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Tetralogy of Fallot is a congenital heart disease affecting newborns and involves stenosis of the right ventricular outflow tract (RVOT). Surgical correction often widens the RVOT with a transannular enlargement patch, but this causes issues including pulmonary valve insufficiency and progressive right ventricle failure. A monocusp valve can prevent pulmonary regurgitation; however, valve failure resulting from factors including leaflet design, morphology, and immune response can occur, ultimately resulting in pulmonary insufficiency. A multimodal platform to quantitatively evaluate the effect of shape, size, and material on clinical outcomes could optimize monocusp design. This study introduces a benchtop soft biorobotic heart model, a computational fluid model of the RVOT, and a monocusp valve made from an entirely biological cell-assembled extracellular matrix (CAM) to tackle the multifaceted issue of monocusp failure. The hydrodynamic and mechanical performance of RVOT repair strategies was assessed in biorobotic and computational platforms. The monocusp valve design was validated in vivo in ovine models through echocardiography, cardiac magnetic resonance, and catheterization. These models supported assessment of surgical feasibility, handling, suturability, and hemodynamic and mechanical monocusp capabilities. The CAM-based monocusp offered a competent pulmonary valve with regurgitation of 4.6 ± 0.9% and a transvalvular pressure gradient of 4.3 ± 1.4 millimeters of mercury after 7 days of implantation in sheep. The biorobotic heart model, in silico analysis, and in vivo RVOT modeling allowed iteration in monocusp design not now feasible in a clinical environment and will support future surgical testing of biomaterials for complex congenital heart malformations. Editor's summary: Congenital heart defects such as Tetralogy of Fallot require surgical correction to address complex and multifactorial deficits in cardiac function. Here, Singh et al. develop a model of impaired right ventricular outflow tract function in a biorobotic heart paired with an in silico hemodynamic model. The hybrid soft robotic and ovine myocardium exhibited anatomical and biomechanical characteristics of the heart for ex vivo iteration of surgical repair strategies with a cell-assembled matrix biomaterial. The optimized surgical approach was further supported by an acute in vivo ovine model. This multimodal platform will support the hemodynamic evaluation of complex cardiac defects, aiding in the translation of new therapeutic approaches. —Molly Ogle [ABSTRACT FROM AUTHOR]

This meta-analysis assesses antiphospholipid antibodies' (aPLs) impact on heart valve disease in Systemic Lupus Erythematosus (SLE) patients. We searched PubMed, Embase, Cochrane, and Web of Science up to January 2024 for comparative studies of heart valve disease in aPL-positive versus aPL-negative SLE patients. Fixed-effect or random-effect models were used to synthesize data, with I2 and sensitivity analyses for heterogeneity and the trim-and-fill method for publication bias. Including 25 studies with 8089 patients, of which 919 had valvular changes, aPLs significantly increased the risk of heart valve disease (OR = 2.24, 95% CI: 1.58–3.18, p < 0.001). Lupus anticoagulant (LA) indicated the highest risk (OR = 4.90, 95% CI: 2.26–10.60, p < 0.001), anticardiolipin antibodies (aCL) doubled the risk (OR = 2.69, 95% CI: 1.47–4.93, p = 0.001), and anti-β2 glycoprotein I (aβ2GPI) showed a 70% increase (OR = 1.70, 95% CI: 1.17–2.45, p = 0.005). Valve-specific analysis indicated the mitral valve was most commonly involved (26.89%), with higher occurrences in aPL-positive patients (33.34% vs. 15.92%, p = 0.053). Aortic and tricuspid valve involvements were 13.11% vs. 5.42% (p = 0.147) and 12.03% vs. 8.52% (p = 0.039), respectively. Pulmonary valve disease was rare and similar across groups (1.01% in aPL-positive vs. 1.52% in aPL-negative). Significantly, only tricuspid valve disease showed increased risk in aPL-positive patients (OR = 2.66, 95% CI: 1.05–6.75, p = 0.039). APLs notably increase the risk of heart valve disease in SLE patients, with a pronounced effect on tricuspid valve involvement. Regular cardiac assessments for aPL-positive SLE patients are crucial for timely intervention and improved prognosis. [ABSTRACT FROM AUTHOR]

OBJECTIVE To evaluate the relationships between the severity of myxomatous mitral valve disease (MMVD) and pulmonary hypertension (PH) and serum angiopoietin (Ang)-1 and Ang-2 concentrations in dogs with MMVD. ANIMALS 74 dogs (control, n = 12; MMVD, n = 62) were included. METHODS Serum Ang-1 and Ang-2 concentrations were estimated using the canine-specific ELISA kit. The concentrations were compared between dogs with MMVD and healthy dogs, and they were analyzed according to the severity of MMVD and PH. RESULTS The median serum Ang-1 concentration did not differ among the study groups. The median serum Ang-2 concentration was higher in dogs with stage B2 MMVD (P = .041) and acute congestive heart failure (P = .002) than in control dogs. In addition, the median serum Ang-2 concentration was higher in MMVD dogs with PH than in those without PH (P = .031). Serum Ang-2 concentration was correlated with vertebral heart score (rs = 0.36, P = .004) and vertebral left atrial score (r = 0.50, P < .001) in dogs with MMVD, and correlated with vertebral heart score (r = 0.63, P = .01), maximum E wave amplitude of the diastolic transmitral flow (rs = 0.61, P = .018), ejection fraction (rs = -0.77, P < .001) and fractional shortening (rs = -0.56, P = .032) in dogs with acute congestive heart failure. CLINICAL RELEVANCE Circulating Ang-2 levels increase in dogs with the severity of MMVD and the presence of PH. [ABSTRACT FROM AUTHOR]

