bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒01‒26
thirteen papers selected by
Richard James
University of Pennsylvania


  1. Int J Cardiol. 2020 Jan 09. pii: S0167-5273(19)32608-7. [Epub ahead of print]
    Saraf A, De Staercke C, Everitt I, Haouzi A, Ko YA, Jennings S, Kim JH, Rodriguez FH, Kalogeropoulos AP, Quyyumi A, Book W.
      BACKGROUND: As the population of adults with congenital heart disease (CHD) grows, cardiologists continue to encounter patients with complex anatomies that challenge the standard treatment of care. Single ventricle Fontan palliated patients are the most complex within CHD, with a high morbidity and mortality burden. Factors driving this early demise are largely unknown.METHODS AND RESULTS: We analyzed biomarker expression in 44 stable Fontan outpatients (29.2 ± 10.7 years, 68.2% female) seen in the outpatient Emory Adult Congenital Heart Center and compared them to 32 age, gender and race matched controls. In comparison to controls, Fontan patients had elevated levels of multiple cytokines within the inflammatory pathway including Tumor Necrosis Factor-α (TNF-α) (p < 0.001), Interleukin-6 (IL-6) (p < 0.011), Growth Derived Factor-15 (GDF-15) (p < 0.0001), β2-macroglobulin, (p = 0.0006), stem cell mobilization: Stromal Derived Factor-1∝ (SDF-1α) (p = 0.006), extracellular matrix turnover: Collagen IV (p < 0.0001), neurohormonal activation: Renin (p < 0.0001), renal dysfunction: Cystatin C (p < 0.0001) and Urokinase Receptor (uPAR) (p = 0.022), cardiac injury: Troponin-I (p < 0.0004) and metabolism: Adiponectin (p = 0.0037). Within 1 year of enrollment 50% of Fontan patients had hospitalizations, arrhythmias or worsening hepatic function. GDF-15 was significantly increased in Fontan patients with clinical events (p < 0.0001). In addition, GDF-15 moderately correlated with longer duration of Fontan (r = 0.55, p = 0.01) and was elevated in atriopulmonary (AP) Fontan circulation. Finally, in a multivariate model, VEGF-D and Collagen IV levels were found to be associated with a change in MELDXI, a marker of liver dysfunction.
    CONCLUSION: Multiple clinical and molecular biomarkers are upregulated in Fontan patients, suggesting a state of chronic systemic dysregulation.
    Keywords:  Biomarkers; Fontan; GDF-15; Liver function
    DOI:  https://doi.org/10.1016/j.ijcard.2020.01.012
  2. Int J Cardiol. 2020 Jan 17. pii: S0167-5273(19)32630-0. [Epub ahead of print]
    Skogby S, Moons P, Johansson B, Sunnegårdh J, Christersson C, Nagy E, Winberg P, Hanséus K, Trzebiatowska-Krzynska A, Fadl S, Fernlund E, Kazamia K, Rydberg A, Zühlke L, Goossens E, Bratt EL.
      BACKGROUND: Providing continuous follow-up care to patients with congenital heart disease (CHD) remains a challenge in many settings. Previous studies highlight that patients with CHD experience discontinuation of follow-up care, but mainly describe a single-centre perspective, neglecting inter-institutional variations. Hospital-related factors above and beyond patient-related factors are believed to affect continuity of care. The present multicentre study therefore investigated (i) proportion of "no follow-up care"; (ii) transfer destinations after leaving paediatric cardiology; (iii) variation in proportions of no follow-up between centres; (iv) the association between no follow-up and outpatient volumes, and (v) its relationship with staffing resources at outpatient clinics.METHODS: An observational, multicentre study was conducted in seven university hospitals. In total, 654 adolescents with CHD, born between 1991 and 1993, with paediatric outpatient visit at age 14-18 years were included. Transfer status was determined 5 years after the intended transfer to adult care (23y), based on medical files, self-reports and registries.
    RESULTS: Overall, 89.7% of patients were receiving adult follow-up care after transfer; 6.6% had no follow-up; and 3.7% were untraceable. Among patients in follow-up care, only one remained in paediatric care and the majority received specialist adult CHD care. Significant variability in proportions of no follow-up were identified across centres. Higher outpatient volumes at paediatric outpatient clinics were associated with better continued follow-up care after transfer (OR = 1.061; 95% CI = 1.001 - 1.124). Medical staffing resources were not found predictive.
