Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights.

Background: The Quality Enhancement Research Initiative (QuERI) in adults with congenital heart disease (ACHD) was developed to improve detection of pulmonary arterial hypertension (PAH) after repair of systemic-to-pulmonary arterial shunt lesions.

Objectives: This study sought to standardize use of accepted criteria for PAH diagnosis and evaluate utility in at-risk patients with ACHD.

Methods: Patients ≥18 years of age with ACHD repaired ≥1 year before enrollment and with additional risk factors for developing PAH were eligible.

Palliative Care in Adult Congenital Heart Disease-Associated Advanced Heart Disease.

The practice of palliative care (PC) strives to mitigate patient suffering through aligning patient priorities and values with care planning and via improved understanding of complex physical, psychosocial, and spiritual stressors and dynamics that contribute to patient-centered outcomes. Through representative 'case examples' and supportive data, the role and value of a PC consultant, within the framework of a comprehensive adult congenital heart disease (ACHD) team caring for ACHD with advanced heart disease, are reviewed.

Feasibility of frailty screening among patients with advanced heart failure.

Background: Frailty is common among patients with advanced heart failure (HF), and screening for frailty to guide care is recommended. Although multiple tools are available to screen for frailty, the feasibility of routinely incorporating frailty screening into daily clinical practice among hospitalised advanced HF patients has not been rigorously tested.

Methods: This was a prospective, single-centre, quality improvement study.

Top Ten Tips Palliative Care Clinicians Should Know About Delivering Specialty-Aligned Palliative Care.

Specialty-aligned palliative care (SAPC) refers to interprofessional palliative care (PC) that is delivered to a specific population of patients in close partnership with other primary or specialty clinicians. As evolving PC models address physical, psychosocial, and spiritual suffering across illnesses and settings, PC clinicians must acquire advanced knowledge of disease-specific symptoms, common treatments, and complications that impact prognosis and outcomes. The tips provided in this article draw on the experience and knowledge of interprofessional PC and other specialist clinicians from diverse institutions across the United States who have developed and studied SAPC services across different disease groups.

Specialty-Aligned Palliative Care: Responding to the Needs of a Tertiary Care Health System.

Background: Expanding specialty palliative care within complex health systems involves consideration of patients' unmet needs, clinicians' perceptions of palliative care, and the availability of palliative care resources. Prior to this quality improvement (QI) project, palliative care services in our health system primarily served oncology patients.

Intervention: We undertook a prospective strategic planning process that included executive sponsorship and engagement of institutional leaders and clinicians to help define which palliative care services were most needed by the health system.

Serial cardiac biomarker assessment in adults with congenital heart disease hospitalized for decompensated heart failure.

Background: Biomarkers are increasingly part of assessing and managing heart failure (HF) in adults with congenital heart disease (CHD).

Objectives: To understand the response of cardiac biomarkers with therapy for acute decompensated heart failure (ADHF) and the relationship to prognosis after discharge in adults with CHD.

Design: A prospective, observational cohort study with serial blood biomarker measurements.

Associations Between Clinical Outcomes and a Recently Proposed Adult Congenital Heart Disease Anatomic and Physiological Classification System.

Background American Heart Association and American College of Cardiology consensus guidelines introduce an adult congenital heart disease anatomic and physiological (AP) classification system. We assessed the association between AP classification and clinical outcomes. Methods and Results Data were collected for 1000 outpatients with ACHD prospectively enrolled between 2012 and 2019.

Management of acute cardiovascular complications in pregnancy.

The growing population of women with heart disease of reproductive age has been associated with an increasing number of high-risk pregnancies. Pregnant women with heart disease are a very heterogeneous population, with different risks for maternal cardiovascular, obstetric, and foetal complications. Adverse cardiovascular events during pregnancy pose significant clinical challenges, with uncertainties regarding diagnostic and therapeutic approaches potentially compromising maternal and foetal health.

Tuning and external validation of an adult congenital heart disease risk prediction model.

Aims: Adequate risk prediction can optimize the clinical management in adult congenital heart disease (ACHD). We aimed to update and subsequently validate a previously developed ACHD risk prediction model.

Methods And Results: A prediction model was developed in a prospective cohort study including 602 moderately or severely complex ACHD patients, enrolled as outpatients at a tertiary centre in the Netherlands (2011-2013).

