Early left atrial dysfunction in idiopathic pulmonary fibrosis patients without chronic right heart failure

No data are actually available regarding the left atrial (LA) functional assessment by two-dimensional speckle tracking echocardiography (2D-STE) in early-stage idiopathic pulmonary fibrosis (IPF). The primary end-point of our study was to assess whether global LA peak strain (GLAPS), measured by 2D-STE analysis, may detect early alterations in LA function in IPF patients without right heart failure (RHF). Between September 2017 and January 2019, 50 consecutive IPF patients (73.8 \ub1 6.8 years, 36 males) without chronic RHF and 30 controls matched by age, sex and cardiovascular risk factors, were enrolled in an observational retrospective case\u2013control study. All patients underwent a complete echocardiographic study implemented with 2D-STE analysis. GLAPS, left ventricular (LV) global longitudinal strain (GLS), right atrial (RA) reservoir strain (GSA+) and right ventricular (RV)-GLS were obtained in each patient. LVFP were significantly increased in IPF patients in comparison to controls (average E/e\u2032 ratio 14.4 \ub1 3.0 vs 9.6 \ub1 1.5, p < 0.0001), while LV-GLS was slightly reduced in IPF patients compared to controls (19.4 \ub1 3.6% vs 21.0 \ub1 2.2%, p = 0.03).Moreover, GLAPS was significantly impaired in IPF patients in comparison to controls (18.4 \ub1 3.7% vs 28.4 \ub1 5.6%, p < 0.0001).Finally, the two groups of patients did not show any statistically significant difference in both RA-GSA + (23.9 \ub1 3.7% vs 24.5 \ub1 4.0%, p = 0.49) and RV-GLS ( 12 22.6 \ub1 3.3% vs 12 23.5 \ub1 3.0%, p = 0.22). Notably, LV-GLS was strongly inversely correlated both with RV/LV basal diameter ratio and TRV in IPF patients (r = 12 0.87 and 12 0.82, respectively) but not in controls (r = 12 0.29 and 12 0.27, respectively). This finding highlights a likely process of ventricular interdependence in non-advanced IPF, with consequent LV diastolic dysfunction and secondary impairment in LV-GLS and GLAPS. Early LA reservoir dysfunction in IPF patients may be secondary to LV diastolic dysfunction induced by ventricular interdependence and may develop before RV diastolic and systolic dysfunction

Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis: The Role of Exercise Stress Echocardiography

Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography. Methods: Fifteen patients with LAM (mean age, 47 \ub1 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 \ub1 8 years; P =.65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance. Results: Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 \ub1 6.5 vs 4.2 \ub1 3.1 mm Hg, P <.0001), pulmonary vascular resistance (+68.3 \ub1 42.1 vs 120.1 \ub1 18.3 dyne-sec/cm5, P <.0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 \ub1 5.3 vs 120.5 \ub1 1.3 mm Hg, P <.0001). Conclusions: Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH)

Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management

The presence of rare comorbidities in patients with cardiovascular disease (CVD) presents a diagnostic challenge to cardiologists. In evaluating these patients, cardiologists are faced with a unique opportunity to shorten diagnosis times and direct patients towards correct treatment pathways. Idiopathic pulmonary fibrosis (IPF), a type of interstitial lung disease (ILD), is an example of a rare disease where patients frequently demonstrate comorbid CVD. Both CVD and IPF most commonly affect a similar patient demographic: men over the age of 60 years with a history of smoking. Moreover, IPF and heart failure (HF) share a number of symptoms. As a result, patients with IPF can be misdiagnosed with HF and vice versa. This article aims to increase awareness of IPF among cardiologists, providing an overview for cardiologists on the differential diagnosis of IPF from HF, and describing the signs and symptoms that would warrant referral to a pulmonologist with expertise in ILD. Once patients with IPF have received a diagnosis, cardiologists can have an important role in managing patients who are candidates for a lung transplant or those who develop pulmonary hypertension (PH). Group 3 PH is one of the most common cardiovascular complications diagnosed in patients with IPF, its prevalence varying between reports but most often cited as between 30% and 50%. This review summarizes the current knowledge on Group 3 PH in IPF, discusses data from clinical trials assessing treatments for Group 1 PH in patients with IPF, and highlights that treatment guidelines recommend against these therapies in IPF. Finally, this article provides the cardiologist with an overview on the use of the two approved treatments for IPF, the antifibrotics pirfenidone and nintedanib, in patients with IPF and CVD comorbidities. Conversely, the impact of treatments for CVD comorbidities on patients with IPF is also discussed.Funding: F. Hoffmann-La Roche, Ltd.Plain Language Summary: Plain language summary available for this article.status: publishe

Correlation Between Doppler Echocardiography and Right Heart Catheterisation-Derived Systolic and Mean Pulmonary Artery Pressures: Determinants of Discrepancies Between the Two Methods