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1–58) days and 11 (22%) underwent outpatient PBV at 51.8 (11–174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02–94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS. [ABSTRACT FROM AUTHOR]

Background Primary intimal sarcomas of the heart are extremely rare and have a dismal prognosis. Their management represents a complex clinical challenge since complete surgical resection is the only reliable possibility of cure but is only possible in 50% of patients. In non-resectable disease, anthracycline-based therapy is the most effective treatment, but pazopanib may be used in patients unfit to receive anthracyclines. Case summary A 38-year-old man presented with acute right heart failure symptoms due to a primary intimal sarcoma of the heart. A definite diagnosis was made after cardiac surgery. Multi-modality cardiac imaging showed early recurrence of disease with mitral valve and pulmonary veins' invasion, and the patient was deemed inoperable. Due to chronic kidney disease and previous heart failure symptoms, he was started on first-line pazopanib palliative treatment. After 11 months of chemotherapy, there was good clinical tolerance and no evidence of disease progression, which occurred after 13 months. Discussion This case highlights the value of a multi-modality imaging approach for cardiac masses. Most importantly, it reports the successful treatment of a young patient with a primary intimal sarcoma of the heart who was started on palliative pazopanib, with a significantly higher progression-free survival than is reported in the literature. This finding may support pazopanib as a good alternative as first-line treatment when there is contraindication for anthracycline-based chemotherapy. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly characterized by rudimentary PV tissue with variable degrees of PV stenosis and regurgitant pulmonary blood flow. In most cases, it is associated with tetralogy of Fallot. In a minority of APVS cases, with an unknown frequency, intact ventricular septum (IVS), patent ductus arteriosus, and possible tricuspid atresia are present. This condition is known as non-Fallot type APVS. We describe a case of an antenatal diagnosis of APVS with IVS, a large patent ductus arteriosus, and ascending aorta dilatation. The mother was referred to our center at 32 weeks of gestation due to cardiomegaly on sonography. Fetal echocardiography revealed cardiomegaly, right atrial and ventricular enlargement, aneurysmal dilatation of the main pulmonary artery, and mild dilatation of the pulmonary artery branches. Also observed were IVS, rudimentary PV tissue with severe to-and-fro turbulence across the PV, a widely open ductus arteriosus, ascending aorta dilatation, and levorotation of the heart. After childbirth, our diagnosis was confirmed by echocardiography and surgery. The baby experienced severe respiratory distress. At 15 days of life, surgical intervention in the form of pulmonary artery arterioplasty was performed, resulting in good outcomes. The patient underwent follow-up for 6 months and showed reasonable health. [ABSTRACT FROM AUTHOR]

Objectives: Pulmonary regurgitation (PR) is common in adult congenital heart disease (ACHD). 2D phase contrast MRI is the reference method for the quantification of PR and helps in the decision of pulmonary valve replacement (PVR). 4D flow MRI can be an alternative method to estimate PR but more validation is still needed. Our purpose was to compare 2D and 4D flow in PR quantification using the degree of right ventricular remodeling after PVR as the reference standard. Methods: In 30 adult patients with a pulmonary valve disease recruited between 2015 and 2018, PR was assessed using both 2D and 4D flow. Based on the clinical standard of care, 22 underwent PVR. The pre PVR estimate of PR was compared using the post-operative decrease in right ventricle end-diastolic volume on follow-up exam as reference. Results: In the overall cohort, regurgitant volume (Rvol) and regurgitant fraction (RF) of PR measured by 2D and 4D flow were well correlated but with moderate agreement in the overall cohort (r = 0.90, mean diff. −14 ± 12.5 mL; and r = 0.72, mean diff. −15 ± 13%; all p < 0.0001). Correlations between Rvol estimates and right ventricle end-diastolic volume decrease after PVR was higher with 4D flow (r = 0.80, p < 0.0001) than with 2D flow (r = 0.72, p < 0.0001). Conclusions: In ACHD, PR quantification from 4D flow better predicts post-PVR right ventricle remodeling than that from 2D flow. Further studies are needed to evaluate the added value of this 4D flow quantification for guiding replacement decision. Key Points: • Using 4D flow MRI allows a better quantification of pulmonary regurgitation in adult congenital heart disease than 2D flow when taking right ventricle remodeling after pulmonary valve replacement as a reference. • A plane positioned perpendicular to the ejected flow volume as allowed by 4D flow provides better results to estimate pulmonary regurgitation. [ABSTRACT FROM AUTHOR]

