No previous meta-analysis has evaluated the efficacy and safety of pulmonary vasodilators in Fontan physiology. Recent relative trials have obtained conflicting results regarding improvements in peak oxygen consumption; the relatively small number of patients in each study may be a limiting factor. We aimed to evaluate the efficacy and safety of pulmonary vasodilators in Fontan patients. Relevant studies were identified by searching the PubMed, Embase, and Cochrane Library databases. Pooled outcomes were determined to assess the efficacy and safety of pulmonary vasodilators in Fontan patients. Nine randomized controlled studies involving 381 patients with Fontan circulation were included. Pulmonary vasodilator therapy led to significant improvement (mean difference = −0.39, 95% CI: [−0.72, −0.05]) in the New York Heart Association (NYHA) functional class. The 6-minute walking distance (6MWD) was significantly increased by 134 m (95% CI: [86.07, 181.94]), and the peak VO2 was also significantly improved (mean difference = 1.42 ml·(kg·min)-1, 95% CI: [0.21, 2.63]). Additionally, the mean pulmonary artery pressure (mPAP) was significantly reduced (mean difference = −2.25 mmHg, 95% CI: [−3.00, −1.50]). No significant change was found in mortality or in brain natriuretic peptide (BNP) or N-terminal pronatriuretic peptide (NT-proBNP). Four studies reported no side effects and good drug tolerance, and two studies reported mild adverse effects. The present meta-analysis indicated that pulmonary vasodilators (primarily the PDE-5 inhibitor and endothelin-1 receptor antagonist) significantly improved the hemodynamics of Fontan patients, reduced the NYHA functional class and increased the 6MWD. The peak oxygen consumption was also improved. No significant change was observed in mortality or in the BNP or NT-proBNP level. Overall, the pulmonary vasodilators were well tolerated. This finding needs to be confirmed in future studies.

PurposePatients frequently undergo radical cystectomy and urinary diversion for treatment of bladder cancer. However, they remain at risk of urethral recurrence (UR). Studies have determined various risk factors leading to urethral recurrence. However, no publications have weighed the predictive values of these factors.Materials and MethodsStudies published between 1971 and 2016 were retrieved from PubMed, EMBASE and MEDLINE. We used STATA software (Version 12.0) to estimate the pooled risk ratio.ResultsTwenty-five publications with 9498 patients were included. Overall, male patients, especially those with concomitant carcinoma in situ, superficial or intravesical bladder cancer, non-orthotopic diversion, prostatic involvement, bladder neck involvement, positive urethral margins or multifocal bladder cancer were at higher risk of urethral recurrence. The overall risks of recurrence, reported as risk ratios, varied widely. Among all 25 studies, 118 (60.2%) cases in 9 studies were diagnosed through routine follow-up. Another 82 (40.8%) patients in 11 studies first reported symptomatic abnormalities. Prognoses were worse for patients with symptomatic recurrence. Urethral cytology was the most common diagnostic method. Treatment after UR was reported for 272 cases in 14 publications, and 190 patients underwent urethrectomy and 52 underwent urethra-sparing treatments. Outcomes after UR were described in 12 studies reporting 180 cases, and 41 patients were alive through the end of follow-up and 65 patients died of bladder cancer.ConclusionsUR following radical cystectomy for bladder cancer was closely related to risk factors. Precautions, strict follow-up protocols and rational therapies were critical to patients with high risks of urethral recurrences.

