bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒12‒23
seventeen papers selected by
Richard James
University of Pennsylvania


  1. Circulation. 2018 Dec 04. 138(23): 2718-2720
    Oster ME, Knight JH, Suthar D, Amin O, Kochilas LK.
      
    Keywords:  heart defects, congenital; heart transplantation; heart ventricles; hypoplastic left heart syndrome; mortality; survival analysis; thoracic surgery
    DOI:  https://doi.org/10.1161/CIRCULATIONAHA.118.036821
  2. Circ J. 2018 Dec 20.
    Motoki H, Yasukochi S, Takigiku K, Takei K, Okamura T, Kimura K, Minamisawa M, Okada A, Saigusa T, Ebisawa S, Seto T, Shoda M, Okada K, Kuwahara K.
      BACKGROUND: Despite the best efforts of pediatricians, healthcare for adult patients with congenital heart disease (ACHD) has proven challenging because of the increased numbers. This study presents the process of establishing an ACHD care system as a collaborative effort between Shinshu University Hospital and Nagano Children's Hospital.Methods and Results: Establishing an outpatient clinic for transition, a cooperation agreement for in-patient care between the 2 hospitals, and quality management of diagnostic imaging and educational meetings for adult cardiologists were the 3 major challenges. Of the 99 patients who visited the transition clinic in the children's hospital between May 2014 and December 2016, 3 returned to the pediatrician's clinic. Between June 2013 and December 2017, 273 patients visited the ACHD center in Shinshu University Hospital. Until December 2017, mortality and fatal arrhythmia were noted in 3 and 2 cases, respectively. Catheter ablation for arrhythmia was performed in 12 cases, and 4 cases of pregnancy with moderate/severe ACHD or estimated as high risk were managed with healthy livebirths. Surgical interventions for moderate/severe ACHD were performed in collaboration with the children's hospital or Sakakibara Heart Institute.CONCLUSIONS: Patients were successfully transferred to adult cardiology departments. Surgical and nonsurgical interventions for ACHD were provided. Collaboration between adult and pediatric cardiologists assists in the establishment of healthcare systems for ACHD.
    Keywords:  Adult congenital heart disease; Patient care system
    DOI:  https://doi.org/10.1253/circj.CJ-18-0705
  3. Ann Thorac Surg. 2018 Dec 14. pii: S0003-4975(18)31828-9. [Epub ahead of print]
    Bates KE, Mahle WT, Bush L, Donohue J, Gaies MG, Nicolson SC, Shekerdemian L, Witte M, Wolf M, Shea JA, Likosky DS, Pasquali SK.
      BACKGROUND: The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation after infant Tetralogy of Fallot (TOF) and coarctation repair overall at participating sites through implementing a clinical practice guideline (CPG). We evaluated variability across sites in CPG implementation and outcomes.METHODS: Patient characteristics and outcomes [time to extubation, length of stay (LOS)] were compared across sites, including pre- to post-CPG changes. Semi-structured interviews were analyzed to assess similarities and differences in implementation strategies across sites.
    RESULTS: A total of 322 patients were included (4 active, 1 model site). Patient characteristics were similar across active sites, while pre-CPG median time to extubation varied from 15.4-35.5 hours. All active sites had a significant post-CPG decline (p<.001), however there was variation in the post-CPG median time to extubation (0.3-5.3 hours, p=0.01) and magnitude of change (-73.3% to -99.2%). Site A achieved the shortest post-CPG time to extubation and had the greatest percent change. Two sites had significant decreases in medical ICU LOS in TOF patients; no hospital LOS changes were seen. All sites valued the collaborative learning strategy, site visits, CPG flexibility, and had similar core team composition. Site A utilized several unique strategies: inclusion of other staff and fellows, regular in-person data reviews, additional data collection, and creation of complementary protocols.
