Descending aorta to right atrial fistula: Transcatheter embolization of a very rare anomaly with coils.

Key Clinical Message: Descending aorta to right atrial (RA) fistula is a rare and distinct clinical entity mimicking patent ductus arteriosus (PDA) and it may lead to rapid development of pulmonary vascular disease. Correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important. Interventional management is the treatment of choice. Abstract: We present a case report of a trisomy 21 infant with atrial and ventricular septal defects and small patent ductus arteriosus (PDA) complicated by the presence of descending aorta to right atrial (RA) fistula with large left to right shunt leading to rapid increase in pulmonary vascular resistance. Transcatheter occlusion of the fistula followed by closure of the PDA with Nit-Occlud coil systems led to decreased pulmonary pressure and resistance permitting successful surgical repair of the patient's intracardiac defects with good outcome over 3 years of follow-up. Descending aorta to RA fistula is a rare and distinct clinical entity mimicking PDA and its correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important as it may lead to rapid development of pulmonary vascular disease.

Outcomes of delayed chest closure after congenital heart surgery in neonates.

We present the outcomes of delayed chest closure in neonates who underwent congenital heart surgery under cardiopulmonary bypass. Eighty-one consecutive neonatal patients (age ≤ 28 days) with congenital heart diseases who underwent heart operations and after surgery, chest remained open in the intensive care unit until DCC. Correction of transposition of the great arteries pathology was the most common surgical procedure (48.1% of patients). Median sternal closure time from surgery was 3 (2-4) days. Median age of neonates was 9 (5-12) days. In addition, in 4 cases (4.9%) there was secretion from the surgical site after DCC and after taking cultures, in 2 (2.4%) of the cases a pathogen was identified. Multivariable linear regression analysis (adjusted to gender and CPB) showed that only the age-predicted the sternum closure time (ß=-0.09, 95%CI: - 0.16 to -0.02, p=0.02). In-hospital mortality was 6 (7.4%) patients. Although the DCC in neonates who underwent CHD surgical correction was related to a high mortality rate, only the age of neonates predicted the sternum closure time in the ICU.

Outcomes after repair of complete atrioventricular canal with a modified single-patch technique: a retrospective study.

BACKGROUND: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. METHODS: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. RESULTS: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). CONCLUSION: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

Giant Cell Arteritis: Focusing on Current Aspects From the Clinic to Diagnosis and Treatment.

Giant cell arteritis (GCA) is a granulomatous arteritis involving large arteries, particularly the aorta and its major proximal branches, including the carotid and temporal arteries. GCA involves individuals over 50 years old. The etiopathogenesis of GCA may involve a genetic background triggered by unknown environmental factors (eg infections), the activation of dendritic cells as well as inflammatory and vascular remodeling. However, its pathogenetic mechanism still remains unclear, although progress has been made in recent years. In the past, inflammatory markers and arterial biopsy were considered as gold standard for the diagnosis of GCA. However, emerging imaging methods have been made more sensitive and specific for the diagnosis of GCA. Treatment includes biological and other modalities including interleukin-6 (IL-6) inhibitors.

Aortic valve neocuspidization using the Ozaki technique: A meta-analysis of reconstructed patient-level data.

BACKGROUND: Aortic valve neocuspidization using the Ozaki technique has shown promising results both in adults and children. METHODS: A systematic search of the PubMed and Cochrane databases was performed up to November 13, 2021. Individual patient data were reconstructed and analyzed from the Kaplan-Meier curves of all eligible studies for time-to-event outcomes. RESULTS: We included a total of 22 studies reporting on 1,891 patients that underwent Ozaki reconstruction. Mean age at the time of surgery was 43.2 ± 24.5 years (65 ± 12.3 years for adult patients and 12.3 ± 3.8 years for pediatric patients). The most common indication was aortic stenosis (46.4%, 95% CI 34.1-58.6). Mean cross-clamp and cardiopulmonary bypass duration were 106.8 ± 24.8 minutes and 135.2 ± 35.1 minutes, respectively. Permanent pacemaker was implanted in 0.7% (95% CI 0.4-1.2) of the patients. At discharge, mean effective orifice area was 2.1 ± 0.5 cm2/m2. At latest follow-up, peak gradient was 15.7 ± 7.4 mm Hg and only 0.25% (95% CI 0-2.3) had moderate aortic insufficiency. In-hospital mortality was 0.7% (95% CI 0.1-1.7). Late mortality was 1.9% during a mean follow-up of 38.1 ± 23.8 months. One-year, 3-year, and 5-year freedom from reoperation rates were 98.0 %, 97.0 % and 96.5%, respectively. More than half of the reoperations were due to infective endocarditis (51.5%, 95% CI 18.3-84.0). In our cohort, the risk of endocarditis per patient per year was 0.5%. CONCLUSIONS: The midterm outcomes of the Ozaki procedure are excellent in terms of hemodynamics, survival, and freedom from reoperation. Acquiring long-term follow-up will help solidify this technique in the cardiac surgery armamentarium.

