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: 1.08

Impact Factor Rankings

2016 Impact Factor Available summer 2017
2014 / 2015 Impact Factor 1.076
2013 Impact Factor 1.202
2012 Impact Factor 1.013
2011 Impact Factor 0.901

Impact factor over time

Impact factor
Year

Additional details

5-year impact 1.24
Cited half-life 3.80
Immediacy index 0.23
Eigenfactor 0.00
Article influence 0.47
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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    • 12 months embargo
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    • On author's personal website, institutional repositories, arXiv, AgEcon, PhilPapers, PubMed Central, RePEc or Social Science Research Network
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    • Non-Commercial
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    • Must link to publisher version with set statement (see policy)
    • If OnlineOpen is available, BBSRC, EPSRC, MRC, NERC and STFC authors, may self-archive after 12 months
    • If OnlineOpen is available, AHRC and ESRC authors, may self-archive after 24 months
    • Publisher last contacted on 07/08/2014
    • This policy is an exception to the default policies of 'Wiley'
  • Classification
    yellow

Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: The atria serve as reservoir, conduit, and active pump for ventricular filling. The performance of the atrial baffles after atrial switch repair for transposition of the great arteries may be abnormal and impact the function of the systemic right ventricle. We sought to assess atrial function in patients after atrial repair in comparison to patients after arterial switch repair (ASO) and to controls. Methods: Using magnetic resonance imaging, atrial volumes and functional parameters were measured in 17 patients after atrial switch repair, 9 patients after ASO and 10 healthy subjects. Results: After the atrial switch operation, the maximum volume of the pulmonary venous atrium was significantly enlarged, but not of the systemic venous atrium. In both patients groups, independently from the surgical technique used, the minimum atrial volumes were elevated, which resulted in a decreased total empting fraction compared with controls (P < .01). The passive empting volume was diminished for right atrium, but elevated for left atrium after atrial switch and normal for left atrium after ASO; however, the passive empting fraction was diminished for both right atrium and left atrium after both operations (P < .01). The active empting volume was the most affected parameter in both atria and both groups and active empting fractions were highly significantly reduced compared with controls. Conclusion: Atrial function is abnormal in all patients, after atrial switch and ASO repair. The cyclic volume changes, that is, atrial filling and empting, are reduced when compared with normal subjects. Thus, the atria have lost part of their capacity to convert continuous venous flow into a pulsatile ventricular filling. The function of the pulmonary venous atrium, acting as preload for the systemic right ventricle, after atrial switch is altered the most.
    No preview · Article · Dec 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: To determine whether implementation of a standardized clinical practice guideline (CPG) for the evaluation of syncope would decrease practice variability and resource utilization. Design: A retrospective review of medical records of patients presenting to our practice for outpatient evaluation of syncope before and after implementation of the CPG. The guideline included elements of history, physical exam, electrocardiogram, and "red flags" for further testing. Setting: Outpatient pediatric cardiology offices of a large pediatric cardiology practice. Patients. All new patients between 3 and 21 years old, who presented to cardiology clinic with a chief complaint of syncope. Interventions: The CPG for the evaluation of pediatric syncope was presented to the providers. Outcome Measures: Resource utilization was determined by the tests ordered by individual physicians before and after initiation of the CPG. Patient final diagnoses were recorded and the medical records were subsequently reviewed to determine if any patients, who presented again to the system, were ultimately diagnosed with cardiac disease. Results: Of the 1496 patients with an initial visit for syncope, there was no significant difference in the diagnosis of cardiac disease before or after initiation of the CPG: (0.6% vs. 0.4%, P=.55). Electrocardiography provides the highest yield in the evaluation of pediatric syncope. Despite high compliance (86.9%), there were no overall changes in costs ($346.31 vs. $348.53, P=.85) or in resource utilization. There was, however, a decrease in the variability of ordering of echocardiograms among physicians, particularly among those at the extremes of utilization. Conclusions: Although the CPG did not decrease already low costs, it did decrease the wide variability in echo utilization. Evaluation beyond detailed history, physical exam, and electrocardiography provides no additional benefit in the evaluations of pediatric patients presenting with syncope.