    CONCLUSIONS: All PHN CLS sites successfully reduced time to extubation. The magnitude of change varied and may be explained in part by different CPG implementation strategies. These data can guide CPG dissemination and design of future improvement projects.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.11.031
  4. J Am Heart Assoc. 2018 Nov 20. 7(22): e010624
    McCracken C, Spector LG, Menk JS, Knight JH, Vinocur JM, Thomas AS, Oster ME, St Louis JD, Moller JH, Kochilas L.
      Background Prior research has focused on early outcomes after congenital heart surgery, but less is known about later risks. We aimed to determine the late causes of death among children (<21 years of age) surviving their initial congenital heart surgery. Methods and Results This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a US-based registry of interventions for congenital heart defects (CHD). Excluding patients with chromosomal anomalies or inadequate identifiers, we matched those surviving their first congenital heart surgery (1982-2003) against the National Death Index through 2014. Causes of death were obtained from the National Death Index to calculate cause-specific standardized mortality ratios (SMRs). Among 31 132 patients, 2527 deaths (8.1%) occurred over a median follow-up period of 18 years. Causes of death varied by time after surgery and severity of CHD but, overall, 69.9% of deaths were attributed to the CHD or another cardiovascular disorder, with a SMR for CHD/cardiovascular disorder of 67.7 (95% confidence interval: 64.5-70.8). Adjusted odds ratios revealed increased risk of death from CHD/cardiovascular disorder in females [odds ratio=1.28; 95% confidence interval (1.04-1.58); P=0.018] with leading cardiovascular disorder contributing to death being cardiac arrest (16.8%), heart failure (14.8%), and arrhythmias (9.1%). Other major causes of death included coexisting congenital malformations (4.7%, SMR: 7.0), respiratory diseases (3.6%, SMR: 8.2), infections (3.4%, SMR: 8.2), and neoplasms (2.1%, SMR: 1.9). Conclusions Survivors of congenital heart surgery face long-term risks of premature mortality mostly related to residual CHD pathology, heart failure, and arrhythmias, but also to other noncardiac conditions. Ongoing monitoring is warranted to identify target factors to address residual morbidities and improve long-term outcomes.
    Keywords:  congenital heart disease; mortality; outcomes research; surgery
    DOI:  https://doi.org/10.1161/JAHA.118.010624
  5. Congenit Heart Dis. 2018 Dec 20.
    Engelhardt EM, Trout AT, Sheridan RM, Veldtman GR, Dillman JR.
      PURPOSE: Patients who have undergone Fontan palliation of single ventricle physiology congenital heart disease are prone to developing focal liver lesions. In our experience, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy-proven liver lesions in patients post-Fontan palliation of single ventricle physiology cared for at our institution.METHODS: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for lesion pathology, background liver findings, pertinent laboratory data, and demographic data. CT and MRI images were reviewed to describe imaging findings of the reported lesions.
    RESULTS: Twelve patients met inclusion criteria; 58% (7/12) of which were female. Fifteen lesions were biopsied including four macroregenerative/benign hepatocellular hyperplastic nodules, two hepatocellular adenomas, two hepatocellular carcinomas, two intrahepatic cholangiocarcinoma (in the same patient), one venous malformation, and one focus of vascularized scar tissue. Two additional lesions in patients postcardiac transplant were posttransplant lymphoproliferative disorder.
    CONCLUSION: Patients who have undergone Fontan palliation of single ventricle physiology are prone to develop a variety of liver lesions, both benign and malignant.
    Keywords:  Fontan operation; congenital heart disease; histopathology; imaging features; liver lesion
    DOI:  https://doi.org/10.1111/chd.12730
  6. JAMA Cardiol. 2018 Dec 19.
    Smith CA, McCracken C, Thomas AS, Spector LG, St Louis JD, Oster ME, Moller JH, Kochilas L.
      Importance: Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse.Objective: To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics.
    Design, Setting, and Participants: Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018.
    Exposures: We examined patient-associated and surgery-associated risk factors affecting survival.
