Major elective non-cardiac operations in adults with congenital heart disease.

OBJECTIVE: To assess the impact of congenital heart disease (CHD) on resource utilisation and clinical outcomes in patients undergoing major elective non-cardiac operations. BACKGROUND: Due to advances in congenital cardiac management in recent years, more patients with CHD are living into adulthood and are requiring non-cardiac operations. METHODS: The 2010-2018 Nationwide Readmissions Database was used to identify all adults undergoing major elective operations (pneumonectomy, hepatectomy, hip replacement, pancreatectomy, abdominal aortic aneurysm repair, colectomy, gastrectomy and oesophagectomy). Multivariable regression models were used to categorise key clinical outcomes. RESULTS: Of an estimated 4 941 203 adults meeting inclusion criteria, 5234 (0.11%) had a previous diagnosis of CHD. Over the study period, the incidence of CHD increased from 0.06% to 0.17%, p<0.001. CHD patients were on average younger (63.3±14.8 vs 64.4±12.5 years, p=0.004), had a higher Elixhauser Comorbidity Index (3.3±2.2 vs 2.3±1.8, p<0.001) and received operations at high volume centres more frequently (66.6% vs 62.0%, p=0.003). Following risk adjustment, these patients had increased risk of in-hospital mortality (adjusted risk ratio (ARR): 1.76, 95% CI 1.25 to 2.47), experienced longer hospitalisation durations (+1.6 days, 95% CI 1.3 to 2.0) and cost more (+$8370, 95% CI $6686 to $10 055). Furthermore, they were more at risk for in-hospital complications (ARR: 1.24 95% CI 1.17 to 1.31) and endured higher adjusted risk of readmission at 30 days (ARR: 1.32 95% CI 1.13 to 1.54). CONCLUSIONS: Adults with CHD are more frequently comprising the major elective operative cohort for non-cardiac cases. Due to the inferior clinical and financial outcomes suffered by this population, perioperative risk stratification may benefit from the inclusion of CHD as a factor that portends unfavourable outcomes.

Race-based disparities in access to surgical palliation for hypoplastic left heart syndrome.

BACKGROUND: Racial disparities in outcomes have been shown to persist in many operative specialties, including the management of congenital heart disease. Using a demographic-adjusted methodology, we examined whether patient race influenced access to high-performing centers for the operative management of hypoplastic left heart syndrome. METHODS: The 2005-2017 National Inpatient Sample was queried to identify all pediatric (≤5 years) hospitalizations with an operation for hypoplastic left heart syndrome. A racial disparity index was generated for each hospital and defined as the proportion of White patients receiving operative management for hypoplastic left heart syndrome divided by the proportion of White patients admitted for respiratory failure. This methodology quantified hospital-level racial variation while adjusting for the local racial makeup of each center. RESULTS: Of the 17,275 patients who met inclusion criteria, 64.1% were managed at high-volume centers. Patients at high-volume centers had a similar distribution of operative type, age, and burden of comorbidities. The mean racial disparity index steadily grew from 1.06 at the lowest volume decile of operative volume to 1.51 at the highest, indicating an increasing proportion of White patients as volume increased. Using risk-adjusted analysis, each decile increase in hospital volume was associated with a 14% relative reduction in odds of mortality and a 0.06 increase in predicted racial disparity index. Increasing volume was further associated with reduced odds of non-home discharge but did not alter resource utilization. CONCLUSION: We demonstrate that high-volume centers disproportionally serve White patients and have superior clinical outcomes compared to low-volume centers. This study highlights the critical importance of equitable access to expert care for high-risk conditions such as hypoplastic left heart syndrome.

Heart and heart-liver transplantation in adults with failing Fontan physiology.

