bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒02‒17
fifteen papers selected by
Richard James
University of Pennsylvania


  1. J Thorac Cardiovasc Surg. 2019 Jan 11. pii: S0022-5223(19)30001-7. [Epub ahead of print]
    Jegatheeswaran A, Jacobs ML, Caldarone CA, Kirshbom PM, Williams WG, Blackstone EH, DeCampli WM, Duncan KF, Lambert LM, Walters HL, Tchervenkov CI, McCrindle BW.
      OBJECTIVES: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.METHODS: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis.
    RESULTS: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution.
    CONCLUSIONS: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.
    Keywords:  CHD; CHD interrupted arch; congenital heart disease; database; outcomes; quality of life; statistics; statistics regression analysis
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.11.152
  2. Cardiovasc Diagn Ther. 2018 Dec;8(6): 754-764
    Hörer J.
      Today, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to valve surgery, which comprises more than half of all operations in ACHD and in treatment of end-stage CHD, which still yields high mortality and morbidity.
    Keywords:  Heart defect congenital; adult; surgery
    DOI:  https://doi.org/10.21037/cdt.2018.10.06
  3. Cardiovasc Diagn Ther. 2018 Dec;8(6): 814-819
    Singh S, Desai R, Fong HK, Sadolikar A, Samani S, Goyal H.
      With a great interest, we read the article by Neidenbach et al. on non-cardiac comorbidities in German adults with congenital heart disease (ACHD). ACHD always bear an increased risk of developing concomitant non-cardiac comorbidities and complications and impose a great healthcare burden. Limited large-scale data from the United States (US) on this focus incited us to write this brief report. Gilboa et al. estimated nearly 2.4 million people living with CHD (1.4 million adults, 1 million children) in the US in 2010. To have a better nationwide prospect of the current scenario, we looked at the extra-cardiac comorbidities among ACHD patients hospitalized in the US using the National Inpatient Sample database (NIS) for years 2013-2014. The burden of extracardiac comorbidities among the NIS cohort in the US was diverse as compared to the German outpatient ACHD cohort. Our study reports a higher burden of endocrinological, hematological, metabolic, pulmonary, psychiatric, renal and rheumatological comorbidities as compared to the German cohort. However, the burden of gastrointestinal and hepatological comorbidities was higher in the German outpatient cohort. In addition, ACHD patients with non-cardiac comorbidities were older except for those suffering from the psychiatric illnesses as compared to ACHD hospitalizations without comorbidities. It is imperative for the clinicians to understand the non-cardiac complications which a patient might encounter during a lifetime, and which could further complicate the management of ACHD and increases the risk of mortality.
    Keywords:  Adult congenital heart disease (ACHD); National Inpatient Sample (NIS); comorbidities and complications; extra-cardiac; healthcare burden; mean age of hospitalizations; non-cardiac
    DOI:  https://doi.org/10.21037/cdt.2018.09.12
  4. Cardiovasc Diagn Ther. 2018 Dec;8(6): 705-715
    Neidenbach R, Niwa K, Oto O, Oechslin E, Aboulhosn J, Celermajer D, Schelling J, Pieper L, Sanftenberg L, Oberhoffer R, de Haan F, Weyand M, Achenbach S, Schlensak C, Lossnitzer D, Nagdyman N, von Kodolitsch Y, Kallfelz HC, Pittrow D, Bauer UMM, Ewert P, Meinertz T, Kaemmerer H.
      Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.
    Keywords:  Congenital heart defect (CHD); endocarditis; heart failure; primary health care; pulmonary hypertension
    DOI:  https://doi.org/10.21037/cdt.2018.10.15
  5. Sci Rep. 2019 Feb 14. 9(1): 2034
    Gabbert DD, Hart C, Jerosch-Herold M, Wegner P, Ravesh MS, Voges I, Kristo I, Bulushi AAL, Scheewe J, Kheradvar A, Kramer HH, Rickers C.
      The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. e., pulsatility). The present study, for the first time, quantified respiratory and cardiac components of the venous flow in the inferior vena cava (IVC) of 14 Fontan patients and 11 normal controls using a novel approach ("physio-matrix"). We found that in contrast to the normal controls, respiration in Fontan patients had a significant effect on venous flow pulsatility, and the ratio of respiration-dependent to the cardiac-dependent pulsatility was positively associated with the retrograde flow. Nevertheless, the main driving force of net IVC flow was the heart beat and not respiration. The separate analysis of the effects of respiration and heart beat provides new insights into the abnormal venous return patterns that may be responsible for adverse effects on liver and bowel of the patients with Fontan circulation.
