-expected mortality.
    CONCLUSIONS: For the first time, we have demonstrated that variation in mortality as a quality metric exists across dedicated cardiac ICUs. These findings can guide efforts to reduce mortality after cardiac surgery.
    DOI:  https://doi.org/10.1097/PCC.0000000000001776
  18. Pediatrics. 2018 Nov 27. pii: e20180482. [Epub ahead of print]
    When Parents Have Misunderstandings About the Risks and Benefits of Palliative Surgery.
    Robins B, Booser A, Lantos JD.
      When a child needs surgery, both the surgeon and the anesthesiologist must obtain informed consent from the parents. In theory, each specialist obtains permission for their respective portion of the procedure, with the anesthesiologist only obtaining informed consent for the administration of anesthesia and management in the operating room and recovery room. However, he or she may occasionally realize that the parents have misunderstandings about what the surgery and perioperative course entail. In such cases, he or she must decide whether their role is only to discuss the issues related to anesthesia care or whether he or she should also clarify the range of expected outcomes and the postoperative course after surgery. We present a case in which such a dilemma arose and on which we sought experts in anesthesia and ethics to comment.
    DOI:  https://doi.org/10.1542/peds.2018-0482
  19. Circulation. 2018 Nov 06. 138(19): 2116-2118
    Risk Factors and Biomarkers of Poor Outcomes.
    Tretter JT, Redington AN.
      
    Keywords:  Editorials; tetralogy of Fallot
    DOI:  https://doi.org/10.1161/CIRCULATIONAHA.118.037000
  20. Arq Bras Cardiol. 2018 Nov;pii: S0066-782X2018001700674. [Epub ahead of print]111(5): 674-675
    Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study.
    Soares AM.
      
    DOI:  https://doi.org/10.5935/abc.20180203
  21. Eur J Cardiothorac Surg. 2018 Nov 27.
    Augmentation of the pulmonary arteries at or prior to the Fontan procedure is not associated with worse long-term outcomes: a propensity-matched analysis from the Australia-New Zealand Fontan Registry.
    Shearer L, Justo RN, Marathe SP, Betts K, Venugopal P, Winlaw DS, Bullock A, Robertson T, Gentles TL, Celermajer D, d'Udekem Y, Alphonso N.
      OBJECTIVES: Long-term data on the impact of pulmonary artery (PA) augmentation in patients who underwent the Fontan procedure are lacking. The aim of this study was to examine whether surgical or transcatheter PA augmentation at or prior to the Fontan procedure adversely affects the outcomes.METHODS: Data of 1436 patients from the Australia-New Zealand Fontan Registry (1975-2015) were analysed. Primary end point was death or Fontan failure. Cox regression with propensity score matching was used to determine risk or benefit conferred by PA augmentation.
    RESULTS: Forty-eight (3.3%) patients underwent PA augmentation following cavopulmonary shunt (surgical n = 14, balloon dilatation n = 16 and stent n = 18) and 105 (7.3%) patients underwent PA augmentation at the time of the Fontan procedure (surgical n = 104, stent n = 1). Median follow-up was 6.4 years with 10 deaths (6.5%) in the augmentation group and 10.5 years with 95 deaths (7.4%) in the non-augmentation group. The unadjusted Kaplan-Meier, log-rank test and Cox regression analysis demonstrated no significant difference in both end points between the 2 groups [death: hazard ratio (HR) 1.35, 95% confidence interval (CI) 0.70-2.60; P = 0.37 death or failure: HR 1.39, 95% CI 0.83-2.34; P = 0.21]. The propensity score matching yielded 131 matched pairs, with adequate balance for all covariates (the median residual bias = 0.05). The subsequent Cox regression demonstrated no significant difference in the risks of death (HR 1.30, 95% CI 0.49-3.41; P = 0.60) and death or Fontan failure (HR 0.92, 95% CI 0.46-1.85; P = 0.82).
    CONCLUSIONS: PA augmentation prior to or at the Fontan procedure does not confer worse long-term outcomes.
    DOI:  https://doi.org/10.1093/ejcts/ezy376
  22. Cardiol Young. 2018 Nov 26. 1-6
    The current practice and care of paediatric patients post cardiac catheterisation.
    Brown L, Hinsley K, Hurtig M, Porter CL, Connor JA.
      BACKGROUND: Literature is lacking to guide standardised care and assessment practices for paediatric patients post cardiac catheterisation. In response to this gap, we sought to describe the current state of practice in cardiology programmes performing paediatric cardiac catheterisations procedures in the United States of America.Materials and methodsA web-based survey was distributed to the Congenital Cardiovascular Interventional Study Consortium Listserv, with representation from 113 identified institutions. A 36-question survey, including fixed-choice and open-ended questions, was developed and piloted for reliability and validity before distribution. Data were summarised descriptively with count and frequency or median and range.RESULTS: Of the 113 identified institutions, 52% (n=59) responded to the survey. Manual pressure is used to achieve haemostasis by 94.9% of the respondents. Pressure dressings are used by a majority of the facilities and the length of time for bed rest is variable, with the majority using 6 hours for arterial access and 4 hours for venous access. Predominantly, respondents use the time of haemostasis as the start time of bed rest while a third of respondents reported using the time the sheath was removed.
    CONCLUSION: In this study, variation in a number of post catheterisation care and assessment practices for paediatric patients was noted across cardiology programmes. Information from this assessment identifies key opportunities to collaborate in developing standardised practices for the care and assessment of the paediatric patients post catheterisation.
    Keywords:  CHD; paediatric cardiac catheterisation; standardised care; state of practice assessment
    DOI:  https://doi.org/10.1017/S104795111800197X
  23. J Pediatr Nurs. 2018 Nov - Dec;43:pii: S0882-5963(18)30016-2. [Epub ahead of print]43 9-15
    Post-Operative Pain and Comfort in Children After Heart Surgery: A Comparison of Nurses and Families Pre-operative Expectations.
    Harvey KA, Kovalesky A.
      PURPOSE: Parents' and nurses' expectations about pain control and comfort in children after heart surgery were explored to contribute to evidence-based family-centered interventions.DESIGN AND METHODS: 20 nurses and 23 parents from a tertiary pediatric center in the Pacific Northwest, were interviewed about their expectations of children's pain control and comfort experience in the hospital after heart surgery. In this descriptive study, data were collected from semi-structured recall interviews and analyzed using content analysis.
    RESULTS: Most parents expected their child be medicated at a level of not feeling any pain. Many expected their child to remain in a heavily sedated state after the surgery. A few parents did not know what to expect. In contrast, nurses expected children to have controlled pain with intermittent discomfort, yet, tolerating recovery activities.
    CONCLUSIONS: Although both parents and nurses expect to partner in the comfort care of the child, there is variation on the expectations around the nurse-parent relationship and the operational definition of pain management and comfort.
    PRACTICE IMPLICATIONS: Awareness of parents' expectations about pediatric post-operative comfort present an opportunity for the development of interventions aimed to enhance alignment of nurse and family strategies for children after heart surgery. Pre-operative preparation for families specific to post-operative recovery and pain management of children hospitalized for heart surgery is needed.
    Keywords:  Pain management; Parent: nurse: expectation; Pediatric cardiac surgery
    DOI:  https://doi.org/10.1016/j.pedn.2018.07.014
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