Congenital Heart Disease

Publisher: Wiley

Journal description

Congenital Heart Disease: Clinical Studies from Fetus to Adulthood is a new, clinical journal focusing on congenital heart disease in children and adults. Though the number of infants born with heart disease each year is relatively small (approximately 1% of the population), advances in the treatment of such malformations have led to increased life spans for this population. Consequently, in the United States today most patients treated for congenital heart disease are over the age of 20. What are the special needs of adults with congenital heart disease? What are the latest developments in the care of the fetus, infants, and children? Who should treat these patients? How should they be treated? Congenital Heart Disease focuses on these questions and more. Conceived as a forum for the most up-to-date information on congenital heart disease, the journal is led by Editor-in-Chief Douglas S. Moodie, MD, Chairman of the Department of Pediatrics at Ochsner Clinic in New Orleans, as well as an international editorial board. Congenital Heart Disease publishes articles on heart disease as it relates to the following areas: Clinical pediatric and adult cardiology; Cardiac imaging; Preventive cardiology; Diagnostic and interventional cardiac catheterization; Electrophysiology; Surgery; Long-term follow-up, particularly as it relates to older children and adult congenital heart disease; Exercise and exercise physiology in the congenital patient; Post-op and critical care; Common disorders such as syncope, chest pain, murmurs, as well as acquired disorders such as Kawasaki syndrome. The journal includes clinical studies, invited editorials, state-of-the-art reviews, case reports, articles focusing on the history and development of congenital heart disease, and CME material. Occasional issues focus on special topics. Congenital Heart Disease was created for pediatric cardiologists; adult cardiologists who care for patients with congenital heart disease; pediatric and pediatric cardiology nurses; surgeons; radiologists; anesthesiologists; critical care physicians and nurses; and adult support staff involved in the care of patients with congenital heart disease.

Current impact factor: 1.01

Impact Factor Rankings

2015 Impact Factor Available summer 2015
2011 Impact Factor 0.901

Additional details

5-year impact 0.00
Cited half-life 3.30
Immediacy index 0.14
Eigenfactor 0.00
Article influence 0.00
Website Congenital Heart Disease website
Other titles Congenital heart disease (Online)
ISSN 1747-0803
OCLC 76166284
Material type Document, Periodical, Internet resource
Document type Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details

Wiley

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Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Acute kidney injury (AKI) is a common complication resulting from cardiopulmonary bypass in infants. Urinary neutrophil gelatinase-associated lipocalin (NGAL) is a sensitive and specific marker of such injury. In this study, we compared the performance of serum cystatin C (Cys C) and serum creatinine (Cr) as early markers of renal dysfunction in infants undergoing cardiac surgery under bypass. The study was designed as a prospective observational study. The study was conducted in the cardiac intensive care unit (ICU) of a tertiary, academic children's hospital in the United States. Infants (age <1 year) undergoing cardiac surgery under cardiopulmonary bypass were included in the study. Acute kidney injury was defined based on postoperative urinary NGAL. A total of 17 infants were included in the study, and five of them developed AKI. Serum Cys C and Cr levels were measured postoperatively on days 1, 2, and 3, and compared with baseline levels. On postoperative day 2, infants with AKI showed significant change from baseline in serum Cys C levels compared with non-AKI infants (28% vs. -9%, P = .03). The two groups did not show significant differences with respect to rise in serum Cr on any of the 3 postoperative days. Serum Cr on days 1 and 2 showed nonspecific increases in both AKI and non-AKI groups. The area under the receiver operating characteristic curve for day 2 Cys C was 0.87 (95% CI 0.67-1.00) in recognizing NGAL-positive AKI. Postoperative serum Cys C appears to be a more specific and sensitive biomarker for NGAL-positive AKI resulting from cardiopulmonary bypass surgery in infants undergoing cardiac surgery. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12253
