Congenital Heart Disease

Publisher: Blackwell Publishing

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.

  • Impact factor
    1.01
  • 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

Blackwell Publishing

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    • Author can archive a pre-print version
  • Post-print
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    • no listing of affected journals available as yet
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    • See Wiley-Blackwell entry for articles after February 2007
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    • Articles in some journals can be made Open Access on payment of additional charge
    • 'Blackwell Publishing' is an imprint of 'Wiley'
  • Classification
    ​ yellow

Publications in this journal

  • [Show abstract] [Hide abstract]
    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;
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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;
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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;
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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;
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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;
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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;
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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;
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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;
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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;
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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;
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    ABSTRACT: Objective High-fidelity patient simulation (HFPS) has been used in medical education to bridge gaps in medical knowledge and clinical skills. Few studies have analyzed the impact of HFPS in subspecialty rotations for pediatric residents. We hypothesized that pediatric residents exposed to HFPS with a structured content curriculum would perform better on a case quiz than residents without exposure to HFPS. Design Prospective randomized controlled Setting Tertiary-care free standing children's hospital Interventions During a cardiology rotation, senior pediatric residents completed an online pediatric cardiology curriculum and a cardiology quiz. After randomization into two groups, the study group participated in a fully debriefed HFPS session. The control group had no HFPS. Outcome Measure Both groups completed a case quiz. Confidence surveys pre- and postsimulation were completed. Results From October 2010 through March 2013, 55 residents who rotated through the pediatric cardiology rotation were used in the final analysis (30 control, 25 in the study group). There was no significant difference between groups on the initial cardiology quiz. The study group scored higher on the case quiz compared with the control group (P = .024). Based on pre- and postsimulation questionnaires, residents' confidence in approaching a pediatric cardiology patient improved from an average Likert score of 5.1 to 7.5 (on scale of 0–10) (P < .001). Conclusions Incorporation of HFPS into a preexisting pediatric cardiology rotation was feasible and well received. Our study suggests that simulation promotes increased confidence and may modestly improve clinical reasoning compared to traditional educational techniques. Targeted simulation sessions may readily be incorporated into pediatric subspecialty rotations.
    Congenital Heart Disease 11/2014;
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    ABSTRACT: Objectives We sought to review our current philosophy that all primary invasive electrophysiologic (EP) studies in patients with atrial switch procedures (ASPs) should undergo hemodynamic evaluation and have interventional expertise available.Background Patients who have undergone an ASP for dextro-transposition of the great arteries have a high incidence of both hemodynamic and EP sequelae. We present our data to support the combined assessment approach for these patients.Methods Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed.ResultsA total of 18 patients underwent concurrent EP invasive procedure and cardiac catheterization. The median age was 31 (14–43 years) with the majority being male (67%). Patients underwent a total of 30 concurrent primary invasive EP procedure and cardiac catheterization; 14 (47%) of the catheterization procedure were interventional. Some of the catheterization procedures required more than one intervention with total of 19 separate interventions. There were nine (47%) unexpected interventions. The majority of patients (n = 14, 77.8%) had one or more abnormal hemodynamic finding including baffle obstruction (n = 13, 72%), elevated filling pressures (n = 3, 17%), and secondary pulmonary hypertension (n = 3, 17%). Non-EP–related interventional procedures included systemic or pulmonary venous baffle stenting for significant obstruction (n = 7). EP-related interventions included transbaffle puncture for ablation of left-sided reentry circuits (n = 5), closure of previously undiagnosed baffle leaks prior to pacemaker/implantable cardioverter defibrillator (ICD) placement to prevent paradoxical embolism (n = 3), superior baffle stenting to facilitate pacemaker/ICD lead placement (n = 2), and retrieval of retained transvenous pacemaker/ICD lead (n = 2).Conclusion Due to the frequency of abnormal hemodynamics or interventional needs, strong consideration for routine concurrent hemodynamic assessment and availability of interventional expertise is warranted during primary invasive EP procedures in patients post ASP.
