ANCA-Associated Vasculitis with Cardiac Valve Vegetations in Two Teenage Males: A Case Report and Literature Review

Background: Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is a term used to describe systemic vasculitides that affect small and medium-sized blood vessels. The three types of ANCA-associated vasculitis (AAV) are Granulomatosis with Polyangiitis (GPA), formerly Wegener’s granulomatosis , Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly Churg-Strauss, with clinical presentation most frequently involving the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in children, with estimated prevalence of 3-4 per million, and even more rare is the manifestation of cardiac abnormalities secondary to ANCA-associated vasculitis in the pediatric population. Case Presentation: We present the cases of two teenage males who presented with cardiac valvular lesions secondary to Granulomatosis with Polyangiitis in addition to sinus, pulmonary, renal, and cutaneous involvement. These findings of cardiac valvular abnormalities in GPA have rarely been described in the literature in pediatrics. Both patients were treated with rituximab, high-dose methylprednisolone, and plasma exchange (PLEX) and showed improvement in their disease manifestations. Conclusions: A review of the literature revealed only five pediatric cases of ANCA-associated vasculitis with cardiac manifestations, and interestingly, three of the five had valvular involvement. Subsequent valvular involvement makes obtaining the diagnosis of ANCA-Associated Vasculitis very difficult due to concern for underlying infectious endocarditis and can lead to misdiagnosis given the rarity of cardiac involvement in ANCA-associated vasculitis. Routine echocardiogram is not always completed in newly diagnosed GPA, yet cardiac involvement can lead to severe consequences as was seen with our first patient in the form of thromboembolic stroke. We discuss the importance of keeping AAV on the differential when cardiac lesions are present as well as the importance of regular cardiac screening in newly diagnosed patients with AAV, as it is a major factor of cardiac morbidity and mortality in the adult population and can contribute substantially to management decisions.

Long-term outcomes following Rheumatic Heart Disease diagnosis in Australia

Aims Rheumatic Heart Disease (RHD) is a major contributor to cardiac morbidity and mortality globally. We aimed to estimate the probability and predictors of progressing to non-fatal cardiovascular complications and death in young Australians after first RHD diagnosis. Methods and Results This retrospective cohort study used linked RHD register, hospital and death data from five Australian states and territories (covering 70% of the whole population and 86% of the Indigenous population). Progression from uncomplicated RHD to all-cause death and non-fatal cardiovascular complications (surgical intervention, heart failure, atrial fibrillation, infective endocarditis, stroke) was estimated for people aged <35years with first-ever RHD diagnosis between 2010 and 2018, identified from register and hospital data. The study cohort comprised 1718 initially uncomplicated RHD cases (84.6% Indigenous; 10.9% migrant; 63.2% women; 40.3% aged 5-14-years; 76.4% non-metropolitan). The composite outcome of death/cardiovascular complication was experienced by 23.3% (95% CI: 19.5-26.9) within 8 years. Older age and metropolitan residence were independent positive predictors of the composite outcome; history of acute rheumatic fever (ARF) was a negative predictor. Population group (Indigenous/migrant/other Australian) and sex were not predictive of outcome after multivariable-adjustment. Conclusion This study provides the most definitive and contemporary estimates of progression to major cardiovascular complication or death in young Australians with RHD. Despite access to the publically-funded universal Australian healthcare system, one-fifth of initially uncomplicated RHD cases will experience one of the major complications of RHD within 8 years supporting the need for programs to eradicate RHD.

Role of Cardioprotective Medications in Patients with Reduced Global Longitudinal Strain Undergoing Autologous Hematopoietic Stem Cell Transplant to Prevent Post-Transplant Cardiac Morbidity

