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Journal of Thoracic Disease Mar 2020Patients with achondroplasia and other causes of dwarfism suffer from increased rates of cardiovascular disease relative to the remainder of the population. Few studies... (Review)
Review
Patients with achondroplasia and other causes of dwarfism suffer from increased rates of cardiovascular disease relative to the remainder of the population. Few studies have examined these patients when undergoing cardiac surgery or percutaneous intervention. This systematic review examines the literature to determine outcomes following cardiac intervention in this unique population. An electronic search was performed in the English literature to identify all reports of achondroplasia, dwarfism, and cardiac intervention. Of the 5,274 articles identified, 14 articles with 14 cases met inclusion criteria. Patient-level data was extracted and analyzed. Median patient age was 55.5 [interquartile ranges (IQR), 43.8, 59.8] years, median height 102.0 [98.8, 112.5] cm, median BMI 32.1 [27.0, 45.9], and 57.1% (8/14) were male. Of these 14 patients, nine had the following documented skeletal abnormalities: 66.7% (6/9) had scoliosis, 66.7% (6/9) had kyphosis, 11.1% (1/9) had lordosis, 11.1% (1/9) pectus carinatum and 11.1% (1/9) spinal stenosis. Coronary artery disease was present in 53.8% (7/13), and 30.8% (4/13) patients previously suffered a myocardial infarction. Of the eight patients who underwent cardiac surgery, 37.5% (3/8) underwent multivessel coronary artery bypass grafting, 37.5% (3/8) underwent aortic valve replacement, 25.0% (2/8) underwent type A aortic dissection repair, and the remaining 12.5% (1/8) underwent pulmonary thromboendarterectomy. Six patients underwent percutaneous intervention. Median cardiopulmonary bypass time was 136.5 [110.0, 178.8] minutes. Median arterial cannula size was 20.0 [20.0, 24.0] Fr. Bicaval cannulation was performed in all cases describing cannulation strategy (5/5). Median superior vena cava cannula size was 28.0 [28.0, 28.0] Fr, and inferior vena cava cannula size was 28.0 [28.0, 28.0] Fr. No mortality was reported with a median follow up time of 6.0 [6.0, 10.5] months. In conclusion, Common cardiac procedures can be performed with reasonable safety in this patient population. Operative adjustments may need to be made with respect to equipment to accommodate patient-specific needs.
PubMed: 32274169
DOI: 10.21037/jtd.2020.02.05 -
Journal of the American Heart... Apr 2022Background Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive... (Meta-Analysis)
Meta-Analysis Review
Background Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive physiology, but is not fully understood. This systematic review and meta-analysis sought to clarify its physiological and clinical correlates, and to define a framework for understanding EDFF and RV restrictive physiology. Methods and Results PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for observational studies published before March 2021. Random-effects meta-analysis was performed to identify factors associated with EDFF. Forty-two individual studies published between 1995 and 2021, including a total of 2651 participants (1132 with EDFF; 1519 with no EDFF), met eligibility criteria. The pooled estimated prevalence of EDFF among patients with repaired tetralogy of Fallot was 46.5% (95% CI, 41.6%-51.3%). Among patients with EDFF, the use of a transannular patch was significantly more common, and their stay in the intensive care unit was longer. EDFF was associated with greater RV indexed volumes and mass, as well as smaller E-wave velocity at the tricuspid valve. Finally, pulmonary regurgitation fraction was greater in patients with EDFF, and moderate to severe pulmonary regurgitation was more common in this population. Conclusions EDFF is associated with dilated, hypertrophied RVs and longstanding pulmonary regurgitation. Although several studies have defined RV restrictive physiology as the presence of EDFF, our study found no clear indicators of poor RV compliance in patients with EDFF, suggesting that EDFF may have multiple causes and might not be the precise equivalent of RV restrictive physiology.
