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Frontiers in Surgery 2023The pulmonary valve is the most frequently replaced cardiac valve in congenital heart diseases. Whether the valve alone or part of the right ventricular outflow tract... (Review)
Review
The pulmonary valve is the most frequently replaced cardiac valve in congenital heart diseases. Whether the valve alone or part of the right ventricular outflow tract have to be repaired or replaced depends on the specific pathological anatomy of the malformation. Once the decision to replace the pulmonary valve has been made, two options are available: the isolated transcatheter pulmonary valve replacement and the surgical implantation of a prosthetic valve either isolated or in combination with a procedure on the right ventricular outflow tract. In this paper, we will focus on the different past and present surgical options and present a new concept called "endogenous tissue restoration," a promising alternative to the hitherto existing implants. From a general point of view, neither the transcatheter nor the surgical valvular implants are magic bullets in the arsenal for the management of valvular diseases. Smaller valves have to be frequently replaced because of outgrowth of the patients, larger tissue valves may present late structural valve deterioration, while xenograft and homograft conduits may calcify and therefore become narrowed within unpredictable incidence and interval following implantation. Based on long-term research efforts combining the knowledge of supramolecular chemistry, electrospinning, and regenerative medicine, endogenous tissue restoration has emerged most recently as a promising option to create long-term functioning implants. This technology is appealing because following resorption of the polymer scaffold and timely replacement through autologous tissue, no foreign material remain at all in the cardiovascular system. Proof-of-concept studies as well as small first-in-man series have been completed and have demonstrated favorable anatomic and hemodynamic results, comparable to currently available implants in the short term. Based on the initial experience, important modifications to improve the pulmonary valve function have been initiated.
PubMed: 37334202
DOI: 10.3389/fsurg.2023.1185324 -
Journal of the Saudi Heart Association 2022Transcatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the...
INTRODUCTION & AIM OF WORK
Transcatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the predictors of outcome in interventions for treatment of duct-dependent right ventricular outflow tract obstruction with intact interventricular septum.
SUBJECTS & METHODS
68 cases with pulmonary atresia with intact interventricular septum (PA/IVS) and 50 cases with critical pulmonary stenosis (CPS), all younger than 3 months of age, were operated during the period of 10 years; excluding patients with tricuspid valve annulus Z-score smaller than -4, evidence of right ventricular-dependent coronary circulation or additional malformations.
RESULTS
Age, weight, body surface area as well as tricuspid & pulmonary valve Z-scores were significantly less in PA/IVS; right ventricular pressure was similar in both groups however procedural success and survival to hospital discharge was higher in the CPS group. Lower age, weight and body surface area were associated with procedural failure. Weight was the only predictor of procedural success; while weight and lower post-procedural right ventricular pressure were independent predictors for survival to hospital discharge. Post-procedural right ventricular pressure and length of stay were less in the CPS group. tricuspid and pulmonary valve annulus Z-scores were the only independent predictors of the post-procedural milrinone duration in PA/IVS.
CONCLUSION
We advocate for the use of larger balloon/pulmonary annulus ratio, to achieve a lower right ventricular pressure not fearing excessive pulmonary regurgitation that might be beneficial for right ventricular growth; and for the combination with ductal stenting in borderline or bipartite right ventricles.
PubMed: 35774726
DOI: 10.37616/2212-5043.1292 -
BMC Veterinary Research Jul 2020Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy currently described in humans and cats. It consists of a spongy myocardium characterized by...
BACKGROUND
Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy currently described in humans and cats. It consists of a spongy myocardium characterized by prominent trabeculation and deep recesses involving more than 50% of the ventricular thickness. We describe the clinical and pathological features of LVNC combined with tricuspid valve dysplasia, double-orifice tricuspid valve and severe pulmonary stenosis in a puppy. In addition, we briefly review the LVNC causes, pathogenesis, forms and current diagnostic criteria.
