Exercise as Medicine: Evaluation and Prescription for Adults with Congenital Heart Disease.

PURPOSE OF REVIEW: Understanding exercise physiology as it relates to adult congenital heart disease (ACHD) can be complex. Here we review fundamental physiologic principles and provide a framework for application to the unique ACHD patient population. RECENT FINDINGS: ACHD exercise participation has changed dramatically in the last 50 years. A modern approach focuses on exercise principles and individual anatomic and physiologic considerations. With an evolving better understanding of ACHD exercise physiology, we can strategize plans for patients to participate in dynamic and static exercises. Newly developed technologies including wearable devices provide additive information for ACHD providers for further assessment and monitoring. Preparation and assessment for ACHD patients prior to exercise require a thoughtful, personalized approach. Exercise prescriptions can be formulated to adequately meet the needs of our patients.

Adults with CHD balancing motivations and concerns in pregnancy decision-making.

BACKGROUND: While the incidence of pregnancy has increased among individuals with adult CHD, little has been described about considerations and experiences of patients with adult CHD regarding pregnancy. OBJECTIVE: We aimed to explore patients' motivations, concerns, and decision-making processes regarding pregnancy. METHODS: In April 2019-January 2020, we conducted in-depth telephone interviews with patients (n = 25) with simple, moderate, or complex adult CHD, who received prenatal care at the University of Washington during 2010-2019 and experienced a live birth. Transcripts were analysed using thematic analysis. RESULTS: Participants described motivations for pregnancy as both internal desires (motherhood, marriage fulfillment, biological connection, fetal personhood, self-efficacy) and external drivers (family or community), as well as concerns for the health and survival of themselves and the fetus. Factors that enabled their decision to maintain a pregnancy included having a desire that outweighed their perceived risk, using available data to guide their decision, planning for contingencies and knowing their beliefs about termination, plus having a trusted healthcare team, social support, and resources. Factors that led to insurmountable risk in subsequent pregnancies included desire having been fulfilled by the first pregnancy, compounding risk with age and additional pregnancies, new responsibility to an existing child, and reduced healthcare team and social support. CONCLUSIONS: Understanding individuals' motivations and concerns, and how they weigh their decisions to become or remain pregnant, can help clinicians better support patients with adult CHD considering pregnancy. Clinician education on patient experiences is warranted.

Patent Foramen Ovale Closure among Patients with Hypercoagulable States Maintained on Antithrombotic Therapy.

BACKGROUND: Percutaneous device closure was shown to effectively prevent recurrent strokes in patients with patent foramen ovale (PFO). Whether this protective effect is relevant for patients with hypercoagulable states (HCSs) is unknown as they were not represented in prior studies. METHODS: Data on 136 consecutive patients with a PFO and clinically significant HCS were retrospectively collected. PFO closure and antithrombotic regimen were decided on an individual basis by the treating physicians, and adherence to therapy was routinely evaluated. The outcome was the occurrence of cerebrovascular accident (CVA) or transient ischemic attack (TIA). RESULTS: HCS types consisted of antiphospholipid syndrome (31%), factor-5 Leiden mutation (22%), prothrombin mutation (18%), protein S deficiency (15%), protein C deficiency (7%), methyl-tetra-hydro folate reductase mutation (5%), and essential thrombocytosis (2%). 102 patients (75%) were maintained on anticoagulants and the remaining on antiplatelet therapy. PFO closure was undertaken in 85 (63%); antithrombotic therapy was not interrupted prior to or after the procedures. At a mean follow-up of 46 ± 8 months, 23 patients (17%; 95% confidence interval, 9.3-22%) experienced an outcome event, mainly in the form of CVAs (n = 15, 65%). In multivariable analysis, PFO closure was associated with a 5-fold decrease in the risk of CVA/TIA (p = 0.02). This effect was independent of the type of HCS or antithrombotic therapy. CONCLUSIONS: Among patients with HCSs maintained on anticoagulant or antiplatelet therapies, PFO closure was associated with a significantly lower risk of CVA or TIA.

Surgical Pericardiectomy for Constrictive Pericarditis: A Single Tertiary Center Experience.

