Background:Early bioprosthetic mitral valve thrombosis (BPVT) is a rare but life-threatening cause of acute prosthetic obstruction after mitral valve surgery. Management is particularly challenging in postcardiotomy shock requiring veno-arterial extracorporeal membrane oxygenation (VA-ECMO), where low native cardiac output and left-sided stasis may promote thrombosis despite systemic anticoagulation. Case summary:A 74-year-old man underwent bioprosthetic mitral valve replacement, tricuspid annuloplasty, pulmonary vein isolation, and left atrial appendage closure for severe primary mitral regurgitation, with intra-operative identification of ascending aortic dissection requiring replacement of the ascending aorta and hemiarch. Because of severe postcardiotomy shock with biventricular dysfunction, peripheral VA-ECMO was initiated. On post-operative Day 2, transoesophageal echocardiography demonstrated severe bioprosthetic mitral obstruction with restricted leaflet motion and a mean transmitral gradient of 25 mmHg. Repeat imaging showed persistent obstruction with extensive left atrial thrombus despite anticoagulation optimization. Surgical reintervention and systemic fibrinolysis were considered prohibitive. After Heart Team discussion, rescue transseptal percutaneous mitral balloon valvuloplasty was performed under cerebral embolic protection using a 26-mm Inoue balloon. Immediate post-procedural imaging showed improved leaflet mobility and reduction of the mean transmitral gradient from 25 to 4 mmHg, without significant mitral regurgitation. The patient was successfully weaned from VA-ECMO and remained clinically stable at 2-month follow-up. Discussion:This case illustrates that, in carefully selected critically ill patients with catastrophic early BPVT during VA-ECMO support, rescue transseptal balloon dilatation may be considered as an exceptional salvage strategy when surgery and fibrinolysis are not viable. In highly selected patients with early BPVT causing severe haemodynamic compromise during VA-ECMO support, transseptal balloon dilatation may serve as a bridge-to-recovery option when surgery and fibrinolysis are contraindicated or carry prohibitive risk.
Background:Left main coronary artery (LMCA) aneurysms are exceedingly rare, found in ∼0.1% of coronary angiograms, and acute coronary syndromes are an uncommon first presentation. When the aneurysm involves the distal LMCA and its bifurcation, percutaneous covered-stent treatment is generally not feasible, leaving surgical and medical therapy as the only realistic options, with no controlled data to favour either. Case summary:We report two male patients presenting with non-ST-elevation myocardial infarction and thrombosed distal LMCA aneurysms. Both patients were evaluated by a multidisciplinary heart team but received divergent therapeutic strategies. Case 1 (44-year-old male) underwent surgical revascularization with coronary artery bypass grafting and aneurysm ligation following unsuccessful percutaneous recanalization of the left circumflex artery. Case 2 (65-year-old male) declined surgery and was managed medically with intensive antiplatelet and anticoagulation therapy. Both remained asymptomatic during three months of follow-up. Discussion:This case series underscores the diagnostic and therapeutic complexity of thrombosed distal LMCA aneurysms. Although surgical exclusion of the aneurysmal sac may, on anatomical grounds, be hypothesized to reduce the risk of recurrent thromboembolism, medical management remains a legitimate option in selected patients who decline or are unsuitable for surgery. The risk of recurrent thrombosis in medically managed patients remains incompletely characterized, and the short follow-up in our cases limits long-term conclusions. In the absence of randomized controlled trials, management decisions must be individualized through multidisciplinary heart team discussion, incorporating aneurysm size, morphology, thrombus burden, clinical presentation, and patient preferences.
