Surgical Management of Aortic Regurgitation in Takayasu's Arteritis: A Systematic Review of Techniques and Outcomes.

IntroductionTakayasu's arteritis (TA) is an inflammatory condition that affects large vessels and frequently involves the aortic valve causing valve regurgitation. Surgical management is recommended for symptomatic severe aortic regurgitation (AR); however, the optimal surgical approach is yet unclear. This study aims to review surgical treatment options for AR in TA and determine which procedure has a lower chance of late postoperative events and/or mortality. MethodsAn electronic database search was performed within PubMed, EMBASE, Web of Science, and SCOPUS to identify articles from 1975 to 2016 focusing on surgical management of the AR in TA. ResultsTwenty seven studies encompassing a total of 194 cases (77% females) were included. Isolated aortic valve replacement (AVR) was performed in 105/194 cases (54%) (Group A), while combined aortic valve and root replacement (CAVRR) was performed in 87/194 (45%) (Group B). Prosthetic valve detachment was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Dilation of the residual aorta was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Any late (≥ 30 d) postoperative cardiac event was reported in 26/105 cases (24.8%) in group A, and in 7/87 cases (8.1%) in group B (p = 0.003). ConclusionsAlthough CAVRR is a more complex procedure, it might offer a better outcome in terms of late postoperative cardiac events compared to isolated AVR procedure. Future prospective studies are required to help determine the best surgical approach in such a population.

Distinct Coagulopathy With Myocardial Injury and Pulmonary Embolism in COVID-19.

In this article, we report a case of a 61-year-old male who was diagnosed with SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), presenting with acute respiratory distress syndrome requiring intubation and hemodynamic support, marked D-Dimer and troponin I elevation, worsening ST-elevation myocardial infarction on repeat electrocardiograms, and a negative coronary angiogram ruling out a coronary artery thrombosis or occlusion. With worsening diffuse ST-segment elevation on electrocardiograms and reduced ejection fraction on echocardiography in the setting of systemic inflammation, fulminant myocarditis was highly suspected. Despite optimal medical treatment, the patient's condition deteriorated and was complicated by cardiac arrest that failed resuscitation. Although myocarditis was initially suspected, the autopsy revealed no evidence of myocarditis or pericarditis but did demonstrate multiple microscopic sites of myocardial ischemia together with thrombi in the left atrium and pulmonary vasculature. Additionally, scattered microscopic cardiomyocyte necrosis with pathological diagnosis of small vessel micro-thrombotic occlusions. These findings are potentially exacerbated by inflammation-induced coagulopathy, hypoxia, hypotension, and stress, that is, a multifactorial etiology. Further research and an improved understanding are needed to define the precise pathophysiology of the coagulopathic state causing widespread micro-thrombosis with subsequent myocardial and pulmonary injury.

Incidental Finding of a Large Right Atrial Thrombus in a Patient With Cerebral Lymphoma.

Right atrial (RA) masses are rare, challenging to diagnose, and potentially life-threatening with high mortality if untreated. We present a patient presenting with diffuse large B-cell lymphoma in the brain that was incidentally found to have a large RA mass. For a better definition of the RA mass, extensive workup using multimodality imaging including chest computed tomography, transthoracic echocardiography, transesophageal echocardiography, cardiac magnetic resonance imaging, and left heart catheterization was warranted. The imaging demonstrated a large RA mass extending through the tricuspid valve into the right ventricle and superior and inferior vena cava without a mobile component. The mass was then successfully resected, and further histology examination was performed to rule out lymphoma and rare subtypes of diffuse large B-cell lymphoma. The comprehensive workup proved the RA mass to be a calcified thrombus rather than a direct metastatic spread of lymphoma.

An Unusual Case of ST Elevation Myocardial Infarction in Angiographically Non-Obstructed Coronary Arteries.

It is routinely believed that patients with electrocardiographic findings of ST elevation myocardial infarction (STEMI) will have evidence of obstructive coronary artery disease on coronary angiography. Although this is the situation in the majority of STEMI instances, in a small percentage of cases, patients presenting with STEMI are found to have minimal to no coronary artery blockage on subsequent angiography, termed MI with non-obstructive coronary arteries (MINOCA). MINOCA is a heterogenous entity with multiple causes, making a focused diagnostic workup important to select an appropriate treatment, with the goal to prevent a mortality similar to obstructive coronary disease. In this case, we describe a unique presentation of MINOCA, after a patient was diagnosed with STEMI.

Decompensated Non-Ischemic Cardiomyopathy Induced by Anabolic-Androgenic Steroid Abuse.