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    ABSTRACT: Transthoracic echocardiography (TTE) plays a key role in adult congenital heart disease (ACHD). However, a significant number of studies are nondiagnostic due to poor image quality. Enhancement of the blood pool-tissue interface with contrast-enhanced TTE (CE-TTE) can improve image quality in suboptimal studies. The aim of this analysis was to evaluate feasibility and clinical utility of CE-TTE in the assessment of patients with ACHD. A retrospective analysis of all CE-TTE performed in ACHD patients at our institution from August 2007 to May 2014 was performed. Endocardial definition scores (EDS) for each segment in the right and left ventricles were graded pre- and postcontrast imaging, as 1 = good, 2 = suboptimal, 3 = not seen. The endocardial border definition score index (EBDSI) was also calculated pre- and postcontrast imaging. Twenty patients with ACHD had 24 CE. Summation data for all ventricular EDS for unenhanced TTE vs. CE-TTE imaging was: EDS 1 = 136 vs. 314, EDS 2 = 119 vs. 72, EDS 3 = 162 vs. 31, respectively. Wilcoxon matched-pairs rank-signed test showed a significant ranking difference (improvement) pre- and postcontrast for the combined ventricular data (P < .0001) and the individual left and right ventricular data (all P < .0001). The EBDSI for combined ventricular data using CE-TTE was significantly lower than for noncontrast imaging (1.23 ± 0.49 vs. 2.06 ± 0.62, P < .0001). There was one minor contrast adverse reaction. CE-TTE resulted in significantly improved right and left ventricular endocardial definition and improved EDBSI. CE-TTE should be viewed as an additional imaging technique that is available to help assess patients with ACHD, especially those with nondiagnostic images. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12254
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    ABSTRACT: This review synthesizes the empirical literature on outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease. A systematic review of papers published between January 2001 and May 2013 that examined outcomes or experiences of transfer and transition among young people with congenital heart disease was conducted. Data were extracted by two independent reviewers with the outcomes data combined using narrative synthesis and the experiences data integrated using thematic synthesis. Thirteen papers were included in the review: six reported outcomes following transfer, six reported experiences of transfer and transition, and one reported both outcomes and experiences. The review data indicate that high proportions of young people were lost to follow-up or experienced long gaps in care after leaving pediatric cardiology. Factors that protected against loss to follow-up or lapse in care included: beliefs that specialized adult care was necessary; poorer health status; attendance at pediatric appointments without parents; and pediatric referral to an adult congenital heart disease center. Data on experiences highlighted that many young people were unconcerned about transition, but lacked knowledge about their condition and were insufficiently prepared for transfer. In terms of adult services, many young people desired continuity in the quality of care, youth-oriented facilities, a personalized approach, and for their parents to remain involved in their care, but in a secondary, supportive capacity. In conclusion, the high proportions of young people lost to follow-up highlight the need for formal transition programs, which ensure a planned and coordinated transfer. Patients with congenital heart disease need education throughout adolescence about the implications of their condition, the differences between pediatric and adult services, and self-care management. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12251
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    ABSTRACT: Objective The electrocardiogram (ECG) is used to detect left ventricular hypertrophy (LVH) in children and young adults, and it is considered an important screening tool for the most common causes of sudden cardiac arrest in the United States. However, the reliability and accuracy of the ECG in the detection of LVH are poorly understood. This study's objective was to assess the reliability and accuracy of ECG parameters predicting LVH in comparison with echocardiographic two-dimensional left ventricular mass (2D-LVM) assessment.DesignTwo hundred fifty patients met study criteria with complete 2D-LVM measurements, temporally matched ECGs and no confounding structural heart defects. The echocardiographic diagnosis of LVH was made by 2D-LVM z-score > 2. The electrocardiographic diagnosis of LVH