bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒09‒06
five papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Clin North Am. 2020 Oct;pii: S0031-3955(20)30068-7. [Epub ahead of print]67(5): 945-962
    Metcalf MK, Rychik J.
      Hypoplastic left heart syndrome (HLHS) is a complex form of congenital heart disease defined by anatomic and functional inadequacy of the left side of the heart with nonviability of the left ventricle to perform systemic perfusion. Lethal if not treated, a strategy for survival currently is well established, with continuing improvement in outcomes over the past 30 years. Prenatal diagnosis, good newborn care, improved surgical skills, specialized postoperative care, and unique strategies for interstage monitoring all have contributed to increasing likelihood of survival. The unique life with a single right ventricle and a Fontan circulation is a focused area of investigation.
    Keywords:  Congenital heart surgery; Fetal echocardiography; Hypoplastic left heart syndrome; Neurocognitive deficits in congenital heart disease; Outcomes for congenital heart disease
    DOI:  https://doi.org/10.1016/j.pcl.2020.06.008
  2. Ann Thorac Surg. 2020 Aug 26. pii: S0003-4975(20)31352-7. [Epub ahead of print]
    Welke KF, Pasquali SK, Lin P, Backer CL, Overman DM, Romano JC, Karamlou T.
      BACKGROUND: Over 150 hospitals perform congenital heart surgery (CHS) in the United States (U.S.). Many hospitals are near to one another and median patient travel distance is 38.5 miles. We began with a theoretical blank slate and used objective methodology guided by population density and volume thresholds to estimate the optimal number and locations of hospitals to provide CHS in the U.S.METHODS: Guided by published data, we estimated the number of CHS operations in the U.S. in to be 32,500 per year. We distributed patients geographically based on population density. Metropolitan Statistical Areas (population centers and surrounding areas with close economic/social ties) were used as potential hospital locations. Patients were assigned to the closest hospital location such that all hospitals had a CHS volume of ≥ 300 operations.
    RESULTS: We estimated 57 hospitals could serve the contiguous U.S. Median theoretical hospital volume following regionalization was 451 operations (IQR 366-648). Median patient travel distance was 35.1 miles. A minority of patients (6396/31895 = 20%) would travel more than 100 miles.
    CONCLUSIONS: Our model suggests the U.S. could be served by approximately 100 fewer CHS hospitals than currently exist. With hospitals optimally placed, patient travel burden would decrease. This model serves as a platform to improve care delivery by regionalization of CHS.
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.06.057
  3. Curr Opin Pediatr. 2020 Aug 29.
    Selamet Tierney ES.
      PURPOSE OF REVIEW: Children and adolescents with congenital heart disease (CHD) often have decreased exercise capacity and muscle mass. Exercise has numerous health benefits and can improve exercise capacity and muscle mass. As such, guidelines recommend 60 min of daily physical activity in the majority of pediatric CHD patients. Despite these known benefits and guidelines, children and adolescents with CHD often do not exercise regularly. The purpose of this review is to summarize the current literature on exercise in this population.RECENT FINDINGS: With recent advancements in the field, clinical focus in pediatric cardiology has expanded from improving mortality to improving long-term outcomes and quality of life. As part of this paradigm shift, there is ongoing research on safety, impact, and effective delivery of exercise to this population with the overarching goal of promoting an active lifestyle.
    SUMMARY: Exercise interventions are generally safe, feasible, and beneficial in children and adolescents with CHD. There is growing evidence demonstrating the benefits and utility of exercise as a prescription rather than restriction in pediatric CHD patients. Novel approaches are needed for effective delivery of exercise in this population. Further studies are warranted for assessment of long-term benefits of exercise in pediatric CHD patients.
    DOI:  https://doi.org/10.1097/MOP.0000000000000942
  4. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020 Jul 28. pii: 1672-7347(2020)07-0812-07. [Epub ahead of print]45(7): 812-818
    Jiang Z, Liu Y, Wu Z, Lu T, Tan L, Hu Y.
      OBJECTIVES: To explore the method and significance of prenatal counseling in cardiac surgery for fetal congenital heart disease (CHD).METHODS: The prenatal counseling should be provided by experienced CHD experts. The preliminary clinical diagnosis based on relevant data was carried out, the prognosis risk for fetal CHD was graded, and the pathophysiological process and potential hazards of the disease were analyzed. The current condition of CHD in the treatment plan, the long-term quality of life, and the special requirements of parturition in place, period and mode were described. A reliable follow-up system of the fetuses was established, the diagnosis after delivery was verified, and surgical treatment was carried out timely.
    RESULTS: From January 2016 to December 2018, 225 parents with fetal CHD received prenatal counseling, including 60 fetuses (26.7%) with simple CHD and 165 (73.3%) with complex CHD, among which 59 cases (98.3%) and 93 cases (56.4%) decided to continue the pregnancy, respectively. During the follow-up, 118 fetuses were born, of which 66 infants received surgical treatment within 6 months after birth, 63 infants (95.5%) recovered and 3 infants (4.5%) died. The rest 52 infants continued to be followed up.
    CONCLUSIONS: The prenatal counseling for fetal CHD can provide the parents a comprehensive medical information about CHD, which is beneficial to making appropriate pregnancy decisions, and can turn the fetuses from unreasonable birth and passive treatment to selective birth and active treatment in CHD.
    Keywords:  cardiac surgery; congenital heart disease; fetus; prenatal counseling
    DOI:  https://doi.org/10.11817/j.issn.1672-7347.2020.190198
  5. Ann Pediatr Cardiol. 2020 Jul-Sep;13(3):13(3): 186-193
    Arunamata A, Tacy TA, Kache S, Mainwaring RD, Ma M, Maeda K, Punn R.
      Objective: To investigate patient-related factors, echocardiographic, and anatomic variables associated with immediate and long-term clinical outcomes after extracardiac Fontan procedure at our institution.Materials and Methods: Retrospective review of preoperative cardiac catheterizations and echocardiograms as well as medical records of all children with hypoplastic left heart syndrome (HLHS) who underwent Fontan between June 2002 and December 2018.
    Results: Seventy-seven patients with HLHS were included (age 4 years [1.5-11.7]). Seventy patients (91%) received a nonfenestrated Fontan and 57 patients (74%) underwent Fontan without cardiopulmonary bypass (CPB). Presence of a Fontan fenestration (P = 0.69) and use of CPB (P = 0.79) did not differ between those with <2 weeks compared to those with ≥2 weeks of chest tube drainage. There were no differences in either pre- or intra-operative hemodynamics between patients who weighed <15 kg compared to those who weighed ≥15 kg at time of surgery; incidence of death, transplant, and transplant listing were similar between weight groups. Inferior vena cava (IVC) diameter z-score did not differ among patients with and without chylous chest tube drainage (P = 0.78), with and without development of protein losing enteropathy (P = 0.23), or death/heart transplant/transplant listing compared to survivors without transplant (P = 0.26).
    Conclusion: In HLHS patients undergoing Fontan, preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes. Performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit. These observations should be considered when deciding optimal timing for Fontan completion.
    Keywords:  Fontan procedure; clinical outcomes; congenital heart disease; hypoplastic left heart syndrome
    DOI:  https://doi.org/10.4103/apc.APC_5_20