Complex Congenital Heart Disease in the Adult.

Congenital heart disease (CHD), a heterogeneous group of structural abnormalities of the cardiovascular system, is the most frequent cause of severe birth defects. Related to improved pediatric outcomes, there are now more adults living with CHD, including complex lesions, than children. Adults with CHD are at high risk for complications related to their underlying anatomy and past surgical palliative interventions. Adults with CHD require close monitoring and proactive management strategies to improve outcomes.

The pathophysiology of patent foramen ovale and its related complications.

The foramen ovale plays a vital role in sustaining life in-utero; however, a patent foramen ovale (PFO) after birth has been associated with pathologic sequelae in the systemic circulation including stroke/transient ischemic attack (TIA), migraine, high altitude pulmonary edema, decompression illness, platypnea-orthodeoxia syndrome (POS) and worsened severity of obstructive sleep apnea. Importantly, each of these conditions is most commonly observed among specific age groups: migraine in the 20 to 40s, stroke/TIA in the 30-50s and POS in patients >50 years of age. The common and central pathophysiologic mechanism in each of these conditions is PFO-mediated shunting of blood and its contents from the right to the left atrium. PFO-associated pathologies can therefore be divided into (1) paradoxical systemic embolization and (2) right to left shunting (RLS) of blood through the PFO. Missing in the extensive literature on these clinical syndromes are mechanistic explanations for the occurrence of RLS, including timing and the volume of blood shunted, the impact of age on RLS, and the specific anatomical pathway that blood takes from the venous system to the left atrium. Visualization of the flow pattern graphically illustrates the underlying RLS and provides a greater understanding of the critical flow dynamics that determine the frequency, volume, and pathway of flow. In the present review, we describe the important role of foramen ovale in in-utero physiology, flow visualization in patients with PFO, as well as contributing factors that work in concert with PFO to result in the diverse pathophysiological sequelae.

Pulmonary Hypertension in Adult Congenital Heart Disease-Related Heart Failure.

Already a challenging condition to define, adult congenital heart disease (ACHD) -associated heart failure (HF) often incorporates specific anatomies, including intracardiac and extracardiac shunts, which require rigorous diagnostic characterization and heighten the importance of clinicians proactively considering overall hemodynamic impacts of using specific therapies. The presence of elevated pulmonary vascular resistance dramatically increases the complexity of managing patients with ACHD-HF. Total circulatory management in patients with ACHD-HF requires input from multidisciplinary care teams and thoughtful and careful utilization of medical, interventional, and surgical approaches.

Novel oral anticoagulants versus vitamin K antagonists in patients with atrial fibrillation after transcatheter aortic valve replacement: A systematic review and meta-analysis.

BACKGROUND: The efficacy and safety of novel oral anticoagulants (NOACs) compared to the current guideline-recommended vitamin K antagonists (VKAs) in atrial fibrillation (AF) patients undergoing transcatheter aortic valve replacement (TAVR) has not been well established. We pooled evidence from all available studies to assess the risks and benefits of this drug class. METHODS: We queried electronic databases (MEDLINE, Scopus, and Cochrane central) up until January 28th, 2022 for studies comparing NOACs to VKAs in AF patients undergoing TAVR. Results from studies were presented as risk ratios (RR) and pooled using a random-effects model. Subgroup analysis by study design and meta-regression analysis were performed to explore heterogeneity. RESULTS: A total of 12 studies (3 RCTs and 9 observational) containing 12,203 patients (mean age 81.2 years; 50.5% men) were identified and included in the analysis. Pooled analysis revealed no significant difference between NOACs and VKAs in terms of stroke or systemic embolism (RR: 0.78; p = 0.18), major bleeding (RR: 0.84; p = 0.32), intracranial hemorrhage (RR 0.61; p = 0.06), all-cause mortality (RR: 0.69; p = 0.07), and myocardial infarction (RR: 1.60; p = 0.24) at a mean length of follow-up of 15.1 months. RCTs and observational studies did not significantly differ across outcomes on subgroup analysis. Meta-regression analysis found heterogeneity in all-cause mortality to be significantly explained by percentage of males (coefficient: 0.049, p = 0.007), mean age (coefficient: 0.221, p < 0.001), and CHA2DS2-VASc score (coefficient: -1.657, p < 0.001). CONCLUSIONS: This meta-analysis suggests that outcomes with NOACs do not significantly differ compared to VKAs following TAVR in patients with AF.

