We present the case of a 52-year-old woman with cardiogenic shock and refractory right ventricular failure due to spontaneous dissection of the right coronary artery. She remained dependent on... Show moreWe present the case of a 52-year-old woman with cardiogenic shock and refractory right ventricular failure due to spontaneous dissection of the right coronary artery. She remained dependent on mechanical support for several weeks. Both a right ventricular assist device implant and a bidirectional cavopulmonary anastomosis were explored as long-term support options. A history of malignancy and possible right ventricular functional recovery resulted in a decision in favour of the bidirectional cavopulmonary anastomosis and concomitant tricuspid valve annuloplasty. Postoperatively her clinical condition improved significantly, and she could be discharged home. Echocardiography showed normalization of right ventricular dimensions and slight improvement of right ventricular function. Show less
Aimo, A.; Teis, A.; Kasa, G.; Juncà, G.; Lupón, J.; Domingo, M.; ... ; Delgado, V. 2023
Background Age-specific and gender-specific reference values for left ventricular (LV) and right ventricle volumes are available. The prognostic implications of the ratio between these volumes in... Show moreBackground Age-specific and gender-specific reference values for left ventricular (LV) and right ventricle volumes are available. The prognostic implications of the ratio between these volumes in heart failure and preserved ejection fraction (HFpEF) have never been evaluated.Methods We examined all HFpEF outpatients undergoing a cardiac magnetic resonance from 2011 to 2021. The left-to-right ventricular volume ratio (LRVR) was defined as the ratio between the LV and right ventricle end-diastolic volume indexes (LVEDVi/RVEDVi).Results Among 159 patients [median age 58 years (interquartile range 49–69), 64% men, LV ejection fraction 60% (54–70%)] the median LRVR was 1.21 (1.07–1.40). Over 3.5 years (1.5–5.0), 23 patients (15%) experienced all-cause death or heart failure hospitalization, and 22 (14%) cardiovascular death or heart failure hospitalization. The risk of all-cause death or heart failure hospitalization increased with an LRVR less than 1.0 or at least 1.4. An LRVR less than 1.0 was associated with a higher risk of all-cause death or heart failure hospitalization [hazard ratio 5.95, 95% confidence interval (CI) 1.67–21.28; P = 0.006] and cardiovascular death or heart failure hospitalization (hazard ratio 5.68, 95% CI 1.58–20.35; P = 0.008) as compared with LRVR 1.0–1.3. Furthermore, an LRVR at least 1.4 was associated with a higher risk of all-cause death or heart failure hospitalization (hazard ratio 4.10, 95% CI 1.58–10.61; P = 0.004) and cardiovascular death or heart failure hospitalization (hazard ratio 3.71, 95% CI 1.41–9.79; P = 0.008) as compared with LRVR 1.0–1.3. These results were confirmed in patients without dilation of either ventricle.Conclusion LRVR values less than 1.0 or at least 1.4 are associated with worse outcomes in HFpEF. LRVR may become a valuable tool for risk prediction in HFpEF. Show less
Meucci, M.C.; Malara, S.; Butcher, S.C.; Hirasawa, K.; Kley, F. van der; Lombardo, A.; ... ; Graziani, F. 2023
BackgroundThere is limited evidence regarding the association between right ventricular-to-pulmonary artery (RV-PA) coupling and outcomes after transcatheter aortic valve replacement (TAVR)... Show moreBackgroundThere is limited evidence regarding the association between right ventricular-to-pulmonary artery (RV-PA) coupling and outcomes after transcatheter aortic valve replacement (TAVR).ObjectivesThis study aimed to explore the evolution of RV-PA coupling in patients with severe aortic stenosis undergoing TAVR and its prognostic impact.MethodsA total of 900 patients who underwent TAVR in 2 tertiary centers and with echocardiographic analysis performed within 3 months before and after the procedure were included. RV-PA coupling was measured as the ratio of tricuspid annular plane systolic excursion (TAPSE) to pulmonary artery systolic pressure (PASP). RV-PA uncoupling was defined by TAPSE/PASP <0.55, whereas a TAPSE/PASP <0.32 identified a severe uncoupling. The primary endpoint was all-cause mortality.ResultsA total of 520 patients (58%) showed RV-PA uncoupling before TAVR, whereas post-TAVR RV-PA uncoupling was observed in 407 patients (45%). During a median follow-up of 40 months, 250 deaths (28%) occurred. Post-TAVR RV-PA uncoupling was independently associated with an increased risk of mortality (adjusted HR: 1.474; 95% CI: 1.115-1.948; P = 0.006), whereas pre-TAVR uncoupling did not. Among patients with post-TAVR RV-PA uncoupling, the presence of severe uncoupling identified a subgroup with the worst survival (P = 0.008). Patients with RV-PA coupling recovery after TAVR showed similar outcomes as compared with patients with normal coupling. Conversely, the presence of either persistent or new-onset RV-PA uncoupling following TAVR was associated with an increased mortality risk.ConclusionsPost-TAVR RV-PA uncoupling is an independent predictor of long-term mortality, irrespective of coupling before intervention. Assessment of TAPSE/PASP response after TAVR may be helpful to improve risk stratification. Show less
