Myxoedematous tamponade as initial presentation of Hashimoto's thyroiditis.

Cardiac tamponade as the initial presentation of hypothyroidism is extremely rare. We report the case of a 48-year-old man admitted for acute respiratory distress, with cardiac ultrasound showing compressive pericardial effusion. Percutaneous pericardiocentesis was performed leading to a rapid clinical improvement. Laboratory tests confirmed severe hypothyroidism related to Hashimoto's disease. Despite hormone replacement therapy, pericardial effusion recurred after 3 weeks, requiring surgical drainage. Pericardial histology highlighted slight chronic fibrous pericarditis. The cardiac ultrasound scan performed 4 months later showed a well-tolerated chronic pericardial effusion. In conclusion, hypothyroidism should be suspected in case of cardiac tamponade especially in the absence of tachycardia, or in winter when myxoedema is prone to decompensation. Prognosis is generally good under hormone replacement therapy but ultrasound monitoring should be carried out at least until euthyroidism is achieved.

Stress Doppler echocardiography in relatives of patients with idiopathic and familial pulmonary arterial hypertension: results of a multicenter European analysis of pulmonary artery pressure response to exercise and hypoxia.

BACKGROUND: This large, prospective, multicentric study was performed to analyze the distribution of tricuspid regurgitation velocity (TRV) values during exercise and hypoxia in relatives of patients with idiopathic and familial pulmonary arterial hypertension (PAH) and in healthy control subjects. We tested the hypothesis that relatives of idiopathic/familial PAH patients display an enhanced frequency of hypertensive TRV response to stress and that this response is associated with mutations in the bone morphogenetic protein receptor II (BMPR2) gene. METHODS AND RESULTS: TRV was estimated by Doppler echocardiography during supine bicycle exercise in normoxia and during 120 minutes of normobaric hypoxia (FIO(2)=12%; approximately 4500 m) in 291 relatives of 109 PAH patients and in 191 age-matched control subjects. Mean maximal TRVs were significantly higher in PAH relatives during both exercise and hypoxia. During exercise, 10% of control subjects but 31.6% of relatives (P<0.0001) exceeded the 90% quantile of mean maximal TRV seen in control subjects. Hypoxia revealed hypertensive TRV in 26% of relatives (P=0.0029). Among control subjects, TRV at rest was not related to age, sex, body mass index, systemic blood pressure, smoking status, or heart rate. Within kindreds identified as harboring deleterious mutations of the BMPR2 gene, a hypertensive TRV response occurred significantly more often compared with those without detected mutations. CONCLUSIONS: Pulmonary hypertensive response to exercise and hypoxia in idiopathic/familial PAH relatives appears as a genetic trait with familial clustering, being correlated to but not caused by a BMPR2 mutation. The suitability of this trait to predict manifest PAH development should be addressed in long-term follow-up studies.

Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension.

Clinical studies have shown the importance of endothelin as a pathogenic mediator in pulmonary arterial hypertension (PAH). We describe the effects of bosentan, an oral dual endothelin receptor antagonist, in patients with PAH associated with human immunodeficiency virus (HIV) infection. In this prospective study, 16 patients with PAH associated with HIV infection in stable condition received bosentan for 16 weeks. Efficacy endpoints included exercise capacity, cardiopulmonary hemodynamics, Doppler echocardiography, New York Heart Association functional class, and quality of life (SF-36 and EQ-5D). Safety was assessed by laboratory tests, vital signs, and adverse events. Improvements were observed from baseline to Week 16 in all efficacy parameters: 6-minute walk distance (+91 +/- 60 m, p < 0.001), New York Heart Association class (14 patients improved), hemodynamics (cardiac index: +0.9 +/- 0.7 L/minute/m(2), p < 0.001), Doppler echocardiographic variables, and quality of life. During the study, no patient died and none required epoprostenol treatment. Hepatic tolerability was similar to that reported in patients with PAH. Bosentan had no negative impact on control of HIV infection. Although limited by uncontrolled design, small sample size and short duration, this study suggests that bosentan may benefit patients with PAH associated with HIV infection, and that endothelin is an important pathogenic mediator in this disease.

Automatic quantification of right ventricular function with gated blood pool SPECT.