Topics: Diastole; Humans; Pulmonary Valve Insufficiency; Tetralogy of Fallot; Tricuspid Valve; Ventricular Dysfunction, Right; Ventricular Function, Right
PubMed: 35301867
DOI: 10.1161/JAHA.121.024036 -
Journal of Personalized Medicine Apr 2022Patients with severe aortic valve stenosis and concomitant pulmonary hypertension show a significantly reduced survival prognosis. Right heart catheterization as a... (Review)
Review
Severe Aortic Valve Stenosis and Pulmonary Hypertension: A Systematic Review of Non-Invasive Ways of Risk Stratification, Especially in Patients Undergoing Transcatheter Aortic Valve Replacement.
Patients with severe aortic valve stenosis and concomitant pulmonary hypertension show a significantly reduced survival prognosis. Right heart catheterization as a preoperative diagnostic tool to determine pulmonary hypertension has been largely abandoned in recent years in favor of echocardiographic criteria. Clinically, determination of echocardiographically estimated systolic pulmonary artery pressure falls far short of invasive right heart catheterization data in terms of accuracy. The aim of the present systematic review was to highlight noninvasive possibilities for the detection of pulmonary hypertension in patients with severe aortic valve stenosis, with a special focus on cardiovascular biomarkers. A total of 525 publications regarding echocardiography, cardiovascular imaging and biomarkers related to severe aortic valve stenosis and pulmonary hypertension were analyzed in a systematic database analysis using PubMed Central. Finally, 39 publications were included in the following review. It was shown that the current scientific data situation, especially regarding cardiovascular biomarkers as non-invasive diagnostic tools for the determination of pulmonary hypertension in severe aortic valve stenosis patients, is poor. Thus, there is a great scientific potential to combine different biomarkers (biomarker scores) in a non-invasive way to determine the presence or absence of PH.
PubMed: 35455719
DOI: 10.3390/jpm12040603 -
PloS One 2021In the last 25 years, numerous tissue engineered heart valve (TEHV) strategies have been studied in large animal models. To evaluate, qualify and summarize all available... (Meta-Analysis)
Meta-Analysis
In the last 25 years, numerous tissue engineered heart valve (TEHV) strategies have been studied in large animal models. To evaluate, qualify and summarize all available publications, we conducted a systematic review and meta-analysis. We identified 80 reports that studied TEHVs of synthetic or natural scaffolds in pulmonary position (n = 693 animals). We identified substantial heterogeneity in study designs, methods and outcomes. Most importantly, the quality assessment showed poor reporting in randomization and blinding strategies. Meta-analysis showed no differences in mortality and rate of valve regurgitation between different scaffolds or strategies. However, it revealed a higher transvalvular pressure gradient in synthetic scaffolds (11.6 mmHg; 95% CI, [7.31-15.89]) compared to natural scaffolds (4,67 mmHg; 95% CI, [3,94-5.39]; p = 0.003). These results should be interpreted with caution due to lack of a standardized control group, substantial study heterogeneity, and relatively low number of comparable studies in subgroup analyses. Based on this review, the most adequate scaffold model is still undefined. This review endorses that, to move the TEHV field forward and enable reliable comparisons, it is essential to define standardized methods and ways of reporting. This would greatly enhance the value of individual large animal studies.
Topics: Animals; Heart Valve Prosthesis; Models, Animal; Pulmonary Valve; Tissue Engineering
PubMed: 34610023
DOI: 10.1371/journal.pone.0258046 -
Annals of Cardiothoracic Surgery Jul 2021The Ross procedure involves autograft transplantation of the native pulmonary valve into the aortic position and reconstruction of the right ventricular outflow tract...
BACKGROUND
The Ross procedure involves autograft transplantation of the native pulmonary valve into the aortic position and reconstruction of the right ventricular outflow tract (RVOT) with a homograft. The operation offers the advantages of a native valve with excellent hemodynamic performance, the avoidance of anticoagulation, and growth potential. Conversely, the operation is technically demanding and imposes the risk of turning single-valve disease into double-valve disease. This systematic review reports outcomes of pediatric patients undergoing the Ross procedure.
METHODS
An electronic search identified studies reporting outcomes on pediatric patients (mean age <18 years, max age <21 years) undergoing the Ross procedure. Long-term outcomes, including early mortality, late mortality, sudden unexpected unexplained death, reoperation due to failure of the pulmonary autograft or RVOT reconstruction, thromboembolic events, bleeding events, and endocarditis-related complications, were evaluated.
