Four versus 3 Cycles of Neoadjuvant Chemotherapy for Muscle-Invasive Bladder Cancer: Implications for Pathological Response and Survival.

PURPOSE: The ideal number of neoadjuvant chemotherapy (NAC) cycles for muscle-invasive bladder cancer is uncertain with 3 to 4 representing the standard of care (SOC). We compared ypT0 rates and survival between patients receiving 4 versus 3 cycles of NAC with evaluation of chemotherapy-related toxicity for correlation with tumor chemosensitivity and pathological response. MATERIALS AND METHODS: Patients receiving NAC followed by radical cystectomy for cT2-4N0M0 urothelial carcinoma from 2 institutions were included. Primary study groups included 4 cisplatin-based NAC cycles, 3 cisplatin-based NAC cycles, and nonSOC NAC (1-2 cycles or noncisplatin-based) to compare ypT0/≤ypT1 rates and survival. A cohort of patients not receiving NAC was included for pathological reference. RESULTS: Of 693 total patients, 318 (45.9%) received NAC. ypT0 and ≤ypT1 rates were 42/157 (26.8%) and 86/157 (54.8%) for 4 cycles, 38/114 (33.3%) and 71/114 (62.3%) for 3 cycles, and 6/47 (12.8%) and 13/47 (27.7%) for nonSOC (p=0.03 and p <0.01, respectively). Pathological response appeared higher among patients receiving 3 cycles due to toxicity (ypT0: 29/77 [37.7%]; ≤ypT1: 51/77 [66.2%]) but did not reach statistical significance. Toxicities leading to treatment modifications were thrombocytopenia (32.1%), neutropenia (27.2%), renal insufficiency (22.2%), and constitutional symptoms (18.5%). NonSOC patients had lower Kaplan-Meier survival (cT2-cT4N0M0: log-rank p=0.07; cT2N0M0: log-rank p=0.02). There were no statistically significant differences in survival between 4 and 3 cycles (HR 1.00 [95% CI 0.57-1.74], p=0.99). CONCLUSIONS: Patients completing 3 cycles of cisplatin-based NAC have similar pathologic response and short-term survival compared to 4 cycles. Further evaluation of patients experiencing toxicity as a potential marker of tumor chemosensitivity is needed.

Perioperative and long-term outcomes of Ross versus mechanical aortic valve replacement.

BACKGROUND: The ideal aortic valve replacement strategy in young- and middle-aged adults remains up for debate. Clinical practice guidelines recommend mechanical prostheses for most patients less than 50 years of age undergoing aortic valve replacement. However, risks of major hemorrhage and thromboembolism associated with long-term anticoagulation may make the pulmonary autograft technique, or Ross procedure, a preferred approach in select patients. METHODS: Data were retrospectively collected for patients 18-50 years of age who underwent either the Ross procedure or mechanical aortic valve replacement (mAVR) between January 2000 and December 2016 at a single institution. Propensity score matching was performed and yielded 32 well-matched pairs from a total of 216 eligible patients. RESULTS: Demographic and preoperative characteristics were similar between the two groups. Median follow-up was 7.3 and 6.9 years for Ross and mAVR, respectively. There were no early mortalities in either group and no statistically significant differences were observed with respect to perioperative outcomes or complications. Major hemorrhage and stroke events were significantly more frequent in the mAVR population (p < .01). Overall survival (p = .93), freedom from reintervention and valve dysfunction free survival (p = .91) were equivalent. CONCLUSIONS: In this mid-term propensity score-matched analysis, the Ross procedure offers similar perioperative outcomes, freedom from reintervention or valve dysfunction as well as overall survival compared to traditional mAVR but without the morbidity associated with long-term anticoagulation. At specialized centers with sufficient expertize, the Ross procedure should be strongly considered in select patients requiring aortic valve replacement.

Bicuspid aortic valve sparing root replacement.