BACKGROUND: Although surgery is the sole therapeutic option for patients with constrictive pericarditis (CP), reports on high postoperative mortality rates have led to hesitant surgery referral. The aim of this study was to report the short- and long-term outcomes of surgical pericardiectomy (SP) from a large tertiary center. METHODS: Between January 2005 and January 2017, 55 consecutive patients underwent SP after comprehensive echocardiography, computed tomography, and hemodynamic studies. Detailed clinical, imaging, surgical techniques and follow-up outcomes were recorded. RESULTS: The most common etiology was idiopathic (n = 27, 49%) and 33 patients (60%) were in functional class 3/4. Sixteen patients (29%) underwent concomitant interventions during SP, and cardiopulmonary bypass (CPB) was used in these, as well as in four additional cases. Complete resection, independent of CPB, was achieved in 96%. One patient died during the index hospitalization, and four (7%) needed re-explorations due to bleeding. While 12 patients (22%) died during a mean follow-up of 52 ± 39 months, only 1 death was due to right heart failure. Functional class significantly improved (with a p-value < 0.001), diuretics were discontinued in all, and significant reductions of right atrial pressures were recorded. None of these outcomes differed as a result of concomitant interventions at the time of SP. CONCLUSION: Short- and long-term outcomes of SP, performed either alone or concomitantly with other procedures, indicate high safety and favorable clinical and hemodynamic efficacy for the treatment of CP.

What Every Cardiologist Should Know About the 2018 Updated Adult Congenital Cardiology Guidelines.

PURPOSE OF REVIEW: Review the recently updated guidelines for the management of patients with adult congenital heart disease (ACHD) with a focus on the changes between these guidelines and the prior guidelines. RECENT FINDINGS: The 2018 guidelines for the management of patients with ACHD focused on utilizing the available data and limiting the number recommendations based only on expert opinion. These guidelines implement a new anatomic and physiological classification scheme to guide management of patients, which takes into account both the underlying anatomy as well as residual cardiac disease and symptoms. Given a lack of robust outcomes data for many types of CHD, the new guidelines provide fewer total recommendations than the prior version, emphasizing the data that is available and drawing attention to the need for additional data. The 2018 guidelines provide the field with a comprehensive update in the management of ACHD patients with an emphasis on the available data.

Utility of biomarkers in adult Fontan patients with decompensated heart failure.

BACKGROUND: The incidence of heart failure is increasing within the Fontan population. The use of serological markers, including B-type natriuretic peptide, has been limited in this patient population. METHODS: This was a single-centre retrospective study of Fontan patients in acute decompensated heart failure. Fontan patients underwent a 1:2 match with non-Fontan patients for each heart failure hospitalisation for comparative analysis. A univariate logistic regression model was used to assess associations between laboratory and echocardiographic markers and a prolonged length of stay of 7 days or greater. RESULTS: B-type natriuretic peptide levels were significantly lower in Fontan patients admitted for heart failure than that in non-Fontan patients [390.9 (±378.7) pg/ml versus 1245.6 (±1160.7) pg/ml, respectively, p < 0.0001] and were higher in Fontan patients with systemic ventricular systolic or diastolic dysfunction than that in Fontan patients with normal systemic ventricular function [833.6 (±1547.2) pg/ml versus 138.6 (±134.0) pg/ml, p = 0.017]. The change from the last known outpatient value was smaller in Fontan patients in comparison with non-Fontan patients [65.7 (±185.7) pg/ml versus 1638.0 (±1444.7) pg/ml, respectively, p < 0.0001]. Low haemoglobin and high blood urea nitrogen levels were associated with a prolonged length of stay. CONCLUSION: B-type natriuretic peptide levels do not accurately reflect decompensated heart failure in Fontan patients when compared to non-Fontan heart failure patients and should, therefore, be used with caution in this patient population.

Echocardiographic evaluation of univentricular physiology and cavopulmonary shunts.

Since the surgical palliation of a univentricular congenital heart defect was first described in the early 1970s, thousands of lives were saved by this groundbreaking operation. Although initially described for the palliation of tricuspid valve atresia, the Fontan operation is now utilized for many other univentricular heart defects involving either hypoplastic right or left ventricles, and the number of babies who survive the surgery and the postsurgical hospitalization continues to grow. Echocardiography constitutes the mainstay follow-up imaging modality for patients with a Fontan system. In order to obtain all the needed information from the echocardiographic studies, close familiarity with the native congenital heart defects, the build of the Fontan systems, and the potential long-term complications is required. Detailed studies provide valuable information on the various components of the Fontan circulation such as the function of the single ventricle and the valves, flow in the Glenn and Fontan pathways, and possible sources of cyanosis and shunts. Three-dimensional and transesophageal echocardiogram studies can assist in delineating the Fontan anatomy and to evaluate smaller intracardiac structures.

