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Neurocognitive and Psychological Outcomes in Adults With Dextro-Transposition of the Great Arteries Corrected by the Arterial Switch Operation.

Ann Thorac Surg. 2018 03;105(3):830-836

Authors: Kasmi L, Calderon J, Montreuil M, Geronikola N, Lambert V, Belli E, Bonnet D, Kalfa D

BACKGROUND: Neurodevelopmental impairments have frequently been described in children and adolescents with dextro-transposition of the great arteries (d-TGA). The arterial switch operation (ASO) to correct d-TGA has been used for more than 30 years, and more than 90% of these patients now reach adulthood. However, very little is known about their long-term functional outcomes. The present study investigated neurocognitive outcomes and the prevalence of psychiatric disorders in adults with d-TGA corrected by ASO.
METHODS: Neurocognitive functioning was comprehensively assessed (general intellectual functioning, language, attention, visual-spatial skills, executive functions, memory) in 67 adults (59.7% men) with d-TGA (aged 22.9 ± 3.4 years) and in 43 healthy individuals. The prevalence of psychiatric disorders, including depression and anxiety, was evaluated using a structured diagnostic interview. We also analyzed patient- and operative-related risk factors associated with outcomes.
RESULTS: Compared with the general population and the control group, adults with d-TGA displayed reduced performance in tasks assessing attention, visual-spatial skills, executive functions, and memory (all p < 0.05). Compared with controls, patients had also a higher lifetime prevalence of depression (43% vs 19%, p = 0.008) and anxiety disorders (54% vs 33%, p = 0.025). Predictors of long-term outcomes included gender and parental socioeconomic and educational status (all p < 0.05).
CONCLUSIONS: Adults who have undergone a neonatal ASO to correct d-TGA have an increased risk of cognitive deficits and psychiatric disorders. Evaluation of long-term neuropsychological and psychosocial outcomes in early adulthood is a crucial step to anticipate for adapted treatment strategies in adults with congenital heart disease.

PMID: 29033017 [PubMed - indexed for MEDLINE]

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Lateral thoracotomy for epicardial pacemaker placement in patients with congenital heart disease.

Interact Cardiovasc Thorac Surg. 2018 05 01;26(5):845-851

Authors: Haight PJ, Stewart RE, Saarel EV, Pettersson GB, Najm HK, Aziz PF

OBJECTIVES: Our institution adopted a lateral thoracotomy approach to epicaridal pacemaker implantation with the objective of avoiding epicardial scar tissue and to achieve adequate lead pacing and sensing. We sought to assess the short-term outcomes of this approach.
METHODS: A single-centre review was conducted in paediatric patients and adults with congenital heart disease or inherited arrhythmia syndromes who underwent a lateral thoracotomy for epicardial pacemaker placement from August 2010 to January 2016. Patient histories were recorded along with outcomes including complications, lead and generator performance.
RESULTS: Twenty-one operations were performed in 20 patients (median age 17 years, range 3 months-49 years), including 19 pacemakers and 2 implantable-cardioverter defibrillators (ICDs). Prior epicardial pacemakers had been placed in 11 (55%) patients, including 3 with multiple pacemakers. Most patients had undergone at least 1 prior cardiac operation, with a mean of 3.2 (range 0-7) prior cardiac operations. Through our lateral thoracotomy approach, 17 of the 19 attempted atrial leads (89%) and 20 of the 20 attempted ventricular leads (100%) were successfully implanted with acceptable pacing thresholds. Complications included 1 (5%) bleeding, 2 (10%) pacemaker pocket revisions and 1 late death at 6 months unrelated to the pacemaker. There were no lead failures at a mean follow-up period of 27.5 months (range of 0.7-56.1 months).
CONCLUSIONS: The lateral thoracotomy is a useful approach for epicardial pacemaker implantation in patients with congenital heart disease or inherited arrhythmia syndromes including those with multiple prior operations.

PMID: 29300890 [PubMed - indexed for MEDLINE]

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Comparison between Three-Dimensional Navigator-Gated Whole-Heart MRI and Two-Dimensional Cine MRI in Quantifying Ventricular Volumes.

