The right ventricle in “Left-sided” cardiomyopathies: The dark side of the moon

The right ventricle (RV) has long been regarded as the forgotten and neglected cardiac chamber and it has been overshadowed by the left ventricle (LV). However, in the last decades, important advances in non-invasive cardiac imaging, from myocardial deformation imaging to cardiovascular magnetic resonance (CMR), have overcome the challenges imposed by the complex anatomy of the right heart, leading to a deep understanding of cardiovascular physiology and pathophysiology. The importance of the RV in different cardiac disease is now unquestionable and the current evidence emphasizes the forgotten interdependent relationship between the right and the left heart and the pivotal role of RV dysfunction in determining functional performance and outcomes in many cardiac disorders and particularly in cardiomyopathies. The purpose of this review is to summarize the current evidence about the diagnostic and prognostic value of the right heart in the “left-sided” cardiomyopathies, highlighting the relevance to assess RV size and function by multimodality imaging techniques in order to obtain useful information for a proper diagnostic workup and for the prognosis.     

Nonvolumetric echocardiographic indices of right ventricular systolic function: validation with cardiovascular magnetic resonance and relationship with functional capacity

Purpose: Right ventricular (RV) systolic function as measured by right ventricular ejection fraction (RVEF) has long been recognized as an important predictor of outcome in heart failure patients. The echocardiographic measurement of RV volumes and RVEF is challenging, however, owing to the unique geometry of the right ventricle. Several nonvolumetric echocardiographic indices of RV function have demonstrated prognostic value in heart failure. Comparison studies of these techniques with each other using RVEF as a benchmark are limited, however. Furthermore, the contribution of these various elements of RV function to patient functional status is uncertain. We therefore aimed to: (1) Determine which nonvolumetric echocardiographic index correlates best with RVEF as determined by cardiac magnetic resonance (CMR) imaging (the accepted gold standard measure of RV systolic function) and (2) Ascertain which echocardiographic index best predicts functional capacity. Methods: Eighty‐three subjects (66 with systolic heart failure and 17 healthy controls) underwent CMR, 2D echocardiography, and cardiopulmonary exercise testing for comparison of echocardiographic indices of RV function with CMR RVEF, 6‐minute walk distance and VO_{2 PEAK}. Results: Speckle tracking strain RV strain exhibited the closest association with CMR RV ejection fraction. Indices of RV function demonstrated weak correlation with 6‐minute walk distance, but basal RV strain rate by tissue velocity imaging had good correlation with VO_{2 PEAK}. Conclusion: Strain by speckle tracking echocardiography and strain rate by tissue velocity imaging may offer complementary information in the evaluation of RV contractility and its functional effects. (Echocardiography 2012;29:455‐463)     

Imaging and modern assessment of the right ventricle

The right ventricular function is difficult to assess owing to its complex morphology, structure and function. The right ventricle (RV) comprises three compartments, the inlet, the apex, and the outlet contracting with a peristaltic motion from the inflow to the outflow chamber and is tightly linked to left ventricular (LV) function through the pulmonary circulation, the interventricular septum and the myocardium inside the pericardial envelop. The relation of RV function to symptom occurrence, exercise capacity and prognosis in a wide variety of cardiac diseases emphasizes the usefulness of its routine assessment. The evaluation of the RV is largely carried out by echocardiography in daily clinical practice despite important limitations inherent to two-dimensional imaging. Multiple views and numerous parameters allow clinicians to integrate the RV function in the clinical decision-making process. Recent modalities of echocardiography such as myocardial deformation and three-dimensional imaging or exercise echocardiography are promising tools for the assessment of the RV. Cardiac magnetic resonance imaging provides the unique opportunity to image the RV in motion and in three dimensions without the limitation of echogenicity. Therefore, cardiac magnetic resonance imaging is taking a growing place in the assessment of the RV in a wide variety of cardio-pulmonary diseases as pulmonary hypertension, ischemia, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, heart failure or congenital heart diseases. Integrating the complex interplay between both ventricles and the pulmonary circulation, this review will discuss the latest results of standard and novel techniques allowing the assessment of RV function by echocardiography and cardiac magnetic resonance imaging, and will provide to the clinicians, facing therapeutic challenges, a comprehensive overview of right heart function.     

