AHA/ACC vs ESC Guidelines for Management of Adults With Congenital Heart Disease: JACC Guideline Comparison.

The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Barriers and Facilitators of Palliative Care and Advance Care Planning in Adults With Congenital Heart Disease.

Palliative care has potential to improve quality of life and goal-concordant care for patients with adult congenital heart disease (ACHD). However, it is rarely employed prior to critical illness because the best methods for implementation are not well-defined. We qualitatively evaluated ACHD patients' understanding of and opinions regarding palliative care and advance care planning (ACP) to better define the needs of this population. We conducted a thematic analysis of 25 semistructured interviews with patients with ACHD in which we assessed participants' perspectives on the need for, and barriers and facilitators to, the use of palliative care and ACP. In a group of participants with ACHD (mean age 38, 48% male) classified as simple (24%), moderate (32%), or complex (44%), we identified 4 major themes: (1) using knowledge to combat future uncertainties; (2) unfamiliarity with and limited exposure to palliative care and ACP; (3) facilitators and barriers to engaging in palliative care and ACP; and (4) importance of timing and presentation of ACP discussions. In conclusion, participants expressed a desire for knowledge about ACHD progression and treatment. They supported routine incorporation of palliative care and ACP and identified related facilitators and barriers to doing so. Importantly, timing and format of these discussions must be individualized using shared decision-making between clinicians, patients, and their families.

Echocardiographic evaluation of univentricular physiology and cavopulmonary shunts.

The term univentricular heart (UH) defines complex congenital heart disease that lacks a pulmonic ventricular chamber either in the original anatomy or the final palliation. The prevalence of patients with this type of physiology continues to increase due to improved surgical palliative procedures. This review will outline the physiology, complications, and echocardiographic approach to image patients with palliated UH.

Age at Fontan procedure impacts exercise performance in adolescents: results from the Pediatric Heart Network Multicenter study.

BACKGROUND: Most single ventricle patients undergo Fontan procedure in a staged manner. However, optimal timing of Fontan completion after an intermediate staging surgery is controversial. Therefore, we investigated the long-term impact of age at Fontan completion on the exercise performance in adolescents. METHODS: We analyzed National Institutes of Health/National Heart, Lung and Blood Institute Pediatric Heart Network Fontan Cross-Sectional Study dataset consisting of children and adolescents 6 to 18 years of age recruited in 2003 to 2004. Multivariate linear regression techniques were used to evaluate association of age at Fontan procedure with percent predicted VO2 maximum, percent predicted maximum O2 pulse, and heart rate reserve in patients who achieved ventilatory anaerobic threshold (VAT). RESULTS: Of the 405 patients who had undergone only one Fontan operation and ramp cycle ergometry, 72% had prior intermediate surgery. Mean age at Fontan completion and exercise testing was 3.4±2 and 12.4±3.2 years. Three hundred twelve patients reached VAT suggesting adequate cardiopulmonary effort. In patients who reached VAT, each year increase in age at Fontan completion was associated with a decline of 1.5 (95% CI -2.5 to -0.5) points in percent-predicted VO2 maximum and a decline of 4.1 (95% CI -6.0 to -2.1) beat/min in heart rate reserve after adjusting for all pertinent variables. CONCLUSIONS: Fontan completion at a younger age is associated with better exercise performance in adolescents.

Recurrent atrioventricular groove intramuscular arteriovenous malformation.

A 17-year-old patient presented with a recurrent right atrioventricular (AV) groove vascular tumor. The tumor was resected en bloc, including the AV groove extending into the right ventricle (RV) and tricuspid valve. The AV groove and tricuspid valve required extensive reconstruction. The histopathologic features were that of an arteriovenous malformation with a proliferative small vessel component--an extremely rare type of cardiovascular anomaly.

Acute dyspnea in a young man with an old sternotomy.

Described is a 42-year-old man with complex congenital heart disease with single-ventricle physiology who underwent a Fontan operation at 27 years of age. He presented with sudden-onset chest pain and dyspnea and was initially misdiagnosed with a pulmonary embolism. This case is described in order to highlight the challenges in the evaluation of common presentations in adults with congenital heart disease.

Management of acute regurgitation in left-sided cardiac valves.

The management of acute, severe cardiac valvular regurgitation requires expeditious multidisciplinary care. Although acute, severe valvular regurgitation can be a true surgical emergency, accurate diagnosis and subsequent treatment decisions require clinical acumen, appropriate imaging, and sound judgment. An accurate and timely diagnosis is essential for successful outcomes and requires appropriate expertise and a sufficiently high degree of suspicion in a variety of settings. Whereas cardiovascular collapse is the most obvious and common presentation of acute cardiac valvular regurgitation, findings may be subtle, and the clinical presentation can often be nonspecific. Consequently, other acute conditions such as sepsis, pneumonia, or nonvalvular heart failure may be mistaken for acute valvular regurgitation. In comparison with that of the right-sided valves, regurgitation of the left-sided valves is more common and has greater clinical impact. Therefore, this review focuses on acute regurgitation of the aortic and mitral valves.