was fulfilled if specific Q, R, or S wave voltages exceeded the normal limits for the respective age groups established by the Davignon, Macfarlane and Rijnbeek datasets, or fulfilled the LVH criteria set forth by adult indices (Sokolow–Lyon, Cornell, Gubner, Romhilt–Estes).ResultsNone of the ECG parameters showed significant correlation with 2D-LVM z-scores. In the detection of LVH, the most commonly used R in V6 voltages showed the following results: sensitivity 13–29%, specificity 77–96%, positive predictive value (PPV) 29–50%, and negative predictive value (NPV) 77%. The overall accuracy ranged 65–75%. The accuracy of adult indices for LVH ranged from 49% to 78%.Conclusions The ECG's prediction of LVH is unreliable with dismal sensitivity, variable specificity, poor PPV, and barely acceptable NPV. With such overall poor reliability, the use of current ECG standards in screening for LVH warrants reassessment.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12249
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    ABSTRACT: Objectives This study was designed to compare outcomes of the most common pediatric cardiac interventions from the time of implementation with the current era.Background Since the introduction of semilunar valve balloon dilation and device closure of the arterial duct and septal defects, development of interventional techniques and devices has been rapid. However, few studies have compared outcomes between those initial interventions and those in the current era.Methods Five validated common catheter-based therapies were chosen for analysis, including atrial and duct device closure, balloon dilation of the aortic and pulmonary valves, and native coarctation of the aorta. A retrospective review of the first and most recent 10 consecutive patients in each group was performed.ResultsThere was a high mortality (30%) among neonates who underwent aortic valve (AV) dilation in the early era, but no mortality noted in other groups. In the early era, transcatheter atrial defect closure and AV dilations were associated with a low success rate (60% for both lesions) and a high complication rate (40% for atrial septal defect, 30% for AV dilations). Among the last 10 children, the atrial defect occlusion was successful in 100% without complications and AV dilations where successful in all children with a 30% complication rate (one major, two minor).ConclusionsA learning curve with device development plays a significant role in the evolution of transcatheter techniques. These data provide baseline estimates of success and may be used as a template in the future when new techniques are adapted into practice.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12247
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    ABSTRACT: Objectives In patients with systemic right ventricles (RVs) in a biventricular circulation, exercise capacity and RV function often deteriorate over time and echocardiographic assessment of systemic RV function is difficult. The purpose of this study was to examine the relationship between exercise capacity and RV function and to determine which noninvasive imaging parameters correlate most closely with exercise capacity.DesignPatients with a systemic RV (D-loop transposition of the great arteries [TGA] after atrial switch procedure or physiologically “corrected” TGA) who underwent cardiopulmonary exercise testing (CPX) and noninvasive imaging (cardiac magnetic resonance [CMR] and echocardiography [echo]) within 1 year of CPX were identified. Regression analysis was used to evaluate the relationship between exercise variables and noninvasive indices of ventricular function.ResultsWe identified 92 patients with 149 encounters (mean age 31.0 years, 61% men, 70% D-loop TGA) meeting inclusion criteria. Statistically significant correlations between % predicted peak oxygen uptake (%pVO2) and RV ejection fraction (EF) (r = 0.29, P = .0007), indexed RV end-systolic volume (r = −0.25, P = .002), and Tei index (r = −0.22, P = .03) were found. In patients without additional hemodynamically significant lesions, the correlations between %pVO2 and RV EF (r = 0.37, P = .0007) and the Tei index (r = −0.28, P = .03) strengthened and a correlation emerged between %pVO2 and dP/dtic (r = 0.31, P = .007). On multivariable analysis, Tei index was the only statistically significant correlate of %pVO2 (P = .04).Conclusions In patients with systemic RVs in a biventricular circulation, CMR-derived RVEF and echo-derived Tei index correlate with %pVO2. On multivariable analysis, the Tei index was the strongest predictor of peak %pVO2 response.
