Outcomes of aortic valve repair in children stratified by complexity: Which outcome for which lesion?

OBJECTIVE: Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques. METHODS: All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups). RESULTS: During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension. CONCLUSIONS: Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity.

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

OBJECTIVES: The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.

Ross procedure or complex aortic valve repair using pericardium in children: A real dilemma.

OBJECTIVE: Difficult to repair aortic valve lesions, requiring the use of a valve substitute, remain controversial in the face of the Ross procedure, despite undeniable technical advances. This study was undertaken to compare midterm outcomes of children treated using the Ross procedure or aortic valvuloplasty for complex aortic valve lesions. METHODS: Between January 2006 and December 2017, 126 patients aged younger than 18 years were treated for complex aortic stenosis and/or aortic insufficiency and were included in this retrospective study. Only aortic valve lesions requiring repair with an autologous or heterologous pericardial patch were considered complex lesions. Propensity score framework analyses were used to compare outcomes of the Ross and aortic valvuloplasty groups while controlling for confounders. RESULTS: Among the 126 patients with complex aortic valve lesions, propensity score matching selected 34 unique pairs of patients with similar characteristics. Survival (aortic valvuloplasty, 94.1%; Ross, 91%; P = .89), freedom from overall reintervention (aortic valvuloplasty, 50.1%; Ross, 69%; P = .32), and freedom from infective endocarditis at 8 years (aortic valvuloplasty, 100%; Ross, 85.9%; P = .21) were similar. However, freedom from reintervention in the left ventricular outflow tract at 8 years was lower after aortic valvuloplasty than after the Ross procedure (50.1% vs 100%, respectively; P = .001). CONCLUSIONS: Aortic valvuloplasty and the Ross procedure yielded similar 8-year outcomes regarding death, reoperation, and infective endocarditis although aortic valvuloplasty tended to be associated with fewer cases of infective endocarditis. Aortic valvuloplasty using a pericardial patch can be chosen as a first-line strategy for treating complex aortic valve lesions and might offer the possibility of a later Ross procedure.

Three-dimensional geometry of coronary arteries after arterial switch operation for transposition of the great arteries and late coronary events.

OBJECTIVE: Using 3-dimensional (3D) modeling to predict late coronary events after the arterial switch operation (ASO) for transposition of the great arteries (TGA). METHOD: We reviewed 100 coronary computed tomography scans performed after ASO randomly selected from free-from-coronary-event patients and 21 coronary computed tomography scans from patients who had a coronary event later than 3 years after ASO. Using 3D modeling software, we defined and measured 6 geometric criteria for each coronary artery: Clockwise position of coronary ostium, First centimeter angle defined as the angle between of the coronary artery ostium and the first centimeter of the vessel, Minimal 3D angle between the coronary first centimeter and the aortic wall, ostium height defined as the distance between the ostium and the aortic valve, distance between the coronary ostium and the pulmonary artery, and distance between the coronary first centimeter and the pulmonary artery. RESULTS: None of the right ostium geometric parameters were associated with coronary events. Four out of 6 criteria of left coronary artery geometry were associated to coronary events: Clockwise position of the left ostium >67° (P < .001), First centimeter angle >62° (P < .01), minimal 3D angle <39° (P = .003), distance between the coronary ostium and the pulmonary artery <1 mm/mm (P = .03). The association of first centimeter angle >62° and minimal angle in 3D <39° had a 88% sensitivity and a 81% specificity to predict coronary events (receiver operator characteristics curve, 0.847; 95% confidence interval, 0.745-0.949; P < .001). CONCLUSIONS: The acquired geometric characteristics of the transferred left coronary artery are associated with coronary events. Imaging coronary arteries after ASO might be useful to select patients at higher risk of coronary events and to tailor surveillance.

Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy.

