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Journal of Clinical Medicine May 2024Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and... (Review)
Review
Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR.
PubMed: 38731211
DOI: 10.3390/jcm13092682 -
Annals of Cardiac Anaesthesia Jan 2024The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other...
The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography.
Topics: Humans; Female; Aortic Valve; Middle Aged; Echocardiography, Transesophageal; Aortic Valve Insufficiency; Heart Defects, Congenital
PubMed: 38722121
DOI: 10.4103/aca.aca_110_23 -
BMJ Open May 2024The aim of the study was to evaluate mortality and morbidity outcomes following open-heart isolated tricuspid valve surgery (TVSx) with medium to long-term follow-up.
Morbidity and mortality outcomes of patients requiring isolated tricuspid valve surgery: a retrospective cohort study of 537 patients in New South Wales between 2002 and 2018.
OBJECTIVES
The aim of the study was to evaluate mortality and morbidity outcomes following open-heart isolated tricuspid valve surgery (TVSx) with medium to long-term follow-up.
DESIGN
Retrospective cohort study.
SETTING
New South Wales public and private hospital admissions between 1 January 2002 and 30 June 2018.
PARTICIPANTS
A total of 537 patients underwent open isolated TVSx during the study period.
PRIMARY AND SECONDARY OUTCOME MEASURES
Primary outcome was all-cause mortality tracked from the death registry to 31 December 2018. Secondary morbidity outcomes, including admission for congestive cardiac failure (CCF), new atrial fibrillation (AF), infective endocarditis (IE), pulmonary embolism (PE) and insertion of a permanent pacemaker (PPM) or implantable cardioverter-defibrillator (ICD), were tracked from the Admitted Patient Data Collection database. Independent mortality associations were determined using the Cox regression method.
RESULTS
A total of 537 patients underwent open isolated TVSx (46% male): median age (IQR) was 63.5 years (43.9-73.8 years) with median length of stay of 16 days (10-31 days). Main cardiovascular comorbidities were AF (54%) and CCF (42%); 67% had rheumatic tricuspid valve. In-hospital and total mortality were 7.4% and 39.3%, respectively (mean follow-up: 4.8 years). Cause-specific deaths were evenly split between cardiovascular and non-cardiovascular causes. Predictors of mortality included a history of CCF (HR=1.78, 95% CI 1.33 to 2.38, p<0.001) and chronic pulmonary disease (HR=2.66, 95% CI 1.63 to 4.33, p<0.001). In-hospital PPM rate was 10.0%. At 180 days, 53 (9.9%) patients were admitted for CCF, 25 (10.1%) had new AF, 7 (1.5%) had new IE and <1% had PE, post-discharge PPM or ICD insertion.
CONCLUSION
Open isolated TVSx carries significant mortality risk, with decompensated CCF and new AF the most common morbidities encountered after surgery. This report forms a benchmark to compare outcomes with newer percutaneous tricuspid interventions.
Topics: Humans; Male; Female; Middle Aged; Retrospective Studies; Aged; Tricuspid Valve; New South Wales; Adult; Postoperative Complications; Tricuspid Valve Insufficiency
PubMed: 38719314
DOI: 10.1136/bmjopen-2023-080804 -
The American Journal of Cardiology Jul 2024Transcatheter aortic valve replacement (TAVR) has emerged as an alternative treatment for patients with pure severe aortic regurgitation (PSAR) who are contraindicated...