Background:Transthyretin amyloid cardiomyopathy (ATTR-CM) coexists in up to 15% of elderly patients with severe aortic stenosis (AS), presenting complex diagnostic and therapeutic challenges due to overlapping clinical phenotypes. Case summary:An 85-year-old female presented with a 2-month history of heart failure and a previous medical history of total knee replacement. Echocardiography and computed tomography confirmed severe AS, alongside left ventricular hypertrophy out of proportion to the valvular load and an 'apical sparing' strain pattern. Due to the accompanying red flags, a Tc99m-PYP bone scintigraphy was done. This showed a Grade 3 cardiac uptake, and further testing confirmed wild-type ATTR-CM. Given her recent symptom onset, preserved ejection fraction, and high-gradient AS, the Heart Team prioritized transcatheter aortic valve implantation (TAVI). Following a successful procedure, she demonstrated excellent functional recovery at one month, which clinically justified the subsequent initiation of transthyretin stabilizers. Discussion:Concomitant ATTR-CM must be suspected in elderly AS patients presenting with disproportionate hypertrophy or other clinical/imaging 'red flags'. A sequential management strategy-prioritizing TAVI to relieve the haemodynamic bottleneck, followed by clinical reassessment-can effectively identify appropriate candidates for long-term, disease-modifying transthyretin stabilizer therapy.
Background:Combined mitral transcatheter edge-to-edge repair (M-TEER) and left atrial appendage occlusion (LAAO) may be considered in selected high-risk patients with severe mitral regurgitation (MR) and atrial fibrillation (AF). However, the optimal transseptal puncture site differs between procedures, potentially limiting the feasibility of a single-session approach. Case summary:An 80-year-old frail woman with severe atrial functional MR, permanent AF, chronic kidney disease, and recent severe anaemia was referred for treatment. Considering the patient's high thromboembolic and bleeding risk and overall frailty, a combined percutaneous approach was selected. A supero-posterior transseptal puncture optimized for mitral repair enabled successful M-TEER with significant MR reduction. Subsequent LAAO was challenged by suboptimal alignment with the appendage. The use of a steerable introducer restored coaxiality, allowing stable device delivery and successful deployment of a 24-mm occluder with complete sealing and no peri-device leak. Discussion:This case demonstrates the feasibility of a combined one-stop strategy in frail patients at high procedural risk. It highlights the importance of procedural planning and the role of steerable delivery systems in overcoming anatomical constraints imposed by a mitral-oriented transseptal puncture, thereby facilitating successful completion of both interventions in a single session.
Background:Fulminant myocarditis (FM) can rapidly progress to cardiogenic shock and cardiac arrest; however, the optimal timing of mechanical circulatory support (MCS) initiation remains uncertain, particularly regarding patients with rapidly progressive disease at risk of sudden haemodynamic deterioration. Case summary:A 57-year-old Asian man presented with chest pain following flu-like symptoms. On admission, he was hypotensive with elevated cardiac biomarkers and global left ventricular hypokinesia with a left ventricular ejection fraction of approximately 40%. Acute coronary syndrome was excluded by emergent coronary angiography. During evaluation in the cardiac catheterization laboratory, sustained ventricular tachycardia, rapidly worsening haemodynamic, and early signs of end-organ hypoperfusion indicated progressive cardiogenic shock with a high risk of further deterioration. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was therefore initiated based on these evolving clinical findings. Despite initial stabilization, the patient developed complete atrioventricular block followed by pulseless electrical activity, resulting in cardiac arrest within approximately 4 h of presentation. Combined support with VA-ECMO and Impella CP was established. Cardiac function gradually recovered, and the patient was successfully weaned from MCS. Endomyocardial biopsy confirmed lymphocytic myocarditis. Discussion:This case highlights the clinical importance of determining the optimal timing of MCS initiation in patients with suspected FM at high risk of rapid deterioration. Early support prior to further haemodynamic deterioration may stabilize circulation, facilitate diagnostic procedures, and provide a bridge to myocardial recovery.
