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Background: Systemic Mastocytosis (SM) is a disorder of excessive mast cell infiltration in multiple organ tissues. Atherosclerosis is a major risk factor for developing acute coronary syndrome. In addition to lipid accumulation in the arterial wall, inflammation plays an important role in the pathogenesis of plaque rupture and activating the thrombosis cascade. The Mast cells contribution to plaque destabilization has been well established in multiple animal and human studies. In a recent study, SM has been proven to be associated with a higher incidence of acute coronary syndrome even with lower plasma lipids levels. The study showed that 20% of patients with SM had cardiovascular events compared to only 6% in the control group with adjustment to all cardiac risk factors. Case: We presented a patient with no risk factors for heart disease other than old age and history of SM who developed acute myocardial infarction. Conclusion: SM can be life-threatening and can result in ACS, anaphylactic reaction, syncope, or cardiac arrest. Clinicians should have a high index of suspicion of acute coronary syndrome (ACS) occurrence in the setting of inflammatory conditions, such as SM and KS, and vice versa, where SM should be considered or ruled out in patients who suffer from anaphylaxis and cardiac arrest or myocardial infarction.

Hydralazine, an arterial vasodilator, is a widely used medication for the management of hypertension and heart failure, especially for patients who cannot tolerate the use of ACEIs or ARBs. It is generally well-tolerated and has a safe profile in pregnancy. However, hydralazine can induce immune-mediated side effects, such as hydralazine-induced lupus and less commonly hydralazine- induced ANCA vasculitis. The latter most commonly affects the kidneys with or without other organ involvement. There are several cases reported in the literature of hydralazine-induced ANCA associated vasculitis (AAV) that have pulmonary manifestations, also known as hydralazine- induced pulmonary-renal syndrome (PRS), a condition with a high risk of mortality. We are reporting a case of Hydralazine-induced ANCA associated glomerulonephritis with severe diffuse alveolar hemorrhage (DAH). In addition, we will review the current literature and discuss the importance of prompt diagnosis and early management to decrease mortality and morbidity associated with this serious condition.

Background: Fibromuscular dysplasia (FMD) is a non-atherosclerotic disease that affects medium-sized arteries and results in stenosis, dissection, aneurysm or occlusion. It is most commonly reported in the renal and carotid arteries. Involvement of coronary arteries is quite rare and and leads to serious consequences. Case: A 62-year-old African American woman with a history of mitral valve prolapse presented with chest discomfort associated with diaphoresis. Her EKG initially showed ST segment changes in leads II, III, and V2-V5 which resolved in approximately 30 minutes. Her troponin peaked to 20 ng/L during her hospital course. A bedside echocardiogram revealed an EF of 45% with mid, distal septal and apical hypokinesis. Decision-making: The patient was admitted to the Cardiac Care Unit for treatment of an NSTEMI. She underwent cardiac catheterization, which revealed single-vessel coronary disease with diffuse narrowing of the distal LAD, beyond the first diagonal branch down to the apex. CT angiography of her abdomen and pelvis showed mild narrowing of the mid-right renal artery with a small fusiform aneurysm measuring approximately 5 mm. Her carotid duplex showed tortuosity in the right internal carotid artery. Given the multiple vascular anomalies, a diagnosis of fibromuscular dysplasia was considered. Conclusion: Acute coronary syndrome in fibromuscular dysplasia requires an integrated approach to management, especially if there is associated malignant hypertension and/or dissection.

The association between atrial fibrillation and stroke is well-known by use of the CHA2DS2VASc risk assessment. However, little is known about the thromboembolic risk in patients who have isolated systolic heart failure (HF) with sinus rhythm. Despite growing literature which shows how HF in the setting of normal sinus rhythm is a risk factor for thromboembolic disease, the recommendation for use of anticoagulation, whether warfarin or direct oral anticoagulation (DOAC) in this setting, is not yet reflected in cardiology guidelines. To reduce the risk of thromboembolism, anticoagulation trials have been done but have not conclusively shown benefit over risk. We present a case of left middle cerebral artery (MCA) stroke after de novo HF in a patient with sinus rhythm, who was previously not on anticoagulation. Case: A 57-year-old male presented to our hospital with worsening shortness of breath for 3 days. He had signs of hypervolemia consistent with an acute heart failure exacerbation. Pertinent physical exam findings included lower extremity edema, rales, and jugular venous distension. He also had fatigue and exertional dyspnea. During his hospital course, he underwent imaging studies that revealed cardiomegaly, absence of pulmonary embolism, and a severely reduced ejection fraction. During his acute heart failure admission, he developed a left MCA stroke suspected to be of cardioembolic etiology and was successfully treated with tissue plasminogen activator (tPA). Conclusion: According to the most recent AHA/ACC cardiovascular disease guidelines, anticoagulation is not indicated in systolic heart failure patients with sinus rhythm. However, our case is rare because the patient had no underlying risk factors for thromboembolism and significant past medical history. He developed new-onset nonischemic cardiomyopathy complicated by a stroke. Therefore, we suggest the need for prophylactic anticoagulation should be assessed on an individual basis, with the assistance of shared decision making, especially when the ejection fraction is acutely and severely reduced.