    DOI:  https://doi.org/10.1038/s41598-019-38848-5
  6. Cardiovasc Diagn Ther. 2018 Dec;8(6): 716-724
    Neidenbach R, Niwa K, Oto O, Oechslin E, Aboulhosn J, Celermajer D, Schelling J, Pieper L, Sanftenberg L, Oberhoffer R, de Haan F, Weyand M, Achenbach S, Schlensak C, Lossnitzer D, Nagdyman N, von Kodolitsch Y, Kallfelz HC, Pittrow D, Bauer UMM, Ewert P, Meinertz T, Kaemmerer H.
      Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group.
    Keywords:  Congenital heart defect (CHD); aortopathy; endocarditis; heart failure; prevention; primary health care; pulmonary hypertension
    DOI:  https://doi.org/10.21037/cdt.2018.10.16
  7. Paediatr Anaesth. 2019 Feb 13.
    Navaratnam M, Maeda K, Hollander SA.
      Pediatric ventricular assist devices (VADs) are evolving as a standard therapy for end stage heart failure in children. Major recent developments include the increased use of continuous flow (CF) devices in children and increased experience with congenital heart disease (CHD) and outpatient management. In the current and future era anesthesiologists will encounter more children presenting for VAD implantation, subsequent procedures and heart transplantation. Successful peri-operative management requires an understanding of the interaction between the patient's physiology and the device and a framework to troubleshoot problems. This review focuses on CF devices, VAD support for CHD and peri-operative management of pulsatile and CF devices in the pediatric population. This article is protected by copyright. All rights reserved.
    DOI:  https://doi.org/10.1111/pan.13609
  8. Cardiovasc Diagn Ther. 2018 Dec;8(6): 799-804
    Andonian C, Beckmann J, Biber S, Ewert P, Freilinger S, Kaemmerer H, Oberhoffer R, Pieper L, Neidenbach RC.
      Due to technological and medical advances the population of adults with congenital heart disease (ACHD) is growing. Worldwide, congenital heart disease (CHD) affects 1.35-1.5 million children each year and more than 90% reach adulthood. Given the heterogeneity of CHD, survivors are faced with not only complex medical but also psychological challenges which may manifest in mental health problems, such as depression, anxiety and posttraumatic stress disorder. This review focuses on the emotional dimension of CHD. More precisely, it summarizes the present state of research on the prevalence of emotional distress in ACHD. Theoretical models provide a framework for possible explanations of mental health issues in ACHD. Additionally, the review examines the relation between psychological processes and overall health considering the latest scientific findings on coping with chronic illness (illness identity). There is still insufficient knowledge on the psychosocial treatment of mental health issues in the growing population of ACHD. This review suggests a vital need to further investigate the psychological situation of ACHD on a large-scale basis in order to establish a holistic treatment approach to accommodate the patients' special needs.
    Keywords:  Congenital heart disease (CHD); adults with congenital heart disease (ACHD); psychological situation, mental health, chronic disease, prevention
    DOI:  https://doi.org/10.21037/cdt.2018.12.06
  9. Cardiovasc Diagn Ther. 2018 Dec;8(6): 771-779
    Havasi K, Ambrus N, Kalapos A, Forster T, Nemes A.
      Treatment of congenital heart diseases has significantly advanced over the last few decades. Due to the continuously increasing survival rate, there are more and more adult patients with congenital heart diseases and these patients present at the adult cardiologist from the paediatric cardiology care. The aim of the present review is to demonstrate the role of echocardiography in some significant congenital heart diseases.
    Keywords:  Echocardiography; adult; congenital heart disease
    DOI:  https://doi.org/10.21037/cdt.2018.09.11
  10. J Perinat Neonatal Nurs. 2019 Feb 06.
    Berry SN, Colorafi K.
      Congenital anomalies are the leading cause of infant death in the United States, accounting for 20% of the annual infant mortality. Advancements in ultrasound diagnostic technology allow practitioners to diagnose fetal anomalies as early as 11 weeks' gestational age, 75% of which are detected in low-risk pregnancies. Communicating a fetal anomaly diagnosis to parents and initiating perinatal end-of-life discussions are difficult for healthcare providers and parents alike. Furthermore, poorly communicated diagnoses have had long-term negative impacts on perinatal grief intensity, which manifested into lifelong symptoms of adverse psychosocial outcomes such as anxiety, depression, substance abuse, and suicidal ideation. The purpose of this integrative review is to examine the impact of communication in discussing an intrauterine diagnosis of a fetal congenital anomaly on perinatal grief. An integrative review was conducted following the distinct 5-stage process of problem identification, searching the literature, evaluating data, analyzing, and presenting findings. A systematic literature review using the PICO model (Population, Intervention, Comparison, Outcome) and structured after the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) framework was completed between November 2017 and May 2018 in the PubMed, CINAHL, and PsycINFO databases. Of the 931 article results, 15 satisfied search criteria. Emerging themes included parental need for appropriate time to assimilate and understand the diagnosis, freedom to explore options and alternatives, and clinicians with expert communication skills. The initial conversation communicating the diagnosis of a congenital anomaly impacts expectant parents for the remainder of their lives. Healthcare professionals are in a unique position to either positively or negatively impact the intensity of perinatal grieving reactions. The application of empathetic, sensitive communication may offer solace and promote healing surrounding perinatal end-of-life discussions.