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in children and young adults. The most threatening anatomy is an interarterial and an intramural course, both probably involved in ischaemic phenomena and sudden death. The treatment of interarterial AAOCA remains controversial. Most of the published studies describe the results of the unroofing technique. Our study aims to evaluate the results of a different surgical approach. METHODS: From 2005 to 2019, 61 patients were operated on for an interarterial AAOCA (median age 14.7 years). Forty patients had a right AAOCA, and 21 patients had a left AAOCA including 5 patients with intraseptal course. Seventy percent of patients were symptomatic. Five patients had an aborted sudden cardiac death. Two surgical techniques were used: an 'anatomical' repair for 35 patients (15 left and 22 right AAOCA) or a coronary translocation with creation of a neo-ostia in 19 patients (1 left and 18 right AAOCA). The 5 left AAOCA patients with an intra-septal course required a complete release of the coronary artery from the septum. RESULTS: There was no early or late postoperative death. Three patients had an acute postoperative ischaemic event. Two patients required immediate angioplasty and stenting: 1 patient (7 years) with a hypoplastic right AAOCA and 1 patient (66 years) for inadequate tailoring after septal release. The third patient required an immediate surgical revision (H-2) for left AAOCA thrombosis at the level of the pericardial patch with full myocardial recovery at discharge. During follow-up, 1 patient with right AAOCA translocation and chronic chest pain required subsequent stenting and finally a coronary artery bypass grafting 2 years after initial surgery. One patient who had an asymptomatic mild right coronary stenosis 1 year after anatomical repair was successfully treated by angioplasty alone. All patients but 1 who underwent coronary translocation are totally asymptomatic. All patients with anatomical repair or septal release are free from ischaemic symptoms. CONCLUSIONS: Anatomical repair might provide a better protective option for these patients. Unlike unroofing, it treats the entire intramural segment, relocates the ostium at the appropriate sinus level and corrects any acute take-off angle.

Prenatal Diagnosis of Aorto-Left Ventricular Tunnel With Dysplastic Bicuspid Aortic Valve: From Fetal Cardiac Failure to Favorable Outcome.

Aorto-left ventricular tunnel (ALVT) is a rare congenital heart defect. Surgery has to be performed early to avoid life-threatening complications. Prenatal diagnosis of this defect is challenging. We report a case of ALVT diagnosed in a fetus showing premature severe cardiac failure at 24 GA. The new born was operated at day 3 of life with good results. Two years later, he is still doing well recovering a complete normal cardiac function. ALVT should be suggested in front of any fetal cardiac failure. Thanks to early diagnosis, prompt neonatal management can be organized and allows positive outcome.

Ascending aorta and aortic root replacement (with or without valve sparing) in early childhood: surgical strategies and long-term outcomes.

OBJECTIVES: Aortic root and ascending aorta replacements (AARs) are rarely required in the paediatric population. We report here a series of AAR performed in young children using different surgical techniques. METHODS: Between 1995 and 2017, 32 children under the age of 10 years (median age 5.4 years) underwent AAR procedures at our institution. Twenty-two (69%) had a connective tissue disease (infantile Marfan syndrome or Loeys-Dietz syndrome). We performed 11 AAR using a composite graft with a mechanical prosthesis and 21 valve-sparing procedures (10 Yacoub operations and 11 David operations). Median follow-up for operative survivors was 7.7 years (interquartile range 4.2-12.8 years). RESULTS: The cardiac-related early mortality rate was 6%. Patient survival was 91% at both 1 and 10 years. Eleven survivors (38%), all with a status of post-valve-sparing procedure, required an aortic root reintervention with an aortic valve replacement after a median interval of 4.2 years. Interestingly, only patients with infantile Marfan syndrome tended to be associated with risk of reoperation. CONCLUSIONS: Aortic root and AARs are safe in young children whatever the surgical procedure. Aortic valve-sparing procedures show good long-term results except in children with infantile Marfan syndrome whose ineluctable aortic annulus dilatation or aortic valve regurgitation requires reintervention after a short period.

