Myocardial cleft in a patient with Takotsubo cardiomyopathy: an unusual association identified by cardiac magnetic resonance.

We present the case of an 84-year-old woman admitted for Takotsubo cardiomyopathy complicated by congestive heart failure. Cardiovascular magnetic resonance (CMR) imaging was performed on day five and confirmed severely depressed left ventricular systolic function with typical apical ballooning. In steady-state free precession long-axis cine imaging, a basal inferior myocardial cleft was also observed, with no signs of myocardial noncompaction or regional wall motion abnormalities involving this segment. The pre-discharge CMR study confirmed the presence of a basal inferior myocardial cleft and significant improvement in left ventricular systolic function. Myocardial clefts are congenital abnormalities that have been described in healthy individuals as well as in the setting of hypertrophic cardiomyopathy, but it is not clear whether it is a benign structural variant or a distinct cardiomyopathy phenotype. To our knowledge this is the first reported case of this abnormality in a patient with Takotsubo cardiomyopathy.

Long-term follow-up after aortic coarctation repair: the unsolved issue of exercise-induced hypertension.

INTRODUCTION: Despite successful repair of aortic coarctation (AC), systemic hypertension (HTN) can persist in a significant percentage of patients. Exercise-induced HTN is also common in these patients, although its clinical significance is still unclear. In this study we aimed to assess the prevalence of exercise-induced HTN in adult patients with repaired AC. METHODS: We retrospectively reviewed the clinical records of patients aged >18 years with repaired AC followed at an adult congenital heart disease outpatient clinic in a tertiary care center. Demographic and clinical data including age at intervention, blood pressure (BP) at rest and on exercise, transthoracic echocardiogram (TTE) and treadmill exercise test results were evaluated. Exercise-induced HTN was defined as peak systolic BP ≥ 210 mmHg for men and ≥ 190 mmHg for women. RESULTS: We analyzed 65 patients (40 [61.5%] male; mean age at follow-up 30 ± 8 years). Median age at AC repair was 7 years (P25-P75: 4-20) and mean follow-up was 20 ± 7 years. Only one patient had diabetes and 10 (15.4%) had dyslipidemia. The majority of patients had controlled BP at rest and only nine (18%) were under antihypertensive medication. Forty-nine patients performed a treadmill exercise test. The mean duration of exercise was 10.7 ± 3.1 minutes and mean peak heart rate was 166 ± 18 beats per minute. Eleven (22%) patients had a hypertensive response, among whom only three (33%) had uncontrolled BP at rest. In our study treatment with angiotensin-converting enzyme inhibitors (ACEI) (OR 4.0 [95% CI 1.9-18.1]) and the peak instantaneous gradient in the descending aorta by TTE (OR 8.2 [95% CI 1.8-37.0]) were predictors of a hypertensive response with exercise. Age at surgery and type of AC repair were not associated with a hypertensive response on exercise. CONCLUSIONS: In this study we found a significant prevalence of exercise-induced HTN in adult patients after successful AC repair despite adequate BP control at rest. Exercise-induced HTN was significantly related to higher peak gradient in the descending aorta and treatment with ACEI. These results highlight the complexity of the adult AC population and show that, even after a good surgical result, several patients remain at high cardiovascular risk and require long-term follow-up.

[Giant left ventricular pseudoaneurysm: the diagnostic contribution of different non-invasive imaging modalities]. / Pseudoaneurisma gigante do ventrículo esquerdo: contributo diagnóstico de diferentes modalidades de imagem não invasivas.

Distinguishing between ventricular aneurysm and pseudoaneurysm, although difficult, is of major importance due to the therapeutic and prognostic implications. The present case highlights the pivotal role of non-invasive imaging modalities for differential diagnosis between these entities in order to ensure appropriate management of these patients.

Cardiac 123I-MIBG scintigraphy and arrhythmic risk in left ventricular noncompaction.