    DOI:  https://doi.org/10.1097/JPN.0000000000000390
  11. J Neurosurg Anesthesiol. 2019 Jan;31(1): 140-143
    Lee KM, Diacovo TG, Calderon J, Byrne MW, Ing C.
      The Pediatric Anesthesia and Neurodevelopment Assessment (PANDA) study team held its biennial symposium in April 2018 to discuss issues on anesthetic neurotoxicity in the developing brain. One of the sessions invited speakers with different areas of expertise to discuss "Outcomes Research in Vulnerable Pediatric Populations." The vulnerable populations included neonates, children with congenital heart disease, children from low socioeconomic status, and children with incarcerated parents. Each speaker presented some of the ongoing research efforts in these groups as well as the challenges encountered in studying them.
    DOI:  https://doi.org/10.1097/ANA.0000000000000544
  12. Can J Cardiol. 2019 Feb;pii: S0828-282X(18)31309-6. [Epub ahead of print]35(2): 217-220
    Rathgeber SL, Harris KC.
      The growing awareness of Fontan-associated liver disease (FALD) in adults with Fontan physiology has provided the impetus to better understand the natural history of FALD and develop a reliable noninvasive method to diagnose and monitor liver health in this population. Biochemical and imaging tests have been investigated to determine their association with liver pathology. The congestive hepatopathy that develops after the Fontan procedure has made interpreting these tests challenging. We have reviewed and summarized the current understanding and ongoing challenges with respect noninvasive measures of liver health in Fontan patients including biochemical tests, elastography, hepatic ultrasound, cross-sectional imaging, and hemodynamics and how they relate to liver pathology. It has been demonstrated from biopsy data that liver disease is universal and progressive in Fontan patients. Traditional biochemical tests, elastography, and imaging methods are often abnormal in Fontan patients but do not reliably indicate significant liver pathology. Although a reliable means for surveillance of FALD remains elusive, this continues to be an active area of investigation, with promising recent developments. Therapeutic options for FALD are limited, with cardiac transplant as the only option that can stabilize FALD pathology and improve symptomatology. Given the limited therapeutic options and the prevalence of liver disease in Fontan patients, there is a compelling case for early routine surveillance of liver health and promotion of global liver health.
    DOI:  https://doi.org/10.1016/j.cjca.2018.11.019
  13. Neth Heart J. 2019 Feb 13.
    Treskes RW, Koole M, Kauw D, Winter MM, Monteiro M, Dohmen D, Abu-Hanna A, Schijven MP, Mulder BJ, Bouma BJ, Schuuring MJ.
      PURPOSE: Mobile health (mHealth) could improve the outcome of grown-up patients with congenital heart disease (GUCH) and reduce their emergency care utilisation. Inappropriate use of mHealth, however, can lead to data overload for professionals and unnecessary data collection for patients, increasing the burden for both. We aimed to determine the clinical characteristics of patients with high emergency care utilisation and to test whether these patients were willing to start using mHealth.METHODS: Clinical characteristics and emergency care utilisation of consecutive GUCH patients who visited one of the two participating cardiologists at the outpatient clinic of the Academic Medical Centre in Amsterdam were studied retrospectively. All patients were approached to fill in an mHealth questionnaire. A frequency of three or more emergency visits in 5 years was defined as high emergency care utilisation.
    RESULTS: In total, 202 consecutive GUCH patients who visited one of the two participating cardiologists were studied. Median age was 41 years, 47% were male, and 51% were symptomatic. In the previous 5 years, 134 emergency visits were identified. Of all patients, 8% had high emergency care utilisation. High emergency care utilisation was associated with patients being symptomatic, using antiarrhythmic drugs or diuretics. In total, 75% of all patients with high emergency care utilisation were willing to start using mHealth.
    CONCLUSION: GUCH patients who are symptomatic, those on antiarrhythmic drug therapy and those on diuretics are suitable candidates for enrolment in future mHealth initiatives because of both high care utilisation and high motivation to start using mHealth.
    Keywords:  Congenital heart disease; Delivery of healthcare; GUCH; Mobile health; Resource utilisation; eHealth
    DOI:  https://doi.org/10.1007/s12471-019-1237-2