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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]

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]

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]