BACKGROUND AND AIM OF STUDY: Mid and long-term data regarding the durability of bicuspid aortic valve sparing root replacement is not completely understood. In this study, our institutional experience is reviewed regarding this procedure with special attention to the operative technique. METHODS: From 2004 to 2019, 1241 patients underwent aortic root replacement and of these 79 patients underwent bicuspid aortic valve sparing root replacement. Early and late outcomes along with echocardiographic data were reviewed. Univariate analysis was used to identify risk factors for mortality. RESULTS: Median age at operation was 43 (inner quartile range: 34.5-50.5) years, with 17% (14) being female. Median follow up time was 4 (inner quartile range: 1-8) years with 359 years of total follow up. Early mortality was 2.5% (2). Overall 10-year freedom from aortic valve intervention was 95.6%. Risk factor analysis did not demonstrate preoperative root diameter, aortic diameter, or aortic insufficiency to be risk factors for mortality. CONCLUSION: Bicuspid aortic valve sparing root replacement can be performed safely in the setting of aortic root pathology and valve insufficiency. With appropriate selection and excellent surgical technique, long-term durable valve function is observed without the need for repeat valve intervention.

Disparities in Benign Prostatic Hyperplasia Progression: Predictors of Presentation to the Emergency Department in Urinary Retention.

PURPOSE: This retrospective cohort study evaluates the characteristics of patients who presented to the emergency department with acute urinary retention. MATERIALS AND METHODS: Using the Healthcare Cost and Utilization Project State Emergency Department Databases we conducted a retrospective cohort study of patients who presented to emergency departments in Florida between 2005 and 2015. Male patients age 45 years or older who presented with diagnosis codes for acute urinary retention and lower urinary tract symptoms/benign prostatic hyperplasia were considered. Information was collected on age, race/ethnicity, primary insurance and rural-urban commuting area codes. RESULTS: The mean age for males presenting with acute urinary retention was 72.2 years, which was 10.6 years older than those presenting for nonurological complaints (p <0.001). Multivariable analysis adjusted for measured confounders found all covariates to be significant. The risk of presenting to the emergency department for acute urinary retention from lower urinary tract symptoms/benign prostatic hyperplasia increased with age, with the 75 to less than 85-year-old age group at the highest risk (OR 15.96, p <0.001). Other factors associated acute urinary retention included African American (OR 1.15, p <0.001) or Hispanic (OR 1.75, p <0.001) race, Medicare (OR 1.27, p <0.001) or private (OR 1.33, p <0.001) insurance, and urban rural-urban commuting area codes (OR 1.31, p <0.001). CONCLUSIONS: Male patients who presented to the emergency department for acute urinary retention with benign prostatic hyperplasia were more likely to be older, of nonwhite race, have Medicare or private insurance, and live in more urban areas. These data suggest that African American and Hispanic patients may be untreated or under treated for benign prostatic hyperplasia in the outpatient setting, resulting in an increased risk of presentation to the emergency department with acute urinary retention.

Penile Prosthesis Implantation and Timing Disparities After Radical Prostatectomy: Results From a Statewide Claims Database.