Burden and Impact of Arrhythmias in Repaired Tetralogy of Fallot.

PURPOSE OF REVIEW: Arrhythmias are a leading cause of morbidity and mortality following repair of tetralogy of Fallot (TOF). This review will highlight current understanding of arrhythmia pathogenesis in this patient population and review novel therapeutic options. RECENT FINDINGS: Risk factors for developing ventricular arrhythmias in repaired TOF have thus far been better defined than for atrial arrhythmias. Growing understanding of the pathophysiology of arrhythmias, development of risk stratification models, and novel techniques such as electrophysiologic ultrahigh-density mapping should help to better identify patients that benefit from advanced therapies such as ablation and implantable cardioverter defibrillators. Atrial and ventricular arrhythmias are common in TOF patients. Methods of risk stratification and therapeutic approaches are rapidly evolving, leading to ever improving clinical outcomes in this patient population.

Invasive Hemodynamic Evaluation of the Fontan Circulation: Current Day Practice and Limitations.

PURPOSE OF REVIEW: Establishing the Fontan circulation has led to improved survival in patients born with complex congenital heart diseases. Despite early success, the long-term course of Fontan patients is complicated by multi-organ dysfunction, mainly due to a combination of low resting and blunted exercise-augmented cardiac output as well as elevated central venous (Fontan) pressure. Similarly, despite absolute hemodynamic differences compared to the normal population with biventricular circulation, the "normal" ranges of hemodynamic parameters specific to age-appropriate Fontan circulation have not been well defined. With the ever-increasing population of patients requiring Fontan correction, it is of utmost importance that an acceptable range of hemodynamics in this highly complex patient cohort is better defined. RECENT FINDINGS: Multiple publications have described hemodynamic limitations and potential management options in patients with Fontan circulation; however, an acceptable range of hemodynamic parameters in this patient population has not been well defined. Identification of "normal" hemodynamic parameters among patients with Fontan circulation will allow physicians to more objectively define indications for intervention, which is a necessary first step to eliminate institutional and regional heterogeneity in Fontan management and potentially improve long-term clinical outcomes.

A multiscale model of vascular function in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by recurrent or unresolved pulmonary thromboemboli, leading to perfusion defects and increased arterial wave reflections. CTEPH treatment aims to reduce pulmonary arterial pressure and reestablish adequate lung perfusion, yet patients with distal lesions are inoperable by standard surgical intervention. Instead, these patients undergo balloon pulmonary angioplasty (BPA), a multisession, minimally invasive surgery that disrupts the thromboembolic material within the vessel lumen using a catheter balloon. However, there still lacks an integrative, holistic tool for identifying optimal target lesions for treatment. To address this insufficiency, we simulate CTEPH hemodynamics and BPA therapy using a multiscale fluid dynamics model. The large pulmonary arterial geometry is derived from a computed tomography (CT) image, whereas a fractal tree represents the small vessels. We model ring- and web-like lesions, common in CTEPH, and simulate normotensive conditions and four CTEPH disease scenarios; the latter includes both large artery lesions and vascular remodeling. BPA therapy is simulated by simultaneously reducing lesion severity in three locations. Our predictions mimic severe CTEPH, manifested by an increase in mean proximal pulmonary arterial pressure above 20 mmHg and prominent wave reflections. Both flow and pressure decrease in vessels distal to the lesions and increase in unobstructed vascular regions. We use the main pulmonary artery (MPA) pressure, a wave reflection index, and a measure of flow heterogeneity to select optimal target lesions for BPA. In summary, this study provides a multiscale, image-to-hemodynamics pipeline for BPA therapy planning for patients with inoperable CTEPH. NEW & NOTEWORTHY This article presents novel computational framework for predicting pulmonary hemodynamics in chronic thromboembolic pulmonary hypertension. The mathematical model is used to identify the optimal target lesions for balloon pulmonary angioplasty, combining simulated pulmonary artery pressure, wave intensity analysis, and a new quantitative metric of flow heterogeneity.

