The concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.

Objectives: Isolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation. Methods: Three adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result. Results: The patient was discharged home after 7 days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8 cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved. Conclusions: Over the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

Complete Isolation of Left Innominate Artery in a Patient With CHARGE Syndrome: Case Presentation and Review of Reported Cases.

We report a rare case of complete isolation of the left innominate artery in a child with CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities) syndrome. This anatomical cluster had been undetected for a relatively large period of time and the patient was referred to us with an incomplete diagnosis even after multiple medical evaluations and a thoracic surgery during the neonatal period. In conclusion, to the best of our knowledge, this is the first case of a complete isolation of left innominate artery treated with a transcatheter approach.

Bentall Endocarditis by C. Lusitaniae After COVID-19: The Finger Covers The Moon.

We report a case of endocarditis months after a Bentall procedure. This was caused by Candida Lusitaniae, in an immunocompetent patient with a recent SARS-CoV-2 infection. The patient underwent a new Bentall procedure. SARS-CoV-2 has been associated with co-infection by Candida species since the beginning of the pandemic, nevertheless, Candida Lusitaniae remains a very uncommon causative agent of prosthetic endocarditis. We suggest a possible role of the SARS-CoV-2, which may have delayed the diagnosis of endocarditis and the appropriate therapy.

Staged approach for correction of anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return into the azygous vein is a rare pathological finding. We describe the case of a 28-year-old girl who had a successful staged approach to treat this rare congenital heart disease. To avoid potential connection of a systemic venous return to the left atrium, the proximal part of the azygous vein was occluded with a percutaneous approach, then the azygous vein flow was redirected into the left atrium with a surgical procedure.

Myocardial oxygen consumption during histidine-tryptophan-ketoglutarate cardioplegia in young human hearts.

OBJECTIVES: Energy demand and supply need to be balanced to preserve myocardial function during paediatric cardiac surgery. After a latent aerobic period, cardiac cells try to maintain energy production by anaerobic metabolism and by extracting oxygen from the given cardioplegic solution. Myocardial oxygen consumption (MVO2) changes gradually during the administration of cardioplegia. METHODS: MVO2 was measured during cardioplegic perfusion in patients younger than 6 months of age (group N: neonates; group I: infants), with a body weight less than 10 kg. Histidine-tryptophan-ketoglutarate crystalloid solution was used for myocardial protection and was administered during a 5-min interval. To measure pO2 values during cardioplegic arrest, a sample of the cardioplegic fluid was taken from the inflow line before infusion. Three fluid samples were taken from the coronary venous effluent 1, 3 and 5 min after the onset of cardioplegia administration. MVO2 was calculated using the Fick principle. RESULTS: The mean age of group N was 0.2 ± 0.09 versus 4.5 ± 1.1 months in group I. The mean weight was 3.1 ± 0.2 versus 5.7 ± 1.6 kg, respectively. MVO2 decreased similarly in both groups (min 1: 0.16 ± 0.07 vs 0.36 ± 0.1 ml/min; min 3: 0.08 ± 0.04 vs 0.17 ± 0.09 ml/min; min 5: 0.05 ± 0.04 vs 0.07 ± 0.05 ml/min). CONCLUSIONS: We studied MVO2 alterations after aortic cross-clamping and during delivery of cardioplegia in neonates and infants undergoing cardiac surgery. Extended cardioplegic perfusion significantly reduces energy turnover in hearts because the balance procedures are both volume- and above all time-dependent. A reduction in MVO2 indicates the necessity of a prolonged cardioplegic perfusion time to achieve optimized myocardial protection.

[Modified Warden procedure for correction of right superior vena cava drainage in the left atrium]. / Warden modificata per la correzione del drenaggio anomalo della vena cava superiore in atrio sinistro.

A newborn with situs solitus, normally related great arteries and intact atrial septum, underwent surgical repair at our institution for anomalous drainage of the right superior vena cava in the left atrium at the level of the right superior pulmonary veins. This rare cyanotic congenital cardiac malformation is herein described with special regard to its anatomical and diagnostic features. A novel surgical approach for achieving correction is also described.

