Bicuspidisation of unicuspid stenotic pulmonary valve in a nine-year-old male.

We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure. The patient was referred for surgical repair.The atrial septal defect was closed by a direct running suture. The repair of the unicuspid valve consisted in bicuspidisation by a large commissurotomy to the left anterior wall of the pulmonary artery. The neo-commissure was created with two separate patches of autologous pericardium secured to the wall of the pulmonary root. The adjustment of the effective height of the pulmonary valve leaflets was done by trimming the patches and a triangular plication of the newly created posterior leaflet. Perioperative echocardiogram showed a peak gradient of 15 mm Hg and trivial pulmonary regurgitation. The total cross-clamp time was 92 min and the bypass time 123 min with a favourable evolution after the surgery.The particularity of the case is represented by the complexity of the bicuspidisation procedure. Using this technique, a tailored approach is needed for every patient.

Aortic transection for resection of middle mediastinal tumor.

BACKGROUND: Visceral mediastinal tumors are rare with challenging surgical approaches due to their location in close proximity with the great vessels and the pulmonary trunk. The aim of this paper is to discuss surgical strategies for complex cases of primary mediastinal tumors. METHODS: We present two cases of patients with middle mediastinal tumor, one synovial sarcoma and one paraganglioma. For both patients, surgical access was performed through a sternotomy with beating heart cardio-pulmonary bypass and aortic transection, allowing optimal exposure of the carina, of the common pulmonary artery and its bifurcation. Both tumors were resected 'en-bloc'. The postoperative course was uneventful and the two patients had a 3 months postoperative follow-up CT-scan showing no evidence of recurrence. RESULTS: Surgery remains the cornerstone of treatment for synovial sarcoma and for paraganglioma of the visceral mediastinum and this location may be difficult to deal with. Many different surgical accesses exist and our approach of ascending aortic transection allows optimal exposure to the pulmonary artery, but also provides access to the upper airways. CONCLUSION: For visceral mediastinal tumor with close contact with vascular and respiratory structures, aortic transection allows an excellent exposure and control of the tumor with oncological resection.

Epstein-Barr Virus-associated Pulmonary Tumor: A Pediatric Case and Discussion of the Literature.

Epstein-Barr virus-associated smooth pulmonary tumor is a rare condition that mostly affects immunosuppressed patients. This case describes a young boy with a history of kidney transplantation who presented recurrent pneumonia. Multiple endobronchial soft tissue tumors affecting both right and left bronchial tree were found and partially removed by bronchoscopy. Immunohistologic analysis demonstrated Epstein-Barr virus-associated smooth pulmonary tumor. Immunosuppressive therapy was changed from tacrolimus to sirolimus. A few months later, new right upper lobe and inferior left lobe tumors were found. Recurrent left lower lobe pneumonia prompted lobectomy. In the present case, complete resection and change of immunosuppressive treatment were effective.

Robotic-Assisted Lobectomy Favors Early Lung Recovery versus Limited Thoracotomy.

BACKGROUND: Postoperative pulmonary recovery after lobectomy has showed early benefits for the video-assisted thoracoscopic surgery and sparing open techniques over nonsparing techniques. Robotic-assisted procedures offer benefits in term of clinical outcomes, but their advantages on pulmonary recovery and quality of life have not yet been distinctly prospectively studied. METHODS: Eighty-six patients undergoing lobectomy over a period of 29 months were prospectively studied for their pulmonary function recovery and pain score level during the in-hospital stay and at 1, 2, and 6 months. Quality of life was evaluated at 2 and 6 months. Forty-five patients were operated by posterolateral limited thoracotomy and 41 patients by robotic approach. The postoperative analgesia protocol differed for the two groups, being lighter for the robotic group. RESULTS: The pulmonary tests were not significantly different during the in-hospital stay. At 1 month, the forced expiratory volume in 1 second, forced vital capacity, vital capacity, and maximal expiratory pressure were significantly better for the robotic group (p = 0.05, 0.04, 0.05, and 0.02, respectively). There was no significant difference left at 2 and 6 months. Pain intensity was equivalent during the in-hospital stay but was significantly lower for the robotic group at 1 month (p = 0.02). At 2 and 6 months, pain and quality of life were comparable. CONCLUSION: Robotic technique can offer similar pulmonary and pain recovery during the in-hospital stay with a lighter analgesia protocol. It clearly favors the early term recovery compared with the open limited technique. The objective and subjective functional recovery becomes equivalent at 2 and 6 months.

