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.

Successful Management of Pregnancies Complicated by Type B Aortic Dissections (Both Acute and Chronic).

We describe 2 pregnancies complicated by descending aortic dissections. Patient 1 suffered an acute dissection at 28 weeks. Patient 2 had residual dissection after ascending dissection repair and conceived after detailed preconception counseling. Both were complicated by hypertension, managed by a multidisciplinary team, and ended uneventfully with cesarean deliveries.

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.

Beta-Blocker Use during Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan's Syndrome.

BACKGROUND: Pregnant patients with Marfan's syndrome (MFS) are at an increased risk for adverse aortic outcomes. While beta-blockers are used to slow aortic root dilatation in nonpregnant MFS patients, the benefit of such therapy in pregnant MFS patients remains controversial. The purpose of this study was to investigate the effect of beta-blockers on aortic root dilatation during pregnancy in MFS patients. METHODS: This was a longitudinal single-center retrospective cohort study of females with MFS who completed a pregnancy between 2004 and 2020. Clinical, fetal, and echocardiographic data were compared in patients on- versus off-beta-blockers during pregnancy. RESULTS: A total of 20 pregnancies completed by 19 patients were evaluated. Beta-blocker therapy was initiated or continued in 13 (65%) of the 20 pregnancies. Pregnancies on-beta-blocker therapy experienced less aortic growth compared with those off-beta-blockers (0.10 [interquartile range, IQR: 0.10-0.20] vs. 0.30 cm [IQR: 0.25-0.35]; p = 0.03). Using univariate linear regression, maximum systolic blood pressures (SBP), increase in SBP, and absence of beta-blocker use in pregnancy were found to be significantly associated with greater increase in aortic diameter during pregnancy. There were no differences in rates of fetal growth restriction between pregnancies on- versus off-beta-blockers. CONCLUSION: This is the first study that we are aware of to evaluate changes in aortic dimensions in MFS pregnancies stratified by beta-blocker use. Beta-blocker therapy was found to be associated with less aortic root growth during pregnancy in MFS patients.

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.

Main operating room deliveries for patients with high-risk cardiovascular disease.

BACKGROUND: High-risk cardiovascular disease (CVD) prevalence in pregnant patients is increasing. Management of this complex population is not well studied, and little guidance is available regarding labour and delivery planning for optimal outcomes. OBJECTIVE: We aimed to describe the process for and outcomes of our centre's experience with the main operating room (OR) caesarean deliveries for patients with high-risk CVD, including procedural and postpartum considerations. STUDY DESIGN: We performed a retrospective evaluation of pregnant patients with high-risk CVD who delivered in the main OR at a large academic centre between January 2010 and March 2021. Patients were classified by CVD type: adult congenital heart disease, cardiac arrest, connective tissue disease with aortopathy, ischaemic cardiomyopathy, non-ischaemic cardiomyopathy or valve disease. We examined demographic, anaesthetic and procedure-related variables and in-hospital maternal and fetal outcomes. Multidisciplinary delivery planning was evaluated before and after formalising a cardio-obstetrics programme. RESULTS: Of 25 deliveries, connective tissue disease (n=9, 36%) was the most common CVD type, followed by non-ischaemic cardiomyopathy (n=5, 20%). Scheduled deliveries that went as initially planned occurred for six patients (24%). Fourteen (56%) were unscheduled and urgent or emergent. Patients in modified WHO Class IV frequently underwent unscheduled, urgent deliveries (64%). Most deliveries were safely achieved with neuraxial regional anaesthesia (80%) and haemodynamic monitoring via arterial lines (88%). Postdelivery intensive care unit stays were common (n=18, 72%), but none required mechanical circulatory support. There were no in-hospital maternal or perinatal deaths; 60-day readmission rate was 16%. Some delivery planning was achieved for most patients (n=21, 84%); more planning was evident after establishing a cardio-obstetrics programme. Outcomes did not differ significantly by CVD group or delivery era. CONCLUSIONS: Our experience suggests that short-term outcomes of pregnant patients with high-risk CVD undergoing main OR delivery are favourable. Multidisciplinary planning may support the success of these complex cases.

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.

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.

Cardiac and Pregnancy Outcomes of Pregnant Patients With Congenital Heart Disease According to Risk Classification System.

Pregnancy risk assessment for patients with adult congenital heart disease (ACHD) must include physiologic and anatomic impacts. We aimed to determine whether maternal cardiac and pregnancy outcomes vary by disease severity defined according to the following 3 different classifications: ACHD anatomic severity, ACHD physiologic class, and modified World Health Organization (mWHO) class. Cardiac outcomes included a composite of arrhythmia, heart failure, stroke, and thromboembolism. Pregnancy outcomes included a composite of intrauterine growth restriction, preterm birth, preeclampsia, or postpartum hemorrhage. We employed generalized estimating equations to account for multiple pregnancies. Of the 245 pregnancies, 17.1% were preterm and 45.7% were cesarean deliveries. Cardiac hospitalizations occurred in 22.0% and arrhythmias in 12.7%. Cardiac outcomes tended to be more prevalent in people with more severe heart disease. Pregnancy outcomes were U-shaped or less prevalent in people with more severe disease. There was a 2.9-fold increased risk for the composite cardiac outcome for complex anatomy (adjusted incidence rate ratio 2.90, 95% confidence interval 1.08 to 7.81, p = 0.04), a 9.4-fold increased risk for physiologic class C or D (9.37, 1.28 to 68.79, p = 0.03), and a fourfold increased risk for mWHO class III or IV (3.99, 1.53 to 10.40, p = 0.005). There was a lower risk for the composite pregnancy outcome for mWHO class II or II to III (0.54, 0.36 to 0.79, p = 0.002) but no association with anatomy or physiology. In conclusion, physiologic class may be most accurately associated with adverse outcomes and therefore efforts to optimize hemodynamics before pregnancy may help to mitigate the risk.

