bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒07‒07
thirteen papers selected by
Richard James
University of Pennsylvania


  1. Am J Cardiol. 2019 Jun 06. pii: S0002-9149(19)30626-5. [Epub ahead of print]
    Oster ME, McCracken C, Kiener A, Aylward B, Cory M, Hunting J, Kochilas LK.
      Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.
    DOI:  https://doi.org/10.1016/j.amjcard.2019.05.047
  2. Heart. 2019 Jul 05. pii: heartjnl-2019-314711. [Epub ahead of print]
    Crossland DS, Jansen K, Parry G, Harper A, Perri G, Davidson A, De Rita F, Hermuzi A, Nassar M, Seller N, MacGowan GA, Hasan A, O'Sullivan JJ, Coats L.
      OBJECTIVES: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT).METHODS: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV).
    RESULTS: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period.
    CONCLUSIONS: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.
    Keywords:  complex congenital heart disease; congenital heart disease; congenital heart disease surgery; heart transplantation
    DOI:  https://doi.org/10.1136/heartjnl-2019-314711
  3. J Thorac Cardiovasc Surg. 2019 Mar;pii: S0022-5223(18)33252-5. [Epub ahead of print]157(3): 1146-1155
    Trusty PM, Wei ZA, Slesnick TC, Kanter KR, Spray TL, Fogel MA, Yoganathan AP.
      OBJECTIVE: Fontan surgical planning is an image-based, collaborative effort, which is hypothesized to result in improved patient outcomes. A common motivation for Fontan surgical planning is the progression (or concern for progression) of pulmonary arteriovenous malformations. The purpose of this study was to evaluate the accuracy of surgical planning predictions, specifically hepatic flow distribution (HFD), a known factor in pulmonary arteriovenous malformation progression, and identify methodological improvements needed to increase prediction accuracy.METHODS: Twelve single-ventricle patients who were enrolled in a surgical planning protocol for Fontan surgery with pre- and postoperative cardiac imaging were included in this study. Computational fluid dynamics were used to compare HFD in the surgical planning prediction and actual postoperative conditions.
    RESULTS: Overall, HFD prediction error was 17 ± 13%. This error was similar between surgery types (15 ± 18% and 18 ± 10% for revisions vs Fontan completions respectively; P = .73), but was significantly lower (6 ± 7%; P = .05) for hepatic to azygous shunts. Y-grafts and extracardiac conduits showed a strong correlation between prediction error and discrepancies in graft insertion points (r = 0.99; P < .001). Improving postoperative anatomy prediction significantly reduced overall HFD prediction error to 9 ± 6% (P = .03).
    CONCLUSIONS: Although Fontan surgical planning can offer accurate HFD predictions for specific graft types, methodological improvements are needed to increase overall accuracy. Specifically, improving postoperative anatomy prediction was shown to be an important target for future work. Future efforts and refinements to the surgical planning process will benefit from an improved understanding of the current state and will rely heavily on increased follow-up data.
    Keywords:  Fontan; congenital heart defect; hepatic flow distribution; preprocedural planning; pulmonary arteriovenous malformations; surgical planning
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.11.102
  4. Circulation. 2019 Jul 01. CIR0000000000000696
    Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia TY, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, d'Udekem Y, .
      It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.
    Keywords:  AHA Scientific Statements; Fontan procedure; congenital heart defects; quality of life
    DOI:  https://doi.org/10.1161/CIR.0000000000000696
  5. Patient Educ Couns. 2019 Jun 26. pii: S0738-3991(19)30256-3. [Epub ahead of print]
    Werner O, Abassi H, Lavastre K, Guillaumont S, Picot MC, Serrand C, Dulac Y, Souletie N, Acar P, Bredy C, Amedro P.
      OBJECTIVE: Transition education programs dedicated to adolescents and young adults with congenital heart disease (CHD) aim to facilitate transfer to adult cardiology and bring more autonomy to teenagers. This prospective controlled multicentre study analysed the factors influencing the participation in a transition education program.METHODS: CHD patients aged 13-25 y were offered to participate in the transition program. A multiple linear regression identified the explanatory factors for participation in the program.