Transcatheter aortic valve replacement (TAVR) has emerged as an alternative treatment for patients with pure severe aortic regurgitation (PSAR) who are contraindicated for surgery or have a high surgical risk. However, the therapeutic efficacy and safety of TAVR in low Society of Thoracic Surgeons (STS) score risk patients remain to be clarified. This study aimed to explore the feasibility of TAVR treatment in different STS-risk patients and to compare the adverse events between the groups. In this study, patients with PSAR who underwent TAVR at Zhongshan Hospital, Fudan University, China, during the inclusion period were included and categorized into 3 groups based on STS scores. The baseline data, imaging results, and follow-up data of the patients were documented. Therefore, of 75 TAVR patients, 38 (50.7%) were categorized as low risk (STS <4), and 37 (49.3%) patients were categorized as intermediate and high risk (STS ≥4). Compared with patients at intermediate and high risk, those in the low-risk group were younger, had a lower body mass index, had a lower prevalence of hypertension, chronic obstructive pulmonary disease, and previous percutaneous coronary intervention, and had better cardiac function (p all <0.05). In the hospital and at the 1-month follow-up, the degree of aortic regurgitation and cardiac function were significantly improved. No significant difference was found between the 2 groups in the hospital or during the 30-day follow-up. In conclusion, TAVR for PSAR in low-STS-risk patients is safe and efficient during 30 days of follow-up compared with intermediate- and high-STS-risk groups. TAVR for PSAR should not be limited to inoperable or STS-defined high-risk patients. Long-term follow-up is needed for further investigation.
Topics: Humans; Transcatheter Aortic Valve Replacement; Aortic Valve Insufficiency; Male; Female; Aged; Treatment Outcome; Severity of Illness Index; Risk Assessment; Retrospective Studies; China; Risk Factors; Follow-Up Studies; Aged, 80 and over; Time Factors
PubMed: 38703883
DOI: 10.1016/j.amjcard.2024.04.056 -
The American Journal of Emergency... Jun 2024The tricuspid regurgitation pressure gradient (TRPG) reflects the difference in pressure between the right ventricle and right atrium (ΔP). Its estimation by...
The tricuspid regurgitation pressure gradient (TRPG) reflects the difference in pressure between the right ventricle and right atrium (ΔP). Its estimation by echocardiography correlates well with that obtained using right-heart catheterization. An elevated TRPG is an important marker for identifying right ventricular dysfunction in both the acute and chronic settings. However, in the "wide-open" variant of TR, the TRPG counterintuitively falls. Failure to recognize this potential pitfall and underlying pathophysiology can cause underestimation of the severity of right ventricular dysfunction. This could lead to erroneous fluid tolerance assessments, and potentially harmful resuscitative and airway management strategies. In this manuscript, we illustrate the pathophysiology and potential pitfall of wide-open TR through a series of cases in which emergency physicians made the diagnosis using cardiac point-of-care ultrasound. To our knowledge, this clinical series is the first to demonstrate recognition of the paradoxically-low TRPG of wide-open TR, which guided appropriate management of critically ill patients in the emergency department.
Topics: Humans; Tricuspid Valve Insufficiency; Emergency Service, Hospital; Ventricular Dysfunction, Right; Echocardiography; Male; Female; Middle Aged; Aged; Point-of-Care Systems; Adult
PubMed: 38702221
DOI: 10.1016/j.ajem.2024.04.039 -
Surgical Case Reports May 2024Although the true prevalence and incidence are not clearly known, mycotic pulmonary artery aneurysm is a potentially devastating condition that leads to high mortality,...
BACKGROUND
Although the true prevalence and incidence are not clearly known, mycotic pulmonary artery aneurysm is a potentially devastating condition that leads to high mortality, over 60% if untreated. Among them, mycotic pulmonary artery pseudoaneurysm, which occurs in relatively central areas, has rarely been reported. We report an extremely rare case of a late complication with a mycotic pulmonary artery pseudoaneurysm, presumably due to infective endocarditis, in a 68-year-old woman 4 months after total arch replacement.
CASE PRESENTATION
A 68-year-old woman was referred to our department for 2 weeks with fever of unknown origin. She had a history of emergency total arch replacement for an acute type A aortic dissection 4 months earlier and chronic rheumatoid arthritis on monthly subcutaneous tocilizumab treatment for several years. Blood culture was positive for Enterococcus faecalis. Transthoracic and transesophageal echocardiography revealed a left ventricular ejection fraction of 58%, severe mitral regurgitation with a 15-mm diameter vegetation on the anterior mitral leaflet, and severe aortic insufficiency with string-like structures. Contrast computed tomography showed a focal saccular outpouching from the right pulmonary artery. On 18F-fluorodeoxyglucose (FDG) positron emission tomography, focal uptake of FDGs was observed along the same lesion of the pulmonary artery and ascending-arch graft. The patient eventually recovered after the surgical intervention of mitral and aortic valve replacement, re-total arch replacement, pulmonary artery repair, application of omental flap, and antibiotics without any evidence of re-infection after 1 year.
