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Little is known about the course of echocardiographic parameters used for the evaluation of valvular heart disease (VHD) during pregnancy, hampering interpretation of possible changes (physiological vs. pathophysiological). Therefore we studied the course of these parameters and ventricular function in pregnant women with aortic and pulmonary VHD. The cohort comprised 66 pregnant women enrolled in the prospective ZAHARA studies or evaluated by an identical protocol who had pulmonary VHD or aortic VHD (stenosis/prosthetic valve). The control group comprised 46 healthy pregnant women. Echocardiography was performed preconception, during pregnancy and 1 year postpartum. Peak gradient, mean gradient, aortic valve area (AVA)/effective orifice area (EOA), left ventricular ejection fraction (LVEF) and right ventricular function (RVF; TAPSE) were assessed. Peak and mean gradients increased during pregnancy compared to preconception in women with aortic VHD and controls (p < 0.0125), but not in women with pulmonary VHD. AVA/EOA remained unchanged. Preconception and postpartum gradients were comparable in all groups. Mean LVEF was normal in pregnant women with VHD and controls. Mean TAPSE was lower (p < 0.001) in women with pulmonary VHD compared to women with aortic VHD and controls (<20 mm vs. ≥23 mm; p < 0.001). In women with pulmonary VHD a decrease of TAPSE was observed during pregnancy (p = 0.005). Physiological changes during pregnancy lead to increased Doppler gradients in women with aortic VHD. This increase was not found in women with pulmonary VHD, probably caused by impaired RVF. Therefore, evaluation of RVF during pregnancy might be important to prevent underestimation of the degree of stenosis. • Serial changes in Doppler gradients and valve area in aortic and pulmonary valve disease were reported throughout pregnancy • Aortic valve gradients increased during pregnancy and returned to baseline postpartum, valve area remained unchanged • Valve gradients did not change in pregnant women with pulmonary valve disease, likely due to worse right ventricular function • Evaluation of the right ventricle might be important to prevent possible underestimation of the degree of pulmonary stenosis [ABSTRACT FROM AUTHOR]

Abstract Background Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy. Methods and results A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs −0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S′ lateral [1.0 (2.0) cm/s vs −0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E′ lateral [0.57 (2.4) cm/s vs −3.3 (3.9) cm/s; p < 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E′ lateral [−0.7 (1.9) vs 1.5 (1.9); p < 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs −5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [−0.18 (0.42) vs 0.23 (0.48); p = 0.037]. Conclusion Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified. Highlights • Left ventricular (LV) function is a known predictor of mortality after tetralogy of Fallot repair. • We aimed to characterise LV reverse remodelling achievable with ramipril therapy. • 6-months' ramipril treatment limits progression of both diastolic and systolic LV function. [ABSTRACT FROM AUTHOR]

Background Patients with congenital or acquired heart defects affecting the pulmonary valve and right ventricular outflow tract (RVOT) commonly require multiple surgical interventions, resulting in significant morbidity. A less invasive alternative is percutaneous pulmonary valve implantation (PPVI). Though studies have previously reported the safety and efficacy of the early generation transcatheter heart valves (THVs), data on more recent devices are severely lacking. Methods and results We performed a multinational, multicentre, retrospective, observational registry analysis of patients who underwent PPVI using the Edwards SAPIEN XT THV. Of the 46 patients that were enrolled, the majority had tetralogy of Fallot as the underlying diagnosis (58.7%), and stentless xenograft as the most common RVOT anatomy (34.8%). Procedural success rate was high (93.5%), with a low frequency of periprocedural complications and adverse events (6.5% and 10.9%, respectively). At 30 days post-procedure, NYHA class had improved significantly (90.6% were at NYHA I or II). The rate of moderate/severe pulmonary regurgitation had decreased from 76.1% at baseline to 5.0% at 30 days, and the calculated peak systolic gradient had decreased from 45.2 (SD ± 21.3) mm Hg to 16.4 (SD ± 8.0) mm Hg, with these values remaining low up to 2 years. Conclusions The data suggest the efficacy and safety of the SAPIEN XT THV in PPVI in common anatomies in patients with conduits, as well as those with native pulmonary valves or transannular patches. Continued data collection is necessary to verify long-term findings. Clinicaltrials.gov identifier NCT02302131 . [ABSTRACT FROM AUTHOR]

