bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒08‒02
nine papers selected by
Richard James
University of Pennsylvania


  1. Eur J Prev Cardiol. 2020 Jul 27. 2047487320942869
    Scheffers LE, Berg LEV, Ismailova G, Dulfer K, Takkenberg JJ, Helbing WA.
      BACKGROUND: Patients with a Fontan circulation have a reduced exercise capacity, which is an important prognostic predictor of morbidity and mortality. A way to increase exercise capacity in Fontan patients might be exercise training. This systematic review assesses the effects of exercise training investigated in Fontan patients in order to provide an overview of current insights.DESIGN AND METHODS: Studies evaluating an exercise training intervention in Fontan patients published up to February 2020 were included in this systematic review.
    RESULTS: From 3000 potential studies, 16 studies reported in 22 publications met the inclusion criteria. In total, 264 Fontan patients with mean age range 8.7-31 years, were included. Different training types including inspiratory muscle training, resistance training and aerobic training were investigated. Main outcome measures reported were peak oxygen uptake, cardiac function, lung function, physical activity levels and quality of life. Peak oxygen uptake increased significantly in 56% of the studies after training with an overall mean increase of +1.72 ml/kg/min (+6.3%). None of the studies reported negative outcome measures related to the exercise programme. In four studies an adverse event was reported, most likely unrelated to the training intervention.
    CONCLUSIONS: Exercise training in Fontan patients is most likely safe and has positive effects on exercise capacity, cardiac function and quality of life. Therefore exercise training in Fontan patients should be encouraged. Further studies are required to assess the optimal training type, intensity, duration and long-term effects.
    Keywords:  Fontan procedure; exercise; heart defects congenital; physical endurance; quality of life
    DOI:  https://doi.org/10.1177/2047487320942869
  2. Heart Lung Circ. 2020 Jul 06. pii: S1443-9506(20)30311-5. [Epub ahead of print]
    Rehan R, Kotchetkova I, Cordina R, Celermajer D.
      BACKGROUND: Although advances in congenital heart disease (CHD) management have allowed survival of children with even highly complex CHD lesions well into adult life, the burden of disease (medical, psychological and social) has not been well characterised, for those living to middle age and beyond.METHODS: We assessed 121 consecutive patients from our adult CHD centre, who survived to age 50 years and who had had moderate or complex CHD lesions. Pre-specified groups included those with repaired tetralogy of Fallot (TOF) (n=56), coarctation of the aorta (CoA) (n=34), systemic right ventricle (RV) (n=9), Fontan surgery for "single ventricle" hearts (n=5), those with repaired Ebstein's Anomaly (n=9) and other complex CHD (n=8).
    RESULTS: The overall burden of disease was very substantial. Of the TOF patients, almost half (46%) had required at least one open-heart reoperation and 41% had had a pacemaker or implantable defibrillator; 20% had had a radiofrequency ablation and 32% were anti-arrhythmic therapy. Over 40% had ≥1 admission for heart failure and 9% had had endocarditis. Only 64% were still employed. Of the CoA survivors, 50% had had a second operation (aortic valve and/or descending aortic surgery), 88% were on medications for hypertension and 62% were still employed. In the more complex groups, approximately half had been diagnosed with depression/anxiety and cerebrovascular event, heart failure and/or significant arrhythmias were common.
    CONCLUSIONS: Despite considerable advances, adults with CHD who survive to age 50 years have experienced high levels of physical and mental health complications.
    Keywords:  Adult congenital heart disease; Arrhythmia; Depression; Survival
    DOI:  https://doi.org/10.1016/j.hlc.2020.05.114
  3. Pediatr Cardiol. 2020 Jul 29.
    Anderson BR, Blancha Eckels VL, Crook S, Duchon JM, Kalfa D, Bacha EA, Krishnamurthy G.
