Outcomes of the Ross procedure in young adults with previous aortic valve interventions.

OBJECTIVES: Contemporarily modified Ross procedure continues to deliver excellent outcomes and remains part of the treatment strategy for aortic valve disease in the young adult population. The aim of this study was to assess whether Ross procedure carried out as a second or subsequent intervention for aortic valve disease carries similar risk and long-term benefit, when compared to Ross procedure for the first-time aortic valve replacement. METHODS: A total of 158 patients aged 16-60 years from a single congenital cardiac centre between 1997 and 2020 were included. The sample was split into 2 subgroups, based on the history of previous aortic valve interventions prior to the Ross procedure. Primary outcomes were defined as survival and pulmonary autograft failure. Coarsened exact matching was used to balance for covariates. RESULTS: A total of 103 patients underwent primary Ross and 55 underwent secondary Ross with a mean follow-up of 7.8 years. Twenty-two patients underwent 28 reoperations during follow-up. Forty-nine well-matched pairs were obtained through matching. Freedom from pulmonary autograft failure at 10 years was 84.5% in the primary group vs 100% in the secondary group (P = 0.021). Five- and ten-year survival were identical, 97.5% in the primary group vs 95.6% in the secondary group (P = 0.53). Male sex and era were found to be predictors of neoaortic root dilatation. CONCLUSIONS: The lower incidence of graft failure seen in secondary Ross could justify a stepwise approach to aortic valve intervention. Ross procedure delivers excellent outcomes in the adult population with no difference in survival for primary or secondary Ross.

Post-infarction ventricular septal defect: percutaneous or surgical management in the UK national registry.

AIMS: Post-infarction ventricular septal defect (PIVSD) is a mechanical complication of acute myocardial infarction (AMI) with a poor prognosis. Surgical repair is the mainstay of treatment, although percutaneous closure is increasingly undertaken. METHODS AND RESUTS: Patients treated with surgical or percutaneous repair of PIVSD (2010-2021) were identified at 16 UK centres. Case note review was undertaken. The primary outcome was long-term mortality. Patient groups were allocated based upon initial management (percutaneous or surgical). Three-hundred sixty-two patients received 416 procedures (131 percutaneous, 231 surgery). 16.1% of percutaneous patients subsequently had surgery. 7.8% of surgical patients subsequently had percutaneous treatment. Times from AMI to treatment were similar [percutaneous 9 (6-14) vs. surgical 9 (4-22) days, P = 0.18]. Surgical patients were more likely to have cardiogenic shock (62.8% vs. 51.9%, P = 0.044). Percutaneous patients were substantially older [72 (64-77) vs. 67 (61-73) years, P < 0.001] and more likely to be discussed in a heart team setting. There was no difference in long-term mortality between patients (61.1% vs. 53.7%, P = 0.17). In-hospital mortality was lower in the surgical group (55.0% vs. 44.2%, P = 0.048) with no difference in mortality after hospital discharge (P = 0.65). Cardiogenic shock [adjusted hazard ratio (aHR) 1.97 (95% confidence interval 1.37-2.84), P < 0.001), percutaneous approach [aHR 1.44 (1.01-2.05), P = 0.042], and number of vessels with coronary artery disease [aHR 1.22 (1.01-1.47), P = 0.043] were independently associated with long-term mortality. CONCLUSION: Surgical and percutaneous repair are viable options for management of PIVSD. There was no difference in post-discharge long-term mortality between patients, although in-hospital mortality was lower for surgery.

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

Background: Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results. Methods: We retrospectively identified neonates with HLHS IS/HRIC requiring LAD between 2005 and 2019. All babies had prenatal/postnatal echocardiography. Our LAD strategy evolved over time from attempt at transcatheter balloon atrial septostomy (BAS) to surgical septectomy with inflow occlusion, to hybrid trans-atrial stent implantation. Results: Twelve neonates required LAD at a median time of 14 (0.5-31) hours after birth. Five patients underwent BAS that proved successful in 2 cases. Of the 3 unsuccessful cases, 2 required extra-corporeal membrane oxygenation (ECMO) support and died subsequently; one underwent hybrid trans-atrial stent implantation. Of the remaining 7 patients, 3 underwent surgical septectomy with inflow-occlusion and 4 underwent hybrid trans-atrial stent implantations. Overall, 8 patients survived LAD and reached Norwood palliation. Three of the 8 required ECMO postoperatively. There was no hospital mortality after Norwood stage 1 palliation and interstage survival was 100%. Six patients successfully underwent Glenn shunt (superior cavopulmonary anastomosis) and 5 have completed total cavopulmonary connection. Conclusions: Our experience suggests that prompt postnatal LAD can be safely achieved with careful multidisciplinary planning and accurate antenatal diagnosis. In our hands, hybrid trans-atrial septal stent insertion appears to be a safe approach which combines the versatility of transcatheter techniques together with the effectiveness of surgical control.

Mechanical Circulatory Support for the Failing Sub-Aortic Right Ventricle in Adults.

Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.

IVC Stent to Improve Ventricular Assist Device Filling Following Pediatric Cardiac Transplant.