    Congenital Heart Disease 02/2015; DOI:10.1111/chd.12248
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    ABSTRACT: Chylothorax complicates the postoperative course of patients after congenital heart surgery. Innominate vein thrombosis and stenosis have been associated with postoperative chylothorax. Revascularization and angioplasty can be accomplished using transcatheter techniques. We report our experience with this procedure for the management of postoperative chylothorax. This is a retrospective case series of patients who underwent catheter revascularization and/or angioplasty of the innominate vein following cardiac surgery at our institution from January 1, 2008 through April 9, 2014. The cardiovascular intensive care unit and cardiac catheterization laboratory at the University of Alabama at Birmingham and Benjamin Russell Hospital for Children in Birmingham, Alabama were used as settings for the study. Out of 112 patients with postoperative chylothorax, 7 (6.3%) underwent transcatheter dilation of the innominate vein for occlusion/stenosis. The median age of the cohort was 1 month (15 days-6 years); median weight was 3 kg (2.7-22.2). Diagnosis was made a median 8 days (2-20) and persisted for a median of 24 days (9-44). Most patients failed medical management (low fat diet, nothing by mouth, and/or octreotide). Cardiac catheterization occurred at a median 9 days (2-29) after chylothorax diagnosis. Median chest tube output on the day prior to procedure was 63 (12-149) cc/kg/day and decreased to 23 (0-64) cc/kg/day 2 days postprocedure (P = .01). Effusions resolved in a median of 5 days (1-16). There were no clinical complications postcatheterization. All patients who have undergone repeat angiography have maintained patency of the innominate vein. There was no mortality. Complications from chylothorax included prolong hospitalization, hyponatremia, hypoproteinemia, coagulopathy, lymphopenia, and infection. Innominate vein occlusion and stenosis associated with chylous effusion are amenable to transcatheter revascularization and/or angioplasty, consistently leading to improvement, if not full resolution of chylothorax. © 2015 Wiley Periodicals, Inc.
    Congenital Heart Disease 01/2015; DOI:10.1111/chd.12246
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    ABSTRACT: Background Single ventricle defects are among the most complex congenital heart defects and the development of advanced surgical procedures in recent decades has created the first generation of adolescents and young adults living with this condition. Yet little is known about how these individuals experience life and what impact the heart defect has on their life in general.Objective The aim was to illuminate and gain a deeper understanding of adolescents' and young adults' experiences of living with a surgically palliated univentricular heart.DesignSeven open-ended in-depth interviews were conducted, transcribed, and analyzed according to the henomenological hermeneutical method. All adolescents and young adults operated before 1995 according to the Fontan procedure or the total cavo-pulmonary connection procedure at one pediatric cardiology unit were included in the study. They were 17–32 years of age (median age 22 years).ResultsThe interpretation of the interview transcripts showed that the participants experienced living with a surgically palliated univentricular heart in terms of feeling exceptional, strong, and healthy. This was supported by two structural analyses, where three themes emerged: happiness over being me, focusing on possibilities, and being committed to life.Conclusion Living with a Fontan circulation included negative experiences but the analyses clearly demonstrated a feeling of being strong and healthy. An appreciation of having survived and being committed to life was found to be an integral part of the development of the interviewees' existential growth. This probably strengthens them further in their ability to balance expectations and hurdles in life. This study provides valuable insights into the experience of patients after the Fontan procedure and the importance of a positive health care environment throughout their lives.
    Congenital Heart Disease 01/2015; DOI:10.1111/chd.12244
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    ABSTRACT: Objective We investigated the type and extent of calcification in a series of heart specimens from adult congenital heart disease patients because recent autopsy observations suggested a high prevalence of calcification.DesignWe used computed tomography to examine seven heart specimens from adults (>18 years old) with a congenital heart defect collected with permission from the family during a recent 3-year period. Clinical data regarding diagnosis, history, and imaging studies were recorded. The 3D data sets were reviewed after reformatting as maximum intensity projection and volumetric renderings to determine the pattern and extent of calcium deposition.ResultsFive of the seven hearts had extensive calcifications in one or more of three patterns: atherosclerosis associated in the three oldest cases; surgery associated in four of five hearts that had undergone heart surgery; and myocardial calcification remote from surgical sites in two cases. Myocardial calcification was associated with regional dysfunction and was present in the three patients that died suddenly and unexpectedly.Conclusion Cardiac calcification was frequent in our series of heart specimens from adults with congenital heart defects, was often but not uniformly associated with prior surgery, and, in our small series, was associated with regional dysfunction and sudden death.