The impact of tricuspid valve regurgitation severity on exercise capacity and cardiac-related hospitalisations among adults with non-operated Ebstein's anomaly.

BACKGROUND: Tricuspid valve regurgitation is an inherent part of Ebstein's anomaly, yet whether the severity of the regurgitation further impairs exercise capacity and contributes to long-term morbidity on top of the lesion severity per se is unknown. METHODS: To evaluate for this potential effect, we included 30 patients with Ebstein's anomaly who did not undergo any form of surgical interventions and had a cardiopulmonary exercise test and echocardiographic studies in this retrospective analysis. Echocardiographic studies and cardiopulmonary exercise tests were critically reviewed for lesion severity grade, tricuspid regurgitation degree, and exercise parameters. Cardiac-related hospitalisations were recorded from computerised medical records and during clinic visits. RESULTS: Fourteen patients (47%) had moderate and 8 (27%) had severe regurgitation. Patients with ≥ moderate regurgitation exhibited significantly lower exercise capacity (median % predicted maximal oxygen consumption, 62 versus 79%, p = 0.03) and venilatory efficiency at exercise. When stratifying exercise results by regurgitation degree, a stepwise decrease in oxygen consumption and ventilatory efficiency with increasing regurgitation severity was observed, regardless of the anatomic lesion severity. During a median follow-up of 4.6 years, > moderate tricuspid regurgitation was associated with significantly lower cumulative probability of freedom from cardiac hospitalisations. CONCLUSIONS: We report that among non-operated Ebstein's anomaly patients, greater tricuspid regurgitation severity was associated with worse exercise capacity and with overall higher probability of cardiac-related hospitalisations independent from the underlying lesion severity.

Leadless Pacemaker Implantation in a Pediatric Patient with Prolonged Sinus Pauses.

Permanent cardiac pacing is the only effective solution for patients with symptomatic bradycardia and heart block. About 10% of patients undergoing implantation of the conventional pacing system develop complications related to the subcutaneous pocket or the leads and in pediatric patients lead problems may rise in up to 30% of the patients. The leadless pacemaker devices were developed in order to minimize some of those complications. We present a case of an 11-year-old patient who presented after the sudden death of his older brother, with recurrent episodes of syncope and documented prolonged sinus pauses. The patient underwent percutaneous implantation of a leadless Micra™ pacemaker device with optimal results.

Exercise Hemodynamics for the Diagnosis of Diastolic Dysfunction in Dyspneic Patients with Systemic Sclerosis.

OBJECTIVES: To assess the added diagnostic value of using exercise hemodynamics during RHC in assessment of patients with symptomatic SSc. METHODS: We performed 22 RHCs in 17 SSc patients with dyspnea and/or pulmonary arterial hypertension (PAH). Exercise was performed in 15 RHCs using isotonic arm exercises while holding a 1 kg weight in each hand. Measurements of pulmonary arterial pressure (PAP), pulmonary arterial wedge pressure (PAWP), and cardiac output (CO) were taken at rest and during peak exercise. RESULTS: Normal resting RHC (PAP 22  3 mmHg, PAWP 11  3 mmHg) was found in seven cases. Of these, exercise induced elevation in PAP was found in three (38  7 mmHg), and exercise induced elevation in PAWP was found in four (24  6 mmHg). Elevated resting PAP was found in 15 (41  11 mmHg) with minor changes in exercise. Of the 22 RHCs, elevation of the PAWP was found in 11 (50%), half of which were in response to exercise. CONCLUSIONS: In symptomatic SSc patients, exercise hemodynamics provides important information on diastolic dysfunction that is not available with non-invasive testing. Findings on exercise RHC can explain patient symptoms in up to 50% of cases. Earlier and more accurate diagnosis of patient symptoms can aid in tailoring the correct therapy for each.

