Optimizing patient care and outcomes through the congenital heart center of the 21st century

Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a stateâ ofâ theâ art congenital heart center of the 21st century. Care center collaboration helps overcome the barrier of relative small volumes at any given program. Patients and families have become active collaborative partners with care centers in the definition of acute and longitudinal outcomes and our quality metrics. Understanding programmatic metrics that create an environment to provide outstanding congenital heart care will allow centers to improve their structure, processes and ultimately outcomes, leading to an increasing number of centers that provide excellent care. This manuscript provides background, as well listing of proposed specialty domain quality metrics for centers, and thus serves as an updated baseline for the ongoing dynamic process of optimizing care and realizing patient value.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/143653/1/chd12575_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/143653/2/chd12575.pd

Hypoplastic Left Heart Syndrome Current Considerations and Expectations

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients

The NANOGrav 15-year Data Set: Evidence for a Gravitational-Wave Background

We report multiple lines of evidence for a stochastic signal that is correlated among 67 pulsars from the 15-year pulsar-timing data set collected by the North American Nanohertz Observatory for Gravitational Waves. The correlations follow the Hellings-Downs pattern expected for a stochastic gravitational-wave background. The presence of such a gravitational-wave background with a power-law-spectrum is favored over a model with only independent pulsar noises with a Bayes factor in excess of $10^{14}$, and this same model is favored over an uncorrelated common power-law-spectrum model with Bayes factors of 200-1000, depending on spectral modeling choices. We have built a statistical background distribution for these latter Bayes factors using a method that removes inter-pulsar correlations from our data set, finding $p = 10^{-3}$ (approx. $3\sigma$) for the observed Bayes factors in the null no-correlation scenario. A frequentist test statistic built directly as a weighted sum of inter-pulsar correlations yields $p = 5 \times 10^{-5} - 1.9 \times 10^{-4}$ (approx. $3.5 - 4\sigma$). Assuming a fiducial $f^{-2/3}$ characteristic-strain spectrum, as appropriate for an ensemble of binary supermassive black-hole inspirals, the strain amplitude is $2.4^{+0.7}_{-0.6} \times 10^{-15}$ (median + 90% credible interval) at a reference frequency of 1/(1 yr). The inferred gravitational-wave background amplitude and spectrum are consistent with astrophysical expectations for a signal from a population of supermassive black-hole binaries, although more exotic cosmological and astrophysical sources cannot be excluded. The observation of Hellings-Downs correlations points to the gravitational-wave origin of this signal.Comment: 30 pages, 18 figures. Published in Astrophysical Journal Letters as part of Focus on NANOGrav's 15-year Data Set and the Gravitational Wave Background. For questions or comments, please email [email protected]

Pulmonary vasodilators: beyond the bounds of pulmonary arterial hypertension therapy in COVID-19.

Pulmonary arterial hypertension (PAH) and novel coronavirus (SARS-CoV-2) disease COVID-19 are characterized by extensive endothelial dysfunction and inflammation leading to vascular remodeling and severe microthrombi and microvascular obliterative disease. It is hypothesized that those patients with underlying lung disease, like PAH, represent a high-risk cohort in this pandemic. However, reports of COVID-19 in this cohort of patient have been scaring and an observational survey showed that the disease was relatively well tolerated. We postulate that specific PAH vasodilator may offer some protection and/or advantage in the case of concomitant COVID-19. Here we review the literature describing mechanisms of action for each of the broad categories of PAH therapy, and offer potential hypothesis about why this therapy may impact outcomes in COVID-19

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