Echocardiographic evaluation of right ventricular-arterial coupling in pulmonary hypertension.

Pulmonary hypertension (PH) is a hemodynamic condition characterized by chronically elevated mean pulmonary artery pressure (m-PAP ≥ 25 mmHg) measured at rest by right heart catheterization (RHC). It includes a pre-capillary and a post-capillary form. Pulmonary artery hypertension (PAH) is a pre-capillary form of PH potentially generated by several heterogeneous systemic disorders, whose main hemodynamic change is represented by severely increased pulmonary vascular resistance (PVR). In order to preserve an efficient right ventricular-arterial (RV-PA) coupling, the right ventricle (RV) adapts to this chronic increase of its afterload, with a compensatory hypertrophy, until RV dilatation and dysfunction occur. Right ventricular (RV) function and especially RV-PA coupling assessment showed to be very important prognostic markers in this subset of patients, especially for those with pre-capillary PH. The aim of this review is to provide a pathophysiological insight into the spectrum of RV adaptive changes occurring in response to chronic increase of RV afterload and to present the role of echocardiographic parameters as possible tools for early non-invasive evaluation of RV-PA coupling, before overt heart failure ensues.

Impaired myocardial strain in early stage of Duchenne muscular dystrophy: its relation with age and motor performance.

Duchenne muscular dystrophy (DMD) is complicated by an early and progressive left ventricular (LV) dysfunction. Despite the reduction of ejection fraction (EF) usually manifests in the second decade, subtle alterations in LV mechanics can be detected earlier. Longitudinal and circumferential LV deformation, evaluated by speckle tracking echocardiography (STE), are considered sensitive markers of early dysfunction. We retrospectively examined clinical and echocardiographic data of 32 DMD children with preserved LV function. According to the median age, patients were then divided into younger and older than 9 years, and compared to 24 age-matched healthy subjects. Six-minute-walk test (6MWT), North Star Ambulatory Assessment (NSAA), and a comprehensive cardiac evaluation were performed. Although EF was within the normal range, DMD patients had significantly lower values than healthy controls, and the same occurred for the remaining conventional systolic and diastolic indices. Global longitudinal strain (GLS) was reduced in all patients (older and younger, both p < 0.001). Global circumferential strain (GCS) was reduced only in older patients (< 0.001). Both GLS and GCS worsened with age in DMD patients (GLS p = 0.005; GCS p = 0.024). GLS was significantly worse in the apical segments and in the postero-lateral wall. GCS in the antero-septal, anterior and antero-lateral segments was significantly reduced in older patients, with a prevalent involvement of the sole septal wall in the younger boys. 6MWT appeared to be correlated inversely to GLS and directly to EF. A longitudinal evaluation should be scheduled in DMD boys to assess the global cardiac performance over time and to evaluate the impact of therapies.

Ten Years of 2D Longitudinal Strain for Early Myocardial Dysfunction Detection: A Clinical Overview.

In recent years, the role of left ventricular ejection fraction (EF) as the gold standard parameter for the evaluation of systolic function has been questioned, and many efforts have been concentrated in the clinical validation of new noninvasive tools for the study of myocardial contractility. Improvement in the accuracy of speckle-tracking echocardiography has resulted in a large amount of research showing the ability of two-dimensional strain to overcome EF limitations in the majority of primary and secondary heart diseases. Currently, global longitudinal strain (GLS) is considered the most accurate and sensitive parameter for the assessment of early left ventricular dysfunction. This review summarizes the advantages that this measurement can provide in several clinical settings. Moreover, the important cautions that should be considered in making the choice to use GLS also are addressed. Finally, a special focus on bull's-eye polar maps for the assessment of regional changes of longitudinal function and the usefulness of these maps in the differential diagnosis of several diseases is provided.

Impairment of elastic properties of the aorta in bicuspid aortic valve: relationship between biomolecular and aortic strain patterns.