    Congenital Heart Disease 01/2015; DOI:10.1111/chd.12243
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    ABSTRACT: Objective Hypertrophic cardiomyopathy is underscored by profound phenotypic and genotypic heterogeneity. Echocardiographically, hypertrophic cardiomyopathy can be categorized into four morphological subtypes: reverse curve, sigmoidal, neutral contour, and apical variant. Previous studies indicate that reverse curve hypertrophic cardiomyopathy is the strongest predictor of a positive genetic test. Little is known about the spectrum and prevalence of mutations and genotype–phenotype correlations in apical hypertrophic cardiomyopathy.DesignBetween 1999 and 2007, 1053 patients with the diagnosis of hypertrophic cardiomyopathy (60% male, age at diagnosis 44.4 ± 19 years) underwent sarcomeric genetic testing. Blinded to the genetic test results, each echocardiogram was scored for septal morphology and phenotyping was performed using the patient's medical record. Subset analysis was performed to elucidate the genotype, phenotype, and outcome of apical hypertrophic cardiomyopathy.ResultsOverall, 71 patients (7%) had apical hypertrophic cardiomyopathy on echocardiography (63% male, mean age 47.8 ± 15 years, mean left ventricular wall thickness 19.8 ± 6 mm). Left ventricular outflow tract obstruction was uncommon (seven patients; 10%). Eighteen patients (25%) had a positive genetic test, with the majority of mutations found in MYBPC3 (six; 35%) and MYH7 (six; 35%). Follow-up was available on 68 patients (96%) with a median age of 57.3 years (range 19.3–82 years). Mean follow-up was 5.5 years (range 0.1–18.2 years). There was no statistical difference between the occurrence rates of adverse events between genotype-positive and genotype-negative groups.Conclusions In this largest cohort of patients with genetic testing for hypertrophic cardiomyopathy, <10% exhibited apical disease. This least common subtype was associated with a negative genetic test result 75% of the time. In contrast to prior publications suggesting a predilection for ACTC1/TPM1 mutations in patients with apical hypertrophic cardiomyopathy, the two most common genotypes (MYBPC3-HCM and MYH7-HCM) remained most common among patients who had a positive genetic test.
    Congenital Heart Disease 01/2015; DOI:10.1111/chd.12242
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    ABSTRACT: Objective Traditionally, karyotype and fluorescence in situ hybridization (FISH) were used for cytogenetic testing of infants with congenital heart disease (CHD) who underwent cardiac surgery at our institution. Recently, chromosome microarray analysis (CMA) has been performed in lieu of the traditional tests. A standardized approach to cytogenetic testing does not exist in this population. The purpose of this study was to assess the utility of CMA based on our current ordering practice.DesignWe reviewed the records of all infants (<1 year old) who underwent cardiac surgery at our institution from January 2010 to June 2013. Data included results of all cytogenetic testing performed. Diagnostic yield was calculated as the percentage of significant abnormal results obtained by each test modality. Patients were grouped by classification of CHD.ResultsTwo hundred seventy-five (51%) of 535 infants who underwent cardiac surgery had cytogenetic testing. Of those tested, 154 (56%) had multiple tests performed and at least 18% were redundant or overlapping. The utilization of CMA has increased each year since its implementation. The diagnostic yield for karyotype, FISH and CMA was 10%, 12%, and 14%, respectively. CMA yield was significantly higher in patients with septal defects (33%, P = .01) compared with all other CHD classes. CMA detected abnormalities of unknown clinical significance in 13% of infants tested.Conclusions In our center, redundant cytogenetic testing is frequently performed in infants undergoing cardiac surgery. The utilization of CMA has increased over time and abnormalities of unknown clinical significance are detected in an important subset of patients. A screening algorithm that risk-stratifies based on classification of CHD and clinical suspicion may provide a practical, data-driven approach to genetic testing in this population and limit unnecessary resource utilization.