Background:Triple M overlap syndrome is a rare, but often fatal, immune-related adverse event. Evidence on the effectiveness of immunosuppressive treatments, including abatacept, and disease monitoring in complex cases remains limited. Case Summary:A man in his 60s developed fulminant immune checkpoint inhibitor (ICI)-related myocarditis after nivolumab plus ipilimumab for recurrent lung adenocarcinoma, complicated by triple M overlap syndrome (myocarditis, myositis, and myasthenia gravis). Despite high-dose corticosteroids and intravenous immunoglobulin, cardiac biomarkers worsened, with progressive conduction abnormalities and a rapid decline in the left ventricular ejection fraction to 23%. Upon transfer to our institution, he developed sustained ventricular tachycardia, requiring direct current cardioversion and intra-aortic balloon pump support. Endomyocardial biopsy (EMB) at admission showed CD8+ T-cell-predominant myocarditis, consistent with a fulminant, steroid-refractory disease course. Abatacept was initiated after a multidisciplinary discussion, resulting in haemodynamic and arrhythmic stabilization. Guided by serial EMB findings of residual myocardial inflammation, abatacept was administered four times by hospital Day 30, along with intravenous methylprednisolone pulse therapy, plasma exchange, intravenous immunoglobulin, and tacrolimus. Left ventricular ejection fraction gradually improved to 56%, and a follow-up EMB on Day 88 demonstrated marked histopathological recovery. Discussion:This case illustrates that abatacept-based multidisciplinary therapy may help stabilize fulminant steroid-refractory ICI-related myocarditis with triple M overlap syndrome. Serial EMB provided a histopathological assessment of residual myocardial inflammation and guided immunosuppressive therapy, suggesting a potential role for serial biopsy in complex cases.
Background:Penetrating cardiac trauma is rare in adolescents, and late acute coronary syndrome after surgical repair, which narrows left anterior descending artery, is even rarer. Managing ischaemia from late coronary stenosis without underlying atherosclerosis is challenging. Multimodal imaging, including coronary computed tomography and intracoronary imaging, is crucial for guiding treatment. Case summary:An 18-year-old male presented with recurrent chest pain. Two years after surgical repair of a penetrating left ventricular injury, a 12-lead electrocardiogram showed deep, symmetrical T-wave inversions in the anterolateral leads. Coronary angiography revealed a myocardial bridge and a significant stenosis in the mid left anterior descending artery (LAD). After recurrent symptoms, coronary computed tomography angiography demonstrated repair material surrounding the stenotic segment. Myocardial perfusion imaging showed a small fixed defect without reported reversibility. Intravascular ultrasound (IVUS) showed a minimum lumen area of 1.85 mm2 without an atherosclerotic phenotype. Following individualized Heart Team review favoring a percutaneous approach, a drug-eluting stent was implanted; the final minimum stent area was 4.83 mm2. At 1 month, exertional chest pain had markedly improved, biomarkers were normal, and the electrocardiogram was unchanged. Discussion:This case may represent one of the initial documented instances of IVUS-guided percutaneous coronary intervention (PCI) for late LAD compression caused by mechanical factors, following pledget repair for penetrating cardiac trauma in a young patient. Multimodal imaging helped clarify the underlying mechanism of this non-atherosclerotic coronary lesion, and a multidisciplinary Heart Team approach directed the personalized treatment decision.
Background:Erdheim-Chester disease (ECD) is a rare clonal non-Langerhans cell histiocytosis driven predominantly by MAPK pathway alterations. Although cardiovascular involvement is frequent, it is usually subclinical, while constrictive pericarditis is exceptionally rare. Case summary:A 57-year-old man with multisystem ECD presented with signs of congestive heart failure. Multimodality imaging demonstrated extensive cardiovascular involvement, with marked pericardial thickening and haemodynamic features of constrictive pericarditis on echocardiography, diffuse late gadolinium pericardial enhancement on cardiac magnetic resonance, and circumferential periarterial infiltration on computed tomography angiography. Despite intensive medical therapy resulting in temporary haemodynamic improvement, persistent constrictive physiology prompted urgent pericardiectomy. Intraoperatively, the pericardium was diffusely thickened and densely adherent to the epicardium. The post-operative course was complicated by refractory cardiogenic shock and multiorgan failure, leading to the patient's death. Autopsy revealed extensive histiocytic infiltration of the pericardium and myocardium, demonstrating that extensive cardiovascular involvement in ECD, rather than isolated pericardial fibrosis, was causative for the constrictive physiology. Discussion:This case illustrates the potentially devastating consequences of cardiovascular involvement in ECD. It suggests that once extensive myocardial infiltration by histiocytes additional to pericardial fibrosis has evolved, pericardiectomy alone may be insufficient to relieve the constrictive physiology and alter clinical course.
