bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒03‒17
eleven papers selected by
Richard James
University of Pennsylvania


  1. ASAIO J. 2019 Mar 06.
    Chen S, Rosenthal DN, Murray J, Dykes JC, Almond CS, Yarlagadda VV, Wright G, Navaratnam M, Reinhartz O, Maeda K.
      Ventricular assist device (VAD) support for children with single ventricle (SV) heart disease remains challenging. We performed a single-center retrospective review of SV patients on VAD support and examined survival to transplant using the Kaplan-Meier method. Patients transplanted were compared with those who died on support. Between 2009 and 2017, there were 14 SV patients with 1,112 patient-days of VAD support. Stages of palliation included pre-Glenn (n = 5), Glenn (n = 5), and Fontan (n = 4). Eight patients (57%) were successfully bridged to transplant at a median 107 days. Deaths occurred early (n = 6, median 16 days) and in smaller patients (10.1 vs. 28.3 kg, P = 0.04). All Fontan patients survived to transplant, whereas only 20% of Glenn patients survived to transplant. Adverse events occurred in 79% (n = 11). Five patients met hospital discharge criteria, with two patients (one pre-Glenn, one Glenn) discharged and transplanted after 219 and 174 days of VAD support. All transplanted patients were discharged at a median 21 days posttransplant. SV patients in various stages of palliation can be successfully bridged to transplant with VAD support. With use of intracorporeal continuous-flow devices, longer-term support and hospital discharge are possible.
    DOI:  https://doi.org/10.1097/MAT.0000000000000983
  2. Circ J. 2019 Mar 08.
    Ochiai R, Kato H, Misaki Y, Kaneko M, Ikeda Y, Niwa K, Shiraishi I.
      BACKGROUND: The aim of this study was to determine preferences regarding transfer of patients with congenital heart disease (CHD) attending a children's hospital in Japan and related factors. Methods and Results: We conducted a self-administered questionnaire survey with CHD patients >15 years of age treated at the pediatric cardiology outpatient clinic of a children's hospital. Logistic regression analysis was used to identify factors related to patient preferences regarding the transfer. One hundred and eleven of the 122 patients given a questionnaire provided valid responses (valid response rate, 91.0%). Sixty-six subjects (64.9%) reported "not being told anything specific" by their physicians about the transfer from the children's hospital, and 72 (59.5%) stated that they "wished to continue attending the children's hospital". Visiting outpatient clinic with parents (OR, 11.00; 95% CI: 2.01-60.97), having low uncertainty about continuing to attend the children's hospital (OR, 0.95; 95% CI: 0.92-0.98), and having high uncertainty about leaving the current physician (OR, 1.04; 95% CI: 1.01-1.07) were significantly related to the patient's wish to continue to attend the children's hospital.CONCLUSIONS: There is a need to improve patient education regarding the opportunities for transfer, and to develop a systematic transition program for children's hospitals and aligned specialized adult CHD centers.
    Keywords:  Congenital heart disease; Health-care delivery; Patient preference; Pediatric hospital; Transition to adult care
    DOI:  https://doi.org/10.1253/circj.CJ-18-1144
  3. Birth Defects Res. 2019 Mar 14.
    Crystal MA, Freud LR.
      Advances in fetal echocardiography have allowed for the prenatal diagnosis of congenital heart disease and an understanding of its natural history in utero. This insight has led to the development of fetal cardiac intervention (FCI) for select defects to prevent significant morbidity or mortality postnatally. Fetal aortic valvuloplasty (FAV) may be performed to prevent progression to hypoplastic left heart syndrome, a severe form of congenital heart disease, in utero. The current review focuses on this type of FCI and discusses the history of FAV, the rationale for intervention, candidate selection, procedural technique, and outcomes to date. Finally, the importance of building a multidisciplinary team to perform FCI is addressed.
    Keywords:  congenital heart disease; fetal cardiac intervention; fetal echocardiography; hypoplastic left heart syndrome; prenatal diagnosis
    DOI:  https://doi.org/10.1002/bdr2.1478
  4. PLoS One. 2019 ;14(3): e0213154
    Ni ZH, Lv HT, Ding S, Yao WY.