BACKGROUND: Many patients with erectile dysfunction (ED) after radical prostatectomy (RP) improve with conservative therapy but some do not; penile prosthesis implantation rates have been sparsely reported, and have used nonrepresentative data sets. AIM: To characterize rates and timing of penile prosthesis implantation after RP and to identify predictors of implantation using a more representative data set. METHODS: The Healthcare Cost and Utilization Project State Inpatient and State Ambulatory Surgery databases for Florida from 2006 to 2015 were used. Patients undergoing RP (2006-2012) were tracked longitudinally for penile prosthesis implantation. Patient and clinical data were analyzed using multivariable logistic regression. OUTCOMES: The primary outcome was risk-adjusted predictors of prosthesis implantation, and the secondary outcome was predictors of the highest quartile of time between RP and penile prosthesis. RESULTS: Of 29,288 men who had RP, 1,449 (4.9%) patients underwent subsequent prosthesis. The mean time from RP to prosthesis was 2.6 years (median: 2.1; interquartile range [IQR]: 1.2-3.5). Adjusted predictors of prosthesis implantation included open RP (odds ratio [OR]: 1.5, P < .01), African American race (OR: 1.7, P < .01) or Hispanic ethnicity (OR: 3.2, P < .01), and Medicare (OR: 1.4, P < .01) insurance. Oler patients (age >70 years; OR: 0.7, P < .01) and those from the highest income quartile relative to the lowest (OR: 0.8, P < .05) were less likely to be implanted. Adjusted predictors of longer RP-to-implantation time (highest quartile: median: 4.7 years; IQR: 3.9-6.0 years) included open RP (OR: 1.78, P < .01), laparoscopic RP (OR: 4.67, P < .01), Medicaid (OR: 3.03, P < .05), private insurance (OR: 2.57, P < .01), and being in the highest income quartile (OR: 2.52, P < .01). CLINICAL IMPLICATIONS: These findings suggest ED treatment healthcare disparities meriting further investigation; upfront counseling on all ED treatment modalities and close monitoring for conservative treatment failure may reduce lost quality of life years. STRENGTHS & LIMITATIONS: This study is limited by its use of administrative data, which relies on accurate coding and lacks data on ED questionnaires/prior treatments, patient-level cost, and oncologic outcomes. Quartile-based analysis of income and time between RP and prosthesis limits the conclusions that can be drawn. CONCLUSION: Less than 5% of post-RP patients undergo penile prosthesis implantation, with open RP, Medicare, African American race, and Hispanic ethnicity predicting post-RP implantation; living in the wealthiest residential areas predicts lower likelihood of implantation compared to the least wealthy areas. Patients with the longest time between RP and prosthesis are more likely to live in the wealthiest areas or have undergone open/laparoscopic RP relative to robotic RP. Bajic P, Patel PM, Nelson MH, et al. Penile Prosthesis Implantation and Timing Disparities After Radical Prostatectomy: Results From a Statewide Claims Database. J Sex Med 2020;17:1175-1181.

Paravertebral block for percutaneous nephrolithotomy: a prospective, randomized, double-blind placebo-controlled study.

PURPOSE: Percutaneous nephrolithotomy (PCNL) is performed commonly in patients with large kidney stones, but the management of their postoperative pain presents a major challenge. While it is not routinely performed in PCNL patients, paravertebral block (PVB) has been described as an effective strategy for pain control after various non-urologic surgeries. This trial aims to assess the effect of paravertebral blockade on intraoperative and postoperative opioid use as well as postoperative pain control in patients undergoing PCNL. METHODS: This was a prospective, randomized, double-blind, placebo-controlled study. Patients who consented to participate were randomly assigned to undergo either PVB or a placebo intervention preoperatively. The patient, surgeon, and anesthesia team were all blinded to the randomization. The outcome parameters were intraoperative opioid requirement, postoperative visual analog scale (VAS) pain scores, postoperative opioid use, and postoperative antiemetic use. RESULTS: 23 patients were enrolled in each arm of the study, and the two groups had no significant differences in baseline demographic or clinical characteristics. Patients in the PVB group had significantly lower intraoperative opioid use, postoperative opioid use, frequency of opioid use, and antiemetic. Patients in the PVB group also had lower postoperative VAS pain scores. There were no patients who suffered from complications attributable to PVB. CONCLUSION: The results of this randomized, double-blind, placebo-controlled trial suggest that PVB should be considered an effective strategy to reduce opioid requirement and improve pain control for patients undergoing PCNL.

Vacuum-assisted extraction of ilio-caval and right heart masses: A 5-year single center experience.

PURPOSE: Despite paucity of data, there exists growing popularity of catheter-based extraction methods for intravascular thrombi and vegetations. We describe a large single center experience with vacuum-assisted extraction techniques (VAET) for right-sided intravascular and cardiac masses. METHODS: We retrospectively reviewed the perioperative course of patients undergoing VAET between 2014 and 2019. Primary outcomes were survival and freedom from recurrent bacteremia. Procedural success was a composite definition of survival, majority of mass extraction, absence of recurrent bacteremia, and valve function not requiring further intervention during index hospitalization. RESULTS: Of the entire cohort (n = 58), 48% and 52% underwent VAET for vegetations and sterile thrombi, respectively. Of those with positive cultures, the most common organism isolated was Staphylococcus aureus (48%). Preoperative active bacteremia was present in 36% (21/58) and of these patients, 76% (16/21) had neither recurrent nor persistent bacteremia post-op. The majority of masses (67%, 38/58) were debulked with an average reduction in size of 42%. Conversion to open surgery occurred in 3.5% (2/58). Intraoperative and 30-day survival were 98% (57/58) and 90% (28/31), respectively. Overall success was 86% (50/58). The prevalence of moderate/severe tricuspid regurgitation was 37% pre-op and 61% post-op. Average length of intensive care unit and overall hospital stay was 5.6 and 16 days, respectively. CONCLUSIONS: In this single center experience, VAET was conducted safely with a high degree of success and freedom from short-term recurrent bacteremia. This minimally invasive procedure is an attractive alternative to traditional open techniques for removal of right-sided intravascular and cardiac masses.