      The aims of this study were (1) to describe the additive risk of performing cardiac surgery in neonates born ≤ 2.0 kg, after accounting for the baseline risks of low birth weight, and (2) to describe the additive risk of being born ≤ 2.0 kg in neonates undergoing cardiac surgery. We used a risk difference analysis in a retrospective cohort, 2006-2016. Neonates born ≤ 2.0 kg undergoing congenital heart surgery during initial postnatal admission were included. Data were standardized alternatingly for birth weight and cardiac surgical risk using national population data to estimate the number of deaths expected had they not required cardiac surgery or were they of normal weight. Of 105 neonates ≤ 2 kg, median birth weight was 1.6 kg (IQR 1.3-1.8 kg). Median gestational age was 33 weeks (IQR 31-35 weeks). Observed operative mortality was 14.3%; 0% for neonates ≤ 1.0 kg (CI 0-33.6%), 20.6% for neonates > 1.0-1.5 kg (CI 8.7-37.9%), and 12.9% for neonates > 1.5-2.0 kg (CI 5.7-23.9%). Among neonates ≤ 2.0 kg not undergoing cardiac surgery, expected mortality was 4.8% (CI 1.6-10.8); cardiac surgery increased the risk of mortality 9.5% (CI 1.7-17.4%). Conversely, the expected risk for normal birth weight neonates undergoing cardiac surgery was 5.7% (CI 2.1-12.0%); low birth weight increased the risk of mortality 8.6% (CI 0.5-16.6%). To continue making advancements in cardiac surgery, we must understand that the rate of mortality observed in normal weight infants is not a realistic target and that, despite advances, the risk attributable to the surgery remains higher among low birth weight patients.
    Keywords:  Birth weight; Congenital heart disease; Neonates; Outcomes; Surgery
    DOI:  https://doi.org/10.1007/s00246-020-02420-0
  4. J Pediatr. 2020 Jul 24. pii: S0022-3476(20)30964-1. [Epub ahead of print]
    Shikany AR, Landis BJ, Parrott A, Miller EM, Coyan A, Walters L, Hinton RB, Goldenberg P, Ware SM.
      OBJECTIVE: To investigate the frequency of genetic diagnoses among infants with critical congenital heart disease (CHD) using a comprehensive cardiovascular genetics approach and to identify genotype-phenotype correlations.STUDY DESIGN: A retrospective chart review of patients evaluated by cardiovascular genetics in a pediatric cardiac intensive care unit from 2010 to 2015 was performed. Infants with CHD who were <1 month of age were included. CHD was classified using structured phenotype definitions. Cardiac and non-cardiac phenotypes were tested for associations with abnormal genetic testing using chi-squared and Fisher exact tests.
    RESULTS: Genetic evaluation was completed in 293 infants with CHD, of whom 213 had isolated CHD (iCHD) and 80 had multiple congenital anomalies (MCA). Overall the yield of abnormal genetic testing was 26%. The MCA cohort had a greater yield of genetic testing (39%) than the iCHD cohort (20%) (odds ratio 2.7). Utilizing a non-hierarchical CHD classification and excluding 22q11.2 deletion and common aneuploidies, right ventricular obstructive defects were associated with abnormal genetic testing (P = .0005). Extracardiac features associated with abnormal genetic testing included ear, nose and throat (p=0.003) and brain (p=0.0001) abnormalities. A diagnosis of small for gestational age or intrauterine growth retardation was also associated with abnormal genetic testing (p=0.0061), as was presence of dysmorphic features (p=0.0033, odds ratio 3.5). Nondysmorphic infants with iCHD or MCA had similar frequencies of abnormal genetic testing.
    CONCLUSION: The present study provides evidence to support a comprehensive cardiovascular genetics approach in evaluating infants with critical CHD while also identifying important genotype-phenotype considerations.
    Keywords:  cardiovascular genetics; chromosome microarray; copy number variant; genetic syndrome; genetic testing
    DOI:  https://doi.org/10.1016/j.jpeds.2020.07.065
  5. Med Care Res Rev. 2020 Jul 29. 1077558720945925
    Hardy RY, Keller D, Gurvitz M, McManus B, Varda D, Lindrooth RC.
