Usefulness of cardiopulmonary exercise test combined with exercise stress echocardiography in mild chronic thromboembolic pulmonary disease.

PURPOSE: Chronic thromboembolic pulmonary disease (CTEPD) can lead to exercise limitations even without right ventricular (RV) dysfunction or pulmonary hypertension at rest. Combining exercise stress echocardiography with cardiopulmonary exercise testing (ESE-CPET) for RV function and pressure changes combined measuring overall function may be useful for CTEPD evaluation. This study aims to investigate CPET and ESE results to elucidate the mechanisms of exercise limitation in mild CTEPD cases. METHODS: Among our CTEPD registry, 50 patients who performed both right heart catheterization data of mild disease (less than 30 mm Hg of mean pulmonary arterial pressure (mPAP)) and ESE-CPET were enrolled. Echocardiography and CPET-derived parameters were compared with hemodynamic parameters measured through right heart catheterization. RESULTS: Peak VO2 (maximal oxygen consumption) was decreased in overall population (71.3 ± 16.3% of predictive value). Peak VO2 during exercise was negatively correlate with mPAP and pulmonary vascular resistance at rest. A substantial increase in RV systolic pressure (RVSP) was observed during exercise (RVSP: pre-exercise 37.2 ± 11.8 mm Hg, postexercise 64.3 ± 24.9 mm Hg, p-value < .001). Furthermore, RV function deteriorated during exercise when compared to the baseline (RV fractional area change: 31.5 ± 10.0% to 37.8 ± 7.0%, p-value < .001; RV global longitudinal strain: -17.1 ± 4.2% to -17.7 ± 3.3%, p-value < .001) even though basal RV function was normal. While an excessive increase in RVSP during exercise was noticed in both groups, dilated RV and RV dysfunction during exercise were demonstrated only in the impaired exercise capacity group. CONCLUSION: CTEPD patients with mild PH or without PH exhibited limited exercise capacity alongside an excessive increase in RVSP during exercise. Importantly, RV dysfunction during exercise was significantly associated with exercise capacity. ESE-CPET could aid in comprehending the primary cause of exercise limitation in these patients.

Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Anaesthetic management of a patient with severe pulmonary hypertension, moderate tricuspid regurgitation and moderate right ventricular dysfunction presenting for a dilation and curettage.

A primigravida woman in her 30s with severe pulmonary hypertension, tricuspid regurgitation and depressed right ventricular function presented for therapeutic termination of pregnancy at 7 weeks gestation after referral and multidisciplinary recommendation. A slowly titrated lumbar epidural was chosen for the primary anaesthetic. Under standard American Society of Anesthesiologists (ASA) monitoring, invasive blood pressure monitoring and continuous transthoracic echocardiography, the patient's right ventricular systolic function was monitored throughout the case using qualitative and quantitative metrics and was notable for a right ventricular systolic pressure of 102.4 mm Hg. Milrinone, started prior to epidural titration, was used for inotropic support. Vasopressin was used in conjunction to sustain systemic vascular resistance while having little effect on the pulmonary vascular resistance. The patient experienced no complications or exacerbation of her pulmonary hypertension and was discharged home the following day.

Prognostic Role of Tricuspid Annular Plane Systolic Excursion to Systolic Pulmonary Artery Pressure Ratio for the Identification of Early Clinical Deterioration in Intermediate-High-Risk Pulmonary Embolism Patients.

The ratio of tricuspid annular plane systolic excursion (TAPSE) to echocardiographically measured systolic pulmonary artery pressure (PASP) has been proposed as a surrogate of RV-arterial coupling. In this analysis, we assess the prognostic role of TAPSE/PASP for early clinical deterioration and short-term mortality in an often clinically challenging population of intermediate-high-risk patients with pulmonary embolism (PE). A post hoc analysis of intermediate-high-risk patients with PE enrolled in the Italian Pulmonary Embolism Registry (ClinicalTrials.gov: NCT01604538) was performed. All patients underwent transthoracic echocardiography at admission. The primary and secondary outcomes were clinical deterioration within 48 hours from admission and 30-day all-cause mortality, respectively. In 422 intermediate-high-risk patients with PE (mean age 71.2 ± 5.3 years, 238 men), 37 (8.7%) experienced clinical deterioration within 48 hours of admission. The 30-day mortality rate was 6.6% (n = 28). The receiver operating characteristic analysis established 0.33 as the optimal cut-off value for the TAPSE/PASP in predicting 48-hour clinical deterioration (area under the curve 0.79 ± 0.1). The sensitivity, specificity, positive predictive value, and negative predictive value were 81%, 88.5%, 40.5%, and 97.9%, respectively. The multivariate Cox regression analysis showed that a TAPSE/PASP ≤0.33 was an independent predictor of 48-hour clinical deterioration (hazard ratio 2.06, 95% confidence interval 1.98 to 2.11, p <0.0001) and 30-day mortality (hazard ratio 2.28, 95% confidence interval 2.25 to 2.33, p <0.001). TAPSE/PASP shows promise as a noninvasive prognostic predictor to identify intermediate-high-risk patients with PE at a higher risk of early clinical deterioration and short-term mortality.

