bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒08‒16
nine papers selected by
Richard James
University of Pennsylvania


  1. Anesthesiol Clin. 2020 Sep;pii: S1932-2275(20)30029-X. [Epub ahead of print]38(3): 531-543
    Adler AC, Nathan AT.
      Children and adults with congenital heart disease undergoing noncardiac surgery are at higher risk of perioperative adverse events. Patients have significant comorbidities and syndromic associations that increase perioperative risk further. The complexity of congenital heart disease requires a thorough understanding of lesion-specific pathophysiology in order to provide safe care. Comprehensive multidisciplinary planning and the use of skilled and experienced teams achieve the best outcomes. The anesthesiologist is a perioperative physician charged with providing safe anesthesia care, instituting appropriate hemodynamic monitoring, and determining appropriate postoperative disposition on an individual basis.
    Keywords:  Congenital heart disease; Fontan; Noncardiac surgery; Pediatric anesthesiology; Pediatrics; Risk assessment
    DOI:  https://doi.org/10.1016/j.anclin.2020.04.001
  2. Curr Opin Organ Transplant. 2020 Aug 06.
    Hofer RE, Christensen JM, Findlay JY.
      PURPOSE OF REVIEW: The success of the Fontan procedure has led to increased survival of patients born with certain congenital heart disease to the point that new sequlae, as a result of Fontan circulation, are being discovered. Included among these is Fontan-associated liver disease (FALD). The purpose of this review is to present available literature on the perioperative management of the combined heart--liver transplantation (CHLT) in patients with FALD.RECENT FINDINGS: The perioperative management of a combined heart-liver transplant in a patient with Fontan circulation is complex. The patient is at risk for hemodynamic disturbances, significant blood loss, coagulopathies, and metabolic derangements. The maintenance of an appropriate transpulmonary pressure gradient is paramount to success. Postoperative management should be accomplished by a multidisciplinary care team. Limited series have demonstrated good outcomes in patients who have undergone CHLT.
    SUMMARY: The perioperative management of CHLT in patients with FALD is complex and available literature is limited. Future studies are needed to further assess proper perioperative management of patients with FALD who undergo CHLT.
    DOI:  https://doi.org/10.1097/MOT.0000000000000800
  3. Circulation. 2020 Aug 11. 142(6): 591-604
    Emamaullee J, Zaidi AN, Schiano T, Kahn J, Valentino PL, Hofer RE, Taner T, Wald JW, Olthoff KM, Bucuvalas J, Fischer R.
      Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan procedure and are alive today, most of whom are <25 years of age. Several unexpected consequences of the Fontan circulation include Fontan-associated liver disease. Surveillance biopsies have demonstrated that virtually 100% of these patients develop clinically silent fibrosis by adolescence. As they mature, there are increasing reports of combined heart-liver transplantation resulting from advanced liver disease, including bridging fibrosis, cirrhosis, and hepatocellular carcinoma, in this population. In the absence of a transplantation option, these young patients face a poor quality of life and overall survival. Acknowledging that there are no consensus guidelines for diagnosing and monitoring Fontan-associated liver disease or when to consider heart transplantation versus combined heart-liver transplantation in these patients, a multidisciplinary working group reviewed the literature surrounding Fontan-associated liver disease, with a specific focus on considerations for transplantation.
    Keywords:  Fontan procedure; heart transplantation; liver diseases; liver transplantation
    DOI:  https://doi.org/10.1161/CIRCULATIONAHA.120.045597
  4. J Am Heart Assoc. 2020 Aug 11. e014548
    Rudd NA, Ghanayem NS, Hill GD, Lambert LM, Mussatto KA, Nieves JA, Robinson S, Shirali G, Steltzer MM, Uzark K, Pike NA, .
      This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.
    Keywords:  AHA Scientific Statements; cardiovascular abnormalities; caregivers; infant; univentricular heart
    DOI:  https://doi.org/10.1161/JAHA.119.014548
  5. Circulation. 2020 Aug 03.
    Lopez KN, Morris SA, Sexson Tejtel SK, Espaillat A, Salemi JL.