Background: Progression to combined post‐ and pre‐capillary pulmonary hypertension (PH) provides prognostic information in human patients with post‐capillary PH. Pulmonary vascular resistance estimated by echocardiography (PVRecho) is useful for the stratification of dogs with myxomatous mitral valve disease (MMVD) and detectable tricuspid regurgitation. Objectives: To evaluate the prognostic value of PVRecho in dogs with MMVD. Animals: Fifty‐four dogs with MMVD and detectable tricuspid regurgitation. Methods: Prospective cohort study. All dogs underwent echocardiography. The PVRecho was calculated based on tricuspid regurgitation and the velocity‐time integral of the pulmonary artery flow. To evaluate the influence of echocardiographic variables on cardiac‐related deaths, Cox proportional hazard analysis was performed. Additionally, Kaplan‐Meier curves classified by PVRecho tertiles were made and compared using log‐rank tests to evaluate the influence of PVRecho on all‐cause mortality and cardiac‐related death. Results: The median follow‐up time was 579 days. Forty‐one dogs with MMVD (PH severity [number]: no or mild, 21/33; moderate, 11/11; severe, 9/10) died during the study. In the multivariable Cox proportional hazard analysis adjusted for age, sildenafil administration, and American College of Veterinary Internal Medicine stage of MMVD, left atrial to aortic diameter ratio and PVRecho remained significant (adjusted hazard ratio [95% confidence interval]: 1.2 [1.1‐1.3] and 2.1 [1.6‐3.0], respectively). Higher PVRecho showed a significant association with lower survival rates. Conclusions and Clinical Importance: Left atrial enlargement and high PVRecho were independent prognostic factors in dogs with MMVD and detectable tricuspid regurgitation. [ABSTRACT FROM AUTHOR]

Implantation of a temporary percutaneous right ventricular assist device (RVAD) in patients with right heart failure after left ventricular assist device (LVAD) implantation is an established technique that may cause complications. We present a 60-year-old male patient who underwent urgent LVAD implantation. On the second postoperative day the patient developed acute right heart failure. We implanted a temporary percutaneous RVAD with two cannulas via the right internal jugular vein and the right femoral vein. Transesophageal echocardiography revealed severe pulmonary insufficiency. After performing re-sternotomy we anastomosed a prosthetic graft to the pulmonary trunk (PT), performed subxiphoid tunneling of the graft and replaced the transjugular outflow cannula. The pulmonary regurgitation caused by the percutaneous transvalvular cannula disappeared. In such case a direct anastomosis to the PT is the solution. [ABSTRACT FROM AUTHOR]

The pulmonary valve (PV) has historically been ignored by imaging studies. Disorders of the PV encountered in adult cardiac patients are increasingly encountered due to advanced care of patients with congenital heart disease and associated PV diseases. Despite advances in PV imaging, multiple challenges remain when it comes to obtaining high quality PV images. While 2D TTE is the usual initial imaging tool for PV, excellent views of the PV annulus and its one or two leaflets are obtained in less than half of patients. The 3D echocardiography en face view allows all three leaflets to be evaluated concurrently, as well as assessments of the RV outflow tract and main pulmonary artery, which has improved quantitative assessment of PV diseases. Increasing image quality and experience with live/real time 3D TEE amplifies its utility in accurate evaluation and helps guide and monitor successful percutaneous PV interventions. 2D TTE remains the first line diagnostic tool; however, 3D TTE and 3D TEE provide better image quality which increases diagnostic accuracy and guidance to therapy. In this review article, we stress improvement in 3D echocardiography and its role in diagnostic and therapeutic options for PV diseases. [ABSTRACT FROM AUTHOR]

Valvular regurgitation represents an important cause of cardiovascular morbidity and mortality. Imaging is pivotal in the evaluation of native valve regurgitation and echocardiography is the primary imaging modality for this purpose. The imaging assessment of valvular regurgitation should integrate quantification of the regurgitation, assessment of the valve anatomy and function, and the consequences of valvular disease on cardiac chambers. In clinical practice, the management of patients with valvular regurgitation largely relies on the results of imaging. It is crucial to provide standards that aim at establishing a baseline list of measurements to be performed when assessing native valve regurgitation. The present document aims to present clinical guidance for the multi-modality imaging assessment of native valvular regurgitation. [ABSTRACT FROM AUTHOR]

Dysfunctional right ventricular outflow tracts after a repair for tetralogy of Fallot using a transannular patch offer limited nonsurgical opportunities due to their large dimensions. A discrete subannular narrowing between a dilated right ventricle and the enlarged pulmonary trunk was a potential anatomical target for the creation of a landing zone using a prestent in a young male with severe pulmonary regurgitation and moderate stenosis. Asymmetric expansion of the prestent in the angulated outflow tract led to distal stent embolization that was stabilized by another telescoping stent before successful valve implantation. This manuscript details the unique challenges posed by a dilated regurgitant outflow tract for implanting a pulmonary valve. [ABSTRACT FROM AUTHOR]