Right ventricular free wall and four-chamber longitudinal strain in relation to incident heart failure in the general population.

AIMS: Right ventricular free wall (RVFWLS) and four-chamber longitudinal strain (RV4CLS) are associated with adverse events in various patient populations including patients with heart failure (HF). We sought to investigate the prognostic value of RVFWLS and RV4CLS for the development of incident HF in participants from the general population. METHODS AND RESULTS: Participants from the 5th Copenhagen City Heart Study (2011-2015) without known chronic ischaemic heart disease or HF at baseline were included. RVFWLS and RV4CLS were obtained using two-dimensional speckle-tracking echocardiography from the right ventricular (RV)-focused apical four-chamber view. The primary endpoint was incident HF. Among 2740 participants (mean age 54 ± 17 years, 42% male), 43 (1.6%) developed HF during a median follow-up of 5.5 years (IQR 4.5-6.3). Both RVFWLS and RV4CLS were associated with an increased risk of incident HF during follow-up independent of age, sex, hypertension, diabetes, body mass index and tricuspid annular plane systolic excursion (TAPSE), (HR 1.06, 95%CI 1.00-1.11, P = 0.034, per 1% absolute decrease and HR 1.14, 95%CI 1.05-1.23, P = 0.001, per 1% absolute decrease, respectively). Left ventricular ejection fraction (LVEF) modified the association between RV4CLS and incident HF (P for interaction = 0.016) such that RV4CLS was only of prognostic importance among those with LVEF < 55% (HR 1.21, 95%CI 1.11-1.33, P < 0.001 vs. HR 0.94, 95%CI 0.80-1.10, P = 0.43 in patients with LVEF ≥ 55%). CONCLUSION: In participants from the general population, both RVFWLS and RV4CLS were associated with a greater risk of incident HF independent of important baseline characteristics and TAPSE, and LVEF modified the relationship between RV4CLS and incident HF.

Prevalence of Cardiac Sarcoidosis in Middle-Aged Adults Diagnosed with High-Grade Atrioventricular Block.

INTRODUCTION: Atrioventricular block may be idiopathic or a secondary manifestation of an underlying systemic disease. Cardiac sarcoidosis is a significant underlying cause of high-grade atrioventricular block, posing diagnostic challenges and significant clinical implications. This study aimed to assess the prevalence and clinical characteristics of cardiac sarcoidosis among younger patients presenting with unexplained high-grade atrioventricular block. METHODS: We evaluated patients aged between 18 and 65 years presenting with unexplained high-grade atrioventricular block, who were systematically referred for cardiac magnetic resonance imaging, positron emission tomography-computed tomography, or both, prior to pacemaker implantation. Subjects with suspected cardiac sarcoidosis based on imaging findings were further referred for tissue biopsy. Cardiac sarcoidosis diagnosis was confirmed based on biopsy results. RESULTS: Overall, 30 patients with high-grade atrioventricular block were included in the analysis. The median age was 56.5 years (interquartile range 53-61.75, years). In 37%, cardiac magnetic resonance imaging, positron emission tomography-computed tomography, or both, were suggestive of cardiac sarcoidosis, and in 33% cardiac sarcoidosis was confirmed by tissue biopsy. Compared with idiopathic high-grade atrioventricular block patients, all cardiac sarcoidosis patients were males (100% vs 60%, P = .029), were more likely to present with heart failure symptoms (50% vs 10%, P = .047), had thicker inter-ventricular septum on echocardiography (12.2 ± 2.7 mm vs 9.45 ± 1.6 mm, P = .002), and were more likely to present with right ventricular dysfunction (33% vs 10%, P = .047). CONCLUSIONS: Cardiac sarcoidosis was confirmed in one-third of patients ≤ 65 years, who presented with unexplained high-grade atrioventricular block. Cardiac sarcoidosis should be highly suspected in such patients, particularly in males who present with heart failure symptoms or exhibit thicker inter-ventricular septum and right ventricular dysfunction on echocardiography.