      Background: Congenital heart disease (CHD) accounts for approximately 40% of deaths in United States (US) children with birth defects. Previous US data from 1999-2006 demonstrated an overall decrease in CHD mortality. Our study aimed to assess current trends in US mortality related to CHD from infancy to adulthood over the last 19 years and determine differences by sex and race/ethnicity. Methods: We conducted an analysis of death certificates from 1999-2017 to calculate annual CHD mortality by age at death, race/ethnicity, and sex. Population estimates used as denominators in mortality rate calculations for infants were based on National Center for Health Statistics live birth data. Mortality rates in individuals ≥1 year of age utilized US Census Bureau bridged-race estimates as denominators for population estimates. We characterized temporal trends in all-cause mortality, mortality resulting directly due to and related to CHD by age, race/ethnicity, and sex using joinpoint regression. Results: There were 47.7 million deaths with 1 in 814 deaths due to CHD (n=58,599). While all-cause mortality decreased 16.4% across all ages, mortality resulting from CHD declined 39.4% overall. The mean annual decrease in CHD mortality was 2.6%, with the largest decrease for those age >65 years. The age-adjusted mortality rate decreased from 1.37 to 0.83 per 100,000. Males had higher mortality due to CHD than females throughout the study, although both sexes declined at a similar rate (~40% overall), with a 3-4% annual decrease between 1999 and 2009, followed by a slower annual decrease of 1.4% through 2017. Mortality resulting from CHD significantly declined among all races/ethnicities studied, although disparities in mortality persisted for non-Hispanic blacks versus non-Hispanic whites (mean annual decrease 2.3% versus 2.6%, respectively; age-adjusted mortality rate 1.67 to 1.05 versus 1.35 to 0.80 per 100,000, respectively). Conclusions: While overall US mortality due to CHD has decreased over the last 19 years, disparities in mortality persist for males compared to females and for non-Hispanic blacks compared to non-Hispanic whites. Determining factors that contribute to these disparities such as access to quality care, timely diagnosis, and maintenance of insurance will be important moving into the next decade.
    Keywords:  lifespan
    DOI:  https://doi.org/10.1161/CIRCULATIONAHA.120.046822
  6. J Perinatol. 2020 Aug 13.
    Siefkes H, Kair LR, Saarinen A, Lakshminrusimha S.
      OBJECTIVE: Assess the quality of critical congenital heart disease (CCHD) screening data reports in California, where CCHD screening is not mandatory but reporting is.STUDY DESIGN: Retrospective review of California hospital-level CCHD screening data to evaluate data reliability and adherence to state screening and reporting recommendations. Data were evaluated for internal consistency and compared to two databases.
    RESULTS: Over one-third of hospitals did not submit data. Only 70.7% of the Vital Records live births were reported in CCHD screening data. Only 46% of reporting hospitals submitted data with matching numbers of completed screens and results, and 22% matched their respective live births in a second database.
    CONCLUSION: CCHD data reporting in California is incomplete, which may miss 359 CCHD cases/year from non-reporting. Data inconsistencies may miss additional cases. Mandatory screening, reporting, and improvements in data reliability are urgently needed to inform screening modifications and enhance timely detection and disease surveillance.
    DOI:  https://doi.org/10.1038/s41372-020-00783-z
  7. Ann Thorac Surg. 2020 Aug 11. pii: S0003-4975(20)31308-4. [Epub ahead of print]
    Stephens EH, Talwar AA, Devlin PJ, Eltayeb O, Mongé MC, Tsao S, Backer CL.
      BACKGROUND: There is an active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the non-fenestrated extracardiac Fontan including Fontan conversion.METHODS: Retrospective review was performed of all non-fenestrated extracardiac Fontan and Fontan conversion operations at our institution from 12/1994 to 12/31/2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis for risk factors for adverse outcomes.
    RESULTS: There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 (57%) were extracardiac non-fenestrated Fontan completion operations and 148 (43%) were Fontan conversions. Length-of-stay was 11 (6) days with ventilator duration of 28 (26) hours. Six (3%) of the completion Fontan patients required Fontan takedown at a median time of 2.5 days. On multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length-of-stay. Kaplan Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients.
