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Orphanet Journal of Rare Diseases Oct 2020A parachute tricuspid valve is a very rare congenital cardiac anomaly. Its morphological features and clinical implications have not been sufficiently described so far.... (Review)
Review
BACKGROUND
A parachute tricuspid valve is a very rare congenital cardiac anomaly. Its morphological features and clinical implications have not been sufficiently described so far. The purpose of the present systematic review is to disclose the morphological and clinical characteristics of parachute tricuspid valve, and to discuss its diagnostic methods, treatments and patients' outcomes.
MAIN BODY
The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement guidelines were followed in this systematic review. Publications were systematically searched in the PubMed, Highwire Press, and the Cochrane Library databases. By comprehensive retrieval of the pertinent literature published between 1979 and 2019, 13 reports were collected with 14 patients recruited into this study. Their ages ranged from neonate to 52 years old with a median age of 23 years. Tricuspid valve regurgitation of a less-than-severe degree was seen in 6 (60%) patients, tricuspid valve stenosis was present in 3 (30%) patients and normally functioning tricuspid valve was noted in 1 (10%) patient. All patients had a single papillary muscle in the right ventricle. The chordae tendineae could be normal in length and thickness, or elongated, or shortened and thickened. Forty percent of the patients were asymptomatic or with only mild symptoms and did not need a surgical or interventional therapy, and 6 (60%) patients were indicated for a surgical/interventional treatment due to their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. Patients' outcomes varied depending on the substantial status of the patients with a survival rate of 70% and mortality rate of 30%.
CONCLUSION
A few patients with a parachute tricuspid valve are asymptomatic or only with mild symptoms and a surgical or interventional treatment is not required. The surgical/interventional indications for parachute tricuspid valve patients are their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. The survival rate of this patient setting is satisfactory.
Topics: Heart Defects, Congenital; Humans; Infant, Newborn; Middle Aged; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 33115523
DOI: 10.1186/s13023-020-01561-y -
European Urology Sep 2020There is a critical need for effective bladder-sparing therapies for bacillus Calmette-Guérin (BCG)-unresponsive non-muscle-invasive bladder cancer (NMIBC). Owing to...
CONTEXT
There is a critical need for effective bladder-sparing therapies for bacillus Calmette-Guérin (BCG)-unresponsive non-muscle-invasive bladder cancer (NMIBC). Owing to the current lack of effective agents that can be used as a control, the US Food and Drug Administration began to accept single-arm trials for patients with carcinoma in situ (CIS), using complete response rate (CRR) and duration of response as the primary endpoints to support marketing applications. Despite the ensuing growth of clinical trials in this space, no consensus exists on a clinically relevant benchmark for CRR.
OBJECTIVE
To elucidate the CRR and recurrence-free rate (RFR) using bladder-sparing agents after BCG failure in order to provide a frame of reference for future clinical trial results.
EVIDENCE ACQUISITION
We performed a systematic review of clinical trials utilizing bladder-sparing therapeutics for NMIBC recurring after intravesical BCG (PROSPERO CRD42019130553). The search was performed in MEDLINE, EMBASE, and Cochrane Library. Relevant studies identified from bibliography search and conference abstracts were searched to complement the systematic review. A total of 42 studies utilizing 24 treatment options and consisting of 2254 patients were included for final analysis.
EVIDENCE SYNTHESIS
Median CRRs in the treatment of CIS-containing tumors were 26% at 6 mo, 17% at 12 mo, and 8% at 24 mo after treatment. In comparison, median RFRs in the papillary-only studies were 67% at 6 mo, 44% at 12 mo, and 10% at 24 mo. Specifically in the BCG-unresponsive population, 6- and 12-mo CRRs in CIS-containing patients treated with Mycobacterium phlei cell wall-nucleic acid complex were 45% and 27%, respectively, and the median 6-, 12-, and 24-mo disease-free rates in the other studies were 43%, 35%, and 18%, respectively. The median progression-free rate was 91%: 95% in the CIS-containing studies and 89% in studies restricted to papillary-only recurrences. Toxicities of intravesical agents were generally mild, with very few dose limiting toxicities.
CONCLUSIONS
We demonstrate that, to date, bladder-sparing therapies achieved modest efficacy in patients with NMIBC after BCG. Results from the current study will serve as a frame of reference for emerging trial results in the BCG-unresponsive space.
PATIENT SUMMARY
In this study, we found that bladder-sparing therapies achieved modest efficacy in patients with non-muscle-invasive bladder cancer after bacillus Calmette-Guérin (BCG). These results will serve to inform future clinical trial results for salvage agents used to treat BCG-unresponsive bladder cancer.
Topics: Adjuvants, Immunologic; Administration, Intravesical; BCG Vaccine; Carcinoma in Situ; Humans; Neoplasm Invasiveness; Organ Sparing Treatments; Treatment Outcome; Urinary Bladder Neoplasms
PubMed: 32143924
DOI: 10.1016/j.eururo.2020.02.012 -
Journal of Thoracic Disease Jun 2017Combining a ring annuloplasty (Ring) with a mitral subvalvular intervention (Ring + subvalvular) in patients with secondary mitral regurgitation (MR) may improve mitral...
Mitral valve repair and subvalvular intervention for secondary mitral regurgitation: a systematic review and meta-analysis of randomized controlled and propensity matched studies.
BACKGROUND
Combining a ring annuloplasty (Ring) with a mitral subvalvular intervention (Ring + subvalvular) in patients with secondary mitral regurgitation (MR) may improve mitral valve (MV) repair durability. However, the outcomes of this strategy compared with a Ring only, have not been clearly defined.
METHODS
A systematic review and meta-analysis was performed utilizing randomized controlled and propensity matched studies which compared a Ring + subvalvular versus Ring MV repair for the treatment of secondary MR. Risk ratio (RR), weighted mean difference (MD), and the 95% confidence interval (CI) were calculated by the Mantel-Haenszel and inverse-variance methods, for clinical outcomes and echocardiographic measures of follow-up MR, left ventricular (LV) reverse remodeling, and MV apparatus geometry.
RESULTS
Five studies were identified, with a total of 397 patients. Baseline characteristics were similar between groups, and all patients had moderate to severe secondary MR, with the vast majority in the setting of ischemic cardiomyopathy. A Ring + subvalvular repair consisted of papillary muscle approximation (n=2), papillary muscle relocation (n=2), or secondary chordal cutting (n=1). Follow-up ranged from 10.1 (mean range =0.25-42) to 69 [interquartile range (IQR) =23-82] months. When compared with Ring only at last follow-up, a Ring + subvalvular MV repair was associated with: (I) a smaller MR grade (MD =-0.44, 95% CI -0.69 to -0.19; P=0.0005); (II) a reduced risk of moderate or greater recurrent MR (RR =0.43, 95% CI, 0.27-0.66; P=0.0002); (III) a smaller mean LV end-diastolic diameter (MD =-3.56 mm, 95% CI -5.40 to -1.73; P=0.0001) and a greater ejection fraction (MD =2.64%, 95% CI, 0.13-5.15; P=0.04); and, (IV) an improved MV apparatus geometry. There were no differences in operative mortality, post-operative morbidity, or follow-up survival between surgical approaches.
CONCLUSIONS
When compared with Ring only, a Ring + subvalvular MV repair is associated with greater LV reverse remodeling and systolic function, less recurrence of moderate or greater MR, and an improved geometry of the MV apparatus at short and mid-term follow-up.
PubMed: 28740711
DOI: 10.21037/jtd.2017.05.56