First experience with real-time magnetic resonance imaging-based investigation of respiratory influence on cardiac function in pediatric congenital heart disease patients with chronic right ventricular volume overload.

BACKGROUND: Congenital heart disease (CHD) is often associated with chronic right ventricular (RV) volume overload. Real-time magnetic resonance imaging (MRI) enables the analysis of cardiac function during free breathing. OBJECTIVE: To evaluate the influence of respiration in pediatric patients with CHD and chronic RV volume overload. METHODS AND MATERIALS: RV volume overload patients (n=6) and controls (n=6) were recruited for cardiac real-time MRI at 1.5 tesla during free breathing. Breathing curves from regions of interest reflecting the position of the diaphragm served for binning images in four different tidal volume classes, each in inspiration and expiration. Tidal volumes were estimated from these curves by data previously obtained by magnetic resonance-compatible spirometry. Ventricular volumes indexed to body surface area and Frank-Starling relationships referenced to the typical tidal volume indexed to body height (TTVi) were compared. RESULTS: Indexed RV end-diastolic volume (RV-EDVi) and indexed RV stroke volume (RV-SVi) increased during inspiration (RV-EDVi/TTVi: RV load: + 16 ± 4%; controls: + 22 ± 13%; RV-SVi/TTVi: RV load: + 21 ± 6%; controls: + 35 ± 17%; non-significant for comparison). The increase in RV ejection fraction during inspiration was significantly lower in RV load patients (RV load: + 1.1 ± 2.2%; controls: + 6.1 ± 1.5%; P=0.01). The Frank-Starling relationship of the RV provided a significantly reduced slope estimate in RV load patients (inspiration: RV load: 0.75 ± 0.11; controls: 0.92 ± 0.02; P=0.02). CONCLUSION: In pediatric patients with CHD and chronic RV volume overload, cardiac real-time MRI during free breathing in combination with respiratory-based binning indicates an impaired Frank-Starling relationship of the RV.

[Anatomy-physiology considerations for cardiogenic shock with right ventricular involvement].

Since the discovery of right ventricular infarction, interest in the characteristics of the right ventricle has been increasing. Right ventricular function is now known to be a predictor of mortality in different settings. The right ventricle is a low-pressure, high-compliance, high-volume chamber. To carry out its normal function, it is coupled to the pulmonary circulation and the left ventricle. In the face of acute changes in pressure, volume overload and ischemia, it dilates to adapt to its new load. Its manifestation may be ventricular dysfunction and/or failure that will progress to cardiogenic shock due to right ventricular involvement. Various entities may be the cause of acute dysfunction: right ventricular infarction (alterations in contractility due to ischemia) and high-risk pulmonary thromboembolism (increased afterload). Both share a similar ventricular pathophysiology and high mortality without treatment. Understanding anatomy and physiology, dysfunction and acute ventricular failure are important to define a convenient diagnosis and treatment oriented towards pathophysiology. In this first part, the anatomy and physiology, acute right ventricular dysfunction/failure and cardiogenic shock are taken into consideration, from the perspective of these two entities. In another paper, treatment aimed at cardiogenic shock due to right ventricular involvement will be reviewed.