Aortopulmonary Collateral Artery from the Proximal Ascending Aorta: A Rare Anatomical Finding.

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken.

[Anomalous origin of the right coronary artery from the pulmonary artery or coronary artery fistula: when the diagnosis is uncertain]. / Quando la diagnosi può essere incerta: origine anomala dell'arteria coronaria destra dall'arteria polmonare o fistola coronarica?

The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.

Tetralogy of Fallot whit a "contralateral" ductus arteriosus.

Two neonates were taken shortly after birth to our unit with a prenatal diagnosis of [S,D,S] Tetralogy of Fallot with pulmonary atresia and "unusual" aorta to pulmonary connection. The echocardiogram confirmed the main diagnosis showing: a left aortic arch with a vascular connection between the right innominate artery and the origin of the right pulmonary artery in patient A; and right aortic arch with a vascular connection between the left innominate artery and the origin of the left pulmonary artery in patient B.

Selective versus standard cerebro-myocardial perfusion in neonates undergoing aortic arch repair: A multi-center study.

The results of neonatal aortic arch surgery using cerebro-myocardial perfusion were analyzed. Selective cerebral and myocardial perfusion, using two separate pump rotors, was compared with standard perfusion, using a single pump rotor with an arterial line Y-connector. Between May 2008 and May 2016, 69 consecutive neonates underwent arch repair using either selective cerebro-myocardial perfusion (Group A, n = 34) or standard perfusion (Group B, n = 35). The groups were similar for age, weight, BSA, prevalence of one-stage or staged repair, and single ventricle palliation; male gender was more frequent in Group A. The duration of the cerebro-myocardial perfusion was comparable (27 ± 8 vs. 28 ± 7 min, P = 0.9), with higher flows in Group A (57 ± 27 vs. 39 ± 19 mL/kg/min, P = 0.01). Although cardioplegic arrest was more common in Group B (13/34 vs. 23/35, P = 0.03), the duration of myocardial ischemia was longer in Group A (64 ± 41 vs. 44 ± 14 min, P = 0.04). There was 1 hospital death in each group, with no permanent neurological injury in either group. Cardiac morbidity (1/34 vs. 7/35, P = 0.02) was more common in Group B, while extracardiac morbidity was similar in both the groups. During follow-up (3.2 ± 2.4 years), 5 late deaths occurred with a comparable 5-year survival rate (75 ± 17% vs. 88 ± 6%, P = 0.7) and freedom from arch reintervention (86 ± 6% vs. 84 ± 7%, P = 0.6). Risk of cardiac morbidity was greater with standard cerebro-myocardial perfusion (OR = 5.2, CI 3.3-6.8, P = 0.001) and with perfusion flows less than 50 mL/kg/min (OR 3.7, CI 1.87-5.95, P = 0.04). Cerebro-myocardial perfusion is a safe and effective strategy to protect the brain and heart in neonates undergoing arch repair. Selective techniques using higher perfusion flows may further attenuate cardiac morbidity.

Dacron Conduit for Extracardiac Total Cavopulmonary Anastomosis: A Word of Caution.

BACKGROUND: The extracardiac conduit technique is a valid option for completing total cavopulmonary anastomosis (TCPC) in patients with a single ventricle. The technique allows for beating heart surgery, optimal flow dynamics, and reduced postoperative atrial arrhythmia. Different types of conduit have been proposed. This study reported a single-centre experience with two different types of conduit. METHODS: Consecutive patients referred for TCPC at the current institution between January 2001 and September 2013 were included. Retrospective extraction of pertinent variables was accomplished through electronic patient chart review. Patients were stratified based on the type of conduit used to perform the TCPC: polytetrafluoroethylene (PTFE) conduit (Group A) and polyethylene-terephthalate (Dacron) conduit (Group B). RESULTS: The patient population included 105 patients: Group A had 80 patients, and Group B had 25 patients. The two groups were similar in major clinical and procedural variables, including conduit size and Nakata index. Eighteen patients (Group A: one [1.25%]; Group B: 17 [68%]) had conduit occlusion or severe stenosis requiring intervention after a mean 46.9±35months after the operation. The percentage of patients in Group A who were free of conduit obstruction at 3, 5, and 10 years was 100%, 100%, and 96%, respectively, whereas these figures were 68%, 52%, and 35% in Group B (log-rank <0.000). Conduit re-intervention was associated with an increased risk of overall mortality after primary intervention (p<0.004). Dacron tube was found to be an independent risk factor for mid-term stenosis or obstruction (hazard ratio, 62.9; 95% CI, 8.2-482.2; p=0.000). CONCLUSION: Dacron conduit for TCPC surgery was associated with a higher risk of obstruction and need for early re-intervention compared with PTFE conduit. Surgical or percutaneous re-interventions for conduit obstruction increased the risk of late mortality.