Right ventricular (RV) systolic function is an essential but neglected component in cardiac evaluation, and its importance to the contribution to overall cardiac function is undermined. It is not only sensitive to the effect of left heart valve disease but is also more sensitive to changes in pressure overload than the left ventricle. Pulmonary Hypertension is the common and well-recognized complication of RV systolic dysfunction. It is also the leading cause of pulmonary valve disease and right ventricular dysfunction. Patients with a high pulmonary artery pressure (PAP) and a low RV ejection fraction have a seven-fold higher risk of death than heart failure patients with a normal PAP and RV ejection fraction. Furthermore, it is an independent predictor of survival in these patients. In this review, we examine the association of right ventricular systolic function with Pulmonary Hypertension by focusing on various pathological and clinical manifestations while assessing their impact. We also explore new 2022 ESC/ERS guidelines for diagnosing and treating right ventricular dysfunction in Pulmonary Hypertension. [ABSTRACT FROM AUTHOR]

Background and Objectives : Infective endocarditis (IE) involving the native pulmonary valve (PV) is extremely rare, with no data in Indian literature. The objective of this communication is to describe the clinical and diagnostic characteristics, underlying risk factors, microbiological features, and management of PVIE. Methods : This is a retrospective analysis of 8 cases of PVIE managed in a tertiary care center from 1992 to 2020. Results : PVIE was observed in 8 patients with underlying congenital cardiac malformation (Group A, 6 Patients) and in patients with central venous catheter (Group B, 2 patients). All the patients had prolonged febrile illness accompanied by right heart failure 4 (50%), septic pulmonary emboli 2 (25%), and pulmonary regurgitation 3 (37.5%). Trans-thoracic echocardiography demonstrated the vegetations, whereas computed tomography of chest diagnosed pulmonary emboli in 2 (25%), and pulmonary artery aneurysm in 1 (12.5%) patient. The early mortality was extremely high (5, 62.5%). Delayed diagnosis, fulminant septicemia, and multi-organ failure resulted in unfavorable outcomes. Conclusions : IE of the native PV is a rare and potentially lethal illness. Diagnosis should be considered in any febrile patient with an underlying congenital defect, central venous line, bacteremia, and comorbidities. Multi-modality imaging should be utilized to enhance the diagnostic yield and detect complications promptly. [ABSTRACT FROM AUTHOR]

Mitral valve disease (MVD) is an important and most frequently acquired heart disease found in dogs. MVD is classified into different stages according to its severity. There is a challenge in differentiation between asymptomatic and symptomatic stages of the MVD. Moreover, pulmonary hypertension (PH) is a common complication in dogs affected by MVD. In clinical practice, there are also some limitations to identify PH. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) is a technique that can characterize specific patterns of peptide mass called peptide barcodes from various samples. Besides, in combination with liquid chromatography-tandem mass spectrometry (LC-MS/MS), potential peptide sequences associated with specific conditions could be identified. The present study aimed to use MALDI-TOF coupled with LC-MS/MS to characterize specific peptide barcodes and potential peptide candidates in serum samples from healthy dogs, dogs with MVD stage B (MVD B, asymptomatic stage), MVD stage C (MVD C, symptomatic stage), MVD stage B with PH (MVD B PH), and MVD stage C with PH (MVD C PH). Discrete clusters of the 5 sample groups were identified by 3D plot analysis. Peptide barcodes also revealed differences in peptide patterns among the 5 groups. Six amino acid sequences of peptide candidates at 1,225.60, 1,363.85, 1,688.71, 1789.52, 2020.21, and 2156.42 Da were identified as part of the proteins CLCN1, CLUL1, EDNRA, PTEN, SLC39A7, and CLN6, respectively. The network interactions between these discovered proteins and common cardiovascular drugs were also investigated. These results demonstrate that MALDI-TOF MS has promise as an optional technique for diagnosing dogs affected by asymptomatic and symptomatic stages of MVD with and without PH. Further studies are required to identify peptide barcodes in dogs with other diseases to create peptide barcode databases in veterinary medicine before using this method as a novel diagnostic tool in the future. [ABSTRACT FROM AUTHOR]

Ventricular tachycardia and premature ventricular complexes (PVCs) arising from right ventricular outflow tract (RVOT) are the most common type of ventricular arrhythmias (VAs) in patients without structural heart disease. Radiofrequency ablation is now the gold standard of treatment in this setting due to high efficacy rates and optimal safety profile. During the last few years, the pulmonary valve (PV) and the pulmonary artery (PA) have attracted much attention as reliable sites of origin of RVOT‐type arrhythmias. In the mean while intracardiac echocardiogram (ICE) has undoubtedly improved our understanding of the cardiac anatomy. Aim of this paper is to provide an illustrated step‐by‐step guide on how to use ICE with the CARTOSOUND module to visualize and reconstruct 3D shell of the RV, the PV, as well of other contiguous anatomical structures (i.e., the aortic valve and coronary arteries) to perform aware and safe ablation in this region. [ABSTRACT FROM AUTHOR]

