A successful desensitization in an adult patient with ARA-C infusion reaction.

INTRODUCTION: Cytarabine (ARA-C) is an antimetabolite agent used especially in the treatment of hematologic malignancies. Infusion reactions have an important place among the side effects that may occur due to treatment. Clinical findings of infusion reactions resemble allergic reactions. CASE REPORT: 47-year-old male patient with a diagnosis of B-cell Acute Lymphoblastic Leukaemia developed infusion reaction during ARA-C treatment. MANAGEMENT & OUTCOME: There was no alternative treatment option for his existing malignant disease, we decided ARA-C desensitization. DISCUSSION: We would like to describe a successful desensitization protocol in an adult patient who experienced a reaction during ARA-C infusion.

The efficiency of cinacalcet treatment in delaying parathyroidectomy in a case with neonatal severe hyperparathyroidism caused by homozygous mutation in the CASR gene.

Neonatal severe hyperparathyroidism (NSHPT) causes severe hypercalcaemia, metabolic bone disease, and potential neurodevelopmental deficits, all of which can be life-threatening. The use of calcimimetic agents can prevent or delay technically difficult parathyroidectomy in the newborn period. We present a 6-day-old male infant who presented with poor feeding, weight loss, and severe hypotonia. His total serum calcium and parathyroid hormone levels were very high (23.6 mg/dl and 1120 ng/dl, respectively). Based on these findings, the patient was diagnosed with NSHPT and was started on cinacalcet therapy until the genetic analysis results were available. Genetic analysis revealed a previously reported homozygous mutation in the CASR gene that was unresponsive to cinacalcet therapy in the literature. However, a normocalcaemic state unexpectantly occurred, which could be maintained with low calcium formula and cinacalcet therapy up to 13 months of age in the patient. Nevertheless, hypercalcaemia developed 2 months after he started a normal calcium-containing diet. Therefore, the patient underwent total parathyroidectomy at 17 months of age. We would like to emphasize, in light of this case, that cinacalcet treatment may be considered as first-line therapy for delaying parathyroidectomy in all cases with NSHPT, even in those who have an unresponsive cinacalcet CASR gene mutation.

Comparison of left ventricular unloading strategies on venoarterial extracorporeal life support.

OBJECTIVES: Our goal was to compare the haemodynamic effects of different mechanical left ventricular (LV) unloading strategies and clinical outcomes in patients with refractory cardiogenic shock supported with venoarterial extracorporeal membrane oxygenation (VA-ECMO). METHODS: A total of 448 patients supported with VA-ECMO for refractory cardiogenic shock between 1 March 2015 and 31 January 2020 were included and analysed in a single-centre, retrospective case-control study. Fifty-three patients (11.8%) on VA-ECMO required LV unloading. Percutaneous balloon atrial septostomy (PBAS), intra-aortic balloon pump (IABP) and transapical LV vent (TALVV) strategies were compared with regards to the composite rate of death, procedure-related complications and neurological complications. The secondary outcomes were reduced pulmonary capillary wedge pressure, pulmonary artery pressure, central venous pressure, left atrial diameter and resolution of pulmonary oedema on a chest X-ray within 48 h. RESULTS: No death related to the LV unloading procedure was detected. Reduction in pulmonary capillary wedge pressure was highest with the TALVV technique (17.2 ± 2.1 mmHg; P < 0.001) and was higher in the PBAS than in the IABP group; the difference was significant (9.6 ± 2.5 and 3.9 ± 1.3, respectively; P = 0.001). Reduction in central venous pressure with TALVV was highest with the other procedures (7.4 ± 1.1 mmHg; P < 0.001). However, procedure-related complications were significantly higher with TALVV compared to the PBAS and IABP groups (50% vs 17.6% and 10%, respectively; P = 0.015). We observed no significant differences in mortality or neurological complications between the groups. CONCLUSIONS: Our results suggest that TALVV was the most effective method for LV unloading compared with PBAS and IABP for VA-ECMO support but was associated with complications. Efficient LV unloading may not improve survival.

Percutaneous ultrasound-guided versus bronchoscopy-guided dilatational tracheostomy after median sternotomy: A case-control study.