Arrhythmogenic cardiomyopathy of left ventricle. A rare event,but possible

Arrhythmogenic right ventricular dysplasia (ARVD) is a form of inherited cardiomyopathy characterized by fibro-fatty substitution mainly right ventricular (RV). Affected patients may succumb to life-threatening ventricular arrhythmias and heart failure. It is even more common among athletes who experience sudden cardiac death (SCD). The disease involvement is not limited only to the RV, but the left ventricle (LV) can also be involved. We have reported a case of a 38 years-old man, with two episodes of syncope in his history. After echocardiographic investigations, the patient was referred to cardiovascular magnetic resonance (CMR). Morphological images showed fatty infiltration of the epicardial layer of LV lateral wall (mid and apical segment). A diagnosis of ‘Isolated Left-Sided Arrhythmogenic Cardiomyopathy’ was made. An ICD implantation was performed, and a medical therapy with enalapril and bisoprolol was started.     

Multimodality Evaluation of the Right Ventricle: An Updated Review

The assessment of the volumes, function, and mechanics of the right ventricle (RV) is very challenging because of the anatomical complexity of the RV. Because RV structure, function, and deformation are very important predictors of cardiovascular morbidity and mortality in patients with heart failure, pulmonary hypertension, congenital heart disease, or arrhythmogenic RV cardiomyopathy, it is of great importance to use an appropriate imaging modality that will provide all necessary information. In everyday clinical practice, 2‐dimensional echocardiography (2DE) represents a method of first choice in RV evaluation. However, cardiac magnetic resonance (CMR) remained the gold standard for RV assessment. The development of new imaging tools, such as 3‐dimensional echocardiography (3DE), provided reliable data, comparable with CMR, and opened a completely new era in RV imaging. So far, 3DE has shown good results in determination of RV volumes and systolic function, and there are indications that it will also provide valuable data about 3‐dimensional RV mechanics, similar to CMR. Two‐dimensional echocardiography–derived strain is currently widely used for the assessment of RV deformation, which has been proven to be a more significant predictor of functional capacity and survival than CMR‐derived RV ejection fraction. The purpose of this review is to summarize currently available data about RV structure, function, and mechanics obtained by different imaging modalities, primarily 2DE and 3DE, and their comparison with CMR and cardiac computed tomography.     

Usefulness of magnetic resonance imaging for the diagnosis of right ventricular dysplasia in children

Cardiac magnetic resonance (CMR) has been helpful in adults in the diagnosis of arrhythmogenic right ventricular dysplasia. Short of direct surgical observation or autopsy, no gold standard exists. CMR diagnostic criteria include right atrial and ventricular dilation, regional right ventricular (RV) wall motion abnormalities, outflow tract ectasia, and myocardial fatty infiltration. To determine whether adult diagnostic criteria are useful in children referred for CMR for this diagnosis, the images and records of 81 patients (aged 11.5 +/- 5.5 years) over an 8-year period were reviewed. Histories included ventricular tachycardia, palpitations, dilated right ventricle, syncope, near sudden death, or family history of RV dysplasia. Four families were studied with parents who had RV dysplasia diagnosed by surgery, explanted heart, or CMR. CMR imaging included T1-weighted imaging, cine, 1-dimensional RV myocardial tagging, and phase-encoded velocity mapping, and 2 patients underwent delayed-enhancement CMR. Only 1 of the 81 patients met 5 of the criteria. None of the others met >2 of the criteria, and only 2 patients met 1 or 2 criteria. For questionable regional wall motion abnormalities, RV myocardial tagging was helpful. In conclusion, CMR of patients with a history suspicious for the diagnosis of RV dysplasia is a low-yield test in children. This may be due to the evolving nature of the disease, which does not manifest itself from a morphologic or ventricular-function standpoint until later in development. Follow-up studies as patients age may be advantageous.     