Korean J Radiol. 2018 Jul-Aug;19(4):704-714

Authors: Goo HW

Objective: To test whether the method utilizing three-dimensional (3D) whole-heart MRI has an additional benefit over that utilizing conventional two-dimensional (2D) cine MRI in quantifying ventricular volumes.
Materials and Methods: In 110 patients with congenital heart disease, a navigator-gated, 3D whole-heart MRI during end-systole (ES) and end-diastole (ED), 2D short-axis cine MRI, and phase contrast MRI of the great arteries were acquired. Ventricular volumes were measured by using a 3D threshold-based segmentation for 3D whole-heart MRI and by using a simplified contouring for 2D cine MRI. The cardiac trigger delays of 3D whole-heart MRI were compared with those of a 2D cine MRI. The stroke volumes calculated from the ventricular volumes were compared with the arterial flow volumes, measured by phase contrast MRI.
Results: The ES and ED trigger delays of whole-heart MRI were significantly less than cine MRI for both the left ventricle (-16.8 ± 35.9 ms for ES, -59.0 ± 90.4 ms for ED; p < 0.001) and the right ventricle (-58.8 ± 30.6 ms for ES, -104.9 ± 92.7 ms for ED; p < 0.001). Compared with the arterial flow volumes, 2D cine MRI significantly overestimated the left ventricular stroke volumes (8.7 ± 8.9 mL, p < 0.001) and the 3D whole-heart MRI significantly underestimated the right ventricular stroke volumes (-22.7 ± 22.9 mL, p < 0.001).
Conclusion: Three-dimensional whole-heart MRI is often subject to early timing of the ED phase, potentially leading to the underestimation of the right ventricular stroke volumes.

PMID: 29962876 [PubMed - indexed for MEDLINE]

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Relating exercise-induced desaturation and gas-exchange in pulmonary artery hypertension.

Respir Physiol Neurobiol. 2019 01;259:58-62

Authors: Balmain BN, Seale H, Harris J, Hall K, Lin ACW, Sabapathy S, Morris NR

We measured pulmonary gas exchange during six minute walk test (6MWT) in patients with idiopathic (IPAH) and congenital heart disease-related pulmonary hypertension (CHDPH), and determined the relationship between the degree of desaturation and changes in minute ventilation to carbon dioxide production V˙EV˙CO2 and end-tidal partial pressure for carbon dioxide (PetCO2) in both groups. Fifty-two patients (IPAH, n = 28; CHDPH, n = 24) completed 6MWT with simultaneous pulmonary gas exchange. Whilst no significant difference in six minute walk distance was observed between groups (IPAH: 529 ± 89; CHDPH: 476 ± 119 m, p = 0.10), oxygen uptake, carbon dioxide production, and minute ventilation were higher in IPAH than CHDPH (p < 0.01). In addition, CHDPH desaturated to a greater extent than IPAH with a lower post-6MWT SPO2 (IPAH: 92.3 ± 7.9; CHDPH: 73.3 ± 14.9%, p < 0.01). The change in SpO2 correlated to the change in V˙E/V˙CO2 (r=-0.44, p = 0.02) and PetCO2 (r = 0.49, p < 0.01) for CHDPH, but not IPAH. The exercise-induced hypoxic stimulus during 6MWT in CHDPH may be associated with an enhanced ventilatory response.

PMID: 30048753 [PubMed - indexed for MEDLINE]

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Should we forget about valve area when assessing aortic stenosis?

Heart. 2019 01;105(2):92-93

Authors: Baumgartner H

PMID: 30076237 [PubMed - indexed for MEDLINE]

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Selective Partial Obstruction of Inferior Vena Cava Blood Flow During Diastole: Cor Triatriatum Dexter, Large Eustachian Valve, or Chiari Network?

J Cardiothorac Vasc Anesth. 2019 Feb;33(2):575-578

Authors: Pagel PS, Telles-Hernandez L, Miller R, Hill GED, Almassi GH

PMID: 30174263 [PubMed - indexed for MEDLINE]

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Understanding the Etiology of Heart Failure Among the Rural Poor in Sub-Saharan Africa: A 10-Year Experience From District Hospitals in Rwanda.

J Card Fail. 2018 Dec;24(12):849-853

Authors: Eberly LA, Rusingiza E, Park PH, Ngoga G, Dusabeyezu S, Mutabazi F, Harerimana E, Mucumbitsi J, Nyembo PF, Borg R, Gahamanyi C, Mutumbira C, Ntaganda E, Rusangwa C, Kwan GF, Bukhman G

