Ventricular assist device implantation in patients with a failing systemic right ventricle: a call to expand current practice.

Ventricular assist device (VAD) implantation is an established treatment modality for patients with end-stage heart failure, and improves symptoms and survival. In the Netherlands, it is not yet routinely considered in patients with congenital heart disease and failing systemic right ventricle (SRV). Recently, a VAD was implanted in 2 SRV patients, one who underwent a Mustard procedure during infancy for transposition of the great arteries (male, 47 years old) and one with a congenitally corrected transposition of the great arteries (male, 54 years old). The first patient is doing well >1 year after implantation; the second patient will be discharged home soon. These examples and other reports demonstrate the feasibility of adopting VAD implantation into routine care for SRV failure. In conclusion, patients with SRV failure may be suitable candidates for VAD implantation: they are relatively young, usually have a preserved subpulmonary left ventricular function, and their specific anatomical and physiological characteristics often make them unsuitable for cardiac transplantation. Therefore it is important to recognise the possibility of VAD implantation early in the process of SRV failure, and to timely refer these patients to a heart failure clinic with experience in VAD implantation in this group of patients for optimisation, screening, and implantation.

The extent of the raphe in bicuspid aortic valves is associated with aortic regurgitation and aortic root dilatation.

BACKGROUND: The clinical course of bicuspid aortic valves (BAVs) is variable. Data on predictors of aortopathy and valvular dysfunction mainly focus on valve morphology. AIM: To determine whether the presence and extent of the raphe (fusion site of valve leaflets) is associated with the degree of aortopathy and valvular dysfunction in patients with isolated BAV and associated aortic coarctation (CoA). METHODS: Valve morphology and aortic dimensions of 255 BAV patients were evaluated retrospectively by echocardiography. RESULTS: BAVs with a complete raphe had a significantly higher prevalence of valve dysfunction (especially aortic regurgitation) than BAVs with incomplete raphes (82.9 vs. 66.7 %, p = 0.01). Type 1A BAVs (fusion of right and left coronary leaflets) and complete raphe had larger aortic sinus diameters compared with the rest of the population (37.74 vs. 36.01, p = 0.031). Patients with CoA and type 1A BAV had significantly less valve regurgitation (13.6 vs. 55.8 %, p < 0.001) and smaller diameters of the ascending aorta (33.7 vs. 37.8 mm, p < 0.001) and aortic arch (25.8 vs. 30.2 mm, p < 0.001) than patients with isolated BAV. CONCLUSIONS: Type 1A BAV with complete raphe is associated with more aortic regurgitation and root dilatation. The majority of CoA patients have incomplete raphes, associated with smaller aortic root diameters and less valve regurgitation.

Detection of pulmonary vasculopathy by novel analysis of oxygen uptake in patients with systemic sclerosis: association with pulmonary arterial pressures.

OBJECTIVES: During cardiopulmonary exercise testing (CPET) compromised pulmonary vasculature in patients with systemic sclerosis (SSc) may lead to increases in pulmonary arterial pressures (PAP) and decreased oxygen uptake. We hypothesised that this may lead into a disproportional heart rate (HR) increase with a corresponding V'O2/HR breakpoint and relates to systolic PAP at rest. METHODS: In a prospective design we evaluated V'O2/HR slopes for breakpoints. To understand its physiological meaning, we evaluated V'O2/HR and V'O2/mPAP slopes for breakpoints in a historic data set of SSc patients, in which CPET and right heart catheterisation was performed simultaneously. V'O2/HR slopes with a peak oxygen uptake outside the normal range were defined as pathologic. RESULTS: A breakpoint occurred in both V'O2/mPAP and V'O2/HR slope in 16/34 patients in the historic dataset and occurred in the V'O2/mPAP slope at a lower V'O2in 15 patients. In the prospective dataset, 73/121 patients showed a V'O2/HR breakpoint and achieved a significantly lower peak oxygen uptake compared to 48/121 patients without a V'O2/HR breakpoint (p=0.036). Mean systolic PAP in 41/121 patients with a pathologic V'O2/HR slope differed significantly from patients without a pathologic V'O2/HR slope (p=0.027). In 27/121 patients with a systolic PAP < 35 mmHg a pathologic V'O2/HR slope was observed. CONCLUSIONS: SSc patients with a V'O2/HR breakpoint are characterised by a decreased oxygen uptake, likely caused by sudden PAP increases during exercise. Importantly, in patients with normal resting SPAP pathologic V'O2/HR slopes were observed. This suggests that these patients are at risk for developing pulmonary hypertension.

