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Decreased Diastolic Ventricular Kinetic Energy in Young Patients with Fontan Circulation Demonstrated by Four-Dimensional Cardiac Magnetic Resonance Imaging.

Pediatr Cardiol. 2017 Apr;38(4):669-680

Authors: Sjöberg P, Heiberg E, Wingren P, Ramgren Johansson J, Malm T, Arheden H, Liuba P, Carlsson M

Abstract
Four-dimensional (4D) flow magnetic resonance imaging (MRI) enables quantification of kinetic energy (KE) in intraventricular blood flow. This provides a novel way to understand the cardiovascular physiology of the Fontan circulation. In this study, we aimed to quantify the KE in functional single ventricles. 4D flow MRI was acquired in eleven patients with Fontan circulation (median age 12 years, range 3-29) and eight healthy volunteers (median age 26 years, range 23-36). Follow-up MRI after surgical or percutaneous intervention was performed in 3 patients. Intraventricular KE was calculated throughout the cardiac cycle and indexed to stroke volume (SV). The systolic/diastolic ratio of KE in Fontan patients was similar to the ratio of the controls' left ventricle (LV) or right ventricle (RV) depending on the patients' ventricular morphology (Cohen´s κ = 1.0). Peak systolic KE/SV did not differ in patients compared to the LV in controls (0.016 ± 0.006 mJ/ml vs 0.020 ± 0.004 mJ/ml, p = 0.09). Peak diastolic KE/SV in Fontan patients was lower than in the LV of the control group (0.028 ± 0.010 mJ/ml vs 0.057 ± 0.011 mJ/ml, p < 0.0001). The KE during diastole showed a plateau in patients with aortopulmonary collaterals. This is to our knowledge the first study that quantifies the intraventricular KE of Fontan patients. KE is dependent on the morphology of the ventricle, and diastolic KE indexed to SV in patients is decreased compared to controls. The lower KE in Fontan patients may be a result of impaired ventricular filling.

PMID: 28184976 [PubMed - indexed for MEDLINE]

Related Articles

Mental health problems among nurses in paediatric cardiac intensive care.

Br J Nurs. 2017 Aug 10;26(15):870-873

Authors: Tito RS, Baptista PCP, da Silva FJ, Felli VEA

Abstract
At present, there are growing rates of psychiatric symptoms among some occupational categories, with emphasis on health professionals who work in hospitals. This study aimed to identify the occurrence of mental health problems (anxiety and depression) among 92 nursing workers in a paediatric cardiac intensive care unit. This is an exploratory, cross-sectional study, with a quantitative approach. The research was conducted in a public university hospital specialising in cardiology, pneumology, and thoracic and cardiac surgery. The data were collected between June and July of 2012 through socio-demographic and Self-Reporting Questionnaire (SRQ-20) instruments. The analysis of the results revealed the occurrence of mental health problems in 45% (41) of the workers. There was the prevalence of tension, nervousness and worry symptoms, followed by headache. Findings highlight the need for protective measures towards the mental health of workers who assist children with serious heart disease.

PMID: 28792829 [PubMed - indexed for MEDLINE]

Updated 2017 European and American guidelines for prosthesis type and implantation mode in severe aortic stenosis.

Heart. 2017 Oct 07;:

Authors: Otto CM, Baumgartner H

PMID: 28988212 [PubMed - as supplied by publisher]

Process of Transition for Congenital Heart Patients: Preventing Loss to Follow-up.

Clin Nurse Spec. 2017 Nov/Dec;31(6):329-334

Authors: Habibi H, Emmanuel Y, Chung N

Abstract
PURPOSE: The aim of this article is to provide an overview of our nurse-led transition clinic provided to congenital heart disease patients moving from pediatric into adult care setting.
DESCRIPTION OF THE SERVICE: Nurse-led transition clinic was analyzed at various stages of young adult care from an early stage of 12 to 14 years to entering adult setting at 16 years or older.
METHODS: Overview of current transition service for young adults being transferred from pediatric into adult services highlights the integral role of clinical nurse specialist as a coordinator of care.
RESULTS: The result of the service overview indicates that nurse-led transition service enables patients to build on their knowledge. This is achieved by providing them time and the opportunities to develop an understanding of their condition and the attitudes required to engage with the adult care setting as indicated in the psychology questionnaire from transition day.
CONCLUSION: A nurse-led transition clinic enhances long-term care of patients by supporting the young adults and their family/carer through the transition and transfer of the care to promote the young adult's understanding of their condition and to prevent any lost to follow-up.

