European Heart Journal: Case Reports最新文献

Background: Granulomatosis with polyangiitis (GPA) is an autoimmune multisystem disorder characterized by small vessel vasculitis with granulomatous inflammation. In this report, we describe a unique case of GPA who presented with complete heart block (CHB) and developed complications due to intracranial large vessel involvement.

Case summary: A 47-year-old gentleman presented with CHB with a background history of arthralgia and blood-tinged nasal discharge. Whole body positron emission tomography-computed tomography scan showed soft tissue thickening with increased fluorodeoxyglucose uptake in basal interventricular septum and mitral leaflet aorta from the root up to the renal arteries. The patient developed subarachnoid haemorrhage and stress-induced cardiomyopathy after pacemaker implantation. The patient responded dramatically to steroids and rituximab and the CHB resolved on follow-up.

Discussion: Cardiac involvement in GPA is very rare as is the large vessel involvement. In this report, we describe the cardiac involvement of GPA in the form of basal interventricular septum and anterior mitral leaflet giving rise to CHB. The patient also had aortitis and vertebral artery aneurysm, which ruptured resulting in subarachnoid haemorrhage. The patient also developed stress-induced cardiomyopathy and monomorphic ventricular tachycardia. The patient improved with steroids and rituximab and is doing well on follow-up.

Background: This is a case report of a patient with Fabry disease (FD). We successfully treated a patient with ventricular septal hypertrophy and left ventricular outflow tract (LVOT) obstruction caused by FD. We report our exclusive new surgery for patients with LVOT obstruction, percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) procedure™ (percutaneous intramyocardial septal radiofrequency ablation). The patient recovered well 7 months after operation, and no LVOT obstruction and arrhythmia occurred.

Case summary: A 51-year-old woman with chest tightness after satiety for ∼10 years, aggravated for 2 months. The patient had a history of multiple syncope, syncope after activity or emotional excitement. Echocardiogram demonstrated ventricular septal thickness of 22 mm by the report. Genetic testing revealed gene mutation, which was consistent with FD (GLA: c.643A > C). Biochemical evaluation reported reduced serum α-galactosidase A activity and abnormal serum Lyso-GL-3 enzyme activity. The patient and her families decided to perform PIMSRA operation. After the operation, the septal thickness steadily decreased to 16 mm over the course of 7 months. However, the patient's symptoms improved significantly.

Discussion: This is the first case of cardiac involvement caused by FD mimicking hypertrophic cardiomyopathy treated with the innovative PIMSRA procedure. Percutaneous intramyocardial septal radiofrequency ablation procedure can be one of the effective treatments to alleviate cardiac hypertrophy and left ventricular obstruction in FD.

Background: Cardiac lymphoma is a rare disease that can present in various ways. Additionally, atypical clinical presentation makes the diagnosis even more challenging. The most common type of cardiac lymphoma is diffuse large B-cell lymphoma. With chemotherapy, the median survival rate can be up to 2 years. In this report, we focus on the diagnostic approach and differential diagnosis.

Case summary: A 56-year-old patient presented with complete heart block and B-symptoms. Implantation of a pacemaker (PM) was initially deferred due to a junctional rhythm (50 b.p.m.). Echocardiography showed thickening of the left heart with small pericardial effusion. For better visualization of the extent and infiltration cardiac magnetic resonance imaging and computer tomography (CT) were performed. In addition to the cardiac mass, enlarged mediastinal lymph nodes were found on staging CT scan, prompting a transbronchial biopsy. Histology revealed diffuse large B-cell non-Hodgkin lymphoma. The patient was referred to a hospital with a Hemato-oncology Department for initiation of chemotherapy.

Discussion: Cardiac lymphoma can be a rare cause of complete heart block. Prior to PM implantation, basic echocardiography is important. In rare conditions like cardiac lymphoma, multimodal imaging, and interdisciplinary decision-making are crucial for management. In the future, lead-less pacemakers could be a safe and effective option for oncology patients.

Background: Immune checkpoint inhibitors (ICIs) are effective antineoplastic agents but can cause adverse effects in many organ systems. Cardiovascular toxicities include arrhythmias, myocarditis, heart failure, takotsubo syndrome, pericarditis, coronary artery disease, and vasculitis.

