bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒08‒18
seven papers selected by
Richard James
University of Pennsylvania


  1. J Mol Cell Cardiol. 2019 Aug 13. pii: S0022-2828(19)30166-X. [Epub ahead of print]
    Saraf A, Book W, Nelson TJ, Xu C.
      Hypoplastic Left Heart Syndrome (HLHS) is a complex Congenital Heart Disease (CHD) that was almost universally fatal until the advent of the Norwood operation in 1981. Children with HLHS who largely succumbed to the disease within the first year of life, are now surviving to adulthood. However, this survival is associated with multiple comorbidities and HLHS infants have a higher mortality rate as compared to other non-HLHS single ventricle patients. In this review we (a) discuss current clinical challenges associated in the care of HLHS patients, (b) explore the use of systems biology in understanding the molecular framework of this disease, (c) evaluate induced pluripotent stem cells as a translational model to understand molecular mechanisms and manipulate them to improve outcomes, and (d) investigate cell therapy, gene therapy, and tissue engineering as a potential tool to regenerate hypoplastic cardiac structures and improve outcomes.
    Keywords:  Congenital heart disease; Hypoplastic left heart syndrome
    DOI:  https://doi.org/10.1016/j.yjmcc.2019.08.005
  2. J Pediatr. 2019 Aug 08. pii: S0022-3476(19)30811-X. [Epub ahead of print]
    Gakenheimer-Smith L, Glotzbach K, Ou Z, Presson AP, Puchalski M, Jones C, Lambert L, Delgado-Corcoran C, Eckhauser A, Miller T.
      OBJECTIVE: To evaluate the association between neonatal neurobehavioral state and oral feeding outcomes following congenital heart disease (CHD) surgery.STUDY DESIGN: This single center retrospective cohort study described neonates undergoing cardiac surgery evaluated perioperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS). We compared NNNS attention scores, which evaluates neonates' ability to orient and fixate on stimuli, with the feeding outcomes percentage of feeds taken orally at discharge and time to reach full oral feeds using regression analyses. Models were constructed for both preoperative and postoperative NNNS evaluations.
    RESULTS: Between August 2015 and October 2017, 124 neonates underwent 89 preoperative and 97 postoperative NNNS evaluations. In multivariable Cox regression, higher preoperative NNNS attention scores were associated with a shorter time to achieve full oral feeds (hazard ratio 1.4; 95% CI 1.0‒2.0; P = .047). This relationship was not seen for post-operative NNNS attention scores or percentage of oral feeds at discharge. Depending on the model, younger age at surgery, increased ventilator days, increased length of stay, and single or 2-ventricle anatomy with aortic arch obstruction were associated with lower percentage of oral feeds at discharge and/or delay in full oral feeds.
    CONCLUSIONS: Higher neonatal attention before cardiac surgery is associated with improved feeding outcomes. Prospective assessment of neonatal neurobehavioral state may be a novel approach to predict and target interventions to improve feeding outcomes in CHD. Future studies should examine the impact of intrinsic neurodevelopmental delay vs environmental adaptation on the neurobehavioral state of neonates with CHD.
    Keywords:  congenital heart disease; neurodevelopment; oral feeding
    DOI:  https://doi.org/10.1016/j.jpeds.2019.06.047
  3. Ann Thorac Surg. 2019 Aug 10. pii: S0003-4975(19)31167-1. [Epub ahead of print]
    Riggs KW, Zafar F, Radzi Y, Yu PJ, Bryant R, Morales DL.
      BACKGROUND: Early mortality has plagued the otherwise good outcomes in adult congenital heart disease (ACHD) transplantation, but perio-operative care is improving. We sought to identify risk factors for 1-year mortality currently and examine the results of patients without those risk factors compared to non-ACHD (nACHD) patients.METHODS: The UNOS database was searched for all adult (>17 yrs) heart transplant recipients from 2000-2018. They were divided into early and late era. A multivariate analysis identified risk factors for 1-year mortality in the late era. Patients without these risk factors were compared to the nACHD community by Kaplan-Meier analysis.
    RESULTS: 495 ACHD patients were identified from 2000-2008 and 666 from 2009-2018. The recent era had better 1-year (p-value<0.001) and overall (p-value=0.003) survival than 2000-2008. ACHD patients were different from the nACHD population in age (37 yrs vs 57 yrs), BMI>25 kg/m2 (45% vs 66%), incidence of renal dysfunction (23% vs 28%) and liver dysfunction (29% vs 23%), sensitization (38% vs 29%), and ischemic times (3.5 hrs vs 3.1 hrs). Multivariate analysis identified BMI>25 kg/m2 (HR: 1.79), renal dysfunction (HR: 1.85), liver dysfunction (HR: 1.69), and longer ischemic time (HR: 1.46) as risk factors for early mortality. Patients with only 1 of the first 3 categorical risk factors had 1-year survival comparable to nACHD patients.
    CONCLUSIONS: ACHD patients have better early and long-term outcomes in the recent era. If only 1 of 3 pre-transplant risk factors for early mortality is present, patients have survival equal to nACHD patients and perhaps better long-term survival.
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.06.067
  4. J Pain Symptom Manage. 2019 Aug 09. pii: S0885-3924(19)30439-7. [Epub ahead of print]
    Ludmir J, Steiner JM, Wong HN, Kloosterboer A, Leong J, Aslakson RA.