Low blood pressure in the very old, a consequence of imminent heart failure: the Leiden 85-plus Study.

Low blood pressure in the very old has been associated with organ dysfunction and excess mortality but the underlying mechanism has yet to be elucidated. We hypothesized that cardiac dysfunction contributes to low blood pressure in the very old. We invited a convenience sample consisting of 82 participants all aged 90 years from a population-based cohort study in the very old. Blood pressure was measured twice, and all but one underwent echocardiography to assess cardiac dimensions and functional cardiac parameters. Some 47 participants were free from haemodynamically significant valvular disease and were included in the present analyses. There were low values for mean cardiac output (2.04 l(-1) min(-1) m(-2), s.e. 0.40) and mean stroke volume (31.4 ml m(-2), s.e. 7.7). For every 10-mm Hg decrease in systolic blood pressure, cardiac output was 0.09 l(-1) min(-1) m(-2) lower (s.e. 0.04, P=0.019), and stroke volume was 1.58 ml m(-2) lower (s.e. 0.68, P=0.024). Mean left ventricular ejection fraction was normal and 2.39% (s.e. 1.16, P=0.046) higher for each 10-mm Hg decrease in systolic blood pressure. Mean left ventricular dimensions were normal but the E/A ratio was reduced (0.68, s.d. 0.21), indicating diastolic dysfunction. In conclusion, among the oldest old, low systolic blood pressure correlates with low cardiac output. Systolic ventricular function is not impaired.

Uncontrolled acromegaly is associated with progressive mitral valvular regurgitation.

INTRODUCTION: Recent cross-sectional studies have documented an association between acromegaly and regurgitant valvular heart disease. The aim of this study was to evaluate the change in prevalence of valvular heart disease in relation to the clinical activity, because the natural history of valvular changes in acromegaly is unknown. PATIENTS AND METHODS: Valvular regurgitation was assessed in 37 acromegalic patients (18 patients with active disease, and 19 with controlled disease) by conventional two-dimensional and Doppler echocardiography before and after an interval of 1.9 years (range 1.5-3.0 years). RESULTS: At baseline, valvular regurgitation (mitral and aortic sites combined) was present in 46% of the patients and increased to 67% at follow-up (P=0.008). Mitral regurgitation increased significantly from 32% to 60% (P=0.002), but no change was noted for the aortic valve (27% vs. 31%, NS). In patients with active disease, valvular regurgitation increased significantly from 56% at baseline to 88% at follow-up (P=0.031) due to a significant increase of mitral regurgitation from 39% to 78% at follow-up (P=0.016). In contrast, no increase in valvular regurgitation was found in patients with controlled disease. CONCLUSION: The prevalence of mitral, but not aortic, valvular regurgitation increased in patients with active acromegaly during follow-up. Patients with acromegaly require adequate cardiac evaluation and follow-up to establish the extent and progression of valvular involvement.

Reversible diastolic dysfunction after long-term exogenous subclinical hyperthyroidism: a randomized, placebo-controlled study.