Case summary: A 66-year-old woman with Stage 3C2 endometrial carcinoma presented for her second cycle of pembrolizumab, carboplatin, and paclitaxel. She subsequently suffered cardiac arrest and was brought to the emergency department. Spontaneous circulation returned following resuscitation, but she was haemodynamically unstable. An electrocardiogram revealed complete heart block. Initial management included intubation, vasopressor support, and transcutaneous pacing before transfer to the catheterization lab. Coronary angiography revealed no coronary artery disease. Right heart catheterization confirmed severe cardiogenic shock despite inotropic support and a temporary transvenous pacemaker in place. A micro-axial flow pump (Impella CP) was implanted for deteriorating cardiogenic shock. She was treated with high-dose corticosteroids (dexamethasone 190 mg i.v.) for suspected ICI-associated myocarditis, with significant improvement in cardiac function. The Impella was weaned and removed on Day 5. Cardiac magnetic resonance imaging showed elevated T1 and T2 signal intensities, consistent with the 2018 Lake Louise Criteria for myocarditis. The complete heart block was resolved, but a leadless pacemaker was implanted due to pre-existing conduction abnormalities.

Discussion: Early recognition of ICI-associated myocarditis can be achieved with biochemical testing, electrocardiography, imaging, and expedited investigation of alternative causes for cardiac decompensation. Our case demonstrates that temporary left ventricular assist devices can support cardiac output for patients in cardiogenic shock due to ICI-associated myocarditis, allowing for recovery following high-dose corticosteroids.

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect (reported incidence of 0.5%-2%) and is commonly associated with proximal aortic dilation. Patients with severe aortic stenosis (AS) of BAV have been shown to have worse pre-operative left ventricular (LV) function as well as a higher incidence of post-operative heart failure hospitalization when compared with analogous patients with tri-leaflet aortic valve disease. While surgical aortic valve replacement (SAVR) may be favoured over transcatheter aortic valve implantation (TAVI) due to anatomical factors or concomitant aortopathy and coronary artery disease, surgical candidacy is often limited by prohibitive operative risk.

Case summary: We report on three cases of severe AS in BAV with concomitant aortopathy and severe left ventricular dysfunction in whom we proceeded with SAVR with a priori planned venoarterial extracorporeal membrane oxygenation (VA-ECMO) support and inotrope-assisted wean. All patients had severe LV dysfunction (ejection fraction < 25%) at baseline with gradual substantial improvement or normalization after successful SAVR.

Discussion: These cases demonstrate the utility of planned VA-ECMO with SAVR and aortic root replacement as an integral component of the operative strategy for high surgical risk patients with severe BAV AS not amenable to TAVI. Appropriate pre-operative planning and consent for VA-ECMO as well as a multi-disciplinary approach involving anaesthesia, intensive care, and heart failure cardiology are the key to offering this option as an alternative to palliative medical therapy to a selected group of patients.

Background: Mitral and tricuspid regurgitation in patients with cardiac amyloidosis (CA) pose significant diagnostic and therapeutic challenges due to its non-specific symptoms and limited treatment options. Transcatheter edge-to-edge repair (TEER) is complicated by altered cardiac geometry, advanced restriction, and potential amyloid valve deposits.

Case summary: We present the case of dual TEER in a 79-year-old male with advanced transthyretin cardiac amyloidosis (ATTR-CA) and severe symptomatic mitral and tricuspid regurgitation. In a staged approach, TEER for both the mitral and tricuspid valves was successfully conducted, resulting in improved valvular function and symptom relief. Transvalvular gradients were 5 mmHg for mitral valve and 2 mmHg for tricuspid valve, each with mild residual regurgitation, improved clinical status, and regressive natriuretic peptides.

Discussion: This case underscores the feasibility of dual TEER in CA patients with valvular involvement. Further research is necessary to optimize treatment strategies and address the multifaceted nature of this complex disease.

Background: The coronary sinus reducer (CSR) is a therapeutic option for patients with coronary artery disease who are not eligible for further revascularization and experience refractory angina. Cardiac resynchronization therapy (CRT) improves symptoms and prognosis in heart failure with reduced ejection fraction, but the presence of a CSR may complicate left ventricular lead placement. Only four cases have been reported so far in this context. This case report introduces a novel technique for left ventricular lead implantation in such patients.

Case summary: A 73-year-old man with a history of chronic coronary syndrome, coronary artery bypass surgery, angioplasties, and CSR implantation presented with heart failure symptoms. His ECG showed sinus rhythm and left bundle branch block (QRS 160 ms), and echocardiography revealed severe systolic dysfunction (ejection fraction 20%). During Cardiac Resynchronization Therapy-Defibrillator (CRT-D) implantation, venography revealed a suitable proximal tributary branch near the CSR, which was successfully used for lead placement without complications.