Echocardiographic assessment of the right ventricle in the current era: Application in clinical practice

The right ventricle has unique structural and functional characteristics. It is now well recognized that the so‐called forgotten ventricle is a key player in cardiovascular physiology. Furthermore, there is accumulating evidence that demonstrates right ventricular dysfunction as an important marker of morbidity and mortality in several commonly encountered clinical situations such as heart failure, pulmonary hypertension, pulmonary embolism, right ventricular myocardial infarction, and adult congenital heart disease. In contrast to the left ventricle, echocardiographic assessment of right ventricular function is more challenging as volume estimations are not possible without the use of three‐dimensional (3D) echocardiography. Guidelines on chamber quantification provide a standardized approach to assessment of the right ventricle. The technique and limitations of each of the parameters for RV size and function need to be fully understood. In this era of multimodality imaging, echocardiography continues to remain a useful tool for the initial assessment and follow‐up of patients with right heart pathology. Several novel approaches such as 3D and strain imaging of the right ventricle have expanded the usefulness of this indispensable modality.     

Anatomy and physiology of the right ventricle

Under normal baseline conditions the unique anatomy, myocardial ultrastructure, and coronary physiology of the right ventricle (RV) reflect a high-volume low-pressure pump. Early work described the RV as a passive conduit with minimal pumping capability. It is now appreciated that through a mechanism of ventricular interdependence, RV systolic function and diastolic load are extremely important in the prognosis and treatment of congestive heart failure, cardiac transplantation, pulmonary hypertension, congenital heart disease, and left ventricle assist devices. Magnetic resonance imaging with three-dimensional analysis has shown the complex geometry of the RV and the interaction of both ventricles within the pericardium.     

Right ventricular function following cardiac resynchronization therapy

The effect of cardiac resynchronization therapy (CRT) on right ventricular (RV) function has not been well described. The purpose of this study was to use tissue Doppler imaging to assess changes in RV function after CRT. Thirty-five patients with heart failure (age 65 +/- 10 years; 26 men) who underwent color tissue Doppler imaging of the right ventricle both immediately before CRT and >3 months (mean 6 +/- 3) after were studied. Myocardial systolic velocity was measured at the tricuspid annulus and basal and midventricular segments of the right ventricle free wall and averaged to obtain a measure of global RV function (RV S(m)). Left ventricular ejection fraction was also measured using biplane Simpson's method before and after CRT. RV S(m) significantly improved after CRT (5.4 +/- 1.9 to 7.1 +/- 2.6 cm/s; p <0.001), as did left ventricular ejection fraction (26 +/- 6% to 34 +/- 10%; p <0.001). Twenty-one of 35 patients (60%) showed an increase in RV S(m) > or =1 cm/s, with an increase > or =2 cm/s in 13 of those patients (37%). Improvement was seen in both ischemic and nonischemic patients and was independent of improvement in left ventricular ejection fraction and baseline left ventricular dyssynchrony. In conclusion, CRT resulted in improved RV function measured as RV S(m). CRT had beneficial effects on RV function independent of improvement in left ventricular ejection fraction.     

