Performance and failure of right ventricle to pulmonary artery conduit in congenital heart disease.

INTRODUCTION: Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery but with limited durability leading to re-intervention. Current single-center, retrospective, cohort study is reporting results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. METHODS: Patients with CHD referred for RV-PA conduits surgical implantation (October 1997 and January 2022) have been included. Primary outcome was conduit failure defined as peak gradient above 64mmHg/severe regurgitation/need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effect linear regression analysis. RESULTS: Two-hundred and fifty-two patients were initially included. One hundred and forty-nine patients were elegible for follow-up data collection. After a median follow-up time of 49 months the primary study endpoint occurred in 44 (29%) patients. Multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft as protective factors (HR 0.11, 95% CI 0.02-0.47 and HR 0.34, 95% CI 0.16-0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (OR 4.29, 95% CI 1.41-13.01). Longterm use of oral anticoagulant was independently associated with slower progression of peak echocardiographic gradient across conduits (mixed effect linear regression p-value 0.027). CONCLUSION: In patients with CHD, surgically implanted RV-PA conduit failure is faster in children and after non-homograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Longterm anticoagulation seems to exert a protective effect.

Utilization and outcomes of expanded criteria donors in adults with congenital heart disease.

BACKGROUND: Use of donation after circulatory death (DCD) and hepatitis C virus (HCV) positive donors in heart transplantation have increased the donor pool. Given poor waitlist outcomes in the adult congenital heart disease (ACHD) population, we investigated waitlist outcomes associated with willingness to consider DCD and HCV+ offers and post-transplant outcomes following HCV+ and DCD transplantation for these candidates. METHODS: Using the United Network for Organ Sharing database, we identified adult ACHD candidates and recipients listed or transplanted, respectively, between 01/01/2016 and 09/30/2023 for the HCV analysis and between 12/01/2019 and 09/30/2023 for the DCD analysis. Among candidates, we compared the cumulative incidence of transplant, with waitlist death/deterioration as a competing risk, by willingness to consider HCV+ and DCD offers. Among recipients of HCV+ (vs HCV-) and DCD (vs brain death [DBD]) transplants, we compared perioperative outcomes and post-transplant survival. RESULTS: Of 1,436 ACHD candidates from 01/01/2016 to 09/30/2023, 37.0% were willing to consider HCV+ heart offers. Of 886 ACHD candidates from 12/01/2019 to 09/30/2023, 15.5% were willing to consider DCD offers. On adjusted analysis, willingness to consider HCV+ offers was associated with 84% increased likelihood of transplant, and willingness to consider DCD offers was associated with 56% increased likelihood of transplant. Of 904 transplants between 01/01/2016 and 09/30/2023, 6.4% utilized HCV+ donors, and of 540 transplants between 12/01/2019 and 09/30/2023, 6.9% utilized DCD donors. Recipients of HCV+ (vs HCV-) and DCD (vs DBD) heart transplants had similar likelihood of perioperative outcomes and 1-year survival. CONCLUSIONS: ACHD candidates who were willing to consider HCV+ and DCD offers were more likely to be transplanted and had similar post-transplant outcomes compared to recipients of HCV- and DBD organs.

Feasibility and Findings of Including Self-Identified Adult Congenital Heart Disease Patients in the INVESTED Trial.

Background: Adult congenital heart disease (ACHD) patients have significant morbidity and rise in cardiac admissions. Their outcome with high-dose influenza vaccination is unknown in comparison to those without ACHD. Objectives: The purpose of this study was to compare all-cause mortality or cardiopulmonary hospitalizations in self-identified ACHD versus non-ACHD patients receiving high- or low-dose influenza vaccination within the INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure trial. Methods: We prospectively included ACHD patients in the INVESTED (INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure) trial. The primary endpoint was all-cause death or hospitalization for cardiovascular or pulmonary causes. Results: Of the 272 ACHD patients, 132 were randomly assigned to receive high-dose trivalent and 140 to standard-dose quadrivalent influenza vaccine. Compared to the non-ACHD cohort (n = 4,988), ACHD patients were more likely to be younger, women, smokers, have atrial fibrillation, and have a qualifying event of heart failure. The primary outcome was 49.8 events versus 42.8 events per 100 person-years (adjusted HR: 1.17; 95% CI: 0.95-1.45; P = 0.144) in the ACHD group and non-ACHD group, respectively. The interaction between ACHD status and randomized treatment effect was not significant for the primary outcome (P = 0.858). Vaccine-related adverse events were similar in both groups. Conclusions: Patients who self-identify as being ACHD had similar primary outcome of all-cause death or hospitalization for cardiovascular or pulmonary causes compared to non-ACHD cohort. High-dose influenza vaccination was similar to standard-dose influenza vaccination on the primary outcome in patients who self-identify as ACHD.