A 30-year-old male presented to the emergency department with dyspnea, fatigue, orthopnea, and paroxysmal nocturnal dyspnea for the past three months. The patient admitted to anabolic steroid use for the past 11 years. Transthoracic echocardiography was significant for severely dilated left ventricle, diffuse hypokinesis, ejection fraction < 15%, and grade II diastolic dysfunction. The patient was diagnosed with decompensated, non-ischemic cardiomyopathy stage C, and New York Heart Classification (NYHA) class III > IV, likely from use of anabolic steroids, after a negative workup for other etiologies. On follow-up after continuation of guideline-directed medical therapy, the patient demonstrated improved heart failure status (NYHA class I > II). Cardiomyopathy is a rare but important adverse effect of anabolic steroids to consider.

An Unusual Case of Neisseria flavescens/subflava Group Tricuspid Valve Endocarditis in a Patient With Previously Treated Methicillin-Resistant Staphylococcus aureus Endocarditis.

Infective endocarditis (IE) is classified as an infection of any cardiac valve or endocardial surface and is often associated with high morbidity and mortality. Certain bacteria such as gram-positive cocci and viridans group streptococci have high predilection for both naïve and damaged cardiac valvular tissues, accounting for the majority of IE cases. In very infrequent instances, gram-negative bacteria (GNB), more specifically non-meningococcal, non-gonococcal GNB, have been shown to cause IE. The following is a case of a young male diagnosed with Neisseria flavescens/subflava tricuspid valve endocarditis after being previously treated for Methicillin-resistant Staphylococcus aureus (MRSA) endocarditis.

Tri-Valvular Endocarditis in a Previously Normal Heart Misdiagnosed as Recurrent Valley Fever.

Infective endocarditis (IE) is classified as an infection of any cardiac valve or endocardial surface. This condition is associated with high morbidity and mortality; hence, early diagnosis and rapid intervention are extremely vital. Although IE is frequently found to infect only one heart valve, rare instances have shown multi-valvular involvement. Many conditions can present similar to IE, often delaying the diagnosis of IE. Coccidioidomycosis (or Valley Fever), a fungal infection endemic in the Southwestern United States, can present with features analogous to those of IE. We present the case of a middle-aged male with no underlying structural heart disease found to have tri-valvular IE after being misdiagnosed with recurrent Valley Fever.

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review.

Solitary fibrous tumors (SFT) represent a unique subset of mostly benign heterogeneous tumors with mesenchymal cell origins. These tumors have been reported in the past as being mostly indolent, with a slowly evolving clinical course and low potential for malignancy. Although found systemically, the incidence of SFT arising intrathoracically, from the pleura of the lung, is relatively poorly documented in the medical literature. SFT is a rare phenomenon, but in even rarer circumstances, these tumors are associated with distinctive paraneoplastic syndromes, such as Pierre-Marie-Bamberger syndrome (PMBS) and Doege-Potter syndrome (DPS). PMBS presents as digital clubbing and hypertrophic pulmonary osteoarthropathy. DPS has been characterized as a non-islet cell tumor hypoglycemia due to the ectopic secretion of insulin-like growth factor 2 (IGF-2), a pattern seen in fewer than 5% of cases of SFT. Treatment is typically through surgical resection. In our research of the medical literature, we found only very few cases in which the association with SFT and both paraneoplastic syndromes were described. Here, we report an uncommon case of a 68-year-old male patient found to have an incidental right hemithoracic tumor with digital clubbing and intermittent severe episodes of fasting hypoglycemia after initially presenting with a syncopal episode.

A Novel Combination of Metachronous Primary Malignancies of the Thyroid and Breast in a Patient with Neurofibromatosis Type 1.

Neurofibromatosis 1 (NF1) is a genetic condition of variable presentations. It has been shown to increase the risk of multiple cancers. Therefore, NF1 has been identified as a tumor-provoking condition. We present a case of a 39-year-old woman with NF1 who was diagnosed initially with papillary thyroid carcinoma (PTC) and subsequently presented with a painful breast lump. Core biopsy revealed an invasive ductal carcinoma (IDC) for which selective estrogen receptor modulator (SERM) therapy was initiated. A lumpectomy was performed soon after, which confirmed IDC. Following surgery, the patient received a combination of anthracycline and cyclophosphamide (AC), which was later followed by a taxol-based chemotherapy regimen. This study aims to throw light on the rare phenomenon of metachronous malignancy: the occurrence of successive primary cancers in the same patient. We believe that raising awareness regarding the different neoplasms associated with NF1 is important to promote appropriate preemptive screening for early detection of a second primary neoplasm, which can help lower the morbidity and mortality associated with this condition through expedited intervention.