Combined Use of Rotational Thromboelastometry (Rotem) and Platelet Impedance Aggregometry (Multiplate Analyzer) in Cyanotic and Acyanotic Infants and Children Undergoing Cardiac Surgery With Cardiopulmonary Bypass: Subgroup Analysis of a Randomized Clinical Trial.

OBJECTIVES: Few studies have investigated the Multiplate platelet function analyzer in pediatrics. The authors evaluated Multiplate combined with Rotem in terms of guiding platelet transfusion after pediatric cardiac surgery with cardiopulmonary bypass (CPB). The authors further compared coagulation parameters between cyanotic and acyanotic patients. DESIGN: Subgroup analysis of a randomized clinical trial. SETTING: Tertiary hospital. PARTICIPANTS: Patients weighing between seven and 15 kg. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Rotem and Multiplate tests were performed (1) after anesthesia induction, (2) upon CPB separation, and (3) upon intensive care unit arrival. Among a total of 59 subjects, 9 patients required platelet transfusion. In multivariate linear regression, analysis EXTEM maximum clot firmness upon CPB separation was associated with the volume of transfused platelets (regression coefficient = -0.348 [95% confidence interval -1.006 to -0.028]; p = 0.039). No such association was found for the Multiplate test. Acyanotic and cyanotic heart disease were present in 32 and 27 children, respectively. There were no significant differences between these two groups in terms of platelet count and function. Postoperative blood loss was significantly higher in the cyanotic group compared with the acyanotic arm (p = 0.015; difference [95% confidence interval -2.40 {-4.20 to -0.60}]). There were no differences between groups regarding transfusion of allogeneic blood products. CONCLUSIONS: This study showed that Rotem, but not Multiplate results, were associated with platelet transfusion in pediatric cardiac surgery with no intake of platelet inhibitors. The usefulness of combining these tests in platelet transfusion decision-making needs to be evaluated in larger populations.

Autologous vascularised pericardial flap tunneling technique in Scimitar syndrome repair with dextrocardia: lessons from the Senning procedure.

Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Surgery is required when pulmonary overcirculation is present. Following repair, Scimitar vein stenosis occurs in approximately 20%. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation.

Fresh Frozen Plasma versus Crystalloid Priming of Cardiopulmonary Bypass Circuit in Pediatric Surgery: A Randomized Clinical Trial.

BACKGROUND: In congenital cardiac surgery, priming cardiopulmonary bypass (CPB) with fresh frozen plasma (FFP) is performed to prevent coagulation abnormalities. The hypothesis was that CPB priming with crystalloids would be different compared with FFP in terms of bleeding and/or need for blood product transfusion. METHODS: In this parallel-arm double-blinded study, patients weighing between 7 and 15 kg were randomly assigned to a CPB priming with 15 ml · kg PlasmaLyte or 15 ml · kg FFP in addition to a predefined amount of packed red blood cells used in all patients. The decision to transfuse was clinical and guided by point-of-care tests. The primary endpoints included postoperative bleeding tracked by chest tubes, number of patients transfused with any additional blood products, and the total number of additional blood products administered intra- and postoperatively. The postoperative period included the first 6 h after intensive care unit arrival. RESULTS: Respectively, 30 and 29 patients in the FFP and in the crystalloid group were analyzed in an intention-to-treat basis. Median postoperative blood loss was 7.1 ml · kg (5.1, 9.4) in the FFP group and 5.7 ml · kg (3.8, 8.5) in the crystalloid group (P = 0.219); difference (95% CI): 1.2 (-0.7 to 3.2). The proportion of patients additionally transfused was 26.7% (8 of 30) and 37.9% (11 of 29) in the FFP and the crystalloid groups, respectively (P = 0.355; odds ratio [95% CI], 1.7 [0.6 to 5.1]). The median number of any blood products transfused in addition to priming was 0 (0, 1) and 0 (0, 2) in the FFP and crystalloid groups, respectively (P = 0.254; difference [95% CI], 0 [0 to 0]). There were no study-related adverse events. CONCLUSIONS: The results demonstrate that in infants and children, priming CPB with crystalloids does not result in a different risk of postoperative bleeding and need for transfusion of allogeneic blood products.

