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.

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 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.

Anomalous Aortic Origin of a Coronary Artery is Always a Surgical Disease.

Anomalous aortic origin of a coronary artery is a congenital anomaly in which a major coronary artery arises from the wrong sinus of Valsalva (left coronary from right sinus or right coronary from left sinus) and courses between the great arteries before reaching its normal epicardial course. Although the risk of sudden death is clearly established, the indications for surgery remain controversial. The risk of sudden death is increased in symptomatic patients, in anomalous left coronary artery, as well as in the presence of some risk factors (intense physical activity, young age [<35 years], aggravating anatomical features [intramural interarterial course, slit-like ostium, acute angle of take-off]). As far as is currently known, surgical management using an anatomical repair can prevent sudden death, provided that normal coronary anatomy and function are achieved and that extensive return-to-play testing is performed. A precise evaluation of the benefit/risk ratio is mandatory on an individual basis, but surgery may be indicated in the vast majority of patients.

Anomalous aortic origin of coronary arteries: 'anatomical' surgical repair.

Anomalous aortic origin of coronary arteries (left coronary from right sinus or right coronary from left sinus) is a rare congenital defect, which carries a high risk of sudden cardiac death. The risk is particularly high when the interarterial course between the great arteries has an intramural segment, or is hypoplastic/stenotic, or has an abnormal orifice. Various surgical techniques have been used, including coronary artery bypass grafting, pulmonary artery translocation, partial or complete unroofing of the intramural course and patch enlargement of the interarterial course. We favour 'anatomical' repair that creates an enlarged neo-ostium into the appropriate sinus, eliminates completely the intramural segment and restores a normal angle of take-off. Reimplantation of the anomalous coronary artery may be indicated in variants without an intramural course. Surgical correction is mandatory for symptomatic and asymptomatic patients with evidence of myocardial ischaemia under stress; it is recommended in asymptomatic patients with high-risk variants (anomalous left coronary artery with the intramural course), particularly in young patients with strenuous activities.

Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot.

OBJECTIVES: Right ventricle to pulmonary artery connection (RVPA connection) without prosthetic material has been our ideal strategy to palliate pulmonary atresia with ventricular septal defect (VSD) or severe tetralogy of Fallot for the last decade. We speculate that RVPA connection ensures adequate postoperative haemodynamics for symptomatic neonates and promotes pulmonary artery rehabilitation. The present study was undertaken to assess the outcome of this strategy. METHODS: Between 2000 and 2010, among 107 patients who benefited from an RVPA connection, 57 were neonates. Forty-eight of these underwent autologous tissue reconstruction, 5 using left atrial appendage. Median weight was 2.9 kg (range 1.8-4.4). Median Nakata index was 100 mm2/m2 (range 17-185 mm2/m2); 12% had major aortopulmonary collaterals. All patients were reviewed retrospectively. End-points were death or complete repair; reintervention for restrictive pulmonary blood flow was considered as failure. At follow-up, we evaluated reintervention after complete repair, and quality of life. RESULTS: There were 2 early deaths (RV hypoplasia and RV failure) and 3 late sudden deaths (range 3-6 months). Pulmonary blood flow required to be increased in 8 patients: 4 underwent shunt after a median delay of 1 month; RVPA connection enlargement was needed in 3; 1 patient had percutaneous angioplasty. Finally, 47 patients (81%) had a complete repair, of which 70% were performed without prosthetic material at a median age of 7 months (range 2-53), with a median Nakata index of 221 mm2/m2 (range 102-891). One patient died early and 1 was a failure with opening of the VSD after intracardiac repair. At last follow-up, 4 patients were still awaiting repair, with 1 late death and 5 who had required reintervention after intracardiac repair; there were 3 conduit replacements and 2 balloon dilatation patch enlargements. CONCLUSIONS: The neonatal RVPA connection approach (i) provides an acceptable survival rate with a satisfactory haemodynamic adaptation, (ii) facilitates rehabilitation of PAs and (iii) avoids the use of prosthetic graft at correction.

Pitfalls in repair of conotruncal anomalies.

Despite a wide anatomic diversity, the complete repair of all conotruncal anomalies includes two surgical steps. 1) An intracardiac tunnel is created to connect the left ventricle to one of the arterial orifices (usually the aortic, sometimes the pulmonary), through the conoventricular ventricular septal defect. Any conal septum should be resected to create a short, large, and straight tunnel. Abnormal insertions of the atrioventricular valves (tricuspid and mitral) on the conal septum should be preserved. "Intramural" residual ventricular septal defects must be avoided by anchoring the intracardiac patch directly to the arterial annulus. 2) To connect the right ventricle to the pulmonary artery, either an intracardiac or an extracardiac reconstruction is carried out, according to the distance between the tricuspid valve and the pulmonary orifice. When extracardiac reconstruction is indicated, it is usually performed without prosthetic conduit (with or without French maneuver, eventually using the left atrial appendage). In most patients, complete repair can be performed as a primary operation during infancy.