Outcomes following the implantation of cardioverter-defibrillator for primary prevention in transposition of the great arteries after intra-atrial baffle repair: a single-centre experience.

AIMS: Patients with D-loop transposition of the great arteries (D-TGA) status post intra-atrial baffling are at an increased risk for sudden cardiac arrest. The benefit of primary implantable cardioverter-defibrillator (ICD) implantation in these patients is questionable due to high burden of adverse events. We aimed to evaluate the incidence and causes of all types of device shocks, as well as of device-related complications among patients with D-TGA implanted with ICDs for primary prevention. METHODS AND RESULTS: Retrospective analysis of all patients with D-TGA who underwent atrial switch procedure and ICD implantation for primary prevention. Eighteen patients (83% males) were identified. Average age at atrial switch was 2.5 years (range 0.1-17) and at ICD implantation 26 years (15-41). During a median follow-up of 4 years, 10 patients (55%) received shocks for non-ventricular arrhythmic events, whereas 1 patient was shocked for ventricular tachycardia, for an annual rate of shock delivery of 7.1%. The most common cause for shock delivery was the occurrence of atrial arrhythmias, mostly in the form of atrial flutter. Elevated systemic ventricular end-diastolic pressures were found to be associated with an increased risk for inappropriate shocks. Five patients (28%) required lead extraction and three required generator change due to device recalls during follow-up. CONCLUSION: Atrial arrhythmias were the most common cause for ICD shocks in a primary prevention population, while ventricular tachycardia was infrequent. The association between elevated end-diastolic pressures and the occurrence of arrhythmias demonstrates the close mechano-electrical relationship in D-TGA and may be an important predictor of arrhythmic events.

Predictors and outcomes of "super-response" to cardiac resynchronization therapy.

BACKGROUND: Cardiac resynchronization therapy (CRT) has been shown to improve heart failure (HF) symptoms and survival. We hypothesized that a greater improvement in left-ventricular ejection fraction (LVEF) after CRT is associated with greater survival benefit. METHODS AND RESULTS: In 693 patients across 2 international centers, the improvement in LVEF after CRT was determined. Patients were grouped as non-/modest-, moderate-, or super-responders to CRT, defined as an absolute change in LVEF of ≤5%, 6-15%, and >15%, respectively. Changes in New York Heart Association (NYHA) functional class and left ventricular end-diastolic dimension (LVEDD) were assessed for each group. There were 395 non-/modest-, 186 moderate-, and 112 super-responders. Super-responders were more likely to be female and to have nonischemic cardiomyopathy, lower creatinine, and lower pulmonary artery systolic pressure than non-/modest- and moderate-responders. Super-responders were also more likely to have lower LVEF than non-/modest-responders. There was no difference in NYHA functional class, mitral regurgitation grade, or tricuspid regurgitation grade between groups. Improvement in NYHA functional class (-0.9 ± 0.9 vs -0.4 ± 0.8 [P < .001] and -0.6 ± 0.8 [P = .02]) and LVEDD (-8.7 ± 9.9 mm vs -0.5 ± 5.0 and -2.4 ± 5.8 mm [P < .001 for both]) was greatest in super-responders. Kaplan-Meier survival analysis revealed that super-responders achieved better survival compared with non-/modest- (P < .001) and moderate-responders (P = .049). CONCLUSIONS: Improvement in HF symptoms and survival after CRT is proportionate to the degree of improvement in LV systolic function. Super-response is more likely in women, those with nonischemic substrate, and those with lower pulmonary artery systolic pressure.

Safety and efficacy of strategic implantable cardioverter-defibrillator programming to reduce the shock delivery burden in a primary prevention patient population.