Meucci, M.C.; Lillo, R.; Lombardo, A.; Lanza, G.A.; Bootsma, M.; Butcher, S.C.; ... ; Graziani, F. 2022
Aims:To perform a comparative analysis of right ventricle (RV) myocardial mechanics, assessed by 2D speckle-tracking echocardiography (2D-STE), between patients with Fabry disease and patients with... Show moreAims:To perform a comparative analysis of right ventricle (RV) myocardial mechanics, assessed by 2D speckle-tracking echocardiography (2D-STE), between patients with Fabry disease and patients with sarcomeric disease.Methods and results: Patients with Fabry cardiomyopathy (FC) (n = 28) were compared with patients with sarcomeric hypertrophic cardiomyopathy (HCM), matched for degree of left ventricle hypertrophy (LVH) and demographic characteristics (n = 112). In addition, patients with Fabry disease and no LVH [phenotype-negative carriers of pathogenic alpha-galactosidase gene mutations (GLA LVH-)] (n = 28) were compared with age and sex-matched carriers of sarcomeric gene mutations without LVH [Phenotype-negative carriers of pathogenic sarcomeric gene mutations (Sarc LVH-)] (n = 56). Standard echocardiography and 2D-STE were performed in all participants. Despite a subtle impairment of RV global longitudinal strain (RV-GLS) was common in both groups, patients with FC showed a more prominent reduction of RV free wall longitudinal strain (RV-FWS) and lower values of difference between RV-FWS and RV-GLS (Delta RV strain), in comparison to individuals with HCM (P <0.001 and P = 0.002, respectively). RV-FWS and Delta RV strain demonstrated an independent and additive value in discriminating FC from HCM, over the presence of symmetric LVH, systolic anterior motion of the mitral valve and RV hypertrophy. Similar results were found in GLA LVH- patients: they had worse RV-FWS and lower values of Delta RV strain as compared to Sarc LVH- patients (both P <0.001).Conclusion: Patients with FC show a specific pattern of RV myocardial mechanics, characterized by a larger impairment of RV-FWS and lower Delta RV strain in comparison to patients with HCM, which may be helpful in the differential diagnosis between these two diseases.[GRAPHICS]. Show less
Assessment of right ventricular (RV) function is crucial for the evaluation of the dyspnoeic patient and/or with systemic venous congestion and provides powerful prognostic insights. It can be... Show moreAssessment of right ventricular (RV) function is crucial for the evaluation of the dyspnoeic patient and/or with systemic venous congestion and provides powerful prognostic insights. It can be performed using different imaging modalities including standard and advanced echocardiographic techniques, cardiac magnetic resonance imaging, computed tomography, and radionuclide techniques, which should be used in a complementary fashion. Each modality has strengths and weaknesses based on which the choice of their use and in which combination may vary according to the different clinical scenarios as will be detailed in this review. The conclusions from multiple studies using different imaging techniques are concordant: RV function can be reliably assessed and is a critical predictor of clinical outcomes.[GRAPHICS]. Show less
Wezenbeek, J. van; Kianzad, A.; Bovenkamp, A. van de; Wessels, J.; Mouratoglou, S.A.; Braams, N.J.; ... ; Man, F.S. de 2022
Background: Heart failure with preserved ejection fraction (HFpEF) is a prevalent disorder for which no effective treatment yet exists. Pulmonary hypertension (PH) and right atrial (RA) and... Show moreBackground: Heart failure with preserved ejection fraction (HFpEF) is a prevalent disorder for which no effective treatment yet exists. Pulmonary hypertension (PH) and right atrial (RA) and ventricular (RV) dysfunction are frequently observed. The question remains whether the PH with the associated RV/RA dysfunction in HFpEF are markers of disease severity. Methods: To obtain insight in the relative importance of pressure-overload and left-to-right interaction, we compared RA and RV function in 3 groups: 1. HFpEF (n=13); 2. HFpEF-PH (n=33), and; 3. pulmonary arterial hypertension (PAH) matched to