Aims: Bicuspid aortic valve (BAV) is associated with aortic wall alterations. We aimed to detect any correlation between aortic elasticity and genetic and biomolecular patterns of elastin. Methods and results: Forty-nine BAV patients (mean age: 38 ± 17.05) were prospectively enrolled. A blood sample was drawn for analysis of a single nucleotide polymorphism of elastin gene (ELN rs2071307) responsible for misfolding of elastin, and for the amount of elastin soluble fragments (ESF) in the plasma. Aortic dimensions and elastic properties were determined by echocardiography, aortic stiffness (AS) by M-mode analysis, and longitudinal strain (LS) of the ascending aorta (AA) by speckle-tracking echocardiography; values of aortic strain were compared with 45 age-matched subjects (mean age: 33 ± 9.67) with tricuspid aortic valve (TAV). BAV patients had greater aortic dimensions [Valsalva sinus (P = 0.004), sinotubular junction (P = 0.013), AA (P < 0.001)] and stiffness (P = 0.002) but lower LS (P = 0.04) than those with TAV. Results from comparisons of mutated genotype patients (AA, n = 10) with heterozygous (GA, n = 21) and wild-types ones (GG, n = 16) revealed that the presence of mutation was associated with increased ESF (P = 0.010 GG vs. GA; P = 0.035 GA vs. AA), larger AA (P = 0.019 GG vs. GA; P = 0.001 GG vs. AA), and lower LS (P = 0.032 GG vs. AA). Patients with a dilated AA showed greater ESF (P < 0.001), greater AS (P = 0.007), and lower LS of the AA (P = 0.002) than those with a normal AA. The same parameters were not significantly different comparing patients with moderate or severe aortic valve disease and patients with less than moderate valve disease. Conclusions: Our results show a close correlation between genetic and biomolecular patterns of elastin and mechanical properties of the aorta in patients with BAV.

An Unusual Case of Aortic-Right Atrium Fistula: A Diagnostic and Therapeutic Challenge.

An aorta-to-right atrium (RA) fistula is an anomalous communication between the ascending or descending thoracic aorta and the RA. In this report, we describe a case of an idiopathic aortic root-to-RA fistula occasionally found during a coronary angiography performed in a young patient admitted for acute chest pain with evidence of multivessel coronary artery disease. The anatomical peculiarity of this fistulous communication is that it gave origin to collateral vessels furnishing the inferolateral wall of the left ventricle. The case represented a diagnostic and therapeutic challenge that required a multimodality imaging and a multidisciplinary team approach.

[A case of pulmonary artery sarcoma and review of the literature]. / Un caso di angiosarcoma polmonare e revisione della letteratura.

Pulmonary artery sarcoma (PAS) is a highly malignant tumor that originates in the pulmonary artery. This disease has a poor prognosis. Early diagnosis followed by radical surgical resection constitutes the only chance of survival for patients. However, owing to the rare and nonspecific clinical manifesta-tions and imaging findings, PAS is frequently misdiagnosed as various pulmonary thromboembolic diseases. We report the case of a 49-year-old woman who presented to our emergency department for dyspnea, hemoptysis, cough, and asthenia. A diagnosis of pulmonary thromboembolism was initially postulated. However, the rapid clinical progression of the disease, characterized by multiorgan involvement, together with the persistence of pulmonary filling defects despite appropriate anticoagulation therapy, induced to a late diagnosis of metastasized PAS. The peculiarity of our case consists of two main factors: the first is the atypical clinical presentation characterized by severe neurological impairment that finally led to patient's death; the second is the histopathological aspect of the lesion with a prevalent histiocytic cell component that is described in the literature as the less frequent pathological variant of this tumor.

[Combined left atrial appendage percutaneous closure and atrial fibrillation ablation: a single center experience]. / Procedura combinata di chiusura percutanea di auricola sinistra e ablazione di fibrillazione atriale: esperienza di un singolo centro.