    Congenital Heart Disease 01/2015; DOI:10.1111/chd.12241
  • Congenital Heart Disease 01/2015; 10(1). DOI:10.1111/chd.12245
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    ABSTRACT: Objective The purpose of this study was to determine the prevalence of growth restriction in infants and young children with congenital heart disease (CHD) and investigate the relationship between poor growth, feeding difficulties, cardiac classification, and nutrition intervention on outcomes.DesignThis is a prospective observational cohort study of infants and young children with CHD aged 0–3 years admitted to hospital for cardiac surgery. Anthropometry, growth history, cardiac classification, cardiac diagnosis, feeding difficulty, and nutrition intervention data were collected for 78 participants.ResultsMany participants demonstrated growth restriction as evidenced by a z-score ≤−2 for population growth parameters including weight/age z-score (n = 18, 23%), height/age z-score (n = 16, 21%), and weight/height z-score (n = 12, 18%). Increased hospital length of stay was associated with factors including faltering growth preadmission (P = .009), tube feeding required preadmission (P = .002), diagnosis of cyanotic CHD (P = .015), and presence of a feeding difficulty (P = .015).Conclusions Growth restriction remains an ongoing problem in children with CHD. Faltering growth preadmission and lower growth parameters were associated with an increased hospital length of stay. Nutritional screening from diagnosis may detect growth faltering, improve access to early nutrition intervention, and improve patient outcomes.
    Congenital Heart Disease 12/2014; DOI:10.1111/chd.12231
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    ABSTRACT: Background Many patients with congenital heart disease (CHD) acquire rhythm abnormalities related to their repair, most commonly intraatrial reentrant tachycardia (IART). Treatment of IART in CHD is often multifaceted, and may include medication, ablation, and pacing. Evidence regarding the use of antitachycardia pacing therapies is limited.Objective The aim of the study is to define the use and efficacy of antitachycardia pacing in patients with CHD at a single center.ResultsEighty implants were performed on 72 patients between 2000 and 2010. Follow-up data of more than 3 months were available for 56 patients; median follow-up time was 2.8 years. Twenty (36%) patients received successful antitachycardia pacing at a median 1.3 years postimplant. For those patients with IART after implant, antitachycardia pacing was successful in 57%. Patients with two-ventricle repairs were more likely to have successful antitachycardia pacing than those with one-ventricle palliation (45% vs. 17%, P = .04). Patients with documented IART had more successful antitachycardia pacing than those with no documented atrial tachycardia prior to implant (46% vs. 7%, P = .006). Early complications of antitachycardia pacemaker implant occurred in six patients (11%); late complications after implant occurred in three patients (5.6%). Of the initial 72 patients implanted, there were six deaths (8%).Conclusions Antitachycardia pacing therapies were successful in the majority of CHD patients who had IART after implant. Patients without documented atrial tachycardia prior to implant were unlikely to require or receive successful therapy from antitachycardia pacemaker. Those patients postatrial switch procedure who had documented IART prior to implant had the highest incidence of successful antitachycardia pacing therapies. Antitachycardia pacemaker implantation is an adjunct to the management of IART in CHD patients, but may not benefit patients who have not yet demonstrated IART.
    Congenital Heart Disease 12/2014; DOI:10.1111/chd.12230
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    ABSTRACT: AimsDiastolic dyssynchrony is increasingly being recognized as another marker for possible adverse cardiac events. Minimal data exist in hypoplastic left heart syndrome (HLHS) patients. The goal of this study was to determine if there were differences in diastolic dyssynchrony in patients with HLHS vs. control patients.Methods/ResultsTissue Doppler imaging (TDI) and strain rate (SR) analysis of the right ventricle in HLHS and control patients were performed. Time interval from onset of QRS complex on electrocardiography to peak TDI e′ wave was obtained. Differences in intervals were calculated: QRSe′(RV) − QRSe′(IVS) and QRSe′(RV) − QRSe′LV). Time interval from onset of QRS to peak strain rate early diastolic wave (SRe) was obtained for the six-segment model RV. Standard deviation of the six SRe time intervals was calculated. t-tests were performed to determine if differences were present between groups. Sixty patients were studied (35 HLHS, 25 control). There were no significant differences between HLHS and control patients in age (6.5 ± 3.2 years vs. 6.1 ± 2.6 years) or heart rate (91 ± 22 bpm vs. 91 ± 13 bpm), respectively. There were no significant differences between HLHS and control patients in QRSe′(RV) − QRSe′(IVS) (19.9 ± 15.4 ms vs. 23.3 ± 13.6 ms) and QRSe′(RV) − QRSe′(LV) (20.7 ± 13.8 vs. 22.8 ± 14.6 ms), respectively. There was a significant difference in SRe standard deviation between the HLHS and control patients (25.3 ± 12.4 ms vs. 15.5 ± 6.9 ms, P = .0007), respectively.Conclusion Patients with HLHS had increased diastolic dyssynchrony compared with control patients as measured via deformation analysis. Future studies are needed to determine the significance of these findings.