Biventricular response to supine physical exercise in young adults assessed with ultrafast magnetic resonance imaging

Simultaneous assessment of left ventricular (LV) and right ventricular (RV) response to exercise is limited with the current imaging modalities. Magnetic resonance imaging (MRI) techniques are now under development that allow near real-time evaluation of biventricular function under physical stress. This approach may open new avenues to study heart function in response to exercise in health and disease. The aim of this study was to evaluate biventricular response to supine physical exercise using ultrafast MRI. Biventricular volumes and function were examined in 16 healthy volunteers (mean age 18 +/- 2 years) using an ultrafast MRI sequence at rest and during an exercise protocol on a MRI compatible bicycle ergometer. Exercise level was individualized at the workload corresponding to 60% of the maximal oxygen uptake. All subjects completed the exercise MRI examination, allowing functional evaluation. Stroke volume of both ventricles increased from rest to exercise (left ventricle, 89 +/- 14 ml vs 102 +/- 19 ml, p < 0.05; right ventricle, 88 +/- 14 ml vs 101 +/- 16 ml, p < 0.05). Ejection fraction also increased in both ventricles from rest to exercise (left ventricle, 63 +/- 6% vs 74 +/- 6%, p < 0.05; right ventricle, 61 +/- 6% vs 70 +/- 6%, p < 0.05). End-systolic volume of the left and right ventricles decreased from rest to exercise (left ventricle, -33 +/- 12%, p < 0.05; right ventricle, -25 +/- 12%, p < 0.05), whereas LV and RV end-diastolic volumes remained unchanged. The results fit well with current concepts of cardiac physiology, and therefore we conclude that ergometer-induced exercise MRI is a valid approach to assess physiologic changes in LV and RV function simultaneously.     

Serial right ventricular thallium-201 imaging after exercise: Relation to anatomy of the right coronary artery

The relation of the appearance of the right ventricle on serium thallium-201 myocardial imaging to coronary artery anatomy was examined in 88 consecutive patients undergoing exercise thallium-201 testing and coronary angiography for the evaluation of chest pain. Transient defects in the right ventricle were found in 8 patients. All had high grade (≥ 90 %) stenosis of the proximal right coronary artery. Nonvisualization of right ventricular (RV) activity occurred in 10 patients. Nine of the 10 (90%) had significant (>- 50% stenosis) disease of the proximal right coronary artery and 7 (70%) had high grade stenosis. The right ventricle appeared normal in 70 patients. Twenty-nine (41 %) of these patients had significant proximal right coronary artery disease. Right ventricular appearance was not affected by the presence or absence of disease of the left anterior descending or left circumflex artery or by the appearance of the left ventricle.

Thus, with serial RV thallium-201 myocardial imaging after exercise, we found that (1) RV transient defects suggest the presence of high grade proximal right coronary artery stenosis, (2) nonvisualization of RV activity also predicts significant proximal right coronary disease, and (3) the right ventricle frequently appears normal despite proximal right coronary artery disease and therefore this finding does not exclude such disease.     

Right ventricular involvement in hypertrophic cardiomyopathy: a case report and literature review

Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) abnormalities have also been reported. However, involvement of the right ventricle in HCM has not been extensively characterized. The literature regarding prevalence, genetics, patterns of involvement, histologic findings, symptoms, diagnosis, and treatment of RV abnormalities in HCM is reviewed. To highlight the salient points, a case is presented of apical HCM with significant RV involvement, with an RV outflow tract gradient and near obliteration of the RV cavity, in the absence of a left intraventricular gradient. Right ventricular involvement in HCM appears to be as heterogeneous as that of the left ventricle. The spectrum extends from mild concentric hypertrophy to more unusual severe, obstructive disease. While in some cases the extent of RV involvement correlates with left ventricular (LV) involvement, predominant RV disease can be seen as well. While the genetics of RV involvement have not been well characterized, histologic findings appear to be similar to those in the left ventricle, suggesting similar pathogenesis. Significant RV involvement may result in RV outflow obstruction and/or reduced RV diastolic filling, with potentially increased incidence of severe dyspnea, supraventricular arrhythmias, and pulmonary thromboembolism. The optimal treatment for patients with significant RV disease is unknown. Medical and surgical therapies have been attempted with variable success; experience with newer techniques such as percutaneous catheter ablation has not been reported. Further characterization of RV involvement in HCM is necessary to elucidate more clearly the clinical features and optimal treatments of this manifestation of HCM.     

