Innominate Artery Pseudoaneurysm Requiring Cartilage Tracheal Repair in a Child.

Mycotic aneurysms are rare but potentially catastrophic. We report a case of an innominate artery pseudoaneurysm in a 4-year-old patient that caused a tracheoinnominate fistula requiring tracheoplasty with a costal cartilage graft and a homograft iliac artery replacement of the diseased innominate artery, with a successful outcome.

Stereo-EEG tailored resection in a child with presumed perinatal post-stroke epilepsy: The complex organization of epileptogenic zone.

Introduction: Only a few studies have focused on tailored resection in post-stroke epilepsy, in which hemispherectomy and hemispherotomy are the most recognized treatments. Case description: We describe the case of a patient with drug-resistant, presumed perinatal, post-stroke epilepsy and moderate right hemiparesis. The seizures were stereotyped, both spontaneous and induced by sudden noises and somatosensory stimuli. Considering the discordant anatomic-electro-clinical data - left perisylvian malacic lesion with electrical onset over the left mesial fronto-central leads - and the patient's functional preservation, SEEG was performed. SEEG revealed sub-continuous abnormalities in the perilesional regions. Several seizures were recorded, with onset over the premotor area, rapidly involving the motor and insular-opercular regions. We decided for a combined surgical approach, SEEG-guided radiofrequency thermocoagulation, on the fronto-mesial structure but also on the central operculum, followed by resective surgery including only the fronto-mesial structures. Discussion and conclusion: The SEEG allowed to localize the epileptogenic zone far away from the anatomical lesion but connected to part of it. A combined surgical approach tailored on SEEG results allowed a good outcome (Engel Ib) without additional deficits.

A case report of truncus arteriosus with intact ventricular septum and crossed branch pulmonary arteries.

Background: Truncus arteriosus (TA) or persistent arterial trunk describes the presence of a solitary arterial trunk arising from the base of the heart, supported by a common ventriculoarterial junction. The trunk gives rise to the coronary arteries, systemic arteries, and at least one pulmonary artery. Truncus arteriosus is a rare congenital cardiac disease, and even rarer is the absence of ventricular septal defect. Case summary: We report a case of a 2-day-old infant who presented with cyanosis and a cardiac murmur. He was diagnosed with TA with intact ventricular septum (IVS), as well as crossed pulmonary arteries which was identified on pre-operative imaging. We describe the surgical management and short-term follow-up. Discussion: Our clinical case demonstrates a unique diagnosis and management of TA with IVS identified by pre-operative imaging with good surgical outcome.

Increasing Use of Neonatal Catheter Intervention for Pulmonary Atresia With Intact Ventricular Septum: Management Trends From a Single Centre.

BACKGROUND: There is increasing use of catheter-based therapy as part of the neonatal treatment algorithm for pulmonary atresia with an intact ventricular septum (PAIVS). The management strategies utilised and outcomes of patients with PAIVS at our centre have not been examined. METHODS: A retrospective case series was undertaken including all infants with PAIVS born January 2009 to July 2019 (follow-up to January 2020) managed at The Children's Hospital at Westmead, New South Wales. Demographic features, anatomical substrate, management pathway and subsequent clinical outcomes were examined. RESULTS: Fifty-two (52) infants were included (male n=28, 53.8%). The right ventricular morphology was classified as normal, mildly, moderately and severely hypoplastic in 3 (5.8%), 13 (25.0%), 19 (36.5%) and 17 (32.7%) patients respectively. Thirty-seven (37) patients underwent an initial aortopulmonary (AP) shunt (surgical shunt or patent ductus arteriosus [PDA] stent). The remaining 15 patients underwent an initial intervention to decompress the right ventricle. Twenty (20) patients underwent a neonatal catheter-based intervention. An initial catheter-based intervention was more likely in the second half of the period. Sixteen (16) patients had an attempted pulmonary valve perforation, 12 as their initial procedure. Median follow-up was 62 months (range 3-119 months). Final circulation status was known in 37 patients; biventricular n=14 (37.8%), "1.5 ventricles" n=4 (10.8%), single n=19 (51.4%). There were five deaths during the period (9.6%), including two during the initial procedural admission attributed to tamponade requiring extracorporeal membrane oxygenation (ECMO) at the time of percutaneous pulmonary valve perforation. CONCLUSION: There has been an overall trend towards including catheter-based strategies in the neonatal period as part of management at our centre. Given the risk of bleeding and ECMO related to this, consideration should be given to the availability of multidisciplinary support when planning the timing of these procedures.