BACKGROUND: We evaluated long-term safety and efficacy of concomitant left atrial appendage (LAA) closure and atrial fibrillation (AF) ablation. METHODS: From February 2013 to June 2017, all patients referred for AF ablation and LAA closure (group 1) were enrolled in the study and compared with a matched control group undergoing AF ablation only (group 2). Pulmonary vein isolation was achieved in all cases with radiofrequency or cryoballoon. LAA was occluded with Watchman or Amplatzer Cardiac Plug or Amulet (ACP) devices. All patients were treated with oral anticoagulation therapy for at least 3 months after the procedure ("blanking period"), and then switched to dual antiplatelet therapy with aspirin and clopidogrel for other 3 months, and then to single antiplatelet therapy with aspirin in case of LAA closure, while group 2 was treated with long-term oral anticoagulation therapy according to CHA2DS2-VASc score. Follow-up was performed with transesophageal echocardiography and clinical visit at 3, 6 and 12 months after the procedure. AF burden was evaluated by loop recorder or pacemaker interrogation in all patients. RESULTS: Overall, 42 patients were enrolled, 21 in each group. Mean age was 66.86 ± 10.35 years in group 1 vs 68.42 ± 10.61 in group 2 (p=NS); mean CHA2DS2-VASc score was 2.8 ± 1.22 in group 1 vs 2.01 ± 0.93 in group 2 (p=NS), mean HAS-BLED score was 3.2 ± 0.83 in group 1 vs 3.1 ± 0.95 in group 2 (p=NS). Persistent AF was present in 80% of patients in group 1 and in 85% in group 2. LAA closure was successful in all cases (14 Watchman, 7 ACP devices). Procedural and fluoroscopy times were shorter in group 2 (68 ± 17 vs 52 ± 15 min, p <0.05; 23 ± 5 vs 18 ± 3 min, p <0.05, respectively). No procedural complications were observed in group 2, while in group 1 one case of self-terminating pericardial effusion and one arteriovenous fistula were observed. At a mean follow-up of 14.93 ± 10.05 months, complete seal of LAA was documented in all patients, with neither dislocations nor thromboembolic events. Similarly, no long-term complications were observed in group 2. Maintenance of sinus rhythm was overlapping, with an AF relapse rate of 36% in group 1 vs 38% in group 2 (p=NS). CONCLUSIONS: Combined LAA percutaneous closure and AF ablation appears to be feasible in high-risk patients.

Apical hypertrophic cardiomyopathy: Present status.

Since the first description of apical hypertrophic cardiomyopathy in Japan 40years ago, contrasting information from all over the world has emerged regarding the natural history of the disease. This review provides an overview of incidence, phenotypic expressions, clinical features, prognosis, and management of this heterogeneous clinical entity, which may play a more relevant role in the burden of sudden cardiac death than previously thought.

PFO: Button me up, but wait Comprehensive evaluation of the patient.

Patent foramen ovale (PFO) is a slit or tunnel-like communication in the atrial septum occurring in approximately 25% of the population. A wide number of pathological conditions have been linked to its presence, most notably, cryptogenic stroke (CS) and migraine. However, in the setting of a neurological event, it is not often clear whether the PFO is pathogenically related to the index event or an incidental finding. Therefore, a detailed analysis of several clues is needed for understanding PFO's clinical significance, with a frequent case-by-case decision about destination therapy. Indeed, the controversy about PFO's pathogenicity prompted a paradigm shift of research interest from medical therapy with antiplatelets or anticoagulants to percutaneous transcatheter closure, in secondary prevention. Observational data and meta-analysis of observational studies had previously suggested that PFO closure with a device was a safe procedure with a low recurrence rate of stroke. To date, however, recent randomized controlled trials have not shown the superiority of PFO closure over medical therapy. Thus, the optimal strategy for secondary prevention of paradoxical embolism in patients with a PFO remains unclear. Moreover, the latest guidelines for the prevention on stroke restricted indications for PFO closure to patients with deep vein thrombosis and high-risk of its recurrence. Given these recent data, in the present review, we critically discuss current treatment options, pointing out the role of a comprehensive patient evaluation in overcoming PFO closure restrictions and planning the best management for each patient.