Desde el conocimiento del infarto del ventrículo derecho, el interés por las características del ventrículo derecho ha sido cada vez mayor. Ahora se sabe que la función ventricular derecha es un predictor de mortalidad en diferentes contextos. El ventrículo derecho es una cavidad de baja presión, alta compliancia y alto volumen. Para llevar a cabo su función normal se encuentra acoplado a la circulación pulmonar y al ventrículo izquierdo. Ante alteraciones agudas de sobrecarga de presión, volumen e isquemia, se dilata para adaptarse a su nueva carga. Su manifestación puede ser disfunción o falla ventricular que progresará a choque cardiogénico por involucro del ventrículo derecho. Diversas entidades pueden ser la causa de la disfunción aguda: el infarto del ventrículo derecho (alteraciones de la contractilidad por isquemia) y la tromboembolia pulmonar de alto riesgo (aumento de la poscarga). Ambas comparten una fisiopatología ventricular similar y alta mortalidad sin tratamiento. Entender la anatomía fisiológica, la disfunción y la falla ventricular aguda es importante para definir un diagnóstico oportuno y un tratamiento orientado a la fisiopatología. En esta primera parte se toma en consideración la anatomía fisiológica y la disfunción/falla aguda ventricular derecha y su desenlace en el choque cardiogénico, desde la perspectiva de estas dos entidades. En otro trabajo se revisará el tratamiento orientado al choque cardiogénico por involucro ventricular derecho.

Right ventricular systolic function and mechanical dyssynchrony in ischemic or non-ischemic dilated cardiomyopathy: A speckle-tracking study.

AIM: This study assessed RV dyssynchrony (irrespective to QRS duration) and RV systolic function in non-ischemic dilated cardiomyopathy (NIDCM) versus ischemic dilated cardiomyopathy (IDCM) patients by using different echo-Doppler modalities. METHODS: Eighty-five cases (48 patients with DCM [whether ischemic or non-ischemic] and 37 age-matched healthy controls) were studied. Conventional echo-Doppler study, tissue Doppler (TDI), and speckle tracking (STE) were carried out to measure LV and RV systolic function. Time-to-peak negative longitudinal strain at the four RV sites were assessed by TDI derived strain and 2D speckle tracking. RESULTS: Patients with DCM (whether ischemic or non-ischemic) had significantly lower fractional area change, RV tricuspid annular systolic velocity (p < .001 for both), tricuspid annular plane systolic excursion (p = .01), RV-GLS whether TDI or 2D derived (p < .001). Twenty-nine patients (60%) showed right intraventricular delay (RV4SD > 60 ms). The RV-dyssynchrony index was negatively correlated to %FAC (r = -.362, p = .01), RV Sm (r = -.312, p = .04), and 2D-RV GLS (r = -.305, p = .05). Insignificant higher RV-dysynchrony index was detected in NIDCM compared to IDCM group; however, the basal septal segment was significantly delayed in dilated group. More impaired RV systolic function was detected in ischemic group. 2D STE and TDI showed a significant correlation in the assessment of the right-intraventricular delay (p = .001). CONCLUSION: Right-intraventricular dyssynchrony are detectable in patients with dilated cardiomyopathy (whether ischemic or non-ischemic) with a higher statistically insignificant value in non-ischemic group by using tissue Doppler imaging and 2D speckle tracking. More impairment of the RV systolic function was noticed in the ischemic group. Impaired RV systolic function was associated with right intraventricular delay.

Long-Term Effects of Pulmonary Endarterectomy on Right Ventricular Stiffness and Fibrosis in Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Surgical removal of thromboembolic material by pulmonary endarterectomy (PEA) leads within months to the improvement of right ventricular (RV) function in the majority of patients with chronic thromboembolic pulmonary hypertension. However, RV mass does not always normalize. It is unknown whether incomplete reversal of RV remodeling results from extracellular matrix expansion (diffuse interstitial fibrosis) or cellular hypertrophy, and whether residual RV remodeling relates to altered diastolic function. METHODS: We prospectively included 25 patients with chronic thromboembolic pulmonary hypertension treated with PEA. Structured follow-up measurements were performed before, and 6 and 18 months after PEA. With single beat pressure-volume loop analyses, we determined RV end-systolic elastance (Ees), arterial elastance (Ea), RV-arterial coupling (Ees/Ea), and RV end-diastolic elastance (stiffness, Eed). The extracellular volume fraction of the RV free wall was measured by cardiac magnetic resonance imaging and used to separate the myocardium into cellular and matrix volume. Circulating collagen biomarkers were analyzed to determine the contribution of collagen metabolism. RESULTS: RV mass significantly decreased from 43±15 to 27±11g/m2 (-15.9 g/m2 [95% CI, -21.4 to -10.5]; P<0.0001) 6 months after PEA but did not normalize (28±9 versus 22±6 g/m2 in healthy controls [95% CI, 2.1 to 9.8]; P<0.01). On the contrary, Eed normalized after PEA. Extracellular volume fraction in the right ventricular free wall increased after PEA from 31.0±3.8 to 33.6±3.5% (3.6% [95% CI, 1.2-6.1]; P=0.013) as a result of a larger reduction in cellular volume than in matrix volume (Pinteraction=0.0013). Levels of MMP-1 (matrix metalloproteinase-1), TIMP-1 (tissue inhibitor of metalloproteinase-1), and TGF-ß (transforming growth factor-ß) were elevated at baseline and remained elevated post-PEA. CONCLUSIONS: Although cellular hypertrophy regresses and diastolic stiffness normalizes after PEA, a relative increase in extracellular volume remains. Incomplete regression of diffuse RV interstitial fibrosis after PEA is accompanied by elevated levels of circulating collagen biomarkers, suggestive of active collagen turnover.