Bovine jugular vein conduit versus pulmonary homograft in the Ross operation.

OBJECTIVES: The Ross procedure involves using the native pulmonary valve for aortic valve replacement then replacing the pulmonary valve with an allograft or xenograft. We aimed to compare our age-matched experience with the bovine jugular vein conduit and the pulmonary homograft for pulmonary valve replacement during the Ross procedure in children. METHODS: Between 1998 and 2016, 15 patients <18 years of age underwent a Ross procedure using the bovine jugular vein conduit (Ross-Bovine Jugular Vein Conduit) at our institution. These patients were age-matched with 15 patients who had the Ross operation with a standard pulmonary homograft for right ventricular outflow tract reconstruction (Ross-Pulmonary Homograft). Paper and electronic medical records were retrospectively reviewed. RESULTS: The median age of the Ross-Bovine Jugular Vein Conduit and Ross-Pulmonary Homograft patients were 4.8 years (interquartile range 1.1-6.6) and 3.3 years (interquartile 1.2-7.6), respectively (p = 0.6). The median follow-up time for the Ross-Bovine Jugular Vein Conduit and Ross-Pulmonary Homograft groups were 1.7 years (interquartile range 0.5-4.9) and 6.8 years (interquartile range 1.9-13.4), respectively (p = 0.03). Overall, 5-year survival, freedom from redo aortic valve replacement, and freedom from pulmonary valve replacement were similar between groups. CONCLUSION: The bovine jugular vein conduit and pulmonary homograft have favourable mid-term durability when used for right ventricular outflow tract reconstruction for the Ross operation. The bovine jugular vein conduit may be a suitable replacement for appropriately sized patients undergoing a Ross aortic valve replacement, though longer follow-up is needed.

Outcomes after Ross procedure in adult patients: A meta-analysis and microsimulation.

OBJECTIVE: We conducted a meta-analysis to estimate the risk of adverse events, life expectancy, and event-free life expectancy after the Ross procedure in adults. METHODS: We searched databases for reports evaluating the Ross procedure in patients aged more than or equal to 16 years of age. A microsimulation model was used to evaluate age- and gender-specific life expectancy for patients undergoing the Ross procedure. RESULTS: Data were pooled from 63 articles totaling 19 155 patients from 20 countries. Perioperative mortality was 2.5% (95% confidence interval [CI]: 1.9-3.1; N = 9978). We found a mortality risk of 5.9% (95% CI: 4.8-7.2) at a mean follow-up of 7.2 years (N = 7573). The rate of perioperative clinically significant bleeding was 1.0% (95% CI: 0.1-3.0); re-exploration for bleeding 4.6% (95% CI: 3.1-6.3); postoperative clinically significant bleeding from 30 days until a mean of 7.1 years was 0.5% (95% CI: 0.2-1.0). At a mean of 6.9 years of follow-up, reintervention rate of any operated valve was 7.9% (95% CI: 5.7-10.3). The risk of valve thrombosis was 0.3% (95% CI: 0.2-0.5) at 7.6 years; peripheral embolism 0.3% (95% CI: 0.2-0.4) at 6.4 years; stroke 0.9% (95% CI: 0.7-1.2) at 6.5 years; and endocarditis 2.1% (95% CI: 1.6-2.6) at 8.0 years. Microsimulation reported a 40-year-old undergoing the Ross procedure to have a life expectancy of 35.4 years and event-free life expectancy of 26.6 years. CONCLUSIONS: Ross procedure in nonelderly adults is associated with low mortality and low risk of adverse events both at short- and long-term follow-up. The surgical community must prioritize a large, expertize-based randomized controlled trial to definitively address the risks and benefits of the Ross procedure compared to conventional aortic valve replacement.