Abstract: Background: Infective endocarditis (IE) following percutaneous pulmonary valve replacement (PPVR) with the Melody valve is rarely reported. Furthermore, there are challenges in this diagnosis; especially echocardiographic evidence of vegetation within the prosthesis may be difficult. Method and result: This study is a retrospective review of all patients with Melody valve implantation in a tertiary centre. Between November 2006 and November 2012, 43 procedures were performed in 42 patients (mean age 25years, 6–67years). At a median follow-up of 27months (2–66months), six patients were suspected for IE. However, repeated transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) showed no evidence of IE and the patients were diagnosed as possible cases according to the modified Duke's criteria. Two patients did not respond to antibiotic treatment and underwent intra-cardiac echocardiography (ICE), which clearly demonstrated vegetations. These two cases required surgical explantation, while the other four patients were treated medically without complications. Conclusion: IE after Melody valve implantation is uncommon, but difficult to verify since TTE and TEE often cannot demonstrate vegetations inside the stent. ICE should be considered in suspected cases of IE following PPVR with negative TTE and TEE examinations in order to early tailor the best treatment for the individual patient suspected for IE. [Copyright &y& Elsevier]

Abstract: Background: Chronic severe pulmonary regurgitation (PR) causes progressive right ventricular (RV) dysfunction and heart failure. Parameters defining the optimal time point for surgery of chronic PR are lacking. The present study prospectively evaluated the impact of preoperative clinical parameters, cardiorespiratory function, QRS duration and NT-proBNP levels on post operative RV function and volumes assessed by cardiac magnetic resonance imaging (CMR) in patients with chronic severe PR undergoing pulmonary valve replacement. Methods and results: CMR was performed pre- and 6months postoperatively in 27 patients (23.6±2.9years, 15 women) with severe PR. Postoperatively, RV endsystolic (RVESVI) and enddiastolic volume indices (RVEDVI) decreased significantly (RVESVI pre 78.2±20.4ml/m² BSA vs. RVESVI post 52.2±16.8ml/m²BSA, p <0.001; RVEDVI pre 150.7±27.7ml/m²BSA vs. RVEDVI post 105.7±26.7ml/m²BSA; p <0,001). With increasing preoperative QRS-duration, postoperative RVEF decreased significantly (r =−0.57; p <0.005). Preoperative QRS-duration smaller than the median (156ms) predicted an improved RVEF compared to QRS-duration≥156ms (54.9% vs 46.8%, p <0.05). Multivariate analysis identified preoperative QRS duration as an independent predictor of postoperative RVEF (p <0.005). NT-proBNP levels correlated with changes in RVEDI (r =0.58 p <0,005) and RVESVI (r =0.63; p <0,0001). Multivariate analysis identified NT-proBNP levels prior to PVR as an independent predictor of volume changes (p <0.05). Conclusion: Valve replacement in severe pulmonary regurgitation causes significant reduction of RV volumes. Both, preoperative NT-proBNP level elevation and QRS prolongation indicate patients with poorer outcome regarding RV function and volumes. [Copyright &y& Elsevier]

Abstract: Objectives: Assessment of the long term outcome of mechanical valve prosthesis at pulmonary position in a population of grown-up congenital heart disease patients from a tertiary referral center. Methods: From 1977 to 2007, 22 consecutive patients underwent a total of 25 pulmonary valve replacements with mechanical prosthesis. The most frequent underlying cardiac condition was tetralogy of Fallot (n =16, 64%) and the mean age at the time of pulmonary valve replacement was 32±11years (range 14–50years). Results: The postoperative mortality rate was 4% ( n = 1) with no late deaths documented after a mean follow-up of 7.6±7.6years (range 0.29–24years). No major bleeding episodes occurred. Three patients presented with valve thrombosis in the setting of long term anticoagulation withdrawal and required valve re-replacement. Two of these patients, both with poor right ventricular function and overt clinical signs of right heart failure at the time of valve re-replacement, experienced further episodes of thrombosis despite correct anticoagulation. All episodes resolved with thrombolysis. After addition of antiplatelet treatment in one case and anticoagulation self-control, in the other, no further thrombosis has been documented. Conclusions: Mechanical valve prosthesis may be an alternative to tissue valve prosthesis in patients with congenital heart disease requiring pulmonary valve replacement. Optimal anticoagulation is crucial and additional antiplatelet treatment should be considered. Our data also suggest that patients with severe right ventricular dysfunction and congestive heart failure might be at particular risk for valve thrombosis. [Copyright &y& Elsevier]

