Cardiovascular Diseases

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• Right atrial thrombus masquerading as myxoma Shahad Alchalabi, Abdulrahman S. Museedi Views: 19 HTML: 0 PDF: 9

  Introduction: In-situ right atrial (RA) thrombus is a rare occurrence typically associated with heightened inflammatory or hypercoagulable states. Here, we present a case of in-situ RA thrombus mimicking atrial myxoma in a patient with sepsis and bacteraemia.
  Case description: A 41-year-old man presented with septic arthritis and bacteraemia caused by methicillin-resistant Staphylococcus aureus (MRSA). A transoesophageal echocardiogram revealed a large pediculated mass resembling atrial myxoma, which was not visible on transthoracic echocardiography performed four days earlier. Cardiac magnetic resonance (CMR) imaging strongly suggested a thrombus, leading to the patient undergoing transcatheter aspiration. Subsequent pathology confirmed an organised fibrin thrombus without evidence of infection.
  Discussion: The patient’s in-situ RA thrombus likely developed in response to a heightened inflammatory state associated with sepsis. Limited data exist on in-situ RA thrombi in the absence of atrial fibrillation, though some reports suggest a correlation between heightened inflammation and a hypercoagulable state.
  Conclusion: CMR imaging is invaluable for characterising such masses and can aid in distinguishing a thrombus from a myxoma.

• Unveiling the deadly dance of hypocalcemia and lactic acidosis in a 59-year-old woman with B-cell lymphoma Yasaman Navari, Efosa Bazuaye, Philip J. McDonald Views: 25 HTML: 1 PDF: 11

  Calcium plays a crucial role in the heart’s electrical conduction system and facilitating the contraction of cardiac muscles. Hypocalcemia can result in electrocardiogram findings such as a prolonged QTC interval and eventually torsade de pointes, which in severe cases can progress to cardiac arrest. In cases of B-cell lymphoma, hypocalcemia may arise from various factors. Tumor infiltration can disrupt calcium homeostasis by affecting the parathyroid glands or bone tissue. Acidosis in the context of B-cell lymphoma can cause significant cardiovascular adverse effects. It will reduce peripheral vascular resistance and cardiac muscle contractility, promote dysrhythmias, and disturb oxygen uptake in the lungs. These combined effects markedly compromise cardiac function, increasing the likelihood of cardiac arrest. These mechanisms necessitate comprehensive management strategies in B-cell lymphoma patients. In this case report we present a case of cardiac arrest in a 59-year-old female woman with hypocalcemia and lactic acidosis secondary to B-cell lymphoma.

• When rhythm correction turns risky: rare right-sided haemorrhagic pleural effusion post MAZE procedure Yasaman Navari, Justine Chinnappan, Thair Dawood, Huda Marcus Views: 23 HTML: 1 PDF: 8

  The maze procedure for atrial fibrillation carries risks, including pleural effusion. We report a case of a 54-year-old woman with right-sided pleural effusion post maze surgery, presenting with dyspnoea. Despite treatment, complications arose, including atrial flutter. Prompt recognition and multidisciplinary management led to a favourable outcome. This case underscores the importance of vigilance for rare post-operative complications and highlights the need for collaborative care in optimising patient outcomes following cardiac surgeries. Further research is warranted to refine management strategies for such occurrences.

• Unmasking a case of sitosterolaemia: an approach for diagnosis and management Mostafa Elbanna, Fahad Eid, Mostafa Zaalouk, Ahmad Nawid Latifi, Gaurav Sharma Views: 63 HTML: 11 PDF: 47

  This report presents a 57-year-old female with a history of dyslipidaemia, intolerant to statins and currently managed on evolocumab. Despite a healthy lifestyle, lipid panel abnormalities persisted, leading to an investigation that revealed heterozygous mutations in the ABCG8 gene, confirming a diagnosis of sitosterolaemia. The patient’s unique response to lipid-lowering medications typified this rare disorder, necessitating specialised genetic testing for diagnosis. Management involved dietary modifications and the introduction of ezetimibe, evolocumab and atorvastatin, demonstrating the personalised nature of treatment. The case underscores the importance of considering sitosterolaemia in unexplained lipid abnormalities and highlights the challenges in diagnosis and management. Ongoing research is crucial for refining diagnostic and therapeutic strategies for this clinically significant disorder, emphasising the need for a multidisciplinary approach to patient care.