BACKGROUND: Quantification of right ventricular (RV) function is clinically relevant for the risk stratification and follow-up of patients with a wide spectrum of disease. This can be achieved with electrocardiography-gated blood pool single photon emission computed tomography (GBPS). We aimed to evaluate the accuracy of the completely automatic QBS GBPS processing software as compared with equilibrium planar radionuclide angiography (RNA) and with a GBPS manual segmentation method (GBPS(35%)) for the measurement of global RV ejection fraction (EF), taking the first-pass RNA (FP-RNA) as the gold standard. In parallel, we compared the RVEF, RV end-diastolic volume (EDV), and RV end-systolic volume (ESV) provided by QBS and GBPS(35%). METHODS AND RESULTS: The population included 85 patients with chronic post-embolic pulmonary hypertension. Twenty-one patients were excluded because of unsuccessful FP-RNA. Intraobserver and interobserver RVEF, RVEDV, and RVESV reproducibilities encountered with planar RNA, QBS, and GBPS(35%) were similar and compared favorably with those calculated with FP-RNA for RVEF. Mean RVEF was different between all methods. RVEF calculated with FP-RNA was better correlated to QBS (r = 0.68) and GBPS(35%) (r = 0.70) than to planar RNA (r = 0.59). RVEDV and RVESV with QBS were lower than with GBPS(35%), by 29% +/- 14% and 36% +/- 13%, respectively. RVEDV and RVESV with QBS were highly correlated to corresponding GBPS(35%) values: r = 0.88 and r = 0.91, respectively. CONCLUSION: As opposed to FP-RNA, GBPS is highly successful for the quantification of RV function. Both QBS and GBPS(35%) provide RVEF values similarly well correlated to FP-RNA and performed better than planar RNA. RVEF, RVEDV, and RVESV provided by QBS and GBPS(35%) are highly correlated. All of these RV functional measurements require further validation versus a better gold standard before their accuracy can be established.

Comparative value of ECG-gated blood pool SPET and ECG-gated myocardial perfusion SPET in the assessment of global systolic left ventricular function.

Both electrocardiographically (ECG) gated blood pool SPET (GBPS) and ECG-gated myocardial perfusion SPET (GSPET) are currently used for the measurement of global systolic left ventricular (LV) function. In this study, we aimed to compare the value of GSPET and GBPS for this purpose. The population included 65 patients who underwent rest thallium-201 GSPET imaging at 15 min after (201)Tl injection followed by planar (planar(RNA)) and GBPS equilibrium radionuclide angiography immediately after 4-h redistribution myocardial perfusion SPET imaging. Thirty-five patients also underwent LV conventional contrast angiography (X-rays). LV ejection fraction (EF) and LV volume [end-diastolic (EDV) and end-systolic (ESV) volumes] were calculated with GBPS and GSPET and compared with the gold standard methods (planar(RNA) LVEF and X-ray based calculation of LV volume). For both LVEF and LV volume, the inter-observer variability was lower with GBPS than with GSPET. GBPS LVEF was higher than planar(RNA) (P<0.01) and GSPET LVEF (P<0.01). Planar(RNA) LVEF showed a slightly better correlation with GBPS LVEF than with GSPET LVEF: r=0.87 and r=0.83 respectively. GSPET LV volume was lower than that obtained using X-rays and GBPS (P<0.01 for both). LV volume calculated using X-rays showed a slightly better correlation with GBPS LV volume than with GSPET LV volume: r=0.88 and r=0.83 respectively. On stepwise regression analysis, the accuracy of GSPET for the measurement of LVEF and LV volume was correlated with a number of factors, including planar(RNA) LVEF, signal to noise ratio, LV volume calculated using X-rays, summed rest score and acquisition scan distance (i.e. the radius of rotation). The accuracy of GBPS for the measurement of LVEF and LV volume was correlated only with the signal level, the signal to noise ratio and the acquisition scan distance. Both GSPET and GBPS provide reliable estimation of global systolic LV function. The better reliability of GBPS and in particular its lower sensitivity to different variables as compared with GSPET favours its use when precise assessment of global systolic LV function is clinically indicated.

Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension.

