Coronary Microvascular Dysfunction Induced by Primary Hyperparathyroidism is Restored After Parathyroidectomy

Background— Symptomatic primary hyperparathyroidism (PHPT) is associated with increased cardiovascular mortality. However, data on the association between asymptomatic PHPT and cardiovascular risk are lacking. We assessed coronary flow reserve (CFR) as a marker of coronary microvascular function in asymptomatic PHPT of recent onset. Methods and Results— We studied 100 PHPT patients (80 women; age, 58±12 years) without cardiovascular disease and 50 control subjects matched for age and sex. CFR in the left anterior descending coronary artery was detected by transthoracic Doppler echocardiography, at rest, and during adenosine infusion. CFR was the ratio of hyperemic to resting diastolic flow velocity. CFR was lower in PHPT patients than in control subjects (3.0±0.8 versus 3.8±0.7; P <0.0001) and was abnormal (≤2.5) in 27 patients (27%) compared with control subjects (4%; P =0.0008). CFR was inversely related to parathyroid hormone (PTH) levels ( r =−0.3, P <0.004). In patients with CFR ≤2.5, PTH was higher (26.4 pmol/L [quartiles 1 and 3, 16 and 37 pmol/L] versus 18 [13–25] pmol/L; P <0.007), whereas calcium levels were similar (2.9±0.1 versus 2.8±0.3 mmol/L; P =0.2). In multivariable linear regression analysis, PTH, age, and heart rate were the only factors associated with CFR ( P =0.04, P =0.01, and P =0.006, respectively). In multiple logistic regression analysis, only PTH increased the probability of CFR ≤2.5 ( P =0.03). In all PHPT patients with CFR ≤2.5, parathyroidectomy normalized CFR (3.3±0.7 versus 2.1±0.5; P <0.0001). Conclusions— PHPT patients have coronary microvascular dysfunction that is completely restored after parathyroidectomy. PTH independently correlates with the coronary microvascular impairment, suggesting a crucial role of the hormone in explaining the increased cardiovascular risk in PHPT

Giant Aneurysm of the Right Atrial Appendage in a 39-Year-Old Woman

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Alström Syndrome: Cardiac Magnetic Resonance findings.

BACKGROUND: Alström Syndrome (ALMS) is an extremely rare multiorgan disease caused by mutations in ALMS1. Dilated cardiomyopathy (DCM) is a common finding but only one series has been investigated by Cardiac Magnetic Resonance (CMR). METHODS: Eight genetically proven ALMS patients (ages 11-41) underwent CMR performed by standard cine steady state, T1, T2 and late gadolinium enhancement (LGE) sequences. Ejection fraction (EF), Diastolic Volume (EDV) and Systolic Volume normalized for body surface area (ESV), and mass indices were determined, as well as EDV/Mass ratio, an index expressing the adequacy of cardiac mass to heart volume. Regional fibrosis was assessed by LGE; diffuse fibrosis was measured by a TI scout sequence acquired at 5, 10 and 15min after gadolinium by comparing inversion time values (TI) at null time in ALMS and control group. RESULTS: In one patient severe DCM was present with diffuse LGE. There were seven cases without clinical DCM. In these patients, EF was at lower normal limits or slightly reduced and ESV index increased; six patients had decreased mass index and EDV/Mass ratio. Mild regional non ischemic fibrosis was detected by LGE in three cases; diffuse fibrosis was observed in all cases, as demonstrated by shorter TI values in ALMS in comparison with controls (5min: 152±12 vs 186±16, p 0.0002; 10min: 175±8 vs 204±18, p 0.0012; 15min: 193±9 vs 224±16, p 0.0002). CONCLUSIONS: Cardiac involvement in ALMS is characterized by progressive DCM, associated with systolic dysfunction, myocardial fibrosis and reduced myocardial mass. Int J Cardiol 2013 Aug 20; 167(4):1257-63

Apicobasal gradient of left ventricular myocardial edema underlies transient T-wave inversion and QT interval prolongation (Wellens\u2019 ECG pattern) in Tako-Tsubo cardiomyopathy

BACKGROUND:Tako-Tsubo cardiomyopathy (TTC) presents with chest pain, ST-segment elevation followed by T-wave inversion and QT interval prolongation (Wellens' electrocardiographic [ECG] pattern), and left ventricular dysfunction, which may mimic an acute coronary syndrome. OBJECTIVE:To assess the pathophysiologic basis of the Wellens' ECG pattern in TTC by characterization of underlying myocardial changes by using cardiac magnetic resonance (CMR).METHODS:The study population included 20 consecutive patients with TTC (95% women; mean age 65.3 \ub1 10.4 years) who underwent CMR studies both in the initial phase and after 3-month follow-up by using a protocol that included cine images, T2-weighted sequences for myocardial edema, and post-contrast sequences for late gadolinium enhancement. Quantitative ECG indices of repolarization, such as maximal amplitude of negative T waves, sum of the amplitudes of negative T waves, and maximum corrected QT interval (QTc max), were correlated to CMR findings. RESULTS:At the time of initial CMR study, there was a significant linear correlation between the apicobasal ratio of T2-weighted signal intensity for myocardial edema and the maximal amplitude of negative T waves (\u3c1 = 0.498; P = .02), sum of the amplitudes of negative T waves (\u3c1 = 0.483; P = .03), and maximum corrected QT interval (\u3c1 = 0.520; P = .02). Repolarization indices were unrelated to either late gadolinium enhancement or quantitative cine parameters. Wellens' ECG abnormalities and myocardial edema showed a parallel time course of development and resolution on initial and follow-up CMR studies. CONCLUSIONS:Our study results show that the ischemic-like Wellens' ECG pattern in TTC coincides and quantitatively correlates with the apicobasal gradient of myocardial edema as evidenced by using CMR. Dynamic negative T waves and QTc prolongation are likely to reflect the edema-induced transient inhomogeneity and dispersion of repolarization between apical and basal left ventricular regions

Alström Syndrome: Cardiac Magnetic Resonance findings.