Significance of End-Diastolic Forward Flow in Patients With Repaired Tetralogy of Fallot　- Its Interaction With the Left Ventricular Property and End Organ Damage.

BACKGROUND: Although right ventricular (RV) enlargement may affect RV diastolic dysfunction assessed by end-diastolic forward flow (EDFF) in patients with repaired tetralogy of Fallot (TOF), EDFF may also be modified by left ventricular (LV) hemodynamics. We hypothesized that EDFF is affected by LV hemodynamics, not limited to RV diastolic stiffening.Methods and Results: Among 145 consecutive patients with repaired TOF who underwent catheterization, hemodynamic properties in 47 with consistent EDFF and 75 without EDFF were analyzed. Compared with patients without EDFF, those with EDFF had a large RV volume with a high regurgitant fraction. Although cardiac index and central venous pressure (CVP) were similar, contrast injection augmented CVP and LV end-diastolic pressure (EDP) in patients with vs. those without EDFF, suggesting compromised diastolic reserve. In patients with EDFF, the velocity-time integral (VTI) of EDFF was positively correlated with LVEDP and systemic vascular resistance, in addition to RV EDP. EDFF-VTI was correlated with hepatic venous wedge pressure and markers of hepatic dysfunction. Subanalysis of the older (≥6 years) half of the study cohort revealed that EDFF was associated with bi-atrial enlargement independent of RV volume, highlighting the pronounced role of EDFF on the diastolic property in the aged cohort. CONCLUSIONS: EDFF-VTI in patients with repaired TOF reflects RV diastolic dysfunction, affected by the left heart system. EDFF-VTI indicates blood stagnation, which may be attributed to end-organ damage.

Restoration of right ventricular function in the treatment of pulmonary arterial hypertension.

OBJECTIVE: A 45% threshold of right ventricular ejection fraction (RVEF) is proposed clinically relevant in patients with pulmonary arterial hypertension (PAH). We aim to determine treatment response, long-term right ventricular (RV) functional stability and prognosis of patients with PAH reaching or maintaining the RVEF 45% threshold. METHODS: Incident, treatment-naive, adult PAH patients with cardiac magnetic resonance imaging at baseline and first follow-up were included (total N=127) and followed until date of censoring or death/lung transplantation. Patients were categorised into two groups based on 45% RVEF. Baseline predictors, treatment response and prognosis were assessed with logistic regression analyses, two-way analysis of variance and log-rank tests. RESULTS: Patients were 50±17 years old, 73% female, of which N=75 reached or maintained the 45% RVEF threshold at follow-up (RVEF≥45%@FU), while N=52 patients did not (RVEF<45%@FU). RV end-diastolic volume and N-terminal pro-B-type natriuretic peptide at baseline were multivariable predictors of an RVEF ≥45% at follow-up. A 40% pulmonary vascular resistance (PVR) reduction resulted in greater improvement in RV function (ΔRVEF 17±11 vs. 5±8; pinteraction<0.001) compared to a PVR reduction <40%, but did not guarantee an RVEF ≥45%. Finally, the 45% RVEF threshold was associated with stable RV function during long-term follow-up and better survival (HR: 1.91 (95% CI: 1.11 to 3.27)). Patients failing to reach or maintain the 45% RVEF threshold at first follow-up mostly stayed below this threshold over the next consecutive visits. CONCLUSION: After treatment initiation, 60% of patients with PAH reach or maintain the 45% RVEF threshold, which is associated with a long-term stable RV function and favourable prognosis.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Prognosis of Right Ventricular Systolic Dysfunction in Patients With Duchenne Muscular Dystrophy.