Background: Acute mesenteric ischemia (AMI) is a life-threatening medical condition that occurs when a sudden decreased perfusion to the intestines which leads to bowel infarction, and acute superior mesenteric artery embolism (ASMAE) is the main cause of AMI. Unfortunately, with the improvement of diagnosis and treatment technology, the mortality remains high due to less frequent clinical suspicion resulted from the unclear clinical manifestation and non-specific laboratory findings. Methods: Relevant studies published were identified by searching the PubMed, Embase and Cochrane Library databases. This review presented the literatures to introduce the research progress of ASMAE in recent years. Results: Patients with the history of atrial fibrillation, heart valve disease and atherosclerosis should be considered as ASMAE. Laboratory findings are insensitive and unspecific, however, angiography and Computed tomography angiography (CTA) can provide a clear diagnosis sensitively and specifically. Endovascular approaches have been increasingly reported in multiple case series. The key to successful treatment of AMI involves early clinical recognition and early intervention to move the embolus, which can reduce the rate of misdiagnosis and save the precious time and lives of patients. Conclusions: Loss of time eventually leads to progression of ischemia to transmural bowel necrosis with peritonitis and septicemia, which may further worsen patients’ outcomes. It is important for physicians to make a timely and accurate diagnosis, which can save precious time and reduce the mortality.

Background: Pulmonary valve replacement is required for patients with right ventricular outflow tract (RVOT) dysfunction. Surgical and percutaneous pulmonary valve replacement are the treatment options. Percutaneous pulmonary valve implantation (PPVI) provides a less-invasive therapy for patients. The aim of this study was to evaluate the effectiveness and safety of PPVI and the optimal time for implantation. Methods: We searched PubMed, EMBASE, Clinical Trial , and Google Scholar databases covering the period until May 2018. The primary effectiveness endpoint was the mean RVOT gradient; the secondary endpoints were the pulmonary regurgitation fraction, left and right ventricular end-diastolic and systolic volume indexes, and left ventricular ejection fraction. The safety endpoints were the complication rates. Results: A total of 20 studies with 1246 participants enrolled were conducted. The RVOT gradient decreased significantly [weighted mean difference (WMD) = −19.63 mmHg; 95% confidence interval (CI): −21.15, −18.11; p < 0.001]. The right ventricular end-diastolic volume index (RVEDVi) was improved (WMD = −17.59 ml/m²; 95% CI: −20.93, −14.24; p < 0.001), but patients with a preoperative RVEDVi >140 ml/m² did not reach the normal size. Pulmonary regurgitation fraction (PRF) was notably decreased (WMD = −26.27%, 95% CI: −34.29, −18.25; p < 0.001). The procedure success rate was 99% (95% CI: 98–99), with a stent fracture rate of 5% (95% CI: 4–6), the pooled infective endocarditis rate was 2% (95% CI: 1–4), and the incidence of reintervention was 5% (95% CI: 4–6). Conclusions: In patients with RVOT dysfunction, PPVI can relieve right ventricular remodeling, improving hemodynamic and clinical outcomes.

Backgrounds No previous meta‐analyses have compared the efficacy and safety of BPA with riociguat therapy in inoperable CTEPH patients. Methods Relevant published studies were searched in the PubMed, Embase and http://clinicaltrial.gov databases. Results Twenty‐three clinical trials including 1454 patients (631 underwent BPA; 823 underwent riociguat therapy) were analyzed. BPA was associated with a greater improvement in RAP (mean difference (MD) = −3.53 mmHg, 95% CI: [−4.85, −2.21] vs MD = −1.05 mmHg, 95% CI: [−1.82, −0.29]); mPAP (MD = −15.02 mmHg, 95% CI: [−17.32, −12.71] vs MD = −4.19 mmHg, 95% CI: [−5.58, −2.80]); PVR (standard MD = −1.32 woods, 95% CI: [−1.57, −1.08] vs standard MD = −0.65 woods, 95% CI: [−0.79, −0.50]); NYHA functional class (RR = 6.78, 95% CI: [3.14, 14.64] vs RR = 1.49, 95% CI: [1.07, 2.07]); and 6MWD (MD = 71.66 m, 95% CI: [58.34, 84.99] vs MD = 45.25 m, 95% CI: [36.51, 53.99]) than riociguat treatment. However, the increase in CO was greater with riociguat (MD = 0.78 L/min, 95% CI: [0.61, 0.96]) than with BPA (MD = 0.33 L/min, 95% CI: [0.06, 0.59]). No significant difference in cardiac index (CI) was found between BPA (MD = 0.40 L/min/m2, 95% CI: [0.21, 0.58]) and riociguat (MD = 0.40 L/min/m2, 95% CI: [0.26, 0.54]). The most common complications of BPA were pulmonary injury (0.3%‐5.6%) and pulmonary edema (0.8%‐28.6%). The most common adverse events of riociguat were headache, dizziness, hypotension and nasopharyngitis. Conclusions Our meta‐analysis indicates that BPA might be associated with greater improvements in exercise tolerance and pulmonary hemodynamics except for cardiac output and cardiac index than riociguat therapy. However, both of them were well tolerated.