Background:Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a multisystemic disorder that can present with diverse clinical features, including cardiomyopathy and chronic intestinal pseudo-obstruction. However, diagnosing MELAS can be challenging when its manifestations overlap with other genetic syndromes, leading to diagnostic anchoring and potential mismanagement. Case summary:A 51-year-old woman with mosaic Turner syndrome presented with concentric left ventricular hypertrophy and mildly reduced systolic function (left ventricular ejection fraction 45%). Her medical history included childhood-onset hearing loss, hypothyroidism, and recurrent intestinal obstruction of unknown etiology, which had been previously attributed to Turner syndrome or its comorbidities. Despite resolution of bowel symptoms during prior hospitalizations, she exhibited persistent hyperlactataemia (4.55 mmol/L), a diagnostic red flag. Cardiac magnetic resonance imaging showed patchy mid-wall late gadolinium enhancement, suggesting a non-ischaemic process. Family history revealed a maternal pattern of cardiomyopathy and stroke-like episodes. Genetic analysis confirmed the m.3243A > G mitochondrial DNA mutation (heteroplasmy 23%), establishing a diagnosis of MELAS. Earlier recognition of mitochondrial dysfunction might have avoided a previous unnecessary laparotomy performed for suspected intestinal ischaemia. Discussion:This case illustrates the clinical challenge of phenotypic masking; features of Turner syndrome masked the underlying MELAS, resulting in a significant diagnostic delay. The coexistence of unexplained cardiomyopathy, recurrent pseudo-obstruction, and persistent hyperlactataemia should prompt consideration of mitochondrial disease, even in patients with an established genetic diagnosis. Clinicians must remain vigilant for multisystemic 'red flags' to avoid diagnostic anchoring and ensure appropriate metabolic and genetic evaluation.
Background:Renal denervation (RDN) is an emerging therapy for selected patients with resistant hypertension. Its broader autonomic effects may extend beyond blood pressure regulation. Case summary:A 57-year-old man with resistant hypertension treated with six antihypertensive drugs and recurrent autonomic and generalized seizures underwent radiofrequency RDN. At 3 months, ambulatory blood pressure monitoring showed sustained improvement (24 h mean 139/87 mmHg), enabling therapy de-escalation. Generalized tonic-clonic seizures resolved, and autonomic/focal events markedly decreased without changes in antiepileptic medication. Discussion:This case illustrates a potential extrapressor effect of RDN mediated by modulation of sympathetic overactivity. Although causality cannot be drawn from a single case, the parallel neurological improvement supports exploring RDN's role in conditions involving autonomic dysregulation, including certain epileptic syndromes.
