Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows: While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

Shared decision making in cardiology: a systematic review and meta-analysis.

OBJECTIVES: To evaluate the effectiveness of interventions to improve shared decision making (SDM) in cardiology with particular focus on patient-centred outcomes such as decisional conflict. METHODS: We searched Embase (OVID), the Cochrane library, PubMed and Web of Science electronic databases from inception to January 2021 for randomised controlled trials that investigated the effects of interventions to increase SDM in cardiology. The primary outcomes were decisional conflict, decisional anxiety, decisional satisfaction or decisional regret; a secondary outcome was knowledge gained by the patients. RESULTS: Eighteen studies which reported on at least one outcome measure were identified, including a total of 4419 patients. Interventions to increase SDM had a significant effect on reducing decisional conflict (standardised mean difference (SMD) -0.211, 95% CI -0.316 to -0.107) and increasing patient knowledge (SMD 0.476, 95% CI 0.351 to 0.600) compared with standard care. CONCLUSIONS: Interventions to increase SDM are effective in reducing decisional conflict and increasing patient knowledge in the field of cardiology. Such interventions are helpful in supporting patient-centred healthcare and should be implemented in wider cardiology practice.

Cardiopulmonary resuscitation.

Performing cardiopulmonary resuscitation is a key competency for healthcare professionals. Training in immediate and advanced life support is a requirement for UK doctors; this is depicted in the Foundation training programme competencies and in the Internal Medicine Training curriculum. It requires being able to identify unwell patients, perform a structured assessment and treatment approach, master relevant procedural aspects and demonstrate non-technical skills including leading the resuscitation team. The Resuscitation Council UK has recently provided updated guidance on basic and advanced life support. These guidelines align with similar international guidelines, taking into account evidence from clinical trials of cardiac arrest management and national data on cardiac arrest outcomes in the community and in the hospital. The guidance includes considerations regarding individuals with suspected or confirmed coronavirus disease (COVID-19). The complex ethical aspects around escalation of care, advance care planning, 'Do Not Attempt Cardiopulmonary Resuscitation' decisions and communication with patients and their loved ones are also discussed. This chapter summarizes the current guidance on cardiopulmonary resuscitation.

ST-elevation myocardial infarction in a young patient with Duchenne's muscular dystrophy: a case report.

Background: Duchenne's muscular dystrophy (DMD) is an X-linked muscular disease which is caused by the absence of dystrophin. This results in the death of muscle cells and cardiomyocytes and consequent substitution by fat and fibrous tissue. The clinical translation of this is muscle weakness and cardiomyopathy. We report on the case of a young patient with dilated cardiomyopathy on a background of DMD who developed ST-elevation myocardial infarction (STEMI). Case summary: A 19-year-old male patient with DMD, known dilated cardiomyopathy, and no risk factors for ischaemic heart disease presented with central crushing chest pain. His electrocardiogram revealed anterior ST elevation. His angiogram revealed distally occluded left anterior descending and second diagonal branch with no evidence of underlying coronary artery disease. He was treated with balloon angioplasty. An echocardiogram raised the suspicion of a left ventricular thrombus, and the mechanism of STEMI was felt to be embolism from the left ventricular thrombus on a background of dilated cardiomyopathy in the context of DMD. The patient was treated with anticoagulants (warfarin). On a repeat echocardiogram a few months later, the thrombus had resolved. At 3 years of follow-up, the patient did not present any more embolic events. Discussion: To our knowledge, this is the first case of STEMI secondary to thrombotic coronary occlusion that has been described in a patient with DMD. This case highlights an unusual complication of DMD. Based on this case, we discuss the dilemmas in the management and follow-up of this complex patient population.

Exercise tolerance and quality of life in patients with known or suspected coronary artery disease.

BACKGROUND: Coronary artery disease (CAD) is known to impact on patients' physical and mental health. The relationship between performance on treadmill exercise tolerance test (ETT) and health-related quality of life (HRQL)has never been specifically investigated in the setting of CAD. METHODS: Consecutive patients undergoing an ETT with the Bruce protocol during a diagnostic workup for CAD (n = 1,631, age 55 ± 12 years) were evaluated. Exercise-related indices were recorded. Detailed information on cardiovascular risk factors and past medical history were obtained. HRQLwas assessed with the use of the validated 36-Item Short Form Survey (SF-36) questionnaire. RESULTS: Increasing age and the presence of cardiovascular risk factors and comorbidities correlated with lower scores on the physical and mental health component of SF-36(all P < 0.05). Subjects with arrhythmias during exercise and slow recovery of systolic blood pressure had lower scores on the physical health indices or the Social Role Functioning component (P < 0.05). Achieved target heart rate and good exercise tolerance were independently associated with better scores of the physical and mental health domains of SF-36 and overall HRQLscores (ß = 0.05 for target HR and PCS-36, ß = 1.86 and ß = 1.66 per increasing stage of exercise tolerance and PCS-36 and MCS-36, respectively, P < 0.001 for all associations). Ischemic ECG changes were associated with worse scores on Physical Functioning (ß = - 3.2, P = 0.02) and Bodily Pain (ß = - 4.55, P = 0.026). CONCLUSION: ETT parameters are associated with HRQL indices in patients evaluated for possible CAD. Physical conditioning may increase patient well-being and could serve as a complementary target in conjunction with cardiovascular drug therapy.