    CONCLUSION: Our findings support the theory of hospital-related factors influencing continuity of care, above and beyond patient-related characteristics.
    Keywords:  Adolescent; Continuity of patient care; Heart defects, congenital; Patient transfer; Transition to adult care; Young adult
    DOI:  https://doi.org/10.1016/j.ijcard.2020.01.016
  3. MCN Am J Matern Child Nurs. 2020 Jan 23.
    Lisanti AJ, Buoni A, Steigerwalt M, Daly M, McNelis S, Spatz DL.
      BACKGROUND: Kangaroo care (KC), or skin-to-skin care, occurs when an infant is dressed in a diaper and held to a parent's bare chest. This form of holding has been shown to have many benefits for hospitalized infants and has been shown to be a safe and feasible intervention to support infants with congenital heart disease. Despite known benefits, KC was not implemented routinely and consistently in our cardiac center for infants with congenital heart disease. The purpose of this project was to support use of KC as a nursing intervention for hospitalized infants with congenital heart disease and their parents.METHODS: A KC quality improvement committee formed to develop strategies to increase frequency of KC, including the creation of a new nursing policy and procedure on KC for infants, adaptation of the electronic health record to facilitate KC documentation, provide education, and promote translation of KC into practice through the cardiac center's first Kangaroo-A-Thon.
    RESULTS: Twenty-six nurses initiated KC 43 times with 14 patients over the 8-week period for the Kangaroo-A-Thon. No adverse events were reported as a result of infants being held by their parents in KC.
    CONCLUSION: Our local initiative provided preliminary evidence that KC can be safely integrated into standard care for hospitalized infants with congenital heart disease. Formal standards and procedures, along with creative initiatives such as a Kangaroo-A-Thon, can be a first step toward fostering the translation of KC into practice.
    DOI:  https://doi.org/10.1097/NMC.0000000000000612
  4. Indian J Pediatr. 2020 Jan 22.
    Arvind B, Saxena A.
      Congenital heart defects (CHD) are the most common form of birth anomalies. About one-fifth of these are critical requiring very early intervention, the classical examples being transposition of great arteries or obstructive total anomalous pulmonary venous connection. On the other hand, relatively milder and simpler lesions, such as small ventricular septal defects or mild pulmonary stenosis, may either not need intervention at all or intervened as and when deemed necessary. Apart from the cardiovascular effects, some CHDs can significantly affect the physical growth and neurodevelopment of the child. Each type of CHD has unique hemodynamic effects and the intervention is, by and large, timed based on the severity and natural history of each cardiac lesion. Some lesions have a "limited" time window beyond which they may become unsuitable for any intervention. Hence it is critical to intervene at the appropriate time so as to prevent the untoward effects of CHDs and at the same time to avoid unnecessary interventions.
    Keywords:  Acyanotic congenital heart defects; Congenital heart defects; Cyanotic congenital heart defects
    DOI:  https://doi.org/10.1007/s12098-019-03133-w
  5. BMJ Open. 2020 Jan 23. 10(1): e034588
    Flocco SF, Caruso R, Barello S, Nania T, Simeone S, Dellafiore F.
      OBJECTIVE: This study explored the lived experiences of women with congenital heart disease (CHD) during pregnancy and early motherhood.DESIGN: Qualitative study using semistructured interviews. Data were analysed according to interpretative phenomenological analysis.
    SETTING: San Donato Milanese, Italy.
    PARTICIPANTS: 12 adult women during pregnancy or early motherhood.
    RESULTS: Three main themes emerged from the analysis that were labelled as follows: 'Being a woman with CHD'; 'Being a mother with CHD'; and 'Don't be alone'. Mothers described both positive and negative feelings about their pregnancies and transitions from childless women to mothers with CHD. They needed supportive care to improve the management of their health during pregnancy and early motherhood.
    CONCLUSION: This study explored the lived experiences of women with CHD during pregnancy and early motherhood. The emerged themes represent an initial framework for implementing theory-grounded educational and supportive strategies that improve self-care, engagement and quality of life for women with CHD. Furthermore, the study's results provide guidance for operationalising the described experiences into items and domains for future cross-national surveys.
    Keywords:  cardiology; congenital heart disease; qualitative research
    DOI:  https://doi.org/10.1136/bmjopen-2019-034588
  6. Pediatr Int. 2020 Jan 19.
    Yoshida T, Hiraiwa A, Ibuki K, Makimoto M, Inomata S, Tamura K, Kawasaki Y, Ozawa S, Hirono K, Ichida F.