      AIMS AND OBJECTIVES: To explore the home care experiences of caregivers taking care of CHD children before and after cardiac surgery.BACKGROUND: Despite the prevalence of congenital heart disease (CHD) in childhood, little is known about the experiences and impacts on the children and their caregivers after CHD diagnosis and surgery. Such knowledge is needed for meaningful support.
    DESIGN: A qualitative descriptive study.
    METHODS: Twenty-two caregivers of CHD children undergoing cardiac surgery participated in semi-structured interviews at a University Children's Hospital in China. Data were collected by an experienced and trained interviewer. Qualitative content analysis was chosen to describe the experiences of the caregivers.
    RESULTS: Caregivers of CHD children experienced significant demands. After the children underwent their CHD operations, the caregivers experienced complex psychological feelings and excessive stress impacting upon theirlives. In addition, caregivers constantly adapted their roles with self-fulfillment in caring activities.
    CONCLUSIONS: CHD surgery has a major impact on the emotions and daily lives of children and their caregivers. This study offers a framework for understanding the importance of actively listening to caregivers so coping strategies can be implemented.
    RELEVANCE TO CLINICAL PRACTICE: Theexperiencesdescribed in this study contribute to a better understanding of the needs of caregivers whose children underwent CHD operations. They also provide valuable information to professional medical care staff that developfuture nursing assessments.
    DOI:  https://doi.org/10.1371/journal.pone.0213154
  5. Acta Anaesthesiol Scand. 2019 Mar 14.
    Matsuda M, Takemura H, Yamashita A, Matsuoka Y, Sawa T, Amaya F.
      BACKGROUND: Advances in medical technology have resulted in an increased life expectancy in pediatric patients with congenital heart diseases. Assessment of health-related quality of life is crucial to improving their healthcare status. We aimed to assess post-surgical pain prevalence and its impact on health-related quality of life in pediatric patients who underwent cardiac surgery during childhood.METHODS: This cross-sectional study recruited patients aged 4 years or older who underwent cardiac surgery for congenital heart disease at least 1 year prior, during the age of 0-10 years, and were admitted for post-surgical follow-up at our institute. The prevalence, intensity, and location of pain and health-related quality of life were assessed in an interview. Perioperative information was collected from the patients' medical records. Health-related quality of life was assessed using the Pediatric quality of life inventory 4.0 (PedsQL).
    RESULTS: Pain was reported by 24 (17%) of the 141 participants. One-third of them reported moderate to severe pain that required medical intervention. After adjustment for several confounding factors, multivariable linear regression analysis demonstrated that the presence of pain and the number of surgeries were associated with lower total PedsQL scores.
    CONCLUSIONS: Pain was present in 17% of the patients who underwent cardiac surgery during childhood. Presence of pain had a negative impact on long-term health-related quality of life after pediatric cardiac surgery.
    Keywords:  pediatric cardiac surgery; persistent post-surgical pain; quality of life
    DOI:  https://doi.org/10.1111/aas.13346
  6. J Pediatr Nurs. 2019 Mar 08. pii: S0882-5963(18)30440-8. [Epub ahead of print]46 62-71
    Shackleford JL, Kelley SJ, Spratling R.
      PURPOSE: The purpose of this study is to examine the relationship among the three innate needs of Self-Determination Theory (SDT), self-management of care and adherence to treatment, and the relationship to health-related quality of life (HRQOL) for adolescents with congenital heart disease (CHD).DESIGN AND METHODS: A non-experimental, cross-sectional, correlational design was conducted in a sample of 92 participants with CHD, ages 13 to 18 years. For data analysis, Pearson's correlations were used to explore associations between variables, and the hypotheses were tested using multiple linear regression.
    RESULTS: Demographic and clinical data were collected: 15 ± 1.6 yrs; 59% male; 65% White; 43% mild CHD; 25% severe CHD. After controlling for covariates, regression analyses revealed relatedness (β = 0.64) and competence (β = 0.79) contributed significant variance to HRQOL, R2 = 0.56, p < .001; however, autonomy and self-management of care and treatment adherence did not contribute significant variance to HRQOL.