Coronary air embolism is a dreadful complication of cardiac intervention despite careful prevention. In literature reported complications of coronary air embolism ranges from clinically non significant events to cardiogenic shock, myocardial infarction and death. We report a case of massive coronary air embolism in right coronary artery, which results in hypotension and complete heart block in a 33 -year-old female undergoing elective closure of atrial septal defect (ASD) by percutaneous transcatheter intervention. The patient stabilized after timely measures like oxygen support, dopamine infusion, cardiac compression and repeated bolus injection of heparinized saline. She then underwent successful percutaneous ASD device closure.

Atrial septal defect (ASD) is a risk factor for multiple vascular thrombotic events, which can occur either sequentially or simultaneously. In this report we present a case of ST-elevation myocardial infarction (STEMI) and cerebrovascular accident (CVA). The severity of adverse cardiovascular or cerebrovascular events can be increased by the presence of specific type of ASD, such as a patent foramen ovale (PFO) or osteum secundum defect. This case report discusses a unique presentation of a 48-year old male on warfarin therapy for a history of cerebral venous thrombosis (CVT) who subsequently presented with simultaneous STEMI with CVA, and who was incidentally found to have an ostium secundum defect on echocardiography. He was emergently taken for cardiac catheterization, which revealed significant proximal LAD occlusion. There has been a long standing debate within the international scientific communities regarding the therapeutic benefit of PFO closure for long-term secondary prevention of recurrence CVA. We discuss the different points of view regarding PFO closure for secondary prevention of CVA with a review of the literature on this rather controversial topic.

Arrhythmias can complicate the course of patients with ST-elevation myocardial infarction. These arrhythmias can include both tachyarrhythmias and bradyarrhythmias. Tachyarrhythmias range from Ventricular premature complexes to life-threatening ventricular tachycardia/ fibrillation. Bradyarrhythmias range from sinus bradycardia to complete heart block. These arrhythmias have the ability to provoke hemodynamic consequences and increase mortality. Tachyarrhythmias are more common with Anterior wall myocardial infarction and bradyarrhythmias are more common with Inferior wall myocardial infarction. We report a case of Mobitz Type 1 (Wenkebach) second-degree atrioventricular block in a patient with Anterior wall myocardial infarction. Angiography showed a significant lesion in Left anterior descending artery, after the first septal and diagonal branch. After the successful percutaneous coronary intervention, this second degree AV block reverted to first degree AV block. To the best of our knowledge, there is no case report describing this association separately.

Coronary artery anomalies occur in 1.3-5.6% of patients undergoing coronary arteriography. An anomalous origin of LCX from right coronary sinus is the most common congenital variant. It is usually considered “benign” since it is not known to predispose individuals to sudden cardiac death. Such vessels are particularly predisposed to atherosclerotic disease in their proximal portion, due to the acute angulation of its origin from the aorta and its posterior retro aortic course. We present a case of 55 years old female admitted with acute coronary syndrome. Coronary angiogram showed the anomalous origin of the left circumflex artery from right coronary sinus. This artery had a significant lesion which was successfully stented with a drug-eluting stent.

Uhl’s anomaly is an extremely rare congenital cardiac malformation and is characterized by the partial or complete absence of right ventricular myocardium. The absence of myocardium may be the result of primary non-development of myocytes or a form of selective apoptosis. It is mainly sporadic although some familial occurrences have been reported. Congestive cardiac failure is the most common mode of presentation. Associated congenital cardiac malformations are also reported. We report a case of a 17-year-old male who presented with symptoms and signs of right heart failure, during evaluation found to have large right ventricle free wall thrombus. Cardiol Res. 2019;10(2):124-127 doi: https://doi.org/10.14740/cr758w