    No preview · Article · Dec 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: Atrial septal defect (ASD) is the second most common congenital heart defect (CHD) and is observed in families as an autosomal dominant trait as well as in nonfamilial CHD. Mutations in the NKX2-5 gene, located on chromosome 5, are associated with ASD, often combined with conduction disturbances, cardiomyopathies, complex CHD, and sudden cardiac death as well. Here, we show that NKX2-5 mutations primarily occur in ASD patients with conduction disturbances and heritable ASD. Furthermore, these families are at increased risk of sudden cardiac death. Results: We screened 39 probands with familial CHD for mutations in NKX2-5 and discovered a novel mutation in one family (2.5%) with ASD and atrioventricular block. A review of the literature revealed 59 different NKX2-5 mutations in 202 patients. Mutations were significantly more common in familial cases compared to nonfamilial cases (P = 7.1 × 10(-9) ). The majority of patients (74%) had ASD with conduction disturbance. Nineteen patients (15%) of 120 with familial ASD and conduction disturbance died from sudden cardiac death of which nine (8%) were confirmed mutation carriers, and 10 were possible carriers. Conclusions: NKX2-5 mutations mainly occur in familial CHD, the signature phenotype is ASD with conduction disturbances and mutation carriers are at increased risk of sudden cardiac death. We suggest that familial ASD patients should be screened for NKX2-5 mutations and, if they are mutation carriers, implantation of an implantable cardioverter-defibrillator should be considered in these patients.
    No preview · Article · Dec 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: The purpose of this study was to describe social-emotional outcomes and the relationship with neurodevelopmental outcomes in a cohort of 2-year-old children who underwent surgery for congenital heart disease (CHD) in infancy, and explore the relationship between the outcomes and parental and surgical factors. Design: A two-center prospective cross-sectional cohort study. Patients: A cohort of 105 2-year-olds who underwent surgery in infancy for severe CHD MEASURES: Social-emotional and neurodevelopment was evaluated with the Infant and Toddler Social and Emotional Assessment tool (ITSEA), and the Bayley Scales of Infant Toddler Development, Third Edition. Results: Neurodevelopment was delayed in the CHD cohort with significantly worse results compared to published Australian-based norms in all domains (P < .001) and in the Cognitive (P < .001) and Language (P < .001) domains with respect to the reported American norms. Social-emotional outcome was similar to Australian norms in all domains but better than the American based norms in the Internalizing domain (P < .05). Higher maternal education was associated to better neurodevelopmental outcome in all domains and better scores in the internalizing and externalizing domains of the ITSEA. There was a moderate correlation (r = 0.43, P < .001) between Language and social-emotional competence. Motor development was influenced by the need for a significant cardiac reoperation. Conclusions: The influences of social factors may be underestimated in the outcome of children with CHD. Language development in those with CHD may be improved with intervention targeting social-emotional competence; further research is needed in this area.
    No preview · Article · Dec 2015 · Congenital Heart Disease
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    ABSTRACT: Cardiology fellowship is filled with opportunities for learning; however, numerous obstacles must be successfully navigated to complete training with the knowledge, skills, and attitudes necessary for a successful career. To assist Cardiology fellows in prioritizing their time and energy in order to fully gain from their training, a list of recommendations with rationales has been provided.
