Cardiovascular Considerations in Caring for Pregnant Patients: A Scientific Statement From the American Heart Association.

Cardio-obstetrics has emerged as an important multidisciplinary field that requires a team approach to the management of cardiovascular disease during pregnancy. Cardiac conditions during pregnancy include hypertensive disorders, hypercholesterolemia, myocardial infarction, cardiomyopathies, arrhythmias, valvular disease, thromboembolic disease, aortic disease, and cerebrovascular diseases. Cardiovascular disease is the primary cause of pregnancy-related mortality in the United States. Advancing maternal age and preexisting comorbid conditions have contributed to the increased rates of maternal mortality. Preconception counseling by the multidisciplinary cardio-obstetrics team is essential for women with preexistent cardiac conditions or history of preeclampsia. Early involvement of the cardio-obstetrics team is critical to prevent maternal morbidity and mortality during the length of the pregnancy and 1 year postpartum. A general understanding of cardiovascular disease during pregnancy should be a core knowledge area for all cardiovascular and primary care clinicians. This scientific statement provides an overview of the diagnosis and management of cardiovascular disease during pregnancy.

Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction. BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited. METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP). RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg. CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties.

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association.

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.

Mechanism for temporal changes in exercise capacity after Fontan palliation: Role of Doppler echocardiography.

BACKGROUND: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o2). METHODS: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o2 and LV function indices. RESULTS: Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak V̇o2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m2, and CI was 2.9±0.9 L/min/m2. Peak V̇o2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak V̇o2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak V̇o2 and chronotropic index improved. CONCLUSIONS: Overall, there was a temporal decline in peak V̇o2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V̇o2 that correlated with improvement in chronotropic index.

Longitudinal Outcomes of Epicardial and Endocardial Pacemaker Leads in the Adult Fontan Patient.

Placement of an epicardial pacemaker system is often preferred over an endocardial system in patients who have undergone a Fontan operation, but data are limited on how these two systems perform over time in patients with Fontan palliation. We performed a retrospective review of adults with Fontan palliation who had pacemaker implantation and interrogation data at Mayo Clinic from 1994 to 2014. Lead parameters, pacing mode, and polarity were collected at the earliest device interrogation report. Clinic notes and device interrogation reports were reviewed at implantation, 6 months, and yearly after implantation to determine impedance, capture threshold (CT), and energy threshold (ET). There were 87 patients with 168 leads in the study cohort. The mean follow-up time was 7.7 years (6 months-19 years). There were 143 epicardial leads (57 atrial and 86 ventricular) and 25 endocardial leads (20 atrial and 5 ventricular). There was no difference in the baseline lead parameters between epicardial and endocardial leads for impedance (610 ± 259 versus 583 ± 156 Ω, p = 0.93), CT (2.0 ± 1.3 versus 1.8 ± 1.3 V, p = 0.28), or ET (7.1 ± 12.5 versus 6.8 ± 18.1 µJ, p = 0.29). Compared to endocardial leads, ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Regarding clinical outcomes, epicardial leads had higher rates of failure but similar generator longevity in comparison to endocardial leads. Ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Epicardial leads had a higher rate of failure but similar generator longevity compared to endocardial leads.

Adult congenital heart disease: the challenges of a lifetime.

The growing population of adults with congenital heart disease (CHD) poses challenges for cardiac surgeons, general cardiologists and CHD sub specialists. The patients themselves, often believing themselves to be 'totally corrected' following operative repair, also face challenges with re-operations and lifelong cardiac problems. This review examines the challenges of the past, present and future for both medical providers and patients.

Outcomes in adult Fontan patients with atrial tachyarrhythmias.

