Mobile health in adults with congenital heart disease: current use and future needs.

OBJECTIVE: Many adults with congenital heart disease (CHD) are affected lifelong by cardiac events, particularly arrhythmias and heart failure. Despite the care provided, the cardiac event rate remains high. Mobile health (mHealth) brings opportunities to enhance daily monitoring and hence timely response in an attempt to improve outcome. However, it is not known if adults with CHD are currently using mHealth and what type of mHealth they may need in the near future. METHODS: Consecutive adult patients with CHD who visited the outpatient clinic at the Academic Medical Center in Amsterdam were asked to fill out questionnaires. Exclusion criteria for this study were mental impairment or inability to read and write Dutch. RESULTS: All 118 patients participated (median age 40 (range 18-78) years, 40 % male, 49 % symptomatic) and 92 % owned a smartphone. Whereas only a small minority (14 %) of patients used mHealth, the large majority (75 %) were willing to start. Most patients wanted to use mHealth in order to receive more information on physical health, and advice on progression of symptoms or signs of deterioration. Analyses on age, gender and complexity of defect showed significantly less current smartphone usage at older age, but no difference in interest or preferences in type of mHealth application for the near future. CONCLUSION: The relatively young adult CHD population only rarely uses mHealth, but the majority are motivated to start using mHealth. New mHealth initiatives are required in these patients with a chronic condition who need lifelong surveillance in order to reveal if a reduction in morbidity and mortality and improvement in quality of life can be achieved.

Perceived health is partially associated with the symptomatological profile in patients with benign and severe conditions: the case of congenital heart disease.

PURPOSE: Individuals with serious medical conditions can perceive their health status as good. This might be explained by the symptomatology inherent to the condition. Research in this respect is scarce. Congenital heart disease (CHD) is a spectrum of mild, moderate, and complex heart defects, representing more benign and severe chronic conditions. We investigated (1) symptomatology (i.e., symptom frequency and symptom distress) of CHD patients; (2) the extent to which symptomatology was independently related to perceived health; and (3) the relative importance of individual symptoms for perceived health. METHODS: A secondary data analysis on two separate patient samples (629 Belgian and 1,109 Dutch patients) was conducted. Patients' symptomatology was measured with the TAAQOL-CHD. Perceived health was measured by the EQ-5Dvas in Belgian patients, and by a single item (EVGFP rating) of the SF-36 in Dutch patients. Linear regression analyses were performed to investigate the relationship between symptoms and perceived health, while controlling for sex, age, disease complexity, and functional status. RESULTS: The most frequently occurring symptoms were dizziness, palpitations, and nycturia. Symptom distress was associated with perceived health, independent of confounders. Symptom distress with respect to shortness of breath while walking; palpitations; and dizziness were independently related to perceived health. CONCLUSIONS: Perceived health in CHD patients is partially associated with their symptomatology. This finding underscores the possibility that differences in perceived health across patient groups with more benign and severe conditions may be caused by the different impact conditions have--in terms of symptoms--on the day-to-day life.

Bicuspid stenotic aortic valves: clinical characteristics and morphological assessment using MRI and echocardiography.

BACKGROUND: Bicuspid aortic valve (BAV) is one of the most common congenital heart defects with a population prevalence of 0.5% to 1.3%. Identifying patients with BAV is clinically relevant because BAV is associated with aortic stenosis, endocarditis and ascending aorta pathology. METHODS AND RESULTS: Patients with severe aortic stenosis necessitating aortic valve replacement surgery were included in this study. All dissected aortic valves were stored in the biobank of the University Medical Centre Utrecht. Additionally to the morphological assessment of the aortic valve by the surgeon and pathologist, echocardiographic and magnetic resonance imaging (MRI) images were evaluated. A total of 80 patients were included of whom 32 (40%) were diagnosed with BAV by the surgeon (gold standard). Patients with BAV were significantly younger (55 vs 71 years) and were more frequently male. Notably, a significant difference was found between the surgeon and pathologist in determining valve morphology. MRI was performed in 33% of patients. MRI could assess valve morphology in 96% vs 73% with echocardiography. The sensitivity of MRI for BAV in a population of patients with severe aortic stenosis was higher than echocardiography (75% vs 55%), whereas specificity was better with the latter (91% vs 79%). Typically, the ascending aorta was larger in patients with BAV. CONCLUSION: Among unselected patients with severe aortic valve stenosis, a high percentage of patients with BAV were found. Imaging and assessment of the aortic valve morphology when stenotic is challenging.

Magnetic resonance imaging of the main pulmonary artery: reliable assessment of dimensions in Marfan patients on a simple axial spin echo image.

OBJECTIVE: To investigate if a simple axial spin echo (SE) image can be used for reliable assessment of pulmonary artery dimensions in patients with Marfan syndrome. METHODS: Fifty Marfan patients (mean age 33 +/- 10 years; 34 men, 16 women) and 15 normal subjects (mean age 28 +/- 4 years; nine men, six women) underwent cardiac magnetic resonance imaging (MRI). Pulmonary artery dimensions were obtained on axial SE images at two different levels: (1) the level of the pulmonary artery root, and (2) the level of the pulmonary artery bifurcation. To evaluate the accuracy of axial plane measurements 10 Marfan patients also underwent contrast-enhanced MR angiography (CE-MRA). RESULTS: In the 10 Marfan patients who also underwent CE-MRA, the mean diameter at the pulmonary bifurcation assessed with CE-MRA (31.5 +/- 3.6 mm) was almost equal to mean diameter assessed with axial SE (30.7 +/- 3.6 mm). Agreement of methodology according to Bland and Altman analysis showed a 95% confidence interval ranging from -2.6 to + 4.4 mm for all distances of the pulmonary artery root. In Marfan patients the mean right-left diameter measured on both axial SE images and CE-MRA was approximately 2.5 mm larger than the anterior-right and anterior-left diameters (p < 0.001). CONCLUSIONS: Axial SE MRI is a reliable and easy acquisition to measure pulmonary artery dimensions in patients with Marfan syndrome, and could be used for follow-up, especially in patients with severe involvement of the cardiovascular system. Not only the pulmonary artery trunk but also the asymmetric pulmonary root should be measured, although the clinical relevance of the asymmetric root is not yet known.

Quantitative assessment of dural ectasia as a marker for Marfan syndrome.

PURPOSE: To establish normal values for lumbosacral dural sac dimensions with magnetic resonance (MR) imaging and to use these values to assess the sensitivity and specificity of dural ectasia as a marker for Marfan syndrome. MATERIALS AND METHODS: MR imaging was performed to measure dural sac diameter (DSD) from L1 through S1 in 44 adult patients with Marfan syndrome and in 44 matched control subjects. DSD values were corrected for vertebral body size, yielding dural sac ratios (DSRs). The control subjects served to establish the upper limit of normal DSR values at the L1 through S1 levels. RESULTS: Cutoff values for normal DSRs for L1 through S1 were 0.64, 0.55, 0.47, 0.48, 0.48, and 0.57. Significant DSR differences were shown at all levels between patients with Marfan syndrome and control subjects (P <.001 at all levels). At L1 through S1, the sensitivity of dural ectasia as a marker for Marfan syndrome was 45%-77%, and the specificity was 95% or greater. By combining levels L3 and S1, dural ectasia as a marker for Marfan syndrome yielded a sensitivity of 95% (42 of 44 patients) and a specificity of 98% (43 of 44). The presence of dural ectasia excelled, compared with the presence of other Marfan syndrome manifestations in the patient population. CONCLUSION: Abnormal DSR values at L3 or S1 can be used to identify Marfan syndrome with 95% sensitivity and 98% specificity.