Imaging and quantifying valvular heart disease using magnetic resonance techniques

Opinion statement Echocardiography remains the cornerstone of noninvasive valvular heart disease evaluation. There are instances where MRI can be of use. Aside from the obvious advantage where limited acoustic windows are present, cardiac magnetic resonance (CMR) allows for imaging in any desired plane, and advantage can be taken of the ability to align with any regurgitant or stenotic flow jet. The high spatial resolution and contrast allow for accurate detail of valvular anatomy, but it must be remembered that the images represent a composite of eight to 12 heart cycles. For visualizing multiple valvular abnormalities simultaneously, cardiac MRI has a distinct advantage. Finally, a CMR valvular examination can be combined with accurate assessments of left and right ventricular function, myocardial stress perfusion imaging, and detailed viability determinations in a single examination. This provides a comprehensive presurgical evaluation of cardiac physiology.     

Echocardiographic knowledge-based reconstruction for quantification of the systemic right ventricle in young adults with repaired D-transposition of great arteries

The systemic right ventricle (RV) in congenital heart disease is susceptible to progressive dilation and dysfunction. A 2-dimensional echocardiographic means for serial monitoring of the RV would be of great value in this clinical setting. We used 2-dimensional echocardiography with knowledge-based reconstruction (2DE-KBR) for evaluation of systemic RV. Patients with d-transposition of great arteries repaired with an atrial switch and without implanted pacemakers were prospectively recruited for same-day 2DE-KBR and cardiac magnetic resonance (CMR) imaging. RV images were acquired in various 2-dimensional imaging planes using a 3-dimensional space-localizing device attached to the imaging transducer and 3-dimensional reconstruction was performed. RV end-diastolic volume, end-systolic volume, and ejection fraction (EF) were calculated and compared to volumetric CMR analysis. Fifteen patients (7 women, 8 men, 24 ± 7 years old, weight 67 ± 12 kg) were studied. There was good agreement of 2DE-KBR and CMR measurements. Mean RV end-diastolic volume was 221 ± 39 ml with 2DE-KBR and 231 ± 35 ml with CMR (r = 0.80); mean end-systolic volume was 129 ± 35 ml with KBR and 132 ± 30 ml with CMR (r = 0.82), and EF was 42 ± 10% with KBR and 43 ± 7% with CMR (r = 0.86). For 2DE-KBR mean interobserver variabilities were 4.6%, 2.6%, and 4.3%; intraobserver variabilities were 3.2%, 3.1%, and 2.3%, respectively, for end-diastolic volume, end-systolic volume, and EF. In conclusion, this study demonstrates the clinical feasibility of quantifying systemic RV volumes and function using 2DE-KBR in adolescents and young adults with repaired d-transposition of great arteries and good agreement of measurements with CMR.     

Hepatopulmonary syndrome and right ventricular diastolic functions: an echocardiographic examination

Aim: Liver functions are affected in the course of cardiac diseases, and similarly, liver diseases affect cardiac functions. Many studies in the literature have shown that left ventricular systolic and/or diastolic dysfunction may develop during chronic liver disease. However, there are limited studies investigating right ventricular functions during chronic liver diseases. Methods: A total of 84 patients who had no systolic and/or diastolic dysfunction in the left ventricle (LV) were evaluated; 46 patients with liver cirrhosis; 10 (21.74%) cirrhotic patients with hepatopulmonary syndrome (HPS) (group 1), 36 (78.26) cirrhotic patients without HPS (group 2), and 38 healthy individuals were treated as control. Results: Right ventricular diastolic dysfunction was determined in all patients of group 1 (100%), 26 of group 2 (72.22 %), and 4 of the controls (10.52%) (P < 0.05). Tricuspid deceleration time (dt) was significantly different between the groups (P < 0.05). In addition, right atrium (RA) diameters, right ventricle (RV) diameters, and RV wall thickness were significantly different between the groups (P < 0.05). Pulmonary artery pressure (P < 0.05) and pulmonary vascular resistance (P < 0.05) were also seen to be higher in group 1 than those in group 2 and control group. Conclusions: Right ventricular diastolic dysfunction rate is high in chronic liver diseases. In the presence of HPS, right ventricular diastolic dysfunction is more remarkable in patients than those without HPS. Right ventricular diastolic dysfunction may result in dilatation and hypertrophy in the right heart.     