Congenital Pulmonary Vein Stenosis: Encouraging Mid-term Outcome.

Congenital pulmonary vein stenosis (PVS) is a rare entity with limited outcome literature. Multiple interventional approaches have evolved including surgical and catheterization techniques. Our objective is to report our center experience and to compare short-term and mid-term outcomes among these therapeutic modalities. Retrospective study on 23 patients (n = 23) with PVS that required intervention over the last 13 years (2000-2013). Patients were divided into three groups based on type of initial intervention. Of these, 10 (43.5%) had balloon angioplasty, 3 (13.0 %) had surgical dilation, and 10 (43.5%) had surgical marsupialization. Mortality and number of re-interventions were our primary outcomes. Mean age at diagnosis was 10.9 ± 18.4 months. Mean age at initial intervention was 14.5 ± 18.0 months. Mean pre- and post-initial intervention PVS gradients were 9.2 ± 3.4 and 3.4 ± 2.2 mmHg, respectively. Mean survival time and re-intervention-free survival time were 4.8 ± 4.0 and 2.8 ± 3.4 years. No statistical significance was found between the interventions with respect to survival time (p = 0.52) and re-intervention free time (p = 0.78). High initial pre- and post-intervention gradients were significantly associated with re-intervention-free survival (p = 0.01 and p = 0.03, respectively). Patients with bilateral disease have increased mortality (p = 0.01) and decreased 5-year survival (p = 0.009) compared to patients with unilateral disease irrespective of type of intervention. No statistically significant difference in mortality or re-intervention rate was present among these different therapeutic modalities. This study has the longest follow-up so far reported in the current literature (58 months) with overall survival of 78%.

Persistent Heart Failure Despite Medical Therapy Leading to a Diagnosis of Cardiac Amyloidosis.

Cardiac amyloidosis is restrictive cardiomyopathy, commonly classified as either light-chain amyloidosis (AL) or transthyretin amyloidosis (ATTR), which can be further subdivided into wild-type (systemic senile amyloidosis) and hereditary ATTR amyloidosis. Advanced-stage, silent, and clinically undiagnosed amyloidosis has a poor prognosis, with a survival rate of six months and up to five years. We present a 72-year-old female with a past medical history of heart failure, with preserved ejection fraction, atrial fibrillation, systemic lupus erythematosus (SLE), and stage 3b chronic kidney disease, who presented with persistent shortness of breath, lower extremity pitting edema, jugular venous distension, and dyspnea despite optimal medical therapy. The patient was diagnosed with preserved heart failure in the past and was on guideline-directed medical therapy for over five years with no history of cardiac disease in the family. The patient's previous echocardiogram revealed an ejection fraction of 65%. In order to determine the etiology of the patient's cardiomyopathy, she underwent cardiac magnetic resonance imaging (CMR), monoclonal gammopathy testing, and a Technetium pyrophosphate (99mTc-PYP) scintigraphy, of which the latter two were unrevealing. The CMR revealed increased wall thickness and multiple segments of midmyocardial to subendocardial late gadolinium enhancement, suggestive of infiltrative disease. Due to inconclusive testing, the patient underwent an endomyocardial biopsy and was determined to have wild-type, systemic senile amyloidosis, which held a poor prognosis. The patient was started on tafamidis, a new Food and Drug Administration (FDA)-approved therapy for systemic senile amyloidosis, and was discharged on the new medication, with frequent follow-up visits scheduled. Current treatment guidelines for cardiac amyloidosis include loop diuretics and spironolactone. Medications such as beta-blockers, angiotensin-converting enzyme inhibitors, and calcium channel blockers are not clinically effective. There are currently new medications on the horizon, such as tafamidis, which stabilizes the transthyretin tetramer and reduces the formation of amyloid. This case highlighted that patients who have persistent symptoms of heart failure, despite guideline-directed medical therapy, and without a history of genetic cardiac conditions, may also have a diagnosis of cardiac amyloidosis. Cardiac amyloidosis is often misdiagnosed or diagnosed late in the disease course; therefore, there is a need for increasing awareness of early diagnosis and treatment, including new FDA-approved medications for a better chance of survival.