    Main Outcomes and Measures: We analyzed the transplant-free survival during early (<6 years) and late (≥6 years) phase after TOF surgical repair.
    Results: Of the 3283 patients who survived repair for simple TOF and met the study's inclusion criteria, 56.4% were male and 43.6% were female. Twenty-five-year survival following TOF repair was 94.5%. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49) and non-valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19). Presence of a genetic abnormality was associated with increased risk of death both in the early (HR, 3.64; 95% CI, 2.05-6.47) and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44).
    Conclusions and Relevance: Long-term survival after simple TOF repair is excellent. Staged repair and non-valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.
    DOI:  https://doi.org/10.1001/jamacardio.2018.4255
  7. Cardiol Young. 2018 Dec 20. 1-8
    Gaies M, Anderson J, Kipps A, Lorts A, Madsen N, Marino B, Costello JM, Brown D, Jacobs JP, Kasnic D, Lihn S, Lannon C, Margolis P, Pearson GD, Kaltman J, Charpie JR, Redington AN, Pasquali SK, .
      Optimising short- and long-term outcomes for children and patients with CHD depends on continued scientific discovery and translation to clinical improvements in a coordinated effort by multiple stakeholders. Several challenges remain for clinicians, researchers, administrators, patients, and families seeking continuous scientific and clinical advancements in the field. We describe a new integrated research and improvement network - Cardiac Networks United - that seeks to build upon the experience and success achieved to-date to create a new infrastructure for research and quality improvement that will serve the needs of the paediatric and congenital heart community in the future. Existing gaps in data integration and barriers to improvement are described, along with the mission and vision, organisational structure, and early objectives of Cardiac Networks United. Finally, representatives of key stakeholder groups - heart centre executives, research leaders, learning health system experts, and parent advocates - offer their perspectives on the need for this new collaborative effort.
    Keywords:  Clinical registries; collaborative quality improvement; other; paediatric cardiology; quality improvement
    DOI:  https://doi.org/10.1017/S1047951118001683
  8. Congenit Heart Dis. 2018 Dec 18.
    Jackson JL, Morack J, Harris M, DeSalvo J, Daniels CJ, Chisolm DJ.
      OBJECTIVE: The current study aims to identify the rates of lapses in care and loss to follow-up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow-up than whites.DESIGN: Patients were from a large pediatric hospital and had (1) at least one outpatient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a diagnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from before age one through age five. Lapse in follow-up was defined as not having at least one outpatient cardiology visit per year, and loss to follow-up was not returning after a lapse in care by age five. Race was categorized as white and nonwhite. Covariates included sex, insurance type, noncardiology inpatient and outpatient hospital utilization, and CHD severity.
    RESULTS: The sample included 1034 patients. Overall, 75.7% experienced a lapse in care with only 41.6% of those returning by age five. Nonwhites experienced lapses in care at younger ages than whites. Nonwhites had a 53% increased risk of lapse in care. Medicaid patients and those with moderate CHD diagnoses also had an increased risk for lapse in care.
    CONCLUSIONS: Lapse in care appears prevalent among CHD survivors by age five, with nonwhites demonstrating elevated risk. Future multisite prospective studies should include the assessment of parental knowledge, barriers to accessing care, and satisfaction with care.
    Keywords:  children; clinic follow-up; congenital heart disease; racial disparities
    DOI:  https://doi.org/10.1111/chd.12732
  9. JMIR Form Res. 2018 Jul-Dec;2(2):pii: e16. [Epub ahead of print]2(2):
    Lopez KN, O'Connor M, King J, Alexander J, Challman M, Lovick DK, Goodly N, Smith A, Fawcett E, Mulligan C, Thompson D, Fordis M.
      Background: Congenital heart diseases (CHDs) are the most common type of birth defects. Improvements in CHD care have led to approximately 1.4 million survivors reaching adulthood. Successful transition and transfer from pediatric to adult care is crucial. Unfortunately, less than 30% of adolescents with CHD successfully transition to adult care; this number is lower for minority and lower socioeconomic status populations. Few CHD programs exist to facilitate successful transition.Objective: The goal of our study was to describe the formative research used to develop a prototype mobile app to facilitate transition to adult care for adolescents with CHD.