Retrieval of neonatal and paediatric patients on extracorporeal membrane oxygenation support in New South Wales, Australia.

New South Wales has recently added the capability of extracorporeal membrane oxygenation to the neonatal and paediatric retrieval process and this paper describes the early experiences and protocol development for the first eight cases transported.

The Congenital Heart Technical Skill Study: Rationale and Design.

BACKGROUND: We report the rationale and design for a peer-evaluation protocol of attending congenital heart surgeon technical skill using direct video observation. METHODS: All surgeons contributing data to The Society of Thoracic Surgeons-Congenital Heart Surgery Database (STS-CHSD) are invited to submit videos of themselves operating, to rate peers, or both. Surgeons may submit Norwood procedures, complete atrioventricular canal repairs, and/or arterial switch operations. A HIPPA-compliant website allows secure transmission/evaluation. Videos are anonymously rated using a modified Objective Structured Assessment of Technical Skills score. Ratings are linked to five years of contemporaneous outcome data from the STS-CHSD and surgeon questionnaires. The primary outcome is a composite for major morbidity/mortality. RESULTS: Two hundred seventy-six surgeons from 113 centers are eligible for participation: 83 (30%) surgeons from 53 (45%) centers have agreed to participate, with recruitment ongoing. These surgeons vary considerably in years of experience and outcomes. Participants, both early and late in their careers, describe the process as "very rewarding" and "less time consuming than anticipated." An initial subset of 10 videos demonstrated excellent interrater reliability (interclass correlation = 0.85). CONCLUSIONS: This study proposes to evaluate the technical skills of attending pediatric cardiothoracic surgeons by video observation and peer-review. It is notable that over a quarter of congenital heart surgeons, across a range of experiences, from almost half of United States centers have already agreed to participate. This study also creates a mechanism for peer feedback; we hypothesize that feedback could yield broad and meaningful quality improvement.

Surgical closure of atrial septal defects.

Surgical repair of an atrial septal defect (ASD) is a safe and effective operation with little to no morbidity and mortality. In an effort to reduce the trauma of surgery, current approaches focus on less invasive surgical techniques, rather than the intracardiac repair. We will describe the different types of ASD, techniques for repair, and options for minimally invasive repair.

The ordeal of left atrioventricular valve replacement in children under 1 year of age.

OBJECTIVES: This study reviews the outcomes of children under 1 year of age who had left atrioventricular valve (LAVV) replacement (LAVVR) in one centre and explores the benefits of an innovative approach for LAVVR in very small patients. METHODS: Thirteen consecutive patients operated for LAVV replacement between 1997 and 2016 were reviewed retrospectively. Indication for surgery was regurgitation in 7, stenosis in 5 and both stenosis and regurgitation in 1. Nine patients (69%) had previous LAVV repair. Median age at surgery was 126 days (39-327 days). In the primary surgery, 7 mechanical valves and 1 mitral homograft were implanted. Five inverted semilunar valve conduits were implanted consisting of a Contegra valve in 4 and a pulmonary homograft in 1. RESULTS: Hospital mortality was 31% (4 of 13). Two patients required postoperative extracorporeal membrane oxygenation. Six patients developed complete atrioventricular block, with 2 survivors requiring a pacemaker. Late mortality was 31% (4 of 13). Two of the 4 patients who received an inverted Contegra conduit died. Median follow-up of the 5 survivors was 4 years (2-16 years). Four patients had 10 further replacements consisting of 6 redo conventional mechanical valves replacement, 3 supra-annular valve implantation, and 1 modified Ross II. The 5 inverted semilunar valve conduits implanted lasted for 1, 5, 6, 22 and 37 months. CONCLUSIONS: LAVVR below 1 year of age is associated with a considerable operative and late mortality. LAVVR with an inverted conduit bearing semilunar valves may be an alternative strategy for patients with the smallest annuli.

Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. RESULTS: Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). CONCLUSIONS: A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.

Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization.

BACKGROUND: This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy. METHODS: From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries. RESULTS: At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91). CONCLUSIONS: Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.

Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience.