Early initiation of peritoneal dialysis in neonates and infants with acute kidney injury following cardiac surgery is associated with a significant decrease in mortality.

Association between early renal replacement therapy and better survival has been reported in adults with postoperative kidney injury, but not in children undergoing cardiac surgery. We conducted a retrospective cohort study of 146 neonates and infants requiring peritoneal dialysis following cardiac surgery in a tertiary referral hospital. A propensity score was used to limit selection bias due to timing of dialysis, and included baseline and intraoperative characteristics, requirement for postoperative extracorporeal membrane oxygenation, and creatinine clearance variation. Inverse probability of treatment weighting resulted in good balance between groups for all baseline and intraoperative variables. After weighting, 30-day and 90-day mortality were compared between the 109 patients placed on dialysis early, within the first day of surgery, and those with delayed dialysis, commencing on the second day of surgery or later, using logistic regression and survival analysis. Mortality was 28.1% at 30 days, and was 36.3% during follow-up. Early dialysis was associated with a 46.7% decrease in the 30-day and a 43.5% decrease in the 90-day mortality rate when compared with delayed dialysis. All other short-term outcome variables were similar. Thus, initiation of peritoneal dialysis on the day of or the first day following surgery was associated with a significant decrease in mortality in neonates and infants with acute kidney injury.

Bidirectional cavopulmonary shunt with additional pulmonary blood flow: a failed or successful strategy?

OBJECTIVES: In patients with single ventricle physiology, Fontan circulation is considered as the optimal surgical approach, although it entails a growing incidence of late complications. It has been speculated that the association of bidirectional cavopulmonary shunt (BCPS) and additional pulmonary blood flow (APBF) might provide long-lasting palliation. The present study was undertaken to assess the long-term outcome of this strategy. METHODS: A group of 70 patients with single ventricle physiology, who underwent BCPS with APBF between 1990 and 2000, were reviewed retrospectively. Median age at operation was 2 years (range: 0.1-27 years). Unilateral BCPS was performed in 60 patients (86%), bilateral BCPS in 9 and the Kawashima procedure in 1. APBF was provided through antegrade pulmonary outflow tract in 43 patients (61%) and by aortopulmonary shunt in 27 (39%). Two patients died early and three were lost to follow-up. Mean follow-up of the 65 remaining patients was 13.5 ± 4 years. End-points were death, need for heart transplantation (HTx) or Fontan completion and functional outcome. RESULTS: Five patients died (two after HTx, three from ventricular failure); overall actuarial survival was 89 ± 4% at 15 years. Six patients underwent HTx (one after Fontan completion) with two early deaths and no late mortality. Fifty-one patients underwent Fontan completion (11 with additional palliative procedures before completion); there was no early or late mortality following Fontan completion; one patient underwent HTx. Among the remaining six patients with BCPS and APBF, two were not suitable for Fontan completion and four remained suitable. Overall, clinical failure (mortality, HTx, unsuitability for Fontan completion) occurred in 13 patients (19%). Risk factors for clinical failure were older age at BCPS (P = 0.01) and postoperative complications after BCPS (P = 0.001). Considering late mortality, HTx and Fontan completion as strategic failures, the actuarial freedom from these events was 20 ± 5% at 10 years. CONCLUSIONS: BCPS with APBF approach: (i) fails as a strategy for definitive palliation, (ii) provides a high survival rate, (iii) does not preclude a successful Fontan completion and (iv) may delay the long-term deleterious consequences of Fontan circulation. Palliation by BCPS with APBF should be achieved early in life.

Adult congenital surgery: current management.

The population of adults with congenital heart disease (CHD) (commonly called grown-ups with congenital heart disease or GUCH) is increasing steadily and exceeds the population of children with CHD already. The specificities of GUCH surgery are multiple and include (1) variety of the anatomo-clinical situations (defects repaired during childhood, malformations either nonoperated or palliated, nonreparable defects), (2) usual multiorgan involvement, and (3) many technical differences related to cardiopulmonary bypass, myocardial protection, and surgical technique. The surgical indications should be taken after a precise evaluation of the risk/benefit ratio on an individual basis; a balanced attitude should be kept between unwise interventionism and excessive waiting policy. It is now agreed that GUCH surgery should be performed in specialized centers with large patient volumes and expertise of both surgical and medical disciplines. Much remains to be done to implement these recommendations and to accumulate experience and evidence-based information to provide optimal outcome.