Abstract: Purpose: To assess biventricular functional reserve (FR), NT-proBNP levels and exercise performance, in relation to right ventricular volume in patients with pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) at young age. Methods: In 53 TOF patients (maximum age at repair 2.0 years, interval since repair 15 (5) years) without residual lesions except PR, biventricular FR (derived from magnetic resonance imaging with dobutamine stress), NT-proBNP levels, maximal workload, and peak oxygen uptake were assessed. Results: Mean right ventricular end-diastolic volume was 140(38) ml/m2. Median pulmonary regurgitant fraction was 37% (range 0–57%). Biventricular systolic stress response was normal: mean (SD) ESV decreased (ΔRVESV −17(8) ml/m2, ΔLVESV −11(5)), SV increased (ΔRVSV +12(9) ml/m2, ΔLVSV +9(6)), FR was positive in all (RV-FR +11(5)%, LV-FR +13(6)). No serious adverse effects to dobutamine were encountered. NT-proBNP was increased in 2 patients. Median level was 10 pmol/L (range 2–42). NT-proBNP correlated with PR-percentage but not with right ventricular size. High-risk levels of NT-proBNP indicated a smaller RV-FR and a smaller decrease of biventricular ESV. Mean (SEM) VO2max was 96(3)%, mean Workloadmax 89(2)% of predicted. Conclusion: At mid to long term follow-up overall NT-proBNP levels are normal and biventricular functional reserve and exercise tolerance are well preserved in TOF repaired at young age, irrespective of RV volume. This questions the validity of isolated PR or RV volume criteria for pulmonary valve replacement in this group. Low-dose dobutamine stress testing is well tolerated and may be a useful additional tool for clinical decision making. [Copyright &y& Elsevier]

Abstract: Background: Pulmonary regurgitation is common after repair of tetralogy of Fallot, predisposing to right ventricular dilatation and potentially fatal arrhythmias. Magnetic resonance studies of such patients led us to hypothesize that the amount of regurgitation, in the absence of an effective valve, depends on pulmonary arterial compliance and on the location of resistance relative to the compliance. Methods and results: Using a pre-existing mathematical model representing the cardiovascular system, removal of the virtual pulmonary valve gave a triphasic pulmonary artery flow curve similar in shape to those recorded in patients with free regurgitation, with a regurgitant fraction of 30%. There was no reversal of flow at pulmonary capillary level, the regurgitant volume originating entirely from the compliance of the virtual pulmonary arteries and arterioles. Doubling their compliance increased regurgitation to 35%, whereas halving it decreased regurgitation to 23%. Doubling the total pulmonary vascular resistance by increasing arteriolar resistance increased regurgitation to 46%, whereas doubling it by simulating pulmonary annular stenosis proximal to the compliance limited regurgitation to 10%, but at the cost of a 32 mmHg peak systolic pressure drop. Conclusions: The model supported our hypotheses, indicating the relevance to pulmonary regurgitation of previously overlooked variables. The virtual pulmonary regurgitation was exacerbated by pulmonary artery compliance and by elevated resistance distal to it, but was limited by more proximal resistance. These relationships merit careful clinical investigation as they would have implications for the initial management, subsequent investigation and decisions on re-intervention in patients with pulmonary regurgitation. [Copyright &y& Elsevier]

Keywords: Cardiomyopathy; Pulmonary hypertension; Valve disease EN Cardiomyopathy Pulmonary hypertension Valve disease 185 185 1 03/06/21 20210415 NES 210415 To the editor, Zhang and colleagues investigated the comorbidities and clinical outcomes in patients with Takayasu arteritis and heart failure [1]. The endo-myocardial biopsy would be an informative procedure to assess whether heart failures were caused by disease-specific cardiomyopathy or any other conventional comorbidities including coronary artery stenosis and valvular disease. References 1 Y. Zhang, P. Fan, H. Zhang, Clinical characteristics and outcomes of chronic heart failure in adult Takayasu arteritis: A cohort study of 163 patients. [Extracted from the article]