• Anomalous bronchial artery origin - canary in the coal mine - for diagnosing chronic thromboembolic pulmonary hypertension Devi Parvathy Jyothi Ramachandran Nair, Shilla Zachariah, Anisha Abraham, David Sacks, Michael Koslow, Rittu Hingorani Views: 265 PDF: 165 HTML: 12

  Anomalous bronchial artery origins may have clinical implications beyond their anatomical curiosity. In this case, the identification of such an anomaly led to the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH).
  A 49-year-old male with a history of recurrent deep vein thrombosis (DVT) and pulmonary embolism (PE) on anticoagulation presented with chest pain and shortness of breath. Laboratory analysis was remarkable for a troponin peak of 14.74 ng/ml, a brain natriuretic peptide level of 602 pg/ml and a D-dimer level of 0.62 µg/ml. Electrocardiogram showed non-specific ST elevation in the anterolateral and inferior leads. Computed tomography angiography (CTA) of the chest was positive for PE involving the right lower lobe pulmonary arterial tree. Echocardiogram showed reduced left ventricular function (ejection fraction 38%) and akinesis of the antero-apical and infero-apical segments. Cardiac catheterization revealed non-obstructive coronary arteries, and an anomalous origin of a right bronchial artery from the right coronary artery. The right bronchial hypertrophied as it supplied collateral flow to the occluded right pulmonary artery. This anomaly and the patient’s history of multiple DVT/PEs while on therapeutic levels of warfarin with near normal D-dimer levels raised suspicion for a false positive PE. Pulmonary angiogram revealed chronic occlusion in branches of the right pulmonary artery, mean pulmonary artery pressure of 36 mmHg and no acute thrombus. Ventilation-perfusion scan confirmed the diagnosis of CTEPH. The patient underwent successful pulmonary thromboendarterectomy and subsequently had normalization of mean pulmonary artery pressure.
  This case underscores the importance of a comprehensive diagnostic approach, and consideration of alternative explanations for imaging findings, that unveiled the diagnosis of a complex and life-threatening condition such as CTEPH.

• Myxoma involving the pulmonary valve – A case report of an atypical cardiac tumor, treated using an unusual approach Geeta Bhagia, Nasir Hussain, Fnu Arty, Mithun Chakravarthy, Moneal Shah Views: 186 HTML: 14 PDF: 125

  Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach.
  Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 x 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma.
  Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients.

• Catastrophic implantable cardioverter-defibrillator misclassification of ventricular tachycardia Zhafran Veliawan, Ardian Rizal, Indra Prasetya, Adhika Prastya Views: 227 HTML: 19 PDF: 126

  Inappropriate therapy is a frequent adverse consequence of implantable cardioverter-defibrillator. Inappropriate therapy often occurs due to the misinterpretation of sinus tachycardia or atrial fibrillation/flutter with rapid atrioventricular conduction by the device. Current implantable cardioverter-defibrillator (ICD) mechanisms integrate various discriminators into algorithms to differentiate supraventricular tachycardia (SVT) from ventricular tachycardia (VT), to prevent such occurrences. A 40-year-old man suffered seizures and cardiac arrest abruptly, without prior complaints of chest pain. Without delay, he initiated cardiopulmonary resuscitation (CPR), resulting in the regaining of spontaneous circulation. The patient had previously received a single-chamber ICD due to recurring VT and a prior episode of cardiac arrest. The patient had a medical background of coronary artery disease with complete revascularisation and no previous occurrence of SVT. Interrogating the ICD revealed captured non-sustained ventricular tachycardia (NSVT) and SVT events but no VT episode or shock therapy. During the specified time period, the patient underwent an electrophysiological study, and no SVT was induced with the normal function of the atrioventricular and sinoatrial nodes. Various causes can lead to errors in morphology discrimination criteria in single-chamber ICDs. Extending the detection interval is highly recommended to avoid misclassification of ICDs.