Discussion: The CSR has shown promise in relieving refractory angina. As ischaemic heart disease progresses, CRT may become necessary in patients with CSR. The CSR's design can reduce the vascular lumen and complicate lead placement. Previous reports have confirmed the technical feasibility of CRT with CSR but lack long-term safety data. This case highlights that, under favourable anatomical conditions, proximal tributaries of the coronary sinus can be used for lead placement, potentially avoiding complications from reduced venous outflow.

Background: Transcatheter aortic valve replacement (TAVR) is a well-established treatment option for patients with severe aortic valve stenosis; however, clinical valve thrombosis is a major challenge.

Case summary: A 92-year-old woman underwent TAVR for severe aortic stenosis. One month later, the patient developed acute heart failure. As the progression of anaemia due to renal anaemia seemed to cause acute heart failure exacerbation, we started an oral hypoxia-inducible factor prolyl hydroxylase (HIF-PH) inhibitor. After 2 weeks, the patient redeveloped shortness of breath. Transthoracic echocardiography revealed that the mean aortic valve pressure gradient (ΔP) increased from 9 to 54 mmHg, and the aortic valve area decreased from 1.93 to 0.86 cm². Blood work revealed a markedly elevated haemoglobin level from 8.0 to 13.2 g/dL, and transoesophageal echocardiography revealed markedly decreased left coronary and non-coronary cusp mobility. We diagnosed that the rapid increase in the haemoglobin level caused by the HIF-PH inhibitor was related to valve thrombosis and bioprosthetic dysfunction of the transcatheter aortic valve. The HIF-PH inhibitor was discontinued, and anticoagulation therapy was started. Transthoracic echocardiography at 16 days later revealed that the mean aortic valve ΔP improved by 15 mmHg, and the subjective symptoms resolved.

Discussion: This is the first report on a successful treatment of TAVR thrombosis formation associated with HIF-PH inhibitor use. When treating renal anaemia in patients undergoing TAVR, care should be taken to avoid rapid anaemia resolution and valve thrombosis development.

Background: In patients with adult congenital heart disease (ACHD), significant atrioventricular valve regurgitation is an important risk factor for poor outcomes, such as heart failure. However, in many cases, transcatheter intervention may reduce the risk profile to avoid a high surgical risk.

Case summary: A 44-year-old man with complex ACHD in the form of a double-inlet left ventricle, congenitally corrected transposition of the great arteries, pulmonary atresia, atrial septal defect, and patent ductus arteriosus was referred for the treatment of severe tricuspid regurgitation. He received an aortopulmonary shunt and a left-sided modified Blalock-Taussig shunt during childhood. Because of the patient's high surgical risk due to seroma formation around the two shunts and intra-mediastinal collateral vessels, the heart team opted for transcatheter edge-to-edge repair (TEER) using a MitraClip (Abbott Vascular, Santa Clara, CA, USA). Tricuspid TEER was successfully performed using the MitraClip G4 system. The postoperative course was uneventful, with significant improvements in the New York Heart Association functional class.

Discussion: Our case demonstrates that tricuspid TEER can be an alternative option for patients with complex ACHD who are at high risk for conventional surgeries; however, careful assessment with multimodality imaging and a heart team approach, including a cardiologist, ACHD specialist, cardiac surgeon, anthologist, and intensivist, should be considered.

Background: ROS1 tyrosine kinase inhibitors are one of the primary immunotherapies for ROS1 fusion-positive cancers. Tyrosine kinase inhibitors have markedly improved outcomes for advanced cancers previously with poor prognosis. Entrectinib is an example of an ROS1 inhibitor that can be used for lung adenocarcinoma. There are numerous adverse effects with rare cardiac side effects reported, such as heart failure and myocarditis.

Case summary: A 27-year-old male being treated for lung adenocarcinoma who presented new congestive heart failure 2 weeks after starting Entrectinib. He developed refractory ventricular tachycardia, cardiogenic shock with an endomyocardial biopsy that showed active lymphohistiocytic myocarditis. With antiarrhythmic therapy, heavy sedation, mechanical circulatory support, and high-dose steroids, the patient had complete resolution of symptoms and return to baseline status.

Discussion: This is a rare case with a severe complication after starting Entrectinib for lung adenocarcinoma. In the literature, this is the first case of its kind presenting with myocarditis and severe heart failure after treatment with Entrectinib. This case highlights not only using cardiac imaging, and biopsy to help guide the diagnosis, but also describe the appropriate management.