CASE PRESENTATION
A seven-week-old intact German Shorthaired Pointer-cross male was presented with a poor body condition, exercise intolerance and dyspnea. Clinical exam identified a bilateral systolic murmur (grade IV/VI over the right heart base and grade III/VI over the left heart base). Echocardiography identified tricuspid valve dysplasia, mild mitral regurgitation, and severe pulmonic stenosis with a trans-valvar systolic pressure gradient of 106 mmHg. Left ventricular noncompaction was diagnosed by necropsy and further confirmed histopathologically by the presence of two distinct myocardial layers: an inner noncompacted zone covering more than 50% of ventricular thickness containing prominent trabeculation and deep recesses, and an outer zone of compact myocardium.
CONCLUSIONS
This is the first case describing LVNC in a canine patient, supporting the introduction of this form of heart disease as a differential diagnosis for cardiomyopathies in juvenile and adult dogs.
Topics: Animals; Cardiomyopathies; Dog Diseases; Dogs; Echocardiography; Heart Defects, Congenital; Heart Ventricles; Male; Pulmonary Valve Stenosis; Tricuspid Valve
PubMed: 32703195
DOI: 10.1186/s12917-020-02480-7 -
Cureus Aug 2021Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder characterized by infantile hypercalcemia, short stature, a varying degree of...
Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder characterized by infantile hypercalcemia, short stature, a varying degree of mental retardation, elfin-like facial features, and cardiovascular abnormalities, including systemic hypertension, aortic hypoplasia, coarctation of the aorta, and valvular heart disease (aortic and pulmonic stenosis, mitral valve prolapsed or bicuspid aortic valve). It is also characterized by friendly and outgoing personality. The majority of WS cases are sporadic, while few are familial. Both sporadic and familial cases are due to deletion of chromosome 7 (7q11.23). Herein, we present an autopsy case of a 16-day-old male infant born to a 25-year-old mother with a history of William syndrome. Prenatal echocardiogram showed supravalvular aortic stenosis and pulmonary stenosis. The postnatal course was complicated by feeding difficulties and desaturation. Gross autopsy findings included generalized edema, macrocephaly with short neck, and multiple facial anomalies (mandibular hypoplasia, depressed nasal bridge, long philtrum, ear malformation, and wide mouth). The heart was hypertrophied with obstructed ventricles and rudimentary, hypoplastic aortic root. An enlarged, dilated, and tortuous left ureter was a unique finding to this case, in addition to variation in the renal arteries' size and an small bowel outpouching located 33 cm from the ileocecal valve. Cytogenetic analysis revealed deletion of chromosome 7 (7q11.23). In conclusion, majority of WS cases are sporadic, and few are familial and are inherited as autosomal dominant.
PubMed: 34540437
DOI: 10.7759/cureus.17210 -
Medicina (Kaunas, Lithuania) Aug 2022Background and Objectives: Since the first transcatheter aortic valve implantation (TAVI) procedure was performed in 2002, advances in technology and refinement of the...
Background and Objectives: Since the first transcatheter aortic valve implantation (TAVI) procedure was performed in 2002, advances in technology and refinement of the method have led to its widespread use in patients with severe aortic stenosis (AS) and high surgical risk. We aim to identify the impact of TAVI on the clinical and functional status of patients with severe AS at the one-month follow-up and to identify potential predictors associated with the evolution of pulmonary hypertension (PH) in this category of patients. Materials and Methods: We conducted a prospective study which included 86 patients diagnosed with severe AS undergoing TAVI treatment. We analyzed demographics, clinical and echocardiographic parameters associated with AS and PH both at enrolment and at the 30-day follow-up. Results: In our study, the decrease of EUROSCORE II score (p < 0.001), improvement of angina (p < 0.001) and fatigue (p < 0.001) as clinical benefits as well as a reduction in NYHA functional class in patients with heart failure (p < 0.001) are prognostic predictors with statistical value. Regression of left ventricular hypertrophy (p = 0.001), increase in the left ventricle ejection fraction (p = 0.007) and improvement of diastolic dysfunction (p < 0.001) are echocardiographic parameters with a prognostic role in patients with severe AS undergoing TAVI. The pulmonary artery acceleration time (PAAT) (p < 0.001), tricuspid annular plane systolic excursion (TAPSE) (p = 0.020), pulmonary arterial systolic pressure (PASP) (p < 0.001) and the TAPSE/PASP ratio (p < 0.001) are statistically significant echocardiographic parameters in our study that assess both PH and its associated prognosis in patients undergoing TAVI. Conclusions: PAAT, TAPSE, PASP and the TAPSE/PASP ratio are independent predictors that allow the assessment of PH and its prognostic implications post-TAVI.
