Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.

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. AP classification was assigned based on consensus definitions. Primary outcomes were (1) all-cause mortality and (2) a composite of all-cause mortality or nonelective cardiovascular hospitalization. Cox regression models were developed for AP classification, each component variable, and additional clinical models. Discrimination was assessed using the Harrell C statistic. Over a median follow-up of 2.5 years (1.4-3.9 years), the composite outcome occurred in 185 participants, including 49 deaths. Moderately or severely complex anatomic class (class II/III) and severe physiological stage (stage D) had increased risk of the composite outcome (AP class IID and IIID hazard ratio, 4.46 and 3.73, respectively, versus IIC). AP classification discriminated moderately between patients who did and did not suffer the composite outcome (C statistic, 0.69 [95% CI, 0.67-0.71]), similar to New York Heart Association functional class and NT-proBNP (N-terminal pro-B-type natriuretic peptide); it was more strongly associated with mortality (C statistic, 0.81 [95% CI, 0.78-0.84]), as were NT-proBNP and functional class. A model with AP class and NT-proBNP provided the strongest discrimination for the composite outcome (C statistic, 0.73 [95% CI, 0.71-0.75]) and mortality (C statistic, 0.85 [95% CI, 0.82-0.88]). Conclusions The addition of physiological stage modestly improves the discriminative ability of a purely anatomic classification, but simpler approaches offer equivalent prognostic information. The AP system may be improved by addition of key variables, such as circulating biomarkers, and by avoiding categorization of continuous variables.

Contraceptive Practices of Women With Complex Congenital Heart Disease.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking.

The care of adults with congenital heart disease across the globe: Current assessment and future perspective: A position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Exercise testing identifies patients at increased risk for morbidity and mortality following Fontan surgery.

OBJECTIVE: The objective of this study was to examine the relationship between exercise test data and mortality in patients who have had the Fontan procedure. DESIGN: The study was designed as a retrospective cohort study. SETTING: The study was set in a tertiary care center. PATIENTS: All study participants were Fontan patients ≥16 years old who had cardiopulmonary exercise tests at our institution between November 2002 and March 2010. The first exercise test with adequate effort during the study period was retained for analysis. We enrolled 146 patients at a median age of 21.5 years (16.0-51.6); 15.8 years (1.2-29.9) after Fontan surgery. OUTCOME MEASURES: The outcome measures were exercise test data (peak oxygen consumption, peak heart rate, etc.); mortality. RESULTS: Peak oxygen consumption averaged 21.2 ± 6.2 mL/kg/min, 57.1 ± 14.1% predicted. Follow-up data were collected 4.0 ± 2.0 years (range 0.3-7.7) after the exercise test. Sixteen patients (11%) died during follow-up; their peak oxygen consumption (16.3 ± 4.0 mL/kg/min) was significantly less than the survivors' (21.8 ± 6.2 mL/kg/min; P < .0001). Recursive partitioning and Cox proportional hazards modeling revealed that the hazard for death for patients with a peak oxygen consumption of <16.6 mL/kg/min was 7.5 (95% confidence interval: 2.6, 21.6; P < .0002) times that of patients with a higher peak oxygen consumption. Similarly, the hazard ratio for patients with peak-exercise heart rates of <122.5 bpm was 10.6 (3.0, 37.1; 0 < 0.0002). Data from exercise tests could also identify patients at increased risk for a combined morbidity/mortality end point. CONCLUSIONS: In adults with Fontan surgery, exercise test data can identify patients at increased risk of midterm morbidity and mortality.