Cor Triatriatum Sinister in a Young Adult: An Unusual Cause of Syncope.

A 25-year-old male with no prior medical history presented with a one-month history of nausea, weight loss, and dyspnea that progressed to syncope. The initial echocardiogram showed a dilated right ventricle with signs of systolic failure. The patient was admitted for suspected pulmonary embolism, but chest computed tomography (CT) revealed interstitial pneumonia. A transthoracic echocardiogram on day 6 of admission diagnosed cor triatriatum sinister (CTS), severe pulmonary hypertension, chronic cor pulmonale, and reduced right ventricular function. The patient was managed conservatively in the intensive care unit (ICU) without the need for mechanical ventilation and discharged after clinical improvement. This case highlights the importance of the early diagnosis of rare congenital heart defects such as cor triatriatum sinister, which can present with nonspecific symptoms and rapidly progress to right heart failure.

Anesthesia for Combined Heart-Liver Transplantation: A Narrative Review.

In 1984, 21 years after the first liver transplantation, Thomas Starzl achieved a milestone by performing the world's first combined heart-liver transplantation. While still uncommon, the practice of combined heart-liver transplants is on the rise globally. In this review, the authors delve into the current literature on this procedure, highlighting the evolving landscape and key considerations for anesthesiologists. Over the years, there has been a remarkable increase in the number of combined heart-liver transplantations conducted worldwide. This surge is largely attributed to the growing population of adult survivors with single-ventricle physiology, palliated with a Fontan procedure, who later present with late Fontan failure and Fontan-associated liver disease. Research indicates that combined heart-liver transplantation is an effective treatment option, with reported outcomes comparable with isolated heart or liver transplants. Managing anesthesia during a combined heart-liver transplant procedure is challenging, especially in the context of underlying Fontan physiology. International experience in this field remains somewhat limited, with most techniques derived from expert opinions or experiences with single-organ heart and liver transplants. These procedures are highly complex and performed infrequently. As the number of combined heart-liver transplants continues to rise globally, there is a growing need for clear guidance on periprocedural surgical and anesthetic management. Anesthesiologists overseeing these patients must consider multiple factors, balancing various comorbidities with significant hemodynamic and metabolic shifts. An increase in (multicenter) studies focusing on specific interventions to enhance patient and organ outcomes is anticipated in the coming years.

Anesthetic Management of an Adult Patient Post a Fontan Procedure in Laparoscopic Surgery: A Case Report.

We report the successful anesthetic management of laparoscopic surgery in a 21-year-old female patient with Fontan circulation. A preoperative careful review of cardiac catheterization results helped assess the risk of the surgery and implement anesthetic management. Intraoperative management focused on minimizing the impact on pulmonary vascular resistance and venous return by optimizing ventilation and applying lower pneumoperitoneum pressure without tilting the position. Milrinone was administered to reduce pulmonary vascular resistance and provide inotropic support with minimally invasive monitoring. The patient remained stable throughout the procedure without complications. This case highlights the importance of thorough preoperative assessment, individualized intraoperative management, and collaboration with the surgical team when caring for adult Fontan patients undergoing laparoscopic surgery.

Experiences and Attitudes Toward Telemedicine in an Adult Congenital Heart Disease Clinic: Lessons Learned from the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has driven a broader adoption of telemedicine (TM). We aim to describe adult congenital heart disease (ACHD) patient experiences with TM and explore factors associated with positive attitude toward future TM visits. This is a cross-sectional, single-center study in an outpatient ACHD clinic from February to June, 2022. Between-group comparisons were made using Wilcoxon-Rank Sum, Chi-Square, or Fisher-Exact tests. Univariate logistic regression was performed for variables that could correlate with a "positive" attitude toward future TM visits. Significance was determined using an alpha level of 0.05. Of 262 patients (median age 33 years, 55% female, 81% White), 115 (44%) had a prior TM visit and 110 (96%) reported a positive experience. There were 64 (24%) with a positive attitude toward future TM visits. Concerns include lack of cardiac testing and limited quality of visit. Patients with visits every 3-6 months (Odds Ratio [OR] 2.44; p < 0.01) and prior TM visit (OR 1.89; p = 0.03) had higher odds of a positive attitude toward future TM, whereas males had lower odds (OR 0.53; p = 0.04). Age, annual income, disease complexity, distance from clinic, and employment status were not associated. There is high rate of satisfaction with TM among ACHD patients but only one-quarter indicated interest in using TM in the future. Factors associated with interest in TM visits are identified, and together with patient feedback, can be used to understand potential role of TM for the ACHD population in the post-pandemic era.