    Congenital Heart Disease 12/2014; DOI:10.1111/chd.12234
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    ABSTRACT: Public health research is an integral part of the study of congenital heart disease. While this type of research has become more popular, particularly over the past decade, it has a history that stretches back to almost the beginnings of pediatric cardiology as a field. This review aims to introduce the concepts and methodologies of public health and how they relate to congenital heart disease, describe some of the challenges of traditional research methods in congenital heart disease, describe the history of public health research, and demonstrate the relevance of public health research, particularly databases, to pediatric cardiology fellows.
    Congenital Heart Disease 12/2014; 9(6). DOI:10.1111/chd.12235
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    ABSTRACT: Objective Although simulation-based education is now commonly utilized in medicine, its use in the instruction of congenital heart disease remains limited. The objective of this study is to evaluate whether heart models created with three-dimensional printing technology can be effectively incorporated into a simulation-based congenital heart disease and critical care training curriculum for pediatric resident physicians.DesignUtilizing heart models created with a three-dimensional printer, pediatric residents participated in a 60-minute simulation seminar with three consecutive components: (1) didactic instruction on ventricular septal defect anatomy; (2) didactic/simulation-based instruction on echocardiographic imaging of ventricular septal defects and anatomical teaching/operative simulation of ventricular septal defect repair; (3) simulation-based instruction on postoperative critical care management of ventricular septal defects.SettingAcademic, free-standing, children's hospital with quaternary care referrals.ParticipantsTwenty-three pediatric resident physicians.Outcome MeasuresSubjective, Likert-type questionnaires assessing knowledge acquisition, knowledge reporting, and structural conceptualization of ventricular septal defects.ResultsThree-dimensional printing technology was successfully utilized to create heart models of five common ventricular septal defect subtypes. After using these models in a simulation-based curriculum, pediatric residents were found to have improvement in the areas of knowledge acquisition (P = .0082), knowledge reporting (P = .01), and structural conceptualization (P < .0001) of ventricular septal defects, as well as improvement in the ability to describe and manage postoperative complications in ventricular septal defect patients in the critical care setting.Conclusions The utilization of three-dimensional printing in a simulation-based congenital heart disease and critical care training curriculum is feasible and improves pediatric resident physicians' understanding of a common congenital heart abnormality.
    Congenital Heart Disease 12/2014; DOI:10.1111/chd.12238
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    ABSTRACT: Objective The objective of this study was to (1) describe the technical aspects of fluoroscopy-guided umbilical venous catheter placement (FGUVCP); and (2) determine the procedural success rate, factors contributing to procedural failure, and risks of the procedure.Background Umbilical venous catheters are advantageous compared with femoral venous access, but can be difficult to place at the bedside.Materials and Methods This was a retrospective chart review from a single tertiary care referral institution.ResultsFGUVCP was successful in 138 of 180 patients (76.7%) over a seven-year period. Patients in whom FGUVCP was successful were younger at the time of procedure compared with patients in whom FGUVCP was unsuccessful (median 18.2 vs. 22.2 hours, P = .03). The optimal age cutoff to predict FGUVCP success was 20 hours with a high positive predictive value (82.4%) but low negative predictive value (32.5%). No other variables were associated with procedural failure, though functional univentricular heart and older gestational age trended toward statistical significance. Median radiation time, contrast exposure, and blood loss were 3.2 minutes, 1 mL, and 1 mL, respectively. A total of 10 complications in 10 patients were associated with FGUVCP.ConclusionsFGUVCP is a safe and highly successful way to obtain central venous access in neonates with congenital heart disease. Older age at the time of procedure is associated with procedural failure, but utilization of an age cutoff may not be clinically useful.
