Real-time 3D echocardiography: an extra dimension in the echocardiographic diagnosis of congenital heart disease

This thesis investigates the role that real-time 3D echocardiography might play in the analysis of structural heart disease. The 3D echocardiographic assessment of patients with a variety of congenital heart disease is described, with specific focus on description and measurement of intracardiac anatomy and functional assessment. In chapter 1, the outline of the thesis is given. This thesis focuses on the feasibility and clinical applicability of real-time 3D echocardiography in daily patient care for congenital heart disease and whether 3D echocardiographic images give a reliable reflection of the intracardiac anatomy in a wide variety of congenital heart defects

Severe hypokalaemic paralysis and rhabdomyolysis due to ingestion of liquorice

Chronic ingestion of liquorice induces a syndrome with findings similar to those in primary hyperaldosteronism. We describe a patient who, with a plasma K+ of 1.8 mmol/l, showed a paralysis and severe rhabdomyolysis after the habitual consumption of natural liquorice. Liquorice has become widely available as a flavouring agent in foods and drugs. It is important for physicians to keep liquorice consumption in mind as a cause for hypokalaemic paralysis and rhabdomyolysis

Echocardiographic chamber quantification in a healthy Dutch population

Aim For accurate interpretation of echocardiographic measurements normative data are required, which are provided by guidelines. For this article, the hypothesis was that these cannot be extrapolated to the Dutch population, since in Dutch clinical practice often higher values are found, which may not be pathological but physiological. Therefore this study aimed to 1) obtain and propose normative values for cardiac chamber quantification in a healthy Dutch population and 2) determine influences of baseline characteristics on these measurements. Methods Prospectively recruited healthy subjects, aged 20–72 years (at least 28 subjects per age decade, equally distributed for gender) underwent physical examination and 2D and 3D echocardiography. Both ventricles and atria were assessed and volumes were calculated. Results 147 subjects were included (age 44 ± 14 years, 50% female). Overall, feasibility was good for both linear and volumetric measurements. Linear and volumetric parameters were consistently higher than current guidelines recommend, while functional parameters were in line with the guidelines. This was more so in the older population. 3D volumes were higher than 2D volumes. Gender dependency was seen in all body surface area (BSA) corrected volumes and with increasing age, ejection fractions decreased. Conclusion This study provides 2D and 3D echocardiographic reference ranges for both ventricles and atria de-rived from a healthy Dutch population. BSA indexed volumes are gender-dependent, age did not influence ventricular volumes and a rise in blood pressure was independently associated with increased right ventricular volumes. The higher volumes found may be indicative for the Dutch population being the tallest in the world

Reply: Letter to the editor: Prognostic value of left atrial size and function in adults with tetralogy of Fallot

_Dear Dr. An,_ We would like to thank you for your interest in our article entitled “Prognostic value of left atrial size and function in adults with tetralogy of Fallot”. We fully agree that accurate prognostication is of paramount importance in these patients.We are pleased to respond to the comments point by point. Indeed, other imaging modalities such as cardiac magnetic resonance (CMR) are probably more accurate than 2D echocardiography in the assessment of LA volumes. We have acknowledged this in our article in the Clinical Implications paragraph, in which we stated “Although other imaging techniques such as CT, CMR or 3D echocardiography could provide a more accurate estimation of LA size, (…)”. We also agree that the cohort of 32 patients that was analyzed in your department is probably too small to demonstrate any association between LA size and cardiovascular events. Future research is therefore certainly needed to evaluate the prognostic value of CMR-derived left atrial volumes in a large prospective cohort of patients with tetralogy of Fallot with a long-term follow-up duration. Nevertheless, the echocardiographic measurements that are investigated in our study can be easily implemented in day-to-day clinical practice and CMR also has its limitations such as higher costs, less availability, and inability to scan patients with intracardiac devices. As described in Tables 1, 67 patients (50%) had received a surgical PVR at the timeof study inclusion. In Table 2,we have described the cardiac function of all patients at the time of study inclusion. It was considered outside the scope of this study to describe cardiac function at the time of surgical PVR, because this was median 7.7 years prior to study inclusion. We hope that this additional information can adequately address the comments raised

Life expectancy and end-of-life communication in adult patients with congenital heart disease, 40-53 years after surgery