Challenges and opportunities in patients with adult congenital heart disease, a narrative review.

Adult congenital heart disease Pregnancy Transition of care Challenges heart failure.

Intra-aortic balloon pump treatment in an adult patient with a Fontan circulation and acute heart failure: a case report.

Background: There is limited evidence for the use of an intra-aortic balloon pump (IABP) in adult patients with a total cavopulmonary, or Fontan circulation. Case summary: A patient in his twenties with a Fontan circulation presented with sepsis, pneumonia, and pulmonary oedema. He was born with a hypoplastic left ventricle, atrioventricular septal defect, and hypoplastic aortic arch, and a total cavopulmonary circulation had been established within his first years of life. Standard of care treatment with antibiotics, non-invasive ventilatory support, loop diuretics, and vasopressors was initiated. Due to persistent pulmonary congestion and increasing general fatigue, an IABP was placed via a femoral artery to offload the failing systemic ventricle. Secondary to IABP treatment, mean arterial pressure rose, and vasodilatory nitroprusside could be introduced. Over 4 days of IABP treatment, the patient's general condition and ventricular systolic function improved significantly. Discussion: This case suggests that IABP treatment was important in the recovery of our patient with a Fontan circulation, pneumonia, and heart failure. We propose that during IABP treatment, an increase in stroke volume and a reduction in ventricular filling pressure is achieved, thereby increasing the transpulmonary pressure gradient that is central to pulmonary blood flow in Fontan patients. More definitive evidence is necessary to confirm our hypotheses.

Transcatheter Interventions in Adult Congenital Heart Disease.

Congenital heart disease (CHD) is the most common congenital birth defect with an incidence of 1 in 100. Current survival to adulthood is expected in 9 out of 10 children with severe CHD as the diagnostic, interventional, and surgical success improves. The adult CHD (ACHD) population is increasingly diverse, reflecting the broad spectrum of CHD and evolution of surgical techniques to improve survival. Similarly, transcatheter interventions have seen exponential growth and creativity to reduce the need for repeat sternotomies. This article focuses on newer data and evolving techniques for transcatheter interventions specific to certain ACHD populations.

Using artificial intelligence and deep learning to optimise the selection of adult congenital heart disease patients in S-ICD screening.

INTRODUCTION: The risk of complications associated with transvenous ICDs make the subcutaneous implantable cardiac defibrillator (S-ICD) a valuable alternative in patients with adult congenital heart disease (ACHD). However, higher S-ICD ineligibility and higher inappropriate shock rates-mostly caused by T wave oversensing (TWO)- are observed in this population. We report a novel application of deep learning methods to screen patients for S-ICD eligibility over a longer period than conventional screening. METHODS: Adult patients with ACHD and a control group of normal subjects were fitted with a 24-h Holters to record their S-ICD vectors. Their T:R ratio was analysed utilising phase space reconstruction matrices and a deep learning-based model to provide an in-depth description of the T: R variation plot for each vector. T: R variation was compared statistically using t-test. RESULTS: 13 patients (age 37.4 ± 7.89 years, 61.5 % male, 6 ACHD and 7 control subjects) were enrolled. A significant difference was observed in the mean and median T: R values between the two groups (p < 0.001). There was also a significant difference in the standard deviation of T: R between both groups (p = 0.04). CONCLUSIONS: T:R ratio, a main determinant for S-ICD eligibility, is significantly higher with more tendency to fluctuate in ACHD patients when compared to a population with normal hearts. We hypothesise that our novel model could be used to select S-ICD eligible patients by better characterisation of T:R ratio, reducing the risk of TWO and inappropriate shocks in the ACHD patient cohort.

Transcatheter management of obstructed baffle repairs of partial anomalous pulmonary veins: a case series.

Background: Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to pulmonary hypertension and right heart dysfunction if not detected and corrected. Case summary: We describe three patients with occluded PAPVR baffles who underwent drug-coated balloon angioplasty and stenting of the obstructed baffle. In each case, baffle obstruction was detected post-operatively on surveillance cross-sectional imaging, and an invasively measured pulmonary capillary wedge-to-left atrium gradient was noted to be elevated. Post-intervention, each patient had an improvement in baffle flow by angiography as well as lung perfusion as assessed by nuclear medicine scintigraphy. Discussion: Given the subtle symptomatology of obstructed PAPVR pericardial baffle repairs, surveillance imaging is necessary to detect occluded baffles and intervene before downstream right heart disease and pulmonary hypertension develops. Given the high rates of re-stenosis in pulmonary vein stenting, pre-treatment of occluded PAPVR baffles with drug-coated balloons may help reduce re-intervention rates.

