Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

[A case of right heart failure: when it is not pulmonary embolism]. / Un caso di insufficienza cardiaca destra: quando l'embolia polmonare non è un'embolia polmonare.

A 66-year-old patient with recent instrumental findings (echocardiogram, cardiac magnetic resonance imaging) of right ventricular failure was hospitalized due to worsening signs and symptoms of right heart failure, while waiting for diagnostic definition. Pulmonary computed tomography angiography revealed findings compatible with bilateral pulmonary thromboembolism involving the main pulmonary artery. Anticoagulant therapy was initiated with initial benefit, partial relief of symptoms, and moderate improvement in right ventricular function. However, after 4 weeks, the patient was readmitted for recurrence of heart failure and signs of low cardiac output. Echocardiography showed the presence of a conspicuous, mobile, isoechoic mass occupying much of the main pulmonary artery, once again suggestive of thrombosis. The patient underwent surgical thromboendoarterectomy; postoperatively, the procedure was complicated by severe refractory heart failure unresponsive to pharmacological treatments and mechanical support, leading to death in the subsequent days. Unexpectedly, histological analysis revealed a primary angiosarcoma of the endothelium of the main pulmonary artery, a very rare cause of pulmonary artery obstruction generally associated with worst prognosis and presenting with clinical features similar to pulmonary thromboembolism.

Surgical Ablation for Atrial Fibrillation During Mitral Valve Surgery: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

Although surgical ablation has been shown to produce excellent outcomes at follow-up for patients with atrial fibrillation who underwent mitral valve replacement/repair (MVR), this procedure is not commonly performed. Our objective was to conduct a systematic review and meta-analysis to evaluate the outcomes of concomitant surgical ablation during MVR. Three databases were systematically reviewed for randomized clinical trials published by August 2022. The primary outcome was sinus rhythm (SR) at 12 months. Secondary outcomes included SR at discharge and 6 months, all-cause mortality, permanent pacemaker implantation, and stroke and thromboembolic events. A random-effects meta-analysis was performed, calculating odds ratios (ORs) for each outcome. Thirteen studies were included, involving 1,089 patients comparing patients who underwent either isolated MVR ("MVR-only") or concomitant surgical ablation during MVR ("MVR+Ablation"). The odds of SR were significantly higher in the MVR+Ablation group at discharge (OR 9.62, 95% confidence interval [CI] 4.87 to 19.02, I2 = 55%), at 6-month follow-up (OR 7.21, 95% CI 4.30 to 12.11, I2 = 34%), and at 1-year follow-up (OR 8.41, 95% CI 5.14 to 13.77, I2 = 48%). All-cause mortality was not different in the groups, as were stroke and thromboembolic events, whereas the odds of permanent pacemaker implantation were slightly higher in the MVR+Ablation group (OR 1.87, 95% CI 1.11 to 3.17, I2 = 0%). Concomitant surgical ablation during MVR showed excellent outcomes at short-term follow-up, despite a slightly higher rate of permanent pacemaker implantation. Further studies with longer follow-ups are needed to assess if the SR is maintained over the years.

Different valve types: tips and tricks for a totally endoscopic aortic valve replacement.

Endoscopic aortic valve replacement is a fascinating and rapidly growing surgical activity. In the setting of minimally invasive surgical procedures, compared with mitral and tricuspid procedures, aortic valve operations increase the challenge for several reasons. If one relies only on the thoracoscope, the surgical planning and set-up, including the placement of the working port and technical surgical manoeuvres like the aortic cross-clamp, the aortotomy and the aortorrhaphy can be difficult and may lead to severe complications or an increased rate of conversion to sternotomy. A well-developed preoperative decision-making process that includes knowledge of the prosthetic valve characteristics and their implications in an endoscopic environment is crucial for a successful endoscopic aortic valve programme. This video tutorial provides tips and tricks useful for endoscopic aortic valve replacement by paying attention to the patient's anatomy, to the available prosthetic valve types and to their impact on the surgical set-up.

De-Airing Maneuvers After Minimally Invasive and Robotic-Assisted Intracardiac Procedures.

In the setting of minimally invasive and robotic-assisted intracardiac procedures, de-airing requires further technical considerations due to limited access to the pericardial space and the subsequent difficulty of directly manipulating the heart. We summarize the technical steps for de-airing according to different cannulation strategies for minimally invasive and robotic-assisted intracardiac procedures.

De-Airing Maneuvers After Minimally Invasive and Robotic-Assisted Intracardiac Procedures

ABSTRACT In the setting of minimally invasive and robotic-assisted intracardiac procedures, de-airing requires further technical considerations due to limited access to the pericardial space and the subsequent difficulty of directly manipulating the heart. We summarize the technical steps for de-airing according to different cannulation strategies for minimally invasive and robotic-assisted intracardiac procedures.

Effects of preoperative high-oral protein loading on short- and long-term renal outcomes following cardiac surgery: a cohort study.