    CONCLUSIONS: Over the past 24 years our strategy of non-fenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation, with a key risk factor being preoperative ventricular dysfunction.
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.06.019
  8. J Card Surg. 2020 Aug 13.
    Evans WN, Acherman RJ, Mayman GA, Galindo A, Rothman A, Ciccolo ML, Lehoux J, Winn BJ, Yumiaco NS, Restrepo H.
      OBJECTIVE: We hypothesized that a relationship might exist between angiographically demonstrable, post-Fontan venovenous collaterals, and hepatic fibrosis.METHODS: We analyzed data from post-Fontan patients that underwent cardiac catheterization and transvenous-hepatic biopsy procedures between March 2012 and March 2020. From innominate vein angiography, we determined those that either had or lacked venovenous collaterals. Additionally, we examined data from post-Fontan patients that underwent hepatic ultrasound, shear-wave elastography between January 2017 and March 2020.
    RESULTS: We identified 164 patients that met inclusion criteria. Of the 164, 101 (62%) had venovenous collaterals. Of the 101 with collaterals, average total fibrosis score (TFS) was 3.2 and the average rate of fibrosis progression was 0.28 vs an average TFS of 2.1 and an average fibrosis progression rate of 0.22 for those without collaterals (P = .00001 and P = .01, respectively). Of the 101 with collaterals, oxygen saturation was 91% ± 4% vs 93% ± 3% (P = .048) without collaterals. Of the 164, 86 (52%) underwent ultrasound shear-wave elastography. Of the 86 patients undergoing elastography, 50 (58%) were performed in those with collaterals, and 36 (42%) in those without collaterals. For the 50 with collaterals, average elastography values were 13.3 vs 11.2 kPa for the 36 without collaterals (P = .006). We found no statistically significant differences for age at biopsy, Fontan duration, Fontan-type, type of functional univentricle, laboratory, clinical, or hemodynamic values between those with or without collaterals.
    CONCLUSIONS: The presence of angiographically demonstrated venovenous collaterals was associated with statistically, significantly more advanced liver fibrosis than those without collaterals.
    Keywords:  Fontan; hepatic fibrosis; liver biopsy; venovenous collaterals
    DOI:  https://doi.org/10.1111/jocs.14951
  9. Asian Cardiovasc Thorac Ann. 2020 Aug 10. 218492320949655
    Sethasathien S, Silvilairat S, Kraikruan H, Sittiwangkul R, Makonkawkeyoon K, Pongprot Y, Woragidpoonpol S.
      BACKGROUND: As a result of the surgical techniques now being employed, the survival rate in patients after undergoing the Fontan operation has improved. The aims of this study were focused on determining the survival rate and predictors of early mortality.METHODS: In a retrospective cohort study, 117 consecutive patients who underwent the Fontan operation were recruited. Multivariate Cox proportional regression analysis was used to assess the predictors of early mortality, defined as death within 30 days after the Fontan operation.
    RESULTS: The median follow-up time was 6.1 years. The median age at the time of the Fontan operation was 5.7 years. Survival rates in the patients at 5, 10, and 15 years postoperatively were 92%, 87% and 84%, respectively. Using univariate Cox regression analysis, the predictors of early mortality were found to be postoperative mean pulmonary artery pressure ≥23 mm Hg (hazard ratio 26.0), renal failure (hazard ratio 9.5), heterotaxy syndrome (hazard ratio 5.3), and uncorrected moderate or severe atrioventricular valve regurgitation (hazard ratio 9.4). After adjusting for confounding factors using multivariate Cox regression analysis, the predictors of early mortality were found to be postoperative mean pulmonary artery pressure ≥23 mm Hg (hazard ratio 23.2) and uncorrected moderate or severe atrioventricular valve regurgitation (hazard ratio 8.2).
    CONCLUSIONS: Uncorrected moderate or severe atrioventricular valve regurgitation and postoperative mean pulmonary artery pressure ≥23 mm Hg are independent predictors of early mortality after the Fontan operation. Patients with these factors should undergo aggressive management to minimize morbidity and mortality.
    Keywords:  Fontan procedure; congenital; heart defects; mortality; risk factors; survival rate
    DOI:  https://doi.org/10.1177/0218492320949655