Background: Cardiotoxicity is one of the side effects of many antineoplastic treatments. Establishing a baseline cardiac function prior to initiating therapy is of paramount importance and may be the limiting factor to choose a certain antineoplastic treatment regimen. Echocardiographic strain techniques including Global Longitudinal Strain (GLS) measurements are sensitive for detecting preclinical cardiac dysfunction and is a predictor for future reduction in ejection fraction and long-term outcome. Autologous transplant is performed as a consolidation strategy for many hematological malignancies and patients who undergo transplant are treated with high doses of chemotherapy including alkylating agents, which can affect cardiac function along with the strain of pancytopenia. Literature that describes the effects of transplant on cardiac function in patients who have reduced Global Longitudinal Strain is scarce if any at all. Most of the data for low GLS in patients receiving cardiotoxic medications suggest poor long-term morbidity and mortality. Objective and design: We present a case series of 3 patients diagnosed with multiple myeloma undergoing an autologous stem cell transplant as part of their treatment with reduced global longitudinal strain on pre-transplant assessment. These patients were treated with cardioprotective medications (Beta Blockers and/or ACEi/ARBs) before and while undergoing conditioning chemotherapy and subsequently autologous stem cell transplant and were followed until day 100. Results: Follow up in the post-transplant period showed improvement of GLS with and none of these patients demonstrated any signs or symptoms of cardiovascular morbidity. Conclusion: Reduced GLS should therapy in patients undergoing ASCT and cardioprotective medications may have a role in reducing cardiac morbidity and mortality in these patients. Our patients displayed improved GLS in the post-transplant period suggesting the need for further research and streamlining the role of cardio-oncology and cardioprotective treatment in similar situations. Figure 1 Figure 1. Disclosures Raval: Abbvie Pharmaceuticals: Speakers Bureau; Adaptive Biotechnologies: Consultancy; ADCT Therapeutics: Consultancy, Speakers Bureau; Alexion Pharmaceuticals: Speakers Bureau; Amgen Biotechnology Company: Research Funding; Astellas Pharmaceuticals: Speakers Bureau; Astrazeneca Pharmaceuticals: Consultancy, Speakers Bureau; Beigene Pharmaceuticals: Speakers Bureau; Bristol Meyers Squibb Pharmaceuticals: Consultancy; Epizyme Pharmaceuticals: Consultancy, Speakers Bureau; Genetech Biotechnology Company: Research Funding; GlaxoSmithKline Pharmaceuticals: Consultancy; Incyte Pharmaceuticals Corporation: Speakers Bureau; Jazz Pharmaceuticals: Consultancy; Karyopharm Therapeutics: Consultancy; Morphosys Biotech Company: Speakers Bureau; Sanofi Genzyme: Consultancy; Seagen Biotechnology Company: Research Funding; Takeda Pharmaceuticals: Consultancy, Speakers Bureau.

AZD2014, a dual mTOR inhibitor, attenuates cardiac hypertrophy in vitro and in vivo

Cardiac hypertrophy is one of the most common genetic heart disorders and considered a risk factor for cardiac morbidity and mortality. The mammalian target of rapamycin (mTOR) pathway plays a key regulatory function in cardiovascular physiology and pathology in hypertrophy. AZD2014 is a small-molecule ATP competitive mTOR inhibitor working on both mTORC1 and mTORC2 complexes. Little is known about the therapeutic effects of AZD2014 in cardiac hypertrophy and its underlying mechanism. Here, AZD2014 is examined in in vitro model of phenylephrine (PE)-induced human cardiomyocyte hypertrophy and a myosin-binding protein-C (Mybpc3)-targeted knockout (KO) mouse model of cardiac hypertrophy. Our results demonstrate that cardiomyocytes treated with AZD2014 retain the normal phenotype and AZD2014 attenuates cardiac hypertrophy in the Mybpc3-KO mouse model through inhibition of dual mTORC1 and mTORC2, which in turn results in the down-regulation of the Akt/mTOR signaling pathway.