    Methods: A literature search about best practices in transition medicine for CHD was conducted to inform app development. Formative research with a diverse group of CHD adolescents and their parents was conducted to determine gaps and needs for CHD transition to adult care. As part of the interview, surveys assessing transition readiness and CHD knowledge were completed. Two adolescent CHD expert panels were convened to inform educational content and app design.
    Results: The literature review revealed 113 articles, of which 38 were studies on transition programs and attitudes and 3 identified best practices in transition specific to CHD. A total of 402 adolescents aged 15 to 22 years (median 16 years) participated in semistructured interviews. The group was racially and ethnically diverse (12.6% [51/402] African American and 37.8% [152/402] Latino) and 42.0% (169/402) female; 36.3% (146/402) received public insurance. Most adolescents (313/402, 76.7%) had moderate or severe CHD complexity and reported minimal CHD understanding (79.0% [275/348] of those aged 15 to 17 years and 61.1% [33/54] of those aged 18 to 22 years). Average initial transition readiness score was 50.9/100, meaning that transition readiness training was recommended. When participants with moderate to severe CHD (313/402, 77.9%) were asked about technology use, 94.2% (295/313) reported having access to a mobile phone. Interviews with parents revealed limited interactions with the pediatric cardiologist about transition-related topics: 79.4% (331/417) reported no discussions regarding future family planning, and 55.2% (230/417) reported the adolescent had not been screened for mental health concerns (depression, anxiety). Further, 66.4% (277/417) reported not understanding how health care changes as adolescents become adults. Adolescents in the expert panels (2 groups of 3 adolescents each) expressed interest in a CHD-specific tailored app consisting of quick access to specific educational questions (eg, "Can I exercise?"), a CHD story-blog forum, a mentorship platform, a question and answer space, and a checklist to facilitate transition. They expressed interest in using the app to schedule CHD clinic appointments and receive medication reminders. Based on this data, a prototype mobile app was created to assist in adolescent CHD transition.
    Conclusions: Formative research revealed that most adolescents with CHD had access to mobile phones, were not prepared for transition to adult care, and were interested in an app to facilitate transition to adult CHD care. Understanding adolescent and parent needs, interests, and concerns helped in the development of a mobile app with a broader, tailored approach for adolescents with CHD.
    Keywords:  adolescent health; chronic disease; health disparities; mHealth; mobile health; patient empowerment; patient involvement; self-efficacy; transitions of care; user-centered design
    DOI:  https://doi.org/10.2196/formative.9963
  10. Cardiol Young. 2018 Dec 21. 1-7
    Drury NE, Stoll VM, Bond CJ, Patel AJ, Hutchinson S, Clift PF.
      OBJECTIVE: To bring together stakeholders in the United Kingdom to establish national priorities for research in single-ventricle heart conditions.METHODS: This study comprised two surveys and a workshop. The initial public online survey asked respondents up to three questions they would like answered for research. Responses were classified as unanswered, already answered, or unable to be answered by scientific research. In the follow-up survey, unanswered questions were divided into categories and respondents were asked to rank categories and questions by priority. A stakeholder workshop attended by patients, parents, healthcare professionals, researchers, and charities was held to determine the final list of research priorities.
    RESULTS: A total of 128 respondents posed 344 research questions, of which 271 were classified as unanswered, and after removing duplicates, 204 questions remained, which were divided into 20 categories. In the second survey, 56 (49.1%) respondents successfully ranked categories and questions. A total of 39 participants attended the workshop, drawing up a list of 30 research priorities across nine priority categories. The nine priority categories are: Associated co-morbidities; Brain &amp; neurodevelopment; Exercise; Fontan failure; Heart function; Living with a single ventricle heart condition; Management of the well-functioning Fontan circulation; Surgery &amp; perioperative care; and Transplantation, mechanical support &amp; novel therapies.