Single-Shot Cold Histidine-Tryptophan-Ketoglutarate Cardioplegia for Long Aortic Cross-Clamping Durations in Neonates.

OBJECTIVE: More than 30% of European pediatric cardiac surgery centers use single-dose cold histidine-tryptophan-ketoglutarate cardioplegia (Custodiol; Dr Franz Köhler Chemie GmbH, Bensheim, Germany). In neonates with transposition of the great arteries, arterial switch surgery (ASO) implies aortic division, and it is unknown whether repeated ostial cannulation causes intimal insult and affects long-term results, and therefore, single-dose Custodiol is appealing. The present study investigated the association among myocardial no-flow duration, postoperative troponins, and postoperative outcomes in neonates undergoing ASO with Custodiol cardioplegia. DESIGN: Retrospective analysis of the association among myocardial no-flow duration, postoperative troponin release (concentration magnitude × measurement duration within 48 h), and outcomes using stratification according to coronary anatomy and attending surgeon. SETTING: Single-institutional, tertiary pediatric cardiac surgery unit of a university hospital. PARTICIPANTS: The study comprised 101 neonates undergoing ASO. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean age of patients was 6.1 ± 5.4 days, the cardiopulmonary bypass duration was 108.7 ± 54.1 minutes, the temperature during cross-clamping was 31.1°C ± 1.7°C, the duration of mechanical ventilation was 4 (3-6) days, the length of intensive care unit stay was 7 (5-8) days, delayed sternal closure occurred in 32 (31.7%) patients, and no patients died. The myocardial no-flow duration averaged 62.3 ± 14.6 minutes and was linked with both troponin release (p = 0.04) and low cardiac output syndrome, as assessed by the requirement for delayed sternal closure (p = 0.03), regardless of cardiopulmonary bypass duration and temperature. Eighty-two percent of the patients with myocardial no-flow duration >74 minutes necessitated delayed sternal closure. CONCLUSIONS: Single-dose Custodiol may be inadequate for prolonged cross-clamping durations without myocardial perfusion in neonates.

Perfusion Study Helps in the Management of the Intraseptal Course of an Anomalous Coronary Artery.

Anomalous origin of a coronary artery from the opposite aortic sinus of Valsalva can present in various ways, ranging from a benign and incidental finding to sudden cardiac death. The variant with an intraseptal subpulmonary course (sometimes referred to as intraconal), is widely perceived to carry a low risk of ischemia and has been considered to be a benign variant, not requiring surgical treatment. In one of our recent patients, however, nuclear scintigraphy highlighted a myocardial perfusion deficit in the territory supplied by the allegedly benign anomalous coronary artery, prompting the need for a more aggressive surgical approach.

Double orifice and atrioventricular septal defect: dealing with the zone of apposition†.

OBJECTIVES: A double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations. METHODS: We performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month-32 years). RESULTS: Associated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination. CONCLUSIONS: Repair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.

Tetralogy of Fallot and abnormal coronary artery: use of a prosthetic conduit is outdated.