Emergency department evaluation of chest pain among adult congenital heart disease patients.

BACKGROUND: Data regarding emergency department (ED) assessment of acute chest pain (CP) and incidence of myocardial infarction (MI) among adult congenital heart disease (ACHD) patients, relative to the non-congenital population, is lacking. OBJECTIVES: To describe MI risk in ACHD patients presenting to the ED with chest pain and to compare clinical characteristics, diagnostic testing patterns, and outcomes to controls. METHODS: We retrospectively identified a cohort of ACHD patients presenting with acute CP and matched them with non-ACHD controls at a large tertiary-level ED during the period 1998-2018. RESULTS: The congenital and control cohorts comprised 297 patients respectively. While MI was less common among ACHD patients (5.2%) than controls (19.7%), P = .01, arrhythmia (14% vs 6%, P < .001) and acute heart failure (3% vs 0.3%, P = .02) were more often the cause of symptoms. Despite more often presenting with non-anginal CP (81% vs 66%, P < .001) and having fewer CAD risk factors (P = .03), ACHD patients underwent more frequent stress testing (22% vs 14%, P < .001) and underwent invasive coronary angiography with equal frequency (7% vs 8%, P = .99). The trend of greater diagnostic scrutiny for acute coronary disease, in the absence of increased risk, strongly correlated with degree of congenital complexity. Both CP character and HEART Score reliably predicted MI for ACHD patients and controls (both P < .001). CONCLUSION: MI is an uncommon cause of CP among ACHD patients presenting to the ED and occurs less frequently than seen in the general population. Established MI predictors, CP character and HEART Score, can reliably identify MI in ACHD patients.

Contribution of individual components to composite end points in contemporary cardiovascular randomized controlled trials.

Cardiovascular randomized controlled trials (RCTs) typically set composite end points as the primary outcome to enhance statistical power. However, influence of individual component end points on overall composite outcomes remains understudied. METHODS: We searched MEDLINE for RCTs published in 6 high-impact journals (The Lancet, the New England Journal of Medicine, Journal of the American Medical Association, Circulation, Journal of the American College of Cardiology and the European Heart Journal) from 2011 to 2017. Two-armed, parallel-design cardiovascular RCTs which reported composite outcomes were included. All-cause or cardiovascular mortality, myocardial infarction, heart failure, and stroke were deemed "hard" end points, whereas hospitalization, angina, and revascularization were identified as "soft" end points. Type of outcome (primary or secondary), event rates in treatment and control groups for the composite outcome and of its components according to predefined criteria. RESULTS: Of the 45.8% (316/689) cardiovascular RCTs which used a composite outcome, 79.4% set the composite as the primary outcome. Death was the most common component (89.8%) followed by myocardial infarction (66.1%). About 80% of the trials reported complete data for each component. One hundred forty-seven trials (46.5%) incorporated a "soft" end point as part of their composite. Death contributed the least to the estimate of effects (R2 change = 0.005) of the composite, whereas revascularization contributed the most (R2 change = 0.423). CONCLUSIONS: Cardiovascular RCTs frequently use composite end points, which include "soft" end points, as components in nearly 50% of studies. Higher event rates in composite end points may create a misleading interpretation of treatment impact due to large contributions from end points with less clinical significance.

Hybrid transcatheter pulmonary valve replacement with a SAPIEN S3 valve after pulmonary artery banding via left lateral thoracotomy.

For many patients with repaired congenital heart disease, the need for reintervention on dysfunctional right ventricular outflow tracts is pervasive. Many such patients are poor candidates for both transcatheter pulmonary valve replacement and cardiopulmonary bypass, and hybrid surgical and transcatheter procedures have evolved to meet this need. We present two cases of hybrid pulmonary valve replacement involving pulmonary artery band placement via left anterior thoracotomy followed by transvenous placement of a SAPIEN S3 valve without prestenting. This approach avoids cardiopulmonary bypass as well as redo sternotomy and will likely see an increase in utilization in the future.