A Case of Double Inlet Left Ventricle in a 63-Year-Old Female Patient.

This case report describes the medical history and presentation of an elderly patient who was born with single ventricle physiology, an anomaly that is both unique and complex. Patients with single ventricle cardiac anomalies may be susceptible to life-threatening complications. However, advances in medical treatment and understanding have allowed for clinicians to develop surgical and medical interventions to treat patients with univentricular cardiac defects. This case is unique in the sense that the patient has been able to demonstrate remarkable adaptability to this condition and have a sustained life with little intervention. This report serves to explore the pathophysiology of this condition as well as highlight the human body's astounding resilience to configure itself to abnormal conditions. The patient's presentation will be discussed as well as diagnostics and management utilized by the care providers. Despite its rare occurrence, understanding the manifestations of this complex cardiac abnormality can allow future providers to improve the prognosis and outcomes of patients born with a single ventricle.

B-type natriuretic peptide levels predict long-term mortality in a large cohort of adults with congenital heart disease.

BACKGROUND AND AIMS: Many adult patients with congenital heart disease (ACHD) are still afflicted by premature death. Previous reports suggested natriuretic peptides may identify ACHD patients with adverse outcome. The study investigated prognostic power of B-type natriuretic peptide (BNP) across the spectrum of ACHD in a large contemporary cohort. METHODS: The cohort included 3392 consecutive ACHD patients under long-term follow-up at a tertiary ACHD centre between 2006 and 2019. The primary study endpoint was all-cause mortality. RESULTS: A total of 11 974 BNP measurements were analysed. The median BNP at baseline was 47 (24-107) ng/L. During a median follow-up of 8.6 years (29 115 patient-years), 615 (18.1%) patients died. On univariable and multivariable analysis, baseline BNP [hazard ratio (HR) 1.16, 95% confidence interval (CI) 1.15-1.18 and HR 1.13, 95% CI 1.08-1.18, respectively] and temporal changes in BNP levels (HR 1.22, 95% CI 1.19-1.26 and HR 1.19, 95% CI 1.12-1.26, respectively) were predictive of mortality (P < .001 for both) independently of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. Patients within the highest quartile of baseline BNP (>107 ng/L) and those within the highest quartile of temporal BNP change (>35 ng/L) had significantly increased risk of death (HR 5.8, 95% CI 4.91-6.79, P < .001, and HR 3.6, 95% CI 2.93-4.40, P < .001, respectively). CONCLUSIONS: Baseline BNP and temporal BNP changes are both significantly associated with all-cause mortality in ACHD independent of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. B-type natriuretic peptide levels represent an easy to obtain and inexpensive marker conveying prognostic information and should be used for the routine surveillance of patients with ACHD.

Prognostic Value and Determinants of High-Sensitivity Cardiac Troponin T in Patients With a Systemic Right Ventricle: Insights From the SERVE Trial.

BACKGROUND: The determinants and prognostic value of high-sensitivity cardiac troponin T (hs-cTnT) among patients with a systemic right ventricle are largely unknown. METHODS AND RESULTS: Ninety-eight patients from the randomized controlled SERVE (Effect of Phosphodiesterase-5 Inhibition With Tadalafil on Systemic Right Ventricular Size and Function) trial were included. The correlation between baseline hs-cTnT concentrations and biventricular volumes and function quantified by cardiac magnetic resonance or cardiac multirow detector computed tomography was assessed by adjusted linear regression models. The prognostic value of hs-cTnT was assessed by adjusted Cox proportional hazards models, survival analysis, and concordance statistics. The primary outcome was time to the composite of clinically relevant arrhythmia, hospitalization for heart failure, or all-cause death. Median age was 39 (interquartile range, 32-48) years, and 32% were women. Median hs-cTnT concentration was 7 (interquartile range, 4-11) ng/L. Coefficients of determination for the relationship between hs-cTnT concentrations and right ventricular end-systolic volume index and right ventricular ejection fraction (RVEF) were +0.368 (P=0.046) and -0.381 (P=0.018), respectively. The sex- and age-adjusted hazard ratio for the primary outcome of hs-cTnT at 2 and 4 times the reference level (5 ng/L) were 2.89 (95% CI, 1.14-7.29) and 4.42 (95% CI, 1.21-16.15), respectively. The prognostic performance quantified by the concordance statistics for age- and sex-adjusted models based on hs-cTnT, right ventricular ejection fraction, and peak oxygen uptake predicted were comparable: 0.71% (95% CI, 0.61-0.82), 0.72% (95% CI, 0.59-0.84), and 0.71% (95% CI, 0.59-0.83), respectively. CONCLUSIONS: Hs-cTnT concentration was significantly correlated with right ventricular ejection fraction and right ventricular end-systolic volume index in patients with a systemic right ventricle. The prognostic accuracy of hs-cTnT was comparable to that of right ventricular ejection fraction and peak oxygen uptake predicted. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03049540.