    No preview · Article · Dec 2015 · Congenital Heart Disease
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    ABSTRACT: Aim: To determine if a transthoracic echocardiography (TTE) can be used as the sole diagnostic imaging modality to evaluate children with congenital heart disease (CHD) undergoing cardiac surgery. Methods: A retrospective study was carried out at the King Abdulaziz Cardiac Center. We reviewed all pediatric patients who underwent cardiac surgery during the period January 2011 to December 2011. Results: Three hundred ninety-one pediatric patients with CHD fulfilled the inclusion criteria. Of these patients, 287 (73%) underwent surgical interventions based on a TTE alone, while 105 (27%) required additional diagnostic imaging modalities, including a cardiac catheterization (68/105; 65%), cardiac computed tomography angiography (36/105; 35%), or cardiac magnetic resonance imaging (1/105; 1%). A TTE was not enough for all the patients who underwent a cardiac catheterization to find out additional anatomical information (22%), either to directly measure pulmonary artery pressures (62%) or to study vascular reactivity in patients with pulmonary hypertension (16%). Of 36 patients who underwent a cardiac computed tomography angiography, five (14%) had additional information to be added to TTE findings. Of all the patients, 81% had enough information using only the TTE compared to 19% in whom the TTE was not enough to provide all needed information. Only seven of 392 (0.02%) patients had additional minor intraoperative findings that did not affect the surgical decision. Conclusion: Despite the emergence of other imaging modalities, a TTE can be used as the sole diagnostic imaging modality for a preoperative assessment in the majority of children with CHD. Other imaging modalities can be employed with limited indications.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Objectives: To evaluate the use of smartphone and tablet technology ("smart technology" or ST) in remote consultation for pediatric and congenital cardiac catheterization. Design: Two online surveys, to early-career (Mentees) and senior (Mentors) pediatric interventional cardiologists, designed to assess the current use and attitudes toward ST for remote consultation. In addition, the current literature is reviewed for use of remote consultations and potential legal and patient privacy concerns. Results: Forty-six (40%) Mentees and 19 (48%) Mentors responded. 74% of Mentees report using ST for remote consultation, 26% for nearly every case, with 45% of communication occurring during a procedure. Over one-third of Mentees report they have performed an intervention for the first time based on ST consultation. Of Mentors that reported receiving ST consultation, 58% were comfortable providing advice via this method, but 42% felt that the imaging sent was inadequate to provide appropriate advice for at least one case. Both groups felt the speed of consultation was much faster using ST. Privacy measures were reportedly used in up to 85% of consultations, while only 12% of respondents obtained informed consent for the consultation. The most common barrier identified by Mentees for use of ST for consultations was patient privacy concerns. Conclusions: ST use is prevalent among early-career pediatric and congenital interventional cardiologists for consultation regarding catheterization procedures. There are many technologic, legal, and patient privacy concerns that will need to be addressed as the use of ST for remote consultation grows.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: No abstract is available for this article.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: The aim of this study was to compare the functional capacity of children with TGA long-term after ASO with that of healthy children. Design: Retrospective study. Patients: All patients that performed cardiopulmonary exercise test (CPET) were included in the study. As a control group, healthy children in evaluation for physical activity that performed CPET during the same period were also enrolled. Results: Thirty-one TGA patients (19 male) were compared with 29 age-matched controls (21 male). Maximum oxygen consumption was higher in the control group (45.47 ± 8.05 vs. 40.52 ± 7.19, P = .017), although within normal limits in both groups (above 90% of predicted value). The heart rate behavior during exercise was different in both groups, with a mean chronotropic index significantly lower in the TGA group (63% ± 14 vs. 81% ± 12, P < .001). Conclusions: Our results showed that exercise capacity long-term after ASO in TGA is well preserved although lower than in healthy children what might be explained by the presence of chronotropic incompetence in the TGA group.