    CONCLUSIONS: This study found that relatedness and competence were significantly associated with HRQOL in adolescents with CHD; however, autonomy and self-management of care and adherence to treatment were not. These findings demonstrate the importance of further examining relatedness and competence in adolescents with CHD.
    PRACTICE IMPLICATIONS: Nursing care should focus on the improvement of social support systems and interventions to increase self-efficacy for adolescents with CHD.
    Keywords:  Adolescents; Congenital heart disease; Quality of life; Self-determination theory
    DOI:  https://doi.org/10.1016/j.pedn.2019.02.037
  7. Ann Thorac Surg. 2019 Mar 07. pii: S0003-4975(19)30260-7. [Epub ahead of print]
    Jorde UP, Shah AM, Sims DB, Madan S, Siddiqi N, Luke A, Saeed O, Patel SR, Murthy S, Shin J, Oviedo J, Watts S, Jakobleff W, Forest S, Vukelic S, Belov D, Puius Y, Minamoto G, Muggia V, Carlese A, Leung S, Rahmanian M, Leff J, Goldstein D.
      BACKGROUND: Continuous flow-left ventricular assist devices (CF-LVADS) have revolutionized the management of advanced heart failure. Device complications continue to limit survival, but enhanced management strategies have shown promise. This study compared outcomes for HeartmateII recipients before and after implementation of a multidisciplinary continuous support Heart Team (HTMCS) strategy.METHODS: Between 1/2012-12/2016, 124 consecutive patients underwent primary HeartmateII implantation at our institution. In 1/2015, we instituted a HTMCS approach consisting of: (1)daily simultaneous cardiology/cardiac surgery/critical care/pharmacy/coordinator rounds (2)pharmacist-directed anticoagulation (3)pre-discharge speed optimization echocardiogram (4)comprehensive device thrombosis screening and early intervention (5)blood pressure clinic with pulsatility-adjusted goals(6) early post-discharge follow-up and individual long-term coordinator/cardiologist assignment (7)systematic basic/advanced/expert training and credentialing of ancillary in-hospital providers. All patients completed one-year follow-up.
    RESULTS: Demographics for pre-HTMCS (n=71) and HTMCS (n=53) groups including age (55.8±12.1 vs. 52.5±14.1 years, p=NS), percent male (77.5% vs. 71.7%, p=NS) and INTERMACS class 3 (84.5% vs. 83.0%, p=NS) were comparable. One-year survival was 74.6% vs. 100% for pre-HTMCS and HTMCS group, respectively (p=0.0002). One-year survival free of serious adverse events (reoperation to replace device or disabling stroke) was 70.4% vs. 84.9% for pre-HTMCS and HTMCS groups, respectively (p=0.059). Event per patient-year rates for disabling stroke (0.15 vs. 0, p=0.019), gastrointestinal bleeding (0.87 vs. 0.51, p=0.11), and driveline infection (0.24 vs. 0.10, p=0.18) were lower for HTMCS group, while pump thrombosis requiring device exchange was higher (0.09 vs. 0.18, p=0.14).
    CONCLUSIONS: Implementing a comprehensive multidisciplinary approach substantially improved outcomes for CF-LVAD recipients.
    Keywords:  End-stage heart failure; Heart team; Left ventricular assist device; Multidisciplinary management
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.01.063
  8. Children (Basel). 2019 Mar 08. pii: E42. [Epub ahead of print]6(3):
    Rao PS.
      Since the description of surgery for patent ductus arteriosus in late 1930s, an innumerable number of advances have taken place in the management of congenital heart defects (CHDs). In this review the current status of treatment of seven of the most common acyanotic CHDs was reviewed. The discussion included indications for, and timing of, intervention and methods of intervention. The indications are, by and large, determined by the severity of the lesion. Pressure gradients in obstructive lesions and the magnitude of the shunt in left-to-right shunt lesions are used to assess the severity of the lesion. The timing of intervention is different for each lesion and largely dependent upon when the criteria for indications for intervention were met. Appropriate medical management is necessary in most patients. Trans-catheter methods are preferable in some defects while surgery is a better option in some other defects. The currently available medical, trans-catheter, and surgical methods to treat acyanotic CHD are feasible, safe, and effective.
