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It's like balancing on a slackline-A description of how adults with congenital heart disease describe themselves in relation to physical activity.

J Clin Nurs. 2018 Aug;27(15-16):3131-3138

Authors: Bay A, Lämås K, Berghammer M, Sandberg C, Johansson B

AIMS AND OBJECTIVES: To illuminate how adults with congenital heart disease (CHD) describe themselves in relation to physical activity.
BACKGROUND: Several studies have shown that adults with CHD have reduced exercise capacity and do not reach the recommended daily level of physical activity. With this in view, it is of immense importance to investigate how this population experiences physical activity.
DESIGN: Qualitative study with semistructured interviews analysed with qualitative content analysis.
METHODS: Semistructured interviews were individually performed with 14 adults (women = 7, age 19-68 years) with complex CHD. Patients were purposively recruited from the clinic waiting list, based on a scheduled follow-up and diagnosis.
RESULTS: The overall theme, It's like balancing on a slackline, illustrates how adults with CHD described themselves in relation to physical activity. This overall theme consisted of four subthemes: (a) Being an adventurer-enjoying the challenges of physical activity; (b) Being a realist-adapting to physical ability; (c) Being a non-doer-lacking prerequisites for physical activity; and (d) Being an outsider-feeling excluded depending on physical ability.
CONCLUSIONS: Adults with CHD seem to have a diverse relationship with physical activity and it involves various aspects throughout the lifespan. The findings point out factors that might constitute as obstacles for being physically active, specific for people with chronic conditions like CHD. This highlights the importance of further exploring the hindering and facilitating factors for being physically active to get a deeper understanding of how to support adults with CHD to be physically active.
RELEVANCE TO CLINICAL PRACTICE: Given the diverse relationship with physical activity, nurses have to further investigate the patients' relationship with physical activity, to support a healthy lifestyle. Nurses and allied health professionals should offer individualised exercise prescriptions and education about suitable physical activities in relation to physical ability.

PMID: 29752846 [PubMed - indexed for MEDLINE]

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Three-dimensional transthoracic echocardiographic identification of individual cusps of the pulmonary valve: Potential misidentification by the two-dimensional technique.

Echocardiography. 2018 06;35(6):855-859

Authors: Elsayed M, Hsiung MC, Nanda NC, Alratroot A, Turaga NSS

We demonstrate the incremental value of live/real time three-dimensional transthoracic echocardiography (3DTTE) over the two-dimensional modality in the identification of all three cusps of the pulmonary valve in patients in whom only two leaflets could be detected by the latter technique. This was because of the ability of 3DTTE to view the cusps enface permitting assessment of relationship to one another and the surrounding cardiac structures. In addition, 3DTTE showed not only the potential errors that can occur in pulmonary valve cusp identification when the two-dimensional modality is used alone but also how some of these errors can be avoided by paying attention to the surrounding structures.

PMID: 29858885 [PubMed - indexed for MEDLINE]

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eHealth in patients with congenital heart disease: a review.

Expert Rev Cardiovasc Ther. 2018 Sep;16(9):627-634

Authors: Kauw D, Koole MAC, van Dorth JR, Tulevski II, Somsen GA, Schijven MP, Dohmen DAJ, Bouma BJ, Mulder BJM, Schuuring MJ, Winter MM

INTRODUCTION: Mobile health (mHealth), an advanced form of eHealth is expected to drastically change the field of traditional healthcare in the near future as wearables and mobile applications are rapidly increasing in number. The majority of patients with congenital heart disease (CHD) now reach adulthood and this relative young patient population seems particularly suited for mHealth, as they require lifelong follow-up, experience high morbidity burden, and were raised in this digital era. In patients with acquired heart disease the potential of eHealth has been demonstrated, yet data are still inconclusive. Areas covered: In this review of the current literature we evaluated the effect of various eHealth interventions in patients with CHD. Our search resulted in a mere 10 studies, which comprised mostly of children or adolescents with severe CHD. Home-monitoring of saturation and weight through mHealth was found to be beneficial in patients after palliation procedures, and video conferencing was found to have a positive effect on anxiety and healthcare utilization. Expert commentary: Due to high morbidity and mortality in patients with CHD and the promising results of eHealth interventions, further research is desperately needed.

PMID: 30079780 [PubMed - indexed for MEDLINE]

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Channelopathies That Lead to Sudden Cardiac Death: Clinical and Genetic Aspects.