      Transitions from pediatric to adult care by young adults with chronic conditions are fraught with challenges. Poor transitions lead to discontinuities of care that are avoidable with better communication between providers. We tested whether exposure to providers with sustained patient-sharing relationships resulted in fewer emergent admissions of young adults with congenital heart disease (CHD). Care transitions are particularly important for young adults with CHD. Though it is not possible to avoid planned admissions for scheduled procedures, emergency admissions are avoidable with proper care. We tested whether several different patient-sharing relationship measures influenced emergent admissions and found that compared with less severe CHD patients, those with severe CHD experienced a 4 to 10 percentage point decline in emergent admissions given a 5 percentage point increase in practice-level patient-sharing relationships. These results are consistent with our hypothesis that patient sharing improves communication and continuity of care across providers, especially for severe CHD patients.
    Keywords:  congenital heart disease; patient-sharing relationships; provider networks
    DOI:  https://doi.org/10.1177/1077558720945925
  6. Cardiorenal Med. 2020 Jul 28. 1-9
    Bojan M, Pieroni L, Mirabile C, Froissart M, Bonnet D.
      BACKGROUND: The onset of chronic kidney disease (CKD) is an important prognostic factor in young adults with congenital heart disease (CHD). Although it is likely that CKD is manifest early in CHD patients, the prevalence among adolescents is still unknown. The National Kidney Foundation's Kidney Disease Improving Global Outcomes guidelines 2012 recommend new equations for the estimated glomerular filtration rate (eGFR) and highlight the importance of albuminuria for CKD screening. The objective of the present study was to estimate the prevalence of CKD in CHD adolescents.METHODS: This observational cross-sectional study included 115 patients aged 10-18 years attending the cardiologic outpatient clinic at our institution as a follow-up after cardiac surgery in infancy related to various CHDs. CKD assessment used the CKD criteria 2012, including eGFR equations based on serum creatinine and cystatin C, and measurement of albuminuria.
    RESULTS: No patient had an eGFR <60 mL min-1 1.73 m-2. However, 28.7% of all patients (95% CI 20.7-37.9) had eGFRbetween 60 and 89 mL min-1 1.73 m-2 when estimated by the bedside Schwartz creatinine-based equation,and 17.4% (95% CI 11.2-24.1) had eGFRbetween 60 and 89 mL min-1 1.73 m-2 when estimated by the Zappitelli equation, combining creatinine and cystatin C. Of all patients, 20.0% (95% CI 12.1-26.7) had orthostatic proteinuria, and none had persistent albuminuria.
    CONCLUSIONS: There was no evidence of CKD in the present population aged 10-18 years. The significance of an eGFR between 60 and 90 mL min-1 1.73 m-2 is not concordant for this age range and requires further investigations.
    Keywords:  Cardiac surgery; Chronic kidney disease; Congenital heart disease
    DOI:  https://doi.org/10.1159/000508177
  7. Pediatr Res. 2020 Jul 25.
    Kuhn VA, Carpenter JL, Zurakowski D, Reitz JG, Tague L, Donofrio MT, Murnick J, Axt-Fliedner R, Limperopoulos C, Yerebakan C.
      BACKGROUND: The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass.METHODS: A retrospective analysis of neonates with HLHS or d-TGA undergoing cardiac surgery including cardiopulmonary bypass between 2009 and 2017 was performed. Perioperative risk factors and Andropoulos' Brain Injury Scores from pre- and postoperative brain magnetic resonant images (MRI) were correlated to outcome assessments on patients between 5 and 23 months of age. Neurologic outcome was measured using the Pediatric Stroke Outcome Measure (PSOM) and Pediatric Version of the Glasgow Outcome Scale-Extended (GOS-E).
    RESULTS: Fifty-three neonates met our enrollment criteria (24 HLHS, 29 d-TGA). Mechanical ventilation > 12 days and DHCA > 40 min were associated with worse outcome. MRI measures of brain injuries were not associated with worse outcome by PSOM or GOS-E.