Bentall versus valve-sparing aortic root replacement for root pathology with moderate-to-severe aortic insufficiency: a propensity-matched analysis.

OBJECTIVES: To examine short- and long-term outcomes of patients with moderate-to-severe aortic insufficiency (AI) undergoing either a Bentall aortic root replacement (ARR) or valve-sparing root replacement (VSRR). METHODS: A two-centre retrospective database of patients undergoing ARR from 2004 to 2021 was reviewed. Patients <18 years old were excluded. A total of 1527 adult patients underwent Bentall ARR (n = 1150, 75%) or VSRR (n = 377, 25%). Propensity score matching based on preoperative comorbidities was used and 195 matched pairs were identified. Perioperative outcomes, reoperation rates, recurrence of AI and long-term survival were evaluated. RESULTS: ARR patients had more concomitant ascending aortic replacement (35% vs 20%, P = 0.002) and shorter cardiopulmonary bypass (189 vs 233 min, P < 0.0001) and aortic cross-clamp (170 vs 204 min, P < 0.0001) times than the VSRR group. Postoperatively, outcomes were similar between groups, including stroke (3% vs 2%) and in-hospital mortality (1.5% vs 2.1%), all P > 0.05. Indications for and rates of reoperation (4% vs 5%, P = 0.62) of the aortic valve and proximal aorta were similar between ARR and VSRR groups with reoperations occurring a mean of 3.2 years after initial root replacement. The ARR group had less moderate-to-severe AI than the VSRR group (1.6% vs 14%, P = 0.002) a mean of 3 years after surgery. Ten-year survival was similar between ARR (84%) and VSRR (82%) (P = 0.69) groups. CONCLUSIONS: Both ARR and VSRR can be performed with acceptable short- and long-term outcomes in patients with moderate-to-severe AI.

Transition from standard robotic prostatectomy to Retzius-sparing prostatectomy: feasibility and early outcomes.

BACKGROUND AND OBJECTIVE: Retzius-sparing robotic-assisted radical prostatectomy (rsRARP) has gained popularity due to superior early continence outcomes compared to standard robotic prostatectomy (sRARP). We evaluate the results of a single surgeon who transitioned from sRARP to rsRARP and compare oncologic and functional outcomes. METHODS: We retrospectively reviewed all prostatectomies performed by a single surgeon between June 2018 and October 2020. Perioperative, oncologic, and functional data were collected and analyzed. Patients who underwent sRARP were compared with those who underwent rsRARP. RESULTS: Both groups contained 37 consecutive patients each. Preoperative patient characteristics and biopsy results were similar between the two groups. Perioperative outcomes were significant for longer operative room time and higher proportion of T3 tumors in the rsRARP group. Thirty-day complication and readmission rates were similar between groups. There was no difference in early oncologic outcomes, including positive surgical margin rate, biochemical recurrence, and need for adjuvant or salvage treatments. The time to urinary continence and immediate continence rate was superior in the rsRARP group. CONCLUSIONS: The Retzius-sparing approach can be safely adopted by surgeons experienced in sRARP without compromising early oncologic outcomes and with the benefit of improved early continence recovery.

True redo-aortic root replacement versus root replacement after any previous surgery.

Objective: The impact of previous aortic root replacement (True-Redo) versus any previous operation (Any-Redo) on outcomes after reoperative aortic root replacement (redo-ROOT) is largely unknown. In this first multi-institutional study, the clinical impact True-Redo versus Any-Redo in the setting of redo-ROOT was reviewed. Methods: From 2004 to 2021, 822 patients underwent redo-ROOT at 2 major academic centers: 638 Any-Redo and 184 True-Redo. Matching based on preoperative demographics and concomitant operations resulted in 174 matched pairs. An independent risk factor analysis was performed to determine risk factors for early and late mortality. Results: Patients in the True-Redo group were younger, at 49.9 ± 15.1 versus 55.3 ± 14.7 years, P < .001. Concomitant operations were largely similar between the 2 groups, P > .05. Median cardiopulmonary bypass time (P < .001) and aortic crossclamp time (P = .03) were longer for True-Redo group. In-hospital mortality was 13% (109) and was without significant difference between groups, P = .41. Ten-year survival was 78% versus 76% for True-Redo versus Any-Redo groups respectively, P = .7. Landmark survival analysis at 4 years' postoperatively on the matched groups found that patients in the True-Redo group had improved survival outcomes (P = .046). Risk factors of in-hospital mortality consisted of older age (P < .0001), lower ejection fraction (P = .02), and male patient (P = .0003). Conclusions: Clinical outcomes following redo-ROOT are excellent. Performance of a True-Redo-ROOT does not result in worse in-hospital morbidity or mortality and has improved survival benefit at midterm follow-up when compared with patients in the Any-Redo group. The decision to perform a redo-ROOT must be taken seriously and must be individualized in a patient-specific manner for optimal outcomes.