BACKGROUND: Post-cardiac surgery acute kidney injury (AKI) is associated with increased mortality. A high-protein meal enhances the renal blood flow and glomerular filtration rate (GFR) and might protect the kidneys from acute ischemic insults. Hence, we assessed the effect of a preoperative high-oral protein load on post-cardiac surgery renal function and used experimental models to elucidate mechanisms by which protein might stimulate kidney-protective effects. METHODS: The prospective "Preoperative Renal Functional Reserve Predicts Risk of AKI after Cardiac Operation" study follow-up was extended to postoperative 12 months for 109 patients. A 1:2 ratio propensity score matching method was used to identify a control group (n = 214) to comparatively evaluate the effects of a preoperative protein load and standard care. The primary endpoints were AKI development and postoperative estimated GFR (eGFR) loss at 3 and 12 months. We also assessed the secretion of tissue inhibitor of metalloproteases-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7), biomarkers implicated in mediating kidney-protective mechanisms in human kidney tubular cells that we exposed to varying protein concentrations. RESULTS: The AKI rate did not differ between the protein loading and control groups (13.6 vs. 12.3%; p = 0.5). However, the mean eGFR loss was lower in the former after 3 months (0.1 [95% CI - 1.4, - 1.7] vs. - 3.3 [95% CI - 4.4, - 2.2] ml/min/1.73 m2) and 12 months (- 2.7 [95% CI - 4.2, - 1.2] vs - 10.2 [95% CI - 11.3, - 9.1] ml/min/1.73 m2; p < 0.001 for both). On stratification based on AKI development, the eGFR loss after 12 months was also found to be lower in the former (- 8.0 [95% CI - 14.1, - 1.9] vs. - 18.6 [95% CI - 23.3, - 14.0] ml/min/1.73 m2; p = 0.008). A dose-response analysis of the protein treatment of the primary human proximal and distal tubule epithelial cells in culture showed significantly increased IGFBP7 and TIMP-2 expression. CONCLUSIONS: A preoperative high-oral protein load did not reduce AKI development but was associated with greater renal function preservation in patients with and without AKI at 12 months post-cardiac surgery. The potential mechanisms of action by which protein loading may induce a kidney-protective response might include cell cycle inhibition of renal tubular epithelial cells. Clinical trial registration ClinicalTrials.gov: NCT03102541 (retrospectively registered on April 5, 2017) and ClinicalTrials.gov: NCT03092947 (retrospectively registered on March 28, 2017).

Preoperative Renal Functional Reserve Predicts Risk of Acute Kidney Injury After Cardiac Operation.

BACKGROUND: Although acute kidney injury (AKI) frequently complicates cardiac operations, methods to determine AKI risk in patients without underlying kidney disease are lacking. Renal functional reserve (RFR) can be used to measure the capacity of the kidney to increase glomerular filtration rate under conditions of physiologic stress and may serve as a functional marker that assesses susceptibility to injury. We sought to determine whether preoperative RFR predicts postoperative AKI. METHODS: We enrolled 110 patients with normal resting glomerular filtration rates undergoing elective cardiac operation. Preoperative RFR was measured by using a high oral protein load test. The primary end point was the ability of preoperative RFR to predict AKI within 7 days of operation. Secondary end points included the ability of a risk prediction model, including demographic and comorbidity covariates, RFR, and intraoperative variables to predict AKI, and the ability of postoperative cell cycle arrest markers at various times to predict AKI. RESULTS: AKI occurred in 15 patients (13.6%). Preoperative RFR was lower in patients who experienced AKI (p < 0.001) and predicted AKI with an area under the receiver operating characteristic curve (AUC) of 0.83 (95% confidence interval [CI]: 0.70 to 0.96). Patients with preoperative RFRs not greater than 15 mL · min-1 · 1.73 m-2 were 11.8 times more likely to experience AKI (95% CI: 4.62 to 29.89 times, p < 0.001). In addition, immediate postoperative cell cycle arrest biomarkers predicted AKI with an AUC of 0.87. CONCLUSIONS: Among elective cardiac surgical patients with normal resting glomerular filtration rates, preoperative RFR was highly predictive of AKI. A reduced RFR appears to be a novel risk factor for AKI, and measurement of RFR preoperatively can identify patients who are likely to benefit from preventive measures or to select for use of biomarkers for early detection. Larger prospective studies to validate the use of RFR in strategies to prevent AKI are warranted. ClinicalTrials.gov identifier: NCT03092947, ISRCTN Registry: ISRCTN16109759.

Redo After Failure of Aortic Homografts With a Rapid Deployment Valve.

Homograft implantation in the aortic position was a common approach for full root aortic surgical procedures in the early 2000s. Reintervention after degeneration of such homografts remains a challenge. We report two cases of successful implantation of the Edwards Intuity Elite rapid deployment valve into patients with degeneration of existing aortic homograft implants leading to severe aortic regurgitation.

[Central venous catheter-related right atrial thrombosis in a patient with Hodgkin's lymphoma]. / Trombosi atriale destra correlata a catetere venoso centrale in un paziente con linfoma di Hodgkin.