BACKGROUND: The optimal management strategy for atrial tachyarrhythmia in the Fontan population is unknown. METHODS: Retrospective review of 264 adult Fontan patients with atrial tachyarrhythmia evaluating 3 clinically adopted scenarios: antiarrhythmic drug (AAD) therapy, catheter ablation (CA), and Fontan conversion (FC). These patients were followed up at Mayo Clinic from 1994 to 2014. The study objective was to compare freedom from atrial tachyarrhythmia recurrence (AR) and occurrence of composite adverse events (stroke, heart failure hospitalization, death, or heart transplant) between treatment groups. RESULTS: The age of atrial tachyarrhythmia onset was 25 ± 4 years, time from Fontan operation was 13 ± 6 years, follow-up was 74 ± 18 months, atriopulmonary Fontan was 215 (81%), and atrial flutter/intra-atrial reentry tachycardia was 173 (65%). In those managed with AAD (n = 110), freedom from AR was 7% at 60 months. Catheter ablation (n = 31) was associated with an acute procedural success of 94%, and freedom from AR was 41% at 60 months. Fontan conversion (n = 33) resulted in a perioperative mortality of 3%, and freedom from AR was 51% at 60 months. Fontan conversion and CA were similar with regard to AR (P = .14) and significantly better compared with AAD (P < .0001). Adverse events were found to occur more frequently in the patients with AR (P < .0001) and the patients treated with AAD only (P < .0001). CONCLUSIONS: Catheter ablation and FC operations are associated with less recurrence of atrial tachyarrhythmia compared with AAD. Atrial tachyarrhythmias are more likely to recur in patients with a longer history of the arrhythmia and are associated with more adverse events. Early referral to a specialty center for these interventions should be considered.

Outcomes of cardiac pacing in adult patients after a Fontan operation.

BACKGROUND: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding pacing in adult Fontan patients. The objectives of our study were to determine risk factors for pacing and occurrence of device-related complications (DRCs) and pacemaker reinterventions. METHODS: We performed a retrospective review of Fontan patients from 1994 through 2014. We defined DRCs as lead failure, lead recall, cardiac perforation, lead thrombus/vegetation, or device-related infection, and cardiovascular adverse events (CAEs) as venous thrombosis, stroke, death, or heart transplant. Pacemaker reintervention was defined as lead failure or recall. RESULTS: Of 439 patients, 166 (38%) had pacemakers implanted (79 during childhood; 87, adulthood); 114 patients (69%) received epicardial leads initially, and 52 (31%), endocardial leads. Pacing was initially atrial in 52 patients (31%); ventricular, 30 (18%); or dual chamber, 84 (51%). There were 37 reinterventions (1.9% per year) and 48 DRCs (2.4% per year). Pacemaker implantation during childhood was a risk factor for DRCs (hazard ratio, 2.01 [CI, 1.22-5.63]; P = .03). There were 70 CAEs (venous thrombosis, 5; stroke, 11; transplant, 8; and death, 46), yielding a rate of 3.5% per year. DRCs, CAEs, and reintervention rates were comparable for patients with epicardial or endocardial leads. CONCLUSIONS: More than one-third of adult Fontan patients referred to Mayo Clinic had pacemaker implantation. Epicardial leads were associated with high rate of pacemaker reinterventions but similar DRC rates in comparison to endocardial leads.

Mixed aortic valve disease: midterm outcome and predictors of adverse events.

AIMS: The aim of this article is to determine freedom from adverse events (AE) defined as symptoms, aortic valve replacement (AVR), or death in the mixed aortic valve disease (MAVD) population. METHODS AND RESULTS: We reviewed patients with moderate/severe MAVD followed at Mayo Clinic from 1994-2013. Only asymptomatic patients with normal ejection fraction and trileaflet aortic valve were included. Cox proportional-hazard models and Kaplan-Meier method were used. We identified 213 patients with moderate/severe MAVD; mean age was 69 years (±11) and 67% were males. An AE endpoint was reached in 172 patients, and 69% of these AEs occurred in moderate MAVD (peak velocity of 3-3.9 m/s). Mean follow-up was 10.1 ± 3 years, and mean time to AE was 2.9 ± 2.1 years. A mechanical prosthesis was implanted in 71/151 (47%), and 54/151 (36%) had concomitant coronary artery bypass grafting and/or aorta replacement during AVR. Early surgical mortality was 0.7%. Freedom from AE endpoint was 42% [confidence interval (CI) 39-45%] and 30% (CI 27-33%) at 3 and 5 years, respectively. Predictors of AE were peak aortic velocity [hazard ratio (HR) 2.73; CI 1.97-2.84, P < 0.0001] for every 1 m/s difference and having severe stenosis or severe regurgitation at presentation (HR 2.58; CI 2.01-4.44, P = 0.001). CONCLUSION: Moderate MAVD had high rate of AEs comparable to severe isolated aortic stenosis and should be followed as such. Patients with severe MAVD should be evaluated at least every 6 months because half of them will become symptomatic and require AVR within 1 year.