Background:Cardiac tumours are exceedingly rare and often present with non-specific clinical manifestations, making diagnosis challenging. Primary cardiac lymphoma represents a small proportion of primary cardiac tumours and may occasionally mimic acute coronary syndromes due to mass effect on coronary arteries or the conduction system. Case summary:We report the case of a 61-year-old woman admitted for syncope who subsequently developed chest pain, inferior ST-segment elevation myocardial infarction, and complete atrioventricular block. Emergency coronary angiography revealed no obstructive coronary disease but demonstrated extrinsic compression and stiffness of the right coronary artery. Transthoracic echocardiography identified a large right ventricular mass with tricuspid valve involvement and right ventricular inflow obstruction, associated with pericardial and pleural effusion. Multimodality imaging with computed tomography and positron emission tomography showed a large hypermetabolic cardiac mass with mediastinal lymphadenopathy. Histological analysis from a computed tomography-guided biopsy confirmed primary cardiac high-grade B-cell lymphoma. Chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone was initiated, leading to significant tumour reduction but complicated by transient chemotherapy-induced left ventricular dysfunction, which was managed through a multidisciplinary cardio-oncology approach. Despite initial clinical and cardiac improvement, the patient later developed central nervous system progression and died 10 months after diagnosis due to therapy-related neurotoxicity. Discussion:This case highlights a rare presentation of primary cardiac lymphoma manifesting as acute coronary syndrome and atrioventricular block secondary to extrinsic coronary compression. It underscores the importance of multimodality imaging, histological confirmation, and adherence to European Society of Cardiology cardio-oncology guidelines. Early multidisciplinary management is essential to balance effective oncological treatment with cardiovascular safety.
Background:Acute Anrep effect describes transient afterload-dependent increase in myocardial contractility with the aim of maintaining a stroke volume during physical exertion. Sustained activation of the Anrep effect has recently been identified as a compensatory mechanism in afterload-driven diseases, such as hypertrophic obstructive cardiomyopathy or aortic stenosis. The aim of this case presentation was to show whether the Anrep effect can also be chronically activated and reversed in the right heart in a patient with pulmonary stenosis. Case summary:A 30-year-old female patient with chronic exertional dyspnoea (New York heart association II) and suspected supravalvular pulmonary stenosis was referred to our hospital for further diagnosis and treatment. Echocardiography revealed a borderline hypertrophied right ventricle with a gradient of more than 100 mmHg across the pulmonary valve. However, it was not possible to distinguish between valvular and supravalvular stenosis. Pulmonary stenosis with characteristic bulging of the valve could finally be diagnosed by transoesophageal echocardiography. Subsequently, a successful balloon valvuloplasty of the pulmonary valve was performed, which significantly reduced the maximum pressure gradient from 70 to 19 mmHg. Detailed pressure-volume analyses demonstrated distinctive increased contractility (end-systolic elastance = 1.47 mmHg/mL), increased afterload effective arterial elastance, prolonged systole duration (480 ms), and increased stroke work prior to valvuloplasty consistent with the Anrep effect. After successful intervention, the Anrep effect abruptly reversed characterized by reduction of afterload, contractility, and systolic duration. Conclusion:This case report of severe pulmonary stenosis identified a possible contribution of the chronic Anrep effect as a regulator of right ventricular contractility and energy expenditure before and after successful pulmonary valve implantation.
Background:Coronary artery fistulas are rare coronary anomalies that can occasionally lead to thrombotic complications and myocardial infarction. Their diagnosis and management remain challenging due to variable anatomy and low prevalence. Case summary:A 29-year-old man presented with inferior ST-segment elevation myocardial infarction 5 days after orthognathic surgery. Coronary angiography revealed a thrombotic occlusion of the distal right coronary artery (RCA) and a thrombotic fistula arising from the proximal RCA. Successful stenting restored distal flow. Cardiac computed tomography angiography confirmed a RCA-superior vena cava fistula containing thrombus. The patient received dual antiplatelet therapy and anticoagulation. Follow-up imaging at 3 months showed a patent RCA stent and persistent occlusion of the fistula. Discussion:This case illustrates that coronary artery fistulas, though rare, can lead to acute myocardial infarction in young adults. Coronary computed tomography angiography is essential for anatomical assessment, and heart team discussion is key for individualized management.