BACKGROUND: Subclinical hyperthyroidism has been reported to affect systolic and diastolic cardiac function. However, the reversibility of these effects is not well established. OBJECTIVE: Our objective was to investigate the presence and reversibility of cardiac abnormalities in patients with long-term exogenous subclinical hyperthyroidism. DESIGN: We conducted a prospective, single-blinded, placebo-controlled randomized trial of 6 months duration with two parallel groups. SETTING: The study occurred at the Leiden University Medical Center, a tertiary referral center for thyroid carcinoma. PATIENTS: As a model for subclinical hyperthyroidism, 25 patients with a history of differentiated thyroid carcinoma with more than 10 yr of TSH suppressive therapy with L-T4 were studied. INTERVENTIONS: L-T4 dose was replaced by study medication containing L-T4 or placebo. Medication was titrated in a single-blinded fashion to establish continuation of TSH suppression (low-TSH group) or euthyroidism (euthyroid group). MEASUREMENTS: We assessed serum levels of free T4 and TSH and used echo Doppler cardiography including tissue Doppler to establish left ventricular (LV) dimensions and function as well as diastolic function. Baseline echocardiography data were compared with 24 controls. RESULTS: There were no differences in baseline cardiac parameters and TSH levels between the two groups. Although mean LV mass index was increased as compared with 24 controls, only four patients had LV hypertrophy at baseline. This was not improved by restoration of euthyroidism. At baseline, diastolic function was impaired in all patients as indicated by abnormal values for the peak flow of the early filling phase (E, 55.3 +/- 9.5 mm/sec), the ratio of E and the peak flow of the atrial filling phase (E/A ratio, 0.87 +/- 0.13), the early diastolic velocity obtained by tissue Doppler (E', 5.7 +/- 1.3 cm/sec), and the peak atrial filling velocity obtained by tissue Doppler (A', 6.8 +/- 1.4 cm/sec), prolonged E deceleration time (234 +/- 34 msec), and isovolumetric relaxation time (121 +/- 15 msec). After 6 months, significant improvements were observed in the euthyroid group in the E/A ratio (+41%; P < 0.001), E deceleration time (-18%; P = 0.006), isovolumetric relaxation time (-25%; P < 0.001), E' (+31%; P < 0.001), and the E'/A' ratio (+40%; P < 0.001). CONCLUSIONS: We conclude that prolonged subclinical hyperthyroidism is accompanied by diastolic dysfunction that is at least partly reversible after restoration of euthyroidism. Because isolated diastolic dysfunction may be associated with increased mortality, this finding is of clinical significance.

Persistent diastolic dysfunction despite successful long-term octreotide treatment in acromegaly.

INTRODUCTION: This study was designed to evaluate potential reversibility of left-ventricular (LV) dysfunction in patients with acromegaly following long-term control of disease. It is unknown whether the cardiac changes induced by acromegaly can be reversed completely by long-term strict control of growth hormone excess by octreotide. PATIENTS AND METHODS: We compared LV systolic and diastolic function in inactive patients with acromegaly (n = 22), who were divided into patients with long-term control by octreotide (n = 14) and patients with long-term cure by surgery/radiotherapy (n = 8). We also assessed these parameters in patients with active acromegaly (n = 17). RESULTS: In patients with active acromegaly, systolic function at rest was decreased by 18% (P < 0.01), LV mass index increased by 40% (P < 0.04) and isovolumetric relaxation time increased by 19% (P < 0.01), compared with patients with inactive acromegaly. These parameters were not different between well-controlled and cured patients. Using tissue Doppler imaging, the ratio between early and late diastolic velocity (E'/A' ratio) was decreased in active, compared with inactive acromegaly (0.75+/-0.07 versus 1.24+/-0.15; P < 0.01). This E'/A' ratio was considerably higher in cured, compared with octreotide-treated, patients (1.75+/-0.41 versus 1.05+/-0.1; P < 0.01). CONCLUSION: Diastolic function is persistently and significantly more impaired in acromegalic patients with long-term control by octreotide than in surgically cured patients, which points to biological effects of subtle abnormalities in growth hormone secretion. Criteria for strict biochemical control of acromegaly should thus be reconsidered.

Comparison of magnetic resonance imaging studies with enzymatic indexes of myocardial necrosis for quantification of myocardial infarct size.