PMID: 28991016 [PubMed - in process]

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Circulation. 2017 Oct 09;:

Authors: Lui GK, Saidi A, Bhatt AB, Burchill LJ, Deen JF, Earing MG, Gewitz M, Ginns J, Kay JD, Kim YY, Kovacs AH, Krieger EV, Wu FM, Yoo SJ, American Heart Association Adult Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young; Council on Cardiovascular Radiology and Intervention; and Council on Quality of Care and Outcomes Research

Abstract
Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

PMID: 28993401 [PubMed - as supplied by publisher]

Intra-atrial re-entrant tachycardia in congenital heart disease: types and relation of isthmus to atrial voltage.

Europace. 2017 Aug 09;:

Authors: Roca-Luque I, Rivas Gándara N, Dos Subirà L, Pascual JF, Domenech AP, Pérez-Rodon J, Subirana MT, Santos Ortega A, Miranda B, Rosés-Noguer F, Ferreira-Gonzalez I, Ferrer JC, García-Dorado García D, Mitjans AM

Abstract
Background: Intra-atrial re-entrant tachycardia (IART) is a frequent and severe complication in patients with congenital heart disease (CHD). Cavotricuspid isthmus (CTI)-related IART is the most frequent mechanism. However, due to fibrosis and surgical scars, non-CTI-related IART is also frequent.
Objective: The main objective of this study was to describe the types of IART and circuit locations and to define a cut-off value for unhealthy tissue in the atria.
Methods and results: This observational study included all consecutive patients with CHD who underwent a first ablation procedure for IART from January 2009 to December 2015 (94 patients, 39.4% female, age: 36.55 ± 14.9 years, 40.4% with highly complex cardiac disease). During the study, 114 IARTs were ablated (1.21 ± 0.41 IARTs per patient). Cavotricuspid isthmus-related IART was the only arrhythmia in 51% (n = 48) of patients, non-CTI-related IART was the only mechanism in 27.7% (n = 26), and 21.3% of patients (n = 20) presented both types of IART. In cases of non-CTI-related IART, the most frequent location of IART isthmus was the lateral or posterolateral wall of the venous atria, and a voltage cut-off value for unhealthy tissue in the atria of 0.5 mV identified 95.4% of IART isthmus locations.
Conclusion: In our population with a high proportion of complex CHD, CTI-related IART was the most frequent mechanism, although non-CTI-related IART was present in 49% of patients (alone or with concomitant CTI-related IART). A cut-off voltage of 0.5 mV could identify 95.4% of the substrates in non-CTI-related IART.

PMID: 29016802 [PubMed - as supplied by publisher]

Related Articles

Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors.

Pediatr Pulmonol. 2016 Dec;51(12):1320-1329

Authors: Bojanić K, Grizelj R, Dilber D, Šarić D, Vuković J, Pianosi PT, Driscoll DJ, Weingarten TN, Pritišanac E, Schroeder DR, Sprung J

Abstract
BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age-matched controls and whether CPET results among CDH survivors differ according to self-reported daily activity.
METHODS: In one medical center in Croatia, CDH survivors-patients with surgically corrected CDH who were alive at age 5 years-were invited to participate in spirometry and CPET. Values were compared with those of controls matched 2:1 by age and sex for each CDH survivor aged 7 years or older.
RESULTS: Among 27 CDH survivors aged 5-20 years, 13 (48%) had continued symptoms or spirometric evidence of pulmonary disease. Compared with controls (n = 44), survivors (n = 22) had lower peak oxygen consumption (V˙O2 mean [SD], 35.7 [6.9] vs. 45.3 [8.2] ml/kg per min; P < 0.001). At peak exercise, V˙O2/heart rate (P < 0.001), tidal volume (P = 0.005), and minute ventilation (P < 0.001) were lower in survivors, but the maximal respiratory rate was not different (P = 0.72). Among survivors, mean (SD) V˙O2peak (ml/kg per min) differed by self-reported activity level: athletic, 40.3 (5.0); normal, 35.8 (6.5); and sedentary, 32.1 (6.8) (by ANOVA, P = 0.10 across three groups and P = 0.04 athletic vs. sedentary).
CONCLUSION: More than half of CDH survivors continue to have chronic pulmonary disease. CDH survivors had lower aerobic exercise capacity than controls. Self-reporting information on daily activities may identify CDH patients with low V˙O2max who may benefit from physical training. Pediatr Pulmonol. 2016;51:1320-1329. © 2016 Wiley Periodicals, Inc.