BACKGROUND: In this study, we aimed to compare ultrasoundguided versus bronchoscopy-guided percutaneous dilatational tracheostomy outcomes in critically ill adult patients undergoing a median sternotomy. METHODS: Between January 2015 and December 2020, a total of 54 patients (17 males, 37 females; mean age: 54.9±13.1 years; range, 39 to 77 years) who underwent elective ultrasound- or bronchoscopy-guided percutaneous dilatational tracheostomy after a median sternotomy were included. We compared the ultrasound-guided group (n=25) with the bronchoscopy-guided group (n=29) regarding all-cause mortality and complications. Safety assessments included major and minor bleeding, procedural hypoxic or hypotensive event, cardiac dysrhythmias, tracheal injury, damage to adjacent structures, and requirement of conversion to open surgical tracheostomy. RESULTS: No tracheostomy procedure-related death was observed in either group. The median time for tracheostomy was 13 (range, 8 to 17) min in the ultrasound-guided group and 10 (range, 7 to 15) min in the bronchoscopy-guided group (p=0.387). There was no need for conversion between the two methods or conversion to surgical tracheostomy for any patient. The overall complication rates did not significantly differ between the groups (p=0.15). CONCLUSION: Ultrasound-guided percutaneous dilatational tracheostomy can be safely performed in patients undergoing sternotomy. Complication rates of the procedure are similar to those guided with bronchoscopy.

Right Ventricular Strain to Assess Early Right Heart Failure in the Left Ventricular Assist Device Candidate.

PURPOSE OF REVIEW: Right heart failure (RHF) following left ventricular assist device implantation (LVAD) remains the primary cause of postoperative mortality and morbidity, and prediction of RHF is the main interest of the transplantation community. In this review, we outline the role and impact of right ventricular strain in the evaluation of the right ventricle function before LVAD implantation. RECENT FINDINGS: Accumulating data suggest that measurement of right ventricular longitudinal strain (RVLS) has a critical role in predicting RHF preoperatively and may improve morbidity and mortality following LVAD implantation. However, the significant intraobserver, interobserver variability, the lack of multicenter, prospective studies, and the need for a learning curve remain the most critical limitations in the clinical practice at present. This review highlighted the importance of right ventricular strain in the diagnosis of RHF preoperatively and revealed that RVLS might have a crucial clinical measurement for the selection and management of LVAD patients in the future with the more extensive multicenter studies.

Right ventricular free wall longitudinal strain and stroke work index for predicting right heart failure after left ventricular assist device therapy.

OBJECTIVES: Right heart failure (RHF) is an important prognostic factor in continuous-flow left ventricular assist device (LVAD) therapy. We aimed to assess the clinical variables associated with RHF after LVAD implantation and to compare their performance against currently available RHF predictive scoring systems. METHODS: The study cohort comprised 57 patients who underwent LVAD therapy between January 2012 and May 2018 in our centre. The mean age of the patients was 39.9 ± 18.3 years, and 43 (81.1%) of them were men. Thirty-eight patients (66.6%) were in the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile I or II. The study cohort was divided into the patients with RHF postoperatively (n = 20, 35.1%) and without RHF (n = 37, 64.9%). RESULTS: Independent predictors for RHF were preoperative right ventricular ejection fraction <25% [odds ratio (OR) 4.68, 95% confidence interval (CI) 1.41-15.5; P = 0.01], right ventricular stroke work index <400 mmHg ml-1 (OR 3.73, 95% CI 1.01-13.7; P = 0.04), right ventricular outflow tract systolic excursion <7 mm (OR 1.55, 95% CI 0.31-0.84; P = 0.002), right ventricular outflow tract fractional shortening <15% (OR 1.62, 95% CI 0.34-0.78; P = 0.02), right ventricular free wall longitudinal strain ≤19% (OR 3.13, 95% CI 1.01-2.43; P = 0.003), right ventricular fractional area change <27% (OR 3.71, 95% CI 1.15-11.9; P = 0.02) and prealbumin <14 mg/dl (OR 3.45, 95% CI 1.07-11.03; P = 0.03). Modest diagnostic performance for RHF was detected in 4 of 7 validated scoring systems with resulting area under the curve values of 0.70 (95% CI 0.55-0.84; P = 0.001) for the Seattle Heart Failure Model; 0.68 (95% CI 0.49-0.81, P = 0.03) for the Fitzpatrick's; 0.68 (95% CI 0.53-0.83, P = 0.028) for the acute physiology and chronic health evaluation (APACHE) II; and 0.66 (95% CI 0.50-0.82, P = 0.04) for the model for end-stage liver disease scoring systems. However, we found best discrimination performance of the score with a resulting area under the curve value of 0.94 (95% CI 0.55-0.89, P = 0.03) for right ventricular free wall longitudinal strain ≥-15.5% and 0.82 for right ventricular stroke work index <400 mmHg ml-1 m-2 in predicting RHF. CONCLUSIONS: Right ventricular free wall longitudinal strain ≥-15.5% and right ventricular stroke work index <400 mmHg ml-1 m-2 were independent predictors of RHF following LVAD implantation. Currently available prediction risk scores had the modest power of accuracy in the low INTERMACS profile Turkish population.

