Hereditary Thoracic Aortic Diseases.

Advances in both imaging techniques and genetics have led to the recognition of a wide variety of aortic anomalies that can be grouped under the term 'hereditary thoracic aortic diseases'. The present review aims to summarize this very heterogeneous population's clinical, genetic, and imaging characteristics and to discuss the implications of the diagnosis for clinical counselling (on sports activity or pregnancy), medical therapies and surgical management.

Coronary unroofing does not fits all anomalous aortic origin of coronary arteries.

INTRODUCTION: Anomalous aortic origin of coronary artery (AAOCA) is the second leading cause of sudden cardiac death in children and young adults. Intramural-interarterial course is the most frequent anatomic variation and coronary unroofing is widest adopted for surgical management. Symptoms recurrence is described regardless of the technique used. This study aims to describe how an anatomic patient-centered approach aimed to restore a normal coronary artery take-off is associated with symptoms resolution. METHODS: From 2008 to 2021, 25 patients were operated on for an AAOCA at a median age of 20 years. Nineteen patients had a right AAOCA and six had left AAOCA. Intramural course was present in 18 patients. Seventy-six percent were symptomatic. No episodes of aborted sudden cardiac death before surgery was described in the population. Surgical technique used were coronary unroofing in 18 patients, coronary neo-ostioplasty in 3, coronary Reimplantation in 3, and main pulmonary artery re-location in 1. RESULTS: No hospital mortality or reoperation was observed in our experience as well as major complications related to surgery. Mean hospital length of stay was 8.5 days. None of patients reported symptoms recurrence at follow-up. Young athletes returned to play competitive sport. Postoperative computed tomography scan evaluation showed a general improvement of the take-off angle. CONCLUSIONS: AAOCA requires a patient anatomic-based surgical management. There is not a single surgical technique that can fits all anatomic subtype of AAOCA. Surgical techniques may be selected on the base of the preoperative images and intraoperative findings. In our experience, this policy is associated with no symptoms recurrence.

Bicuspid Aortic Valve in Children and Adolescents: A Comprehensive Review.

Bicuspid aortic valve (BAV) is the most common congenital heart defect. Prevalence of isolated BAV in the general pediatric population is about 0.8%, but it has been reported to be as high as 85% in patients with aortic coarctation. A genetic basis has been recognized, with great heterogeneity. Standard BAV terminology, recently proposed on the basis of morpho-functional assessment by transthoracic echocardiography, may be applied also to the pediatric population. Apart from neonatal stenotic BAV, progression of valve dysfunction and/or of the associated aortic dilation seems to be slow during pediatric age and complications are reported to be much rarer in comparison with adults. When required, because of severe BAV dysfunction, surgery is most often the therapeutic choice; however, the ideal initial approach to treat severe aortic stenosis in children or adolescents is not completely defined yet, and a percutaneous approach may be considered in selected cases as a palliative option in order to postpone surgery. A comprehensive and tailored evaluation is needed to define the right intervals for cardiologic evaluation, indications for sport activity and the right timing for intervention.

Neo-aortic posterior sinus of Valsalva reduction and closed coronary artery transfer as a method to approach aortopulmonary mismatch in transposition of great arteries.