Aims: Although survival of patients with congenital heart disease (CHD) improved significantly over time, life expectancy is still not normal. We aimed to investigate how adult patients, their partners, and treating cardiologists estimated the individual life expectancy of CHD patients. Furthermore, preferences regarding end-of-life (EOL) communication were investigated. Methods and results: In this study, we included 202 patients (age: 50 ± 5) who were operated in childhood (&lt;15 years old) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot, or transposition of the great arteries. A specific questionnaire was administered to both the patients and their partners, exploring their perceived life expectancy and EOL wishes. Two cardiologists independently assessed the life expectancy of each patient. Most adults with CHD believed their life expectancy to be normal. However, significant differences were found between estimated life expectancy by the cardiologist and patients (female: P = 0.001, male: P = 0.002) with moderate/severe defects, as well as for males with mild defects (P = 0.011). Regarding EOL communication, 85.1% of the patients reported that they never discussed EOL with a healthcare professional. Compared with patients with mild CHD, significantly more patients with moderate/severe defect discussed EOL with a physician (P = 0.011). The wish to discuss EOL with the cardiologist was reported by 49.3% of the patients and 41.7% of their partners. Conclusion: Adult patients, especially with moderate/severe CHD, perceived their life expectancy as normal, whereas cardiologists had a more pessimistic view than their patients. Increased attention is warranted for discussions on life expectancy and EOL to improve patient-tailored care.</p

Prognostic significance of anterior mitral valve leaflet length in individuals with a hypertrophic cardiomyopathy gene mutation without hypertrophic changes

Purpose: Previous studies suggest that anterior mitral valve leaflet (AMVL) elongation is a primary phenotypic feature in hypertrophic cardiomyopathy (HCM). Our aim was to assess AMVL length in individuals with HCM gene mutations and in healthy controls and to identify predictors of the development of HCM during follow-up. Methods:

Echocardiographic findings associated with mortality or transplant in patients with pulmonary arterial hypertension: A systematic review and meta-analysis

Background Identification of patients at risk of deterioration is essential to guide clinical management in pulmonary arterial hype

Systolic and diastolic strain measurements show left ventricular dysfunction in women with Turner syndrome

Aim: This study aimed to describe the systolic left ventricular global longitudinal strain (LvGLS) and left ventricular peak early diastolic strain rate (Sre) in adult women with Turner syndrome (TS) and to determine its relationship with exercise capacity and clinical parameters. Methods: In this cross-sectional cohort study, consecutively included adult TS women underwent an electrocardiogram, transthoracic echocardiogram (TTE) and cardiopulmonary exercise test (CPET) on the same day. LvGLS and Sre were measured using 2D speckle tracking analysis (STE) and compared with age-matched healthy female controls. Results: Ninety-four adult women ( age 36 +/- 13 years) with TS and 32 healthy age-matched female controls were included. Women with TS had a significantly impaired/reduced systolic LvGLS (-17.82 +/- 2.98% vs. -21.80 +/- 1.85%, p 0.001), compared to healthy female controls. Furthermore, TS women had reduced diastolic function as measured by conventional echocardiographic parameters: A higher A-wave ( p < 0.001), lower E/A-ratio (p = 0.001), longer deceleration time (p = 0.006), and a higher E/E'-ratio (p < 0.001). Women with TS also had a significantly reduced maximal workload (p = 0.033), reduced oxygen uptake (p < 0.001) and a reduced maximal heart rate (p < 0.001) during exercise. Multivariable linear regression analysis revealed that Age, karyotype and QT-duration were significantly associated with Sre, but not with LvGLS, in the TS population. Conclusion: Systolic and diastolic strain and exercise capacity were significantly reduced in TS women compared to healthy women. No correlation between strain itself and exercise capacity could be demonstrated, but correlations with conventional TTE parameters and baseline characteristics were found.Cardiolog

Assessment of pulmonary valve and right ventricular outflow tract with real-time three-dimensional echocardiography

Aim: Assessment of pulmonary valve (PV) and right ventricular outflow tract (RVOT) using real-time 3-dimensional echocardiography (RT3DE). Methods: Two-dimensional echocardiography (2DE) and RT3DE were performed in 50 patients with congenital heart disease (mean age 32 ± 9.5 years, 60% female). Measurements were obtained at parasternal views: short axis (PSAX) at aortic valve level and long axis (PLAX) with superior tilting. RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Diameters of PV annulus (PVAD), and RVOT (RVOTD) were measured by both 2DE and RT3DE, while areas (PVAA) and (RVOTA) by RT3DE only. Results: By RT3DE, PV was visualized sufficiently in 68% and RVOTexcellently in 40%. PVAD and PVAA were measured in 88%. RVOTD and PVAD by 2DE at PLAX were significantly higher than PSAX (P < 0.0001) and lower than that by RT3DE (P < 0.001). Conclusion: RT3DE helps inRVOT and PV assessment adding more details supplemental to 2DE