Left ventricular noncompaction is an unusual but increasingly recognized cardiomyopathy, the etiology of which is still not definitely established. Clinical presentation includes a wide spectrum of scenarios, including heart failure, thromboembolism and malignant arrhythmias, with half of deaths occurring suddenly. Early detection of LVNC is therefore essential to prevent sudden cardiac death. To our knowledge, this is the first report of the presence of cardiac sympathetic nervous dysfunction, assessed by 123iodine-metaiodobenzylguanidine myocardial scintigraphy, in a patient with LVNC, preserved left ventricular systolic function and exercise-induced nonsustained ventricular tachycardia. This finding may be related to the increased arrhythmic risk observed in this cardiomyopathy, giving a new insight into the pathophysiology of LVNC.

Cardiac metastasis from epidermoid esophageal cancer mimicking anterior myocardial infarction.

Cardiac metastases are more common than primary tumors. Several types of malignant tumors have been reported to metastasize to the heart, mainly lung cancer, but in the setting of esophageal cancer, myocardial metastasis is comparatively rare. We report a case of a cardiac metastasis from esophageal squamous cell carcinoma detected 9 months after surgically curative esophagectomy, which presented mimicking acute myocardial infarction. The use of different imaging modalities was fundamental to a correct diagnosis considering the challenging presentation.

Long-term survival, autonomy, and quality of life of elderly patients undergoing aortic valve replacement.

AIMS: We reviewed the long-term survival, autonomy, and quality of life (QoL) of elderly patients undergoing aortic valve replacement (AVR). METHODS: Records of patients ≥75 years old that underwent AVR from 2002 to 2006 were retrospectively analyzed. Functional status was classified with Barthel Index (BI). QoL was presumed as the self-perception of well-being after AVR. Independent predictors of mortality were identified using the Cox proportional hazards model. RESULTS: We included 114 patients, with a mean age of 78.5 ± 2.5 years. Seventy (59.8%) patients were females. Mean additive and logistic EuroSCORE were 7 ± 2 and 9 ± 7, respectively. Follow-up on vital status was achieved for 113 (99.1%) patients after a mean period of 47.2 ± 23.4 months. Twenty-seven (23.7%) patients died (including three operative deaths). Survival up to one, three, and five years of follow-up was 94.4%, 86.7%, and 76.1%, respectively. Multivariate analysis showed that pulmonary hypertension and diabetes were independent predictors of all-cause mortality. Information on BI score and QoL was obtained for 77 (89.5%) and patients. Among those, 69 (89.6%) were autonomous according to BI and 72 (93.5%) considered having had an improvement in QoL. CONCLUSION: Patients ≥75 years old undergoing AVR presented good medium-term survival. Predictors of an adverse outcome were significant pulmonary hypertension and diabetes. At follow-up, most achieved improvement of QoL and remained autonomous. These results stress that excellent long-term outcomes with AVR can be achieved in appropriately selected elderly patients.

The role of cardiovascular magnetic resonance in the evaluation of chest pain etiology: a case report.

In the evaluation of patients with chest pain it is crucial to differentiate between cardiac and non-cardiac etiology. In turn, acute chest pain of cardiac origin includes many clinical entities. The combination of elevated cardiac serum markers, particularly troponin, with typical clinical presentation and electrocardiographic abnormalities most frequently establishes a diagnosis of acute coronary syndrome. However, when coronary angiography reveals only non-significant lesions, this may present a diagnostic dilemma. The authors present the case of a patient admitted with chest pain, initially interpreted as an acute coronary syndrome, based on laboratory parameters and electrocardiographic abnormalities. Coronary angiography showed normal coronary arteries. This result, combined with the findings of subsequent cardiovascular magnetic resonance (myocardial edema and subepicardial delayed enhancement), established a diagnosis of myocarditis. The case illustrates the application of this noninvasive imaging technique in the diagnostic work-up of patients with acute chest pain.

Spontaneous coronary artery dissection: a diagnosis to consider in acute coronary syndromes.