Central venous catheters (CVC) are commonly used in clinical practice. Although long-term complications are uncommon, catheter-related right atrial thrombosis is a rare but potentially life-threatening one. The optimal management is still controversial. We report the case of a young woman affected by Hodgkin lymphoma with CVC-related right atrial thrombosis diagnosed during routine echocardiography. After initial anticoagulation treatment, she complicated with pulmonary embolism, and the mass was surgically removed via a minimally invasive approach with right minithoracotomy access. Surgery was well tolerated, without complications and with prompt recovery. This case confirms how CVC can lead to thrombosis in the right atrium and how this complication can rapidly deteriorate. Moreover, the possible treatment options for the successful management of this complication are discussed, along with the available literature, showing the advantages of a minimally invasive approach.

Cardiac surgery-associated acute kidney injury.

Cardiac surgery-associated acute kidney injury (CSA-AKI) is a common and serious postoperative complication of cardiac surgery requiring cardiopulmonary bypass (CPB), and it is the second most common cause of AKI in the intensive care unit. Although the complication has been associated with the use of CPB, the etiology is likely multifactorial and related to intraoperative and early postoperative management including pharmacologic therapy. To date, very little evidence from randomized trials supporting specific interventions to protect from or prevent AKI in broad cardiac surgery populations has been found. The definition of AKI employed by investigators influences not only the incidence of CSA-AKI, but also the identification of risk variables. The advent of novel biomarkers of kidney injury has the potential to facilitate the subclinical diagnosis of CSA-AKI, the assessment of its severity and prognosis, and the early institution of interventions to prevent or reduce kidney damage. Further studies are needed to determine how to optimize cardiac surgical procedures, CPB parameters, and intraoperative and early postoperative blood pressure and renal blood flow to reduce the risk of CSA-AKI. No pharmacologic strategy has demonstrated clear efficacy in the prevention of CSA-AKI; however, some agents, such as the natriuretic peptide nesiritide and the dopamine agonist fenoldopam, have shown promising results in renoprotection. It remains unclear whether CSA-AKI patients can benefit from the early institution of such pharmacologic agents or the early initiation of renal replacement therapy.

Cardiac surgery-associated acute kidney injury.

Cardiac surgery-associated acute kidney injury (CSA-AKI) is a common and serious postoperative complication of cardiac surgery requiring cardiopulmonary bypass (CPB), and it is the second most common cause of AKI in the intensive care unit. Although the complication has been associated with the use of CPB, the etiology is likely multifactorial and related to intraoperative and early postoperative management including pharmacologic therapy. To date, very little evidence from randomized trials supporting specific interventions to protect from or prevent AKI in broad cardiac surgery populations has been found. The definition of AKI employed by investigators influences not only the incidence of CSA-AKI, but also the identification of risk variables. The advent of novel biomarkers of kidney injury has the potential to facilitate the subclinical diagnosis of CSA-AKI, the assessment of its severity and prognosis, and the early institution of interventions to prevent or reduce kidney damage. Further studies are needed to determine how to optimize cardiac surgical procedures, CPB parameters, and intraoperative and early postoperative blood pressure and renal blood flow to reduce the risk of CSA-AKI. No pharmacologic strategy has demonstrated clear efficacy in the prevention of CSA-AKI; however, some agents, such as the natriuretic peptide nesiritide and the dopamine agonist fenoldopam, have shown promising results in renoprotection. It remains unclear whether CSA-AKI patients can benefit from the early institution of such pharmacologic agents or the early initiation of renal replacement therapy.

How to ensure a good flow to the arm during direct axillary artery cannulation.

We herein describe a simple and safe technique to avoid compartment syndrome/arm ischemia during direct right axillary artery cannulation, especially in patients who require long-term extracorporeal membrane oxygenation support.

Prophylactic intra-aortic balloon pump in high-risk patients undergoing coronary artery bypass grafting: a propensity score analysis.

The optimal use of prophylactic intra-aortic balloon pump (IABP) to prevent postcardiotomy low cardiac output syndrome (LCOS) is still debated and poorly defined. The aim of this study was to evaluate whether prophylactic IABP reduces the rate of postcardiotomy LCOS and improves the early outcome in hemodynamically stable, high-risk patients undergoing coronary artery bypass grafting (CABG). From May 2004 to August 2007, 141 consecutive risk patients underwent CABG. Of these 38 (27%) received prophylactic IABP. The remaining 103 patients underwent operation without preoperative insertion of the device. Prophylactic IABP patients were more likely to be younger (P<0.0001), had a recent myocardial infarction (P<0.0001), lower ejection fraction (P=0.006), and higher New York Heart Association (NYHA) functional class (P=0.05). After risk-adjusting for propensity score, prophylactic IABP patients had a lower incidence of postcardiotomy LCOS (adjusted OR 0.07, P=0.006), postoperative myocardial infarction (adjusted OR 0.04, P=0.04), a shorter length of hospital stay (10.4+/-0.8 vs. 12.2+/-0.6 days, P<0.0001) than those who did not receive IABP. This study shows that prophylactic IABP treatment for hemodynamically stable high-risk patients undergoing CABG may improve postoperative course reducing postcardiotomy LCOS, postoperative myocardial infarction and length of hospital stay.