Converting the Fontan Circulation: Challenges and Evolution.

The Fontan operation, the final palliative step after a series of complex operations in patients with univentricular hearts, has undergone multiple modifications throughout the last decades, with the goal of finding the method which combines the optimal hemodynamic effects of the Fontan circulation with minimal long term side effects. An understanding of the operative evolution and subsequent side effects, as well as the management thereof seems imperative. Since its inception by Francis Fontan the, now obsolete, initial atriopulmonary connection has passed through several milestones into having now reached the era of total cavopulmonary connection. However, recently published results bring to light the new option of biventricular conversion which comes to challenge the management of Fontan patients as we know it. Currently, there is no consensus as to whether to continue with palliation in this challenging group of patients or proceed with heart transplantation. In this collective review, we provide a historic overview of the Fontan evolution as well as future insights, discussing the perspectives of options for patients with failing Fontan, including the latest addition of biventricular correction.

Evaluating the Effectiveness of Stenting for Aortic Coarctation.

BACKGROUND: Coarctation of the aorta (CoA) is a congenital cardiovascular malformation involving narrowing of the thoracic aorta just distal to the left subclavian artery. The aim of our study was to evaluate the hemodynamic effects of endovascular treatment for CoA by using invasive aortic catheterization. METHODS: All patients with CoA who underwent treatment by aortic stent implantation between September 1, 2003, and February 1, 2019, at the "Onassis Cardiac Surgery Center," in Athens, Greece, were evaluated. Patients were treated with either bare (uncovered) Cheatham-Platinum (bCP) or covered Cheatham-Platinum (cCP) stent implantations. Invasive aortic pressure measurements were recorded before and after the endovascular intervention. RESULTS: A total of 48, eight zig CP stents, comprising 24 bCP and 24 cCP stents were implanted in 47 patients. The mean aortic diameter (mm) at the CoA lesion increased from 9.7 ± 3.3 to 19.2 ± 2.9 mm (p <0.01) after the endovascular procedure. The invasive mean blood pressure (BP; mm Hg) from catheterization in the descending aorta increased (before = 114.2 ± 12.8 vs. after = 135.5 ± 28.1; p <0.01), while the invasive mean BP (mm Hg) from catheterization in the ascending aorta was decreased (before = 156.8 ± 25.0 vs. after = 138.4 ± 27.5; p <0.01) after the intervention. The mean aortic BP gradient decreased in both types of stents after intervention (BP gradient among patients with cCP stents = 30.9 +/- 23.6 mmHg and BP gradient among patients with bCP stents = 38.0 +/-23.1 mmHg). However, there was no statistically significant difference between the two types of stents; p = 0.36. CONCLUSIONS: Invasive aortic catheterization provided evidence that endovascular stenting with either bare or covered stents is efficient in treating patients with CoA.

Acute Kidney Injury Predictors and Outcomes after Cardiac Surgery in Children with Congenital Heart Disease: An Observational Cohort Study.