OBJECTIVE: In transposition of great arteries (TGA), aortopulmonary mismatch (APM) can determine postoperative neo-aortic insufficiency after arterial switch operation (ASO). The distortion of sinu-tubular junction may be the geometric mechanism responsible. We developed a strategy able to reduce the mismatch at the timing of ASO, and in this study, we aimed to describe our indications and results. METHODS: Preoperative root circumferences at the level of the mid-portion of sinus of Valsalva and ascending aorta circumference were used to define APM. Indication to surgery was a neo-aortic root (NAR) to ascending aorta ratio ≥ 1.4. Along with standard ASO, posterior neo-aortic sinus inverted conal resection and punch technique for coronary reimplantation was used in all patients to re-establish the more geometric ratio possible between the two components. Hypoplastic aortic arch (HAA) and aortic coarctation (CA) were managed by aortic arch enlargement with an autologous pericardial patch. RESULTS: Twenty patients (20 male), 19 with diagnoses TGA (17 with ventricular septal defect, 85%) and 1 with Taussig-Bing anomaly underwent ASO. HAA was present in three (15%) and CA in two (10%). The mean preoperative neo-aortic to ascending aorta ratio was 1.8 versus 1.1 postoperatively (p < .01). No moderate or severe neo-aortic insufficiency was observed before discharge and at a mean follow-up of 4.3 years (interquartile range = 0.5-12 years). CONCLUSION: Neo-aortic reduction plasty with coronary reimplantation by punch technique is an effective strategy to approach preoperative APM in TGA. This technique confers a more harmonious geometry to NAR that can improve neo-aortic valve function.

Impact of the coronavirus disease 2019 (COVID-19) pandemic on the Italian congenital cardiac surgery system: a national survey.

OBJECTIVES: Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS: Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS: Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres. CONCLUSIONS: Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results.

Three-dimensional printing for surgical planning of a double aortic arch case.

BACKGROUND AND AIM: Over the past years, three-dimensional (3D) models of patient-specific anatomical conditions are being used to improve the comprehension and surgical management of a variety of diseases. It is an additional diagnostic tool that aids clinical decision-making. Furthermore, this technology is still not routinely used in the medical field since its availability is limited by cost and complex process. METHODS AND RESULTS: We describe a patient with a balanced-type double aortic arch encircling trachea and esophagus. Considering the clinical symptoms, surgical decompression of these structures and defined aortic arch reconstruction was indicated. The 3D printed model revealed narrowing of the left aortic arch at the junction of the descending thoracic aorta that did not clearly appear on the conventional images reconstruction. The left aortic arch was divided and the symptoms completely disappeared. No immediate or late complications occurred. CONCLUSION: 3D printed models can be helpful in surgical planning of congenital heart malformations. It should be strongly considered as an additional tool in complex cases.

Combined Surgical and Endoscopic Approach for Ring-Sling Complex.

BACKGROUND: Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated with long-segment tracheal stenosis (TS). Mortality rate in operated children is still high, especially in cases of severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric cohort of patients affected by PAS with severe tracheobronchial involvement. METHODS: From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring surgical intervention. Echocardiography, computed tomography, and bronchoscopy were performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant surgical repair for CHD was performed in three children. RESULTS: Over a mean follow-up of 30 months (range: 3-75 months), a late mortality of 18% was registered; no early death occurred. Good flow through LPA could be documented in all patients. Ten children required operative bronchoscopies (mean: 16/patients) aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial dilatation. CONCLUSIONS: Severe tracheobronchial stenosis and associated CHD were the main determinants for hospitalization time, intensive assistance, and repeated endoscopic procedures.Patients affected by PAS/TS complex require a careful management at high-specialized centers providing multidisciplinary team.Respiratory endoscopy may play a central role both in preoperatory assessment and in postoperative management of patients showing severe tracheobronchial involvement.

Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

OBJECTIVES: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound. DESIGN: Retrospective study. SETTING: Single center. PARTICIPANTS: Total cavopulmonary surgeries were reviewed from January 2016 to May 2019. INTERVENTIONS: Thoracic ultrasound with careful evaluation of the retrosternal area was performed at different postoperative times (12-36 hours, 5-7 days, and before discharge) as completion of routine echocardiography. MEASUREMENTS AND MAIN RESULTS: Among 37 children undergoing total cavopulmonary connection (mean age 5.5 ± 1.8 years [range 2.4-11.7]; mean body surface area 0.7 ± 0.1 m2 [range 0.3-1.6 m2]), retrosternal clots were detected in 18 (48.6%). Of these, 7 (13.5%) had small clots (<1 cm), 2 (5.4%) small to moderate sized clots (>1 cm-<2 cm), 3 (8.1%) moderate sized clots (>2-<3 cm), and 6 (16.2%) large clots (>3 cm). Four of the 6 detected large clots required surgical revision, and in the other 2 patients, the clots were not treated because the patients' conditions were clinically stable. When 3 major groups (group 1-no or small clots, group gropu 2 are small to moderate or moderate, group 3-large clots) were evaluated, no significant differences were noted in age, body surface area, CPB time, conduit type, or the number of previous surgeries. CONCLUSIONS: With thoracic ultrasound diagnosis, existence of retrosternal clots was found to be very common after total cavopulmonary connection. Most clots were small or moderate with no clinical effect; however, large clots that required redo surgery also were detected.