Integrative miRNA and whole-genome analyses of epicardial adipose tissue in patients with coronary atherosclerosis.

BACKGROUND: Epicardial adipose tissue (EAT) is an atypical fat depot surrounding the heart with a putative role in the development of atherosclerosis. METHODS AND RESULTS: We profiled genes and miRNAs in perivascular EAT and subcutaneous adipose tissue (SAT) of metabolically healthy patients without coronary artery disease (CAD) vs. metabolic patients with CAD. Compared with SAT, a specific tuning of miRNAs and genes points to EAT as a tissue characterized by a metabolically active and pro-inflammatory profile. Then, we depicted both miRNA and gene signatures of EAT in CAD, featuring a down-regulation of genes involved in lipid metabolism, mitochondrial function, nuclear receptor transcriptional activity, and an up-regulation of those involved in antigen presentation, chemokine signalling, and inflammation. Finally, we identified miR-103-3p as candidate modulator of CCL13 in EAT, and a potential biomarker role for the chemokine CCL13 in CAD. CONCLUSION: EAT in CAD is characterized by changes in the regulation of metabolism and inflammation with miR-103-3p/CCL13 pair as novel putative actors in EAT function and CAD.

Conventional versus frozen elephant trunk surgery for extensive disease of the thoracic aorta.

OBJECTIVE: To compare early and mid-term outcomes after repair of extensive aneurysm of the thoracic aorta using the conventional elephant trunk or frozen elephant trunk (FET) procedures. METHODS: Fifty-seven patients with extensive thoracic aneurysmal disease were treated using elephant trunk (n = 36) or FET (n = 21) procedures. Patients with aortic dissection, descending thoracic aorta (DTA) diameter less than 40 mm, and thoracoabdominal aneurysms were excluded from the analysis, as were those who did not undergo antegrade selective cerebral perfusion during circulatory arrest. Short-term and mid-term outcomes were compared according to elephant trunk/FET surgical management. RESULTS: Preoperative and intraoperative variables were similar in the two groups, except for a higher incidence of female sex, coronary artery disease and associated procedures in elephant trunk patients. Hospital mortality (elephant trunk: 13.9% versus FET: 4.8%; P = 0.2), permanent neurologic dysfunction (elephant trunk: 5.7% versus FET: 9.5%; P = 0.4) and paraplegia (elephant trunk: 2.9% versus FET: 4.8%; P = 0.6) rates were similar in the two groups. Follow-up was 100% complete. In the elephant trunk group, 68.4% of patients did not undergo a second-stage procedure during follow-up for a variety of reasons. Of these patients, the DTA diameter was greater than 51 mm in 72.2% and two (6.7%) died due to aortic rupture while awaiting stage-two intervention. Endovascular second-stage procedures were successfully performed in all FET patients with residual DTA aneurysmal disease (n = 3), whereas nine of 11 elephant trunk patients who returned for second-stage procedures required conventional surgical replacement through a lateral thoracotomy. Kaplan-Meier estimate of 4-year survival was 75.8 ±â€Š7.6 and 72.8 ±â€Š10.6 in elephant trunk and FET patients, respectively (log-rank P = 0.8). CONCLUSION: In patients with extensive aneurysmal disease of thoracic aorta, elephant trunk and FET procedures seem to be associated with similar satisfactory early and mid-term outcomes. The FET approach leads to single-stage treatment of all aortic disease in most patients, and facilitates endovascular second-stage treatment in patients with residual DTA disease. The elephant trunk staged-approach appears to leave a considerable percentage of patients at risk for adverse aortic events.