Coronary Sinus Reducer system™: A new therapeutic option in refractory angina patients unsuitable for revascularization.

A challenge of modern cardiovascular medicine is to find new, effective treatments for patients with refractory angina pectoris (RAP), a clinical condition characterized by severe angina despite optimal medical therapy and "no option" for a surgical or percutaneous revascularization. Although the relevant advance of both pharmaceutical and interventional treatments for patients affected by symptomatic coronary artery disease has greatly contributed to prolong survival, the increasing number of patients experimenting persistent and invalidating angina symptoms, highlights that quality of life of these patients has not been equally improved. Clinical limitations of the efficiency of conventional and relatively new approaches justify the search for new therapeutic options. In this review, we will focus on the epidemiology of RAP, and we will provide a brief update on the different options actually available to these patients with particular interest to an innovative device that narrow the coronary sinus: the Reducer system (Neovasc Inc., Richmond B.C., Canada). The efforts of present and future clinical studies will ultimately answer the question of whether this intriguing therapy is a suitable strategy for treatment of patients with RAP.

Usefulness of atrial function for risk stratification in asymptomatic severe aortic stenosis.

AIM: We aimed to evaluate the usefulness of left atrial (LA) mechanics and stiffness over global left ventricular (LV) longitudinal strain (GLS) for risk stratification in severe aortic stenosis (AS). METHODS: From a cohort of 89 prospective asymptomatic patients with severe AS and normal LV ejection fraction, 82 (32 men, mean age 73±10 years) truly asymptomatic patients, scheduled after a negative exercise echocardiogram, were enrolled. Forty age- and gender-matched prospective, asymptomatic subjects served as controls. Predefined end points were the occurrence of symptoms (dyspnea, angina, syncope), and death during follow-up. RESULTS: At study entry, patients had: impaired LV GLS (p=0.001), reduced LA reservoir (p<0.001), high LA stiffness (p<0.001), and increased valvulo-arterial impedance (p<0.001) compared to controls. During follow-up [16±14.9 months (ranging from 1 month to 4.2 years)], 53 patients (64.6%) reached one of the endpoints. Patients with events showed lower LV GLS (p>0.001), lower LA reservoir (p<0.001), and greater LA stiffness (p<0.001) than those asymptomatic. On univariate Cox regression analysis, LV GLS (p<0.001), LA reservoir (p<0.001), and LA stiffness (p=0.004) were strong predictors of adverse events. Kaplan-Meier curves showed that event-free survival was significantly higher in patients with a LV GLS ≥16.8% [p<0.001; area under the curve (AUC)=0.922; sensitivity=86%, specificity=80%], a LA reservoir ≥19.8% (p=0.001; AUC=0.860, sensitivity=71%, specificity=84%), and a LA stiffness <0.78 (p<0.001; AUC 0.819, sensitivity 70%, specificity 89%). On multivariate analysis, only LV GLS remained significantly associated with patients' prognosis (hazard ratio=1.49, 95% CI=1.11-2.01, p=0.008). CONCLUSIONS: In asymptomatic patients with severe AS, an efficient cardiovascular system is based on an effective atrial-ventricular interplay. LA function assessment is useful for early identification of risk in these patients. LV GLS however was confirmed to be the best predictor of patients' outcome.

An unusual case of cardiogenic shock late following surgical aortic valve replacement.

The present case describes a rare but potentially life-threatening complication following surgical aortic valve replacement (AVR): iatrogenic coronary ostial stenosis (ICOS). The incidence of ICOS is estimated to be between 0.3% and 5% of all AVR and it generally occurs within 6 months after the procedure. In most cases, either the left main stem or the ostium of the right coronary artery (RCA) is affected, although stenosis of the ostium of the left anterior descending artery can also occur. However, to the best of our knowledge, the simultaneous involvement of both coronary ostia, presenting with rapid onset cardiogenic shock requiring inotropic support and intra-aortic balloon pump placement, has never been reported. .

