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

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

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

Cardiac dysfunction during exercise in young adults with bronchopulmonary dysplasia

Background Worldwide, 1–2% of children is born premature and at risk for developing bronchopulmonary dysplasia (BPD). Preterm born adults are at risk for early cardiovascular disease. The role of BPD is unclear.Aim This study aims to examine cardiorespiratory function during submaximal exercise in young adult survivors of extreme prematurity, with or without BPD.Methods Forty premature born young adults, 20 with BPD (median[IQR] gestational age (GA), 27 [26–28] weeks) and 20 without BPD (GA age 28 [27–29] weeks) were prospectively compared to age-matched at term born (AT) adults (GA 39 [38–40] weeks). Participants underwent exercise testing and cardiovascular magnetic resonance (CMR) with submaximal exercise.Results Resting heart rate in BPD subjects was higher than in AT born subjects (69±10 versus 61±7 mL, p=0.01). Peak oxygen uptake at maximal CPET was decreased in BPD patients (91±18 versus 106±17% of predicted, p=0.01). In BPD subjects cardiac stroke volume (SV) change with exercise was impaired compared to AT subjects (11±13% versus 25±10%; p&lt;0.001). With exercise, left ventricular end-diastolic volume decreased more in preterm borns with versus without BPD (−10±8% versus −3±8%; p=0.01) and compared to AT subjects (0±5%; p&lt;0.001). Exploratory data analysis revealed that exercise SV and end-diastolic volume change were inversely correlated with oxygen dependency in those born prematurely.Conclusions In preterm born young adults –particularly those with BPD– resting cardiac function, exercise performance and cardiac response to exercise is impaired compared to controls. Exercise CMR may reveal an important predisposition for heart disease later in life

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