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Background: Percutaneous closure of atrial septal defect (ASD) has become the preferred method in treatment of the majority of cases. The aim of this study was to evaluate the echocardiographic effects of percutaneous closure of secundum ASD in adults and assess which parameters predict good response to closure. Design: We prospectively included 42 patients with secundum ASD treated successfully with percutaneous device closure. All patients underwent transthoracic echocardiography examination with tissue Doppler imaging before, 24 hours after and within 3 months of intervention. Measurements of arterial stiffness were carried out using a Mobil-O-Graph arteriography system. Results: Remodeling of heart chambers occurred immediately and persisted at 3 months after ASD closure. Significant decreases were observed in right ventricle (RV) end-diastolic diameter, right atrium volume index, and tricuspid annular plane systolic excursion both after the procedure and at 3 months (P < .01 for all). The RV and left ventricle (LV) tissue Doppler-myocardial performance index demonstrated to decline during follow up (P = .0001). Significant correlations were found between pulse-wave velocity, augmentation index, pulmonary artery stiffness, and LV-RV tissue Doppler-myocardial performance index at third month. Linear regression analyses showed that pulse-wave velocity is the most effective parameter of LV and pulmonary artery stiffness is the most effective parameter of RV functional recovery, respectively, assessed by tissue Doppler-myocardial performance index. Conclusions: Percutaneous closure of secundum ASD in adults has various sustained benefits on multiple echocardiographic parameters within 3 months. The changes in RV and LV function after device closure were significantly correlated with the degree of pulmonary artery stiffness and pulse-wave velocity, respectively.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Background: Lymphopenia is common in patients with Fontan circulation and no history of protein-losing enteropathy, but this phenomenon has not been significantly described in the literature. Methods: We retrospectively identified patients with Fontan circulation who underwent catheterization between January 2003 and January 2013 at our center. Patients who had complete blood count with differential drawn within 12 months of the catheterization were included. Patients were excluded if complete blood count with differential was drawn in setting of possible infection or if there was history of protein-losing enteropathy (PLE). Possible associations between patient characteristics and absolute lymphocyte count (ALC) were examined. Results: Fifteen patients were included. The median age at catheterization was 10.2 years (3.8-26.9) and median time from Fontan operation was 6.5 years (0.7-22.1). Twelve (80%) patients had undergone extracardiac Fontan and 9 (60%) had fenestration placed. The median time between complete blood count with differential and catheterization was 2 days (0-346). The median inferior vena cava (IVC) pressure was 13 mm Hg (7-20). The median ALC was 1.5 × 10(3) /μL (0.8-4.5). Four patients (26.7%) met criteria for lymphopenia with ALC < lower limit of normal and 7 (46.7%) patients had an ALC ≤ lower limit of normal. ALC was not associated with any hemodynamic variables but was associated with platelet count (rho = 0.5, P = .04), total white blood cell count (rho = 0.8, P ≤ .001), and absolute monocyte count (0.7, P = .002). Conclusions: In a cohort of patients with Fontan circulation and no history of protein-losing enteropathy who underwent catheterization, lymphopenia was common and positively associated with low platelet count. Thrombocytopenia has been shown to correlate with the degree of hepatic fibrosis in those with Fontan and, thus, hepatic fibrosis may underlie lymphopenia in these patients.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Transparency-sharing data or information about outcomes, processes, protocols, and practices-may be the most powerful driver of health care improvement. In this special article, the development and growth of transparency within the National Pediatric Cardiology Quality Improvement Collaborative is described. The National Pediatric Cardiology Quality Improvement Collaborative transparency journey is guided by equal numbers of clinicians and parents of children with congenital heart disease working together in a Transparency Work Group. Activities are organized around four interrelated levels of transparency (individual, organizational, collaborative, and system), each with a specified purpose and aim. A number of Transparency Work Group recommendations have been operationalized. Aggregate collaborative performance is now reported on the public-facing web site. Specific information that the Transparency Work Group recommends centers provide to parents has been developed and published. Almost half of National Pediatric Cardiology Quality Improvement Collaborative centers participated in a pilot of transparently sharing their outcomes achieved with one another. Individual centers have also begun successfully implementing recommended transparency activities. Despite progress, barriers to full transparency persist, including health care organization concerns about potential negative effects of disclosure on reputation and finances, and lack of reliable definitions, data, and reporting standards for fair comparisons of centers. The National Pediatric Cardiology Quality Improvement Collaborative's transparency efforts have been a journey that continues, not a single goal or destination. Balanced participation of clinicians and parents has been a critical element of the collaborative's success on this issue. Plans are in place to guide implementation of additional transparency recommendations across all four levels, including extension of the activities beyond the collaborative to support transparency efforts in national cardiology and cardiac surgery societies.