Implantable pulmonary artery pressure monitoring device in patients with palliated congenital heart disease: Technical considerations and procedural outcomes.

OBJECTIVE: We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). BACKGROUND: Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. METHODS: We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. RESULTS: CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. CONCLUSIONS: Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

Novel uses for implanted haemodynamic monitoring in adults with subaortic right ventricles.

BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with subaortic right ventricles (sRVs), echocardiography is a poor screening tool for PH; implantable invasive haemodynamic monitoring (IHM) could be used for this purpose, but data are limited. The aim of this study is to report on novel uses of IHM in patients with sRV. METHODS: This retrospective study describes the uses of IHM, impact of IHM on heart failure hospitalisation (HFH) and device-related complications in adults with sRV from a single centre (2015-2022). RESULTS: IHM was placed in 18 patients with sRV (median age 43 (range 30-54) years, 8 female, 16 with D-TGA/AS, 2 with ccTGA); 16 had moderate or severe sRV systolic dysfunction, 13 had PH on catheterisation. IHM was used for (1) Medical therapy titration, (2) Medical management after ventricular assist device in patients with transplant-limiting PH and (3) Serial monitoring of pulmonary artery pressures without repeat catheterisations to help identify the optimal time for heart transplant referral. In follow-up (median 23 months), HFHs/year were similar to the year prior to IHM (median 0 (IQR 0-1.0) before vs 0 (0-0.8) after, p=0.984). Device migration occurred in one, without long-term sequelae. CONCLUSIONS: Uses of IHM in patients with sRV are described which may minimise the need for serial catheterisations in a population where PH is prevalent. HFHs were low overall but not impacted by IHM. One device-related complication occurred without long-term consequence.

Early Experience and Lessons Learned Using Implanted Hemodynamic Monitoring in Patients With Fontan Circulation.

BACKGROUND: Data on the use of implanted hemodynamic monitoring (IHM) in patients with Fontan circulation are limited. This study reports our experience using the CardioMEMS HF system in adults with Fontan circulation. METHODS AND RESULTS: This single-center, retrospective study evaluated heart failure hospitalizations, procedural complications, and device-related complications in patients with Fontan circulation referred for IHM placement (2015-2022). The association of pulmonary artery pressure (by most recent catheterization and median IHM pressure within 30 days of placement) with both death and follow-up Model for End-Stage Liver Disease Excluding International Normalized Ratio score were evaluated. Of 18 patients referred for IHM placement, 17 were successful (median age, 30 [range 21-48] years, 6 women). Procedural complications (access site hematomas, pulmonary artery staining) occurred in 3 patients, without device-related procedural complications. In follow-up (median, 35 [range, 6-83] months), 1 patient developed a pulmonary embolism (possibly device-related). Heart failure hospitalizations/year were similar before and after IHM (median, 1 [interquartile range, 0-1.0] versus 0.6 [0-2.3]; P=0.268), though only 46% of heart failure hospitalizations had associated IHM transmissions. IHM pressures were associated with Model for End-Stage Liver Disease Excluding International Normalized Ratio scores (R2=0.588, P<0.001), though catheterization pressures were not (R2=0.140, P=0.139). The long-term mortality rate was 53% in this cohort. On unadjusted survival analysis, IHM pressures ≥18 mm Hg were associated with mortality (log rank P=0.041), which was not reproduced with catheterization pressures (log rank P=0.764). CONCLUSIONS: In patients with Fontan circulation, IHM did not reduce heart failure hospitalizations, though patient adherence to transmission was low. Device-related complications were low. IHM pressures may better represent real-life conditions compared with catheterization given associations with mortality and Model for End-Stage Liver Disease Excluding International Normalized Ratio score.

Patient Preference and Perception of Care Provided by Advance Nurse Practitioners and Physicians in Outpatient Adult Congenital Clinics.

OBJECTIVE: Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic. DESIGN: A prospective study conducted in two outpatient adult CHD clinics to assess patient satisfaction through standardized surveys. Demographic data included CHD diagnosis, age, and New York Heart Association functional class. All patients completed a Short-Form-12 to assess health status. RESULTS: Of the 371 patients recruited (52% men; median age 29 years, severe CHD complexity 31.5%) and seen by NP (n = 187) or physician (n = 184), physician-managed practices had higher perceived: overall experience, courtesy of provider (P < 0.05) and confidence (trust) in provider (P < 0.1). Overall, patients reported satisfaction with an NP providing care (98%), the NP was able to effectively deal with illness/CHD (95%), and an increased chance (94%) or willingness to see an NP at a future visit. Only 73% reported an understanding of NP training and how an NP differed from a registered nurse. There was a stronger perception of how an NP differed from a physician (83%). CONCLUSION: Patient satisfaction was high regardless of whether care was provided by physicians or NPs. However, patients appear to make distinctions in what they believe the type of care each practitioner is best at providing. Patient education regarding competence of the different health care providers may continue to improve patient satisfaction.