Long-Term Fate of the Truncal Valve.

Background Long-term survival in patients with truncus arteriosus is favorable, but there remains significant morbidity associated with ongoing reinterventions. We aimed to study the long-term outcomes of the truncal valve and identify risk factors associated with truncal valve intervention. Methods and Results We retrospectively reviewed patients who underwent initial truncus arteriosus repair at our institution from 1985 to 2016.

Interobserver agreement of the anatomic and physiological classification system for adult congenital heart disease.

Unlabelled: The Anatomic and Physiological (AP) classification system proposed in the 2018 American College of Cardiology/American Heart Association adult congenital heart disease (ACHD) guidelines assigns 2 dimensions to each patient: anatomic class (AnatC) and physiological stage (PhyS). This approach has not been tested in practice; we assessed interrater reliability and identified sources of disagreement.

Methods: Consensus definitions for AP categories were developed with input from 4 experts.

Advanced Heart Failure Therapies for Adults With Congenital Heart Disease: JACC State-of-the-Art Review.

In the contemporary era, nearly 85% of children with congenital heart disease will reach adulthood. Despite optimal medical and surgical treatment, many will experience a progressive decline in cardiopulmonary function leading to advanced heart failure. These patients present unique anatomic and physiological challenges to the care team, and unlike adults with acquired heart disease who progress to severe heart failure, advanced treatment options such as mechanical circulatory support and cardiac transplant may be limited.

Standardized outcomes in reproductive cardiovascular care: The STORCC initiative.

Background: Validated protocols for diagnostic testing and management of pregnant women with cardiovascular disease (CVD) do not exist. Our objective was to establish a prospective standardized protocol for the clinical evaluation of pregnant women with CVD.

Methods: The Standardized Outcomes in Reproductive Cardiovascular Care (STORCC) initiative prospectively enrolled pregnant women with CVD into a standardized diagnostic testing and assessment protocol.

Reducing radiation dose from myocardial perfusion imaging in subjects with complex congenital heart disease.

Introduction: The prevalence of defects and effective radiation dose from various myocardial perfusion imaging (MPI) strategies in congenital heart disease (CHD) is unknown.

Methods: We studied 75 subjects with complex CHD (ages 5 to 80 years) referred for MPI between 2002 and 2015. A rest and exercise or pharmacologic stress MPI was performed using Technetium sestamibi, rubidium or N-ammonia, and Sodium iodide SPECT (single-photon emission computed tomography), SPECT/CT or Cadmium zinc telluride (CZT) SPECT or PET (positron emission tomography)/CT scanners.

Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease.

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption.

Planned vaginal delivery and cardiovascular morbidity in pregnant women with heart disease.

Background: Although consensus guidelines on the management of cardiovascular disease in pregnancy reserve cesarean delivery for obstetric indications, there is a paucity of data to support this approach.

Objective: The objective of the study was to compare cardiovascular and obstetric morbidity in women with cardiovascular disease according to the plan for vaginal birth or cesarean delivery.

Study Design: We assembled a prospective cohort of women delivering at an academic tertiary care center with a protocolized multidisciplinary approach to management of cardiovascular disease between September 2011 and December 2016.

Disparities in Wait-List Outcomes for Adults With Congenital Heart Disease Listed for Heart Transplantation Before and Since Revision of Status I Listing.

Medical and surgical advances in the late 20th century that allowed the majority of children born with congenital heart disease (CHD) to survive have resulted in a shift of the burden of morbidity and mortality into adulthood. Heart failure is the leading cause of death in adults with CHD. This study aimed to address the gap in understanding of outcomes of adults with CHD who have heart failure and are listed for heart transplantation compared with those without CHD.

Relationship of Red Cell Distribution Width to Adverse Outcomes in Adults With Congenital Heart Disease (from the Boston Adult Congenital Heart Biobank).

Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.

A Pilot Study of Inspiratory Muscle Training to Improve Exercise Capacity in Patients with Fontan Physiology.

While the Fontan procedure has improved life expectancy, patients with single ventricle physiology have impaired exercise capacity due to limited increase in pulmonary blood flow during activity. Enhancing the "thoracic pump" using inspiratory muscle training (IMT) may ameliorate this impairment. Adult nonsmokers with Fontan physiology were recruited through Boston Children's Hospital's outpatient clinic.