Spontaneous coronary artery dissection is an extremely rare cause of acute coronary syndrome, with a reported angiographic incidence ranging between 0.1 and 1.1%. It mainly affects young patients, in particular women in the peripartum period or taking oral contraceptives, without possessing a definite relation with the classic risk factors for coronary artery disease. The left coronary artery system is the most frequently diseased, although the pattern of artery involvement is gender-based. Clinically, the presentation spectrum ranges from mild symptoms to sudden cardiac death. The major pathophysiologic processes underlying medial dissection are the presence of an intimal tear or the formation of an intramural hematoma. Several treatment modalities have been reported with varying success, although owing to the rarity of this condition, the optimal treatment options remain to be established.

Thymulin inhibits monocrotaline-induced pulmonary hypertension modulating interleukin-6 expression and suppressing p38 pathway.

The pathogenesis of pulmonary hypertension (PH) includes an inflammatory response. Thymulin, a zinc-dependent thymic hormone, has important immunobiological effects by inhibiting various proinflammatory cytokines and chemokines. We investigated morphological and hemodynamic effects of thymulin administration in a rat model of monocrotaline (MCT)-induced PH, as well as the pattern of proinflammatory cytokine gene expression and the intracellular pathways involved. Adult Wistar rats received an injection of MCT (60 mg/kg, sc) or an equal volume of saline. One day after, the animals randomly received during 3 wk an injection of saline, vehicle (zinc plus carboxymethyl cellulose), or thymulin (100 ng/kg, sc, daily). At d 23-25, the animals were anesthetized for hemodynamic recordings, whereas heart and lungs were collected for morphometric and molecular analysis. Thymulin prevented morphological, hemodynamic, and inflammatory cardiopulmonary profile characteristic of MCT-induced PH, whereas part of these effects were also observed in MCT-treated animals injected with the thymulin's vehicle containing zinc. The pulmonary thymulin effect was likely mediated through suppression of p38 pathway.

Left atrial stiffness and its implications for cardiac function.

Proper atrial function is essential for overall cardiovascular performance, mainly by its four major functions, namely, bioelectrical, hormonal, metabolic and hemodynamic. With regard to the latter, atria modulate ventricular filling by smoothing the transformation of the continuous venous return to the intermittent filling pattern of the ventricles during diastole through three main components: a phase of reservoir mainly during ventricular systole, a conduit phase during ventricular diastole and an active phase in late ventricular diastole. Although the atria assume a dynamic role in ventricular filling progression, atrial function and emptying pattern is, conversely, highly influenced by the ventricular diastolic wall stress, underlying the close connection observed between these chambers. This review focuses essentially on left atrial mechanical role, particularly on the physiological and clinical consequences of disturbed atrial compliance.

Thrombin induces rapid PAR1-mediated non-classical FGF1 release.

Thrombin induces cell proliferation and migration during vascular injury. We report that thrombin rapidly stimulated expression and release of the pro-angiogenic polypeptide fibroblast growth factor 1 (FGF1). Thrombin failed to induce FGF1 release from protease-activated receptor 1 (PAR1) null fibroblasts, indicating that this effect was dependent on PAR1. Similarly to thrombin, FGF1 expression and release were induced by TRAP, a specific oligopeptide agonist of PAR1. These results identify a novel aspect of the crosstalk between FGF and thrombin signaling pathways which both play important roles in tissue repair and angiogenesis.

Endogenous production of ghrelin and beneficial effects of its exogenous administration in monocrotaline-induced pulmonary hypertension.

