Brachial artery transposition versus catheters as tertiary vascular access for maintenance hemodialysis: a single-center retrospective study

Some hemodialysis patients are not suitable for creation of an arteriovenous fistula (AVF) or arteriovenous graft (AVG). However, they can receive a tunneled cuffed central venous catheter (tcCVC), but this carries risks of infection and mortality. We aimed to evaluate the safety and effectiveness of brachial artery transposition (BAT) versus those of tcCVC. This retrospective study evaluated hemodialysis patients who underwent BAT or tcCVC placement because of severe heart failure, hand ischemia, central venous stenosis or occlusion, inadequate vessels for creating standard arteriovenous access, or limited life expectancy. The primary outcome was whole access circuit patency. Thirty-eight patients who underwent BAT and 25 who underwent tcCVC placement were included. One-year patency rates for the whole access circuit were 84.6% and 44.9% in the BAT and tcCVC groups, respectively. The BAT group was more likely to maintain patency (unadjusted hazard ratio: 0.17, 95% confidence interval: 0.05–0.60, p = 0.006). The two groups did not have significantly different overall survival (log-rank p = 0.146), although severe complications were less common in the BAT group (3% vs. 28%, p = 0.005). Relative to tcCVC placement, BAT is safe and effective with acceptable patency in hemodialysis patients not suitable for AVF or AVG creation.

Temporary Veno-Venous ECMO for Acute Respiratory Illness in Pediatric Berlin Heart Patient

We report the unique case of a 2-year-old male with severe heart failure requiring mechanical circulatory support with a left ventricular assist device, who developed adenovirus pneumonitis infection requiring veno-venous extracorporeal membrane oxygenation (ECMO) support. He progressed to acute respiratory failure and refractory hypoxemia despite intubation with maximum respiratory support. The patient was placed on ECMO with improvement in lung function over four days with subsequent successful decannulation. During the ECMO run, anticoagulation required escalation given the increased circuit surface area. Patient has since recovered and undergone heart transplantation.

Complications of Surgical Treatment of Aortic Arch Hypoplasia in Infants

Aortic arch surgery in neonatal patients remains problematic despite the constant evolution and improvement of treatment methods. Even after successful correction, complications associated with aortic arch reconstruction are more common in young children. This is especially true for newborns and infants with concomitant complex congenital intracardiac abnormalities. Despite the risk of postoperative complications, the risk of surgical treatment of aortic arch hypoplasia is lower than the cumulative hazards associated with the natural course of this defect. That is why pediatric cardiologists are obliged to constantly monitor the operated patients in order to timely identify and treat complications. The aim. To analyze the complications in the early and remote postoperative periods after the reconstruction of the aortic arch in the neonatal period. Materials and methods. The work is based on a study of 445 patients under 1 year of age, who underwent surgical treatment of aortic arch hypoplasia from 2010 to 2019. The criterion for inclusion in the study group was the presence of isolated hypoplasia of the aortic arch and combination with other defects, which were corrected by two-ventricular repair. The majority of the treated patients were male (284 [63.8% of the total number of patients]). The median age of the patients was 0.7 months (0.3; 2.7). The median body weight of the patients was 3.7 kg (3.25; 4.59). Results. At the hospital stage, 12 patients died, which accounted for 3.1% of the entire cohort of operated patients. Complications occurring at the hospital stage were recorded in 75 (16.8%) patients. In the long term, a complicated course was observed in 72 (16.6%) children. Among them, the most frequent complications were: respiratory failure requiring prolonged mechanical ventilation (36.9%), dilated sternum due to severe heart failure (17.4%), diaphragmatic paresis (8.7%), chylothorax (5.4%). In the long term, 47 (10.5%) patients developed aortic arch restenosis, which required reinterventions. The proportion of patients without reoperations in the follow-up period according to the KaplanMeier analysis was 93.4% after 1 year, 91.2% after 4 years, and 76.5% after 9 years. Residual hypertension requiring medical treatment was reported in 59 (13.2%) patients. Conclusions. Aortic arch hypoplasia is a complex congenital heart disease; its surgical treatment is accompanied by the development of complications both in the early and in the long-term period. The main complications at the hospital stage were respiratory and heart failures which were associated with the presence and correction of concomitant congenital heart defects. The main complication of the follow-up period was reobstruction at the level of the aortic arch. Anatomical correction of reobstruction is safe with both endovascular and surgical treatments.