      CONTEXT: Little is known about advance care planning (ACP) and palliative care needs among adults with congenital heart disease (ACHD).OBJECTIVES: To identify and synthesize studies concerning palliative care among ACHD patients.
    METHODS: We searched five electronic databases (PubMed, Embase, SCOPUS, Web of Science, and CINAHL) using the keywords palliative care and congenital heart disease. Inclusion criteria were adults (age >18) with congenital heart disease and publications in English through March 3, 2019.
    RESULTS: Our search yielded 2872 studies, and after removal of duplicates, we screened 2319 abstracts and identified 7 for inclusion. Study findings were grouped into three domains: ACP, symptomatology, and End-of-Life care. Among the 5 cross-sectional studies, only 1-28% of ACHD patients recalled participating in ACP discussions with their doctors but 69-78% reported a strong interest and desire to participate in ACP. In one study, 46% (n=67) of patients had elevated anxiety symptoms (Hospital Anxiety and Depression Scale (HADS-A) ≥ 8) and 11% (n=15) had elevated depressive symptoms (HADS-A ≥ 8). ACHD patients who had a documented goals of care conversation prior to cardiac decompensation had a lower incidence of resuscitation and aggressive treatments at end-of-life (12% (n=3) vs 100% (n=12), p<0.001).
    CONCLUSION: While few ACHD patients complete advance directives, our findings support that many ACHD patients recognize the value of initiating end-of-life and goals of care conversations early on in the course of illness. Future studies investigating communication and implementation strategies of ACP as well as the symptom experience of patients with ACHD are needed.
    Keywords:  Advance care planning; adult congenital heart disease; palliative care
    DOI:  https://doi.org/10.1016/j.jpainsymman.2019.07.025
  5. Ann Thorac Surg. 2019 Aug 09. pii: S0003-4975(19)31161-0. [Epub ahead of print]
    Sower CT, Romano JC, Yu S, Lowery R, Pasquali SK, Zampi JD.
      BACKGROUND: While overall outcomes have improved for single ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients.METHODS: This single-center nested case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity <34 weeks, birth weight <2.5 kg, restrictive/intact atrial septum, ≥moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extra-cardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight <2 kg. Early and mid-term outcomes were compared in the matched hybrid versus Norwood groups.
    RESULTS: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved thirty-day survival in hybrid patients (91% versus 66%, p<0.01), one-year survival was similar between the hybrid and Norwood groups (46% versus 48%, p=0.9). No hybrid patients required dialysis or extracorporeal membrane oxygenation following stage I palliation as compared to 19% and 22% of Norwood patients, respectively (both p<0.01). Hybrid patients, however, required more unplanned reinterventions (43% versus 21%, p=0.02).
    CONCLUSIONS: There remains significant morbidity and mortality among high-risk single ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved mid-term outcomes at our center.
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.06.061
  6. Semin Thorac Cardiovasc Surg. 2019 Aug 09. pii: S1043-0679(19)30253-9. [Epub ahead of print]
    Gellings JA, Johnson WK, Ghanayem NS, Mitchell M, Tweddell J, Hoffman G, Hraska V, Kuhn EM, Woods RK.
      Difficulty weaning from cardiopulmonary bypass (CPB) or the need to return to CPB (collectively D-CPB) may occur after the Norwood procedure. We sought to evaluate the relationship between D-CBP and survival. This was a retrospective chart review of all patients undergoing a Norwood procedure at our institution during the interval 2005-2017. Primary outcome was survival for the Norwood procedure. Secondary outcomes included various measures of morbidity. Successful wean from CBP (S-CPB) was defined as no need to return to full flow CPB during the initial definitive wean or after separation from CPB; otherwise the classification was difficulty with wean (D-CBP). Successful rescue in the D-CPB group was defined as not requiring extracorporeal life support (ECLS) either in the operating room or within the first three postoperative days. Of the 196 patients in the cohort, 49 were D-CPB. Survival for S-CPB was 92.5% (136/147) vs 71.4%% (35/49) for D-CPB (p = 0.001). Major morbidity occurred in 29.9% (44/147) in S-CPB vs 69.4 % (34/49) in D-CPB (p < 0.001). With multivariable analysis, D-CPB was significantly associated with mortality (OR=8.09; CI 2.72 - 24.05; p <0.001). Successful rescue occurred in 30 of 49 patients in the D-CPB group and demonstrated survival similar to the S-CPB group. In the Norwood patient, D-CPB is an important intraoperative event and prognostic factor for mortality and morbidity. Successful rescue appears to ameliorate the impact of D-CPB on survival.
    Keywords:  Mortality; Norwood; Single ventricle
    DOI:  https://doi.org/10.1053/j.semtcvs.2019.08.005
  7. Int J Cardiol. 2019 Aug 02. pii: S0167-5273(19)33166-3. [Epub ahead of print]
    Menachem JN, Opotowsky AR.
      
    Keywords:  Adult congenital heart disease; Decision making; Epidemiology; Heart failure; Neprilysin/antagonists & inhibitors; Renin-angiotensin system/drug effects
    DOI:  https://doi.org/10.1016/j.ijcard.2019.07.100