Background: There is still controversy about whether transthoracic echocardiography (TTE) can provide reliable estimations of pulmonary artery pressures (PAP). The primary endpoint of this study was to evaluate the correlation between TTE and right heart catheterisation (RHC) in estimating systolic (SPAP) and mean (MPAP) pulmonary artery pressures. Methods: Between January 2011 and December 2018, 141 consecutive patients (average age 63.6±11.5 years; 84 women) with suspected or confirmed pulmonary hypertension (PH) were enrolled into this retrospective observational monocentric study. All patients underwent TTE and, within 3 hours, RHC. The correlation between TTE and RHC in estimating both SPAP and MPAP was retrospectively determined. Results: Seventeen (17) of the patients were excluded due to insufficient TTE signal quality. Of the remaining 124 patients, 18 had no PH. There was moderate correlation between both SPAP and MPAP estimated by TTE and those assessed by RHC (r=0.65 and r=0.60, respectively). Bland-Altman analysis revealed a bias of –11.9 mmHg (with the 95% limits of agreement ranging –45.4 to +21.5 mmHg) for SPAP estimation and –4.6 mmHg (with the 95% limits of agreement ranging –27.9 to +18.8 mmHg) for MPAP estimation, suggesting a general overestimation of PAP by TTE. The main factors responsible for discrepancies between TTE and RHC were: female gender, arrhythmic cardiac electrical activity, systemic arterial hypertension, and diuretic treatment. Conclusions: Transthoracic echocardiography frequently overestimated PAP in comparison with RHC, especially in hypertensive women with arrhythmias and under diuretic treatment

Reduced Myocardial Strain Parameters in Subjects With Pectus Excavatum: Impaired Myocardial Function or Methodological Limitations Due to Chest Deformity?

Pectus excavatum (PE) may cause symptoms and alter cardiopulmonary function. Left ventricular (LV) and right ventricular (RV) function have been reported to be impaired in PE subjects. However, this issue has not been systematically investigated with respect to the degree of chest wall abnormality. We aimed to evaluate the influence of severity of chest shape abnormality on myocardial strain parameters in PE subjects. We studied 30 healthy subjects (55.8 \ub1 14.0 year/old, 18 males) with PE, assessed by the ratio of chest transverse diameter over the distance between sternum and spine (modified Haller index, MHI, >2.5), and 30 controls (MHI 642.5) matched by age, sex, and cardiovascular risk factors. Participants underwent 2-dimensional (2D) transthoracic echocardiography implemented with 2D-speckle tracking echocardiography. Right-heart and left-heart chamber dimensions, and stroke volume, were significantly reduced in PE subjects (all P< 0.0001). While LV ejection fraction, E/A, and E/e\u2019, did not significantly differ between the 2 groups, all LV and RV strain and strain rate parameters were severely reduced in subjects with PE (P < 0.0001). Importantly, in PE subjects, but not in controls, LV global longitudinal strain, LV global circumferential strain, LV global radial strain, and RV free wall systolic strain, were all linearly correlated to MHI (all P < 0.0001). In healthy subjects with PE, abnormal chest anatomy progressively impairs myocardial strain. However, this impairment is not due to subclinical myocardial dysfunction; it might reflect intraventricular dyssynchrony due to compressive phenomena, or technical limitations of strain methodology, due to chest wall abnormality

Incremental prognostic value of global left atrial peak strain in women with new-onset gestational hypertension

Background:Left atrial strain and strain rate parameters, measured by bidimensional-speckle tracking echocardiography, have been proposed as predictors of atrial fibrillation, stroke, congestive heart failure and cardiovascular death. However, they have not yet been tested in hypertensive disorders of pregnancy. The aim of this study was to assess the prognostic role of global left atrial peak strain (GLAPS) in a population of pregnant women with new-onset hypertension in a medium-term follow-up.Methods:Twenty-seven consecutive women with new-onset hypertension after 20 weeks pregnancy and 23 age-matched, race-matched and gestational week-matched consecutive normotensive pregnant women were enrolled in this prospective study. All participants underwent a complete echocardiographic study with bidimensional-speckle tracking echocardiography and carotid examination. At 1-year follow-up, we evaluated the occurrence of persistent hypertension.Results:In comparison with normotensive women, those hypertensive had a higher burden of cardiovascular risk factors, similar left atrial volume indexed (P = 0.14), but severely impaired left atrial strain (P < 0.0001) and strain rate values (P < 0.0001). At 1-year follow-up, persistent hypertension was documented in 59.3% of patients. At the univariate Cox analysis, the variables associated with the occurrence of the investigated outcome in all hypertensive pregnancies were SBP (hazard ratio 1.04, P = 0.04), DBP (hazard ratio 1.11, P = 0.01), mean arterial pressure (hazard ratio 1.09, P = 0.01) values and the GLAPS value (hazard ratio 0.85, P = 0.0019). The latter was significantly associated with the investigated outcome both in preeclamptic (hazard ratio 0.84, P = 0.02) and nonpreeclamptic pregnant women (hazard ratio 0.83, P = 0.04). The receiver operating characteristics curve analysis highlighted that a GLAPS value of 23.5% or less predicted persistent hypertension with sensitivity of 100% and specificity of 90.90%.Conclusion:In hypertensive pregnant women a GLAPS value of 23.5% or less reveals a greater severity of atrial cardiomyopathy and might predict postpregnancy persistent hypertension