Impact of 3D printing on the surgical management of tracheal stenosis associated to pulmonary sling: a case report.

Congenital tracheal stenosis (CTS) is rare lesions frequently associated with pulmonary sling (PS). Despite the recent improvement in the results by the introduction of slide tracheoplasty (ST) and multidisciplinary approach, surgical management remains difficult and several authors, for this reason, debate for a conservative approach in these cases. Preoperative planning, require hence the use of the most innovative technology to gain the best possible result. This report describes a complicated, unsuccessful surgically treated case of CTS associated to PS. Based on this result and in order to improve the outcomes, we have built a 3D model of patient's trachea simulating preoperative surgical planning. We have hypnotized that having the model preoperatively the choice of surgical technique would have been different. Since that case, 3D models are introduced in our practice and built before tracheal or cardiac cases considered to have complex anatomy. Future investigations are required but at the moment this experience confirms the utility of 3D model in the evaluation of children considered for tracheal surgery.

Outcome of long-term complications after permanent metallic left bronchial stenting in children.

OBJECTIVES: We describe the way we treated 7 children with critical long-term complications after metallic balloon-expandable stenting in the left mainstem bronchus. METHODS: Endoscopic follow-up included a first bronchoscopy 3 weeks after stenting, then monthly for 3 months, every 4-6 months up to 1 year and at scheduled times to calibrate stent diameter up to final calibration. When major complications occurred, patients underwent chest computed tomographic angiography. RESULTS: In 1 of the 7 children (median age 2.8 years), metallic left bronchial stenting served as a bridge to surgery. After a median 4-year follow-up, all 7 children experienced recurrent stent ovalizations with stent breakage in 3 and erosion in 1. In 4 children, computed tomographic angiography showed abundant peribronchial fibrous tissue, in 2 left mediastinal rotation and in 1 displacement along the left bronchus after pulmonary re-expansion as the cause of stent-related complication. Of the 7 children, 6 underwent surgery (5 posterior aortopexy and 1 section of the ligamentum arteriosus) and 3 required nitinol stents placement within the metallic ones. One patient completed the follow-up, and 1 patient was lost to follow-up. All 5 remaining children still have permanent bronchial stents in place, patent and re-epithelialized after a median 10.5-year follow-up. There were no deaths. CONCLUSIONS: Satisfactory anatomical relationships when children have stents placed in the left mainstem bronchus alone do not guarantee the final success. Several mechanisms intervene to cause critical stent-related complications in children during growth. Permanent metallic stents should be used carefully, and only in selected patients.

Tracheal surgery in children: outcome of a 12-year survey.