Coronary artery aneurysm is defined as the localized dilatation of a coronary artery segment more than 1.5 times the size of adjacent normal segments. The aneurysms of the coronary arteries are rare. Coronary aneurysms can be congenital or acquired. The majority are atherosclerotic in origin. The primary complication is myocardial ischemia or infarction, sudden cardiac death with rupture being rare. Some aneurysms are diagnosed incidentally in arteries other than the culprit artery. Treatment options include medical management with anticoagulation, percutaneous intervention with covered stents or surgery. We report a case of 67-year-old male who presented with acute coronary syndrome. Coronary angiogram showed a moderate size aneurysm of the proximal left anterior descending artery. This aneurysm was successfully managed percutaneously with a covered stent. Cardiol Res. 2018;9(5):318-323 doi: https://doi.org/10.14740/cr764w

Dynamic obstruction to left ventricular outflow in patients with hypertrophic cardiomyopathy usually occurs when the anterior mitral leaflet moves forward in systole and approaches or contacts the ventricular septum. This is called as systolic anterior motion (SAM) of anterior mitral leaflet. However, we have recently identified, by M mode and two-dimensional echocardiography a patient with hypertrophic cardiomyopathy who had a unique pattern of mitral valve motion characterized by abnormal mitral valve coaptation and systolic anterior motion of the abnormally elongated posterior mitral leaflet (PML) leading to left ventricular outflow tract obstruction.This was due to intrinsic abnormality of posterior mitral leaflet. Recognition of systolic anterior motion (SAM) of the posterior mitral leaflet (PML) due to intrinsic abnormality may alter the operative approach in selected patients with hypertrophic cardiomyopathy and subaortic obstruction.

The posterior descending artery (PDA) supplying the posterior one-third of the inter-ventricular septum usually arises from the right coronary artery (RCA) or the left circumflex artery (LCX). PDA arising from the left anterior descending artery (LAD) is an extremely rare anomaly. Here we report a rare type of left dominant circulation in which a large LAD is continuing as PDA after winding round the apex in the presence of a diminutive RCA. Such a large LAD continuing as PDA is referred as "hyperdominant" or "superdominant". A 32-year-old male chronic smoker presented with acute onset retrosternal pain of 4 h duration with profuse sweating in primary health center with electrocardiography (ECG) changes in inferior leads and was thrombolysed with intravenous streptokinase 15 lacs IU over one hour and was referred to our center for further management and coronary intervention. Coronary angiogram revealed PDA as a continuation of the LAD beyond the crux and a non-dominant right coronary as well as LCX. The LAD had plaque in mid-LAD course. Intravascular ultrasound study (IVUS) showed insignificant plaque in mid-LAD (30%). Hence, we decided to keep him on medical therapy only. Cardiol Res. 2018;9(4):253-257 doi: https://doi.org/10.14740/cr738w

Among the congenital anomalies of the coronary arteries, absent left circumflex artery (LCX) defect is extremely rare. Only a few cases have been reported in the literature. We report a case of a 48-year-old female who presented with a 4-month history of exertional chest pain with positive stress (treadmill) test. Conventional coronary angiogram showed a normal left anterior descending, absent LCX and a super-dominant right coronary artery (RCA) with prominent branches. Aortography also failed to show a separate ostium for the left circumflex artery. Multi-detector computed tomographic coronary angiography was performed to confirm the diagnosis of congenital absence of the LCX. It is a benign incidental finding, however some patients present with angina-like symptoms often resulting in detection of this rare anatomy on coronary angiography. Precise morphological and functional evaluation of the anomalous coronary artery is important for selecting the best treatment modality and better prognosis.

It is generally believed that the occlusion of a non-dominant right coronary artery is not associated with significant consequences. Atherosclerotic disease of non-dominant right coronary in the absence of significant left coronary artery disease is rare, and the prevalence is substantially lower as compared to dominant right coronary artery. We report a case of young male presenting with acute coronary syndrome and hemodynamically significant atrial fibrillation. Coronary angiography showed thrombotic occlusion of non dominant right coronary artery. After the stenting of this artery, patient improved hemodynamically with complte resolution of ECG changes.