Heart Lung Circ. 2018 Oct 04;:

Authors: Skinner JR, Winbo A, Abrams D, Vohra J, Wilde AA

Forty per cent (40%) of sudden unexpected natural deaths in people under 35 years of age are associated with a negative autopsy, and the cardiac ion channelopathies are the prime suspects in such cases. Long QT syndrome (LQTS), Brugada syndrome (BrS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are the most commonly identified with genetic testing. The cellular action potential driving the heart cycle is shaped by a specific series of depolarising and repolarising ion currents mediated by ion channels. Alterations in any of these currents, and in the availability of intracellular free calcium, leaves the myocardium vulnerable to polymorphic ventricular tachycardia or ventricular fibrillation. Each channelopathy has its own electrocardiogram (ECG) signature, typical mode of presentation, and most commonly related gene. Long QT type 1 (gene, KCNQ1) and CPVT (gene, RyR2) typically present with cardiac events (ie syncope or cardiac arrest) during or immediately after exercise in young males; long QT type 2 (gene, KCNH2) after startle or during the night in adult females-particularly early post-partum, and long QT type 3 and Brugada syndrome (gene, SCN5A) during the night in young adult males. They are commonly misdiagnosed as seizure disorders. Fever-triggered cardiac events should also raise the suspicion of BrS. This review summarises genetics, cellular mechanisms, risk stratification and treatments. Beta blockers are the mainstay of treatment for long QT syndrome and CPVT, and flecainide is remarkably effective in CPVT. Brugada syndrome is genetically a more complex disease than the others, and risk stratification and management is more difficult.

PMID: 30389366 [PubMed - as supplied by publisher]

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Outcomes of children supported with an intracorporeal continuous-flow left ventricular assist system.

J Heart Lung Transplant. 2018 Sep 25;:

Authors: VanderPluym CJ, Adachi I, Niebler R, Griffiths E, Fynn-Thompson F, Chen S, O'Connor MJ, Machado D, Hawkins B, Bleiweis MS, Koehl DA, Cantor RS, Morales D, Lorts A

BACKGROUND: Since 2012, there has been growing use of the HeartWare (Medtronic, Mounds View, MN) intracorporeal continuous flow (CF) ventricular assist device (VAD) in children, despite it not being labeled for use in pediatric patients. We sought to describe the use and outcomes of children with HeartWare VADs.
METHODS: We identified all patients aged < 19 years and young adults aged 19 to 30 years supported with HeartWare who were entered into the pediatric portion (Pedimacs) of the Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) and the Intermacs registries, respectively, between September 2012 and June 2017. Adverse events and outcomes were analyzed and compared.
RESULTS: We identified 192 children and 247 young adult HeartWare patients. Baseline characteristics of children differed from young adults, with lower median weight of 51.5 kg (range, 13.1-162) vs 75.8 kg (range, 29.8-191; p ≤ 0.0001) and body surface area of 1.5 m2 (range, 0.6-2.9 m2) vs 1.9 m2 (range, 1.1-3.2 m2; p ≤ 0.0001) . At the time of implant, 12 children weighed < 20 kg, and 58.3% of these children had congenital heart disease compared with 11.7% in children who weighed ≥ 20 kg and 6.1% in young adults (p ≤ 0.0001). Median duration of support was 2.8 months (IQR, 1.3-6.0 months) in children and 9.7 months (IQR 4.0-19.2 months) in young adults (p ≤ 0.0001). Serious adverse events in children and young adults included infection in 27% and 44% of patients, respectively (p=0.0002), major bleeding in 23% and 23%, respectively (p = 0.9), device malfunction/pump thrombosis in 11% and 19.0%, respectively (p = 0.04), and stroke in 10% and 12%, respectively (p = 0.5). Of the children who weighed < 20 kg at time of implant, 0% had major bleeding, 16.7% had infections, and 8.3% had stroke. Overall survival was not statistically different between children and young adults, and there was no increased mortality in children who weighed < 20 kg. Rate of discharge on HeartWare was 80% in young adults vs 48% in children who weighed ≥ 20 kg and only 33% in children who weighed < 20 kg.
CONCLUSIONS: Survival in children supported with HeartWare is encouraging and comparable to young adults; however, adverse events are not uncommon in children. Ongoing evaluation of the HeartWare use in children is necessary to further decrease the rate of adverse events and understand obstacles to discharge.

PMID: 30391197 [PubMed - as supplied by publisher]

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Adult Congenital Heart Disease in Australia and New Zealand A Call for Optimal Care.