BACKGROUND: Heart failure is a significant cause of morbidity and mortality in sub-Saharan Africa. Our understanding of the heart failure burden in this region has been limited mainly to registries from urban referral centers. Starting in 2006, a nurse-driven strategy was initiated to provide echocardiography and decentralized heart failure care within noncommunicable disease (NCD) clinics in rural district hospitals in Rwanda.
METHODS AND RESULTS: We conducted a retrospective review of patients with cardiologist-confirmed heart failure treated at 3 district hospital NCD clinics in Rwanda from 2006 to 2017 to determine patient clinical characteristics and disease distribution. Over 10 years, 719 patients with confirmed heart failure were identified. Median age was 27 years overall, and 42 years in adults. Thirty-six percent were children (age <18 years), 68% were female, and 78% of adults were farmers. At entry, 39% were in New York Heart Association functional class III-IV. Among children, congenital heart disease (52%) and rheumatic heart disease (36%) were most common. In adults, cardiomyopathy (40%), rheumatic heart disease (27%), and hypertensive heart disease (13%) were most common. No patients were diagnosed with ischemic cardiomyopathy.
CONCLUSIONS: The results of the largest single-country heart failure cohort from rural sub-Saharan Africa demonstrate a persistent burden of rheumatic disease and nonischemic cardiomyopathies.

PMID: 30312764 [PubMed - indexed for MEDLINE]

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Long-term follow-up in adults after tetralogy of Fallot repair.

Cardiovasc Ultrasound. 2018 Oct 29;16(1):28

Authors: Dłużniewska N, Podolec P, Skubera M, Smaś-Suska M, Pająk J, Urbańczyk-Zawadzka M, Płazak W, Olszowska M, Tomkiewicz-Pająk L

BACKGROUND: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Adults with repaired ToF develop late complications. The aim of this study was to describe and analyze long-term follow-up of patients with repaired ToF.
METHODS: This is a retrospective cohort study. Consecutive 83 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 30.5 ± 10.7. There were 49 (59%) male. Patients were divided into two groups according to the time since the repair (< 25 years and ≥ 25 years). The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively.
RESULTS: In CPET values were not significantly different in the two groups. In CMR volumes of left and right ventricles were not significantly different in the two groups. There were no differences between the groups in ventricular ejection fraction, mass of ventricles, or pulmonary regurgitation fraction. Among all the patients, ejection fraction and left and right ventricle mass, indexed pulmonary regurgitation volume measured by CMR did not correlate with the time since repair. In ECG among all the patients, ejection fraction of the RV, measured in CMR, negatively correlated with QRS duration (r = - 0.43; p < 0.001). There was a positive correlation between QRS duration and end diastolic volume of the RV (r = 0.30; p < 0.02), indexed end diastolic volume of the RV (r = 0.29; p = 0.04), RV mass (r = 0.36; p < 0.001) and left ventricle mass (r = 0.26; p = 0.04).
CONCLUSION: Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. QRS duration could be an utility and easy factor to assessment of right ventricular function.
TRIAL REGISTRATION: The study protocol was approved by the local Ethics Committee. Each participant provided informed consent to participate in the study (license number 122.6120.88.2016 from 28.04.2016).

PMID: 30373624 [PubMed - indexed for MEDLINE]

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Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

Congenit Heart Dis. 2019 Apr 04;:

Authors: Ezzeddine FM, Moe T, Ephrem G, Kay WA

BACKGROUND: Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.
METHODS: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status.
RESULTS: There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana.
CONCLUSIONS: The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.

PMID: 30945809 [PubMed - as supplied by publisher]

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Maternal factors and preoperative nutrition in children with mild cases of congenital heart disease.

Jpn J Nurs Sci. 2019 Jan;16(1):37-46

Authors: Qin C, Li Y, Wang D, Shi Z, Yao R, Wang D, Tang S

AIM: The preoperative poor nutrition of children with congenital heart disease (CHD) impacts the postoperative rehabilitation process of pediatric CHD cases. The factors of these children's preoperative poor nutrition, excluding the disease, have been underreported. The aim was to investigate the preoperative nutritional status of children with CHD who required a simple surgical repair and to analyze the maternal characteristics that are associated with poor nutrition in these sick children.
METHODS: This was a cross-sectional survey. The weight and height of the children were measured, maternal data were collected via a questionnaire and a univariate analysis and multivariate logistic regression were used to analyze the association between maternal factors and the preoperative poor nutrition of the children with CHD.
RESULTS: A total of 119 children with simple CHD were recruited to the study. The prevalence of poor nutrition was higher in the children with CHD ("cases") than in the healthy children ("controls"). An increased risk of poor nutrition was associated with lower mothers' perception, education level, understanding of the disease, and higher anxiety.
CONCLUSIONS: Paying attention to maternal anxiety, depression, and knowledge and providing interventions for the mothers of children with CHD are important in order to promote the nutritional status of these children.

PMID: 29569860 [PubMed - indexed for MEDLINE]