bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒07‒29
nine papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2018 Jul 19. pii: S0003-4975(18)31003-8. [Epub ahead of print]
    Lopez L, Houyel L, Colan SD, Anderson RH, Béland MJ, Aiello VD, Bailliard F, Cohen MS, Jacobs JP, Kurosawa H, Sanders SP, Walters HL, Weinberg PM, Boris JR, Cook AC, Crucean A, Everett AD, Gaynor JW, Giroud J, Guleserian KJ, Hughes ML, Juraszek AL, Krogmann ON, Maruszewski BJ, St Louis JD, Seslar SP, Spicer DE, Srivastava S, Stellin G, Tchervenkov CI, Wang L, Franklin RCG.
      The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past nine years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases (ICD-11). The scheme to categorize a ventricular septal defect utilizes both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.06.020
  2. Prog Cardiovasc Dis. 2018 Jul 18. pii: S0033-0620(18)30150-6. [Epub ahead of print]
    Cotts TB.
      Advances in the care of infants and children with congenital heart disease (CHD) have resulted in significantly improved survival of this population into adulthood. Although the majority of patients with CHD have undergone surgical intervention or transcatheter intervention, patients are not "cured" and almost all require lifelong cardiac care. In many parts of the world, robust systems of care have been developed for infants and children with CHD. Although there has been progress in the development of programs for the care of adults with CHD, the time of transition and transfer of care remains a time of high risk for lapses in care and becoming lost to follow-up. Transition is a process of education and preparation for young patients to assume their own care as they enter adulthood, while transfer is the actual event of a patient moving their care from a pediatric to an adult environment. The purpose of this review is to summarize what is known of best practices related to the transition and transfer of patients with CHD to adult services and to provide a practical approach to the sometimes daunting task of preparing patients for the transfer of care.
    Keywords:  Adult Congenital Heart Disease;Transition; Congenital Heart Disease
    DOI:  https://doi.org/10.1016/j.pcad.2018.07.016
  3. Curr Cardiol Rep. 2018 Jul 21. 20(9): 76
    Gerardin JF, Earing MG.
      PURPOSE OF REVIEW: Highlight the extracardiac comorbidities that adult congenital heart disease patients and summarize the current recommendations for non-cardiac surgery.RECENT FINDINGS: Adult congenital heart disease patients are living longer, becoming more complex and developing adult comorbidities as they age. These patients have multiorgan involvement including higher prevalence of kidney disease and decreased lung function. Non-cardiac comorbidities can complicate surgery in this patient population. Most patients have non-cardiac surgery in community settings. These settings may increase adverse events during and in the post-operative period. Survival is improved when moderate and complex patients are seen in regional referral centers. Improved awareness of long-term complications of congenital heart disease and extracardiac comorbidities for adult congenital heart disease patients is needed. Appropriate care settings with both congenital heart disease expertise and adult subspecialty care reduces morbidity and mortality in these complex patients.
    Keywords:  Adult congenital heart disease; Extracardiac comorbidities; On-cardiac surgery; Preoperative evaluation
    DOI:  https://doi.org/10.1007/s11886-018-1016-5
  4. Ann Thorac Surg. 2018 Jul 19. pii: S0003-4975(18)31004-X. [Epub ahead of print]
    Hongu H, Yamagishi M, Miyazaki T, Maeda Y, Taniguchi S, Asada S, Fujita S, Yaku H.
      BACKGROUND: Conventional Rastelli, Lecompte, and Nikaidoh operations are accepted as standard techniques for complete transposition of the great arteries (TGA) with left ventricular outflow tract (LVOT) obstruction. However, these operations show serious drawbacks, including postoperative obstruction of both ventricular outflow tracts. We developed the half-turned truncal switch operation (HTTSO) to address these problems.METHODS: Between 2002 and 2017, 14 patients underwent HTTSO. Median age was 1.2 years and median body weight was 8.3 kg. Diagnosis was TGA with pulmonary stenosis in nine cases, TGA-type double-outlet right ventricle in four and TGA with degenerative pulmonary valve after pulmonary arterial banding in one. The coronary artery was Yacoub type A in 13 and type D in one. Four patients had a small right ventricle. Pulmonary-aortic annular diameter ratio ranged from 0.43 to 1.00. The right ventricular outflow tract was augmented using a monocuspid polytetrafluoroethylene valved patch in eight cases. Autologous pulmonary annulus was preserved in six cases.
    RESULTS: Median follow-up was 5.2 years. No early mortality was encountered. Only one patient was lost due to arrhythmia, 11 months after HTTSO. No patients showed coronary insufficiency and no outflow tract obstruction was identified. Aortic regurgitation was within mild degree in 12 cases. Additional mitral valvuloannuloplasty was required in three cases late after HTTSO for moderate-to-severe mitral regurgitation. Risk factors for late death and reoperation were low age and body weight at HTTSO.
    CONCLUSIONS: HTTSO is useful for TGA with LVOT obstruction, ensuring wide, straight ventricular outflow tracts and growth potential.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.06.021
  5. Resuscitation. 2018 Jul 18. pii: S0300-9572(18)30346-0. [Epub ahead of print]
    Sutton RM, Reeder RW, Landis W, Meert KL, Yates AR, Berger JT, Newth CJ, Carcillo JA, McQuillen PS, Harrison RE, Moler FW, Pollack MM, Carpenter TC, Notterman DA, Holubkov R, Dean JM, Nadkarni VM, Berg RA, , .