Background: We performed a meta-analysis to evaluate the efficacy and safety of pulmonary vasodilators in pediatric pulmonary hypertension (PH) patients.Methods: We searched electronic databases including PubMed, EMBASE, and the Cochrane Library up to May 2020, and conducted a subgroup analysis for pulmonary vasodilators or underlying disease.Results: Fifteen studies with 719 pediatric PH patients were included in the meta-analysis. Adverse events did not differ (p = 0.11, I2 = 15%) between the pulmonary vasodilators group and the control group, neither in the subgroups. In total, compared with the control group treatment, pulmonary vasodilators significantly decreased the mortality (p = 0.002), mean pulmonary artery pressure (mPAP, p = 0.02), and mechanical ventilation duration (p = 0.03), also improved the oxygenation index (OI, p = 0.01). In the persistent pulmonary hypertension of the newborn (PPHN) subgroup, phosphodiesterase type 5 inhibitors (PDE5i) significantly reduced mortality (p = 0.03), OI (p = 0.007) and mechanical ventilation duration (p = 0.004). Administration of endothelin receptor antagonists (ERAs) improved OI (p = 0.04) and mechanical ventilation duration (p &lt; 0.00001) in PPHN. We also found that in the pediatric pulmonary arterial hypertension (PPAH) subgroup, mPAP was pronouncedly declined with ERAs (p = 0.006). Systolic pulmonary artery pressure (sPAP, p &lt; 0.0001) and pulmonary arterial/aortic pressure (PA/AO, p &lt; 0.00001) were significantly relieved with PDE5i, partial pressure of arterial oxygen (PaO2) was improved with prostacyclin in postoperative PH (POPH) subgroup (p = 0.001). Compared with the control group, pulmonary vasodilators could significantly decrease PA/AO pressure (p &lt; 0.00001) and OI (p &lt; 0.00001) in the short-term (duration &lt;7 days) follow-up subgroup, improve mPAP (p = 0.03) and PaO2 (p = 0.01) in the mid-term (7–30 days) follow-up subgroup, also decrease mortality, mPAP (p = 0.0001), PA/AO pressure (p = 0.0007), duration of mechanical ventilation (p = 0.004), and ICU stay (p &lt; 0.00001) in the long-term follow subgroup (&gt;30 days).Conclusion: Pulmonary vasodilators decrease the mortality in pediatric PH patients, improve the respiratory and hemodynamic parameters, reduce the mechanical ventilation duration.

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Rationale:A single atrium is a rare congenital heart disease (CHD) involving zero atrial septal traces and preserved intact ventricular septum and atrioventricular valves, requiring careful surgical intervention. However, developing to Eisenmenger syndrome (ES) makes the surgery complicated. Based on bidirectional cardiac shunting, vegetation easily develops in case of bacterial infection.Patient concern and diagnoses:We reported a 35-year-old woman with a single atrium, patent ductus arteriosus, pulmonary hypertension, and ES who developed infective endocarditis on her left ventricular outflow tract and complicated cerebral abscess and who underwent challenged medical treatment.Intervention:Infection was successfully controlled after 4-time change in antibiotics over 4 months. However, surgery is complicated for her.Outcomes:The patient presented a relatively good outcome during follow-up for >6 months.Lessons:This case report suggests that patients with complex CHD should accept surgery therapy earlier before developing ES. It is imperative to avoid invasive interventions to prevent infectious endocarditis.