    CONCLUSIONS: Through a multi-stage process, we engaged a wide range of interested parties to establish a list of research priorities in single-ventricle heart conditions. This provides a platform for clinicians, researchers, and funders in the United Kingdom and elsewhere to address the most important questions and improve outcomes in these rare but high-impact CHDs.
    Keywords:  Fontan circulation; Single-ventricle heart conditions; patient involvement; research priorities
    DOI:  https://doi.org/10.1017/S104795111800224X
  11. J Thorac Cardiovasc Surg. 2019 Jan;pii: S0022-5223(18)32353-5. [Epub ahead of print]157(1): 329-340
    Myers PO, Mokashi SA, Horgan E, Borisuk M, Mayer JE, Del Nido PJ, Baird CW.
      OBJECTIVES: There are little recent data on the outcomes of mechanical aortic valve replacement (AVR) in children and young adults with congenital aortic valve disease. We sought to review the survival and associated thromboembolic or bleeding complications after mechanical AVR at a single center.METHODS: Data were retrospectively collected for 121 patients undergoing prosthetic AVR from 2000 to 2014. Kaplan-Meier estimates and Cox proportional hazards were employed.
    RESULTS: Median age at AVR was 16 years (interquartile range, 12-22.8 years). The valves implanted were the St Jude valve (St Jude Medical Inc, St Paul, Minn) in 79 patients (62%), the On-X valve (On-X Life Technologies Inc, Austin, Tex) in 45 patients (35%), and CarboMedics (Sorin SpA, Milan, Italy) in 3 patients (2.4%). Median valve size was 23 mm (range, 21-25 mm). There were 5 early deaths (3.9%). Median follow-up was 5 years (range, 1.6-9.2 years; 600 patient-years). There were 14 deaths during follow-up. Survival was 90.6% ± 2.8% at 1 year, 85.4% ± 3.7% at 5 years, and 81.5% ± 4.5% at 10 years. Freedom from aortic valve reoperation was 98% ± 1.4% at 1 and 5 years, 91.5% ± 3.9% at 7 years, and 78.4% ± 6.9% at 10 years and at latest follow-up. Univariable analysis identified younger age, lower weight, and use of a 16-mm CarboMedics valve as predictors of reoperation. Valve sizes of 16 or 17 mm have a significantly higher risk of reoperation compared with larger valves (log-rank test, P < .001). At multivariable analysis, only younger age was a significant independent predictor of reoperation (hazard ratio, 0.84; 95% confidence interval, 0.71-0.99; P = .038). All patients were treated with warfarin to a goal international normalized ratio of 2.0 to 3.0. Four patients (3.1%; 0.66% per patient-year) had thromboembolic complications, and 5 patients (3.9%; 0.83% per patient-year) had bleeding events during follow-up.
    CONCLUSIONS: Mechanical AVR in patients with congenital heart disease has excellent short- and midterm outcomes. Younger age was an independent predictor of reoperation.
    Keywords:  aortic valve; aortic valve replacement; mechanical valve; pediatric cardiac surgery
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.08.077
  12. Circulation. 2018 Nov 20. 138(21): e653-e711
    Pierpont ME, Brueckner M, Chung WK, Garg V, Lacro RV, McGuire AL, Mital S, Priest JR, Pu WT, Roberts A, Ware SM, Gelb BD, Russell MW, .