      BACKGROUND: Both congenital heart disease (CHD) and very low birth weight (VLBW) infants are very high risk for neurodevelopmental delay. We investigated neurological development at 3 years among pediatric patients with CHD after surgical intervention, very low birth weight (VLBW), and healthy controls.METHODS: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birth weights of <1000 and 1000-1499 g. Neurodevelopmental outcomes at 3 years were evaluated using the Bayley Scales of Infant and Toddler Development, Third Edition.
    RESULTS: Compared with healthy controls, a significant deficit in the language, cognition, and motor skills scores were observed in infants with CHD and VLBW. Infants with a single ventricle exhibited significantly low scores in language and gross motor skills. No statistically significant difference was observed between the birth weight groups of <1000 and 1000-1499 g.
    CONCLUSION: Neurodevelopmental outcomes for infants with both CHD and VLBW showed impairment. Notably, neurodevelopmental delays in infants with a single ventricle were remarkable. Thus, because infants with both CHD and VLBW are at high risk for neurodevelopmental disorders, periodic developmental screenings and support are warranted for these children. (215 words).
    Keywords:  Bayley Scales; Congenital heart disease; Neurodevelopmental outcomes; Very low birth weight infant
    DOI:  https://doi.org/10.1111/ped.14160
  7. J Card Surg. 2020 Jan 23.
    Hernandez GA, Lemor A, Clark D, Blumer V, Burstein D, Byrne R, Fowler R, Frischhertz B, Sandhaus E, Schlendorf K, Zalawadiya S, Lindenfeld J, Menachem JN.
      INTRODUCTION: Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait-list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients.METHODS: Using the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We then identified those with specific ICD-9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle. Multivariate regression models were created to adjust for potential confounders.
    RESULTS: A total of 93 Fontan patients underwent heart transplant during the study time (0.5% of all heart transplants). Compared to non-Fontan heart transplantations, Fontan patients were younger, with a higher incidence of liver disease and coagulopathy. Fontan patients receiving heart transplant had higher mortality during transplant hospitalization compared to non-Fontan patients (26.3% vs 5.3% OR, 18.10, CI, 5.06-65.0 P < .001). Extracorporeal membrane oxygenator (ECMO) usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (P = .016) and 5.32 (P = .015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts.
    CONCLUSION: Adults with Fontan palliation undergoing heart transplantation have exceptionally high inpatient mortality, which is nearly five times that of non-Fontan heart transplant recipients, perhaps related to a delayed referral, surgical complexity, and coexistent, underrecognized liver failure.
    Keywords:  Fontan procedure; congenital heart defect; heart failure; heart transplantation
    DOI:  https://doi.org/10.1111/jocs.14430
  8. Int J Cardiol. 2020 Jan 10. pii: S0167-5273(19)34728-X. [Epub ahead of print]
    Khuong JN, Wilson TG, Grigg LE, Bullock A, Celermajer D, Disney P, Wijesekera VA, Hornung T, Zannino D, Iyengar AJ, d'Udekem Y.
      BACKGROUND: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown.METHODS: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV.
    RESULTS: Serum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR <90 mL/min/1.72m2. Renal dysfunction was present in 67/328 (20%) and 3/328 (1%) patients had an eGFR <60 mL/min/1.72m2. The 10-year survival and 10-year freedom from death and transplantation were the same, 96% (95% CI: 0.9-1) for those with renal dysfunction, and 89% (0.83-0.95; p = 0.1) and 87% (95% CI: 0.81-0.94; p = 0.05) for patients without dysfunction. The 10-year freedom from failure were also similar, 83% (95% CI: 0.70-0.97) for those without renal dysfunction vs 80% (95% CI: 0.74-0.89; p = 0.84). There was no change in mean eGFR for the renal dysfunction group over a mean of 8 ± 5.5 years.
    CONCLUSION: By the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated.
    Keywords:  Congenital heart disease; Fontan procedure; Kidney; Renal function
    DOI:  https://doi.org/10.1016/j.ijcard.2020.01.014
  9. Lancet Child Adolesc Health. 2020 Jan 21. pii: S2352-4642(19)30429-8. [Epub ahead of print]
    Rossano JW.
      
    DOI:  https://doi.org/10.1016/S2352-4642(19)30429-8
  10. Pediatr Cardiol. 2020 Jan 24.
    Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S.