Background: Thrombosis is a complication of prosthetic valves on oral anticoagulants which is associated with significant morbidity and mortality. A re-operation carries a substantial risk, with mortality rate from 10% to 15% in selected series, which may be 2- or 3-folds higher in critically ill patients. This study conducted in a tertiary care cardiology unit aimed to evaluate the effectiveness and safety of thrombolytic therapy in stuck mitral bileaflet heart valves. Methods: As a prospective observational study, clinical symptoms and fluoroscopy were the mainstay in diagnosis of stuck mitral valve. Gradient across the valve by transthoracic echocardiography was used to monitor the therapy every 6 h. Fall of mean gradient more than 50% was considered as successful thrombolysis. And final results were again checked by fluoroscopy with documentation of improved leaflet movement. Results: Totally we studied 34 patients. Patients receiving thrombolytic therapy with streptokinase achieved an overall 91.2% freedom from a repeat operation or major complications, a large subcutaneous hematoma occurred in one ( 2.9%), reoperation required in two due to failure of treatment (5.9%), allergic reaction in one (2.9%), one patient developed transient neurologic dysfunction (2.9%) and one patient died during therapy due to refractory cardiogenic shock(2.9%). All patients including those with delayed presentation (> 14 days) and hemodynamically unstable patients had good results similar to those who presented within 14 days and hemodynamically stable. Mortality was higher in unstable patients and reoperation was higher with delayed presentation. Conclusions: Thrombolysis with streptokinase is highly successful and safe therapy in hemodynamically stable as well as unstable patients, or those with early or delayed presentation with stuck bileaflet mitral valves, especially in centers where round the clock cardiothoracic surgery backup is not available. Cardiol Res. 2018;9(3):161-164 doi: https://doi.org/10.14740/cr708w

Introduction: Congenital coronary artery anomalies are quite uncommon with estimates ranging from 0.2% to 1.3% on coronary angiography. The rarity of these anomalies makes their diagnosis a formidable challenge. Variable arterial courses have been described. In this report, we present a case with unique arterial course, which starts as a hyperacute take-off of the right coronary artery (RCA) from the left main coronary artery (LMCA), with subsequent coursing, without external compression, between the right ventricular outflow tract and aorta. Our case is relevant to the concept of whether we should keep a reasonable index of suspicion for coronary artery anomalies during cardiac evaluations of patients undergoing non-cardiac surgeries. Clinical case: This is an asymptomatic 47-year-old African American female who presented for cardiac clearance for renal transplantation. She had a past medical history of hypertension, bronchial asthma, and former smoking of 10 years (quitting 15 years prior to presentation). She also has end-stage renal disease on hemodialysis. Cardiac workup revealed left ventricular hypertrophy on EKG, multi-chamber dilation seen on echo, and anomalous RCA course seen on CT coronary angiography. Cardiac catheterization revealed non-obstructive coronary artery disease of the LCX and RCA. After consulting with cardiothoracic surgery, conservative medical management was decided based on the patient’s risk stratification. She was advised to have close monitoring of her condition. Discussion: Coronary artery anomalies represent the second most common cause of sudden cardiac death in young athletes. At this time, the prevalence of right coronary artery (RCA) take off from the left coronary sinus occurs at a percentage of 0.019% to 0.49%. The RCA origination from the left main coronary artery (LMCA) accounts for only 0.65% of these anomalies. Our patient had high-risk anatomy consisting of a hyper-angulated take-off of the RCA from the LMCA as well as course between the pulmonary artery and right ventricular outflow tract (RVOT). CT coronary angiography is the most useful imaging modality that characterizes coronary artery anomalies. Although this patient exhibited no signs or symptoms of cardiorespiratory compromise, she warranted a full cardiac workup preoperatively that incidentally revealed a coronary anomaly. Recognition of this disease is critical for timely prevention of potential complications as well as discussion of goals of care. Guidelines for medical versus surgical management are available, but the management strategy should be individualized, with the highest consideration given to risk-benefit analysis.

Spontaneous coronary artery dissection (SCAD) is a rare illness often misdiagnosed, that accounts for up to 4% of cases of acute coronary syndrome in young women. Interestingly, SCAD is the most common cause of myocardial infarction related to pregnancy. Here, we present a case of a 35-year old postpartum patient who presented in cardiac arrest due to ventricular fibrillation and was found to have a ST-segment elevation myocardial infarction. Cardiac catheterization revealed significant coronary vasospasm and dissection of the right circumflex and distal left anterior descending artery. We discuss clinical diagnosis and management of spontaneous coronary dissection along with literature review.

Accurate recognition and documentation of coronary artery anomalies and their course at the time of coronary angiography is essential to determine the significance of such findings and to avoid therapeutic complications. The identification of this anomaly demands a high level of anticipation during the performance of selective coronary angiography to ensure that an adequate study is obtained. Failure to recognize and properly demonstrate the anomaly may result in improper therapeutic decisions that may be hazardous to the patients. Special surgical considerations must be made when performing the valvular replacement or coronary artery bypass grafting, if desired, in such patients.