      This review provides an updated summary of the state of our knowledge of the genetic contributions to the pathogenesis of congenital heart disease. Since 2007, when the initial American Heart Association scientific statement on the genetic basis of congenital heart disease was published, new genomic techniques have become widely available that have dramatically changed our understanding of the causes of congenital heart disease and, clinically, have allowed more accurate definition of the pathogeneses of congenital heart disease in patients of all ages and even prenatally. Information is presented on new molecular testing techniques and their application to congenital heart disease, both isolated and associated with other congenital anomalies or syndromes. Recent advances in the understanding of copy number variants, syndromes, RASopathies, and heterotaxy/ciliopathies are provided. Insights into new research with congenital heart disease models, including genetically manipulated animals such as mice, chicks, and zebrafish, as well as human induced pluripotent stem cell-based approaches are provided to allow an understanding of how future research breakthroughs for congenital heart disease are likely to happen. It is anticipated that this review will provide a large range of health care-related personnel, including pediatric cardiologists, pediatricians, adult cardiologists, thoracic surgeons, obstetricians, geneticists, genetic counselors, and other related clinicians, timely information on the genetic aspects of congenital heart disease. The objective is to provide a comprehensive basis for interdisciplinary care for those with congenital heart disease.
    Keywords:  AHA Scientific Statements; genetics; heart defects, congenital
    DOI:  https://doi.org/10.1161/CIR.0000000000000606
  13. Congenit Heart Dis. 2018 Dec 21.
    Steiner JM, Stout K, Soine L, Kirkpatrick JN, Curtis JR.
      BACKGROUND: Patients with adult congenital heart disease (ACHD) report that advance care planning (ACP) is important, and that they want information about prognosis. However, recognizing importance and being willing to participate are different constructs, and how and when to begin ACP and palliative care discussions remains ill-defined.METHODS: We conducted a cross-sectional survey of 150 consecutive outpatients to assess willingness to participate in ACP, with whom, and important barriers and facilitators to these discussions.
    RESULTS: The majority of participants (69%) reported being willing to participate in ACP; 79% to have a meeting to discuss goals and care preferences; and 91% to speak to a clinician who specializes in palliative care. Being married and anticipating a shorter lifespan were associated with increased reported willingness to participate in ACP. The health care provider with whom most participants preferred to have these discussions was their ACHD clinician. Participants identified important barriers and facilitators to these discussions.
    CONCLUSION: Patients with ACHD report being willing to participate in ACP and palliative care discussions. Patients prefer to have these discussions with their ACHD clinicians, thus ACHD clinicians need to be prepared to address these issues as part of routine care.
    Keywords:  adult congenital heart disease; advance care planning; palliative care
    DOI:  https://doi.org/10.1111/chd.12735
  14. Pediatrics. 2018 Dec 18. pii: e20181551. [Epub ahead of print]
    Mabel H, Harrison CH, Ahmad MU, Blume ED, Boyle GJ, Bester JC, Lantos JD.
      We present the case of a 19-month-old boy with complex congenital heart disease. His single father is skeptical of traditional medicine and does not offer the social support needed to make heart transplantation successful for his son. After the father demonstrates commitment to transplant success and provides enhanced social support, doctors place the child on a Berlin Heart (a biventricular assist device) as a bridge to transplantation and list him for transplant. When the child is matched to a donor heart, the father refuses transplantation, despite that it is the child's only chance for survival. His doctors report the case to child protective services, but they decline to take protective custody. The father then changes his mind and asks that the child be put back on the waiting list for transplant. By this time, the social supports the father implemented are no longer in place. This case raises a number of issues. First, should courts order heart transplantation when doctors believe that it is in the child's best interest and parents do not consent? Second, once parents refuse a transplant, can they change their minds? Third, if there are uncertainties regarding whether the child has the social support to make transplantation successful, should the child be relisted? Finally, should a child who is not currently a transplant candidate but who may become one in the future be supported with ventricular assist devices?
    DOI:  https://doi.org/10.1542/peds.2018-1551
  15. Pediatr Cardiol. 2018 Dec 17.
    Ma MH, He W, Benavidez OJ.