OBJECTIVES: Repair of tetralogy of Fallot (ToF) can be challenging in the presence of an abnormal coronary artery (CA) in 5-12% of cases. The aim of this study was to report our experience with ToF repair without the systematic use of a right ventricle-to-pulmonary artery (RV-PA) conduit. METHODS: We conducted a monocentric retrospective study from 2000 to 2016, including 943 patients with ToF who underwent biventricular repair, of whom 8% (n = 76) presented with an abnormal CA. Mean follow-up time was 50 months (1 month-18 years). RESULTS: The most frequent CA anomaly was the left descending artery arising from the right CA (n = 47, 61.8%). The median age at repair was 7.7 months (1.8 months-16 years). Thirteen patients (17%) required prior palliation, mostly systemic pulmonary shunts for anoxic spells in the neonatal period. Surgical repair allowed us to preserve the annulus in 40 patients (53%) by combining PA trunk plasty, commissurotomy and infundibulotomy under the abnormal CA. If the annulus had to be opened (n = 35, 46%), a transannular patch was inserted after a vertical incision of the PA trunk and extended obliquely on the RV over the anomalous crossing CA (with an infundibulotomy under the abnormal CA). Three patients (4%) required the insertion of an RV-PA conduit (1 valved tube and 2 RV-PA GORE-TEX tubes with annulus conservation). The early mortality rate was 4% (n = 3); none of the deaths was coronary related. Four patients (5%) required reoperation (2 early and 2 late reoperations) for residual pulmonary stenosis, 3 of whom had annulus preservation during the initial repair. The mean RV/left ventricle (LV) pressure ratio and an RV/LV pressure ratio >2/3 were identified as risk factors for right ventricular outflow tract (RVOT) reinterventions (P = 0.0026, P = 0.0085, respectively), RVOT reoperations (P = 0.0002 for both) and reoperation for RVOT residual stenosis (P = 0.0002, P = 0.0014, respectively). Two patients underwent pulmonary valve replacement. Freedom from late reoperation was 100% at 1 year, 97% at 5 years and 84% at 10 and 15 years. CONCLUSIONS: Repair of ToF and abnormal CA can be performed without an RV-PA conduit, with an acceptable low reintervention rate. The high early mortality rate in this series remains a concern. If any doubt remains about the surgical relief of the RVOT obstruction, the RV/LV pressure ratio should always be measured in the operating room.

Mitral valve replacement with a pulmonary autograft: long-term follow-up in an infant.

A 7-month-old boy with a complete atrioventricular septal defect presented with severe left atrioventricular valve regurgitation 4 months after complete repair. As the valve was unsuitable for the repair and the annulus was too small to accommodate a mechanical prosthesis, the modified mitral Ross operation was performed. The long-term outcome was uneventful for 12 years. The mitral Ross procedure is an old-described technique in which classically the pulmonary autograft is encased in a prosthetic conduit preventing any growth potential. On the contrary, the modified technique used in this case shows that the long-term function can be obtained. This procedure may be a valuable option when mitral valve replacement is necessary in infants.

Crossed pulmonary arteries as additional cause of dysphagia in association with right aortic arch and Kommerell diverticulum.

We describe an uncommon association of crossed pulmonary arteries and a right aortic arch with a Kommerell diverticulum and a left ligamentum arteriosum, resulting in disabling dysphagia in a 33-year-old woman. First, endovascular exclusion of the Kommerell diverticulum was performed using a thoracic stent graft, associated with left subclavian-carotid transposition. Second, open aneurysmorrhaphy and division of the left ligamentum arteriosum allowed a proper release of the oesophageal compression. Dysphagia completely disappeared in the postoperative course. Control computed tomography angiography at 6-month follow-up showed a satisfactory hybrid repair. A complete understanding of the combined effects of these two anatomical variations on oesophageal compression led to a suitable surgical management.

Long-term results of the modified Konno procedure in high-risk children with obstructive hypertrophic cardiomyopathy.