Stroke in patients with secundum atrial septal defect and sequelae after transcatheter closure.

OBJECTIVE: To evaluate the frequency of and risk factors for stroke as a presenting feature in adult patients with secundum atrial septal defect (ASD); rates of post-closure atrial fibrillation (AF) and stroke were also assessed. METHODS: We retrospectively reviewed adult patients who presented with an ASD between 2002 and 2018, excluding those with known atrial arrhythmias. Risk factors for stroke were identified using multivariable logistic regression. Post-closure stroke was evaluated using survival analysis stratified by the presence of post-procedure AF. RESULTS: Of 346 patients with ASD (median age 44 years), 34 (10%) presented with a history of stroke. Independent risk factors included elevated body mass index over 25 (OR: 18.2; 95% CI: 4.0 to 82.2; p<0.001), smoking (OR: 9.5; 95% CI: 3.8 to 23.9; p<0.001) and a prominent Eustachian valve (OR: 9.2; 95% CI: 3.4 to 25.2; p<0.001). A scoring system based on these three parameters provided robust stroke risk stratification. During a median follow-up of 12 months after closure, 12 patients (4%) experienced AF and 4 patients (1%) had a new stroke. AF was highly associated with development of stroke post-closure (p<0.001). CONCLUSIONS: In this study population, the incidence of stroke prior to ASD closure among patients without atrial arrhythmias was 10%. Risk factors included obesity, smoking and prominent Eustachian valve anatomy. Lifestyle changes should be recommended for at-risk patients, and it may be reasonable to consider ASD closure in the absence of haemodynamic indications in patients at increased risk of stroke.

Experience With Advance Care Planning Discussions Among Pregnant Women With Congenital Heart Disease.

CONTEXT: Women with adult congenital heart disease (ACHD) have an increased risk of adverse events during pregnancy. Advance care planning may therefore be an appropriate component of prenatal care. OBJECTIVE: The aim of this study was to describe the perspectives of women with ACHD surrounding advance care planning during pregnancy. METHODS: We conducted a thematic analysis of 25 semi-structured interviews with women with ACHD who had been pregnant. Purposive sampling was used to gain diversity in ACHD lesion complexity, race, age at pregnancy, and marital status. RESULTS: Mean age at pregnancy was 29 years (range 15-41 years), and ACHD was classified as simple (24%), moderate (44%), or complex (32%). We identified three primary themes: 1) the role of advance care planning in being prepared and providing security for family; 2) reasons for avoiding advance care planning, including its lower priority among more pressing concerns and the impact it might have on their current psychological state; and 3) varied openness to advance care planning discussions during pregnancy. CONCLUSION: Advance care planning is not a routine part of prenatal care in ACHD, and its role in this population requires further assessment.

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.

Health Service Utilization Patterns Among Adults With Congenital Heart Disease: A Population-Based Study.

Background Several studies have examined hospitalizations among patients with adult congenital heart disease (ACHD). Few investigated other services or utilization patterns. Our aim was to study service utilization patterns and predictors among patients with ACHD. Methods and Results We identified 11 653 patients with ACHD aged ≥18 years (median, 47 years), through electronic records of 2 large Israeli healthcare providers (2007-2011). The association between patient, disease, and sociogeographic characteristics and healthcare resource utilization were modeled as recurrent events accounting for the competing death risk. Patients with ACHD had high healthcare utilization rates compared with the general population. The highest standardized service utilization ratios (SSRs) were found among patients with complex congenital heart disease including primary care visits (SSR, 1.53; 95% CI, 1.47-1.58), cardiology outpatient visits (SSR, 5.17; 95% CI, 4.69-5.64), hospitalizations (SSR, 6.68; 95% CI, 5.82-7.54), and days in hospital (SSR, 15.37; 95% CI, 14.61-16.12). Adjusted resource utilization hazard increased with increasing lesion complexity. Hazard ratios (HRs) for complex versus simple disease were: primary care (HR, 1.14; 95% CI, 1.06-1.23); cardiology outpatient visits (HR, 1.40; 95% CI, 1.24-1.59); emergency department visits (HR, 1.19; 95% CI, 1.02-1.39); and hospitalizations (HR, 1.75; 95% CI, 1.49-2.05). Effects attenuated with age for cardiology outpatient visits and hospitalizations and increased for emergency department visits. Female sex, geographic periphery, and ethnic minority were associated with more primary care visits, and female sex (HR versus men, 0.89 [95% CI, 0.84-0.94]) and periphery (HR, 0.72 [95% CI, 0.58-0.90] for very peripheral versus very central) were associated with fewer cardiology visits. Arab minority patients also had high hospitalization rates compared with the majority group of Jewish or other patients. Conclusions Healthcare utilization rates were high among patients with ACHD. Female sex, geographic periphery, and ethnicity were associated with less optimal service utilization patterns. Further research should examine strategies to optimize service utilization in these groups.

Acute Increase in Deaths Among Patients With Adult Congenital Heart Disease During COVID-19: Single-Center Experience.

Fear of acquiring severe acute respiratory syndrome coronavirus-2 infection is a major contributor to underutilization of the health care system during the current pandemic. In this report, we describe 4 cases of unexpected deaths that occurred within a short time period in patients with adult congenital heart disease without warning symptoms. (Level of Difficulty: Intermediate.).