• Parachute mitral valve and mid-aortic syndrome – unusual associations of Alagille syndrome Geeta Bhagia, Nasir Hussain, Fnu Arty, Puneet Bansal, Robert Biederman Views: 235 HTML: 10 PDF: 126

  Background: Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome.
  Case description: Case 1: A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms.
  Case 2: A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13–14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management.
  Conclusion: PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.

• Delayed closure of ventricular septal defect with prolonged mechanical support Dawood Shehzad, Mustafa Shehzad, Muhammad Ahmad, Abdul Wassey, Noor Zara, Humna Younis, Haider Ali Babar Khan Views: 209 HTML: 20 PDF: 166

  Introduction: Ventricular septal defect (VSD) is a severe complication following acute myocardial infarction (MI) resulting from mechanical disruption of the interventricular septum due to extensive myocardial necrosis. Despite advances in management, the mortality rate approaches 50%. We report a case of a 58-year-old male with VSD following MI who was successfully treated with a delayed surgical approach after haemodynamic support using Impella.
  Case description: A 58-year-old man with type 2 diabetes mellitus and hypertension presented with three days of chest pain. Testing revealed late presenting acute anterior ischaemic infarction and left-to-right shunt in the apical ventricular septum. Urgent cardiac catheterisation showed near-total occlusion of the left anterior descending artery. An Impella CP^® was placed before angioplasty with a drug-eluting stent to optimise haemodynamics. After a multidisciplinary discussion, the Impella CP^® was upgraded to Impella 5.5^®, and surgery was delayed allowing for scar formation. The patient remained in the intensive care unit, where he underwent physical therapy, showing improvements in exercise tolerance by the time of surgery. He underwent a left ventriculotomy with a successful repair via an endocardial patch 28 days after initial presentation. Post-operative recovery was uneventful, with the patient discharged five days later, reporting no physical limitations one month post-discharge.
  Conclusion: The successful management of VSD post-MI relies on interdisciplinary collaboration, careful timing of surgical intervention and the strategic use of mechanical support devices such as the Impella. This case highlights the potential for favourable outcomes when tailored treatment approaches are employed.

• Left anterior descending coronary T-wave inversion pattern (Wellens’ syndrome) associated with myopericarditis and a normal left coronary artery Geeta Bhagia, Nasir Hussain, Fnu Arty, Victor Farah, Robert Biederman Views: 219 HTML: 12 PDF: 151

  Background: Wellens’ syndrome is characterised by a history of chest pain with an abnormal electrocardiogram (EKG), demonstrating biphasic or deeply inverted T waves in leads V2–3 (may extend to involve all precordial and lateral limb leads – the type B Wellens’ pattern). A Wellens’ EKG pattern is considered highly specific for critical stenosis involving the ostial/proximal left anterior descending artery (LAD). However, there are no reported cases of an association of a Wellens’ EKG pattern with myopericarditis. Here, we present such a rare case.
  Case description: A thirty-one-year-old female with known essential hypertension and psoriatic arthritis presented with a constant, central chest pain radiating to the shoulders and back. The patient’s physical examination was unremarkable at presentation other than elevated blood pressure at 170/68 mmHg. An EKG at presentation demonstrated deep symmetric T-wave inversions in anterolateral leads with elevated high-sensitivity troponin, and an elevated erythrocyte sedimentation rate. The patient was referred to the cardiac catheterisation laboratory for concerns of a Wellens’ EKG pattern; however, invasive angiography demonstrated only obtuse marginal branch disease – no LAD disease was noted. Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of myopericarditis and absence of myocardial infarction. The patient was medically managed and discharged home in a stable condition.
  Conclusion: In literature and established clinical practice, the Wellens’ EKG pattern is considered highly concerning for critical ostial/proximal LAD stenosis. However, we now propose that myopericarditis may be considered in a differential diagnosis for this EKG pattern.