Cardiac late effects after modern 3D-conformal radiotherapy in breast cancer patients: a retrospective cohort study in Germany (ESCaRa)

Purpose Radiotherapy (RT) was identified as a risk factor for long-term cardiac effects in breast cancer patients treated until the 1990s. However, modern techniques reduce radiation exposure of the heart, but some exposure remains unavoidable. In a retrospective cohort study, we investigated cardiac mortality and morbidity of breast cancer survivors treated with recent RT in Germany. Methods A total of 11,982 breast cancer patients treated between 1998 and 2008 were included. A mortality follow-up was conducted until 06/2018. In order to assess cardiac morbidity occurring after breast cancer treatment, a questionnaire was sent out in 2014 and 2019. The effect of breast cancer laterality on cardiac mortality and morbidity was investigated as a proxy for radiation exposure. We used Cox Proportional Hazards regression analysis, taking potential confounders into account. Results After a median follow-up time of 11.1 years, there was no significant association of tumor laterality with cardiac mortality in irradiated patients (hazard ratio (HR) for left-sided versus right-sided tumor 1.09; 95% confidence interval (CI) 0.85–1.41). Furthermore, tumor laterality was not identified as a significant risk factor for cardiac morbidity (HR = 1.05; 95%CI 0.88–1.25). Conclusions Even though RT for left-sided breast cancer on average incurs higher radiation dose to the heart than RT for right-sided tumors, we found no evidence that laterality is a strong risk factor for cardiac disease after contemporary RT. However, larger sample sizes, longer follow-up, detailed information on individual risk factors and heart dose are needed to assess clinically manifest late effects of current cancer therapy.

Transcatheter Tricuspid Valve-in-Valve Procedure—An Illustrative Case Report and Review

Severe tricuspid commitment is no longer understood as merely a marker of disease but is now widely thought of as a significant contributor to cardiac morbidity and mortality. However, isolated tricuspid valve surgery remains rare and to this day continues to be associated with the highest surgical risk among all valve procedures and high operative mortality rates, especially in reoperations. Therefore, the development of tricuspid transcatheter procedures is as necessary as it was for the other valves a couple of years ago. Recently, multiple percutaneous therapies have been developed for the management of severe tricuspid disease, initially only repair and more recently replacement, thus creating a new branch for the management of patients who have already undergone surgery and who present with dysfunctional bioprostheses. The purpose of this review and report is to demonstrate current and possible future challenges, and to show that the valve-in-valve procedure of the tricuspid valve is feasible and safe, and now can be performed in all its range, in the smallest to the largest sizes of presentation, without incurring the untoward risks of conventional surgery.

ECG Abnormalities in Patients with Acute Exacerbation of Bronchiectasis and Factors Associated with High Probability of Abnormality

Background. Bronchiectasis is an important reason for morbidity and mortality according to the last records that referred to high incidence rate of disease. Cardiovascular problems are common in pulmonary diseases, in general, and it can symptom by ECG abnormalities. The objective of this study was to define the most ECG abnormalities in patients with acute exacerbation of bronchiectasis and to study the correlation between the cardiac disorder and the other risk factors of the exacerbation. Materials and Methods. A prospective single-center observational cohort study was done at Aleppo University Hospital for patients with AEB between October 2017 and September 2018. They were divided into 2 groups (normal ECG vs. abnormal). Patients with COPD, cystic fibrosis, new diagnosis of ischemic accident through the last 6 months of the study, and treatment with macrolides or fluoroquinolones through the last 3 months of the study were excluded. We study the percent of abnormalities through the AEB and the percentage of the most common abnormalities. Results. 67 patients were included in the study (44 males and 23 females) with a mean age of 52.85 ± 21.456 . ECG abnormalities were recorded in 43 patients, and it was more common in men (67.44% of cases). Advanced age and survival state had a statistical significance ( p = 0.003 , 0.023), respectively, between the 2 groups. Right axis deviation (RAD) is the most common abnormality (23.3%) followed by sinus tachycardia (20.9%), and it is close to T-depression (18.6%). AF was the most common arrhythmia from all recorded arrhythmias (6.98% from all cases). Positive sputum cultures were recorded in 55.8%, and the most common isolated pathogen factor was Pseudomonas aeruginosa. Recurrent pneumonia was seen in 30.2% of all patients with abnormal ECG. We find a high prevalence of ECG abnormalities in patients with Oximetry (90-95%, 39.5%), and the opportunity for abnormalities is equal in the 2 age groups (45–59 and more than 75) that reflexed the possibility of cardiac disorders in any age in patients with AEB. Conclusions. ECG abnormalities are common in AEB, and it can happen in any age and any value of Oximetry. It needs more attention because of the prognosis of the cardiac morbidity.