    Congenital Heart Disease 11/2014;
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    ABSTRACT: Objective The aim of this study was to compare the effects of anesthesia by dexmedetomidine and propofol on the hemodynamics and oxygen balance in children with complex congenital heart disease who were undergoing cardiac surgery.Methods Fifty-seven children were randomized to receive either a continuous infusion of propofol (6–8 mg/kg/h) or dexmedetomidine (0.5–0.7 μg/kg/h) after anesthesia induction. Hemodynamic data were recorded. Oxygen balance parameters were assessed at baseline after midazolam sedation, before and immediately after skin incisions were made, after sternotomy, 5 minutes after protamine administration, and at the end of surgery.ResultsCompared with the dexmedetomidine group, the propofol group exhibited decreases in blood pressure, cardiac output, and cardiac index before skin incision (P < .05) and increases in blood pressure, heart rate, cardiac output, and cardiac index after sternotomy (P < .01). However, very similar trends in oxygen dynamics were obtained in both groups (P > .05), and the cardiac index was not correlated with total oxygen consumption (r = −0.109, P = .066) or the oxygen extraction ratio (r = −0.107, P = .072).Conclusions Dexmedetomidine infusion may be superior to propofol anesthesia in children with complex congenital heart disease who are undergoing cardiopulmonary bypass because dexmedetomidine was associated with less variability in heart rate or blood pressure during surgery. However, the oxygen balance was similar when either agent was used.
    Congenital Heart Disease 11/2014;
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    ABSTRACT: Background Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist.Objective This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD.Methods This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed.ResultsThe cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%.Conclusions Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.
    Congenital Heart Disease 11/2014;
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    ABSTRACT: Background Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth.MethodsA previously identified Nutritional Bundle was spread among NPC-QIC sites. Primary outcome: interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth.ResultsFour hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008–December 2010) and 249 in Period 2 (December 2010–April 2013). Median age at S2 was 4.9 months (2.6–12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ <0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was <0 in six sites while in Period 2 no site had median interstage ΔWAZ <0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively).Conclusions Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.
    Congenital Heart Disease 11/2014;
  • Congenital Heart Disease 11/2014; 9(6):497.
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    ABSTRACT: Background The prognostic value of cardiopulmonary exercise testing (CPX) for mortality risk remains controversial in Fontan patients.PurposeOur goal of the present study was to clarify the prognostic value of major CPX variables and the factors determining exercise capacity in a large cohort of Fontan patients.Methods Since 1990, heart rate (HR), oxygen uptake (VO2), and ventilatory equivalent for carbon dioxide production (VE/VCO2) at peak exercise were determined in 335 Fontan patients (18 ± 5 years old), and the CPX variables were compared with the clinical profile and events.ResultsWhen compared with 209 controls, peak HR (148 ± 24 bpm), VO2 (27 ± 7 mL/kg/minute, [61 ± 15%]), and VE/VCO2 (40 ± 8 [117 ± 24%]) were markedly impaired in the Fontan patients (P < .0001). During a follow-up of 3.1 ± 2.7 years, 62 Fontan pathophysiological-associated events requiring unscheduled hospitalization (USH) occurred, and 24 patients died. All the CPX variables predicted the USH and mortality (P < .001–.0001). On multivariate analysis, in addition to use of diuretics (P = .0007) and low cardiac index (P = .0426), peak VO2 independently predicted the USH (hazard ratio: 0.95 per %, 95% confidence interval: 0.91–0.99, P = .014), while for mortality, multivariate analysis revealed that, in addition to heterotaxy syndrome (P = .0128) and year at first Fontan operation (P = .0532), peak VO2 independently predicted mortality (hazard ratio: 0.88 per %, 95% confidence interval: 0.76–0.98, P = .0217).Conclusion Fontan patients exhibit markedly impaired CPX variables, and all the major variables, especially peak VO2, predicted the risk of both morbidity and mortality.
    Congenital Heart Disease 10/2014;