To evaluate the potential of gadolinium-diethylene triamine pentaacetic acid (DTPA)-enhanced magnetic resonance imaging (MRI) in the quantification of infarct size in patients with a first acute myocardial infarction, 24 patients with a first acute myocardial infarction were studied by electrocardiographic gated MRI at a mean of 4.3 days after the acute event. Multislice, single-phase, T1-weighted, spin-echo MRI in the true short-axis plane was performed 20 minutes after intravenous injection of gadolinium-DTPA (0.15 mmol/kg of body weight). Circumscript myocardial regions of increased signal intensity on gadolinium-DTPA-enhanced images were considered to be infarcted. Infarct size (in g) was determined using Simpson's rule, and was compared with that based on cumulative release of alpha-hydroxybutyrate dehydrogenase activity in plasma and with peak creatine kinase-MB level in plasma. Infarct size quantified with MRI correlated well with "enzymatic" infarct size (in g equivalents) (y = 0.99 x + 0.71; r = 0.93; p = 0.0001) and peak creatine kinase-MB levels (r = 0.72; p = 0.002). It is concluded that gadolinium-DTPA-enhanced MRI enables accurate quantification of infarct size in patients with a first acute myocardial infarction.

Assessment of acute myocardial infarction in man with magnetic resonance imaging and the use of a new paramagnetic contrast agent gadolinium-BOPTA.

To assess the feasibility of and characterize the new paramagnetic contrast agent gadolinium-BOPTA/dimeglumine (Gd-BOPTA) to detect acute myocardial infarctions with MR imaging, 24 patients (53.3 +/- 8.3 yr) were examined 9.3 +/- 3.6 days after a first myocardial infarction. Short-axis T1-weighted and T2-weighted MR imaging was performed at three slice levels. T1-weighted images were obtained before, immediately after, 15, 30, and 45 min after injection. Patients received either 0.05 or 0.1 mmol/kg body weight Gd-BOPTA. Images were qualitatively and quantitatively analyzed. Two patients showed no signs of infarction on T2-weighted images as opposed to contrast-enhanced T1-weighted images. Contrast-to-noise ratio was not affected by the dosage level. Signal intensity (SI) of normal to infarcted myocardium was significantly improved by both dosages (p < .0005) but a dosage of 0.05 mmol/kg produced significantly higher SI inf/norm (1.42 +/- 0.07 vs. 1.34 +/- 0.06, respectively, p = .015). SI of normal and infarcted myocardium enhanced immediately after administration of 0.05 mmol/kg (29.3 +/- 5.1% and 53.8 +/- 9.6% respectively), which decreased thereafter to 5.3 +/- 4.8% and 40.2 +/- 8.5% respectively, at 45 min (p < .002 for normal myocardium). SI enhancement immediately after 0.1 mmol/kg Gd-BOPTA showed no decrease within the first 45 min. Gd-BOPTA enables the detection of myocardial infarction. Optimal infarct delineation is achieved from 15 to 45 min after administration of 0.05 mmol/kg body weight Gd-BOPTA. Gd-BOPTA at 0.05 mmol/kg does improve image quality as measured by contrast-to-noise ratio and SI enhancement as compared to 0.10 mmol/kg.

Sequential analysis of infarcted and normal myocardium in piglets using in vivo gadolinium-enhanced MR images.

Gadolinium-enhanced magnetic resonance (MR) imaging was performed before, and 1 and 3 wk after coronary occlusion in domestic piglets. After administration of the contrast agent gadopentetate dimeglumine, two different enhancement patterns within the infarcted region were observed. The first pattern, showing peripheral enhancement of the infarcted region with absence of contrast in the center, was seen at 1 wk after occlusion at 5 min after administration of the contrast agent. The second pattern showed signal enhancement of the center of the infarcted region and was observed at 1 wk after occlusion, 30 min following contrast administration, and at 3 wk after occlusion, both 5 and 30 min following contrast administration. Infarct size and left ventricular (LV) mass by MR imaging, measured 3 wk after infarction, corresponded well with pathologic assessment. LV mass, measured by static and dynamic MR imaging, increased during the period of investigation. It is concluded that gadolinium-enhanced MR imaging clearly identifies infarcted myocardium early and late after coronary occlusion in the piglet. Combined results of infarct size and LV mass can be obtained simultaneously during one imaging procedure.