Heart Lung Circ. 2018 Oct 28;:

Authors: Nicolae M, Gentles T, Strange G, Tanous D, Disney P, Bullock A, Grigg L, Chen SSM, Pressley L, Cordina R, Hornung T, O'Donnell C, Celermajer DS

BACKGROUND: Adult congenital heart disease (ACHD) is a relatively new subspecialty in the cardiology field. The prevalence of ACHD is estimated at ∼ 3,000 per million adult population. The ACHD patient group is estimated to grow at ∼ 5% per year and in the next decade it is forecast that 1 in 150 young adults will carry some form of ACHD diagnosis. These estimates translate to ∼ 72,000 ACHD patients in Australia and ∼ 14,000 in New Zealand, although no current numbers are available. The Cardiac Society of Australia and New Zealand (CSANZ) has recently published Recommendations for Standards of Care for Adult Congenital Heart Disease (ACHD) in 2016. There is currently no long-term plan or proposal to address this huge health care burden within the federal government. This document details the size of the problem insofar as it is known and recommends solutions to be implemented.
METHODS: This document was developed by the Adult Congenital Heart Disease Working Group of the Paediatric and Congenital Council (the Congenital Heart Alliance of Australia and New Zealand) as a response to the chronic under resourcing in this area, the risk this poses to patients and clinicians, and the clear need for long-term planning to develop safe care pathways.
RESULTS: These issues were raised with the CSANZ Board in December 2015 and the document was developed in response to the Board's request for more information. The current iteration was finalised on 14 November 2017.
CONCLUSIONS: The authorship group comprised participants in the CSANZ adult CHD standards of care recommendations from 2013 with the inclusion of some newly trained ACHD cardiologists, who represented most states and territories across ANZ. None of the authors has any academic or professional conflict of interest.

PMID: 30392984 [PubMed - as supplied by publisher]

Subcutaneous defibrillators in the adult congenital heart disease population: one size does not fit all: Comment on "Eligibility for subcutaneous implantable cardioverter- defibrillators in the adult congenital heart disease population".

Pacing Clin Electrophysiol. 2018 Nov 05;:

Authors: Afzal MR, Lastinger L, Weiss R

PMID: 30394546 [PubMed - as supplied by publisher]

Eligibility for subcutaneous implantable cardioverter-defibrillators in the adult congenital heart disease population.

Pacing Clin Electrophysiol. 2018 Nov 05;:

Authors: Garside H, Leyva F, Hudsmith L, Marshall H, de Bono J

BACKGROUND: Patients with adult congenital heart disease (ACHD) have an increased risk of arrhythmic, sudden cardiac death. The subcutaneous implantable cardioverter defibrillator (S-ICD) provides a potentially safer alternative to transvenous ICDs in ACHD. Suitability for S-ICD depends on the surface ECG which is often abnormal in ACHD patients. This study investigates the proportion of ACHD patients who meet the screening criteria for S-ICD METHODS: A standard screening ECG was performed in 102 patients with complex ACHD (Tetralogy of Fallot, Fontan Circulation, Transposition of the Great Arteries). This process was repeated post-exercise for patients who also had an exercise test.
RESULTS: Three quarters (75.4%) of ACHD patients meet screening criteria for an S-ICD with at least 1 suitable vector. The most common number of acceptable vectors in the eligible group was 2 (35% of total population). In only 12% were all three vectors suitable whilst 28% had only 1. The primary vector (equivalent of ECG lead III) was the most common suitable vector, found in 62% of participants who had appropriate sensing vectors. 25 (24.5%) patients failed to meet the S-ICD screening criteria. Of these, 14 had repaired tetralogy of Fallot. 92% of patients with a Fontan circulation met ECG screening criteria. Of those who had the protocol repeated following their cardiopulmonary exercise test (n = 14), only 1 additional patient failed eligibility criteria.
CONCLUSIONS: A quarter of ACHD patients do not meet eligibility criteria for the S-ICD. However, more than 90% of patients with a Fontan circulation are suitable for an S-ICD. This article is protected by copyright. All rights reserved.

PMID: 30394548 [PubMed - as supplied by publisher]

Transaortic Flow in Aortic Stenosis: Stroke Volume Index versus Flow Rate.

Cardiology. 2018 Nov 05;141(1):71-74

Authors: Lazaros G, Drakopoulou MI, Tousoulis D

PMID: 30396182 [PubMed - as supplied by publisher]

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Ischaemic cardiomyopathy and embolic stroke in a young adult with suspected synthetic cannabinoid use.

BMJ Case Rep. 2018 Jun 07;2018:

Authors: Khan S, Hanif A, Wilson MF

The incidence of cardiovascular disease is increasing in young adults. We are reporting a case of acute stroke in a young patient with severe ischaemic cardiomyopathy in the absence of traditional risk factors. After ruling out atherosclerotic disease, his presentation was attributed to synthetic cannabinoid use. We then discussed the typical barriers in early diagnosis and limitations of laboratory testing in this condition. Due to the increase in abuse of these synthetic drugs among young adults, there is a need for high clinical suspicion which can help with early recognition and improve morbidity and mortality associated with these chemicals.

PMID: 29880579 [PubMed - indexed for MEDLINE]