Usefulness of Left Atrial Reservoir Size and Left Ventricular Untwisting Rate for Predicting Outcome in Primary Mitral Regurgitation.

The present study proposed to evaluate whether analysis of cardiac mechanics through speckle-tracking imaging is useful for risk stratification in asymptomatic patients with chronic primary mitral regurgitation (MR). We prospectively enrolled 67 patients (mean age 57 ± 18 years) and followed them over time. MR was mild in 20 patients (30%), moderate in 24 (36%), and severe in 23 (34%). After a mean time of 24.8 ± 17 months, 34 patients (51%) remained asymptomatic, whereas 33 (49%) developed events (19 underwent mitral valve surgery, 9 required hospitalization for acute heart failure, and 5 patients died). Compared to asymptomatic patients, those with events at baseline showed more severe MR, larger and spherical ventricles, diastolic dysfunction, and greater systolic pulmonary arterial pressure. Moreover, patients with events had decreased left atrial (LA) reservoir (p <0.001) and left ventricular (LV) untwisting rate (p <0.001). On univariate Cox regression analysis, effective regurgitant orifice area (p <0.001), vena contracta (p <0.001), systolic pulmonary arterial pressure (p = 0.003), LV end-systolic diameter (p <0.001), E/E' ratio (p = 0.004), LA volume (p = 0.001), LA reservoir (p <0.001), and LV untwisting rate (p <0.001) were associated with an increased risk of events. On multivariate analysis, only LA reservoir (p = 0.013) and LV untwisting rate were independent predictors (p = 0.017) of outcome. Moreover, LA reservoir evaluation significantly improved (p = 0.013) risk stratification compared to recommended parameters. In conclusion, impaired cardiac mechanics is more closely associated than severity of MR with the occurrence of events in asymptomatic chronic primary MR.

Longitudinal Strain by Automated Function Imaging Detects Single-Vessel Coronary Artery Disease in Patients Undergoing Dipyridamole Stress Echocardiography.

BACKGROUND: The aim of this study was to investigate the incremental value of global longitudinal strain (GLS) by automated function imaging in respect to wall motion (WM) for the detection of coronary artery disease (CAD) during dipyridamole stress echocardiography. METHODS: Fifty-two patients (mean age, 65.3 ± 8.7 years; 22 men) underwent dipyridamole stress echocardiography followed by coronary angiography within 1 week. Diagnostic accuracy for the identification of single-vessel CAD was evaluated for WM and GLS. The study population was divided into two groups according to coronary angiographic findings: those with CAD (n = 38; mean age, 67.2 ± 5.9 years; 19 men) and those without CAD (n = 14; mean age, 63.3 ± 6.4 years; three men). RESULTS: A trend toward lower resting GLS values was found in patients with CAD than in those without (-18.7 ± 2.2% vs -20 ± 2.8%, P = .061). In patients without CAD, GLS progressively increased up to peak dose (from -20 ± 2.8% at rest to -20.7 ± 1.9% at low dose, P = .045; from -20.7 ± 1.9% at low dose to -21.5 ± 3.1% at peak dose, P = .032), whereas in patients with CAD, an increase of GLS from rest to low dose (from -18.7 ± 2.2% to -19.2 ± 3.9%, P = .046) followed by a decrease from low to peak dose (from -19.2 ± 3.9% to -17.5 ± 2.4%, P = .007) was observed. In addition, with regard to diagnostic accuracy in detecting CAD, WM yielded sensitivity of 44%, specificity of 55%, positive predictive value of 73%, and negative predictive value of 26%, whereas GLS, alternatively evaluated as the difference between peak dose and resting values or between peak and low-dose values, provided sensitivity of 61%, specificity of 90%, positive predictive value of 94%, and negative predictive value of 47% (respectively, P = .020, P = .001, P = .023, and P = .031, all vs WM) and sensitivity of 84%, specificity of 92%, positive predictive value of 96%, and negative predictive value of 68% (respectively, P < .001, P < .001, P = .001, P < .001, all vs WM). CONCLUSIONS: GLS analysis, particularly performed by comparing peak-dose with low-dose values, improves the accuracy of dipyridamole stress echocardiography in the detection of single-vessel CAD compared with the sole assessment of WM changes.