    Congenital Heart Disease 12/2014; DOI:10.1111/chd.12233
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    ABSTRACT: Indications for sudden cardiac death (SCD) primary prevention are unknown in patients with repaired tetralogy of Fallot (ToF). The role of microvolt T-wave alternans (MTWA) in SCD risk stratification was documented. However, the prevalence of spectral MTWA and its association with ventricular arrhythmia (VA) in adults after ToF repair were not elucidated. Microvolt T-wave alternans, electrocardiogram (ECG), ambulatory ECG monitoring, echocardiography, and spiroergometry were evaluated in 102 adults after ToF repair. Microvolt T-wave alternans results were classified as normal: negative(-), abnormal: positive(+), and indeterminate(ind). Owing to similar prognostic significance, MTWA(+) and MTWA(ind) due to patient factors were combined into nonnegative group: MTWA(abnormal). Microvolt T-wave alternans(abnormal) was more frequent in the studied group as compared with controls (P = .0005). The MTWA(abnormal) group had greater right ventricular end-diastolic diameter (P = .005), higher incidence of pulmonary regurgitation (P = .015), lower peak oxygen consumption (P = .01), and higher VE/VCO2 slope (P = .04) in comparison with MTWA(normal). Univariate logistic regression proved pulmonary regurgitation (OR = 3.57, 95% CI 1.27-10.04), VA (OR = 3.26, 95% CI 1.06-10.05), right ventricular end-diastolic enlargement (OR = 1.11, 95% CI 1.03-1.2), increase in VE/VCO2 slope (OR = 1.08, 95% CI 1.01-1.17), and decrease in peak oxygen uptake (OR = .91, 95% CI 0.83-0.99) to increase MTWA(abnormal) prevalence. In adults after ToF repair, abnormal MTWA occurred more often than in controls. Probability of abnormal MTWA did not rise with prevalence of malignant VA; however, presence of abnormal MTWA was associated with VA risk factors: pulmonary regurgitation, right ventricular enlargement, and consequent heart failure. The role of MTWA in selecting patients late after ToF repair at risk of SCD needs further observation. © 2014 Wiley Periodicals, Inc.
    Congenital Heart Disease 11/2014; DOI:10.1111/chd.12240
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    ABSTRACT: Neurocardiogenic syncope (NCS) is the most common cause of syncope in children and adolescents. Neurocardiogenic syncope occurs secondary to cerebral hypotension because of bradycardia, hypotension, or both. Head-up tilt-table test (HUTT) is the primary diagnostic test. Near-infrared spectroscopy (NIRS) is a noninvasive technology that directly monitors trends in regional tissue oxygen saturations over a specific body region. Placing an NIRS probe over the temporal region allows an indirect measurement of cerebral perfusion. Our hypothesis is that regional tissue oxygen saturation will decrease during an NCS episode and will remain stable in patients without syncope. The investigators conducted a retrospective review of all HUTT utilizing cephalic NIRS performed at our institution from August 2012 to January 2013. Tests were classified as positive, negative, or psychogenic reactions. Paired t-test was used to determine statistical significance of NIRS changes and one-way analysis of variance was used to analyze baseline characteristics among the three groups. Twelve patients were included in the study (female = 10). The average age was 14.4 years (range: 12-17). Five tests were positive for NCS, four were negative, and three demonstrated psychogenic reactions. Patients with a positive test had a sudden, significant decrease in regional tissue oxygen saturations (P = .009) by an average of 11.3 ± 5.2% compared with baseline. The decrease in regional tissue oxygen saturation preceded symptoms, hypotension, and bradycardia in all patients. Regional tissue oxygen saturation levels remained stable in patients with a negative test or psychogenic syncope. NIRS monitoring during HUTT produces a reliable, positive result that precedes clinical signs and symptoms. Further, it helps distinguish NCS from psychogenic syncope. © 2014 Wiley Periodicals, Inc.
    Congenital Heart Disease 11/2014; DOI:10.1111/chd.12236