pulmonary artery pressures of HFpEF-PH (PH limited to mPAP >= 30 and <= 50 mmHg) (n=47). Patients underwent right heart catheterization and cardiac magnetic resonance imaging. Results: The right ventricle in HFpEF-PH was less dilated and hypertrophied than in PAH. In addition, RV ejection fraction was more preserved (HFpEF-PH: 52 +/- 11 versus PAH: 36 +/- 12%). RV filling patterns differed: vena cava backflow during RA contraction was observed in PAH only. In HFpEF-PH, RA pressure was elevated throughout the cardiac cycle (HFpEF-PH: 10 [8-14] versus PAH: 7 [5-10] mm Hg), while RA volume was smaller, reflecting excessive RA stiffness (HFpEF-PH: 0.14 [0.10-0.17] versus PAH: 0.08 [0.06-0.11] mm Hg/mL). RA stiffness was associated with an increased eccentricity index (HFpEF-PH: 1.3 +/- 0.2 versus PAH: 1.2 +/- 0.1) and interatrial pressure gradient (9 [5 to 12] versus 2 [-2 to 5] mm Hg). Conclusions: RV/RA function was less compromised in HFpEF-PH than in PAH, despite similar pressure-overload. Increased RA pressure and stiffness in HFpEF-PH were explained by left atrial/RA-interaction. Therefore, our results indicate that increased RA pressure is not a sign of overt RV failure but rather a reflection of HFpEF-severity. Show less
OBJECTIVES The aim of this study was to determine the prognostic impact of right ventricular (RV)-pulmonary arterial (PA) coupling in patients with heart failure (HF) with severe secondary mitral... Show moreOBJECTIVES The aim of this study was to determine the prognostic impact of right ventricular (RV)-pulmonary arterial (PA) coupling in patients with heart failure (HF) with severe secondary mitral regurgitation (SMR) enrolled in the COAPT (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients With Functional Mitral Regurgitation) trial.BACKGROUND RV contractile function and PA pressures influence outcomes in patients with SMR, but the impact of RV-PA coupling in patients randomized to transcatheter edge-to-edge repair (TEER) vs guideline-directed medical therapy (GDMT) is unknown.METHODS RV-PA coupling was assessed by the ratio of RV free wall longitudinal strain derived from speckle-tracking echocardiography and noninvasively measured RV systolic pressure. Advanced RV-PA uncoupling was defined as RV free wall longitudinal strain/RV systolic pressure <= 0.5%/mm Hg. The primary endpoint was a composite of all-cause mortality or HF hospitalization at 24-month follow-up.RESULTS A total of 372 patients underwent speckle-tracking echocardiography, and 70.2% had advanced RV-PA uncoupling. By multivariable analysis, advanced RV-PA uncoupling was strongly associated with an increased risk for the primary 24-month endpoint of death or HF hospitalization (HR: 1.87; 95% CI: 1.31-2.66; P = 0.0005). A similar association was present for all-cause mortality alone (HR: 2.57; 95% CI: 1.54-4.29; P = 0.0003). The impact of RV-PA uncoupling was consistent in patients randomized to TEER and GDMT alone. Compared with GDMT alone, the addition of TEER improved 2-year outcomes in patients with (48.0% vs 74.8%; HR: 0.51; 95% CI: 0.37-0.71) and those without (28.8% vs 47.8%; HR: 0.51; 95% CI: 0.27-0.97) advanced RV-PA uncoupling (P-interaction = 0.98).CONCLUSIONS In the COAPT trial, advanced RV dysfunction assessed by RV-PA uncoupling was a powerful predictor of 2-year adverse outcomes in patients with HF and SMR. (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients With Functional Mitral Regurgitation [The COAPT Trial]; NCT01626079) (C) 2021 by the American College of Cardiology Foundation. Show less
Butcher, S.C.; Fortuni, F.; Montero Cabezas, J.M.; Abou, R.; Mahdiui, M. el; Bijl, P. van der; ... ; Delgado, V. 2021
Aims Right ventricular myocardial work (RVMW) is a novel method for non-invasive assessment of right ventricular (RV) function utilizing RV pressure-strain loops. This study aimed to explore the... Show moreAims Right ventricular myocardial work (RVMW) is a novel method for non-invasive assessment of right ventricular (RV) function utilizing RV pressure-strain loops. This study aimed to explore the relationship between RVMW and invasive indices of right heart catheterization (RHC) in a cohort of patients with heart failure with reduced left ventricular ejection fraction (HFrEF), and to compare values of RVMW with those of a group of patients without cardiovascular disease.Methods and results Non-invasive analysis of RVMW was performed in 22 HFrEF patients [median age 63 (59-67) years] who underwent echocardiography and invasive RHC within 48 h. Conventional RV functional measurements, RV global constructive work (RVGCW), RV global work index (RVGWI), RV global wasted work (RVGWVV), and RV global work efficiency (RVGWE) were