Acute Kidney Injury (AKI) commonly complicates cardiac surgery in children with congenital heart disease (CHD). In this study we assessed incidence, risk factors, and outcomes of postoperative AKI, while testing the hypothesis that, depending on the underlying diagnosis, there would be significant differences in AKI incidence among different diagnostic groups. We conducted an observational cohort study of children with CHD undergoing cardiac surgery in a single tertiary center between January 2019 and August 2021 (n = 362). Kidney Disease Improving Global Outcome (KDIGO) criteria were used to determine the incidence of postoperative AKI. Diagnosis was incorporated into multivariate models using an anatomic-based CHD classification system. Overall survival was estimated using Kaplan−Meier curves. Log-rank test and adjusted Cox proportional hazard modelling were used to test for differences in survival distributions and determine AKI effect on survival function, respectively. AKI occurred in 70 (19.3%), with 21.4% in-hospital mortality for AKI group. Younger age, lower weight, longer cardiopulmonary bypass time, preoperative mechanical ventilation and diagnostic category were associated with postoperative AKI. Resolution rate was 92.7% prior to hospital discharge for survivors. AKI was associated with longer duration of mechanical ventilation, ICU and hospital length of stay. AKI patients had significantly higher probability of all-cause mortality postoperatively when compared to the non-AKI group (log-rank test, p < 0.001). Adjusted hazard ratio for AKI versus non-AKI group was 11.08 (95% CI 2.45−50.01; p = 0.002). Diagnostic category was associated with cardiac surgery-related AKI in children with CHD, a finding supporting the development of lesion specific models for risk stratification. Postoperative AKI had detrimental impact on clinical outcomes and was associated with decreased survival to hospital discharge.

Risk Factors for Acute Postoperative Delirium in Cardiac Surgery Patients >65 Years Old

Background: Acute postoperative delirium is the most common neuropsychiatric disorder in cardiac surgery patients in the intensive care unit (ICU). The purpose of this study was to evaluate the possible risk factors of postoperative delirium (POD) for cardiac surgery patients in the ICU. Materials and Methods: The study population was composed of 86 cardiac surgery patients managed postoperatively in the cardiac surgery ICU. Presence of POD in patients was evaluated by the CAM-ICU scale. Results: According to the CAM-ICU scale, 22 (25.6%) patients presented POD; history of smoking, alcohol use, COPD, and preoperative permanent atrial fibrillation were associated with POD (for all, p < 0.05). The type of cardiac surgery operations, type of analgesia, and red blood cell transfusion in the ICU were not associated with POD (p > 0.05), while cardiac arrhythmia in the ICU, hypoxemia in the ICU after extubation (pO2 < 60 mmHg), and heart rate after extubation were predisposing factors for POD (for all, p < 0.05). Multivariable logistic regression analysis (adjusted to risk factors) showed that hypoxemia after extubation (OR = 20.6; 95%CI: 2.82−150), heart rate after extubation (OR = 0.95; 95% CI: 0.92−0.98), and alcohol use (OR = 74.3; 95%CI: 6.41−861) were predictive factors for acute postoperative delirium (for all, p < 0.05). Conclusion: Alcohol use and respiratory dysfunction before and after heart operation were associated with acute postoperative delirium in cardiac surgery ICU patients.

Does deep hypothermic circulatory arrest with versus without retrograde cerebral perfusion affect the outcomes after proximal aortic arch aneurysm and acute type A aortic dissection repair? Different pathologies and cerebral protection techniques with similar results.

Hypothermic circulatory arrest is used for proximal and total aortic arch correction in patients with aortic arch aneurysm and acute or chronic type A aortic dissection. Different cerebral perfusion techniques have been proposed for reducing morbidity and mortality rate. The study of Arnaoutakis et al. showed that deep hypothermic circulatory arrest with or without retrograde cerebral perfusion for proximal aortic aneurysm and acute type A aortic dissection correction had similar results with regard to morbidity and mortality rate. In addition, the short circulatory arrest time contributes for favorable outcomes of these patients. Although antegrade cerebral perfusion with hypothermic circulatory is widely used by many cardiac surgeons, deep hypothermic circulatory arrest with or without retrograde cerebral perfusion remains an alternative and safe method for brain protection in patients undergoing proximal aortic arch aneurysm or acute type A aortic dissection repair.

Postoperative Chylothorax in Neonates and Infants after Congenital Heart Disease Surgery-Current Aspects in Diagnosis and Treatment.