Beyond Thrombus Detection: The Role of Multimodality Imaging Approach.

We present a very rare case of paraneoplastic syndrome characterized by the unusual coexistence of a left ventricular apical thrombus and pulmonary embolism as the first manifestation of an unrecognized lung adenocarcinoma.

New diagnostic perspectives on heart failure with preserved ejection fraction: systolic function beyond ejection fraction.

Although preserved ejection fraction is found in more than 50% of patients with heart failure, its acceptance as a specific clinical entity is limited. More understanding of the physiopathology, early diagnosis and medical management is needed. With no existing systematic information in the literature, the aim of this review is to provide a comprehensive overview of the new imaging techniques for diagnosing heart failure with preserved ejection fraction, particularly in the early stages of the disease, underlying the pivotal role of new technologies such as two-dimensional speckle tracking echocardiography and vascular stiffness.

Interplay between arterial stiffness and diastolic function: a marker of ventricular-vascular coupling.

AIMS: We evaluated the interplay between left ventricular diastolic function and large-artery stiffness in asymptomatic patients at increased risk of heart failure and no structural heart disease (Stage A). METHODS: We divided 127 consecutive patients (mean age 49 ±â€Š17 years) with risk factors for heart failure who were referred to our laboratory to rule out structural heart disease into two groups according to presence (Group 1, n = 35) or absence (Group 2, n = 92) of grade I left ventricular diastolic dysfunction. Doppler imaging with high-resolution echo-tracking software was used to measure intima-media thickness (IMT) and stiffness of carotid arteries. RESULTS: Group 1 had significantly higher mean age, blood pressure, left ventricular mass index, carotid IMT and arterial stiffness than Group 2 (P < 0.05). Overall, carotid stiffness indices (ß-stiffness index, augmentation index and elastic modulus) and 'one-point' pulse wave velocity each showed inverse correlation with E-wave velocity, E' velocity and E/A ratio, and direct correlation with A-wave velocity, E-wave deceleration time and E/E' ratio (P < 0.05). Arterial compliance showed negative correlations with the echocardiographic indices of left ventricular diastolic function (P < 0.05). On logistic regression analysis, age, hypertension, SBP, pulse pressure, left ventricular mass index, carotid IMT and stiffness parameters were associated with grade I left ventricular diastolic dysfunction (P < 0.05 for each). However, on multivariate logistic analysis, only 'one-point' pulse wave velocity and age were independent predictors (P = 0.038 and P = 0.016, respectively). CONCLUSION: An independent association between grade I left ventricular diastolic dysfunction and increased arterial stiffness is demonstrated at the earliest stage of heart failure. Hence, assessment of vascular function, beyond cardiac function, should be included in a comprehensive clinical evaluation of these patients.

The practical role of echocardiography in selection, implantation, and management of patients requiring LVAD therapy.

Viable treatment options for advanced heart failure have not emerged as the number of people afflicted with this condition has grown. Although heart transplantation is the only curative strategy for patients with end-stage heart failure, the relative shortage of donors has led to a worldwide plateau of this option over the past 20 years. The result is an unacceptably high mortality rate among patients with advanced heart failure. Interest in developing alternative curative strategies based on chronic circulatory support, with the aim of prolonging and improving quality of life for these patients, has grown. Patients supported with left ventricular assist devices require structured longitudinal care from a team of providers. An integrated approach using basic echocardiography is critical to patient selection, implantation, and continued surveillance and success of patients with left ventricular assist devices.