Late management of the aortic root after repair of tetralogy of Fallot: A European multicentre study.

OBJECTIVES: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). METHODS: Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). RESULTS: The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). CONCLUSIONS: Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.

An unusual case of cardiac tamponade: Bronchogenic cyst infection due to Salmonella bredeney.

We present an unusual case of cardiac tamponade in a 17-year-old girl immunocompetent patient due to Salmonella enterica ssp. bredeney following infection of a bronchogenic cyst. The patient was admitted to hospital with pleuritic chest pain, dyspnoea and fever. Pulmonary angio-CT showed a bronchogenic cyst compressing the left atrium. The echocardiography showed diffuse pericardial effusion with right ventricular collapse consistent with cardiac tamponade. Pericardiocentesis was performed and microbiological cultures of the pericardial fluid became positive for Salmonella species confirmed later as bredeney subspecies by PCR. Empirical antibiotherapy was started with intravenous (IV) ceftriaxone. Bronchogenic cyst infection was suspected and confirmed by 18FDG PET CT. The patient was successfully treated by complete resection of the cyst and continuation of IV ceftriaxone followed by oral amoxicillin/clavulanate for a total duration of 6 weeks. She then completely recovered and didn't present any relapse after 6 months of follow up.

Comparing the Host Reaction to CorMatrix and Different Cardiac Patch Materials Implanted Subcutaneously in Growing Pigs.

BACKGROUND: Comparing the structural changes, and local host reactions to CorMatrix (CorMatrix Cardiovascular Inc., Roswell, Georgia, United States) and different biomaterials implanted subcutaneously in growing pig model. METHODS: Four pigs harboring implanted patches of CorMatrix, Vascutek porcine pericardium (Vascutek; Scotland, United Kingdom), SJM bovine pericardium (St. Jude Medical, Inc., Minnesota, United States), and Gore-Tex (W. L. Gore & Associates GmbH, Flagstaff, Arizona, United States) were studied for 1, 3, 6, and 12 months. The explants were examined histologically. RESULTS: CorMatrix showed gradual and consistent patch resorption and subsiding inflammatory and fibrosis process. Full scaffold degradation and replacement by mild fibrosis and subcutaneous tissue were seen by 1 year. Xenopericardial patches remained intact, and the initially severe inflammatory and fibrotic reactions reduced gradually to moderate fibrosis and chronic inflammation. Gore-Tex showed foreign body reaction. CONCLUSIONS: Patches were biotolerated by pigs. Xenopericardial patches elicited encapsulating fibrosis and no remodeling. CorMatrix resorbs completely and degrades consistently without leaving residues. Lack of encapsulating fibrosis toward CorMatrix allows tissue ingrowth and matrix remodeling.

Multilevel factors are associated with immunosuppressant nonadherence in heart transplant recipients: The international BRIGHT study.

Factors at the level of family/healthcare worker, organization, and system are neglected in medication nonadherence research in heart transplantation (HTx). The 4-continent, 11-country cross-sectional Building Research Initiative Group: Chronic Illness Management and Adherence in Transplantation (BRIGHT) study used multistaged sampling to examine 36 HTx centers, including 36 HTx directors, 100 clinicians, and 1397 patients. Nonadherence to immunosuppressants-defined as any deviation in taking or timing adherence and/or dose reduction-was assessed using the Basel Assessment of Adherence to Immunosuppressive Medications Scale© (BAASIS© ) interview. Guided by the Integrative Model of Behavioral Prediction and Bronfenbrenner's ecological model, we analyzed factors at these multiple levels using sequential logistic regression analysis (6 blocks). The nonadherence prevalence was 34.1%. Six multilevel factors were associated independently (either positively or negatively) with nonadherence: patient level: barriers to taking immunosuppressants (odds ratio [OR]: 11.48); smoking (OR: 2.19); family/healthcare provider level: frequency of having someone to help patients read health-related materials (OR: 0.85); organization level: clinicians reporting nonadherent patients were targeted with adherence interventions (OR: 0.66); pickup of medications at physician's office (OR: 2.31); and policy level: monthly out-of-pocket costs for medication (OR: 1.16). Factors associated with nonadherence are evident at multiple levels. Improving medication nonadherence requires addressing not only the patient, but also family/healthcare provider, organization, and policy levels.

