Surgical Experience With Nine Cases of Obstructed Right Aortic Arch.

BACKGROUND: Right-sided aortic arch obstruction is an extremely rare congenital anomaly. A variety of surgical approaches have been described. This study reviews our institutional experience over the last 30 years. METHODS: Our surgical database at the University of Alabama at Birmingham and Children's Hospital of Alabama from 1992 to 2022 was reviewed to include all patients who underwent surgical repair for right-sided aortic arch obstruction. RESULTS: A total of nine patients underwent surgical repair for right-sided aortic arch obstruction. Surgical approach was via thoracotomy (n = 2, 22%), sternotomy (n = 5, 56%), or combined (n = 2, 22%). Primary extended end-to-end anastomosis was utilized for patients with discrete coarctation (n = 1, 11%), reverse subclavian flap for coarctation with associated distal arch hypoplasia (n = 2, 22%), GORE-TEX® tube graft for circumflex aorta (n = 1, 11%), and aortic arch advancement (n = 5, 56%) with or without patch augmentation for those with an interrupted or severely hypoplastic aortic arch. Reintervention was required in one patient (11%) for recoarctation. All patients were discharged in good condition. There was no hospital mortality and at 10.5 years (mean) follow-up there was one late death. CONCLUSION: Right aortic arch obstruction is a rare entity. Surgical approach should be tailored to the anatomy and associated intracardiac defects. Preoperative imaging with a CT angiogram is useful for operative planning. Sternotomy with single-stage primary repair is safe, effective, and our preferred surgical approach for patients with right aortic arch obstruction and associated intracardiac pathology.

Association Between Venous Homografts and Allosensitization After Norwood Procedure.

BACKGROUND: Right ventricle (RV) to pulmonary artery (PA) shunts have become the shunt of choice at many centers for use during the Norwood procedure for single ventricle palliation. Some centers have begun to use cryopreserved femoral or saphenous venous homografts as an alternative to polytetrafluoroethylene (PTFE) for shunt construction. The immunogenicity of these homografts is unknown, and potential allosensitization could have significant implications on transplant candidacy. METHODS: All patients undergoing Glenn procedure at our center between 2013 and 2020 were screened. Patients who initially underwent Norwood procedure with either PTFE or venous homograft RV-PA shunt and had available pre-Glenn serum were included in the study. The primary outcome of interest was panel reactive antibody (PRA) level at the time of Glenn surgery. RESULTS: Thirty-six patients met inclusion criteria (N = 28 PTFE, N = 8 homograft). Patients in the homograft group had significantly higher median PRA levels at the time of Glenn surgery (0% [IQR 0-18] PTFE vs 94% [IQR 74-100] homograft, P = .003). There were no other differences between the two groups. CONCLUSIONS: Despite potential improvements in PA architecture, the use of venous homografts for RV-PA shunt construction at the time of Norwood procedure is associated with significantly elevated PRA level at the time of Glenn surgery. Centers should carefully consider the use of currently available venous homografts given the high percentage of these patients who may require future transplantation.

Health-Related Quality of Life After Single Ventricle Palliation or Tetralogy of Fallot Repair.

Single ventricle (SV) cardiac lesions and tetralogy of Fallot (TOF) are both common forms of cyanotic congenital heart disease. With advances in perioperative care and longitudinal follow-up, survival of these patients has dramatically improved and the majority survive to adulthood. This study compares health-related quality of life (HRQoL) of adult SV and TOF patients to each other and the general population. HRQoL of all surviving, non-transplanted SV and TOF patients 21 years of age and older at our institution was assessed with the SF-36 questionnaire via phone. Additional data including demographic parameters and information related to comorbidities and healthcare utilization were also analyzed. Among 81 eligible SV patients and 207 TOF patients, 33 (41%) and 75 (36%) completed the SF-36 phone survey, respectively. The mean age of SV patients was 32 vs. 38 years in the TOF group (p=0.01). SV patients reported more hepatic, pulmonary, and renal comorbidities. TOF patients were more likely to complete advanced degrees and more likely to have children (p=0.03). SV physical functioning scores were worse compared to TOF. In other domains of the SF-36 questionnaire, SV and TOF scores were similar. Compared to the general population, both groups reported worse bodily pain and mental health, but other aspects of psychosocial and general health were comparable. Overall HRQoL is good for both SV and TOF patients through early and mid-adulthood. Some QoL metrics were modestly worse in the SV patients. While these patients may have some physical limitations, psychosocial wellbeing appears preserved.