RESULTS
Upon review of 2,035 publications, 30 studies and 3,156 pediatric patients were included. Patients had a median age of 9.5 years and median follow-up period of 5.7 years. Early mortality rates varied from 0.0 to 17.0% and were increased in the neonatal population. Late mortality rates were much lower (0.04-1.83%/year). Reoperation due to pulmonary autograft failure occurred at rates of 0.37-2.81%/year and reoperation due to RVOT reconstruction failure was required at rates of 0.34-4.76%/year. Thromboembolic, bleeding, and endocarditis events were reported to occur at rates of 0.00-0.58, 0.00-0.39, and 0.00-1.68%/year, respectively.
CONCLUSIONS
The Ross operation offers a durable aortic valve replacement (AVR) option in the pediatric population that offers favorable survival, excellent hemodynamics, growth potential, decreased risk of complications, and avoidance of anticoagulation. Larger multi-institutional registries focusing on pediatric patients are necessary to provide more robust evidence to further support use of the Ross procedure in this population.
PubMed: 34422554
DOI: 10.21037/acs-2020-rp-23 -
Four-dimensional flow cardiovascular magnetic resonance in tetralogy of Fallot: a systematic review.Journal of Cardiovascular Magnetic... May 2021Patients with repaired Tetralogy of Fallot (rTOF) often develop cardiovascular dysfunction and require regular imaging to evaluate deterioration and time interventions... (Review)
Review
BACKGROUND
Patients with repaired Tetralogy of Fallot (rTOF) often develop cardiovascular dysfunction and require regular imaging to evaluate deterioration and time interventions such as pulmonary valve replacement. Four-dimensional flow cardiovascular magnetic resonance (4D flow CMR) enables detailed assessment of flow characteristics in all chambers and great vessels. We performed a systematic review of intra-cardiac 4D flow applications in rTOF patients, to examine clinical utility and highlight optimal methods for evaluating rTOF patients.
METHODS
A comprehensive literature search was undertaken in March 2020 on Google Scholar and Scopus. A modified version of the Critical Appraisal Skills Programme (CASP) tool was used to assess and score the applicability of each study. Important clinical outcomes were assessed including similarities and differences.
RESULTS
Of the 635 articles identified, 26 studies met eligibility for systematic review. None of these were below 59% applicability on the modified CASP score. Studies could be broadly classified into four groups: (i) pilot studies, (ii) development of new acquisition methods, (iii) validation and (vi) identification of novel flow features. Quantitative comparison with other modalities included 2D phase contrast CMR (13 studies) and echocardiography (4 studies). The 4D flow study applications included stroke volume (18/26;69%), regurgitant fraction (16/26;62%), relative branch pulmonary artery flow(4/26;15%), systolic peak velocity (9/26;35%), systemic/pulmonary total flow ratio (6/26;23%), end diastolic and end systolic volume (5/26;19%), kinetic energy (5/26;19%) and vorticity (2/26;8%).
CONCLUSIONS
4D flow CMR shows potential in rTOF assessment, particularly in retrospective valve tracking for flow evaluation, velocity profiling, intra-cardiac kinetic energy quantification, and vortex visualization. Protocols should be targeted to pathology. Prospective, randomized, multi-centered studies are required to validate these new characteristics and establish their clinical use.
Topics: Heart Ventricles; Humans; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Predictive Value of Tests; Prospective Studies; Retrospective Studies; Tetralogy of Fallot
PubMed: 34011372
DOI: 10.1186/s12968-021-00745-0 -
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 -
World Journal of Surgical Oncology Mar 2023Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few... (Review)
Review
BACKGROUND
Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma.
METHODS
Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively.
RESULTS
This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively.
CONCLUSIONS
Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.