Multi-Modality Imaging in Dilated Cardiomyopathy: With a Focus on the Role of Cardiac Magnetic Resonance.

Heart failure (HF) is recognized as a leading cause of morbidity and mortality worldwide. Dilated cardiomyopathy (DCM) is a common phenotype in patients presenting with HF. Timely diagnosis, appropriate identification of the underlying cause, individualized risk stratification, and prediction of clinical response to treatment have improved the prognosis of DCM over the last few decades. In this article, we reviewed the current evidence on available imaging techniques used for DCM patients. In this direction, we evaluated appropriate scenarios for the implementation of echocardiography, nuclear imaging, and cardiac computed tomography, and we focused on the primordial role that cardiac magnetic resonance (CMR) holds in the diagnosis, prognosis, and tailoring of therapeutic options in this population of special clinical interest. We explored the predictive value of CMR toward left ventricular reverse remodeling and prediction of sudden cardiac death, thus guiding the decisions for device therapy. Principles underpinning the use of state-of-the-art CMR techniques such as parametric mapping and feature-tracking strain analysis are also provided, along with expectations for the anticipated future advances in this field. We also attempted to correlate the evidence with clinical practice, with the intent to address questions on selecting the optimal imaging method for different indications and clinical needs. Overall, we recommend a comprehensive assessment of DCM patients at baseline and at follow-up intervals depending on the clinical status, with the addition of CMR as a second-line modality to other imaging techniques. We also provide an algorithm to guide the detailed imaging approach of the patient with DCM. We expect that future guidelines will upgrade their clinical recommendations for the utilization of CMR in DCM, which is expected to further improve the quality of care and the outcomes. This review provides an up-to-date perspective on the imaging of dilated cardiomyopathy patients and will be of clinical value to training doctors and physicians involved in the area of heart failure.

Cardiovascular Disease in Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA), is a term used to describe a group of inflammatory disorders beginning before the age of 16 years. Although for the majority of children remission is achieved early, those with systemic or polyarticular form of the disease may present persistent symptoms in adulthood. Considering that there is overlap in the pathogenesis of JIA with adult rheumatic diseases, concerns have been raised as to whether JIA patients could be at increased cardiovascular (CV) risk in the long-term. In this review, we summarize evidence for CV involvement in JIA and present data on CV risk factors and surrogate markers of arterial disease. We also provide information on beneficial and harmful CV effects of anti-inflammatory medications in the context of JIA and suggest strategies for CV screening. Overall, patients with systemic forms of JIA demonstrate an adverse lipid profile and early arterial changes relevant to accelerated arterial disease progression. Although there is paucity of data on CV outcomes, we recommend a holistic approach in the management of JIA patients, which includes CV risk factor monitoring and lifestyle modification as well as use, when necessary, of antiinflammatory therapies with documented CV safety.

A concealed carcinoid cardiac metastasis uncovered by comprehensive cardiovascular magnetic resonance-based tissue characterization: a case report.

BACKGROUND: Cardiac metastases of carcinoid tumours are extremely rare, and their diagnosis poses a significant challenge. A variety of techniques has been reported in the literature for this purpose, ranging from echocardiogram to the Indium-111 Octreotide, positron emission tomography using specific tracers, and biopsy. Occasionally, the diagnosis is only made post-mortem. Recently, CMR (cardiovascular magnetic resonance) has been added to the diagnostic toolkit. This case report describes the CMR sequences that can be used to characterize cardiac metastases of carcinoid tumours. CASE SUMMARY: A 55-year-old woman with an antecedent history of resected carcinoid tumour of the ileocecal junction underwent whole-body In-111 Octreoscan single-photon emission computed tomography in the context of her follow-up. This raised the suspicion of pericardial involvement, which prompted a CMR study. Comprehensive CMR findings were consistent with isolated carcinoid tumour metastasis embedded within the anterior papillary muscle. We describe the CMR sequences that were used to characterize the metastasis. DISCUSSION: The rarity of cardiac metastasis of carcinoid tumour makes its diagnosis challenging and warrants a high level of clinical suspicion. Cardiovascular magnetic resonance imaging proves to be an indispensable tool in the tissue characterization of such tumours.