OBJECTIVES: Despite the fact that team management has improved the results in recent years, perioperative deaths and complications remain high in paediatric tracheal surgery. We reviewed our institutional experience by comparing our results with those in the literature. METHODS: Between 2005 and 2017, 30 children underwent surgery for tracheal disease. Fifteen were boys and fifteen were girls (50% vs 50%). The median age at operation was 7 months (15 days-9.6 years), and the median weight was 5.2 kg (2.8-34 kg). Congenital tracheal stenosis was diagnosed in 25 children (83.3%), and 5 (16.7%) had acquired lesions. The mean internal diameter in congenital tracheal stenosis was 1.5 mm, with complete tracheal rings present in all patients. Associated malformations were bronchopulmonary in 11 cases (36.7%) and cardiovascular in 16 (53.3%). RESULTS: No in-hospital deaths occurred in our data set. Overall mortality was 4 of 30 cases (13.3%). Twenty-four endoscopic reinterventions were required in 19 children (63%) and consisted of stent positioning in 13 (43.3%), balloon dilatation in 5 (16.7%), granulation removal in 4 (13.3%) and tracheostomy in 2 (6.7%). Of the survivors (26 of 30, 86.7%), 11 children (42.3%) did not require further examination on adequate tracheal diameter for age and absence of symptoms after a median follow-up period of 3.5 years. CONCLUSIONS: The result of paediatric tracheal surgery depends on several factors. The number of cases treated at a particular centre is an important one, but our experience, although limited, can be compared with that at centres with a higher volume of cases. We emphasize the need for applying a multidisciplinary approach to master the surgical command of different reconstructive tracheal procedures, to manage associated defects, particularly cardiovascular defects, and to manage complications under endoscopic guidance. These can be considered the mainstays of building a successful tracheal programme.

Arterial Switch Operation and Plasma Biomarkers: Analysis and Correlation with Early Postoperative Outcomes.

The aims of our study were to describe plasma brain natriuretic peptide (BNP), Troponin I (TnI), and Cystatin C (Cys-C) concentration kinetics in the postoperative period after arterial switch operation in neonate, and to test the correlation between the plasma biomarkers and early clinical outcomes. We prospectively enrolled 29 neonates who underwent ASO. All patients received Custodiol cardioplegia. Blood samples were collected preoperatively (one day before) and in the ICU immediately after admission, and then 6, 12, 24, and 48 h after surgery. TnI peak (mean 17.23 ± 7.0 ng/mL) occurred between the arrival in the ICU and the 6th hour, then we had a constant decrease. TnI had a good correlation with the inotropic support time (r = 0.560, p = 0.0015) and ICU time (r = 0.407, p = 0.028), less than with ventilation and Hospital stay (r = 0.37, p = 0.0451 and r = 0.385, p = 0.0404). BNP peak (mean 4773.79 ± 2724.52 ng/L) was in the preoperative time with a constant decrease after the operation and it had no significant correlations with clinical outcomes. The CyS-C had the highest preoperative values, which decreased during the operating phase, and then constantly increased upon arrival to the ICU with a peak at 48 h (mean 1.76 ± 0.35 mg/L). CyS-C peak had a good correlation with a plasmatic creatinine peak (r = 0.579, p = 0.0009) but not with other clinical outcomes. Our study demonstrated significant correlations between the Tnl peak and early clinical outcomes in neonates undergoing arterial switch operation. Other plasma biomarkers such as the BNP and CyS-C had no direct correlation.

The role of posterior aortopexy in the treatment of left mainstem bronchus compression.

OBJECTIVES: We reviewed the role of posterior aortopexy for left mainstem bronchus compression in infants and children. METHODS: Eighteen children with respiratory symptoms were enrolled between 2005 and 2015 for surgical decompression of the left mainstem bronchus. The children were managed from diagnosis to follow-up by a dedicated tracheal team. Primary outcomes were the complete relief of symptoms or improvement with respect to preoperative clinical status. RESULTS: The median age was 4 years (0.3-15.4) and the median weight was 13.2 kg (3, 1-40). Symptoms or indications for bronchoscopy included difficult weaning from mechanical ventilation (n = 3, 17%), difficult weaning from tracheotomy (n = 4, 22%), recurrent pneumonia (n = 4, 22%), wheezing (n = 3, 17%), atelectasis (n = 1, 5.5%), bitonal cough (n = 1, 5.5%) and stridor (n = 2, 11%). Associated malformations were present in 88.7%. The diagnosis was made by bronchoscopy and computed tomography. Indication for surgery was the presence of pulsations and reduction in the diameter of the left mainstem bronchus compression of more than 70%. Surgery was performed by left posterolateral thoracotomy. Aortopexy was done under bronchoscopic control. No early or late deaths were observed, nor were reoperations necessary. Residual malacia was observed in 8 children (44%). Median follow-up was 4.1 years (0.1-7.1). At last follow-up, 17/18 (94.4%) children showed adequate airway patency. CONCLUSIONS: The intrathoracic location of the left mainstem bronchus predisposes it to compression. Vascular anomalies represent the most frequent causes. Aortopexy has been advocated as a safe and useful method to relieve the compression, and our results confirmed these findings. Management of these patients is challenging and requires a multidisciplinary team.