Recent Experience and Follow-Up After Surgical Closure of Secundum Atrial Septal Defect in 120 Children.

While percutaneous catheter closure proves an effective treatment for secundum atrial septal defect (ASD2), some child patients require surgical closure. We assessed the risks associated with isolated surgical ASD2 closure by reviewing the outcomes of 120 children operated on between 1999 and 2011 (mean age 4.6 ± 3.9 years, mean weight 17 ± 12 kg). Direct sutures were performed in 4% and patch closures in 96%. The mean cardiopulmonary bypass duration was 38 ± 14 min, aortic cross-clamp time 19 ± 9 min, intensive care unit length of stay 1.6 ± 1.1 days, hospital stay 11.2 ± 5.1 days. There were no complications in 60 patients (50%) and major complications in 8 (6.7%), with 1 patient (0.8%) dying of pneumonia-induced sepsis, 2 (1.7%) requiring revision surgery, 3 (2.5%) requiring invasive treatment (2 pericardial drainage, 1 successful resuscitation), and 2 (1.7%) presenting thromboembolisms (1 cerebral stroke, 1 cardiac thrombus). In hospital minor complications occurred in 22 patients: 17 pericardial effusions (15%), 15 infections requiring treatment (12.5%), 1 sternal instability (0.8%), 4 anemias requiring transfusion (3.3%), 7 pulmonary atelectasis (6%), and 2 post-extubation glottis edema (1.7%). At early outpatient follow-up, complications occurred in 21 patients: 16 (13.3%) pericardial effusions, 4 (3.3%) infections requiring treatment, and 3 (2.5%) keloid scarring. No complications occurred during long-term follow-up. In line with published data, mortality was low (0.8%), yet major complications (6.7%) were more common in these cases than those following percutaneous ASD2 closure. Minor complications were frequent (43%) with no long-term sequelae.

Experimental Aortic Valve Cusp Extension with CorMatrix in a Porcine Model.

Background We tested the feasibility of using porcine small intestinal submucosal extracellular matrix (CorMatrix) for aortic valve (AV) repair in porcine model examining its resorption and remodeling potential. Methods The non-coronary cusp was replaced with CorMatrix in four animals for 120 days. Valve function was assessed by echocardiography. Explants were examined by histology, immunohistochemistry, and collagen assessment. Results CorMatrix was almost totally replaced with tissue resembling the native cusp with a partial two-layer architecture. However, function was lost due to thickening and calcification. Conclusions Tested in high-pressure AV position in a pig model, CorMatrix degrades and remodels, but also loses function.

Pain, Quality of Life, and Clinical Outcomes after Robotic Lobectomy.

Background To evaluate pulmonary function, pain, and quality of life at midterm after robotic lobectomy performed in a single institution. Methods Sixty-five consecutive patients underwent robotic thoracic surgery over 32 months using a complete four-arm portal technique. Sixty-one patients underwent lobectomies predominantly for stage I non-small cell lung cancer. Pulmonary function tests were repeated at midterm follow-up. Pain and quality of life were evaluated during the follow-up on a subgroup of 39 patients, excluding the learning period. Results At a mean of 7-month follow-up, there was no significant difference in preoperative and midterm postoperative pulmonary function. A total of 62.5% of the patients reported a variable intensity of discomfort or pain at the surgical site, with a mean pain intensity score of 2.1 ± 1.4. Mean pain interference score were weak (1.8 ± 1.9), with patients with moderate pain reporting significantly higher pain interference scores than those with mild pain (p = 0.0025). Only one patient suffered from neuropathic-like pain. Quality of life was globally favorable and related to the pain level, with a significant interference on the physical component. Conclusion Robotic lobectomy does not appear to have an impact on midterm pulmonary function. Persistent postoperative pain is mild, nonneuropathic-like, with weak interference on daily activities. Quality of life is satisfactory but related to the pain level.