AIMS: Strategically chosen ventricular tachycardia (VT)/ventricular fibrillation (VF) detection and therapy parameters aimed at reducing shock deliveries were proven effective in studies that utilized single manufacturer devices with a follow-up of up to 1 year. Whether these beneficiary effects can be generalized to additional manufacturers and be maintained for longer periods is to be determined. Our aim was to evaluate the durability and applicability of the programming of strategic implantable cardioverter-defibrillators (ICDs) of various manufacturers, which is aimed at reducing the shock delivery burden in primary prevention ICD recipients. METHODS AND RESULTS: A retrospective analysis of prospectively collected data of 300 ICD recipients of various manufacturers was conducted; 160 devices were strategically programmed to reduce shocks and 140 were not. The primary endpoint was the composite of death and appropriate shocks. Additional outcomes were inappropriate shocks, syncope events, and non-sustained VTs. At a median follow-up of 24 months, 19 patients died, 31 received appropriate shocks, and 41 received inappropriate shocks. Multivariate analysis showed that strategic programming dedicated to shock reduction was associated with a 64% risk reduction in the primary endpoint [hazard ratio (HR): 0.13-0.93; P = 0.03] and a 70% reduction in inappropriate shock deliveries (HR: 0.16-0.72; P = 0.01). Very few syncope events occurred (five patients, 1.6%), and there was no between-group difference in this outcome. CONCLUSION: Utilization of strategically chosen VT/VF detection and therapy parameters was found to be effective and safe in ICDs of various manufacturers at a median follow-up period of 2 years among primary prevention patients.

Morphological features of the P-waves at surface electrocardiogram as surrogate to mechanical function of the left atrium following a successful modified maze procedure.

AIMS: Absent left atrium (LA) mechanical contraction may occur following the modified Cox-maze operation, and was found to impose a potential risk for the occurrence of thrombo-embolic stroke. It is unknown whether certain morphological P-wave characteristics can surrogate absent LA mechanical activity. The aim of this study was to evaluate the morphological features of the P-waves on the surface electrocardiogram (ECG) of patients who underwent the maze operation and to relate them to the contractile profile of the LA. METHODS AND RESULTS: Electrocardiogram tracings of 150 consecutive patients that were in sustained sinus rhythm following the maze operation were evaluated. P-waves were scrutinized for morphology, duration, axis, and amplitude. Clinical, surgery-related, and echocardiographic data were collected and analysed. Forty-seven patients (31%) had no evidence of LA contraction at 3 months after surgery (baseline assessment) and on follow-up echocardiography. Multivariate analysis showed that a positive-only P-wave deflection at lead V1 (P = 0.03), a negative-only deflection at aVL, and a P-wave amplitude of ≤ 0.05 mV at the septal-anterior leads (P < 0.001 for both) were associated with absent LA mechanical contraction. In a secondary analysis, a risk score involving the above three parameters was developed for the prediction of stroke occurrence. Patients at the high-risk score group had a 30% survival freedom of stroke compared with 70% for patients at intermediate risk (P < 0.001). CONCLUSION: Absent LA mechanical contraction following the modified maze operation may be accompanied by a distinguished pattern of the P-waves on the surface ECG.

Exercise physiology and testing in adult patients with congenital heart disease.

As the longevity of patients with congenital heart disease improves, the number surviving to adulthood will continue to rise. Consequently, practicing physicians can expect to encounter an increasing number of adult patients with various congenital cardiac conditions. Impaired exercise tolerance in this patient population is exceptionally common; adult patients with congenital heart disease have reduced exercise capacity compared with healthy, age-matched counterparts. The different methods of evaluating exercise capacity, the characteristic physiologic abnormalities encountered in patients with various congenital cardiac conditions, the pathophysiologic mechanisms that may account for these abnormalities, and the clinical implications of these findings are discussed.

Peak oxygen consumption by smartwatches compared with cardiopulmonary exercise test in complex congenital heart disease.

OBJECTIVE: To evaluate for correlation between exercise capacity as assessed by peak oxygen consumption (pVO2) measurement during a cardiopulmonary exercise test (CPET) and smartwatches reporting this parameter in patients with adult congenital heart disease (ACHD) complex lesions. METHODS: A prospective study that included patients with ACHD either a Fontan circulation or a right ventricle supporting the systemic circulation who underwent two separate CPETs at least 1 year apart. Generalised estimating equations linear regression was performed to identify factors associated with correlation between smartwatch and CPET-derived pVO2. RESULTS: 48 patients (71% with a Fontan circulation, 42% females, mean age 33±9 years) underwent two CPETs between May 2018 and May 2022 with echocardiograms performed within 6 months of each CPET. Apple Watch was the predominant smartwatch used (79%). Smartwatch and CPET measured peak heart rate (Pearson correlation=0.932, 95% CI (0.899, 0.954)) and pVO2 (0.8627, 95% CI (0.8007, 0.9064) and 0.8634, 95% CI (0.7676, 0.9215) in the first and second CPET, respectively) correlated well, with smartwatch-measured pVO2 values measuring higher by a mean of 3.146 mL/kg/min (95% CI (2.559, 3.732)). Changes in pVO2 between the first and the second CPET also correlated well (Pearson correlation=0.9165, 95% CI (0.8549, 0.9525)), indicating that for every 1 mL/(min kg) change in CPET-measured pVO2, there was a corresponding 0.896 mL/(min kg) change in the smartwatch-measured pVO2. CONCLUSION: Both absolute values and changes over time in pVO2 as measured by smartwatches and CPETs correlate well in patients with complex ACHD.