    RESULTS: A total of 123 patients (mean age 19.6 ± 3.4 y) were included in the study, with 57 participants and 66 non-participants. Both groups showed similar socio-demographic and quality of life characteristics, low level of physical activity with muscular deconditioning and high exposure to risk behaviours (71% patients with ≥1 risk factor). Patients with complex CHD (OR = 4.1, P = 0.03), poor disease knowledge (OR = 0.3, P = 0.02), risk behaviours (body piercing, OR = 5.53, P = 0.01; alcohol, OR = 3.12, P = 0.06), and aged <20 y (OR = 0.29, P = 0.03), were more likely to join the program.
    CONCLUSION: Many risk factors influencing the participation of adolescents and young adults with CHD in transition education programs are controllable.
    PRACTICE IMPLICATION: Further randomized studies are necessary to evaluate the impact of transition education program on quality of life, successful transfer to adult centre and, ultimately, prognosis.
    Keywords:  Attitudes; Congenital; Health knowledge; Heart defects; Patient education as topic; Practice; Quality of life; Transition to adult care
    DOI:  https://doi.org/10.1016/j.pec.2019.06.023
  6. Pediatr Crit Care Med. 2019 Jul 05.
    Justice LB, Callow L, Loomba R, Harvey J, FitzGerald M, Cooper DS.
      OBJECTIVES: The education, training, and scope of practice of cardiac ICU advanced practice providers is highly variable. A survey was administered to cardiac ICU advanced practice providers to examine specific variations in orientation format, competency assessment during and at the end of orientation, and scope of clinical practice to determine gaps in resources and need for standardization.DESIGN: This study was a cross-sectional descriptive study utilizing survey responses.
    SETTING: Pediatric cardiac ICUs in the United States.
    SUBJECTS: The survey was delivered to a convenience sample of advanced practice providers currently practicing in pediatric cardiac ICUs.
    INTERVENTIONS: A list of pediatric cardiothoracic surgery programs was generated from the Society of Thoracic Surgery database. A self-administered, electronic survey was delivered via email to advanced practice providers at those institutions. Descriptive data were compared using a chi-square test or Fisher exact test depending on the normalcy of data. Continuous data were compared using a Student t test or Mann-Whitney U test.
    MEASUREMENTS AND MAIN RESULTS: Eighty-three of 157 advanced practice providers responded (53% response rate, representing 36 institutions [35% of institutions]). Sixty-five percent of respondents started as new graduates. Ninety-three to one-hundred percent obtain a history and physical, order/interpret laboratory, develop management plans, order/titrate medications, and respiratory support. Ability to perform invasive procedures was highly variable but more likely for those in a dedicated cardiac ICU. Seventy-seven percent were oriented by another advanced practice provider, with a duration of orientation less than 4 months (66%). Fifty percent of advanced practice providers had no guidelines in place to guide learning/competency during orientation. Sixty-seven percent were not evaluated in any way on their knowledge or skills during or at the end of orientation. Orientation was rated as poor/fair by the majority of respondents for electrophysiology (58%) and echocardiography (69%). Seventy-one percent rated orientation as moderately effective or less. Respondents stated they would benefit from more structured didactic education with clear objectives, standardized management guidelines, and more simulation/procedural practice. Eighty-five percent were very/extremely supportive of a standardized cardiac ICU advanced practice provider curriculum.
    CONCLUSIONS: Orientation for cardiac ICU advanced practice providers is highly variable, content depends on the institution/preceptor, and competency is not objectively defined or measured. A cardiac ICU advanced practice provider curriculum is needed to standardize education and promote the highest level of advanced practice provider practice.
    DOI:  https://doi.org/10.1097/PCC.0000000000002069
  7. Acta Paediatr. 2019 Jun 29.
    Brida M, Gatzoulis MA.
      The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success story of modern medicine. In the 1950s survival of children born with CHD was only approximately 15%, whereas nowadays more than 90% of these children survive well into adulthood. Consequently the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients with CHD over 60 years old. Many adult patients are afflicted by residual haemodynamic lesions and also face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non-cardiac pathology etc., necessitating integrated care and all medical disciplines. We are faced with a 'tsunami' in terms of adult CHD numbers, disease heterogeneity, and complexity of work and interventions needed. We need to secure resources, welcome more people in our field, learn from 'marching with our patients', and educate better patients, public, and ourselves so that every single patient with CHD, born anywhere in the world, may reach their full life potential. This article is protected by copyright. All rights reserved.