Imaging of ventricular function by cardiovascular magnetic resonance

Over the past 15 years, cardiac magnetic resonance imaging (CMR) has vaulted to the forefront as the ideal diagnostic modality for the evaluation of both left and right ventricular function. The accumulated literature supports this contention for the left ventricle. However, for the right ventricle, typically poorly visualized accurately by traditional imaging techniques, CMR has emerged as the test of choice. Although earlier CMR sequences have become even more robust, resulting in further improvements in spatial and temporal resolution, CMR has avidly remained the gold standard. Yet, these attributes that have so benefited investigations of the systole need not be so constrained. In this review, we discuss recent applications of CMR to the study of lusitropy, demonstrating the potential for further advances in our understanding of diastole.     

Volume measurement by CARTO compared with cardiac magnetic resonance.

AIMS: The CARTO electrophysiological mapping system has demonstrated accurate results for end-diastolic ventricular volumes in casts and animals. However, in humans, a comparison with cardiac magnetic resonance (CMR), the non-invasive gold standard for volumetric analysis, has not yet been performed. METHODS AND RESULTS: A total of 34 (29 male) heart failure patients (NYHA class III/IV) underwent an electrophysiological mapping procedure with the CARTO system in the left ventricle (LV) (n = 34) and right ventricle (RV) (n = 12) and CMR for RV and LV end-diastolic volume (RVEDV and LVEDV) measurements another day. Mean LVEDV was comparable between CMR and CARTO (328 +/- 95 and 320 +/- 92 mL, respectively; P = NS), whereas RV volumes measured by CARTO were larger (CMR 140 +/- 48 vs. CARTO 176 +/- 47 mL; P < 0.01). Overall, we found a good correlation between CMR and CARTO measurements for both chambers; however, the Bland-Altman analysis showed a non-interchangeability of these methods. Measurement differences were independent of chamber size, but significantly affected by the number of acquired mapping points. CONCLUSION: Although CMR and CARTO showed a good correlation in the measurement of RVEDV and LVEDV in a group of heart failure patients, the clinical interchangeability of the two methods may be questioned.     

Imaging the right heart: the use of integrated multimodality imaging

During recent years, right ventricular (RV) structure and function have been found to be an important determinant of outcome in different cardiovascular and also pulmonary diseases. Currently, echocardiography and cardiac magnetic resonance (CMR) imaging are the two imaging modalities most commonly used to visualize the RV. Most structural abnormalities of the RV can be reliably described by echocardiography but due its complex geometrical shape, echocardiographic assessment of RV function is more challenging. Newer promising echocardiographic techniques are emerging but lack of validation and limited normal reference data influence their routine clinical application. Cardiac magnetic resonance is generally considered the clinical reference technique due to its unlimited imaging planes, superior image resolution, and three-dimensional volumetric rendering. The accuracy and reliability of CMR measurements make it the ideal tool for serial examinations of RV function. Multidetector computed tomography (MDCT) plays an important role in the diagnosis of pulmonary emboli but can also be used for assessing RV ischaemic disease or as an alternative for CMR if contra-indicated. Radionuclide techniques have become more obsolete in the current era. The different imaging modalities should be considered complimentary and each plays a role for different indications.     