Objectives: Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects. Aim of our study was to evaluate the effect of concomitant PVR with TOF repair on right ventricular (RV) parameters, cardiopulmonary exercise capacity, and bioprosthetic valve durability at mid-term. Materials and Methods: Between January 2013 and August 2018, 37 adolescents and adults with TOF who had hypoplastic pulmonary annulus underwent concomitant TOF repair with PVR at our institute. We retrospectively collected the data from the hospital records including follow-up. Results: Mean age of the patients was 18.48 ± 7.53 years. Bioprosthetic valve size ranged from 19 mm to 25 mm. There was no early or late mortality. No patient had developed significant perioperative complications. At a mean follow-up of 53.3 ± 16.4 months, there was no significant change in mean QRS duration, RV function, RV end-systolic and end-diastolic dimensions, RV myocardial performance index, and functional status (including NYHA class and 6-min walk test) compared to at-discharge values. Four patients developed prosthetic valve degeneration with mild PR and without significant increase in gradient. Conclusion: Concomitant PVR with TOF repair in adult provides excellent mid-term outcome, with a minimal rate of pulmonary valve degeneration. It not only eases the early postoperative course but also preserves the RV function as well as functional status at mid-term. [ABSTRACT FROM AUTHOR]

Severe tricuspid regurgitation (TR) is often associated with significant morbidity and mortality. It is a relatively common valvular disease, which can be the result of structural abnormalities of any anatomical part of the tricuspid valve. Severe TR is associated with congestive heart failure and hemodynamic impairment, resulting in high mortality when repaired by elective surgery. Given the clinical importance of severe symptomatic TR, significant efforts are being made to establish several effective transcatheter solutions that would avoid the need for high-risk tricuspid valve surgery. If left untreated, patients with severe TR face a poor prognosis. Percutaneous transcatheter therapeutic procedures have expanded the treatment options for patients with heart valve disease. Percutaneous interventional therapy for aortic, mitral, and pulmonary valve diseases is well established; however, catheter-based approaches to tricuspid regurgitation (TR) are still in the early stages of development. Transcatheter tricuspid valve intervention has recently emerged as a viable alternative to surgery for patients with symptomatic severe tricuspid regurgitation. Although usually performed on a compassionate basis, expansion of its use as an elective option in patients with severe atrial functional tricuspid regurgitation is now being investigated. Caval valve implantation (CAVI) can reduce venous regurgitation and improve right heart hemodynamics. TricValve is a transcatheter system of 2 self-expanding valves made of bovine pericardial tissue mounted on nitinol stents intended for placement in the superior and inferior vena cava. Initial studies showed an increase in the quality of life, a decrease in the number of hospitalizations and the absence of signs of perforation or structural damage of valvular stents after 6 months of follow-up in patients with symptomatic functional TR. We are pleased to report the inaugural utilization of the TricValve system in Croatia, as applied to a 66-year-old patient who had experienced recurrent hospital admissions due to severe TR and accompanying symptoms of right-sided heart failure, including edema and ascites. We also aim to highlight the unique aspects and complexities of the nurses role in the implementation of this novel heart failure treatment technology [ABSTRACT FROM AUTHOR]

Background: Isolated pulmonary valve endocarditis (IPE) is rare, accounting for 1.5-2% of all cases of infective endocarditis. Herein, we describe a case of isolated pulmonary valve endocarditis with rapid progression in a 28-year-old male. Unlike most patients reported previously who were cured with only anti-infective therapy, without surgery at an early stage, multiple complications occurred in this patient in less than 2 weeks.Case Presentation: The patient was diagnosed with pulmonary valve endocarditis with blood cultures showing Staphylococcus aureus and echocardiography revealing 2 masses (measuring 14*13 mm、11*16 mm in size). Only 12 days later, acute massive pulmonary embolism occurred. Then, repeated echocardiography revealed multiple masses attached to the pulmonary valve with severe pulmonary insufficiency and the possibility of pulmonary valve destruction. Finally, pulmonary valve replacement, vegetation removal, and right pulmonary thromboendarterectomy together with resection of the middle and lower lobes of the right lung were performed.Conclusions: The role of surgery at an early stage might need to be reconsidered, and it may be viable to combine medical and surgical approaches. [ABSTRACT FROM AUTHOR]

Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups – polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up. [ABSTRACT FROM AUTHOR]

In the past decade, balloon-expandable percutaneous pulmonary valves have been developed and applied in clinical practice. However, all the existing products of pulmonary artery interventional valves in the market have a straight structure design, and they require a preset support frame and balloon expansion. This shape design of the valve limits the application range. In addition, the age of the population with pulmonary artery disease is generally low, and the existing products cannot meet the needs of anti-calcification properties and valve material durability. In this study, through optimization of the support frame and leaflet design, a self-expanding pulmonary valve product with a double bell-shaped frame was designed to improve the match of the valve and the implantation site. A loading and deployment study showed that the biomaterial of the valve was not damaged after being compressed. Pulsatile flow and fatigue in vitro tests showed that the fabricated pulmonary valve met the hydrodynamic requirements after 2 × 108 accelerated fatigue cycles. The safety and efficacy of the pulmonary valve product were demonstrated in studies of pulmonary valve implantation in 11 pigs. Angiography and echocardiography showed that the pulmonary valves were implanted in a good position, and they had normal closure and acceptable valvular regurgitation. The 180 days' implantation results showed that the calcium content was 0.31–1.39 mg/g in the anti-calcification treatment group, which was significantly lower than that in the control valve without anti-calcification treatment (16.69 mg/g). Our new interventional pulmonary valve product was ready for clinical trials and product registration. [ABSTRACT FROM AUTHOR]