analysed and compared with invasively measured stroke volume and stroke volume index. Non-invasive analysis of RVMW was also performed in 22 patients without cardiovascular disease to allow for comparison between groups. None of the conventional echocardiographic parameters of RV systolic function were significantly correlated with stroke volume or stroke volume index. In contrast, one of the novel indices derived non-invasively by pressure-strain Loops, RVGCW, demonstrated a moderate correlation with invasively measured stroke volume and stroke volume index (r = 0.63, P=0.002 and r = 0.59, P= 0.004, respectively). RVGWI, RVGCW, and RVGWE were significantly lower in patients with HFrEF compared to a healthy cohort, while values of RVGWVV were significantly higher.Conclusion RVGCW is a novel parameter that provides an integrative analysis of RV systolic function and correlates more closely with invasively measured stroke volume and stroke volume index than other standard echocardiographic parameters. Show less
Fortuni, F.; Dietz, M.F.; Prihadi, E.A.; Bijl, P. van der; Ferrari, G.M. de; Knuuti, J.; ... ; Marsan, N.A. 2020
OBJECTIVES A novel tricuspid regurgitation (TR) grading system, using vena contracta (VC) width and effective regurgitant orifice area (EROA), was proposed and validated based on its prognostic... Show moreOBJECTIVES A novel tricuspid regurgitation (TR) grading system, using vena contracta (VC) width and effective regurgitant orifice area (EROA), was proposed and validated based on its prognostic usefulness.BACKGROUND The clinical need of a new grading system for TR has recently been emphasized to depict the whole spectrum of TR severity, particularly beyond severe TR (massive or torrential).METHODS TR severity was characterized in 1,129 patients with moderate or severe secondary TR (STR). Recently proposed cutoff values of VC width were more effective in differentiating the prognosis of patients with moderate STR, whereas EROA cutoff values performed better in characterizing the risk of patients with more severe STR. Therefore, these 2 parameters were combined into a novel grading system to define moderate (VC <7 mm), severe (VC >= 7 mm and EROA <80 mm(2)), and torrential (VC >= 7 mm and EROA >= 80 mm(2)) STR.RESULTS A total of 143 patients (13%) showed moderate STR, whereas 536 patients (47%) had severe STR, and 450 (40%) had torrential STR. Patients with torrential STR had larger right ventricular (RV) dimensions, lower RV systolic function, and were more likely to receive diuretics. The cumulative 10-year survival rate was 53% for moderate, 45% for severe, and 35% for torrential STR (p = 0.007). After adjusting for potential confounders, torrential STR retained an association with worse prognosis compared with other STR grades (hazard ratio: 1.245; 95% confidence interval: 1.023 to 1.516; p = 0.029).CONCLUSIONS A novel STR grading system was able to capture the whole range of STR severity and identified patients with torrential STR who were characterized by a worse prognosis. (C) 2021 by the American College of Cardiology Foundation. Show less
Prihadi, E.A.; Delgado, V.; Leon, M.B.; Enriquez-Sarano, M.; Topilsky, Y.; Bax, J.J. 2019
Tricuspid regurgitation (TR) is classified according to different morphologic types based on the underlying mechanisms: primary, secondary, and isolated TR. Primary TR is caused by an anatomical... Show moreTricuspid regurgitation (TR) is classified according to different morphologic types based on the underlying mechanisms: primary, secondary, and isolated TR. Primary TR is caused by an anatomical abnormality of the tricuspid valve apparatus. Secondary TR is caused by dilation of the tricuspid valve annulus, related to right ventricular (RV), or right atrial remodeling and increased RV pressures (often secondary to left-sided heart disease). Isolated TR can exist in patients without increased RV pressures and is frequently associated with atrial fibrillation. Two-dimensional echocardiography plays a pivotal role in the assessment of the etiology and severity of TR. Views from 3-dimensional techniques have significantly increased the understanding of the pathophysiology of each morphologic type of TR (leaflet damage, annular dilation, and distinct patterns of right-heart remodeling). The following review will describe the etiology, anatomical and functional characteristics, and outcomes of each morphologic type of TR, and furthermore addresses challenging pitfalls in the referral for tricuspid valve intervention. (C) 2019 by the American College of Cardiology Foundation. Show less
Ruohonen, S.; Koskenvuo, J.W.; Wendelin-Saarenhovi, M.; Savontaus, M.; Kahonen, M.; Laitinen, T.; ... ; Raitakari, O. 2016