CONCLUSIONS
We report a successful surgical repair of mycotic pulmonary artery pseudoaneurysm 4 months after total arch replacement for acute type A aortic dissection. This report describes an effective treatment for an extremely rare postoperative condition.
PubMed: 38700748
DOI: 10.1186/s40792-024-01896-9 -
Arteriosclerosis, Thrombosis, and... Jul 2024Myxomatous valve disease (MVD) is the most common cause of mitral regurgitation, leading to impaired cardiac function and heart failure. MVD in a mouse model of Marfan...
BACKGROUND
Myxomatous valve disease (MVD) is the most common cause of mitral regurgitation, leading to impaired cardiac function and heart failure. MVD in a mouse model of Marfan syndrome includes valve leaflet thickening and progressive valve degeneration. However, the underlying mechanisms by which the disease progresses remain undefined.
METHODS
Mice with gene variant recapitulate histopathologic features of Marfan syndrome, and Wnt (Wingless-related integration site) signaling activity was detected in (T-cell factor/lymphoid enhancer factor-β-galactosidase) reporter mice. Single-cell RNA sequencing was performed from mitral valves of wild-type and mice at 1 month of age. Inhibition of Wnt signaling was achieved by conditional induction of the secreted Wnt inhibitor Dkk1 (Dickkopf-1) expression in periostin-expressing valve interstitial cells of -Cre; mice. Dietary doxycycline was administered for 1 month beginning with MVD initiation (1-month-old) or MVD progression (2-month-old). Histological evaluation and immunofluorescence for ECM (extracellular matrix) and immune cells were performed.
RESULTS
Wnt signaling is activated early in mitral valve disease progression, before immune cell infiltration in mice. Single-cell transcriptomics revealed similar mitral valve cell heterogeneity between wild-type and mice at 1 month of age. Wnt pathway genes were predominantly expressed in valve interstitial cells and valve endothelial cells of mice. Inhibition of Wnt signaling in mice at 1 month of age prevented the initiation of MVD as indicated by improved ECM remodeling and reduced valve leaflet thickness with decreased infiltrating macrophages. However, later, Wnt inhibition starting at 2 months did not prevent the progression of MVD.
CONCLUSIONS
Wnt signaling is involved in the initiation of mitral valve abnormalities and inflammation but is not responsible for later-stage valve disease progression once it has been initiated. Thus, Wnt signaling contributes to MVD progression in a time-dependent manner and provides a promising therapeutic target for the early treatment of congenital MVD in Marfan syndrome.
Topics: Animals; Wnt Signaling Pathway; Disease Progression; Fibrillin-1; Disease Models, Animal; Mitral Valve; Mice; Intercellular Signaling Peptides and Proteins; Mice, Transgenic; Marfan Syndrome; Mitral Valve Insufficiency; Mice, Inbred C57BL; Inflammation; Male; Female; Cell Adhesion Molecules; Adipokines
PubMed: 38660802
DOI: 10.1161/ATVBAHA.123.320388 -
Circulation May 2024Tricuspid valve disease is an often underrecognized clinical problem that is associated with significant morbidity and mortality. Unfortunately, patients will often... (Review)
Review
Tricuspid valve disease is an often underrecognized clinical problem that is associated with significant morbidity and mortality. Unfortunately, patients will often present late in their disease course with severe right-sided heart failure, pulmonary hypertension, and life-limiting symptoms that have few durable treatment options. Traditionally, the only treatment for tricuspid valve disease has been medical therapy or surgery; however, there have been increasing interest and success with the use of transcatheter tricuspid valve therapies over the past several years to treat patients with previously limited therapeutic options. The tricuspid valve is complex anatomically, lying adjacent to important anatomic structures such as the right coronary artery and the atrioventricular node, and is the passageway for permanent pacemaker leads into the right ventricle. In addition, the mechanism of tricuspid pathology varies widely between patients, which can be due to primary, secondary, or a combination of causes, meaning that it is not possible for 1 type of device to be suitable for treatment of all cases of tricuspid valve disease. To best visualize the pathology, several modalities of advanced cardiac imaging are often required, including transthoracic echocardiography, transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, to best visualize the pathology. This detailed imaging provides important information for choosing the ideal transcatheter treatment options for patients with tricuspid valve disease, taking into account the need for the lifetime management of the patient. This review highlights the important background, anatomic considerations, therapeutic options, and future directions with regard to treatment of tricuspid valve disease.