A fetal echocardiogram noted valvar pulmonary atresia vs critical pulmonary valve stenosis, confluent pulmonary arteries, patent ductus arteriosus, and an apical muscular ventricular septal defect vs coronary sinusoid. Postnatal echocardiogram documented an absent pulmonary valve, coronary artery fistula to the right ventricle, confluent pulmonary arteries, and a patent ductus arteriosus. Catheterization confirmed the postnatal echocardiographic findings except there was no antegrade flow from the right ventricle to the pulmonary arteries. Close evaluation of all imaging modalities revealed the final diagnosis of absent pulmonary valve with a double‐chambered right ventricle with no egress from the right ventricle to pulmonary artery. [ABSTRACT FROM AUTHOR]

Objective: This study sought to investigate the variation of right heart structure pre‐ and post‐operation as risk factors for moderate to severe pulmonary regurgitation (PR) after repaired Tetralogy of Fallot and the best "cutoff" values for the transannular patch (TAP). Methods: We collected surgical, echocardiographic, and computed tomographic data of Teralogy of Fallot (TOF) patients over two years and calculated z‐score values based on the echocardiographic data. Based on the PR level after follow‐up, the patients were divided into two groups, trivial to mild PR and moderate to severe PR. A multivariate logistic regression analysis was performed, and the receiver operating characteristic curve analysis was used to find the best "cutoff" value for risk factors. Results: A total of 104 TOF patients were included in our cohort study. From the multivariate analysis, correction strategy (P =.002), difference in zRVOT (OR 1.974, 95% CI 1.354 to 2.878, P <.0001), and zPVA (OR 3.605, 95% CI 1.980 to 6.562, P <.0001) were the significant risk factors for moderate to severe PR. The "cutoff" value for the difference in zPVA that could predict moderate to severe PR in the TAP group was 3, and the optimal "cutoff" value for TAP was −1.4. Conclusions: The TAP is a risk factor for significant PR after surgery. We recommend the optimal "cutoff" value for TAP is −1.4 calculated using Shan‐Shan Wang's data set. During the procedure, to limit the RVOT resection and restrict the enlargement of pulmonary annulus within a variation of z‐score as 3 would reduce significant PR. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. Methods: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation Results: There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81-249] vs. 150 [82-260] mL/m²) and end systolic volumes indexed for BSA (RVESVi) (75 [20-186] vs. 76 [39-189] mL/m²) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56-137] vs. 81 [57-128] mL/m² and LV end-systolic volume index 34 [23-68] vs. 35 [18-61] mL/m²). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥20 mL/m²) in twenty patients (24%) from 154 mL/m² (87-237) to 184 mL/m² (128-260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m² (57-98) to 85 ml/m² (72-105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation. Conclusion: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair. [ABSTRACT FROM AUTHOR]

A case study of 9-month-old 6.2-kg sexually intact male Cavalier King Charles Spaniel with pulmonary valve stenosis was referred for cardiac consultation regarding treatment with balloon valvuloplasty is presented. Topics include Transthoracic echocardiography was repeated at facility by a cardiology resident (AVC), and physical examination of the dog revealed a regularly irregular heart rhythm.

Patients after the Ross procedure are at risk for right (RV) and left ventricular (LV) dysfunction due to neo-aortic and pulmonary dysfunction. While neo-aortic root dilatation has been related to LV dysfunction, the potential contributions of aortic stiffness and ventricular interactions have not been evaluated. Patients status post Ross procedure up to age 18 years with cardiac magnetic resonance (CMR) exam from 2007 to 2018 were retrospectively reviewed. Aortic pulse wave velocity (PWV) was calculated from phase contrast and angiogram images. RV and LV peak global longitudinal (GLS) and circumferential strain (GCS) were measured using tissue tracking software. Multivariable regression was performed for variables associated with parameters of LV function. In 58 patients (median age 20.5 years at CMR exam), male gender, longer time since Ross procedure, aortic root dilatation, and lower RV ejection fraction (EF) were associated with decreased LV EF. There was no association with LV late gadolinium enhancement or neo-aortic or conduit regurgitation. LV GCS and GLS also correlated with RV GCS, RV GLS and PWV. In multivariable analysis, the relation of RV and LV systolic function, but not aortic measurements, remained significant. In conclusion, in long-term follow-up after pediatric Ross procedure, RV function rather than aortic root size or aortic stiffness most closely relates to LV function. Ventricular interactions may impact decision-making on timing of conduit intervention, which could differ from established criteria in populations with only aortic or pulmonary valve disease. Further study is warranted to evaluate possible association with clinical outcome. [ABSTRACT FROM AUTHOR]