Poor ventricular assist device filling is often seen in patients supported with the Berlin Heart Excor. Caval stenosis is an uncommon complication following the bicaval approach to orthotopic heart transplantation. We report the case of a five-year-old female patient post heart transplantation whose poor right ventricular assist device filling immediately resolved following management of caval stenosis.

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care.

There is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review. The authors are from 3 different centres; each centre has an established subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant, and mechanical support for patients with ACHD. Appropriate care of this complex population requires multidisciplinary ACHD-HF teams evaluate all possible treatment options. The risks and benefits of nontransplant ACHD surgery, percutaneous structural and electrophysiological intervention, and ongoing conservative management must be considered alongside those of transplant strategies. In our approach, advanced care planning and palliative care coexist with the consideration of advanced therapies. An ethos of shared decision making, guided by the patient's values and preferences, strengthens clinical care, but requires investment of time as well as skilled communication. In this review, we aim to offer practical real-world advice for managing these patients, supported by scientific data where it exists.

Impact of mode of failure and end-organ dysfunction on the survival of adult Fontan patients undergoing cardiac transplantation.

OBJECTIVES: Adults with failing Fontan circulation (FFC) represent a heterogeneous, high-risk group for cardiac transplantation with poor reported outcomes. We studied the impact of mode of Fontan failure (preserved versus impaired systolic ventricular function) and end-organ dysfunction on early survival in adults undergoing cardiac transplantation for FFC. METHODS: A single-centre, retrospective study of 26 adults (≥16 years) with FFC undergoing cardiac transplantation between 1990 and 2015. Patients were classified by the presence or absence of preserved systolic ventricular function (PVF). End-organ dysfunction was assessed by serum markers, including albumin, liver ultrasound and the presence of varices, ascites, splenomegaly and thrombocytopaenia (VAST score for portal hypertension). RESULTS: Thirty-day survival rate for the entire cohort was 69.2%, with 76.2% survival for the recent era. One-year Kaplan-Meier survival rate was 65.4%. Actuarial survival was poorer in those with PVF or heterotaxy (P = 0.01; log-rank test). Cox multivariable regression analysis confirmed PVF as an independent predictor for death (odds ratio, OR 5.38; confidence interval, CI 1.08-26.96; P = 0.04). In examining the PVF subset further, these patients had significantly higher VAST and liver ultrasound scores and lower serum albumin, compared with patients with impaired function. Patients with PVF and ≥moderate liver fibrosis on ultrasound or VAST score ≥2 accounted for two-thirds of the total mortality. CONCLUSIONS: Favourable cardiac transplantation outcomes can be achieved in adults with failing Fontan circulation. Patients with PVF may represent a distinct subset with more perturbed failing Fontan physiology and higher cardiac transplant mortality. We continue, however, to evolve and refine our strategies and are optimistic concerning future improvement in outcomes even in those with PVF.

Fontan-associated liver disease: Implications for heart transplantation.

Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury. Pathology studies have generally shown that FALD worsens as time post-Fontan increases, but the prevalence of FALD is not well defined because the majority of Fontan patients, even those with significant hepatic fibrosis, appear to be asymptomatic and biochemical or functional hepatic abnormalities are usually subtle or absent. Alternate non-invasive investigations, derived from the study of other chronic liver diseases, have been tested in small series of pediatric and adult Fontan patients, but they have been confounded by congestion and do not correlate well with liver biopsy findings. Liver disease can complicate Fontan circulatory failure and may even be significant enough to be considered a contraindication to heart transplantation or require combined heart-liver transplantation. The search for the optimal management strategy continues in the setting of increasing numbers of Fontan patients surviving to adulthood and being referred for heart transplantation. Thus, in this review we attempt to define the scope and significance of FALD and address transplant-related assessment and management of this challenging disorder.

Single-centre experience of 101 paediatric and adult Ross procedures: mid-term results.

We aimed to determine outcomes for the Ross procedure in paediatric and adult patients, with particular emphasis on survival, complication and reintervention rates. A retrospective review of 101 patients who had the Ross procedure in a congenital cardiac surgical centre serving a population of approximately 2.5 million was performed. There were 69 adults and 32 children with a mean age of 24.8 ± 13.9 years. Indications for surgery were aortic stenosis (48), regurgitation (10), mixed disease (35) and complex left outflow tract obstruction (8). The mean follow-up duration was 4.7 ± 3.7 years. The mini-inclusion technique was used to incorporate the autograft, and in all cases, pulmonary homografts were placed in the right ventricular outflow tract. Sub-aortic resection was also performed in six and Ross-Konno operations in eight patients. There were no early deaths and there was one late death secondary to endocarditis. Freedom from reintervention was 92% at 5 years and 77% at 10 years. Children were significantly more likely to require reintervention (16%, 5 of 32 versus 4%, 3 of 69, P = 0.05). The Ross procedure carries low early and mid-term mortality, and reintervention rates appear acceptable. The Ross procedure should be considered a feasible alternative to prosthetic valves in patients who require aortic valve replacement.