      Congenital heart defects are common among patients with trisomy 13 and 18; surgical repair has been controversial and rarely studied. We aimed to assess the frequency of cardiac surgery among admissions with trisomy 13 and 18, and evaluate their associations with resource use, complications, and mortality compared to admissions without these diagnoses. We evaluated congenital heart surgery admissions of ages < 18 years in the 1997, 2000, 2003, 2006, and 2009 Kids' Inpatient Database. Bivariate and multivariate analyses examined the adjusted association of trisomy 13 and 18 on resource use, complications, and inpatient death following congenital heart surgery. Among the 73,107 congenital heart surgery admissions, trisomy 13 represented 0.03% (n = 22) and trisomy 18 represented 0.08% (n = 58). Trisomy 13 and 18 admissions were longer; trisomy 13: 27 days vs. 8 days, p = 0.003; trisomy 18: 16 days vs. 8 days, p = 0.001. Hospital charges were higher for trisomy 13 and 18 admissions; trisomy 13: $160,890 vs. $87,007, p = 0.010; trisomy 18: $160,616 vs. $86,999, p < 0.001. Trisomy 18 had a higher complication rate: 52% vs. 34%, p < 0.006. For all cardiac surgery admissions, mortality was 4.5%; trisomy 13: 14% and trisomy 18: 12%. In multivariate analysis, trisomy 18 was an independent predictor of death: OR 4.16, 95% CI 1.35-12.82, p = 0.013. Patients with trisomy 13 and 18 represent 0.11% of pediatric congenital heart surgery admissions. These patients have a 2- to 3.4-fold longer hospital stay and double hospital charges. Patients with trisomy 18 have more complications and four times greater adjusted odds for inpatient death.
    Keywords:  Congenital heart surgery; Outcomes; Pediatric cardiology; Trisomy 18; Trisomy 21
    DOI:  https://doi.org/10.1007/s00246-018-2032-3
  16. BJOG. 2018 Dec 21.
    Hautala J, Gissler M, Ritvanen A, Tekay A, Pitkänen-Argillander O, Stefanovic V, Sarkola T, Helle E, Pihkala J, Pätilä T, Mattila I, Jokinen E, Räsänen J, Ojala T.
      OBJECTIVE: To evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA) and whether maternal risk factors for severe fetal heart disease affect prenatal detection.DESIGN: Population-based cohort study.
    SETTING: Nationwide data from Finnish registries 2004 to 2014.
    POPULATION: 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly.
    METHODS: Prenatal detection rates were calculated in three time periods (prescreening, transition, and screening phase). The effect of maternal risk factors (obesity, in vitro fertilization, pregestational diabetes, and smoking) was evaluated.
    MAIN OUTCOME MEASURES: Change in detection rates and impact of maternal risk factors on screening programme efficacy.
    RESULTS: In total 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and TGA from 12.3% to 41.0% (P<0.0001). Maternal risk factors did not affect prenatal detection rate, but detection rate differed substantially by region.
    CONCLUSIONS: A nationwide screening programme improved overall UVH and TGA detection rates, but regional differences were observed. Obesity or other maternal risk factors did not affect the screening programme efficacy. The establishment of structured guidelines and recommendations are essential when implementing the screening programme. In addition, prospective screening register is highly recommended to ensure high quality of screening. This article is protected by copyright. All rights reserved.
    Keywords:  Congenital heart disease; Prenatal diagnosis; Prenatal screening; Transposition of great arteries; Univentricular heart
    DOI:  https://doi.org/10.1111/1471-0528.15589
  17. Echo Res Pract. 2019 Mar 01. pii: ERP-18-0049. [Epub ahead of print]6(1): R9-R23
    Bedair R, Iriart X.
      Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, affecting 3 in 10,000 live births. Surgical correction in early childhood is associated with good outcomes, but lifelong follow-up is necessary to identify the long-term sequelae that may occur. This article will cover the diagnosis of TOF in childhood, the objectives of surveillance through adulthood and the value of multi-modality imaging in identifying and guiding timely surgical and percutaneous interventions.
    Keywords:  congenital heart disease; multi-modality imaging; pulmonary regurgitation; tetralogy of Fallot
    DOI:  https://doi.org/10.1530/ERP-18-0049