Background Constrictive pericarditis (CP) is a pathological condition of the pericardium, resulting from fibrosis, scarring, and calcification of the pericardium. Other conditions have been reported to mimic 'constrictive physiology' despite the presence of an intact pericardium. However, there has been no report of pulmonary regurgitation (PR) mimicking the haemodynamic characteristics of CP. Case summary A 51-year-old woman was admitted to our institute because of severe right-sided heart failure. Transthoracic echocardiography revealed severe PR concomitant with significant dilatation of the right-sided heart. Septal bounce and the respiratory reciprocation of the transmitral and transtricuspid inflow velocities were also observed, indicating exacerbated ventricular interdependence. Cardiac catheter examination demonstrated elevated right atrial pressure with a prominent y descent, dip, and plateau waveform in the right ventricular pressure, and equalization of the diastolic pressure of all cardiac chambers, which are quite consistent with CP. On surgical inspection, however, there was no pericardial thickening or adhesion, indicating no obvious signs of CP. Discussion Pericardial constriction results from the relative relationship between intrapericardial volume and pericardial reserve. When the intrapericardial volume exceeds the physiological limit, the cardiac chambers compete with each other in a fixed pericardial space. In this case, prominent dilation of the right-sided chambers caused by severe PR resulted in overstretching of the pericardium above the pericardial reserve, which led to a characteristic haemodynamic picture that resembled CP. Thus, it is important to recognize the diagnostic pitfall in the preoperative evaluation of a 'CP mimic physiology'. [ABSTRACT FROM AUTHOR]

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]

Background: Pulmonary endarteritis is a rare clinical phenomenon with congenital heart that can potentially lead to major complications.Case Presentation: We report a 47-year-old man with pulmonary endarteritis. This patient presented with hypertension, chest pain and a previous history of pulmonary valve disease during childhood. Also, eight-months prior, he was hospitalized with dyspnea (Functional Class III), cough, phlegm, and night sweats without fever. Echocardiographic diagnosis in the first transtransthoracic echocardiography (TTE) was intense pulmonary valve stenosis (PVS) an, thus, the pulmonary valve vegetation and PVS, established by transesophageal echocardiography (TEE). He was referred for surgery after 1 weeks of intravenous antibiotic therapy for removal of the vegetation.Conclusions: Finally he was asymptomatic at 3-months of follow-up and was clinically in good condition. Therefore, the detection of infective endocarditis of the lung valve must not lengthy be prolonged. [ABSTRACT FROM AUTHOR]

Quadricuspid pulmonary valve is a rare entity that can be associated with pulmonary artery aneurysm. It is usually asymptomatic and diagnosed incidentally. Association with other congenital or acquired heart diseases has been reported. Echocardiography and gated CT angiography can provide useful functional and anatomic information that can help in the diagnosis and management. [ABSTRACT FROM AUTHOR]

• Development of a 3D dynamic computational bi-ventricle model. • Effects of pulmonary regurgitation on cardiac motion and function. • Dilation of right ventricle and high myofibre stress agree with the literature. • Interaction of left ventricle and right ventricle and motion of the septum. Background and objective: Assessing the severity of pulmonary regurgitation (PR) and identifying optimal clinically relevant indicators for its treatment is crucial, yet standards for quantifying PR remain unclear in clinical practice. Computational modelling of the heart is in the process of providing valuable insights and information for cardiovascular physiology research. However, the advancements of finite element computational models have not been widely applied to simulate cardiac outputs in patients with PR. Furthermore, a computational model that incorporates both the left ventricle (LV) and right ventricle (RV) can be valuable in assessing the relationship between left and right ventricular morphometry and septal motion in PR patients. To enhance our understanding of the effect of PR on cardiac functions and mechanical behaviour, we developed a human bi-ventricle model to simulate five cases with varying degrees of PR severity. Methods: This bi-ventricle model was built using a patient-specific geometry and a widely used myofibre architecture. The myocardial material properties were described by a hyperelastic passive constitutive law and a modified time-varying elastance active tension model. To simulate realistic cardiac functions and the dysfunction of the pulmonary valve in PR disease cases, open-loop lumped parameter models representing systemic and pulmonary circulatory systems were designed. Results: In the baseline case, pressures in the aorta and main pulmonary artery and ejection fractions of both the LV and RV were within normal physiological ranges reported in the literature. The end-diastolic volume (EDV) of the RV under varying degrees of PR was comparable to the reported cardiac magnetic resonance imaging data. Moreover, RV dilation and interventricular septum motion from the baseline to the PR cases were clearly observed through the long-axis and short-axis views of the bi-ventricle geometry. The RV EDV in the severe PR case increased by 50.3% compared to the baseline case, while the LV EDV decreased by 18.1%. The motion of the interventricular septum was consistent with the literature. Furthermore, ejection fractions of both the LV and RV decreased as PR became severe, with LV ejection fraction decreasing from 60.5% at baseline to 56.3% in the severe case and RV ejection fraction decreasing from 51.8% to 46.8%. Additionally, the average myofibre stress of the RV wall at end-diastole significantly increased due to PR, from 2.7 ± 12.1 kPa at baseline to 10.9 ± 26.5 kPa in the severe case. The average myofibre stress of the LV wall at end-diastole increased from 3.7 ± 18.1 kPa to 4.3 ± 20.3 kPa. Conclusions: This study established a foundation for the computational modelling of PR. The simulated results showed that severe PR leads to reduced cardiac outputs in both the LV and RV, clearly observable septum motion, and a significant increase in the average myofibre stress in the RV wall. These findings demonstrate the potential of the model for further exploration of PR. [ABSTRACT FROM AUTHOR]