Custodiol Solution and Cold Blood Cardioplegia in Arterial Switch Operation: Retrospective Analysis in a Single Center.

BACKGROUND: The cardioplegia is one of the most significant tools used to increase myocardial protection. The aim of our study is to compare the use of Custodiol solution versus intermitted blood cardioplegia in a retrospective analysis of data for patients who underwent arterial switch operation in our institution. MATERIAL AND METHODS: From January 2008 to March 2011, myocardial protection was performed in 44 neonates (blood group) with intermittent blood cardioplegia. From March 2011 to November 2014, myocardial protection was performed in 50 neonates (Custodiol group) with one-shot anterograde Custodiol cardioplegia. RESULTS: Cardiopulmonary bypass and aortic cross-clamp were more favorable in Custodiol group (p-value 0.005 and ≤ 0.00001). The rate of delayed sternal closure was 63.6% in the blood group and 52% in the Custodiol group (p = 0.25). In the postoperative outcomes we did not find differences between the two groups. The 30-day mortality was one patient in the blood group (p = 0.46). We observed a transient ischemic electrocardiogram in 10 patients of the blood group and in 14 of the Custodiol group (p = 0.72), all cases with full resolution during hospitalization without coronary reoperation. A trend of higher peak of troponin-I and brain natriuretic peptide in Custodiol group has been reported. CONCLUSION: No prefect cardioplegia exists, the Custodiol solution does not cause extra/additional myocardial damage in arterial switch operation. In our experience this strategy seems warranted to simplify the procedure and to be more comfortable for the surgeon.

Prognostic role of BNP in children undergoing surgery for congenital heart disease: analysis of prediction models incorporating standard risk factors.

BACKGROUND: The routine use of brain natriuretic peptide (BNP) in pediatric cardiac surgery remains controversial. Our aim was to test whether BNP adds information to predict risk in pediatric cardiac surgery. METHODS: In all, 587 children undergoing cardiac surgery (median age 6.3 months; 1.2-35.9 months) were prospectively enrolled at a single institution. BNP was measured pre-operatively, on every post-operative day in the intensive care unit, and before discharge. The primary outcome was major complications and length ventilator stay >15 days. A first risk prediction model was fitted using Cox proportional hazards model with age, body surface area and Aristotle score as continuous predictors. A second model was built adding cardiopulmonary bypass time and arterial lactate at the end of operation to the first model. Then, peak post-operative log-BNP was added to both models. Analysis to test discrimination, calibration, and reclassification were performed. RESULTS: BNP increased after surgery (p<0.001), peaking at a mean of 63.7 h (median 36 h, interquartile range 12-84 h) post-operatively and decreased thereafter. The hazard ratios (HR) for peak-BNP were highly significant (first model HR=1.40, p=0.006, second model HR=1.44, p=0.008), and the log-likelihood improved with the addition of BNP at 12 h (p=0.006; p=0.009). The adjunction of peak-BNP significantly improved the area under the ROC curve (first model p<0.001; second model p<0.001). The adjunction of peak-BNP also resulted in a net gain in reclassification proportion (first model NRI=0.089, p<0.001; second model NRI=0.139, p=0.003). CONCLUSIONS: Our data indicates that BNP may improve the risk prediction in pediatric cardiac surgery, supporting its routine use in this setting.