Assessment of the cerebral circulation in adults with coarctation of the aorta.

AIMS: There is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted. The purpose of our study was to prospectively perform CTA of the intracranial vessels in adults with CoA to evaluate the prevalence and identify high-risk features of this complication. METHODS AND RESULTS: From January 2008 to February 2011, adults ≥18 years of age with CoA were prospectively enrolled in a screening program with CTA of the intracranial vessels. Analyses of prognostic variables were performed with both Fisher's exact and two sample t-test. Forty-three patients (58% female, 33.55 ± 10.21 years) with CoA completed CTA of the intracranial vessels. Five patients (11%) were found to have IA. Patients with IA were older than those without (45.6 ± 8.17 vs. 30.89 ± 7.89, P = 0.0003). There were no statistically significant differences detected between measurements of fasting lipid profiles, C-reactive protein, brain natriuretic peptide, and homocysteine levels among CoA patients with and without IA (P = not significant). CONCLUSION: Prospective screening of adults with CoA confirmed the increased prevalence of IA but also identified increased age as the sole risk factor. These data suggested that screening is justified particularly in the fourth and fifth decades of life. Further studies are required that focus on the development, natural history, and treatment of IA.

Shock-related anxiety and sexual function in adults with congenital heart disease and implantable cardioverter-defibrillators.

BACKGROUND: An increasing number of adults with congenital heart disease (CHD) require implantable cardioverter-defibrillators (ICDs), yet little is known about their impact on psychological well-being and sexual function. OBJECTIVE: To assess shock-related anxiety in adults with CHD and its association with depression and sexual function. METHODS: A prospective, multicenter, cross-sectional study was conducted on adult patients with CHD with (ICD(+)) and without (ICD(-)) ICDs. The Florida Shock Anxiety Scale was administered to patients with ICD(+) and the Beck Depression Inventory-II to all patients. Men completed the Sexual Health Inventory for Men, and women completed the Female Sexual Function Index. RESULTS: A total of 180 adults with CHD (ICD(+): n = 70; ICD(-): n = 110; median age 32 years [interquartile range 27-40 years]; 44% women) were enrolled. The complexity of CHD was classified as mild in 32 (18%), moderate in 93 (52%), and severe in 54 (30%) subjects. In ICD recipients, a high level of shock-related anxiety was identified (Florida Shock Anxiety Scale score 16; interquartile range 12-23.5), which was slightly higher than the median score for ICD recipients in the general population (P = .057). A higher level of shock-related anxiety was associated with poorer sexual function scores in both men (Spearman's ρ =-.480; P<.001) and women (Spearman's ρ =-.512; P<.01). It was also associated with self-reported depressive symptomatology (Spearman's ρ = .536; P< .001). CONCLUSIONS: Adults with CHD and ICDs demonstrate a high level of shock-related anxiety, which is associated with lower sexual functioning scores in men and women. These results underscore the need for increased clinical attention related to ICD-related shock anxiety and impaired sexual function in this population.

Quality outcomes of ACHD patients undergoing cardiovascular procedures and hospital admissions in a free-standing children's hospital.

OBJECTIVES: Describe quality outcomes of adults with congenital heart disease (ACHD) undergoing cardiovascular procedures and admissions in a free-standing children's hospital with a multi-disciplinary ACHD program and compared with pediatric outcomes. BACKGROUND: A challenge for the U.S. healthcare system is where to treat the >1 million ACHD patients (pts): adult hospitals without CHD care, or pediatric hospitals without adult services. METHODS: We reviewed all CHD pts ≥ 18 yrs of age from 2002-2007. Procedural and hospital related morbidity and mortality were recorded. ANOVA and t-test compared adult with pediatric pts. RESULTS: Overall, 782 pts, mean age of 29.8 ± 9.9 yrs, encountered 1490 procedures/admissions. For 178 cardiac surgeries (72% reoperations), mortality was 1.8% and complication rate was 7.3%. There was 0% mortality for 412 cardiac catheterizations, 311 electrophysiological procedures, 401 transesophageal echocardiograms (TEE), 244 exercise tests (ETT) and 54 medical admissions. Major adverse event rate was 0.6% for cardiac catheterization and electrophysiological procedures. No adverse events occurred during TEE and ETT. Only 4 pts required transfer to an adult institution (0.25%). There was no significant difference in mortality or adverse events between pediatric and adult CHD pts, p>0.05. CONCLUSIONS: The optimal setting to provide ACHD care remains a complex issue. Our study is the first to demonstrate 1) a low incidence of morbidity and mortality for ACHD pts undergoing cardiovascular procedures or admissions at a free-standing children's hospital, 2) outcomes comparable to pediatric CHD pts. Future models incorporating ACHD programs within pediatric heart centers should be considered to care for this complex population.