Topics: Humans; Hypertension, Pulmonary; Prognosis; Prospective Studies; Transcatheter Aortic Valve Replacement
PubMed: 36143859
DOI: 10.3390/medicina58091182 -
Structural Heart : the Journal of the... Jun 2022Patients with paradoxical low-flow low-gradient aortic stenosis (pLFLG-AS) have high mortality and high degree of TAVR futility. Computed tomography (CT) enables...
BACKGROUND
Patients with paradoxical low-flow low-gradient aortic stenosis (pLFLG-AS) have high mortality and high degree of TAVR futility. Computed tomography (CT) enables accurate simultaneous right ventricular (RV) and parenchymal lung disease evaluation which may provide useful objective markers of AS severity, concomitant pulmonary comorbidities, and transcatheter aortic valve replacement (TAVR) improvement. However, the prevalence of RV dysfunction and its association with pulmonary disease in pLFLG-AS is unknown. The study objective was to test the hypothesis that pLFLG-AS patients undergoing TAVR have decreased RV function without significant parenchymal lung disease.
METHODS
Between August 2016 and March 2020, 194 consecutive AS patients completed high-resolution computed tomography (CT) imaging for TAVR evaluation. Subjects were stratified based on echocardiographic criteria as the study group, pLFLG (n=27), and two consecutive control groups: classic severe, normal-flow, high-gradient (n=27) and normal-flow, low-gradient (NFLG) (n=27) AS. Blinded biventricular function and lung parenchymal disease assessments were obtained by high-resolution CT imaging.
RESULTS
Patient demographics were similar between groups. pLFLG-AS had lower RV ejection fraction (49±10%) compared to both classic severe (58±7%, p<0.001) and NFLG AS (55±65%, p=0.02). There were no significant differences on lung emphysema (p=0.19), air fraction (p=0.58), and pulmonary disease presence (p=0.94) and severity (p=0.67) between groups.
CONCLUSION
pLFLG-AS patients have lower RV ejection fraction, than classic severe and normal-flow low-gradient AS patients in the absence of significant parenchymal lung disease on CT imaging. These findings support the direct importance of RV function in the pathophysiology of aortic valve disease.
PubMed: 36212028
DOI: 10.1016/j.shj.2022.100014 -
JACC. Cardiovascular Imaging Aug 2019Aortic stenosis (AS) causes left ventricular remodeling (hypertrophy, remodeling, fibrosis) and other cardiac changes (left atrial dilatation, pulmonary artery and... (Review)
Review
Aortic stenosis (AS) causes left ventricular remodeling (hypertrophy, remodeling, fibrosis) and other cardiac changes (left atrial dilatation, pulmonary artery and right ventricular changes). These changes, and whether they are reversible (reverse remodeling), are major determinants of timing and outcome from transcatheter or surgical aortic valve replacement. Cardiac changes in response to AS afterload can either be adaptive and reversible, or maladaptive and irreversible, when they may convey residual risk after intervention. Structural and hemodynamic assessment of AS therefore needs to evaluate more than the valve, and, in particular, the myocardial remodeling response. Imaging plays a key role in this. This review assesses how multimodality imaging evaluates AS myocardial hypertrophy and its components (cellular hypertrophy, fibrosis, microvascular changes, and additional features such as cardiac amyloid) both before and after intervention, and seeks to highlight how care and outcomes in AS could be improved.
Topics: Aortic Valve Stenosis; Echocardiography; Fibrosis; Heart Valve Prosthesis Implantation; Heart Ventricles; Humans; Hypertrophy, Left Ventricular; Magnetic Resonance Imaging; Multimodal Imaging; Predictive Value of Tests; Prognosis; Tomography, X-Ray Computed; Ventricular Function, Left; Ventricular Remodeling
PubMed: 31395243
DOI: 10.1016/j.jcmg.2019.02.034 -
Brazilian Journal of Cardiovascular... May 2023Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal... (Review)
Review
INTRODUCTION
Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome.