Targeted Minimally Invasive Parathyroidectomy for Ectopic Aortopulmonary Adenoma Under Gamma Probe Guidance.

We report a case of 53-year-old woman with the parathyroid adenoma (PA) located in the aortopulmonary window with an aberrant right subclavian artery. Her preoperative calcium level was 11.3 mg/dL (reference range = 8.8-10.6 mg/dL). The parathyroid hormone level was significantly elevated at 127.4 pg/mL (reference range = 12-88 pg/mL) as measured on immunoradiometric assay. Dual-phase technetium-99m-labeled sestamibi parathyroid scintigraphy and fluorine-18 F-fluorocholine positron emission tomography/computed tomography revealed a mediastinal tumor measuring 3 × 5 × 6 mm located anterolateral to the aortopulmonary window. Minimally invasive removal of PA was performed through 3.5-cm reverse J-shaped partial upper sternotomy to the third intercostal space. Postoperative recovery was uneventful, and serum calcium and parathyroid hormone levels normalized within 24 hours of surgery. Our case represents the rare occurrence of a mediastinal PA associated with aberrant right subclavian artery treated with targeted minimally invasive approach using different imaging modalities including technetium-99m-sestamibi scintigraphy and fluorine-18 F-fluorocholine positron emission tomography/computed tomography, and intraoperative use of gamma probe for precise localization PA.

Sutureless aortic valve replacement with concomitant valvular surgery.

OBJECTIVES: Sutureless aortic valve replacement (SU-AVR) is an alternative technique to standard aortic valve replacement. We evaluated our experience with the Perceval SU-AVR with concomitant mitral valve surgery, with or without tricuspid valve surgery, and aimed to discuss the technical considerations. METHODS: From January 2013 through June 2016, 30 patients with concomitant severe mitral valve disease, with or without tricuspid valve disease, underwent SU-AVR with the Perceval prosthesis in a single center. RESULTS: The mean age was 73.0 ± 6.6 years, ranging from 63 to 86 years, and 60% (n = 18) were male. Mean logistic EuroScore of the study cohort was 9.8 ± 4.6. Concomitant procedures consisted of mitral valve repair (n = 8, 26.6%), mitral valve replacement (n = 22, 73.3%), tricuspid valve repair (n = 18, 60%), tricuspid valve replacement (n = 2, 6.6%), and cryoablation for atrial fibrillation (n = 21, 70%). Median prosthesis size was 25 mm (large size). At 1 year, there were 2 deaths from noncardiac causes. One patient (3.3%) had third-degree atrioventricular block requiring permanent pacemaker implantation. Three patients (10%) had intraoperative supra-annular malpositioning of the aortic prosthesis, which was safely removed and reimplanted in all cases. Mean follow-up was 18 ± 4.5 for months (maximum 3 years). During the postoperative period, sinus rhythm restoration rate in patients who underwent the cryo-maze procedure was 76.1% (n = 16) at discharge. There was no structural valve deterioration or migration of the prosthesis at follow-up. CONCLUSIONS: Perceval SU-AVR is a technically feasible and safe procedure in patients with severe aortic stenosis with good results even in the presence of multivalvular disease and atrial fibrillation surgery.

Increased free Zn2+ correlates induction of sarco(endo)plasmic reticulum stress via altered expression levels of Zn2+ -transporters in heart failure.