PMID: 27228382 [PubMed - indexed for MEDLINE]

Related Articles

Appropriateness and diagnostic yield of inpatient pediatric echocardiograms.

Congenit Heart Dis. 2017 Mar;12(2):210-217

Authors: Lang SM, Bolin E, Daily JA, Tang X, Thomas Collins R

Abstract
OBJECTIVE: Multiple reports have shown echocardiograms for certain indications are neither cost-effective nor of high diagnostic yield. Given the ease with which tests can be obtained at a tertiary academic children's hospital, our aims were to: (1) determine the diagnostic yield of inpatient studies by in-hospital location; (2) evaluate inpatient echocardiograms to determine indications and level of appropriateness; and (3) evaluate the frequency of cardiology involvement prior to those echocardiograms.
DESIGN: All initial inpatient echocardiograms interpreted at our institution from February 2009 to December 2014 were reviewed retrospectively. Patient location was grouped as pediatric intensive care (PICU), emergency department (ED), and general floor.
RESULTS: There were 727 first-time inpatient echocardiograms that met inclusion criteria. Pathology was identified in 25% of the study echocardiograms, with 11% of all studies demonstrating pathology that could alter patient management (moderate or severe pathology). The studies performed in the PICU and ED had more severe pathology compared with those from the general floor (P < .001, .003; respectively). Few echocardiograms were performed for rarely appropriate indications on the general floor (7%) and PICU (2.2%). Over 75% of general floor echocardiograms performed for a pathologic murmur yielded normal or incidental findings. Cardiology consultation was documented in only 7.5% of general floor studies.
CONCLUSION: The diagnostic yield of inpatient, first-time pediatric echocardiograms is relatively low. The majority of studies that identified pathology were performed on patients located in higher acuity units. General floor echocardiograms for murmurs had a low diagnostic yield, raising the question of cardiology consultation versus direct echocardiogram ordering for subjective physical exam signs.

PMID: 27863016 [PubMed - indexed for MEDLINE]

Related Articles

Trends, microbiology, and outcomes of infective endocarditis in children during 2000-2010 in the United States.

Congenit Heart Dis. 2017 Mar;12(2):196-201

Authors: Gupta S, Sakhuja A, McGrath E, Asmar B

Abstract
BACKGROUND: We studied the incidence, trend, underlying conditions, microbiology, and outcomes of infective endocarditis (IE) in children during 11 years using Nationwide Inpatient Sample (NIS) database. This is the largest all-payer inpatient care database in the United States containing data for more than 8 million hospital stays from over 1000 hospitals.
METHODS: NIS data from 2000 to 2010 of primary discharge diagnosis of IE in children aged ≤19 years old were studied. Children with underlying congenital heart defects and acquired heart conditions were identified. Microbiological causative agents were recorded. Linear regression was used to assess trend of incidence over time.
RESULTS: An estimated 3,840 (95% CI: 3,395-4,285) children had a discharge diagnosis of IE. The overall incidence was 0.43 per 100 000 children. The incidence was stable over the study period (P = .4 for trend). The majority of patients 56.2% were ≥11 years old and 15.4% were ≤ 1 year. Underlying cardiac conditions were present in 53.5% of patients. Overall 30.2% of cases were culture-negative. Among those with identified pathogens, Staphylococcus species were most common (43.1%) followed by Streptococcus species (39.5%). Viridans Streptococcus group was most common in those with underlying heart disease (32.7%) and S. aureus was most common in those without heart disease (46.9%). Among culture-positive patients, there was a decline in proportion of Staphylococcal IE (P = .03) and an increase in proportion of Streptococcal IE (P = .04). Overall mortality was 2.8%. Patients with Staphylococcal IE had longer median length of stay (12 vs. 9 days; P < .01) and the highest mortality (4.7%).
CONCLUSION: The incidence of IE in children has remained unchanged in the United States during the 11-year study period. Among culture-positive patients there was a significant decrease in Staphylococcal IE and a significant increase of Streptococcal IE. Staphylococcal IE was associated with increased LOS and highest mortality.

PMID: 27885814 [PubMed - indexed for MEDLINE]

Related Articles

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

Congenit Heart Dis. 2017 Mar;12(2):153-158

Authors: Lewis MJ, Hecht E, Ginns J, Benton J, Prince M, Rosenbaum MS

Abstract
BACKGROUND: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.
METHODS AND RESULTS: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004).
CONCLUSIONS: Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.

PMID: 27893192 [PubMed - indexed for MEDLINE]

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