OBJECTIVE: Transaortic septal myectomy is the gold standard surgery in obstructive hypertrophic cardiomyopathy, but it is not optimal for children aged less than 5 years and with right ventricular outflow tract obstruction. We evaluated outcomes with the modified Konno procedure in children with severe forms of obstructive hypertrophic cardiomyopathy. METHODS: A total of 79 consecutive children who underwent the modified Konno procedure in our center between 1991 and 2016 were included. RESULTS: Clinical features included age less than 5 years (38%), maximal septal thickness 25 mm or more (32%), extension to the left ventricular apex (29%), and right ventricular outflow tract obstruction (28%). In total, 25% of patients had Noonan syndrome. Five children (6%) aged less than 15 months with Noonan syndrome and biventricular obstruction died in the hospital. Mean follow-up was 6 ± 5.7 years. Survival without death and heart transplantation was 82% at 20 years. Atrioventricular block occurred in 9 patients (11%) and was associated with right ventricular outflow tract obstruction and surgery before 2010. Death, resuscitated sudden cardiac death, and appropriate implantable defibrillator shock were associated with maximal septal thickness before surgery (adjusted odds ratio, 1.20; 95% confidence interval, 1.07-1.35; P = .002) and need for an associated procedure (adjusted odds ratio, 8.84; 95% confidence interval, 2.01-38.93; P = .004). There was no case of recurrent obstruction. Reoperation was required in 4 patients (5%) for other reasons. CONCLUSIONS: The modified Konno procedure provided durable obstruction relief and good long-term survival in children with severe forms of obstructive hypertrophic cardiomyopathy. Children with Noonan syndrome undergoing surgery early in life were at higher risk of early mortality.

Outcomes of the anatomical repair in patients with congenitally corrected transposition of the great arteries: lessons learned in a high-volume centre.

OBJECTIVES: The physiological repair of the congenitally corrected transposition of the great arteries (ccTGA) has been associated with a long-term risk of the right ventricular dysfunction and tricuspid valve regurgitation. On the other side, the anatomical repair with the restoration of the left ventricle in a systemic position has been hypothesized to improve long-term outcomes. The aim of this study was to determine the results of the anatomical repair. METHODS: Between 1990 and 2015, 39 patients underwent an anatomical repair of a ccTGA. The median age was 7 years (range from 8 days to 42.8 years). Eighteen (46%) patients had a double switch. Of these, 21 (54%) patients had an atrial switch with a Rastelli technique (n = 16, 41%), with a réparation à l'étage ventriculaire (REV) procedure (n = 4, 10%) or a truncal rotation (n = 1, 3%). The follow-up was 100% complete at 2 years with a median follow-up of 3.17 years (range 0-26 years). RESULTS: The early mortality was 10% (n = 4) and the late mortality was 20% (n = 8). The long-term survival was 77 ± 10% at 10 years for the double switch group and 62 ± 11% at 10 years for the Rastelli group (P = 0.25). Eight (20%) patients required reintervention. Freedom from reoperation at 10 years was 82 ± 11% in the double switch group and 88 ± 11% in the Rastelli group. At the last follow-up, 23 (82%) patients were in New York Heart Association (NYHA) functional class I and 2 (7%) patients in NYHA class II. CONCLUSIONS: The anatomical repair of the ccTGA is associated with significant early mortality and morbidity. However, the long-term functional status is satisfactory. The anatomical repair of the ccTGA should be reserved for a carefully selected group of patients.

Particular surgical aspects of endocarditis due to Kingella kingae with cerebral complication.

We report 2 cases of Kingella kingae endocarditis leading to valvular mitral perforation in previously healthy children. Kingella kingae belongs to the HACEK (Haemophilus aphrophilus, Actiobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and K. kingae) group of organisms known to cause endocarditis.

Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect.