Postoperative chylothorax is a rare complication following cardiac surgery for congenital heart disease (CHD) in the pediatric population, including neonates and infants. Multiple mechanisms are involved in the development of postoperative chylous effusions, mainly the traumatic injury of lymphatic vessels, systemic venous obstruction and dysfunction of the right ventricle. In this review, we focus on the existing evidence regarding the definition and diagnosis of postoperative chylothorax in children with CHD, as well as current therapeutic approaches, both nutritional and interventional, for the management of these patients. As part of nutritional management, we specifically comment on the use of defatted human milk and its effect on both chylothorax resolution and patient growth. A consensus with regard to several key aspects of this potentially significant complication is warranted given its impact on the cost, morbidity and mortality of children with CHD.

Can regional cerebral oxygen saturation monitoring during circulatory arrest time predict postoperative neurological dysfunction in patients undergoing surgical pulmonary thromboendarterectomy? Useful index for short- and long-term outcomes.

A hypothermic circulatory arrest is usually used to correct thoracic aorta pathologies. The emergency treatment of acute type A aortic dissection and elective repair of aortic arch pathologies are the most common indications for using hypothermic circulatory arrest. A hypothermic circulatory arrest can also be used for surgical pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Intervals with total circulatory arrest offer a clear surgical field for thrombus and emboli removal from the pulmonary artery branches. The price to pay for intermittent circulatory arrest during pulmonary thromboendarterectomy is postoperative neurological dysfunction due to brain hypothermia and hypoperfusion. A noninvasive method for cerebral monitoring during cardiac surgery is real-time regional cerebral oxygen saturation (rSO2 ). Liu et al. report that continuous monitoring of rSO2 during surgical pulmonary thromboendarterectomy may reduce the long cerebral hypoperfusion time and prevent postoperative neurological dysfunction.

Acute renal failure after acute type A aortic dissection repair. Insidious postoperative complication with poor short- and long-term prognosis.

Acute type A aortic dissection (ATAAD) is a life-threatening aortic disease. Many systems and organs are affected by malperfusion which presents preoperatively and postoperatively. Postoperative acute renal failure after ATAAD constitutes a severe and insidious complication. Acute renal damage is observed in many patients with ATAAD preoperatively and it burdens the renal function postoperatively. Renal replacement therapy represents an additional risk factor for short-, mid-, and long-term outcomes after ATAAD repair. Brown et al.'s present study highlight the clinical significance of this complication. Also, they remind us of the importance of optimizing perioperative renal protective strategies in patients undergoing ATAAD repair.

Does systemic immune-inflammation index predict the short outcomes after an acute type A aortic dissection repair? Promising biomarker for acute aortic syndrome.

The retrospectively presented by Xu et al. assessed the value of the systemic immune-inflammation index (SII) in the prediction of short-term outcomes in patients undergoing surgery for acute type A aortic dissection (ATAAD). The authors concluded that SII is a reliable biomarker that can predict postoperative short-term outcomes and this marker could be potentially applied to stratification and patient selection with ATAAD. Although the study is retrospective, it is well-matched and conducted in a large volume center and the surgical technique was standard for all procedures. In addition, there was no statistical difference in cardiopulmonary bypass, aortic cross-clamp and deep hypothermic time, comorbidities, blood and blood products transfusion between the study groups. In addition, 90.7% of patients underwent elephant trunk technique for ATAAD repair, while the 30-day mortality and postoperative temporary and permanent neurological dysfunctions were 14.8% and 11.1%, respectively. Randomized controlled and prospective studies are warranted to clarify these well-documented results to apply this useful biomarker in clinical practice for patients with the acute aortic syndrome.

Congenital, acquired, or both? The only two congenitally based, acquired heart diseases.

Discrete subaortic stenosis (DSS) is a type of left ventricular outflow tract obstruction whereas double-chambered right ventricle is a form of right ventricular outflow tract obstruction. Both of these cardiac malformations share lots of similar characteristics which classify them as acquired developmental heart diseases despite their congenital anatomical substrate. Both of them are frequently associated to ventricular septal defects. The initial stimulus in their pathogenetic process is anatomical abnormalities or variations. Subsequently, a hemodynamic process is triggered finally leading to an abnormal subaortic fibroproliferative process with regard to DSS or to hypertrophy of ectopic muscles as far as double-chambered right ventricle is concerned. In many cases, these pathologies are developed secondarily to surgical management of other congenital or acquired heart defects. Moreover, high recurrence rates after initial successful surgical therapy, particularly regarding DSS, have been described. Finally, an interesting coexistence of DSS and double-chambered aortic ventricle has also been reported in some cases.

