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Background d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with unrepaired cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma. Case summary A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew Streptococcus mitis-oralis and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed normal intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation. Discussion A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient’s unique cardiac haemodynamics and after shared decision-making with the patient.

A 79-year-old woman presents with recurrent pericardial and pleural effusions for several years. She was noted to have exudative pleural effusions and bilateral nailbed discoloration. The constellation of her presenting symptoms and existing physical examination findings revealed a diagnosis of yellow nail syndrome, a rare cause of recurrent pericardial effusions. (Level of Difficulty: Advanced.)

ObjectiveChylopericardium (CPE) is a rare condition associated with accumulation of triglyceride-rich chylous fluid in the pericardial cavity. Due to minimal information on CPE within the literature, we conducted a systematic review of all published CPE cases to understand its clinical characteristics, management and outcomes.MethodsWe performed a literature search and identified cases of patients with CPE from 1946 until May 2021 in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We identified relevant articles for pooled analyses of clinical, diagnostic and outcome data.ResultsA total of 95 articles with 98 patients were identified. Patient demographics demonstrated male predominance (55%), with a mean age of 37±15 years. Time from symptom onset to diagnosis was 5 (Q1 4.5, Q3 14) days, with 74% of patients symptomatic on presentation. Idiopathic CPE (60%) was the most common aetiology. Cardiac tamponade secondary to CPE was seen in 38% of cases. Pericardial fluid analysis was required in 94% of cases. Lymphangiography identified the leakage site in 59% of patients. Medical therapy (total parenteral nutrition, medium-chain triglycerides or octreotide) was undertaken in 63% of cases. In our cohort, 32% progressed towards surgical intervention. During a median follow-up of 180 (Q1 180, Q3 377) days, CPE recurred in 16% of cases. Of the patients with recurrence, 10% were rehospitalised.ConclusionCPE tends to develop in younger patients and may cause serious complications. Many patients fail medical therapy, thereby requiring surgical intervention. Although overall mortality is low, associated morbidities warrant close follow-up and possible reintervention and hospitalisations.

Systemic autoimmune diseases are an important cause of pericardial involvement and contribute to up to ∼22% cases of pericarditis with a known aetiology. The underlying mechanism for pericardial involvement varies with each systemic disease and leads to a poor understanding of its management. Multimodality imaging establishes the diagnosis and determines the type and extent of pericardial involvement. In this review, we elaborate upon various pericardial syndromes associated with different systemic autoimmune and autoinflammatory diseases and the multitude of imaging modalities that can be used to further characterize autoimmune pericardial involvement. Lastly, these forms of pericarditis have a greater likelihood of recurrence, and clinicians need to understand their unique treatment approaches to improve patient outcomes.

Heart failure with preserved ejection fraction (HFpEF) affects half of all patients with heart failure. While previously neglected, the right ventricle (RV) has sparked interest in recent years as a means for better understanding this condition and as a potential therapeutic target.Right ventricular dysfunction (RVD) is present in 4%–50% of patients with HFpEF. The RV is intimately connected to the pulmonary circulation, and pulmonary hypertension is commonly implicated in the pathophysiology of RVD. The development of RVD in HFpEF may also be driven by comorbidities, such as chronic obstructive pulmonary disease, obesity, obstructive sleep apnoea and atrial fibrillation. The evaluation of RVD is particularly challenging due to anatomical and structural factors, as well as unique physiological characteristics of this chamber like load and interventricular dependency. Fractional area change, tricuspid annular plane systolic excursion and tricuspid annular systolic velocity are commonly used measurements of RV function. Speckle tracking echocardiography and cardiac magnetic resonance (CMR) are also gaining attention as important tools for the assessment of RV structure, fibre deformation and systolic performance. Further research is needed to confirm the utility and prognostic significance of RV [18F]fluorodeoxyglucose (FDG) positron emission tomography imaging as FDG accumulation is suggested to increase with progressive RVD. Targeted pharmacotherapy with phosphodiesterase inhibitors, guanylate–cyclase stimulators, nitrates and inhaled inorganic nitrites have yet to demonstrate improvement in RVD, compelling the need for evaluation and discovery of novel pharmacological interventions for this entity.

Background Myocardial infarction with non-obstructive coronary arteries is a working diagnosis for several heart disorders. Previous studies on anxiety and depression in patients with myocardial infarction with non-obstructive coronary arteries are lacking. Our aim was to investigate the prevalence of anxiety and depression among patients with myocardial infarction with non-obstructive coronary arteries. Methods We included 99 patients with myocardial infarction with non-obstructive coronary arteries together with age- and sex-matched control groups who completed the Beck Depression Inventory and the Hospital Anxiety and Depression Scale (HADS) 3 months after the acute event. Results Using the Beck Depression Inventory, we found that the prevalence of depression in patients with myocardial infarction with non-obstructive coronary arteries (35%) was higher than in healthy controls (9%; P = .006) and similar to that of patients with coronary heart disease (30%; P = .954). Using the HADS anxiety subscale, we found that the prevalence of anxiety in patients with myocardial infarction with non-obstructive coronary arteries (27%) was higher than in healthy controls (9%; P = .002) and similar to that of patients with coronary heart disease (21%; P = .409). Using the HADS depression subscale, we found that the prevalence of depression in patients with myocardial infarction with non-obstructive coronary arteries (17%) was higher than in healthy controls (4%; P = .003) and similar to that of patients with coronary heart disease (13%; P = .466). Patients with myocardial infarction with non-obstructive coronary arteries and takotsubo syndrome scored higher on the HADS anxiety subscale than those without (P = .028). Conclusions This is the first study on the mental health of patients with myocardial infarction with non-obstructive coronary arteries to show that prevalence rates of anxiety and depression are similar to those in patients with coronary heart disease.