Cardiac Arrest in Pregnancy: A Scientific Statement From the American Heart Association.

This is the first scientific statement from the American Heart Association on maternal resuscitation. This document will provide readers with up-to-date and comprehensive information, guidelines, and recommendations for all aspects of maternal resuscitation. Maternal resuscitation is an acute event that involves many subspecialties and allied health providers; this document will be relevant to all healthcare providers who are involved in resuscitation and specifically maternal resuscitation.

Right Parasternal Lead Placement Increases Eligibility for Subcutaneous Implantable Cardioverter Defibrillator Therapy in Adults With Congenital Heart Disease.

BACKGROUND: The subcutaneous implantable cardioverter defibrillator (S-ICD) provides an attractive option for patients with congenital heart disease (CHD) in whom a transvenous defibrillator is contraindicated. Given the unusual cardiac anatomy and repolarization strain, the surface electrocardiogram (ECG) is frequently abnormal, potentially increasing the screen failure rate. METHODS AND RESULTS: We prospectively screened 100 adult CHD patients regardless of the presence of clinical indication for ICD utilizing a standard left sternal lead placement, as well as a right parasternal position. Baseline patient and 12-lead ECG characteristics were examined to assess for predictors of screen failure. Average patient age was 48±14 years, average QRS duration was 134±37 ms, and 13 patients were pacemaker dependent. Using the standard left parasternal electrode position, 21 patients failed screening. Of these 21 patients with screen failure, 9 passed screening with the use of right parasternal electrode positioning, reducing screening failure rate from 21% to 12%. QT interval and inverted T wave anywhere in V2-V6 leads were found to be independent predictors of left parasternal screening failure (P=0.01 and P=0.04, respectively). CONCLUSIONS: Utilization of both left and right parasternal screening should be used in evaluation of CHD patients for S-ICD eligibility. ECG repolarization characteristics were also identified as novel predictors of screening failure in this group. (Circ J 2016; 80: 1328-1335).

Pregnancy and Delivery in Women With Congenital Heart Disease.

Because of the growing population of patients with congenital heart disease (CHD), most maternal cardiac disease is now congenital in origin. For women with complex CHD, pregnancy poses an increased risk for both the mother, with complications of arrhythmias and heart failure being the most common, and the baby, with a higher chance of miscarriage, intrauterine growth retardation, and the need for early delivery. Pre-pregnancy counseling must be performed by cardiologists who have expertise in both CHD and pregnancy, with a detailed clinical assessment of the patient and the current hemodynamic situation, including echocardiography and an exercise test. In each case the approach must be individualized with consideration of the risks in each case. In some cases, such as Eisenmenger syndrome, pregnancy is contraindicated. Optimum outcomes in these complex patients are achieved when a multidisciplinary approach is used, involving maternal-fetal medicine specialists, cardiologists with expertise in CHD and obstetric anesthesia.

Amiodarone-induced thyrotoxicosis in adults with congenital heart disease--clinical presentation and response to therapy.

OBJECTIVE: The development of amiodarone-induced thyrotoxicosis (AIT) can threaten the hemodynamic stability of adult patients with congenital heart disease (CHD). Here, we describe the natural history and treatment response of AIT in this at-risk population. METHODS: We studied retrospectively all cases of AIT that occurred in CHD patients at our institution after a minimum of 3 months on amiodarone. Subjects were identified from the cohort of adults with CHD who were treated at the Mayo Clinic Adult CHD clinic between 1987 and 2009. RESULTS: We identified 23 cases of AIT: 7 were type 1, 13 were type 2, and 3 were undefined due to insufficient data. Most patients were symptomatic (17 of 23, 74%), with arrhythmia and weight loss as the most common symptoms. The majority (12 of 23, 52%) were initially observed; 10 patients (43%) were treated medically and 1 patient (5%) underwent thyroidectomy. Four patients from the observation group eventually required active treatment and 3 patients from the medical group required surgery. Asymptomatic patients tended to resolve under observation (5 of 7, 71.4%) rather than progress to active treatment (0 of 4) (P = .06). Discontinuation of amiodarone, AIT type, or use of perchlorate did not impact AIT duration. CONCLUSION: AIT in CHD patients exhibits a wide range of severity and sensitivity to medical therapy. Asymptomatic patients display a trend toward AIT resolution with observation alone. Amiodarone continuation does not appear to impact management outcome or disease duration. Additional studies in this high-risk population could identify elements of pathophysiology that would point toward better disease prevention and treatment.