Background Chronic respiratory failure and heart involvement may occur in Duchenne muscular dystrophy. We aimed to assess the prognostic value of the right ventricular (RV) systolic dysfunction in patients with Duchenne muscular dystrophy. Methods and Results We studied 90 genetically proven patients with Duchenne muscular dystrophy from 2010 to 2019, to obtain respiratory function and Doppler echocardiographic RV systolic function. Prognostic value was assessed in terms of death and cardiac events. The median age was 27.5 years, and median forced vital capacity was at 10% of the predicted value: 83 patients (92%) were on home mechanical ventilation. An RV systolic dysfunction was found in 46 patients (51%). In patients without RV dysfunction at inclusion, a left ventricular systolic dysfunction at inclusion was associated with a higher risk of developing RV dysfunction during follow-up with an odds ratio of 4.5 (P=0.03). RV systolic dysfunction was significantly associated with cardiac events, mainly acute heart failure (62%) and cardiogenic shock (23%). In a multivariable Cox model, the adjusted hazard ratio was 4.96 (95% CI [1.09-22.6]; P=0.04). In terms of death, we found a significant difference between patients with RV dysfunction versus patients without RV dysfunction in the Kaplan-Meier curves (log-rank P=0.045). Conclusions RV systolic dysfunction is frequently present in patients with Duchenne muscular dystrophy and is associated with increased risk of cardiac events, irrespective of left ventricular dysfunction and mechanical ventilation. Registration URL: https://www.clinicaltrials.org; unique identifier: NCT02501083.

What echocardiographic findings differentiate acute pulmonary embolism and chronic pulmonary hypertension?

BACKGROUND: Pulmonary embolism (PE) and pulmonary hypertension (PH) are potentially fatal disease states. Early diagnosis and goal-directed management improve outcomes and survival. Both conditions share several echocardiographic findings of right ventricular dysfunction. This can inadvertently lead to incorrect diagnosis, inappropriate and potentially harmful management, and delay in time-sensitive therapies. Fortunately, bedside echocardiography imparts a few critical distinctions. OBJECTIVE: This narrative review describes eight physiologically interdependent echocardiographic parameters that help distinguish acute PE and chronic PH. The manuscript details each finding along with associated pathophysiology and summarization of the literature evaluating diagnostic utility. This guide then provides pearls and pitfalls with high-quality media for the bedside evaluation. DISCUSSION: The echocardiographic parameters suggesting acute or chronic right ventricular dysfunction (best used in combination) are: 1. Right heart thrombus (acute PE) 2. Right ventricular free wall thickness (acute ≤ 5 mm, chronic > 5 mm) 3. Tricuspid regurgitation pressure gradient (acute ≤ 46 mmHg, chronic > 46 mmHg, corresponding to tricuspid regurgitation maximal velocity ≤ 3.4 m/sec and > 3.4 m/sec, respectively) 4. Pulmonary artery acceleration time (acute ≤ 60-80 msec, chronic < 105 msec) 5. 60/60 sign (acute) 6. Pulmonary artery early-systolic notching (proximally-located, higher-risk PE) 7. McConnell's sign (acute) 8. Right atrial enlargement (equal to left atrial size suggests acute, greater than left atrial size suggests chronic). CONCLUSIONS: Emergency physicians must appreciate the echocardiographic findings and associated pathophysiology that help distinguish acute and chronic right ventricular dysfunction. In the proper clinical context, these findings can point towards PE or PH, thereby leading to earlier goal-directed management.

Quantitative Measures of Right Ventricular Size and Function by Echocardiogram Correlate with Cardiac Catheterization Hemodynamics in Congenital Diaphragmatic Hernia.