We investigated the endogenous production of ghrelin as well as cardiac and pulmonary vascular effects of its administration in a rat model of monocrotaline (MCT)-induced pulmonary hypertension (PH). Adult Wistar rats randomly received a subcutaneous injection of MCT (60 mg/kg) or an equal volume of vehicle. One week later, animals were randomly assigned to receive a subcutaneous injection of ghrelin (100 mug/kg bid for 2 wk) or saline. Four groups were analyzed: normal rats treated with ghrelin (n=7), normal rats injected with saline (n=7), MCT rats treated with ghrelin (n=9), and MCT rats injected with saline (n=9). At 22-25 days, right (RV) and left ventricular (LV) pressures were measured, heart and lungs were weighted, and samples were collected for histological and molecular analysis. Endogenous production of ghrelin was almost abolished in normal rats treated with ghrelin. In MCT-treated animals, pulmonary expression of ghrelin was preserved, and RV myocardial expression was increased more than 20 times. In these animals, exogenous administration of ghrelin attenuated PH, RV hypertrophy, wall thickening of peripheral pulmonary arteries, and RV diastolic disturbances and ameliorated LV dysfunction, without affecting its endogenous production. In conclusion, decreased tissular expression of ghrelin in healthy animals but not in PH animals suggests a negative feedback in the former that is lost in the latter. A selective increase of ghrelin mRNA levels in the RV of animals with PH might indicate distinct regulation of its cardiac expression. Finally, ghrelin administration attenuated MCT-induced PH, pulmonary vascular remodeling, and RV hypertrophy, indicating that it may modulate PH.

Opioid receptors and preconditioning of the heart.

Recent studies have demonstrated that opioids and their receptors, investigated for many years for their analgesic effects on central nervous system receptors, also play a role in the regulation of the cardiovascular system. Although some of these actions may be mediated by activation of peripheral opioid receptors, others seem to result from direct or independent effects of their receptors on cardiac tissue and the peripheral vascular system. This review will focus on the effects of opioid receptor stimulation in the heart, particularly those mimicking the cardioprotective effect of ischemic preconditioning.

Determinants of afterload-induced diastolic dysfunction.

Severe left ventricular (LV) afterload elevations induce diastolic dysfunction (DD). This decompensatory response, observed in both dogs and rabbits, seems to be related to cytosolic calcium levels. As rats have different calcium kinetics, we investigated the effects of acute LV afterload elevation in rats. Anaesthetized Wistar rats (n = 10) were instrumented in order to record simultaneously LV pressure and septal-free wall dimensions. Afterload was elevated through partial or total occlusions of the ascending aorta. In afterloaded heartbeats, we measured the time constant tau, the necessary (NTR) and the available (ATR) time intervals to complete LV relaxation. DD was evaluated through analysis of the position of the end-diastolic pressure-volume relation. Afterload elevations elicited acceleration of relaxation and DD was observed only in isovolumetric heartbeats. This DD could be explained by the deficit in time to complete relaxation in isovolumetric heartbeats. We conclude, therefore, that the time constant tau and ATR are the major determinants of afterload-induced DD. The calcium kinetics of the rat provide a better adaptation to afterload elevations in this than in other species.

Evaluation of biventricular function in the rat: a new experimental model.

The use of small animals in cardiovascular research has increased over recent years. This might be a limitation when evaluation of biventricular function is required. Although evaluation of left ventricular (LV) pressure and volume is already possible in small animals, concomitant evaluation of right ventricle function has been limited to large animals. The study describes a new model to assess pressures and dimensions of both ventricles simultaneously in the adult rat. Adult Wistar rats (n = 12), weighing 372 +/- 16 g, were anesthetized with pentobarbital (60 mg/kg, i.p.) and ventilated through a tracheostomy (60 cpm, 1 ml/100 g). Under a dissecting microscope (6x) the right jugular vein was catheterized. After sternotomy and pericardiotomy, three crystals were placed along the major cardiac transverse diameter: in the right subendocardium of the interventricular septum and on the epicardial surfaces of the RV and LV free walls. In addition, two high-fidelity catheters were introduced through the apex into the RV (2F, Millar) and LV (3F, Millar) cavities. This allowed the measurement of all parameters derived from pressure and dimension curves of the RV and LV, including pressure-dimension loops. This study describes, for the first time, a model that allows simultaneous evaluation of biventricular pressure and dimensions in an animal model as small as an adult rat. This model opens up new perspectives for the establishment of correlations between molecular biology and hemodynamic data in both ventricles, which is particularly important as more differences between the two ventricles are being found.