OBJECTIVE: Management of pulmonary atresia with intact ventricular septum (PAIVS) can be directed to either biventricular repair or univentricular palliation. The optimal management strategy has yet to be defined. METHODS: All patients operated at the Royal Children's Hospital, Melbourne for PAIVS between 1990 and 2006 (n = 81) were reviewed. Patients were retrospectively stratified into a simple three-tiered classification based on right ventricle (RV) size. Multivariate logistic regression analysis was performed to identify risk factors of mortality. RESULTS: The distribution of RV sizes was normal in 11 (14%), moderate hypoplasia in 45 (56%), and severe hypoplasia in 25 (31%) patients. RV-to-coronary-artery connections were present in 33 (41%) and RV coronary dependence in six patients (7%). Sixteen patients died (20%). The end-status of the remaining patients was biventricular repair in 31/81 (38%), 1½-ventricle repair in 10/81 (12%), Fontan circulation in 14/81 (17%), transplantation in 1/81 (1%), and still awaiting repair in 9/81 (11%). Ten-year survival was 80% (95% confidence interval (CI): 71-87%). Independent predictors of mortality were lower tricuspid valve (TV) annulus size Z-score and the presence of RV-to-coronary-artery connections. CONCLUSIONS: A simple three-tiered classification based on RV size may allow initial stratification into biventricular or univentricular repair for patients with normal RV size and severe RV hypoplasia. In patients with moderate RV hypoplasia, the presence of RV-to-coronary-artery connections or a TV Z-score<-2 should caution one against attempting biventricular repair.

Extended three-patch technique for congenital supravalvular aortic stenosis.

We describe a modified extended 3-patch surgical procedure to relieve supravalvular aortic stenosis. This simple technique provides effective and lasting enlargement of the ascending aorta in patients with severe congenital supravalvular aortic stenosis.

Pulmonary atresia, VSD and Mapcas: repair without unifocalization.

The last three decades have seen considerable development in the management of infants with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collaterals. The technical difficulty of surgical treatment lies in the fashioning of a pulmonary vascular bed capable of providing suitably low right ventricular pressure for repair of the intracardiac defect. With the current trend toward early and complete unifocalization of major aortopulmonary collateral arteries (mapcas), we describe an alternative approach - "repair without unifocalization" - and explain our reasoning for pursuing this technique.

Surgical management of invasive pulmonary fungal infection in hematology patients.

BACKGROUND: The purpose of this study was to analyze our institutional results with pulmonary resection in neutropenic patients with hematologic malignancies and suspected invasive pulmonary fungal infections. METHODS: We performed a retrospective medical record review of 25 immunocompromised patients with hematologic malignancies who underwent pulmonary resection between 2000 and 2007. We analyzed preoperative diagnostic technique, degree of pulmonary resection, and postoperative morbidity and mortality to determine whether surgery is a viable treatment option in this subset of patients. RESULTS: Twenty-three of 25 patients had a minithoracotomy compared with 2 who had video-assisted thorascopic surgery resection only. Thirteen had wedge resections, 9 had lobectomies, and 3 had segmentectomies. Early surgical morbidity was 2 of 25, involving 1 pneumothorax and 1 empyema. In-hospital mortality was 2, with 1 death primarily related to surgery. Median survival was 342 days, and survival was significantly better in patients with only one lesion. No patient experienced late recurrence of invasive pulmonary fungal infection. Resected pulmonary tissue also provided the best chance for a proven diagnosis in 19 of 25 (76%). CONCLUSIONS: This study confirms that pulmonary resection in high-risk immunocompromised patients with suspected invasive fungal infection can be carried out with excellent operative morbidity and mortality.

Re-implantation of an anomalous right coronary artery arising from the left coronary sinus and coursing between the great arteries.

The patient with a coronary artery anomaly remains a treatment dilemma. We present a 62-year-old woman who underwent re-implantation of her anomalous right coronary artery (ARCA) from the left coronary sinus and describe our techniques according to potential anatomic variations of ARCA. The ARCA from the left coronary sinus is increasingly being recognized as a cause of angina, acute myocardial infarction, syncope, and sudden death. We describe a case that was treated by direct coronary artery re-implantation into the right coronary sinus and suggest that this technique be the first considered when planning surgical correction.

Late presentation digital ischemia after radial artery harvest for coronary artery bypass.

The radial artery is increasingly being used as a coronary artery bypass graft. Morbidity from harvesting is rare, yet it does occur. We present a case of digital ischemia presenting late after surgery and suggest that although preoperative assessment may be normal, comorbidities such as collagen vascular disease in conjunction with atherosclerotic peripheral vascular disease should be carefully considered as a contraindication to radial artery harvest.

Cutaneous microangiopathic thrombosis complicated by pyoderma gangrenosum in post-cardiac surgery heparin-induced thrombocytopaenia.

Thrombotic cutaneous gangrene is a rare complication of heparin-induced thrombocytopaenia after cardiac surgery. We report a case and discuss management issues with cardiopulmonary bypass for cardiac surgery in this condition.