Analysis of risk factors for in-hospital mortality in 177 patients who underwent isolated coronary bypass grafting and received intra aortic balloon pump.

BACKGROUND AND AIM OF THE STUDY: Intra-aortic balloon pump (IABP) is the most commonly used device for circulatory support of patients with heart disease. The aim of this study evaluated the risk factors for outcomes of patients who underwent isolated coronary bypass grafting and received IABP. METHODS: One hundred-seventy-seven patients underwent isolated coronary bypass grafting and received intraoperative IABP. All perioperative data were recorded. The primary end-point was in-hospital mortality, while the secondary end-points were the length of intensive care unit (ICU) stay and hospital length of stay (LOS). RESULTS: In-hospital mortality was 5.6% (10 patients). Multivariable logistic regression analysis showed that the risk factors for in-hospital mortality were preoperative plasma creatinine level and cardiopulmonary bypass (CPB) time (for both p < .05). Multivariable linear regression analysis showed that postoperative acute kidney injury (AKI), immediate plasma troponin after operation, numbers of grafts, and CPB time were predicted the hospital LOS, while postoperative AKI, preoperative left ventricular ejection fraction, and immediate plasma troponin after operation were predicted the lengths of ICU stay. CONCLUSION: Our analysis showed that increased preoperative plasma creatinine, postoperative renal dysfunction, and CPB time negatively affected the outcomes of patients who received intraoperative IABP insertion.

Antegrade and Retrograde Cerebral Perfusion During Acute Type A Aortic Dissection Repair in 290 Patients.

AIM: Hypothermia and selective brain perfusion is used for brain protection during an acute type A aortic dissection (ATAAD) correction. We compared the outcomes between antegrade and retrograde cerebral perfusion techniques after ATAAD surgery. METHOD: Between January 1995 and August 2017, 290 patients underwent ATAAD repair under deep hypothermic circulatory arrest/retrograde cerebral perfusion (DHCA/RCP) in 173 patients and moderate hypothermic circulatory arrest/antegrade cerebral perfusion (MHCA/ACP) in 117 patients. Outcomes of interest were: 30-day mortality, new-onset postoperative neurological complications, and length of intensive care unit (ICU) and in-hospital stays. RESULTS: No differences were observed between the preoperative details of both groups (p>0.05). Thirty-day (30-day) mortality did not differ between groups (RCP vs ACP, 22% vs 21.4%; p=0.90). New-onset postoperative permanent neurological dysfunctions and coma was similar in two group in 6.9% versus 10.3% of patients and 3.8% versus 6.8% patients of patients, respectively (p=0.69). The incidence of 30-day mortality and new postoperative neurological complications were similar in the RCP and ACP groups (odds ratio [OR], 1.0; 95% confidence interval [CI], 0.39-2.83 [p=0.91] and OR, 1.7; 95% CI, 0.87-3.23 [p=0.11], respectively). There was no difference between length of stay in the ICU and overall stay in hospital between the RCP and ACP groups (p=0.31 and p=0.14, respectively). No difference in survival rate was observed between the RCP and ACP groups (hazard ratio, 1.2; 95% CI, 0.76-2.01 [p=0.39]). CONCLUSIONS: Thirty-day (30-day) mortality rate, new-onset postoperative neurological dysfunctions, ICU stay, and in-hospital stay did not differ between the MHCA/ACP and DHCA/RCP groups after ATAAD correction. Although the rates of 30-day mortality and postoperative neurological complications were high after ATAAD repair, ACP had no advantages over the RCP technique.

Intrathoracic schwannoma originating from intrathoracic vagus nerve.

In differential diagnosis of posterior mediastinal mass should be included the intrathoracic vagus nerve tumor. Surgical excision of intrathoracic vagus nerve schwannoma is associated with a low recurrence rate and excellent long-term results.

Right atrial thrombus mimicking cardiac tumor in a neonate.

Does the cardiac mass should be operated in neonates? When diagnosis of cardiac tumor or mass has been made, the surgical excision should not be delayed for prevention of life-treating complications.