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    ABSTRACT: Background Adolescents and young adults with congenital heart disease (CHD) are often restricted from physical activity and sports participation, which may have adverse effects.Objectives To determine the amount of physical activity, type of sports participation, and reasons for sports restrictions, and to evaluate the effect of sports participation on quality of life (QoL) in a cohort of patients with CHD.Methods Individuals with CHD aged 13–30 years were recruited at outpatient visits or via mailings. They completed a questionnaire addressing physical activity, sports participation, sports restrictions, and QoL (Pediatric Quality of Life Inventory). We also reviewed the patient's medical record.ResultsOf the 177 patients who responded (mean age 20 years), 31% have mild CHD, 40% have moderate CHD, and 29% have severe CHD. In the cohort, 52% participate in competitive sports, 25% recreational sports, and 23% no sports. Among patients with severe CHD, 29% participate in competitive sports that would be restricted by published guidelines (36th Bethesda Conference). After controlling for age, sex, CHD severity, residual hemodynamic disease, and comorbidities, participation in competitive sports and increased frequency of physical activity are independently associated with a higher QoL (P = .003 and P = .001, respectively). In an identical model, competitive sports participation and frequency of physical activity are associated with higher maximum predicted oxygen consumption (VO2) (n = 40; P = .002 and .02) and slightly lower body mass index (BMI) (P = .02 and .01). All findings were similar when analyses were stratified by recruitment method.Conclusions Patients with CHD commonly participate in competitive sports, and such participation is associated with higher QoL, improved exercise capacity, and lower BMI.
    Congenital Heart Disease 10/2014;
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    ABSTRACT: Background The evaluation of right ventricular (RV) function is important in patients with hypoplastic left heart syndrome (HLHS). Echocardiographic qualitative grading has been the prevalent method used in the past, but newer technologies allowing for quantitative assessment of RV function may have changed this fact. The goal of this study was to determine the current routine noninvasive evaluation of patients with HLHS and what, if any, methods are used to assess systolic and diastolic function in this population.Methods Web-based survey was conducted using various listservs. Timing of echocardiograms between surgical stages was assessed. Methods of assessing systolic and diastolic function were evaluated.ResultsTwo hundred seventy-seven physicians who averaged 12.8 ± 9.6 years removed from training responded. Largest percentage of respondents was echocardiographers (44.2%) in a university-based practice (73.3%) from North America (91.7%). There were 54.3% of respondents who performed echocardiograms monthly between stages I and II, 48.8% who performed echocardiograms every 6 months between stages II and III, and 67.0% who performed echocardiograms annually after stage III procedure. The main method for systolic grading was qualitative grading (95.5%) and for diastolic grading were tricuspid blood inflow velocities (56.8%). Qualitative grading was considered the method of choice for systolic grading for 38.8% of respondents and tissue Doppler velocities was the method of choice for diastolic grading for 35.3% of respondents. There were 4.0% of respondents who routinely perform a cardiac magnetic resonance imaging (cMRI) between stages I and II, 8.0% between stages II and III, and 24.2% after stage III procedure.Conclusion Variability in the noninvasive assessment of the RV in patients with HLHS continues to exist. Qualitative RV systolic assessment was still the predominant method used to assess function despite newer imaging techniques to allow for quantification. Future studies are needed to determine which values are most useful in reviewing function in this complex patient population.
    Congenital Heart Disease 10/2014;
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    ABSTRACT: Objective Congenital heart defects (CHDs) affect a large number of newborns and account for a high proportion of infant mortality worldwide. There are regional differences in the prevalence and distribution pattern of CHDs. The aim of this study is to estimate the distribution pattern and prevalence of CHDs among the population of north-central India and to compare the results with studies in other regions of the country to get an overview of prevalence of CHDs in India.DesignWe carried out a prospective study in the outpatient department of a tertiary care referral center in north-central India. This study was carried out from January 2011 to April 2014, with 34 517 individuals being recruited for the study. All patients were examined by chest x-ray, electrocardiogram, and 2D echocardiography. Prevalence rate per 1000 individuals examined was calculated. Relative frequencies of individual CHD types as a proportion of total CHDs were also calculated.ResultsOut of 34 517 individuals examined, 661 were diagnosed with CHDs, giving a prevalence of 19.14 per 1000 individuals. The most common defect was ventricular septal defect (33%), followed by atrial septal defect (19%) and tetralogy of Fallot (16%). The majority of CHD cases (58%) diagnosed were between 0 and 5 years of age. The prevalence of CHDs in adults was 2.4 per 1000 individuals in this cohort, with atrial septal defect (44.5%) being the most frequent defect.Conclusion The prevalence of CHDs in our cohort was high, possibly because of the power of the diagnostic methods we used and the inclusion of all age groups. Adults with CHDs may significantly contribute to the prevalence of CHDs in the next generation, and this needs to be considered when estimating prevalence rates. Although several small regional studies have been carried out in India, there is an urgent need to establish a nationwide registry/database for congenital heart defects.
    Congenital Heart Disease 10/2014;