Effects of tricuspid valve regurgitation on clinical and echocardiographic outcome in patients with cardiac resynchronization therapy.

AIMS: The severity of tricuspid regurgitation (TR) is a predictor of outcome among heart failure patients. The interaction between cardiac resynchronization therapy (CRT) and TR has not been described. In this study, we examined the effect of pre-implant TR, and worsened TR post-implant, on response to CRT and overall survival. METHODS AND RESULTS: We included all patients with successfully implanted CRT systems between 2007 and 2010. Patients were divided into two groups pre-implant: (Gp 1) no-or-mild TR; and (Gp 2) moderate-or-severe TR. Post-implant, patients were divided into two groups: (Gp A) improved or stable TR; and (Gp B) worsened TR. The clinical and echocardiographic outcome of all patients was assessed. The study included 193 patients. Thirty-five subjects (18%) had moderate or severe TR pre-implant (Gp 2). Baseline echo parameters and 6 min walk distance were worse in Gp 2 compared with Gp 1 (mild or no TR). There was no significant difference in clinical response to CRT between the two groups. However, Gp 2 had a significantly lower echocardiographic response (35 vs. 60%, P = 0.01) and higher mortality over 3 years (OR = 6.70, 95% CI = 1.8-24.5, P = 0.004). Post-implant, 25 patients (13%) developed worsened TR (Gp B), not associated with deterioration in right ventricle function or elevation in pulmonary artery pressure. Worsened TR predicted a reduced clinical response to CRT (42 vs. 70%, P = 0.006), when compared with Gp A. CONCLUSIONS: The presence of baseline moderate or severe TR is associated with increased mortality but does not predict clinical or echocardiographic response to CRT. Patients with worsened TR following CRT are less likely to clinically respond to CRT. Pacing leads passing through the tricuspid valve may worsen TR. It is conceivable that avoidance of lead-induced TR by alternative implantation techniques could improve the response rate to CRT.

Cardiopulmonary exercise testing for heart failure: pathophysiology and predictive markers.

Despite the numerous recent advancements in therapy, heart failure (HF) remains a principle cause of both morbidity and mortality. HF with preserved ejection fraction (HFpEF), a condition that shares the prevalence and adverse outcomes of HF with reduced ejection fraction, remains poorly recognised in its initial manifestations. Cardiopulmonary exercise testing (CPET), defined as a progressive work exercise test that includes non-invasive continuous measurement of cardiovascular and respiratory parameters, provides a reliable mode to evaluate for early features and for the assessment of prognostic features of both forms of HF. While CPET measurements are standard of care for advanced HF and transplant programmes, they merit a broader clinical application in the early diagnosis and assessment of patients with HFpEF. In this review, we provide an overview of the pathophysiology of exercise intolerance in HF and discuss key findings in CPETs used to evaluate both severity of impairment and the prognostic implications.

Common congenital heart problems in acute and intensive care.

Over the past five decades, there have been multiple advances in the treatment of congenital heart defects, resulting in an increasing population of adults living with congenital heart disease (CHD). Despite improved survival, CHD patients often have residual haemodynamic sequelae and limited physiologic reserve and are at increased risk for acute decompensation with occurrence of arrhythmias, heart failure, and other medical conditions. Comorbidities occur more frequently and at an earlier age in CHD patients than in the general population. The management of the critically ill CHD patient requires an understanding of the unique aspects of congenital cardiac physiology as well as the recognition of other organ systems that may be involved. Certain patients may be candidates for mechanical circulatory support, and goals of care should be established with advanced care planning.