Impact of different cannulation strategies on in-hospital outcomes of aortic arch surgery: a propensity-score analysis.

BACKGROUND: The impact of different cannulation strategies on outcomes of aortic arch surgery remains controversial. This retrospective study sought to evaluate central cannulation (ascending aorta, right axillary, and innominate artery) compared with femoral artery cannulation for aortic arch surgery, and to identify among preoperative and intraoperative variables the independent predictors of death and permanent neurologic dysfunction (PND) in aortic arch surgery. METHODS: All patients were operated through a median sternotomy using antegrade selective cerebral perfusion with moderate hypothermia as a method of brain protection. Treatment bias was addressed by use of propensity-score matching and multivariate regression analysis. Logistic regression models were used to identify the independent predictors of hospital mortality and PND. RESULTS: Of the 473 patients undergoing aortic arch surgery, 273 (57.7%) underwent femoral cannulation (FC), and 200 (42.3%) underwent central cannulation (CC). The CC and FC cannulation were associated with similar risk of in-hospital death (absolute risk reduction [ARR]: 0.7%; p = 0.880) and PND (ARR:-2.6%, p = 0.361) in the overall cohort and after adjusting for propensity-based matching (ARR for hospital mortality: 2.2%, p = 0.589; ARR for PND: 3.4%, p = 0.271). Female gender (odds ratio [OR]:2.1, p = 0.030), type A acute dissection or intramural hematoma (OR: 2.2; p = 0.041), and CPB time (OR: 1.010/minute, p = 0.015) were independent predictors of in-hospital death. Female gender (OR: 2.4; p = 0.033), type A acute dissection or intramural hematoma (OR: 4.2; p = 0.005), and diabetes (OR: 6.6, p = 0.007) were independent predictors of PND. CONCLUSIONS: During aortic arch surgery, CC and FC are associated with a similar risk of postoperative death and PND. Type A acute aortic dissection and cardiopulmonary bypass time remain strong risk factors for mortality and PND.

Delayed management of blunt traumatic aortic injury: open surgical versus endovascular repair.

BACKGROUND: A growing body of evidence has shown that delayed management of traumatic injury of the thoracic aorta determines survival benefits as compared with immediate treatment. However, few data exist comparing outcomes after delayed open surgical or endovascular management. Accordingly, we reviewed our experience with delayed management, stratifying the data according to type of repair; open surgical versus endovascular. METHODS: Since 1992, delayed aortic repair has represented our first-line management for all blunt traumatic thoracic aortic injury (BTTAI) patients, except for those who presented with or became unstable due to impending aortic rupture. These patients were converted to urgent primary aortic repair. Thus, between 1992 and 2010, a total of 77 BTTAI patients were managed according to this policy. There were 57 (74%) men having a mean age of 33.4 years. Thirty-one (41.3%) patients underwent open surgical repair (SR), 44 (58.6%) underwent endovascular repair (ER), and 2 died while awaiting aortic repair. At admission, the clinical and trauma characteristics were similar in both groups. The trauma-to-repair time span (in days) was 200 (Q1-Q3: 27 to 340) and 10 (Q1-Q3: 2 to 79) for SR and ER patients, respectively (p = 0.001). Due to unpaired hemodynamic or imaging signs of impending aortic rupture, 15 patients required urgent repair, which was endovascular in 11 (25%) cases and surgical in 4 (12.9%). RESULTS: Overall, hospital mortality was 3.9% (n = 3), being 0% in SR patients and 2.3% (n = 1) in ER patients (p = 0.398). No new postoperative paraplegia occurred; a cerebellar stroke occurred in 1 (2.3%) ER patient receiving intentional coverage of the left subclavian artery. During follow-up (96.1% complete at 95 ± 70 months), no late deaths occurred. At 15 years, the estimates of survival and freedom from secondary aortic procedures were 96% and 100%, respectively. CONCLUSIONS: Delayed management of traumatic aortic injury was associated with satisfactory short- and long-term results without significant differences between open surgical and endovascular repair. However, the reduced invasiveness of endovascular repair can optimize operative timing allowing prompt aortic repair in unstable patients, earlier repair in stable patients, and, when indicated, easier concomitant non-aortic surgery.