    Keywords:  Adult congenital heart disease; catheter interventions in congenital heart disease; history of congenital heart disease; surgery in congenital heart disease
    DOI:  https://doi.org/10.1111/apa.14921
  8. J Thorac Cardiovasc Surg. 2019 Jul;pii: S0022-5223(19)30532-X. [Epub ahead of print]158(1): 220-229
    Mascio CE, Irons ML, Ittenbach RF, Gaynor JW, Fuller SM, Kaplinski M, Kennedy AT, Steven JM, Nicolson SC, Spray TL.
      OBJECTIVE: Hypoplastic left heart syndrome is one of the most common and challenging lesions requiring surgical intervention in the neonatal period. The Norwood procedure for hypoplastic left heart syndrome was first reported in 1983. The objective of this study was to describe early outcomes after the Norwood procedure at a single institution over 30 years.METHODS: This retrospective cohort study included all patients with hypoplastic left heart syndrome (and variants) who underwent the Norwood procedure between January 1984 and May 2014 at a single institution. The study period was divided into 6 eras: era 1, 1984 to 1988; era 2, 1989 to 1993; era 3, 1994 to 1998; era 4, 1999 to 2003; era 5, 2004 to 2008; and era 6, 2009 to 2014. The primary outcome was in-hospital mortality after the Norwood procedure. Binomial point estimates complete with 95% confidence intervals (CL0.95) were computed for the entire cohort and by era.
    RESULTS: During the study period, 1663 infants underwent the Norwood procedure. Overall in-hospital mortality was 25.9% (CL0.95, 23.8-28.0). Mortality by chronologic era was 40.4% (CL0.95, 34.9-45.9), 33.6% (CL0.95, 29.2-37.9), 28.7% (CL0.95, 22.8-34.6), 14.9% (CL0.95, 10.4-19.3), 11.2% (CL0.95, 7.4-15.0), and 15.7% (CL0.95, 10.3-21.1). Survival was improved in eras 4 to 6 compared with eras 1 to 3 (P all < .03). Anomalous pulmonary drainage, moderate to severe atrioventricular valve regurgitation, lower birth weight, earlier era, younger gestational age, genetic anomaly, preterm birth, race other than white or African-American, and lower weight at the Norwood procedure were associated with increased mortality. Mortality was greatest in patients with 3 or more risk factors. In the best-fitting multiple covariate model, anomalous pulmonary venous drainage, gestational age in weeks, genetic anomaly, and race other than white and African American were statistically significant contributors, after adjusting for era.
    CONCLUSIONS: Survival after the Norwood procedure has plateaued despite improvements in diagnosis, perioperative care, and surgical techniques. Nonmodifiable patient characteristics are important determinants of the risk of mortality.
    Keywords:  Norwood procedure; hypoplastic left heart syndrome; outcomes
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.12.117
  9. Pediatr Rev. 2019 Jul;40(7): 344-353
    Javed R, Cetta F, Said SM, Olson TM, O'Leary PW, Qureshi MY.
      Hypoplastic left heart syndrome is one of the most complex congenital heart diseases and requires several cardiac surgeries for survival. The diagnosis is usually established prenatally or shortly after birth. Each stage of surgery poses a unique hemodynamic situation that requires deeper understanding to manage common pediatric problems such as dehydration and respiratory infections. Careful multidisciplinary involvement in the care of these complex patients is improving their outcome; however, morbidity and mortality are still substantial. In this review, we focus on the hemodynamic aspects of various surgical stages that a primary care provider should know to manage these challenging patients.
    DOI:  https://doi.org/10.1542/pir.2018-0005
  10. Heart. 2019 Jul 04. pii: heartjnl-2018-313928. [Epub ahead of print]
    Buber J, Shafer K.
      
    Keywords:  congenital heart disease
    DOI:  https://doi.org/10.1136/heartjnl-2018-313928
  11. BMJ Open. 2019 Jul 02. 9(7): e028139
    Bakker MK, Bergman JEH, Krikov S, Amar E, Cocchi G, Cragan J, de Walle HEK, Gatt M, Groisman B, Liu S, Nembhard WN, Pierini A, Rissmann A, Chidambarathanu S, Sipek A, Szabova E, Tagliabue G, Tucker D, Mastroiacovo P, Botto LD.