BACKGROUND: As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart-liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients has improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology. METHODS AND RESULTS: Retrospective analysis of patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center who underwent OHT or CHLT for failing Fontan physiology from January 1, 2002 to May 31, 2017. We identified 20 patients with single-ventricle physiology and Fontan palliation who underwent OHT or CHLT. The median age was 29.5 years (range 19-44). Five patients underwent CHLT because of biopsy proven hepatic cirrhosis. The median length of hospital stay was 23 days (range 8-76) post-OHT and 51 days (range 26-77) post-CHLT. During a median follow-up of 56 months (range 2-178), there was one mortality occurring at 34 months post-OHT due to coronary vasculopathy. Most frequent early postoperative complications included bleeding and infection (55% and 20%, respectively) and surgical reintervention for bleeding complications (n = 8, 40%). One CHLT patient experienced clinically significant hepatic rejection requiring admission and steroid treatment. CONCLUSIONS: Despite inherent risks and complexities of OHT or CHLT in patients with a failed Fontan, transplant is a reasonable therapy. Peri- and postoperative complications are common and may require surgical reintervention. Continued observation of practices and unifying themes may help improve patient selection, pre- and postoperative treatment and ultimately outcomes.

Ventricular arrhythmia burden after transcatheter versus surgical pulmonary valve replacement.

OBJECTIVE: Comparative ventricular arrhythmia (VA) outcomes following transcatheter (TC-PVR) or surgical pulmonary valve replacement (S-PVR) have not been evaluated. We sought to compare differences in VAs among patients with congenital heart disease (CHD) following TC-PVR or S-PVR. METHODS: Patients with repaired CHD who underwent TC-PVR or S-PVR at the UCLA Medical Center from 2010 to 2016 were analysed retrospectively. Patients who underwent hybrid TC-PVR or had a diagnosis of congenitally corrected transposition of the great arteries were excluded. Patients were screened for a composite of non-intraoperative VA (the primary outcome variable), defined as symptomatic/recurrent non-sustained ventricular tachycardia (VT) requiring therapy, sustained VT or ventricular fibrillation. VA epochs were classified as 0-1 month (short-term), 1-12 months (mid-term) and ≥1 year (late-term). RESULTS: Three hundred and two patients (TC-PVR, n=172 and S-PVR, n=130) were included. TC-PVR relative to S-PVR was associated with fewer clinically significant VAs in the first 30 days after valve implant (adjusted HR 0.20, p=0.002), but similar mid-term and late-term risks (adjusted HR 0.72, p=0.62 and adjusted HR 0.47, p=0.26, respectively). In propensity-adjusted models, S-PVR, patient age at PVR and native right ventricular outflow tract (RVOT) (vs bioprosthetic/conduit outflow tract) were independent predictors of early VA after pulmonary valve implantation (p<0.05 for all). CONCLUSION: Compared with S-PVR, TC-PVR was associated with reduced short-term but comparable mid-term and late-term VA burdens. Risk factors for VA after PVR included a surgical approach, valve implantation into a native RVOT and older age at PVR.

Atrial septal hematoma: after minimally invasive aortic valve replacement.

Interatrial septal hematoma is a very rare complication after mitral valve surgery. Unusually, it is the result of aortic valve disease, including aortic dissection. We report a case wherein interatrial septal hematoma followed minimally invasive aortic valve replacement in a 68-year-old woman. The hematoma was recognized upon intraoperative transesophageal echocardiography, but there was no evidence of accompanying aortic dissection. The interatrial septal hematoma was at first drained by needle, but recurrence prompted reoperation and plication of the interatrial septum. Finally, the hematoma resolved after correction of the coagulopathy. Catheter injury to the coronary sinus exacerbated by the retrograde administration of cardioplegic solution is thought to have caused the origin of the interatrial septal dissection.

Obstruction of lateral tunnel fontan by an adjustable occluder device.

Innovative ways of adjusting Fontan fenestrations have been developed over the last two decades. Long-term complications have not been described. We successfully treated one such complication of Fontan conduit obstruction due to an adjustable occluder device.