      We developed a Fontan surveillance catheterization protocol as part of routine assessment of stable patients 10 years after Fontan completion. The surveillance catherization includes hemodynamic assessment with inhaled nitric oxide, angiography, liver biopsy, and transcatheter intervention if indicated. We aimed to describe hemodynamic and liver biopsy findings, response to pulmonary vasoreactivity testing, rates of transcatheter intervention, and changes in medical therapy following surveillance catheterization in stable Fontan patients. A single-center retrospective review of Fontan patients undergoing surveillance catheterization between November 2014 and May 2019 was performed. Liver biopsies were independently scored by two pathologists. Sixty-three patients underwent surveillance catheterization (mean age 14.6 ± 3.0 years). The mean Fontan pressure was 11.8 ± 2.1 mmHg. The mean cardiac index was 2.9 ± 0.6 L/min/m2. In the 51 patients who underwent pulmonary vasoreactivity testing, there was a significant decrease in median pulmonary vascular resistance (1.8 [range 0.8-4.1] vs 1.4 [range 0.7-3.0] Wood units × m2; p < 0.001). The mean cardiac index increased (3.0 ± 0.6 vs 3.2 ± 0.7 L/min/m2, p = 0.009). The Fontan pressure did not change significantly. Fifty-seven patients underwent liver biopsy, and all but one showed fibrosis. Nineteen patients (33.3%) demonstrated bridging fibrosis or cirrhosis. Twenty-five patients underwent 34 transcatheter interventions. Pulmonary artery or Fontan stent placement was performed in 19 patients. Phosphodiesterase type 5 inhibitors were initiated in nine patients following surveillance catheterization. Routine surveillance catheterization with liver biopsy in adolescent Fontan patients reveals information that can guide interventional and medical management. Further long-term follow-up and assessment are indicated to assess the benefit of these interventions.
    Keywords:  Cardiac catheterization and angiography; Complex congenital heart disease; Fontan physiology
    DOI:  https://doi.org/10.1007/s00246-020-02293-3
  11. Lancet Child Adolesc Health. 2020 Jan 21. pii: S2352-4642(19)30402-X. [Epub ahead of print]
    .
      BACKGROUND: Previous congenital heart disease estimates came from few data sources, were geographically narrow, and did not evaluate congenital heart disease throughout the life course. Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study 2017, this study aimed to provide comprehensive estimates of congenital heart disease mortality, prevalence, and disability by age for 195 countries and territories from 1990 to 2017.METHODS: Mortality estimates were generated for aggregate congenital heart disease and non-fatal estimates for five subcategories (single ventricle and single ventricle pathway congenital heart anomalies; severe congenital heart anomalies excluding single ventricle heart defects; critical malformations of great vessels, congenital valvular heart disease, and patent ductus arteriosus; ventricular septal defect and atrial septal defect; and other congenital heart anomalies), for 1990 through to 2017. All available global data were systematically analysed to generate congenital heart disease mortality estimates (using Cause of Death Ensemble modelling) and prevalence estimates (DisMod-MR 2·1). Systematic literature reviews of all types of congenital anomalies to capture information on prevalence, associated mortality, and long-term health outcomes on congenital heart disease informed subsequent disability estimates.
    FINDINGS: Congenital heart disease caused 261 247 deaths (95% uncertainty interval 216 567-308 159) globally in 2017, a 34·5% decline from 1990, with 180 624 deaths (146 825-214 178) being among infants (aged <1 years). Congenital heart disease mortality rates declined with increasing Socio-demographic Index (SDI); most deaths occurred in countries in the low and low-middle SDI quintiles. The prevalence rates of congenital heart disease at birth changed little temporally or by SDI, resulting in 11 998 283 (10 958 658-13 123 888) people living with congenital heart disease globally, an 18·7% increase from 1990 to 2017, and causing a total of 589 479 (287 200-973 359) years lived with disability.
    INTERPRETATION: Congenital heart disease is a large, rapidly emerging global problem in child health. Without the ability to substantially alter the prevalence of congenital heart disease, interventions and resources must be used to improve survival and quality of life. Our findings highlight the large global inequities in congenital heart disease and can serve as a starting point for policy changes to improve screening, treatment, and data collection.
    FUNDING: Bill & Melinda Gates Foundation.
    DOI:  https://doi.org/10.1016/S2352-4642(19)30402-X