Exercise-induced hypertension after aortic coarctation repair: Our experience and a systematic review.

BACKGROUND: The exact prevalence and clinical significance of excessive increase in blood pressure in response to exercise in patients with repaired coarctation of aorta (CoA) remains unknown. AIM: This study aimed to investigate the impact of different definitions of exercise-induced hypertension (EIH) on the prevalence rates in our adult patients with repaired CoA. A systematic review of the available literature was also performed. METHODS: We retrospectively analyzed exercise test data from adult patients with repaired CoA followed at the national referral center for adult congenital heart disease between 1998 and 2021. The three most reported definitions of EIH in patients with repaired CoA were used for the analysis of EIH prevalence. We also performed a systematic search of the PubMed electronic bibliographic database. Full-text versions of all potentially relevant articles on EIH in CoA were reviewed for relevance. RESULTS: Our registry included 161 adult CoA patients. Complete exercise test results were available in 74 patients (59% male, median age 39 years [range 20-68 years]). The prevalence of EIH in our cohort varied from 24 to 41%, depending on the definition used. We identified eleven eligible articles from 184 publications. The reported prevalence rate of EIH in the studies ranged from 13% to 82%. CONCLUSION: EIH is common in patients with repaired CoA; however, the rates of EIH vary greatly depending on the definition used. A standardized and uniform EIH definition is needed to accurately assess the prevalence and clinical relevance of EIH in this population.

Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation.

BACKGROUND: Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs. CASE PRESENTATION: A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock-Taussig shunt at 2 years of age with transannular patching at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient's new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation. CONCLUSION: It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.

Caring for the Critically Ill Adult Congenital Heart Disease Patient.

PURPOSE OF REVIEW: This review aims to discuss the unique challenges that adult congenital heart disease (ACHD) patients present in the intensive care unit. RECENT FINDINGS: Recent studies suggest that ACHD patients make up an increasing number of ICU admissions, and that their care greatly improves in centers with specialized ACHD care. Common reasons for admission include arrhythmia, hemorrhage, heart failure, and pulmonary disease. It is critical that the modern intensivist understand not only the congenital anatomy and subsequent repairs an ACHD patient has undergone, but also how that anatomy can predispose the patient to critical illness. Additionally, intensivists should rely on a multidisciplinary team, which includes an ACHD specialist, in the care of these patients.

Characteristics and Outcomes of a Single-Centre Cohort of Adult Congenital Heart Disease Patients Referred for Heart Transplant.

BACKGROUND: Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. METHOD: Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021). Database analysis of patient demographics, characteristics, wait-listing, and transplantation outcomes was performed. RESULTS: A total of 45 patients (mean age 37±9.9 years old; 69% male) were referred for HTx with a mean follow-up of 5.9±6.3 years. Of these, 22 of 45 (49%) were listed and transplanted, including one heart-lung transplant. The commonest diagnosis was dextro-transposition of the great arteries (13/45, 29%). Most patients, 33 of 45 (73.3%) had undergone at least one cardiac surgery in childhood. Indications for HTx referral included HF in 34 of 45 (75%), followed by pulmonary hypertension in 7 of 45 (11%). Median transplant wait-list time was 145 days (interquartile range, 112-256). Of the 23 patients not wait-listed, the reasons included clinical stability in 13 of 45 (29%), psychosocial factors in 2 of 45 (4.4%) and prohibitive surgical risk, including multiorgan dysfunction, in 8 of 45 (17.7%). Transplant was of a single organ in most, 21 of 22 (95.5%). Overall mortality was 5 of 22 (22.7%) in those after HTx, and 14 of 23 (60.9%) in those not listed (p=0.0156). CONCLUSIONS: Increasingly, ACHD patients demonstrate the need for advanced HF treatments. HTx decision-making is complex, and increased mortality is seen in those not wait-listed. Ultimately, the referral of ACHD patients for HTx is underpinned by local decision-making and experience, wait-list times and outcomes.