OBJECTIVES: To determine the early, intermediate and long-term outcomes of pulmonary atresia with ventricular septal defect (PA/VSD) Types I, II and III initially palliated by a right ventricle to pulmonary artery (RVPA) connection. METHODS: We performed a retrospective study from 2000 to 2014 that included 109 patients with PA/VSD who had undergone an RVPA connection (tetralogy of Fallot and PA/VSD Type IV excluded). The end-points of this strategy were adequate pulmonary artery tree post-palliation, second palliation, biventricular repair, right ventricular pressure post-biventricular repair and late reoperation. Mean follow-up was 5.4 years (1 day to 14-78 years). RESULTS: Early mortality after an RVPA connection was 2.7% (3 of 109). The interstage mortality rate was 6.6% (7 of 106). Eighty-four (77%) patients had a biventricular repair and 8 patients (7%) are awaiting repair. Overall survival was 90% at 1 year and 81% at 10 years. The RVPA connection allowed significant growth of the native pulmonary artery with a Nakata index of 101 mm2/m2 before the RVPA connection and 274 mm2/m2 after (P = 0.001). Twenty-nine reinterventions for restrictive pulmonary blood flow have been done (9 before 2 months and 20 after 2 months). Of the 84 patients who had a repair, 22 patients (26%) initially had a right ventricular pressure greater than 40 mmHg. Twenty-eight patients (33%) required late reoperation. CONCLUSIONS: Hospital deaths after the RVPA connection were low. The procedure allowed good growth of the native pulmonary artery. Biventricular repair was possible in a large number of cases. The late morbidity rate remains significant. Early reinterventions could be avoided by appropriate calibration. This technique appears to be suitable for any type of PA/VSD with central pulmonary arteries.

Hypoplastic left heart syndrome: a novel surgical strategy for small-volume centres?

OBJECTIVES: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach. METHODS: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia. Initial results led to tightening of the pulmonary artery band from 3.5 mm to 2.5 mm. RESULTS: From July 2014 to May 2016, 15 patients were initially palliated (13 patients with aortic atresia/mitral atresia, 1 with double inlet right ventricle, 1 with tricuspid atresia) at a median age of 5 days (1-8 days) and a median weight of 3.0 kg (2.3-3.9 kg). All but 2 patients were discharged from the hospital: There was 1 early in-hospital death and 1 patient required hospitalization in the intensive care unit until stage 2. There were also 4 interstage deaths. Nine patients underwent Stage 2 and 1 patient is still waiting. Compared to the classic Norwood procedure, early mortality decreased significantly (43.3-6.7%; P = 0.0074) and overall mortality before Stage 2 was halved (61.6-33.3%). Postoperative morbidity was also reduced. CONCLUSIONS: This new surgical palliative approach to hypoplastic left heart syndrome, particularly useable technically, seems to combine the advantages of the hybrid procedure by avoiding cardiac ischaemia and ventriculotomy without the complications of PDA stenting and restrictive atrial septectomy. Although the mortality rate decreased significantly, it remains substantial in small-volume centres, especially in the interstage period.

Single coronary artery and neonatal arterial switch operation: early and long-term outcomes.

OBJECTIVES: The presence of single coronary artery (CA) in the arterial switch operation (ASO) for neonatal treatment with transposition of the great arteries (TGA) has been reported to be an independent risk factor for early death after surgical repair and late reintervention. The study objective was to evaluate the mortality and the CA stenosis risk at early and long term in neonatal ASO for TGA and single CA. METHODS: Between January 1987 and January 2010, 979 neonates underwent an ASO, of which 73 had a single CA (7.5% of all cohort): right ostium with posterior left CA loop was the most frequent pattern (63%), followed by left ostium with an anterior right CA loop (26%). Mean age at operation was 9.3 ± 5.7 days. Mean follow-up was 9.8 years (range: 1-20 years). RESULTS: Eight patients (11%) died, 6 of coronary-related death. Overall, survival was 90.1 ± 1.9% at 1 year and 88.6% ± 3.8% at 2, 5, 10 and 15 years, respectively. Independent risk factor for mortality was associated surgery before 2001. Freedom from coronary events was 91.6 ± 3.3% at 1 year and 88.7 ± 3.8% at 5, 10 and 15 years respectively. No patients required late reintervention for CA surgery or angioplasty. CONCLUSIONS: All coronary-related death occurred within the first 6 months after ASO, and all patients but 1 were operated before 2001. In our experience, it appears that a single CA is not any more a risk factor for early and late mortality after ASO for TGA. Mortality has drastically reduced since 2001 and is now close to that found in TGA with standard coronary patterns. The acquired experience shared between the surgeons and the institution offsets the undeniable surgical difficulty.