Tripeptidyl Peptidase 1 (TPP1) Deficiency in a 36-Year-Old Patient with Cerebellar-Extrapyramidal Syndrome and Dilated Cardiomyopathy

We report on a 36-year-old man with cerebellar-extrapyramidal syndrome and severe heart failure because of dilated cardiomyopathy of unknown origin. Dysarthria and cardiac arrhythmia began at early childhood (4 years of age). Brain MRI (28 years of age) demonstrated severe cerebellar atrophy. At the age 32, he presented with dysarthria, ataxia, dystonia, and tremor of the right hand, bilateral slowed neural conduction in the visual pathways, and decreased mental acuity. At the age of 33 years, the patient underwent cardiac transplantation because of severe dilated cardiomyopathy. In the TPP1 gene, biallelic variants were identified: previously reported p.(Leu13Pro) and novel p.(Tyr508Cys) variant. Additionally, hemizygous novel missense variant in the ABCD1 gene was inherited from the mother p.(Arg17His). Normal very-long-chain fatty acids (VLCFA) levels both in patient and his mother excluded ABCD1 mutation as the pathogenic one. Tripeptidyl peptidase 1 (TPP1) activity was reduced (8,8 U/mg protein/h; reference range: 47.4 ± 10.7). In light microscopy the biopsy specimens obtained from explanted heart showed severe myocyte hypertrophy with perinuclear vacuolization with inclusions. Electron microscopy revealed absence of lipofuscin accumulation, no ultrastructural curvilinear profiles, fingerprint bodies, or granular osmiophilic deposits (GRODs) in lysosomes. As described here, the patient presents clinical symptoms observed in benign forms of ceroid lipofuscinosis type 2 (CLN2) and simultaneously some features of autosomal recessive spinocerebellar ataxia type 7 (SCAR7), which is also caused by mutations in the TPP1 gene.

Repair of Anomalous Single Coronary Artery From the Pulmonary Artery (ASCAPA)

We describe the management of an infant presenting with severe heart failure at 6 weeks of age found to have an anomalous single coronary artery originating from the main pulmonary artery (MPA). This patient was transferred to our hospital and ultimately had their coronary artery translocated to the ascending aorta successfully. Preoperative severe left ventricular (LV) dysfunction and moderate/severe mitral regurgitation (MR) improved to normal function and mild-to-moderate MR 6 weeks postrepair. Three-dimensional CT reconstructions proved valuable and allowed for accurate preoperative planning leading to successful coronary transfer.

Sacubitril/ Valsartan in Advanced Heart Failure: Is it Just a Matter of Contractility or are there Effects on the Pulmonary Circulation? A Real Life Monocentric Experience

Background: Sacubitril/valsartan (S/V), an angiotensin receptor neprilysin inhibitor (ARNI), is the first drug to demonstrate a mortality benefit in patients with chronic heart failure and reduced ejection fraction. S/v had a 20% reduction in the primary composite endpoint of cardiovascular death or heart failure hospitalization, compared with subjects receiving enalapril. However, the mechanisms are not clear. The aim of this prospective, non-randomized study was to assess the clinical and instrumental effects of this agent in patients with HFrEF and pulmonary hypertension. Methods: To investigate the effects of S/V in HFrEF, we selected 40 consecutive patients (31 males, 9 females, mean age 64±19 years) in the NYHA class II-III, because they had left ventricular ejection fraction ≤35% at echocardiography. Etiology: 22 CHD, 3 Myocarditis, 15 IDCM. Results: 2 patients took the maximum dose of 97/103 mg, 2 stopped the therapy due to a creatinine increase, all the others took the dose of 49/52 mg. During a mean ± SD follow-up of 24±6 months, no patients died. PASP decreased from 42.71 to 36 mm/Hg (p <0.0001); 6MWT improved from 402 m to 453 m (p <0.0001). Mean LVEF increased from 28.9% to 31.5% p <0.005); NYHA mean class improved from 1.95 to 1.70. An AICD was implanted in 20 patients. Conclusion: These preliminary data suggest that in patients with severe heart failure, S/V is able to improve 6MWT and PASP, even in the absence of a significant improvement of ventricular contractility. S/v may reduce the fluid retention and pulmonary vasoconstriction that contribute to heart failure symptoms.