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

Three-dimensional echocardiography in congenital heart disease

Three-dimensional echocardiography is a valuable tool for the assessment of cardiac function where it permits calculation of chamber volume and function. The anatomy of valvar and septal structures can be presented in unique and intuitive ways to enhance surgical planning. Guidance of interventional procedures using the technique has now become established in many clinical settings. Enhancements of image processing to include intracavity flow, image fusion and true 3D displays look set to further improve the contribution of this modality to care of the patient with congenital heart disease

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

The Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

Aims: To assess the prognostic value of left ventricular (LV) global longitudinal strain (GLS) and global longitudinal early diastolic strain rate (GLSre) with regard to cardiovascular events, as congenital aortic stenosis (AoS) is associated with significant mortality and morbidity but predictors for clinical outcome are scarce. Strain analysis provides a robust and reproducible method for early detection of LV dysfunction, which might be of prognostic value. Methods: This prospective study, included clinically stable patients with congenital AoS between 2011– 2013. LV GLS and GLSre was performed in the apical 4, 3 and 2-chamber views using Tomtec software. The end-point was a composite of death, heart failure, hospitalization, arrhythmia, thrombo-embolic events and re-inter-vention. Results: In total 138 patients were included (33[26–43] years, 86(62%) male), NYHA class I: 134(97%). Mean LV GLS was –15.3 ± 3.2%, GLSre 0.66 ± 0.18 s–1. Both correlated with NT-proBNP, LV volumes and ejection fraction (strongest LV GLS with LV EF: r –0.539, p &lt; 0.001, strongest LV GLSre with age: r –0.376, p &lt; 0.001). During median follow-up of 5.9[5.5–6.2] years, the endpoint occurred in 53(38%) patients: 4 patients died, 9 developed heart failure, 22 arrhythmias, 8 thrombo-embolic events and 35 re-interventions. Both LV GLS (stan-dardized HR (sHR 0.62(95%CI 0.47–0.81) and GLSre (sHR 0.62(95%CI 0.47–0.83) were associated with the end-point. Additional multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume, NT-proBNP and prior re-interventions. Conclusion: Left ventricular GLS and GLSre are reduced in adult patients with congenital AoS. Both markers are associated with adverse cardiac events and have clear clinical relevance.</p

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

Sexual function is impaired in women and men with pulmonary hypertension

Background: Sexual health related quality of life (SHRQoL) is an important pillar of health related quality of life (HRQoL). The aim of this study was to investigate sexual functioning in men and women with pulmonary hypertension (PH). Methods and results: In this cross-sectional study, a total of 78 patients were included, 49 were diagnosed with pulmonary arterial hypertension and 29 with chronic thromboembolic pulmonary hypertension (median age 53 [IQR: 46–67 years], 66.7% female). All patients completed SHRQoL questionnaires; for women: ASEX, FSFI, and FSDS and for men: ASEX and IIEF. A PH-specific SHRQoL questionnaire was created based on 4 semi-structured interviews to investigate PH-specific barriers in sexuality. More than half of the patients experienced symptoms during sexual activity, mainly dyspnea (52.6%) and palpitations (32.1%). Sexual dysfunction was present, according to the FSFI-questionnaire, in 63.0% of women. All of the men experienced at least mild dysfunction in one of the domains of the IIEF and erectile dysfunction was present in 48.0%. Sexual dysfunction occurred more often in both men and women with PH than in the general population. PAH-specific medication was not associated with sexual dysfunction, nor was subcutaneous or intravenous pump therapy (OR 1.14, 95%-CI: 0.75–1.73). Diuretics were associated with sexual dysfunction in women (OR 4.01, 95%-CI: 1.04–15.41). Of all patients committed in a relationship, 69.0% would like to discuss sexuality with their healthcare provider. Conclusion: This study showed a high prevalence of sexual dysfunction in men and women with PH. It is important for healthcare providers to discuss sexuality with patients. Graphical abstract: [Figure not available: see fulltext.].</p

Left ventricular high frame rate echo-particle image velocimetry: clinical application and comparison with conventional imaging

BACKGROUND: Echo-Particle Image Velocimetry (echoPIV) tracks speckle patterns from ultrasound contrast agent(UCA), being less angle-sensitive than colour Doppler. High frame rate (HFR) echoPIV enables tracking of high velocity flow in the left ventricle (LV). We aimed to demonstrate the potential clinical use of HFR echoPIV and investigate the feasibility and accuracy in patients. METHODS: Nineteen patients admitted for heart failure were included. HFR contrast images were acquired from an apical long axis view (ALAX), using a fully-programmable ultrasound system. A clinical UCA was continuously infused with a dedicated pump. Additionally, echocardiographic images were obtained using a clinical system, including LV contrast-enhanced images and pulsed-wave (PW) Doppler of the LV inflow and outflow in ALAX. 11 patients underwent CMR and 4 cardiac CT as clinically indicated. These CMR and CT images were used as reference. In 10 patients with good echoPIV tracking and reference imaging, the intracavitary flow was compared between echoPIV, conventional and UCA echocardiography. RESULTS: EchoPIV tracking quality was good in 12/19 (63%), moderate in 2/19 (10%) and poor in 5/19 (26%) subjects. EchoPIV could determine inflow velocity in 17/19 (89%), and outflow in 14/19 (74%) patients. The correlation of echoPIV and PW Doppler was good for the inflow (R(2) = 0.77 to PW peak; R(2) = 0.80 PW mean velocity) and moderate for the outflow (R(2) = 0.54 to PW peak; R(2) = 0.44 to PW mean velocity), with a tendency for echoPIV to underestimate PW velocities. In selected patients, echoPIV was able in a single acquisition to demonstrate flow patterns which required multiple interrogations with classical echocardiography. Those flow patterns could also be linked to anatomical abnormalities as seen in CMR or CT. CONCLUSION: HFR echoPIV tracks multidirectional and complex flow patterns which are unapparent with conventional echocardiography, while having comparable feasibility. EchoPIV tends to underestimate flow velocities as compared to PW Doppler. It has the potential to provide in one acquisition all the functional information obtained by conventional imaging, overcoming the angle dependency of Doppler and low frame rate of classical contrast imaging. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12947-022-00283-4