Congenital heart disease and the liver.

There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug-related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart-liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long-term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population.

Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation.

OBJECTIVES: To describe the experience of a single, tertiary care institution in the care of patients with Fontan physiology undergoing anesthesia for noncardiac surgery. BACKGROUND: The Fontan procedure was developed in 1971 to palliate patients with univentricular cardiac physiology leading to long-term survival of these patients, who may now present as adults for noncardiac surgery. METHODS: We retrospectively reviewed the medical records of Fontan patients 16 years and older who underwent general anesthesia for noncardiac surgery at Mayo Clinic in Rochester, Minnesota. Preoperative data, perioperative course, intraoperative and postoperative hemodynamic, pulmonary, cardiovascular, and renal complications were described. RESULTS: Thirty-nine general anesthetics were administered to 31 patients for noncardiac surgery after Fontan palliation. Perioperative complications occurred in 12 of the 39 (31%) noncardiac surgeries, and there was one postoperative death that occurred on day 13 after ventral hernia repair. The two patients who had complications that did not resolve (long-term dialysis and death) had ejection fractions well below the mean for the group (22% and 28%). CONCLUSION: It may be more appropriate for Fontan patients to undergo anesthesia for noncardiac surgery in a tertiary institution, particularly patients with an ejection fraction of <30%. Intraoperative arterial blood pressure monitoring and overnight admission are likely appropriate for most cases.

Mortality and Morbidity of Heart Failure Hospitalization in Adult Patients With Congenital Heart Disease.

BACKGROUND: Little is known about outcomes following heart failure (HF) hospitalization among adults with congenital heart disease (CHD) in the United States. We aim to compare the outcomes of HF versus non-HF hospitalizations in adults with CHD. METHODS AND RESULTS: Using a national deidentified administrative claims data set, patients with adult congenital heart disease (ACHD) hospitalized with and without HF (ACHDHF+, ACHDHF-) were characterized to determine the predictors of 90-day and 1-year mortality and quantify the risk of mortality, major adverse cardiac and cerebrovascular events, and health resource use. Cox proportional hazard regression was used to compare ACHDHF+ versus ACHDHF- for risk of events and health resource use. Of 26 454 unique ACHD admissions between January 1, 2010 and December 31, 2020, 5826 (22%) were ACHDHF+ and 20 628 (78%) were ACHDHF-. The ACHD HF+ hospitalizations increased from 6.6% to 14.0% (P<0.0001). Over a mean follow-up period of 2.23 ± 2.19 years, patients with ACHDHF+ had a higher risk of mortality (hazard ratio [HR], 1.86 [95% CI, 1.67-2.07], P<0.001), major adverse cardiac and cerebrovascular events (HR, 1.73 [95% CI, 1.63-1.83], P<0.001) and health resource use including rehospitalization (HR, 1.09 [95% CI, 1.05-1.14], P<0.001) and increased postacute care service use (HR, 1.56 [95% CI, 1.32-1.85], P<0.001). Cardiology clinic visits within 30 days of hospital admission were associated with lower 90-day and 1-year all-cause mortality (odds ratio [OR], 0.62 [95% CI, 0.49-0.78], P<0.001; OR, 0.69 [95% CI, 0.58-0.83], P<0.001, respectively). CONCLUSIONS: HF hospitalization is associated with increased risk of mortality and morbidity with high health resource use in patients with ACHD. Recent cardiology clinic attendance appears to mitigate these risks.