Zn2+ -homoeostasis including free Zn2+ ([Zn2+ ]i ) is regulated through Zn2+ -transporters and their comprehensive understanding may be important due to their contributions to cardiac dysfunction. Herein, we aimed to examine a possible role of Zn2+ -transporters in the development of heart failure (HF) via induction of ER stress. We first showed localizations of ZIP8, ZIP14 and ZnT8 to both sarcolemma and S(E)R in ventricular cardiomyocytes (H9c2 cells) using confocal together with calculated Pearson's coefficients. The expressions of ZIP14 and ZnT8 were significantly increased with decreased ZIP8 level in HF. Moreover, [Zn2+ ]i was significantly high in doxorubicin-treated H9c2 cells compared to their controls. We found elevated levels of ER stress markers, GRP78 and CHOP/Gadd153, confirming the existence of ER stress. Furthermore, we measured markedly increased total PKC and PKCα expression and PKCα-phosphorylation in HF. A PKC inhibition induced significant decrease in expressions of these ER stress markers compared to controls. Interestingly, direct increase in [Zn2+ ]i using zinc-ionophore induced significant increase in these markers. On the other hand, when we induced ER stress directly with tunicamycin, we could not observe any effect on expression levels of these Zn2+ transporters. Additionally, increased [Zn2+ ]i could induce marked activation of PKCα. Moreover, we observed marked decrease in [Zn2+ ]i under PKC inhibition in H9c2 cells. Overall, our present data suggest possible role of Zn2+ transporters on an intersection pathway with increased [Zn2+ ]i and PKCα activation and induction of HF, most probably via development of ER stress. Therefore, our present data provide novel information how a well-controlled [Zn2+ ]i via Zn2+ transporters and PKCα can be important therapeutic approach in prevention/treatment of HF.

Tricuspid valve surgery in implantable cardiac electronic device-related endocarditis: Repair or replace?

BACKGROUND: The aim of this study was to investigate lead endocarditis-related tricuspid valve regurgitation, to identify underlying causes, and to report our surgical approaches to tricuspid valve endocarditis. METHODS: Between March 2010 and August 2016, medical records of a total of 43 patients (23 males, 20 females; mean age: 63.2±13.6 years; range 48 to 72 years) who underwent tricuspid valve surgery for severe tricuspid regurgitation caused by lead endocarditis, which was previously placed as an implantable cardiac electronic device were reviewed. We removed all systems including infected leads and generators, revised infected wounds and tissues, performed tricuspid valve surgery for lead endocarditis, and applied long-term intravenous antibiotic regimen for the culprit agent, as confirmed by the culture. RESULTS: Of 43 patients, 18 underwent tricuspid valve repair and 25 underwent tricuspid valve replacement for lead endocarditisrelated severe tricuspid valve regurgitation. During followup (range, 2 to 62 months), two patients required temporary mechanical support due to postoperative acute right heart failure, while eight patients died due to sepsis (n=6; 14%) and stroke (n=2; 4.6%) in the early postoperative period. The remaining patients showed significant improvement in signs and symptoms of heart failure. CONCLUSION: Our study results suggest that incompetent experience and inaccurate decision for valve repair may result in delayed valve replacement and prolonged operation time.

Comparison of aortic cross-clamping versus beating heart surgery in tricuspid valve repair.

BACKGROUND: The aim of this study was to evaluate the clinical outcomes of tricuspid valve repair using aortic cross-clamping versus using beating heart surgery. METHODS: A total of 208 patients (67 males, 141 females; mean age 61.5±9.2 years; range, 29 to 81 years) who underwent concomitant cardiac surgery and tricuspid valve repair between January 2007 and January 2016 at a single center were included. Two surgical strategies for tricuspid valve repair with aortic cross-clamping (n=102) or on beating heart (n=106) were compared. Primary endpoints were in-hospital mortality and the rate of permanent pacemaker placement after surgery. Secondary endpoints were cross-clamp and cardiopulmonary bypass times, postoperative inotropic support, temporary pacemaker requirement, and residual tricuspid regurgitation at discharge and at one year. RESULTS: Overall hospital mortality was 7% (n=14) (cross-clamping 7% vs. beating heart 7%; p>0.05). The mean cross-clamp and cardiopulmonary bypass times were significantly longer in the aortic cross-clamping group (p=0.0001). Also, a higher number of patients in this group needed inotropic support (78/102) than the beating heart group (57/106) (p<0.05). The rate of postoperative left bundle branch block was higher in the cross-clamping group (14% vs. 5%, respectively; p<0.05). The rate of permanent pacemaker placement was also significantly higher in the cross-clamping group than the beating heart group (11.8% vs. 2.8%, respectively; p<0.05). At discharge, residual >2 tricuspid regurgitation was more commonly seen in the cross-clamping group (16% vs. 3%, respectively; p=0.0023). At one year of follow-up, residual >2 tricuspid regurgitation was present in 22 patients (23%) in the aortic crossclamping group and in eight patients (8%) in the beating heart group (p=0.0048). CONCLUSION: Tricuspid valve repair on beating heart offers less inotropic support and a lower rate of postoperative permanent pacemaker placement requirement and residual tricuspid regurgitation, although both techniques yield similar postoperative clinical outcomes. These results support the use of tricuspid valve repair on a beating heart in concomitant left-sided valvular heart surgery.