Autonomic nervous system dysfunction correlates with microvascular damage in systemic sclerosis patients

Objectives: Cardiac autonomic neuropathy is among the known cardiovascular complications of systemic sclerosis and may affect the whole prognosis of the disease. The aim of our study was to assess cardiac autonomic neuropathy prevalence in our cohort of systemic sclerosis patients and compare its main features with clinical and epidemiological data, particularly with the severity of microvascular damage, as detected by nailfold videocapillaroscopy. Methods: Twenty-six patients with definite systemic sclerosis were consecutively enrolled at our outpatient rheumatology clinic. All patients underwent physical examination, nailfold videocapillaroscopy, and autonomic neuropathy diagnostic tests (orthostatic hypotension test, deep breathing test, lying-to-standing, and Valsalva maneuvers). Results: Cardiac autonomic neuropathy prevalence was 50% (13 cases). On univariate analysis, cardiac autonomic neuropathy was shown to be significantly associated with an active pattern on nailfold videocapillaroscopy (odds ratio 5.86, 95% confidence interval 1.59–9.24; p = 0.032), whereas anti-Scl-70 positivity (odds ratio, 0.24; 95% confidence interval, 0.03–2.12; p = 0.049) and C-reactive protein (odds ratio, 19.32; 95% confidence interval, 1.79–56.71; p = 0.036) reached only a borderline statistical association. The time-dependent Cox multivariate regression model showed cardiac autonomic neuropathy development to be independently associated with an active pattern on nailfold videocapillaroscopy (odds ratio, 7.19; 95% confidence interval, 1.87–8.96; p = 0.042) and anti-Scl-70 positivity (odds ratio, 5.92; 95% confidence interval, 1.06–18.43; p = 0.048). Conclusions: Severe microvascular damage, as detected by nailfold videocapillaroscopy, may suggest the coexistence of autonomic dysfunction and should be considered as a red flag for the identification of patients particularly at risk of cardiac morbidity and mortality.

Large Incidental Intracardiac Thrombus in a Patient with Ischemic Heart Disease

Rheumatic heart disease contributes to significant cardiovascular morbidity and mortality and remains a predominant cause of cardiac morbidity in developing nations. Coronary angiography is indicated as a routine procedure in the preoperative assessment of patients with valvular heart disease, who are older than 40 years age. Though transthoracic echocardiography (TTE) is routinely done preoperative in every patient, transesophageal echocardiography (TEE) is an important imaging modality especially for the identification of thrombus. The diagnosis of associated left atrial thrombus changes the approach to the surgery, regardless of the symptoms. Rheumatic mitral valve disease contributes to significant cardiovascular morbidity and mortality worldwide. It is the predominant cause of cardiac morbidity in developing nations. This case demonstrates incidental finding of large left atrial thrombus, diagnosed incidentally through transesophageal echocardiography in a patient who was previously diagnosed as a case of severe mitral stenosis with coronary artery disease.

Anaesthetic Management of Ischemic Heart Disease with Ejection Fraction of 30 % Posted for Fracture Femur Surgery – A Case Report

The incidence of ischemic heart disease (IHD) is increasing and it is the leading cause of morbidity and mortality worldwide.1 These patients have increased risk of myocardial ischemia, myocardial infarction (MI), conduction disturbances and cardiac arrest perioperatively. Preoperative cardiovascular assessment and prediction of short- and long-term risks affects the perioperative anaesthetic management and also surgical decision making. Risk factors include recent MI, congestive cardiac failure, peripheral vascular disease, angina pectoris, diabetes mellitus (DM), hypertension, renal dysfunction, age, obesity and sedentary lifestyle.2 The choice of anaesthesia is general anaesthesia so that myocardial oxygen supply can be kept greater than demand by preventing tachycardia and high blood pressures, which can lead to ischemia.3 Few studies have shown that Epidural anaesthesia can decrease cardiac morbidity and mortality in IHD patients. We report a successful anaesthetic management of IHD with ejection fraction of 30 % posted for fracture femur surgery under Combined Spinal Epidural (CSE) anaesthesia.