Topics: Male; Humans; Child; Aged; Middle Aged; Pulmonary Valve; Echocardiography; Heart Neoplasms; Myxoma; Heart Atria
PubMed: 36941612
DOI: 10.1186/s12957-023-02984-0 -
Cureus Jan 2023Patients with symptomatic aortic stenosis (AS) commonly have an associated finding of pulmonary hypertension (PH), and it has been previously shown to have increased... (Review)
Review
Patients with symptomatic aortic stenosis (AS) commonly have an associated finding of pulmonary hypertension (PH), and it has been previously shown to have increased morbidity and mortality following surgical aortic valve repair (SAVR) as well as transcatheter aortic valve implantation (TAVI). There are no guidelines stating the cut-off point for PH at which the patient can safely undergo TAVI with benefits outweighing the risks. This is partly due to the lack of uniformity in the PH definition used in various studies. This systematic review sought to study the effect of preprocedural pulmonary hypertension on early and late all-cause and cardiac mortality in patients undergoing TAVI. We performed a systematic review of studies comparing patients with AS undergoing TAVI having PH. The review was undertaken as per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Articles were identified from PubMed, Pubmed Central (PMC), Cochrane, and Medline on January 10, 2022, for literature published until January 10, 2022. MeSH strategy was used on PubMed to search the literature, and filters were applied to search only Observational Studies, randomized controlled trials (RCT), and meta-analysis. A total of 170 unique articles were identified and screened. Of the 33 full-text articles that were reviewed, 18 articles, including duplicates, were excluded. Fifteen articles fulfilled the selection criteria and were included in this review. The study design included two meta-analyses, one randomized control trial, one prospective cohort study, and 11 retrospective cohort studies. The studies involved a total of approximately 30,000 patients. The observational studies in our review were of good to fair quality, the RCT had a low to moderate bias, and the meta-analysis was of moderate quality. Baseline PH and persistence of PH post-TAVI are strongly associated with all-cause and cardiac mortality. Few studies have shown that a decrease in post-TAVI PH carries mortality benefits. Therefore, efforts should be made to identify mechanisms of persistent PH post-TAVI and whether interventions to reduce PH pre-TAVI will have any clinical implications or not by conducting RCT.
PubMed: 36860229
DOI: 10.7759/cureus.34300 -
Frontiers in Cardiovascular Medicine 2021Postoperative pulmonary complications remain a leading cause of increased morbidity, mortality, longer hospital stays, and increased costs after cardiac surgery;...
Comparative Evaluation of the Incidence of Postoperative Pulmonary Complications After Minimally Invasive Valve Surgery vs. Full Sternotomy: A Systematic Review and Meta-Analysis of Randomized Controlled Trials and Propensity Score-Matched Studies.
Postoperative pulmonary complications remain a leading cause of increased morbidity, mortality, longer hospital stays, and increased costs after cardiac surgery; therefore, our study aims to analyze whether minimally invasive valve surgery (MIVS) for both aortic and mitral valves can improve pulmonary function and reduce the incidence of postoperative pulmonary complications when compared with the full median sternotomy (FS) approach. A comprehensive systematic literature research was performed for studies comparing MIVS and FS up to February 2021. Randomized controlled trials (RCTs) and propensity score-matching (PSM) studies comparing early respiratory function and pulmonary complications after MIVS and FS were extracted and analyzed. Secondary outcomes included intra- and postoperative outcomes. A total of 10,194 patients from 30 studies (6 RCTs and 24 PSM studies) were analyzed. Early mortality differed significantly between the groups (MIVS 1.2 vs. FS 1.9%; = 0.005). Compared with FS, MIVS significantly lowered the incidence of postoperative pulmonary complications (odds ratio 0.79, 95% confidence interval [0.67, 0.93]; = 0.004) and improved early postoperative respiratory function status (mean difference -24.83 [-29.90, -19.76]; < 0.00001). Blood transfusion amount was significantly lower after MIVS ( < 0.02), whereas cardiopulmonary bypass time and aortic cross-clamp time were significantly longer after MIVS ( < 0.00001). Our study showed that minimally invasive valve surgery decreases the incidence of postoperative pulmonary complications and improves postoperative respiratory function status.
PubMed: 34497838
DOI: 10.3389/fcvm.2021.724178