OBJECTIVES: The purpose of this study was to investigate the effects of bosentan (125 or 250 mg twice daily) on echocardiographic and Doppler variables in 85 patients with World Health Organization class III or IV pulmonary arterial hypertension (PAH). BACKGROUND: Bosentan, an orally active dual endothelin-receptor antagonist, improves symptoms, exercise capacity, and hemodynamics in patients with PAH. METHODS: Patients had primary pulmonary hypertension (84%) or PAH associated with connective tissue disease. Of these, 29 patients received placebo and 56 received bosentan (1:2 randomization). Six-minute walk tests and echocardiograms were performed at baseline and after 16 weeks of treatment. RESULTS: Baseline characteristics were similar in the placebo and bosentan groups, and echocardiographic and Doppler findings were consistent with marked abnormalities of right ventricular (RV) and left ventricular (LV) structure and function that were due to PAH. The treatment effect on 6-min walking distance was 37 m in favor of bosentan (p = 0.036). Treatment effects of bosentan compared with placebo on other parameters were as follows: Doppler-derived cardiac index = +0.4 l/min/m(2) (p = 0.007), LV early diastolic filling velocity = +10.5 cm/s (p = 0.003), LV end-diastolic area = +4.2 cm(2) (p = 0.003), LV systolic eccentricity index = -0.12 (p = 0.047), RV end-systolic area = -2.3 cm(2) (p = 0.057), RV:LV diastolic areas ratio = -0.64 (p = 0.007), Doppler RV index = -0.06 (p = 0.03), and percentage of patients with an improvement in pericardial effusion score = 17% (p = 0.05). CONCLUSIONS: Bosentan improves RV systolic function and LV early diastolic filling and leads to a decrease in RV dilation and an increase in LV size in patients with PAH.

Performance of OSEM and depth-dependent resolution recovery algorithms for the evaluation of global left ventricular function in 201Tl gated myocardial perfusion SPECT.

UNLABELLED: It is unknown whether the use of ordered-subsets expectation maximization (OSEM) and depth-dependent resolution recovery (RR) will increase the accuracy of (201)Tl electrocardiogram-gated SPECT (GSPECT) for the measurement of global left ventricular (LV) function. METHODS: Fifty-six patients having both rest (201)Tl GSPECT and planar equilibrium radionuclide angiography (planar(RNA)) on the same day were studied. Twenty-nine patients also had LV conventional contrast angiography (Rx). LV ejection fraction (LVEF), end-diastolic volume (EDV), and end-systolic volume (ESV) were calculated with the quantitative gated SPECT software (QGS) using 4 different processing methods: filtered backprojection (FBP), OSEM, RR + FBP, and RR + OSEM. LVEF calculated with planar(RNA) and LV EDV and ESV calculated with Rx were considered gold standards. LVEF and volumes provided with the GSPECT methods were compared with the gold standard methods. RESULTS: LVEF calculated with GSPECT methods (FBP, OSEM, RR + FBP, and RR + OSEM) were similar (not statistically significant) and correlated well with planar(RNA). On Bland-Altman analysis, the mean +/- SD of absolute difference in LVEF with GSPECT FBP, OSEM, RR + FBP, and RR + OSEM methods versus planar(RNA) were similar, with relatively large limits of agreement. LV volumes calculated with the 4 GSPECT methods were significantly lower but correlated well with Rx LV volumes. LV volumes calculated with FBP and OSEM were lower than those calculated with RR + FBP and RR + OSEM (P < 0.01). On Bland-Altman analysis, the mean +/- SD of absolute difference in LV volumes with FBP, OSEM, RR + FBP, and RR + OSEM versus Rx was, respectively, 56 +/- 45 mL (P < 0.01 vs. the other 3 methods), 57 +/- 45 mL (P < 0.01 vs. the other 3 methods), 43 +/- 48 mL, and 46 +/- 47 mL, with correspondingly large limits of agreement. The variance of random error did not differ between FBP, OSEM, RR + FBP, and RR + OSEM for either LVEF or volumes. CONCLUSION: OSEM and FBP presented similar accuracy for LVEF and volume measured with the QGS software. Their combination with depth-dependent RR provided similar LVEF but more accurate LV volumes.