Marked aneurysmal dilation of the central and branch pulmonary arteries in utero in patients with tetralogy of Fallot with absent pulmonary valve can often exhibit extrinsic compression of the trachea and bronchi. The major morbidity in these patients remains postoperative ventilation issues. This case report highlights the role of intraoperative bronchoscopy in providing guidance for obtaining optimal bronchial decompression that was achieved by an initial pulmonary arteriopexy followed by an aortopexy. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome is a rare congenital heart disease characterized by absent or rudimentary pulmonary valve leaflets and hypoplastic pulmonary annulus. The most common variant, associated with tetralogy of Fallot, implies dilatation of pulmonary branches and usually absent ductus arteriosus. Rarely, absent pulmonary valve occurs with intact ventricular septum: pulmonary branches are normally sized or mildly dilated and ductus arteriosus is usually patent. The rarest type is associated with intact ventricular septum and tricuspid atresia. A close connection has been raised between pulmonary regurgitation and development of tricuspid valve. We describe a case of prenatal diagnosed absent pulmonary valve with intact ventricular septum and patent ductus arteriosus in which severe pulmonary regurgitation caused reversible functional atresia of tricuspid valve. Postnatally, satisfactory biventricular circulation was obtained with inotropic support for a few days while ductus arteriosus closed spontaneously. At a 3‐year follow up the child is asymptomatic without therapy. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation (PR) is a frequent complication after repair of congenital heart disease. Three different GRK isoforms (GRK2, GRK5, and GRK3) and two β-adrenoceptors (β1-AR and β2-AR) are present in peripheral blood mononuclear cells (PBMC) and their expression changes as a consequence of the hemodynamic and neurohumoral alterations that occur in some cardiovascular diseases. Therefore, they could be useful as biomarkers in PR. A prospective study was conducted to describe the expression (TaqMan Gene Expression Assays) of β-ARs and GRKs in PBMC isolated (Ficoll® gradient) from patients with severe PR before and after pulmonary valve replacement and establish if this expression correlates to clinical status. 23 patients with severe PR were included and compared with 22 healthy volunteers (controls). PR patients before the PVR had a significantly lower expression of β2-AR (513.8 ± 261.2 mRNA copies) vs. controls (812.5 ± 497.2 mRNA copies), so as GRK2 expression (503.4 ± 364.9 copies vs. 858.1 ± 380.3 mRNA copies). The expression of β2-AR and GRK2 significantly decreases in symptomatic and asymptomatic patients, as well as in patients under treatment with beta-blockers and non-treated patients. The expression of β2-AR and GRK2 in PR patients recovers the normal values after pulmonary valve replacement (754,8 ± 77,1 and 897,8 ± 87,4 copies, respectively). Therefore, changes in the expression of β2-AR and GRK2 in PBMC of PR patients, could be considered as potential biomarkers to determine clinical decisions. [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: Valve implantation provides a reversible effective therapy in a selected group of emphysema patients. Knowing predictors for successful therapy, the rate of treatment failure has decreased. Some patients, however, do not benefit, so that the valves may have to be removed. Objectives: To assess implant-related events, complications during valve removal, and clinical outcome after endoscopic procedure. Methods: The data of 76 consecutive emphysema patients who underwent valve removal > 6 months since implantation were collected. Results: Seventy-six patients (mean age 62 years, 54% male) underwent removal of all valves after a mean time of 624 days (193–3,043 days) since implantation. Granulation tissue was observed in 39.5% (30/76) and significant secretion in 34.2% (26/76). In 5.3% (4/76), valve removal was complicated requiring another bronchoscopy in 2 of them. In 5.3% (4/76) of the patients, one valve could not be removed and remained in situ. Bleeding requiring intervention occurred in 3.9% (3/76) during valve removal. Following bronchoscopy, there was a need for antibiotics in 34.2% (26/76), glucocorticosteroids in 1.3% (1/76), and both in 6.6% (5/76) due to productive cough or chronic obstructive pulmonary disease (COPD) exacerbation. Due to respiratory failure, invasive ventilation or noninvasive ventilation was necessary in 2.6% (2/76) and 6.6% (5/76), respectively, following procedure. No statistical significant change in lung function was observed following valve removal. Conclusions: Valve removal after > 6 months since implantation is feasible and associated with an acceptable safety profile. However, close monitoring of these patients with limited pulmonary reserve is recommended with particular attention to COPD exacerbations and respiratory failure. [ABSTRACT FROM AUTHOR]