OBJECTIVE: To evaluate associations between cardiac catheterization (cath) hemodynamics, quantitative measures of right ventricular (RV) function by echocardiogram, and survival in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This single-center retrospective cohort study enrolled patients with CDH who underwent index cath from 2003 to 2022. Tricuspid annular plane systolic excursion z score, RV fractional area change, RV free wall and global longitudinal strain, left ventricular (LV) eccentricity index, RV/LV ratio, and pulmonary artery acceleration time were measured from preprocedure echocardiograms. Associations between hemodynamic values, echocardiographic measures, and survival were evaluated by Spearman correlation and Wilcoxon rank sum test, respectively. RESULTS: Fifty-three patients (68% left-sided, 74% liver herniation, 57% extracorporeal membrane oxygenation, 93% survival) underwent cath (39 during index hospitalization, 14 later) including device closure of a patent ductus arteriosus in 5. Most patients (n = 31, 58%) were on pulmonary hypertension treatment at cath, most commonly sildenafil (n = 24, 45%) and/or intravenous treprostinil (n = 16, 30%). Overall, hemodynamics were consistent with precapillary pulmonary hypertension. Pulmonary capillary wedge pressure was >15 mm Hg in 2 patients (4%). Lower fractional area change and worse ventricular strain were associated with higher pulmonary artery pressure while higher LV eccentricity index and higher RV/LV ratio were associated with both higher pulmonary artery pressure and higher pulmonary vascular resistance. Hemodynamics did not differ based on survival status. CONCLUSIONS: Worse RV dilation and dysfunction by echocardiogram correlate with higher pulmonary artery pressure and pulmonary vascular resistance on cath in this CDH cohort. These measures may represent novel, noninvasive clinical trial targets in this population.

A Novel Composed Index to Evaluate the Right Ventricle Free-Wall Adaptation Against Ventricular Wall Stress in Acute Pulmonary Embolism.

BACKGROUND: Pulmonary embolism severity index and simplified pulmonary embolism severity index have been utilized in initial risk evaluation in patients with acute pulmonary embolism. However, these models do not include any imaging measure of right ventricle function. In this study, we proposed a novel index and aimed to evaluate the clinical impact. METHODS: Our study population comprised retrospectively evaluated 502 patients with acute pulmonary embolism managed with different treatment modalities. Echocardiographic and computed tomographic pulmonary angiography evaluations were performed at admission to the emergency room within maximally 30 minutes. The formula of our index was as follows: (right ventricle diameter × systolic pulmonary arterial pressure-echo)/(right ventricle free-wall diameter × tricuspid annular plane systolic excursion). RESULTS: This index value showed significant correlations to clinical and hemodynamic severity measures. Only pulmonary embolism severity index, but not our index value, independently predicted in-hospital mortality. However, an index value higher than 17.8 predicted the long-term mortality with a sensitivity of 70% and specificity of 40% (areas under the curve = 0.652, 95% CI, 0.557-0.747, P =.001). According to the adjusted variable plot, the risk of long-term mortality increased until an index level of 30 but remained unchanged thereafter. The cumulative hazard curve also showed a higher mortality with high-index value versus low-index value. CONCLUSIONS: Our index composed from measures of computed tomographic pulmonary angiography and transthoracic echocardiography may provide important insights regarding the adaptation status of right ventricle against pressure/wall stress in acute pulmonary embolism, and a higher value seems to be associated with severity of the clinical and hemodynamic status and long-term mortality but not with in-hospital mortality. However, the pulmonary embolism severity index remained as the only independent predictor for in-hospital mortality.

The prognostic role of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) primarily affects the left ventricle (LV) sparing the right ventricle (RV) in vast majority of cases. However, several studies employing CMR have revealed that myocardial hypertrophy may also involve the RV. To assess RV size and function in a large prospectively cohort of HCM patients and to evaluate whether these parameters in association with other MR findings can predict cardiac events. Two participating centers prospectively included patients with known or suspected HCM between 2011 and 2017. CMR studies were performed with three different scanners. Outcome measures were a composite of ventricular arrhythmias, hospitalization for HF and cardiac death. Of 607 consecutive patients with known or suspected HCM, 315 had complete follow-up information (mean 65 ± 20 months). Among them, 115 patients developed major cardiac events (MACE) during follow-up. At CMR evaluation, patients with events had higher left atrium (LA) diameter (41.5 ± 8 mm vs. 37.17 ± 7.6 mm, p < 0.0001), LV mass (156.7 vs. 144 g, p = 0.005) and myocardial LGE (4.3% vs. 1.9%, p = 0.001). Similarly, patients with events had lower RV stroke volume index (42.7 vs. 47.0, p = 0.0003) and higher prevalence of both RV hypertrophy (16.4% vs. 4.7%, p = 0.0005) and reduced RV ejection fraction (12.2% vs. 4.4%, p = 0.006). In the multivariate analysis, LA diameter and RV stroke volume index were the strongest predictors of events (p < 0.001 and p = 0.0006, respectively). Anatomic and functional RV anomalies detected and characterized with CMR may have may have a major role in predicting the prognosis of HCM patients.