Intensive Care Unit Management of the Adult with Congenital Heart Disease.

PURPOSE: Adults with congenital heart disease (ACHD) are a rapidly growing population with ever-increasing complexity, and intensive care unit (ICU) management is often necessary. This review summarizes common cardiovascular and non-cardiovascular complications in ACHD and provides a framework for ICU care. RECENT FINDINGS: Heart failure is the leading cause of hospitalization and mortality in ACHD. Varied anatomy and repairs, as well as differing physiological complications, limit generalized application of management algorithms. Recent studies suggest that earlier mechanical support in advanced cases is feasible and potentially helpful. Cardiac arrhythmias are poorly tolerated and often require immediate attention. Other complications requiring intensive care include infections such as endocarditis and COVID-19, pulmonary hypertension, renal failure, hepatic dysfunction, coagulopathy, and stroke. Successful ICU care in ACHD requires a multi-disciplinary approach with careful consideration of anatomy, physiology, and associated comorbidities. Few studies have formally examined ICU management in ACHD and further research is necessary.

Approaching End-of-Life Decisions in Adults with Congenital Heart Disease.

PURPOSE: Despite tremendous advances in medical and surgical care, some adults with congenital heart disease (ACHD) develop terminal conditions where therapy is limited. This paper reviews the important role of palliative care, advance care planning (ACP), and end-of-life (EOL) care in ACHD. RECENT FINDINGS: Recent studies suggest that ACP is infrequently utilized in ACHD. Patients generally express interest in learning more about EOL care, though few ACHD providers have received adequate training to confidently conduct these discussions. Most barriers to communication are largely addressable, and an organized approach to ACP that encourages active patient participation followed by clear documentation is more likely to be successful. Palliative care appears complementary to standard medical care and can be introduced at any stage of illness, with proven benefit in similar patient populations. ACP is an important part of the routine care for all ACHD. Patient preferences should be identified early and palliative methods incorporated whenever necessary.

Transcatheter closure of patent foramen ovale following cryptogenic stroke: An updated meta-analysis of randomized controlled trials.

BACKGROUND: Transcatheter closure of patent foramen ovale (PFO) after cryptogenic stroke has long been a contentious issue. Herein, we pool aggregate data examining safety and efficacy of transcatheter closure of PFO compared with medical therapy following initial cryptogenic stroke. METHODS: We searched for randomized clinical trials (RCT) that compared device closure with medical management and reported on subsequent stroke and adverse events. Stroke was considered as the primary efficacy endpoint, whereas bleeding and atrial fibrillation were considered primary safety endpoints. Data were pooled by the random effects model and I2 was used to assess heterogeneity. RESULTS: A total of 5 RCT investigating 3630 patients met inclusion criteria. Pooled analysis revealed that device closure compared to medical management was associated with a significant reduction in stroke (RR=0.3, 95% CI=0.02-0.57). There was, however, a significant increase in atrial arrhythmias with device therapy (RR=4.8, 95% CI=2.2-10.7). We found no increase in bleeding (RR=0.80, 95% CI=0.5-1.4), death (RR=0.76, 95% CI=0.3-1.99) or "any adverse events" (RR=1.02, 95% CI=0.85-1.23) with device therapy. Sub-group analysis revealed that device closure significantly reduced the incidence of the composite primary endpoint among patients who had moderate to large shunt sizes (RR=0.22, 95% CI=0.02-0.42). CONCLUSIONS: Transcatheter closure is associated with a significant reduction in the risk of stroke compared to medical management at the expense of an increased risk of atrial arrhythmias.

Heart Transplantation and Mechanical Circulatory Support in Adults with Congenital Heart Disease.