Bioprosthetic Pulmonary Valve Dysfunction in Congenital Heart Disease.

BACKGROUND: We aim to identify the incidence and timing of dysfunction and failure of stented bioprosthetic valves in the pulmonary position in congenital heart disease patients. METHODS: A total of 482 congenital heart disease patients underwent 484 stented bioprosthetic pulmonary valve implantations between 2008 and 2018. There were 164 porcine valves (Porcine) and 320 bovine pericardial valves (Pericardial) implanted. Primary endpoints were survival, valve dysfunction, and valve failure. RESULTS: Pericardial valves were implanted in older patients (22.0, interquartile range [IQR] 14-33 vs 16.0, IQR 11-23 years, P < 0.001). Five-year survival (96.7% vs 97.9%) for the Pericardial and Porcine groups, respectively, were similar, P > 0.05. Forty-six (34%) Porcine and 75 (27%) Pericardial group patients met criteria for valve dysfunction at a median echocardiographic follow-up time of 7.43 years (IQR 4.1-9.5 years) and 3.26 years (IQR 1.7-4.7 years), respectively. More Pericardial group patients suffered from at least mild late PR while late median peak gradient was higher in the Porcine group, P < .001 for both. Risk factors for valve dysfunction included decreasing patient age for the entire cohort (hazard ratio [HR] 1.02, 95% confidence interval [CI] 1.00-1.04, P = .015) and lack of anticoagulation at discharge for the Porcine group (HR 3.06, 95% CI 1.03-9.10, P = .044) but not the Pericardial group. Five-year cumulative incidence of dysfunction was 39% for the Pericardial group and 17% for the Porcine group. CONCLUSIONS: Porcine stented and bovine pericardial stented valves can be implanted in the pulmonary position in all age groups safely. However, despite similar rates of valve failure, bovine pericardial stented valves have a higher incidence of valve dysfunction at mid-term follow-up.

Presentation of Sjogren Syndrome as Nodular Pulmonary Amyloidosis.

Sjogren syndrome is an autoimmune disorder that leads to dryness in the eyes and mouth. Nodular pulmonary amyloidosis is a localized amyloid deposition pathology commonly seen with monoclonal lymphoproliferative disorders. We present a patient who came in with dyspnea and was found to have nodular pulmonary amyloidosis on biopsy. Commonly associated lymphoproliferative pathologies were ruled out and on further workup, the patient was found to have Sjogren syndrome. This case demonstrates pulmonary nodular amyloidosis as a rare presentation of Sjogren syndrome in the setting of relatively well-controlled symptoms. Detection of pulmonary nodular amyloidosis should prompt evaluation of associated conditions such as malignancy and autoimmune disorders to guide further management.

Thrombotic Thrombocytopenic Purpura After Ad6.COV2.S Vaccination.