Does Postoperative Cardiac Troponin-I Have Any Prognostic Value in Predicting Midterm Mortality After Congenital Cardiac Surgery?

OBJECTIVES: This study evaluated the prognostic value of postoperative cardiac troponin-I (cTnI) in predicting all-cause mortality up to 3 months after normothermic congenital cardiac surgery. DESIGN: Prospective observational study. SETTING: University hospital. PARTICIPANTS: All children ages 0 to 10 years. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: cTnI was measured after the induction of anesthesia but before the surgery, at the pediatric intensive care unit arrival, and at 4, 12, and 24 hours postoperatively. Follow-up was extended up to 6 months. Overall, 169 children were analyzed, of whom 165 were survivors and 4 were nonsurvivors. cTnI levels were significantly higher in nonsurvivors only at 24 hours (p = 0.047). Children undergoing surgery with cardiopulmonary bypass (CPB) had significantly higher cTnI concentrations compared with those without CPB (p<0.001). Logistic regression analysis was performed on the 146 children in the CPB group with the following predictive variables: CPB time, postoperative cTnI concentrations, the presence of a cyanotic malformation, and intramyocardial incision. None of the variables predicted mortality. Postoperative cTnI concentrations did not predict 6 months׳ mortality. Only cTnI at 24 hours predicted the length of stay in the pediatric intensive care unit. CONCLUSIONS: This study did not find that postoperative cTnI concentration predicted midterm mortality after normothermic congenital heart surgery. (ClinicalTrials.gov identifier: NCT01616394).

Prenatal diagnosis of isolated total anomalous systemic venous connection to the left atrium.

We report the prenatal diagnosis and the neonatal follow-up of a patient with isolated total abnormal systemic venous connection to the left atrium. Right-sided and left-sided superior caval veins and the inferior caval vein were all connected to the left atrium. Pulmonary venous return was normal. This was associated with some right ventricular underdevelopment. To our knowledge, this is the first fetal description of this very rare congenital cardiac malformation.

Hypothermic continuous machine perfusion improves metabolic preservation and functional recovery in heart grafts.

The number of heart transplants is decreasing due to organ shortage, yet the donor pool could be enlarged by improving graft preservation. Hypothermic machine perfusion (MP) has been shown to improve kidney, liver, or lung graft preservation. Sixteen pig hearts were recovered following cardioplegia and randomized to two different groups of 4-hour preservation using either static cold storage (CS) or MP (Modified LifePort© System, Organ Recovery Systems, Itasca, Il). The grafts then underwent reperfusion on a Langendorff for 60 min. Energetic metabolism was quantified at baseline, postpreservation, and postreperfusion by measuring lactate and high-energy phosphates. The contractility index (CI) was assessed both in vivo prior to cardioplegia and during reperfusion. Following reperfusion, the hearts preserved using CS exhibited higher lactate levels (56.63 ± 23.57 vs. 11.25 ± 3.92 µmol/g; P < 0.001), increased adenosine monophosphate/adenosine triphosphate (AMP/ATP) ratio (0.4 ± 0.23 vs. 0.04 ± 0.04; P < 0.001), and lower phosphocreatine/creatine (PCr/Cr) ratio (33.5 ± 12.6 vs. 55.3 ± 5.8; P <0.001). Coronary flow was similar in both groups during reperfusion (107 ± 9 vs. 125 + /-9 ml/100 g/min heart; P = ns). CI decreased in the CS group, yet being well-preserved in the MP group. Compared with CS, MP resulted in improved preservation of the energy state and more successful functional recovery of heart graft.