Reoperative surgery on the thoracic aorta.

OBJECTIVE: The objective of our study was to report our hospital and long-term results after reinterventions on the thoracic aorta. METHODS: Between 1986 and 2011, 224 reoperations on the proximal thoracic aorta after previous aortic surgery were performed in our institution. The number of reinterventions quadrupled during the course of the study period. Mean patient age was 58.1 years, and 174 patients (77.7%) were male. An urgent/emergency operation was performed in 39 patients (17.4%). Indications for surgery included degenerative and chronic postdissection aneurysm (n = 166), false aneurysm (n = 31), active prosthetic infection (n = 16), acute dissection (n = 10), and other (n = 1). Surgical procedures involved the aortic root in 40.6% of patients, the ascending aorta in 9.4%, the aortic arch in 24.6%, and the entire proximal thoracic aorta in 25.4%. RESULTS: Hospital mortality was 12.1%. On multivariate analysis, cardiopulmonary bypass time (odds ratio, 1.1023/minute; P < .001), and urgent/emergency status (odds ratio, 5.6; P < .001) emerged as independent predictors of hospital mortality. The follow-up was 98.7% complete. Estimated 1-, 5-, and 10-year survival rates were 84.4%, 72.5%, and 48.5%, respectively. Eighteen reinterventions were performed during follow-up-16 because of the progression of aortic disease at the proximal aorta (n = 2) and downstream aorta (n = 14). Freedom from reoperation at 1, 5, and 10 years was 95.6%, 90.2%, and 81.5%, respectively. CONCLUSIONS: Reoperative aortic surgery was associated with satisfactory short- and long-term results, especially if carried out on an elective basis. The extent of the aortic replacement did not impact survival and was associated with a reduced need for reintervention. The progressive nature of aortic disease and the favorable results of elective primary aortic interventions suggest favoring aggressive aortic resections at initial surgery.

Acute kidney injury following transcatheter aortic valve implantation: incidence, predictors and clinical outcome.

BACKGROUND: Limited data exist on renal complications of transcatheter aortic valve implantation (TAVI) within a comprehensive program using different valves with transfemoral, transapical, and trans-subclavian approach. METHODS: Prospective single-center registry of 102 consecutive patients undergoing TAVI using both approved bioprostheses and different access routes. The main objective was to assess the incidence, predictors and the clinical impact of acute kidney injury (AKI). AKI was defined according to the valve academic research consortium (VARC) indications. RESULTS: Mean age was 83.7 ± 5.3 years, logistic EuroSCORE 22.6 ± 12.4%, and STS score 8.2 ± 4.1%. Chronic kidney disease at baseline was present in 87.3%. Periprocedural AKI developed in 42 patients (41.7%): 32.4% stage 1, 4.9% stage 2 and 3.9% stage 3. The incidence of AKI was 66.7% in transapical, 30.3% in transfemoral, and 50% in trans-subclavian procedures. The only independent predictor of AKI was transapical access, with a hazard ratio (HR) between 4.57 and 5.18 based on the model used. Cumulative 1-year survival was 88.2%. At Cox regression analysis, the only independent predictor of 30-day mortality was diabetes mellitus (HR 7.05, 95% CI 1.07-46.32; p=0.042), whilst the independent predictors of 1-year death were baseline glomerular filtration rate<30 mL/min (HR 5.74, 95% CI 1.42-23.26; p=0.014) and post-procedural AKI 3 (HR 8.59, 95% CI 1.61-45.86, p=0.012). CONCLUSIONS: TAVI is associated with a high incidence of AKI. Although in the majority of the cases AKI is of mild entity and reversible, AKI 3 holds a strong negative impact on 1-year survival. The incidence of AKI is higher with transapical access.