Thrombotic thrombocytopenic purpura (TTP) is caused by the deficiency of ADAMTS13, a von Willebrand factor cleaving protease, which results in thrombotic microangiopathy. It is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis leading to organ damage. It has an extremely high mortality rate if left untreated, making early diagnosis and treatment of the utmost importance. We report a case of TTP that developed after vaccination with Ad26.COV2.S COVID vaccine. We present a case of a 50-year-old African American female who presented with dyspnea one week after receiving the first dose of Ad26.COV2.S vaccine. Initial labs showed anemia, thrombocytopenia, and markers of intravascular hemolysis. The suspicion for thrombotic thrombocytopenic syndromes (TTS), vaccine-induced thrombotic thrombocytopenia (VITT), TTP, and Immune thrombocytopenic purpura (ITP) was high based on the history and laboratory results. Computed tomography (CT) of the chest and ultrasound of bilateral lower extremities did not show any evidence of thrombosis. The absence of thrombosis in the presence of a high PLASMIC score increased the suspicion of TTP over the other differentials. Diagnosis of TTP was confirmed when the ADAMTS13 level was low with an elevated autoantibody inhibitor level. The patient underwent treatment with corticosteroids, plasmapheresis, and rituximab with improvement in symptoms and platelet count. TTP and VITT are the possible differential diagnosis for a patient presenting with anemia, thrombocytopenia, and signs of hemolysis after vaccination with Ad26.COV2.S. It is necessary to differentiate these two clinical entities as the management varies based on the diagnosis.

Using Intraoperative Portable CT Scan to Minimize Reintervention Rates in Percutaneous Nephrolithotomy: A Prospective Trial.

Background and Purpose: More than 40% of patients undergoing percutaneous nephrolithotomy (PCNL) are left with residual stone fragments and often require secondary procedures. Portable CT (PCT) technology allows surgeons to obtain intraoperative cross-sectional imaging, identify and extract residual stones immediately, and thereby reduce the need for subsequent procedures. This prospective trial evaluates how incorporation of PCT during PCNL affects perioperative outcomes. Patients and Methods: We prospectively enrolled eligible patients undergoing initial PCNL for this trial (n = 60), which entailed a single intraoperative CT abdomen and ipsilateral antegrade ureteroscopy when the surgeon felt stone treatment was visually complete. If residual fragments were identified, the surgeon continued nephroscopy to find and remove them; if not, the procedure was concluded. These patients were compared with a retrospective cohort (n = 174) who underwent initial PCNL with postoperative imaging performed the following day. Results: The two cohorts had similar demographic properties and stone characteristics, and location of percutaneous access. In the prospective arm, 50% of intraoperative PCT scans identified residual fragments, prompting continuation of surgery to remove them. This cohort had significantly higher stone-free rate (82% vs 36%, p < 0.01), lower rate of planned reintervention (7% vs 32%, p < 0.01), lower rate of urgent presentation with ureteral obstruction (0% vs 7%, p = 0.04), lower total CT-based effective radiation dose (8.4 mSv vs 14.6 mSv, p < 0.01), and shorter length of stay (2.3 days vs 3.5 days, p < 0.01) when compared with the retrospective cohort that did not use intraoperative PCT. Conclusions: Obtaining an intraoperative PCT scan during PCNL can substantially improve perioperative outcomes. Further evaluation of this modality through a randomized controlled trial is warranted. Clinical Trial Registration Number: NCT04556396.

Socioeconomic Disparities and Risk Factors in Patients Presenting With Ischemic Priapism: A Multi-Institutional Study.

OBJECTIVES: To evaluate contemporary clinical presentations of priapism, their association with socioeconomic characteristics, and the role of prescribing providers in priapism episodes in a large cohort of patients managed at 3 major academic health systems. METHODS: We identified all consecutive patients presenting with ischemic priapism to the emergency departments of three major academic health systems (2014 -2019). Demographic characteristics, priapism etiologies, and clinical management were evaluated. Univariable and multivariable analyses were used to assess the contribution of socioeconomic characteristics and the role of prescribing providers in priapism episodes. RESULTS: We identified 102 individuals with a total of 181 priapism encounters. Hispanic race, lower income quartile, sickle-cell disease, and illicit drug use were associated with increased risk of recurrent episodes. Of ICI users, 57% received their prescriptions from non-urological medical professionals (NUMPs); the proportion with recurrent episodes was higher for NUMPs compared to urologists (24% vs 0%, P = 0.06) with no demographic differences identified between patients treated by either group. CONCLUSION: Socioeconomic disparities exist among patients presenting with recurrent episodes of priapism, potentially highlighting systemic issues with access to care and patient education. With most patients who developed ischemic priapism from ICI being prescribed these medications by NUMPs, further investigation is required to elucidate the prescribing and counseling patterns of these providers. Increased awareness of disparities and complications may improve patient safety.