    CONCLUSION: For HLHS and d-TGA patients, duration of mechanical ventilation and DHCA are associated with adverse neurologic outcome. Neonatal brain MRI commonly demonstrates acquired brain injuries, but the clinical impact of these abnormalities are not often seen before 2 years of age.
    IMPACT: Acquired brain injury is common in high-risk neonates with CHD but poor neurological outcome was not predicted by severity of injury or lesion subtype. Longer stay in ICU is associated with postoperative brain injuries on MRI.Total duration of ventilation > 12 days is predictive of adverse neurological outcome scores.DHCA > 40 min is associated with adverse neurological outcome scores.Neurological outcome before 2 years of age is more affected by the clinical course than by cardiac diagnosis.
    DOI:  https://doi.org/10.1038/s41390-020-1085-1
  8. Front Psychol. 2020 ;11 1119
    Journiac J, Vioulac C, Jacob A, Escarnot C, Untas A.
      Background: Studies interested in patients coping with a cardiac illness usually focus on children, teenagers, and adults above the age of 55. Apart from the field of congenital heart diseases, there is a general lack of literature regarding young adult cardiac patients (18-55 years old) who seem to cope with psychosocial issues. Therefore, the objective of this paper was to gather all the research carried out concerning the psychological experiences of young adult cardiac patients. Methods and Results: A comprehensive, systematic review was conducted on quantitative, qualitative, and mixed-method studies in PsycINFO, PubMed, ScienceDirect, and Cochrane Library databases. Out of the 10,747 articles found, 32 were included. While we aimed to include many cardiac diseases, coronary patients dominated the data. Five main themes emerged: emotional states (depression, anxiety, emotional distress, and stress), quality of life (health-related quality of life, physical functioning, and sexuality), adjusting to the medical environment (coping with the disease, health behavior change, financial barriers, and interactions with medical professionals), social life (social support and work), and identity (parenthood, new challenges, and new meanings). The results highlighted that their levels of depression, anxiety, stress, and quality of life were sometimes worse than in the general population and than in older and younger patients coping with a cardiac illness. Social isolation, identity changes, work, and parenthood were the specific challenges that this population had to face. Furthermore, young adult cardiac patients showed worse health behavior profiles than the general population and felt that they lacked information from professionals, especially regarding sexuality. Compared to men, women had worse psychosocial outcomes, especially regarding depression, stress, emotional distress, and quality of life. Conclusions: Young adult cardiac patients are to be considered with their own identity and challenges. They may be in need of specific interventions, some dedicated to women, and better communication is necessary with their families and professional caregivers so as to improve the patient's mental health, quality of life, coping skills, and adherence.
    Keywords:  adults; cardiac; experience; review; young
    DOI:  https://doi.org/10.3389/fpsyg.2020.01119
  9. Heart Surg Forum. 2020 Jun 16. 23(4): E430-E434
    Lee SK, Choi KH.
      BACKGROUND: Although postoperative chylothorax following congenital heart surgery occurs rarely, it is associated with substantial morbidity and mortality. The incidence of postoperative chylothorax has been reported as 2% to 5%. Therefore, we aimed to evaluate the incidence of postoperative chylothorax at our center and compared our results with those of other studies.METHODS: Between January 2009 and December 2018, there were 2,515 congenital heart repair surgeries performed at our center. Thirty-six patients with postoperative chylothorax were enrolled in this study, and their medical records retrospectively were reviewed. We calculated the overall and surgery-specific incidences. We compared the data of the medical management group with those of the surgical management group.
    RESULTS: The overall incidence of postoperative chylothorax was 1.4%. The incidence was highest for patients who underwent vascular ring repair (3/32). Moreover, the incidence was higher for single ventricle-related procedure than bi-ventricle-related procedures (5.6% versus 1.0%, P < .0001). Chylothorax was predominantly found on the left side (20/36). Among these cases, six patients died; three of these six were in the surgical management group.
    CONCLUSIONS: The postoperative chylothorax incidence at our center was comparable to those of other centers. However, a reasonable protocol for postoperative chylothorax management to improve outcomes is necessary.
    DOI:  https://doi.org/10.1532/hsf.3043