      OBJECTIVES: To assess international trends and patterns of prenatal diagnosis of critical congenital heart defects (CCHDs) and their relation to total and live birth CCHD prevalence and mortality.SETTING: Fifteen birth defect surveillance programmes that participate in the International Clearinghouse for Birth Defects Surveillance and Research from 12 countries in Europe, North and South America and Asia.
    PARTICIPANTS: Live births, stillbirths and elective terminations of pregnancy for fetal anomaly diagnosed with 1 of 12 selected CCHD, ascertained by the 15 programmes for delivery years 2000 to 2014.
    RESULTS: 18 243 CCHD cases were reported among 8 847 081 births. The median total prevalence was 19.1 per 10 000 births but varied threefold between programmes from 10.1 to 31.0 per 10 000. CCHD were prenatally detected for at least 50% of the cases in one-third of the programmes. However, prenatal detection varied from 13% in Slovak Republic to 87% in some areas in France. Prenatal detection was consistently high for hypoplastic left heart syndrome (64% overall) and was lowest for total anomalous pulmonary venous return (28% overall). Surveillance programmes in countries that do not legally permit terminations of pregnancy tended to have higher live birth prevalence of CCHD. Most programmes showed an increasing trend in prenatally diagnosed CCHD cases.
    DISCUSSION AND CONCLUSIONS: Prenatal detection already accounts for 50% or more of CCHD detected in many programmes and is increasing. Local policies and access likely account for the wide variability of reported occurrence and prenatal diagnosis. Detection rates are high especially for CCHD that are more easily diagnosed on a standard obstetric four-chamber ultrasound or for fetuses that have extracardiac anomalies. These ongoing trends in prenatal diagnosis, potentially in combination with newborn pulse oximetry, are likely to modify the epidemiology and clinical outcomes of CCHD in the near future.
    Keywords:  critical congenital heart defects; epidemiology; prenatal diagnosis
    DOI:  https://doi.org/10.1136/bmjopen-2018-028139
  12. Int J Cardiol. 2019 Jun 13. pii: S0167-5273(19)31187-8. [Epub ahead of print]
    Gavotto A, Abassi H, Rola M, Serrand C, Picot MC, Iriart X, Thambo JB, Iserin L, Ladouceur M, Bredy C, Amedro P.
      BACKGROUND: Systemic right ventricle (RV) is a rare and complex congenital heart disease (CHD). Patients with a systemic RV present with a significant decrease of their exercise capacity. We aimed at identifying clinical and paraclinical factors associated with maximum oxygen uptake (VO2max) in adults with a systemic RV.METHODS: This multicentre cross-sectional study was performed in 2017 in three French tertiary care CHD centres. Adult patients with a D-transposition of the great artery (d-TGA) or a congenitally corrected TGA (cc-TGA) were included. Demographic, clinical, laboratory and imaging data were collected. Univariate and multivariate analyses were performed to identify predictors of impaired VO2max, as measured by cardiopulmonary exercise test (CPET).
    RESULTS: A total of 111 patients were included in the study (85% d-TGA, median age 37.2 ± 8.2 years). Most patients presented with impaired physical capacity (mean VO2max of 23.3 ± 6.9 ml/kg/min, representing 68.4 ± 16.6% of predicted values) and ventilatory anaerobic threshold (VAT) impaired (mean VAT of 32.7 ± 10.9% of the predicted values). In univariate analysis, VO2max correlated with professional status, NYHA functional class, BNP level, the type of systemic RV, decreased RV function values in cardiac imaging, the severity of tricuspid regurgitation, the presence of a pacemaker or an implantable defibrillator, the VAT, the maximum load, and the maximal heart rate during exercise. In multivariate analysis, the VO2max remained associated with the NYHA functional class. The final multivariate model explained 49% of the variability of VO2max.
    CONCLUSION: NYHA functional class and RV function are predictors of impaired exercise capacity in adult patients with systemic RV.
    Keywords:  Cardiopulmonary exercise test; Congenital heart disease; NYHA functional class; Systemic right ventricle
    DOI:  https://doi.org/10.1016/j.ijcard.2019.06.030