Topics: Humans; Tricuspid Valve; American Heart Association; United States; Heart Valve Diseases; Tricuspid Valve Insufficiency; Heart Valve Prosthesis Implantation
PubMed: 38660790
DOI: 10.1161/CIR.0000000000001232 -
International Journal of Cardiology Aug 2024In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment...
BACKGROUND
In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment using 2D and 4D MR flow measurements, with a focus on vortices.
METHODS
We retrospectively analysed 31 patients with congenital aortic valve disease, performing 2D and 4D MR flow measurements at the aortic valve, sinotubular junction (STJ), ascending aorta (AAo), and using midpulmonary artery measurements as a reference. We assessed percentage AR and net forward volumes, calculated linear correlations, and plotted Bland-Altman plots. Net forward flow at all aortic sites were correlated with the main pulmonary artery. Differences in AR between 2D and 4D flows were linked to vortices detected by 4D streamlines.
RESULTS
The best agreement in % AR between 2D and 4D flows was at the aortic valve (mean difference 4D2D -2.9%, limits of agreement 8.7% to -14.3%; r = 0.7). Correlations weakened at STJ and AAo. Vortices in the ascending aorta led to AR overestimation in 2D measurements. Net forward flow at the aortic valve by 4D flow correlated closer with main pulmonary artery than did 2D flow. (Mean difference for 2D and 4D MR flow 7.5 ml and 4.2 ml, respectively).
CONCLUSIONS
For congenital aortic valve disease, the most accurate AR quantification occurs at the aortic valve using 2D and 4D MR flow. Notably, vortices in the ascending aorta can result in AR overestimation with 2D MR flow.
Topics: Humans; Aortic Valve Insufficiency; Retrospective Studies; Female; Male; Adult; Aortic Valve; Magnetic Resonance Imaging, Cine; Blood Flow Velocity; Middle Aged; Adolescent; Young Adult; Child; Aortic Valve Disease
PubMed: 38653434
DOI: 10.1016/j.ijcard.2024.132084 -
Current Cardiology Reports Jun 2024Speckle-tracking echocardiography (STE) can assess myocardial motion in non-LV chambers-including assessment of left atrial (LA) and right ventricular (RV) strain. This... (Review)
Review
PURPOSE OF REVIEW
Speckle-tracking echocardiography (STE) can assess myocardial motion in non-LV chambers-including assessment of left atrial (LA) and right ventricular (RV) strain. This review seeks to highlight the diagnostic, prognostic, and clinical significance of these parameters in heart failure, atrial fibrillation (AF), diastolic dysfunction, pulmonary hypertension (PH), tricuspid regurgitation, and heart transplant recipients.
RECENT FINDINGS
Impaired LA strain reflects worse LV diastolic function in individuals with and without HF, and this is associated with decreased exercise capacity. Initiating treatments targeting these functional aspects may enhance exercise capacity and potentially prevent heart failure (HF). Impaired LA strain also identifies patients with a high risk of AF, and this recognition may lead to preventive strategies. Impaired RV strain has significant clinical and prognostic implications across various clinical scenarios, including HF, PH, tricuspid regurgitation, or in heart transplant recipients. STE should not be limited to the assessment of deformation of the LV myocardium. The use of LA and RV strain is supported by a substantial evidence base, and these parameters should be used more widely.
Topics: Humans; Echocardiography; Heart Atria; Atrial Fibrillation; Heart Transplantation; Hypertension, Pulmonary; Heart Failure; Tricuspid Valve Insufficiency; Prognosis; Heart Ventricles; Ventricular Dysfunction, Right; Ventricular Function, Right; Atrial Function, Left
PubMed: 38647564
DOI: 10.1007/s11886-024-02058-x