BACKGROUND: Alström Syndrome (ALMS) is an extremely rare multiorgan disease caused by mutations in ALMS1. Dilated cardiomyopathy (DCM) is a common finding but only one series has been investigated by Cardiac Magnetic Resonance (CMR). METHODS: Eight genetically proven ALMS patients (ages 11-41) underwent CMR performed by standard cine steady state, T1, T2 and late gadolinium enhancement (LGE) sequences. Ejection fraction (EF), Diastolic Volume (EDV) and Systolic Volume normalized for body surface area (ESV), and mass indices were determined, as well as EDV/Mass ratio, an index expressing the adequacy of cardiac mass to heart volume. Regional fibrosis was assessed by LGE; diffuse fibrosis was measured by a TI scout sequence acquired at 5, 10 and 15min after gadolinium by comparing inversion time values (TI) at null time in ALMS and control group. RESULTS: In one patient severe DCM was present with diffuse LGE. There were seven cases without clinical DCM. In these patients, EF was at lower normal limits or slightly reduced and ESV index increased; six patients had decreased mass index and EDV/Mass ratio. Mild regional non ischemic fibrosis was detected by LGE in three cases; diffuse fibrosis was observed in all cases, as demonstrated by shorter TI values in ALMS in comparison with controls (5min: 152±12 vs 186±16, p 0.0002; 10min: 175±8 vs 204±18, p 0.0012; 15min: 193±9 vs 224±16, p 0.0002). CONCLUSIONS: Cardiac involvement in ALMS is characterized by progressive DCM, associated with systolic dysfunction, myocardial fibrosis and reduced myocardial mass

Coronary flow reserve is related to the extension and transmurality of myocardial necrosis and predicts functional recovery after acute myocardial infarction

Background: Few studies have examined the effect of transmurality of myocardial necrosis on coronary microcirculation. The aim of this study was to examine the influence of cardiac magnetic resonance-derived (GE-MRI) structural determinants of coronary flow reserve (CFR) after anterior myocardial infarction (STEMI), and their predictive value on regional functional recovery. Methods: Noninvasive CFR and GE-MRI were studied in 37 anterior STEMI patients after primary coronary angioplasty. The wall motion score index in the left descending anterior coronary artery territory (A-WMSI) was calculated at admission and follow-up (FU). Recovery of regional left ventricular (LV) function was defined as the difference in A-WMSI at admission and FU. The necrosis score index (NSI) and transmurality score index (TSI) by GE-MRI were calculated in the risk area. Baseline (BMR) and hyperemic (HMR) microvascular resistance, arteriolar resistance index (ARI), and coronary resistance reserve (CRR) were calculated at the Doppler echocardiography. Results: Bivariate analysis indicated that the CPK and troponin I peak, heart rate, NSI, TSI, BMR, the ARI, and CRR were related to CFR. Multivariable analysis revealed that TSI was the only independent determinant of CFR. The CFR value of &gt;2.27, identified as optimal by ROC analysis, was 77% specific and 73% sensitive with accuracy of 76% in identifying patients with functional recovery. Conclusions: Preservation of microvascular function after AMI is related to the extent of transmurality of myocardial necrosis, is an important factor influencing regional LV recovery, and can be monitored by noninvasive CFR

Apico-Basal Gradient Of Left Ventricular Myocardial Edema Underlies Transient T-Wave Inversion And Qt Interval Prolongation (Wellens' Ecg Pattern) In Tako-Tsubo Cardiomyopathy.

BACKGROUND: Tako-Tsubo cardiomyopathy (TTC) presents with chest pain, ST-segment elevation followed by T-waves inversion and QT interval prolongation (Wellens' ECG pattern) and left ventricular (LV) dysfunction, which may mimic an acute coronary syndrome. OBJECTIVE: This study assessed the pathophysiologic basis of the Wellens' ECG pattern in TTC by characterization of underlying myocardial changes using cardiac magnetic resonance (CMR). METHODS: The study population included 20 consecutive patients with TTC (95% females,mean age 65.3\ub110.4 years) who underwent CMR studies both in the initial phase and after 3-months of follow-up, using a protocol which included cine images, T2-weighted sequences for myocardial edema and post-contrast sequences for late gadolinium enhancement (LGE). Quantitative ECG indexes of repolarization, such as maximal amplitude of negative T-waves (NTWm), sum of the amplitudes of negative T waves (NTWs) and maximum QT interval (QTc max), were correlated to CMR findings. RESULTS: At the time of initial CMR study, there was a significant linear correlation between apico-basal ratio of T2 signal intensity (SI) for myocardial edema and NTWm (\u3c1=0.498;p=0.02), NTWs (\u3c1=0.483;p=0.03) and QTc max (\u3c1=0.520;p=0.02). Repolarization indexes were unrelated to either LGE or quantitative cine parameters. Wellens' ECG abnormalities and myocardial edema showed a parallel time course of development and resolution on initial and follow-up CMR studies. CONCLUSIONS: Our study results show that the ischemic-like Wellens' ECG pattern in TTC coincides and quantitatively correlates with the apico-basal gradient of myocardial edema as evidenced by CMR. Dynamic negative T-waves and QTc prolongation are likely to reflect the edema-induced transient inhomogeneity and dispersion of repolarization between apical and basal LV regions