      AIM: The primary aim of this study was to evaluate the association between chest compression rates and 1) arterial blood pressure and 2) survival outcomes during pediatric in-hospital cardiopulmonary resuscitation (CPR).METHODS: Prospective observational study of children ≥37 weeks gestation and <19 years old who received CPR in an intensive care unit (ICU) as part of the Pediatric Intensive Care Unit Quality of CPR Study (PICqCPR) of the Collaborative Pediatric Critical Care Research Network (CPCCRN). Arterial blood pressure and compression rate were determined from manually extracted arterial line waveform data during the first 10 minutes of CPR. The primary outcome was survival to hospital discharge. Modified Poisson regression models assessed the association between rate categories (80-<100, 100-120 [Guidelines], >120-140, >140) and outcomes.
    RESULTS: Compression rate data were available for 164 patients. More than half (98/164; 60%) were < 1 year old. Return of circulation was achieved in 148/164 (90%); survival to hospital discharge in 77/164 (47%). Percentage of events with average rate within Guidelines was 32.9%. Compared to Guidelines, higher rate categories were associated with lower systolic blood pressures (>120-140, p = 0.010; >140, p = 0.077), but not survival. A rate between 80-<100 per minute was associated with a higher rate of survival to hospital discharge (aRR 1.92, CI95 1.13, 3.29, p = 0.017) and survival with favorable neurological outcome (aRR 2.12, CI95 1.09, 4.13, p = 0.027) compared to Guidelines.
    CONCLUSION: Non-compliance with compression rate Guidelines was common in this multicenter cohort. Among ICU patients, slightly lower rates were associated with improved outcomes compared to Guidelines.
    Keywords:  cardiac arrest; cardiopulmonary resuscitation; intensive care unit; pediatric
    DOI:  https://doi.org/10.1016/j.resuscitation.2018.07.015
  6. Prog Cardiovasc Dis. 2018 Jul 18. pii: S0033-0620(18)30149-X. [Epub ahead of print]
    Fathallah M, Krasuski RA.
      Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.
    Keywords:  Adult congenital heart disease; Diagnosis; Eisenmenger syndrome; Pulmonary arterial hypertension; Therapy
    DOI:  https://doi.org/10.1016/j.pcad.2018.07.017
  7. Pediatr Cardiol. 2018 Jul 21.
    Ackerman T, Geerts A, Van Vlierberghe H, De Backer J, François K.
      We tried to identify structural and functional liver aberrances in a palliated Fontan population and sought to determine useful screening modalities, in order to propose a screening protocol to detect patients at risk. Twenty nine patients, median age 23.7 years (interquartile range (IQR) 20.5-27.2) and median Fontan interval 19.7 years (IQR 4.5-21.4), were prospectively studied with echocardiography, blood analysis (including serum fibrosis scores Forns, APRI and FIB4), liver imaging (ultrasound (US), Doppler), and shear wave elastography to determine liver stiffness (LS). Laboratory tests predominantly showed abnormal values for gamma-glutamyltransferase. Forns index indicated moderate fibrosis in 29% of patients and correlated with Fontan interval (p = 0.034). US liver morphology was deviant in 46% of patients, with surface nodularity in 21% and nodular hyperplasia in 29%. Doppler assessment of flow velocities was within normal ranges for most patients. LS (mean 10.4 ± 3.7 kPa) was elevated in 96% of our population and higher LS values were significantly related to longer Fontan interval (p = 0.018). Adolescent and adult Fontan patients show moderate signs of liver dysfunction. Usefulness of serum parameters and fibrosis scores in post-Fontan screening remains ambiguous. The high percentage of morphologic liver changes in palliated patients supports the use of US in periodic follow-up. LS likely overestimates fibrosis due to liver congestion, arguing for the need of validation through sequential measurements. Screening should minimally encompass US assessment in combination with selective liver fibrosis scores. The role of LS measurement in Fontan follow-up and liver screening needs to be further elucidated.
    Keywords:  Cardiac hepatopathy; Elastography; Fontan circulation; Screening; Serum markers; Univentricular heart
    DOI:  https://doi.org/10.1007/s00246-018-1937-1
  8. Reprod Toxicol. 2018 Jul 18. pii: S0890-6238(18)30071-6. [Epub ahead of print]
    Liu Z, He C, Chen M, Yang S, Li J, Lin Y, Deng Y, Li N, Guo Y, Yu P, Li X.
      OBJECTIVES: This study aimed to analyze the association between fetal lead or aluminum exposure and congenital heart disease (CHD) occurrence as well as to explore the mechanism of oxidative stress in heart development.METHODS: Lead and aluminum concentrations were measured by ICP-MS in umbilical serum. The oxidative stress statuses were analyzed by measuring SOD, GPx and MDA with colorimetric assays.
    RESULTS: Higher concentrations of Al were seen in the CHD groups compared to the controls. The risk of CHD occurrence increased markedly in the highly elevated Al group (aOR 2.08, 95%CI 1.11-3.88). With increasing Al and Pb levels, the activity of SOD decreased, and the level of MDA increased. Significantly decreased activity of SOD and GPx were found in the CHD groups (P < 0.05).
    CONCLUSION: Fetal aluminum exposure may contribute to CHD occurrence. Oxidative stress was related to the concentration of Pb and Al, which may be involved in the occurrence of CHD.
    Keywords:  Birth defects; Congenital heart disease; Developmental toxicity; Metal element; Oxidative stress
    DOI:  https://doi.org/10.1016/j.reprotox.2018.07.081
  9. Semin Thorac Cardiovasc Surg. 2018 Jul 19. pii: S1043-0679(18)30124-2. [Epub ahead of print]
    Lacour-Gayet F, Kandachar P, Al-Bulushi A, Maddali MM.
      Aortic atresia (AA) with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is Type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery (SCA). Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an AP window, an aberrant right SCA or a bilateral PDA. We report a case of AA with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.
    DOI:  https://doi.org/10.1053/j.semtcvs.2018.07.009