METHODS
Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal.
RESULTS
Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality.
CONCLUSION
The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.
Topics: Humans; Heart Defects, Congenital; Pulmonary Atresia; Aortic Valve Stenosis; Pulmonary Valve Stenosis; Risk Assessment
PubMed: 36592074
DOI: 10.21470/1678-9741-2022-0273 -
Journal of Imaging Oct 2022The pulmonary valve (PV) is the least imaged among the heart valves. However, pulmonary regurgitation (PR) and pulmonary stenosis (PS) can occur in a variety of patients... (Review)
Review
Multimodality Imaging of the Neglected Valve: Role of Echocardiography, Cardiac Magnetic Resonance and Cardiac Computed Tomography in Pulmonary Stenosis and Regurgitation.
The pulmonary valve (PV) is the least imaged among the heart valves. However, pulmonary regurgitation (PR) and pulmonary stenosis (PS) can occur in a variety of patients ranging from fetuses, newborns (e.g., tetralogy of Fallot) to adults (e.g., endocarditis, carcinoid syndrome, complications of operated tetralogy of Fallot). Due to their complexity, PR and PS are studied using multimodality imaging to assess their mechanism, severity, and hemodynamic consequences. Multimodality imaging is crucial to plan the correct management and to follow up patients with pulmonary valvulopathy. Echocardiography remains the first line methodology to assess patients with PR and PS, but the information obtained with this technique are often integrated with cardiac magnetic resonance (CMR) and computed tomography (CT). This state-of-the-art review aims to provide an updated overview of the usefulness, strengths, and limits of multimodality imaging in patients with PR and PS.
PubMed: 36286372
DOI: 10.3390/jimaging8100278 -
The Journal of Thoracic and... Apr 2022Pulmonary homograft dysfunction is a limitation after the Ross procedure. Decellularized pulmonary homografts can potentially mitigate this complication. The aim of this...
OBJECTIVES
Pulmonary homograft dysfunction is a limitation after the Ross procedure. Decellularized pulmonary homografts can potentially mitigate this complication. The aim of this study was to examine the incidence, predictors, progression, and morphology of pulmonary homograft dysfunction using data from the Canadian Ross Registry.
METHODS
From 2011 to 2019, 466 consecutive patients (mean age: 47 ± 12 years, 73% male) underwent a Ross procedure using a decellularized cryopreserved pulmonary homograft (SynerGraft SG; CryoKife, Kennesaw, Ga). Pulmonary homograft dysfunction was defined as any of the following: peak pulmonary gradient ≥30 mm Hg, pulmonary regurgitation >2, or pulmonary homograft reintervention. Patients meeting ≥1 of these criteria (n = 30) were compared with the rest of the cohort (n = 436). Median follow-up is 2.2 years (maximum = 8.5 years) and 99% complete (1176 patient-years).
RESULTS
The cumulative incidence of pulmonary homograft dysfunction was 11 ± 2% at 6 years. Pulmonary homograft stenosis was the most frequent presentation (n = 28 patients, 93%). Morphologically, stenosis occurred most often along the conduit (59%). Overall, 4 patients required homograft reintervention. At 6 years, the cumulative incidence of homograft reintervention was 3 ± 1%. The instantaneous risk was greatest in the first year after surgery (3.5%/year) and decreased to <1%/year thereafter. Patient age <45 years was the only independent risk factor associated with pulmonary homograft dysfunction (hazard ratio, 3.1, 95% confidence interval, 1.1-8.6, P = .03).
CONCLUSIONS
The use of decellularized cryopreserved pulmonary homografts results in a low incidence of dysfunction and reintervention after the Ross procedure. The risk is greater in the first postoperative year. Younger age is the only independent risk factor for pulmonary homograft dysfunction.
Topics: Adult; Age Factors; Allografts; Cryopreservation; Female; Graft Survival; Heart Valve Prosthesis Implantation; Humans; Male; Middle Aged; Pulmonary Valve; Pulmonary Valve Stenosis; Registries; Reoperation; Risk Factors
PubMed: 32888704
DOI: 10.1016/j.jtcvs.2020.06.139