Effect of intramyocardial bone marrow cell injection on left ventricular dyssynchrony and global strain.

OBJECTIVE: To evaluate the effect of bone marrow cell injection on global strain and left ventricular (LV) dyssynchrony. METHODS: In 14 patients with severe postinfarction heart failure, 93 (14) x 10(6) autologous bone marrow cells were percutaneously injected in the infarction border zone. LV ejection fraction (LVEF), LV dyssynchrony and echocardiographic global strain were assessed at baseline and 3 months in patients and in a non-randomised control group of 10 patients with a history of infarction who developed heart failure and were treated medically. RESULTS: No periprocedural complications occurred during bone marrow cell injection. At 3 months mean (SD) LVEF increased from 23 (8)% to 27 (9)% (p = 0.02) and global strain improved from -7.7 (4.7)% to -8.5 (4.9)% (p = 0.04). In patients with > or =5% improvement in LVEF after bone marrow cell injection, global strain improved from -8.7 (4.6)% to -10.6 (4.5)% (p<0.01). Global strain remained unchanged in patients with <5% improvement in LVEF (-6.6 (4.9)% vs -6.4 (4.5)%, p = NS). The relation between the increase in LVEF and improvement in global strain was significant (r = 0.84, p<0.01). In patients with > or =5% improvement in LVEF, LV dyssynchrony decreased from 173 (64) ms to 116 (64) ms (p = 0.01). In patients with <5% improvement in LVEF, LV dyssynchrony remained unchanged (155 (67) ms vs 177 (81) ms, p = NS). The correlation between improvement in LVEF and reduction in LV dyssynchrony was good (r = -0.77, p<0.01). In the control group, LVEF, global strain and LV dyssynchrony did not improve. CONCLUSIONS: Bone marrow cell injection improves LVEF in patients with severe postinfarction heart failure. The improvement in LVEF was related to reduced LV dyssynchrony and increased global strain.

No increased risk of valvular heart disease in adult poststreptococcal reactive arthritis.

OBJECTIVE: Poststreptococcal reactive arthritis (ReA) is a (poly)arthritis presenting after a Streptococcus group A infection. Acute rheumatic fever (ARF), albeit caused by the same pathogen, has different risk characteristics and is considered to be a separate entity. Whereas ARF is known to cause carditis, the risk of carditis in adult poststreptococcal ReA is unknown. Consequently, the prevailing recommendations regarding long-term antibiotic prophylaxis in poststreptococcal ReA are imprecise and derived from the data on ARF. This study was undertaken to investigate the development of valvular heart disease in an unselected cohort of adult patients with poststreptococcal ReA who did not receive antibiotic prophylaxis and were followed up prospectively. METHODS: All patients presenting with early arthritis to an inception cohort of >2,000 white patients were evaluated. Patients presenting with poststreptococcal ReA (n = 75) were selected. After a median followup of 8.9 years, the occurrence of valvular heart disease was evaluated by transthoracic echocardiography in 60 patients. Controls were matched for age, sex, body surface area, and left ventricular function, with a patient-to-control ratio of 1:2. RESULTS: No differences were seen in left ventricular dimensions. Morphologic abnormalities of the mitral or aortic valves were not more prevalent among patients than among controls. Mild mitral regurgitation was present in 23% and 21% of patients and controls, respectively. Mild aortic regurgitation was present in 10% and 11%, and mild tricuspid regurgitation in 43% and 39%, respectively, revealing no significant differences. CONCLUSION: Our findings indicate that there is no increased risk of valvular heart disease in adult poststreptococcal ReA. Based on these data, routine long-term antibiotic prophylaxis is not recommended in adult poststreptococcal ReA.

Triplane tissue Doppler imaging: a novel three-dimensional imaging modality that predicts reverse left ventricular remodelling after cardiac resynchronisation therapy.