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Background: Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. Methods: A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values were provided. AHr values were recorded for patients and compared with z-scores results obtained with Gautier's and Campens's nomograms. Results: AHr values in the group of normal subjects were 2.6 ± 0.6 at SoV and 2 ± 0.5 at pAA. Categorization of z-scores and AHr showed good correspondence between AHr and Gautier's method (P = .341 at SoV and .185 at pAA) and AHr and Campens method (P =.465 at SoV and 0.110 at pAA). Conclusions: There was a good correspondence of AHr results with two different z-scores. AHr is a simple to use and valid option to quantify aortic root dilatation in pediatric patients.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Background and aims: Patients who underwent a successful repair of the aortic coarctation show chronic hyperdynamic state and normal left ventricular (LV) geometry; however, there are few data regarding the LV systolic function in the long term. Accordingly, we assessed LV systolic mechanics and factors associated with LV systolic dysfunction (LVSD) in patients with repaired CoA. Methods: Clinical and echocardiographic data from 19 repaired CoA were analyzed 28 ± 13 years after surgery. Stress-corrected midwall shortening (sc-MS) and mitral annular peak systolic velocity (S') were analyzed as indexes of LV circumferential and longitudinal systolic function, respectively. Echocardiographic data of CoA patients were compared with 19 patients matched for age and hypertension and 38 healthy controls. Sc-MS was considered impaired if <89%, S' if <8.5 cm/s (10th percentiles of healthy controls, respectively). Results: There were no statistical differences between study groups in LV volumes, mass and geometry. LV ejection fraction and Sc-MS were similar in all groups, however, CoA group had a significantly lower peak S' in comparison with matched and healthy controls (7.1 ± 1.3, 10.3 ± 1.9, and 11.1 ± 1.5, respectively; all P < 0.001). Prevalence of longitudinal LVSD defined as low S' was 84% in CoA, 13% in matched, and 5% in healthy control group (all P<0.05). Multivariate logistic regression analysis revealed that low peak S' was independently related to higher E/E' ratio and the presence of CoA. Conclusions: Patients who underwent a successful repair of CoA commonly show asymptomatic longitudinal LVSD associated with worse LV diastolic function in the long-term follow-up.
    No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. Design: A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. Setting: Academic and community outpatient cardiology clinics. Patients: Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. Intervention: None. Outcomes measures: The compliance with health maintenance and transfer of care recommendations in the outpatient setting. Results: For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. Conclusion: Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists.
    No preview · Article · Nov 2015 · Congenital Heart Disease

  • No preview · Article · Nov 2015 · Congenital Heart Disease
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    ABSTRACT: Objective: There is a paucity of data on clinical correlates and outcomes of pulmonary hypertension (PH) in patients with D-transposition of the great arteries (D-TGA) in the era of inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Our objective was to compare clinical characteristics and outcomes of infants with D-TGA with and without PH, defined as hypoxemia that required iNO and/or ECMO. Methods: We undertook a single-center retrospective chart review involving infants with gestational age ≥32 weeks with D-TGA who, underwent arterial switch operation over a 12-year period. Demographic and clinical data, details of the repair and postoperative complications were abstracted. Results: Our cohort (n = 93), 61 (66%) of whom were males, had a mean (SD) gestational age and birth weight of 38.7 (1.8) weeks and 3.2 (0.6) kg, respectively. PH requiring iNO and/or ECMO was noted in 20 (21.5%) infants. Infants with PH had significantly lower birth weight [2.8 (0.56) vs. 3.33 (0.61)] and gestational age [37.7 (2.1) vs. 38.9 (1.7)] than those without PH. Rates of postoperative complications (duration of pressors, sedative medicaiton and duration of hospital stay, and mechanical ventilation were higher in the group with PH. Of the five (5.4%) infants who died, four received iNO and ECMO. Death or postoperative complications tended to be associated with lower gestational age [OR 0.689; 95% CI: 0.469-1.012, P = 0.058] but not with D-TGA category or bypass duration. Conclusions: Despite aggressive treatment with iNO and ECMO, the coexistence of PH in this population is associated with higher rates of mortality and postoperative complications. Our results also suggest that an early term birth may be associated with PH in infants with D-TGA.
    No preview · Article · Nov 2015 · Congenital Heart Disease