    Keywords:  acyanotic congenital heart defects; aortic stenosis; atrial septal defect; atrioventricular septal defect; coarctation of the aorta; patent ductus arteriosus; pulmonary stenosis; ventricular septal defect
    DOI:  https://doi.org/10.3390/children6030042
  9. Sci Rep. 2019 Mar 11. 9(1): 4137
    Schlatterer SD, Murnick J, Jacobs M, White L, Donofrio MT, Limperopoulos C.
      Congenital heart disease (CHD) is an independent risk factor for brain injury, including stroke, and poor neurodevelopmental outcomes, and placental abnormalities may represent an additional risk factor for brain injury in neonates. The incidence and scope of placental pathology and relationship to fetal brain abnormalities in pregnancies complicated by fetal CHD has not been explored to our knowledge. In order to determine the prevalence of placental pathology findings and whether placental findings are associated with postnatal brain injury in pregnancies complicated by fetal CHD, we reviewed placental pathology reports for 51 pregnancies complicated by CHD and scored available postnatal, pre-operative brain MRI for brain pathology. Overall, 57% of CHD infants had abnormal placental pathology. Pregnancies complicated by CHD with aortic obstruction (AO) were significantly more likely than those with no obstruction to have abnormal placental pathology (79% vs. 44%). There was a trend toward more severe brain lesions amongst patients with brain lesions and placental abnormality (55% moderate/severe) compared to those without placental abnormality (11% moderate/severe). These data suggest that placental abnormalities are common in CHD and may have a compounding effect on brain lesions in this high-risk population.
    DOI:  https://doi.org/10.1038/s41598-019-40894-y
  10. J Thorac Cardiovasc Surg. 2019 Jan 31. pii: S0022-5223(19)30288-0. [Epub ahead of print]
    Bacha EA.
      
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.01.074
  11. BJOG. 2019 Mar 14.
    Keepanasseril A, Pillai AA, Yavanasuriya J, Raj A, Satheesh S, Kundra P.
      OBJECTIVE: To study maternal complications and pregnancy outcome in women with pulmonary hypertension, attending a tertiary centre in south India.STUDY DESIGN: Retrospective Observational study.
    SETTING: Tertiary centre in south India.
    POPULATION: Pregnant women with pulmonary hypertension.
    METHOD: Data regarding demographics, clinical course, medications received and echocardiographic diagnosis regarding pulmonary hypertension, and antenatal care received were collected from the records. Details of labour and delivery and post-partum follow-up were retrieved. We compared the outcome based on the presence or absence of cyanosis and right ventricular systolic pressure levels.
    MAIN OUTCOME MEASURES: Maternal mortality, Occurrence of complications such as heart failure, fetal growth restriction.
    RESULTS: There were 81 pregnancies in 73 women with pulmonary hypertension. Majority of them had pulmonary hypertension secondary to congenital heart disease (80.8%); 17.8% had Eisenmenger's syndrome. Advanced pulmonary artery hypertension (PAH) medication, Sildenafil was administered in 25(31.3%) pregnancies. There were four maternal deaths, of which three had Eisenmenger's syndrome. Heart failure complicated 6.3% and fetal growth restriction 26.3% of pregnancies. Morbidity was significantly increased in women with pulmonary hypertension associated with a cyanotic cardiac lesion or in those with right ventricular systolic pressure >70mmHg.
    CONCLUSION: Despite advances in care, mortality in pregnant women with pulmonary hypertension is a matter of concern, especially in those with Eisenmenger's syndrome. Multidisciplinary team management in tertiary centres and the use of advanced PAH medications even in low to middle-income countries with limited resources, might lead to a reduction in morbidity and mortality related to pulmonary hypertension.
    FUNDING: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. This article is protected by copyright. All rights reserved.
    Keywords:  Eisenmenger's syndrome; Pregnancy; Pulmonary Hypertension
    DOI:  https://doi.org/10.1111/1471-0528.15681