PURPOSE OF REVIEW: To assess current management strategies for advanced heart failure in adults with congenital heart disease, including heart transplantation and mechanical circulatory support. RECENT FINDINGS: Current data demonstrate that adults with CHD generally experience higher short-term mortality after heart transplantation and MCS implantation, but enjoy superior long-term survival. Such patients are nonetheless less likely to receive a transplant than non-ACHD peers due to a variety of factors, including lack of applicability of current listing criteria to HF in ACHD. MCS is underutilized in ACHD, but provides similar quality of life benefits for ACHD and non-ACHD patients alike. Heart failure in ACHD is complex and difficult to treat, and both heart transplantation and mechanical circulatory support are often challenging to implement in this patient population. However, long-term results are encouraging, and existing data supports increasing use of MCS and transplant earlier in their disease course. Multidisciplinary care is critical to success in these complex patients.

Pulmonic Valve Disease: Review of Pathology and Current Treatment Options.

PURPOSE OF REVIEW: Our review is intended to provide readers with an overview of disease processes involving the pulmonic valve, highlighting recent outcome studies and guideline-based recommendations; with focus on the two most common interventions for treating pulmonic valve disease, balloon pulmonary valvuloplasty and pulmonic valve replacement. RECENT FINDINGS: The main long-term sequelae of balloon pulmonary valvuloplasty, the gold standard treatment for pulmonic stenosis, remain pulmonic regurgitation and valvular restenosis. The balloon:annulus ratio is a major contributor to both, with high ratios resulting in greater degrees of regurgitation, and small ratios increasing risk for restenosis. Recent studies suggest that a ratio of approximately 1.2 may provide the most optimal results. Pulmonic valve replacement is currently the procedure of choice for patients with severe pulmonic regurgitation and hemodynamic sequelae or symptoms, yet it remains uncertain how it impacts long-term survival. Transcatheter pulmonic valve replacement is a rapidly evolving field and recent outcome studies suggest short and mid-term results at least equivalent to surgery. The Melody valve® was FDA approved for failing pulmonary surgical conduits in 2010 and for failing bioprosthetic surgical pulmonic valves in 2017 and has been extensively studied, whereas the Sapien XT valve®, offering larger diameters, was approved for failing pulmonary conduits in 2016 and has been less extensively studied. Patients with pulmonic valve disease deserve lifelong surveillance for complications. Transcatheter pulmonic valve replacement is a novel and attractive therapeutic option, but is currently only FDA approved for patients with failing pulmonary conduits or dysfunctional surgical bioprosthetic valves. New advances will undoubtedly increase the utilization of this rapidly expanding technology.

Contributors to and impact of residual shunting after device closure of atrial septal defects.

BACKGROUND: The prevalence of residual shunt in patients after device closure of atrial septal defect and its impact on long-term outcome has not been previously defined. METHODS: From a prospective, single-institution registry of 408 patients, we selected individuals with agitated saline studies performed 1 year after closure. Baseline echocardiographic, invasive hemodynamic, and comorbidity data were compared to identify contributors to residual shunt. Survival was determined by review of the medical records and the Social Security Death Index. Survival analysis according to shunt included construction of Kaplan-Meier curves and Cox proportional hazards modeling. RESULTS: Among 213 analyzed patients, 27% were men and age at repair was 47 ± 17 years. Thirty patients (14%) had residual shunt at 1 year. Residual shunt was more common with Helex (22%) and CardioSEAL/STARFlex (40%) occluder devices than Amplatzer devices (9%; P = .005). Residual shunts were more common in whites (79% vs 46%, P = .004). At 7.3 ± 3.3 years of follow-up, 13 (6%) of patients had died, including 8 (5%) with Amplatzer, 5 (25%) with CardioSEAL/STARFlex, and 0 with Helex devices. Patients with residual shunting had a higher hazard of death (20% vs 4%, P = .001; hazard ratio 4.95 [1.59-14.90]). In an exploratory multivariable analysis, residual shunting, age, hypertension, coronary artery disease, and diastolic dysfunction were associated with death. CONCLUSIONS: Residual shunt after atrial septal defect device closure is common and adversely impacts long-term survival.