Long-Term Results of Balloon Valvuloplasty as Primary Treatment for Congenital Aortic Valve Stenosis: a 20-Year Review.

In the presence of new surgical techniques, the treatment of congenital valvular aortic stenosis is under debate. We reviewed the results and late outcomes of all 93 patients aged 1 day to 18 years, treated with balloon valvuloplasty (BAV) as first-line therapy for congenital aortic valve stenosis in our center from January 1991 to May 2012. Mean age at procedure time was 2.4 years; 37 patients underwent BAV at age ≤30 days (neonates), 29 patients at age ≥1 month and <1 year (infants), and 27 patients were older than 1 year (children). The invasive BAV peak-to-peak aortic valve gradient (mean 59 ± 22 mmHg) was immediately reduced (mean 24 ± 12 mmHg). The observed diminution of gradient was similar for each age group. Four patients had significant post-BAV AI. Mean follow-up after BAV was 11.4 ± 7 years. The last echo peak aortic gradient was 37 ± 18 mmHg and mean gradient was 23 ± 10 mmHg, and two patients had significant AI. Actuarial survival for the whole cohort was 88.2 and 72.9 % for the neonates. All infants, except one, and all children survived. Sixty-six percent of patients were free from surgery, and 58 % were free from any reintervention, with no difference according to age. Freedom from surgery after BAV at 5, 10, and 20 years, respectively, was 82, 72, and 66 %. Our study confirms that BAV as primary treatment for congenital AS is an efficient and low-risk procedure in infants and children. In neonates, the prognosis is more severe and clearly related to "borderline LV."

Long-term outcome and anaesthetic management for non-cardiac surgery after Fontan palliation: a single-centre retrospective analysis.

OBJECTIVES: The improved management of Fontan patients has resulted in good outcome. As such, these patients may necessitate care for non-cardiac surgery. We sought to determine the long-term outcome of our Fontan series palliated with the most recent surgical techniques. Our second objective was to report the incidence and the perioperative course after non-cardiac procedures. We reviewed the records of all patients with either a lateral tunnel or an extracardiac conduit Fontan between 1996 and 2008. Follow-up was recorded until June, 2013, including records regarding non-cardiac interventions. RESULTS: Overall, 58 patients were included. Of them, one patient underwent a takedown of his Fontan, and five patients died in the immediate postoperative course. The cumulative survival of the remaining 52 patients was 81%. There was no significant difference in survival between right and left ventricle morphologies (p=0.56), nor between both types of Fontan (p=0.9). Chronic arrhythmias (25%), fatigue/dyspnoea (40%), thrombotic complications (19%), and embolic events (10%) were among the most recurrent comorbidities. In total, 45 non-cardiac interventions were performed on 26 patients, with three bleeding complications and one death. CONCLUSIONS: This study shows excellent long-term survival after both lateral tunnel and extracardiac conduit Fontan. The incidence of cardiovascular morbidity remains high, however. We also report a high number of non-cardiac interventions. Thorough understanding of the Fontan physiology is mandatory when non-cardiac anaesthesiologists are in charge of these patients.

Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience.

Background: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. We report our center experience of vascular ring management over three decades. Methods: In this single-center retrospective study, data were extracted from patient medical records. To obtain information on symptoms/medication at follow-up, clinical records from pediatric clinics were reviewed. For patients followed in other institutions, a web-based questionnaire was sent to referring pediatricians. Results: Out of 82 patients, 69 were symptomatic (84%). Common symptoms included recurrent respiratory tract infections (43%), stridor (32%), gastro-esophageal reflux (33%), and dysphagia (26%). Diagnosis relied on cardiac ultrasound, barium swallow studies, and chest computerized tomography scan. Surgical repair (thoracotomy 91%) was performed in 79 patients (96%). Median age at repair was 13 [interquartile range (IQR), 4.4-48] months. There was no mortality. Minor complications occurred in 14 patients (18%). Median hospital length of stay was 7 (IQR, 6-9) days. In total, 24% of patients remained symptomatic (median follow-up 54 months). Half of those were asthma-related, with nearly 90% freedom from ring-related symptoms. Conclusions: Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair. Level of Evidence: III.