Anatomical Risk Factors for Reintervention after Arterial Switch Operation for Taussig–Bing Anomaly

Background: This study aimed to determine the factors related to reintervention, especially for pulmonary artery stenosis (PS), in patients with Taussig–Bing anomaly (TBA) after arterial switch operation (ASO). Methods:This retrospective study included 34 patients with TBA who underwent ASO between 1993 and 2018. Preoperative anatomical and physiological differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries (TGA) with ventricular septal defect (VSD) and TBA with an anterior and rightward aorta. Results: The median age and body weight at ASO were 43 (16–102) days and 3.6 (2.8–3.8) kg, respectively. Aortic arch obstruction and coronary anomalies were present in 64% and 41% patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery: aortic annulus size ratio than those not receiving reintervention (1.69 vs. 1.41, P = 0.02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the TGA/VSD group. PS-related reintervention was required more in the TBA group than in the TGA/VSD group. Conclusions: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomical relationship during ASO. (243 words

Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience

Objectives: The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex (HLHC). Methods: A retrospective observational study was performed in 136 patients with HLHC who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analyzed. Results: Stage 1 survival was 91.9% (125/136). Reintervention for pulmonary artery stenosis was needed for 22% and 30% at stage 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while four were not considered to be Fontan candidates. Risk factors for overall mortality included weighing Conclusions: Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain pulmonary artery growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion

Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes

Background This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot (TOF) on pulmonary valve annulus (PVA) growth, the rate of valve-sparing repair (VSR) at the time of intracardiac repair (ICR), and long-term functional outcomes. Methods This retrospective study included 330 patients with TOF who underwent ICR between 1991 and 2019, including 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0±7.3 years. We compared the data of patients who underwent BTS and those who did not undergo BTS before ICR. Results The median age and body weight before BTS were 71 (28–199) days and 4.3 (3.3–6.8) kg respectively. There were no in-hospital or interstage deaths after BTS. The PVA Z-scores of patients with BTS revealed significant growth after BTS (from -4.2±1.8 to -3.0±1.7, P Conclusions A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. BTS may have contributed to the avoidance of primary transannular patch repair (TAP) and facilitated PVA growth; therefore, approximately half of the symptomatic neonates and infants were recruited for VSR. Staged repair may have led to functionally-reliable delayed TAP repair, thereby resulting in less surgical reinterventions

Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

Background: There is limited information on outcomes of adult patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the low incidence of disease and the large variation of surgical histories. Methods: Among 58 patients with repaired PA-IVS, a total of 32 patients aged ≥16 years and who were followed at our institution between January 2003 and December 2018 were reviewed. Surgical history, clinical outcomes, and laboratory, echocardiographic and electrocardiographic data were obtained by chart review. Results: Follow-up was from the age of 16 years and the median age at the latest follow-up was 23.7 years. Twenty-four patients had undergone biventricular repair (BVR), 3 had undergone one-and-a half ventricular repair (1.5VR), and 5 had undergone univentricular repair. Over a median follow-up period of 7.7 years (interquartile range: 4.1–11.0 years), 1 BVR patient died suddenly and 7 patients had heart failure. Arrhythmias were present in 5 patients. Ten patients underwent surgical re-interventions, including 4 BVR take-downs with conversion to 1.5VR and 3 Fontan conversions. Overall survival, heart failure-free, arrhythmia-free, and surgical re-intervention-free rates at 5 years and 10 years from the age of 16 years were 96.2% (95% confidence interval [CI], 77.2–99.4) and 96.2% (95% CI, 77.2– 99.4), 81.4% (95% CI, 62.1–92.1) and 74.6% (95%CI, 52.3–88.7), 88.7% (95% CI, 70.1–96.3) and 75.9% (95% CI, 51.7–90.2), and 80.7% (95% CI, 60.8–91.8) and 70.8% (95% CI, 49.7–85.7), respectively. Conclusion: Adults with PA-IVS have preserved long-term survival regardless of the early operative strategy, while they are at risk for heart failure, arrhythmia, and surgical re-intervention. Thus, detailed and continued follow-up is mandatory for all PA-IVS patients from childhood to adulthood

Intracoronary Autologous Cardiac Progenitor Cell Transfer in Patients With Hypoplastic Left Heart Syndrome (TICAP) : A Prospective Phase 1 Controlled Trial

RATIONALE: Hypoplastic left heart syndrome (HLHS) remains a lethal congenital cardiac defect. Recent studies have suggested that intracoronary administration of autologous cardiosphere-derived cells (CDCs) may improve ventricular function. OBJECTIVE: The aim of this study was to test whether intracoronary delivery of CDCs is feasible and safe in patients with hypoplastic left heart syndrome. METHODS AND RESULTS: Between January 5, 2011, and January 16, 2012, 14 patients (1.8±1.5 years) were prospectively assigned to receive intracoronary infusion of autologous CDCs 33.4±8.1 days after staged procedures (n=7), followed by 7 controls with standard palliation alone. The primary end point was to assess the safety, and the secondary end point included the preliminary efficacy to verify the right ventricular ejection fraction improvements between baseline and 3 months. Manufacturing and intracoronary delivery of CDCs were feasible, and no serious adverse events were reported within the 18-month follow-up. Patients treated with CDCs showed right ventricular ejection fraction improvement from baseline to 3-month follow-up (46.9%±4.6% to 52.1%±2.4%; P=0.008). Compared with controls at 18 months, cardiac MRI analysis of CDC-treated patients showed a higher right ventricular ejection fraction (31.5%±6.8% versus 40.4%±7.6%; P=0.049), improved somatic growth (P=0.0005), reduced heart failure status (P=0.003), and lower incidence of coil occlusion for collaterals (P=0.007). CONCLUSIONS: Intracoronary infusion of autologous CDCs seems to be feasible and safe in children with hypoplastic left heart syndrome after staged surgery. Large phase 2 trials are warranted to examine the potential effects of cardiac function improvements and the long-term benefits of clinical outcomes