BACKGROUND: Several two-dimensional (2-D) tissue Doppler imaging (TDI) echocardiographic techniques have proved useful to identify responders to cardiac resynchronisation therapy (CRT). Recently a 3-D probe allowing simultaneous acquisition of TDI data in three imaging planes became available. OBJECTIVE: To evaluate the value of triplane TDI to predict reverse left ventricular (LV) remodelling after CRT. METHODS: Sixty patients with heart failure, scheduled for CRT, underwent triplane echocardiography with simultaneous TDI acquisition before and 6 months after implantation. From the triplane dataset a 3-D LV volume was generated and LV volumes and ejection fraction were calculated. Intraventricular dyssynchrony was quantitatively analysed by evaluating time from onset of the QRS complex to peak myocardial systolic velocity in 12 LV segments from the triplane dataset and calculation of the standard deviation (Ts-SD-12). Clinical response was defined as an improvement of at least one New York Heart Association class. Reverse LV remodelling was defined as >/=15% decrease of LV end-systolic volume at 6 months' follow-up. RESULTS: Responders to CRT had significantly more LV dyssynchrony at baseline than non-responders (mean (SD) Ts-SD-12: 42 (14) vs 22 (12), p<0.001). A cut-off value of 33 ms for baseline Ts-SD-12, acquired from the triplane TDI dataset, yielded a sensitivity of 89% with a specificity of 82% to predict clinical response to CRT; sensitivity and specificity to predict reverse LV remodelling were 90% and 83%, respectively. CONCLUSION: Triplane TDI echocardiography predicts clinical response and reverse LV remodelling 6 months after CRT implantation.

Underestimation of severity of mitral regurgitation with varying hemodynamics.

Transesophageal echocardiography (TEE) is a valuable technique to assess mitral valve anatomy and the mechanism of mitral regurgitation (MR). We present the case of a 35-year-old woman with severe MR due to restrictive motion of the posterior mitral leaflet, who was referred for mitral annuloplasty. Under physiologic circumstances, a severe (grade 3+) MR was present, whereas in the operating room during general anesthesia, the MR had disappeared almost completely. The downgrading of MR due to general anesthesia and the associated mechanisms of this phenomenon are discussed in this case.

Contrast echocardiography as a useful additional diagnostic tool in evaluating a primary cardiac tumor.

The current report describes a case of a primary cardiac lymphoma. For early and appropriate treatment of a cardiac mass it is not only important to determine its localization and extension but also to differentiate between malignant and benign lesions. This report demonstrates that not only transthoracic echocardiography but also the other different forms of echocardiography such as transesophageal echocardiography, as well as contrast and intracardiac echocardiography, are useful tools in the diagnostic workup of cardiac masses.

Quantitation of global and regional left ventricular function by cine magnetic resonance imaging during dobutamine stress in normal human subjects.

Magnetic resonance imaging (MRI) provides high-resolution images of the heart. However, physical exercise during MRI is difficult due to space restriction and motion artefacts. To evaluate the feasibility of MRI during stress conditions, dobutamine was used as an alternative to exercise. Haemodynamics, ventricular volumes and wall thickening were measured at rest and during peak dobutamine infusion (15 micrograms.kg-1 x min-1) in 23 normal human subjects. To calculate left ventricular volumes, eight short-axis views were obtained encompassing the left ventricle from base to apex. At six levels, percent systolic wall thickening (%WTh) was measured in 18 segments (20 degrees intervals). Heart rate, systolic and diastolic blood pressures, stroke index, cardiac output and left ventricular ejection fraction increased significantly during dobutamine infusion (all P values < 0.001). In addition, %WTh increased significantly (P < 0.001) during dobutamine compared to the control state at all levels except in the apical and low-left ventricular levels. Both in control conditions and during dobutamine, segmental wall motion analysis showed the highest %WTh at the posterolateral area and the lowest %WTh at the septal region (P < 0.05). MRI clearly identifies wall motion dynamics and provides calculations of segmental wall thickening and haemodynamic parameters. Dobutamine is a useful stress agent by virtue of its safety, operator control and its effects which resemble physical exercise.