The Impact of Cystic Fibrosis- and Noncystic Fibrosis-Bronchiectasis on Pulmonary Artery Wall Thickness and Right Heart Functions Assessed by Speckle-Tracking Echocardiography.

BACKGROUND: Right heart functions are affected in patients with bronchiectasis as a result of pulmonary hypertension induced by chronic hypoxemia. Pulmonary artery wall thickness has recently been introduced as a sign of intensive and prolonged inflammation. The aim of this study was to analyze right ventricular and right atrial functions and to mea-sure pulmonary artery wall thickness in patients with cystic fibrosis-bronch iecta sis in comparison to those with noncystic fibrosis-bronchiectasis and healthy individuals. METHODS: We studied 36 patients with cystic fibrosis-bronchiectasis, 34 patients with noncystic fibrosis-bronchiectasis, and 32 age- and sex-matched control subjects. Lung function tests were performed. All subjects underwent comprehensive echocardiographic evaluation including conventional, tissue Doppler, speckle-tracking, and pulmonary artery wall thickness measurements. RESULTS: Right ventricular global longitudinal strain and global longitudinal right atrial strain during ventricular systole decreased in cystic fibrosis-bronchiectasis group compared with noncystic fibrosis-bronchiectasis and control groups (P <.001, both). Conversely, pulmonary artery wall thickness was increased in cystic fibrosis-bronchiectasis group in comparison to other groups (P <.001). Moreover, right ventricular global longitudinal strain was lower and pulmonary artery wall thickness was higher in patients with airflow obstruction (P <.001 and P =.025, respectively) than in those without. Only right ventricular global longitudinal strain was significantly correlated with pulmonary function test parameters. The negative effect of cystic fibrosis on right ventricular and right atrial functions was independent of age, gender, and disease duration. CONCLUSION: Our study showed that right ventricular and right atrial functions were deteriorated and pulmonary artery wall was thickened in cystic fibrosis-bronchiectasis patients more than noncystic fibrosis-bronchiectasis patients. Right ventricular global longitudinal strain detected subclinical right ventricular dysfunction and was associated with the severity of pulmonary disease.

The impact of right ventricular hemodynamics on the performance of a left ventricular assist device in a numerical simulation model.

OBJECTIVES: Left ventricular assist devices (LVADs) have been established as alternative to heart transplantation for patients with end-stage heart failure refractory to medical therapy. Right heart failure (RHF) after LVAD implantation is associated with inferior outcome. Its preoperative anticipation may influence the selection between a pure left ventricular and a biventricular device type and, thus, improve outcomes. Reliable algorithms to predict RHF are missing. METHODS: A numerical model was used for simulation of a cardiovascular circulation. The LVAD was placed as parallel circuit between left ventricle and aorta. In contrast to other studies, the dynamic hydraulic behavior of a pulsatile LVAD was replaced by that of a continuous LVAD. A variety of hemodynamic states was tested mimicking different right heart conditions. Adjustable parameters included heart rate (HR), pulmonary vascular resistance (PVR), tricuspid regurgitation (TR), right ventricular contractility (RVC) and pump speed. Outcome parameters comprised central venous pressure (CVP), mean pulmonary artery pressure (mPAP), cardiac output (CO) and occurrence of suction. RESULTS: Alteration of HR, PVR, TR, RVC and pump speed resulted in diverse effects on CO, CVP and mPAP, resulting in improvement, impairment or no change of the circulation, depending on the degree of alteration. CONCLUSIONS: The numerical simulation model allows prediction of circulatory changes and LVAD behaviour following variation of hemodynamic parameters. Such a prediction may be of particular advantage to anticipate RHF after LVAD implantation. It may help preoperatively to choose the appropriate strategy of only left ventricular or both left and right ventricular support.