Background:Cardiac angiosarcoma, a rare and highly malignant primary cardiac tumour with poor prognosis, often presents diagnostic challenges owing to nonspecific clinical manifestations, with some cases initially presenting with acute pericarditis. Minoxidil, a commonly used treatment for androgenetic alopecia (AGA), has been associated with drug-induced pericarditis. Case summary:A 23-year-old man with no significant medical history presented with chest pain at rest and pericardial effusion. The patient had been taking 5 mg/day of oral minoxidil for AGA; thus, drug-induced pericarditis was initially suspected. His symptoms improved with aspirin and colchicine. However, he experienced recurrent episodes of pericarditis. On the third presentation, echocardiography revealed cardiac tamponade, and pericardiocentesis showed haemorrhagic pericardial effusion with elevated tumour markers. Imaging studies supported the suspicion of a primary cardiac malignancy. Surgical biopsy confirmed the diagnosis of cardiac angiosarcoma. The patient underwent extensive tumour resection followed by adjuvant chemotherapy and proton beam therapy and was discharged without evidence of recurrence. However, metastatic recurrence subsequently developed, and he died 30 months after surgery. Discussion:This case highlights the importance of considering malignancy in the differential diagnosis of recurrent pericarditis in young adults. Although minoxidil-related pericarditis was initially suspected, multimodal imaging and histopathological evaluation led to the diagnosis of cardiac angiosarcoma. Early identification and multidisciplinary management are essential in such rare presentations to improve patient outcomes.
Background:Pulsed field ablation (PFA) is a novel non-thermal ablation modality that has demonstrated efficacy and safety in the treatment of common arrhythmias such as atrial fibrillation (AF) and atrial flutter (AFL). However, evidence regarding its use in patients with dextrocardia remains scarce. Case summary:A 63-year-old woman with dextrocardia and situs in vs. totalis, and with prior surgical atrial septal defect (ASD) repair, presented with heart palpitations that did not respond to antiarrhythmic drugs. Ambulatory and Holter monitoring documented short episodes of AF and AFL. Computed tomography (CT) of the heart and its structures before the procedure enabled anatomical orientation and planning of the procedure. Catheter ablation was performed using a Pentaspline PFA system (FARAPULSE, Boston Scientific) under fluoroscopic guidance without electroanatomical mapping. Pulmonary vein isolation, along with ablation of the left atrial roof, posterior wall of the left atrium, and cavotricuspid isthmus, was successfully achieved. The procedure was successful, and no recurrence of arrhythmia was observed on 6-month Holter monitoring. Discussion:This case demonstrates that combined PFA for AF and AFL is feasible in patients with complex congenital anatomy, including dextrocardia following ASD repair. Mirror-image cardiac anatomy presents unique challenges for catheter navigation and anatomical orientation. Pre-procedural cardiac CT was essential for procedural planning and accurate spatial orientation, facilitating successful PFA in this anatomically complex setting.
Background:Cardiac sarcoidosis remains a major diagnostic challenge when myocardial inflammation occurs in the absence of clinically active extracardiac disease. In this setting, non-invasive tissue characterization and metabolic imaging may provide critical incremental value over conventional structural assessment. Case summary:A 62-year-old woman with progressive exertional dyspnoea and long-standing ventricular ectopy underwent multimodality evaluation. Echocardiography showed mild biventricular systolic dysfunction, reduced global longitudinal strain, and focal basal-to-mid septal abnormalities. Coronary computed tomography (CT) angiography excluded obstructive coronary artery disease and showed inactive pulmonary granulomatous sequelae. Cardiac magnetic resonance demonstrated mild-to-moderate biventricular dysfunction and patchy non-ischaemic late gadolinium enhancement in the basal-to-mid septum with elevated native T1 values but no T2 evidence of oedema. 18F-fluorodeoxyglucose positron emission tomography/CT subsequently revealed focal and multifocal myocardial uptake on a suppressed background, confirming active myocardial inflammation without extracardiac hypermetabolic disease. Immunosuppressive therapy was initiated, and guideline-directed heart failure therapy was optimized. Discussion:This case illustrates how stepwise multimodality imaging can establish the diagnosis of active cardiac sarcoidosis, provide precise non-invasive tissue characterization, distinguish active inflammation from fibrosis, and directly guide immunosuppressive treatment when extracardiac disease is clinically silent.
