Transplant-Free Survival and Interventions at 6 Years in the SVR Trial

Abstract

Background In the Single Ventricle Reconstruction (SVR) trial, one-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS) in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. Methods Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. Results Transplant-free survival for the RVPAS vs. MBTS groups did not differ at 6 years (64% vs. 59%, P=0.25) or with all available follow-up of 7.1±1.6 years (log-rank P=0.13). The RVPAS vs. MBTS treatment effect had non-proportional hazards (P=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before Stage II surgery (HR=0.66; 95% CI 0.48–0.92). The effect of shunt type on death or transplant was not statistically significant between Stage II to Fontan surgery (HR 1.36, 95% CI 0.86–2.17, p=0.17) or after the Fontan procedure (HR 0.76, 95% CI 0.33–1.74, p=0.52). By 6 years, RVPAS patients had a higher incidence of catheter interventions (0.38 vs. 0.23/patient-year, P<0.001), primarily due to more interventions between the Stage II and Fontan procedures (HR=1.72, 95% CI 1.00–3.03). Complications did not differ by shunt type; by 6 years, one in five patients had had a thrombotic event and one in six, seizures. Conclusions By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS vs. MBTS groups. Children assigned to the RVPAS group had 5% higher transplant-free survival but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications. Clinical Trial Registration Registered with ClinicalTrials.gov number, NCT00115934 URL: http://clinicaltrials.gov/show/NCT00115934

Long‐term results of aortic arch reconstruction with branch pulmonary artery homograft patches

“…aortic arch reconstructions but different diagnostic entities. Our Norwood patients had a 15-year overall survival of 75.7%, similar to the 6-year transplantation-free survival of 59% to 64% demonstrated by the SVR trial investigators 3. Continued multidisciplinary advances in the care in the first year of life in these patients will obviously offer the most substantial improvements in their care.…”

“…We have favored reconstructive techniques using prosthetic material to augment the aortic arch over those employing primary anastomotic techniques, as reported by others. [1][2][3][4][5][6] Various nonbiologic and biologic prosthetic materials have been used in aortic arch reconstruction. 2,[7][8][9][10][11] Due to reports describing more favorable outcomes of Norwood patients using pulmonary homograft as the reconstructive material 2,10 in patients operated on in the 1980s and 1990s, we adopted its use for the vast majority of our pediatric patients requiring aortic arch reconstruction.…”

Toward a solution for cardiac failure in the newborn

“…One of us (REC) (16) reviewed the dramatic progress made over three decades in the staged palliation and reconstruction of the hypoplastic left heart beginning with a modification of the procedure first described in 1980 by Norwood (23) or other procedures and culminating in a third major operation, the Fontan procedure, performed at 18 months to 4 years of age. Reconstructive procedures continue to evolve and newer approaches to reconstruction are debated today (14); however, physiology is never fully restored by these procedures. When staged reconstruction fails, cardiac transplantation may be performed as a “salvage” procedure, but in this setting cardiac transplants pose a significantly greater than average risk (24, 25) because the recipients are less healthy, the anatomy and tissue integrity are compromised by prior surgery and the patients are often allo-sensitized (16).…”

Commentary: The Norwood operation from 1982 to 2019: Continued surgical triumph or stagnation?

“…If we as a profession want to be ready and push the field forward, these detailed analyses will have to be done. Thirty-six years after the first successful Norwood operation was performed, we are still dealing with the same plateaued early survival numbers, a 20% rate of serious neurologic events, a 5-year survival in the range of 65%, 4 and adult survivors with liver fibrosis and requiring high-risk transplants. Dr Norwood showed the way of true innovation back then; it is time for others to pick up that mantle.…”