Utility of right ventricular tissue Doppler imaging: correlation with right heart catheterization

Objectives: The objective of this study was to correlate tissue Doppler imaging of the right ventricle (RV) with pulmonary hemodynamics in patients referred for right heart catheterization. Methods: Seventy subjects (mean age 54 ± 13; 35 males) prospectively underwent tissue Doppler imaging of the RV and right heart catheterization within 1 day of each other. Peak systolic velocity and strain were measured at the RV free wall and correlated with pulmonary hemodynamics. Results: RV myocardial velocity demonstrated no correlation with any hemodynamic variable. While RV strain demonstrated significant correlation with cardiac index (r =−0.61; P < 0.001), correlations with transpulmonary gradient (r = 0.26; P < 0.05) and pulmonary vascular resistance (r = 0.30; P < 0.05) were weaker. Subgroup analysis revealed that in patients with left ventricular systolic dysfunction (n = 31), RV strain showed no correlation with any hemodynamic variable. In patients with normal left ventricular systolic function (n = 39), correlations were significant between RV strain and mean pulmonary artery pressure (r = 0.59; P < 0.001), pulmonary vascular resistance (r = 0.60; P < 0.001), and cardiac index (r =−0.67; P < 0.001). Conclusions: RV myocardial strain correlates significantly with pulmonary hemodynamics in patients with pulmonary hypertension and normal left ventricular function. However, there is no correlation with RV performance in patients with left ventricular dysfunction.     

Right ventricular thickness as predictor of global myocardial performance in systemic sclerosis: A Doppler tissue imaging study

Background

Cardiopulmonary involvement in systemic sclerosis (SSc) is a poor prognostic factor, due to pulmonary hypertension and right ventricular dysfunction. We assessed the echocardiographic parameters of right ventricular (RV) function in SSc and correlated echocardiographic findings to clinical features of the disease.

Methods

Thirty patients with SSc (cases) and 30 healthy, age-matched subjects (controls) were studied. Echocardiography, including tissue Doppler imaging, was used to evaluate cardiac function.

Results

Pulmonary hypertension could be documented in only 5 cases by Doppler echo, using Bernoulli principle. RV diastolic function was significantly deranged in cases. RV systolic function and left ventricle (LV) diastolic function were also significantly deranged in the cases. RV thickness was increased in patients with SSc. There were no significant differences in the echocardiographic variables between diffuse and limited subtypes of SSc. Myocardial performance index (MPI) of both ventricles were increased in cases. We could demonstrate RV thickness as the single most important predictor of MPI of both ventricles with sensitivity of 82% and specificity of 72% for RV-MPI and 63% for LV-MPI. Diastolic function was not found to be affected by disease duration or Rodnan skin score.

Conclusion

Patients with SSc exhibit abnormal RV and LV diastolic functions as well as abnormal RV systolic function. RV wall thickness was found to be simple and the single best predictor of global myocardial performance. RV dysfunction may be a response to intermittent pulmonary arterial hypertension, lung parenchymal involvement, or secondary to LV diastolic dysfunction in SSc.Abbreviations: ACE-I, angiotensin converting enzyme inhibitor; DT, deceleration time; DTI, Doppler tissue imaging; E/A ratio, early diastolic/atrial component velocity ratio; ET, ejection time; FVC, forced vital capacity; Hct, hematocrit; HRCT, high-resolution computed tomography; IVCT/ICT, isovolumic contraction time; ILD, interstitial lung disease; IVRT/IRT, isovolumic relaxation time; LV, left ventricle/ventricular; LVEDD, left ventricular end diastolic dimension; LVEDV, left ventricular end diastolic volume; LVEF, left ventricular ejection fraction; LVESD, left ventricular end systolic dimension; LVESV, left ventricular end systolic volume; MPI, myocardial performance index; PAH, pulmonary arterial hypertension; PAP, pulmonary artery pressure; PASP, pulmonary artery systolic pressure; PAT, pulmonary acceleration time; RR, electrocardiographic R–R interval; RVEF, right ventricular ejection fraction; RV, right ventricle/ventricular; SSc, systemic sclerosis