European Heart Journal: Case Reports最新文献

Background: Transcatheter tricuspid valve repair is an increasingly performed procedure, which improves quality of life in selected patients with severe tricuspid regurgitation (TR). For the evaluation of the appropriateness of this procedure, identification of the exact mechanism(s) underlying TR is mandatory. Guidelines recommend right heart catheterization (RHC) as part of the work-up in these patients.

Case summary: A 80-year-old man with shortness of breath, atrial fibrillation, and severe TR was referred for transcatheter repair. Echocardiography showed a preserved left ventricular ejection fraction, a dilated right ventricle with impaired function, severe functional TR, and a high probability of pulmonary hypertension (PH). Right heart catheterization revealed a mean pulmonary artery (PA) pressure of 34 mmHg and a mean PA wedge pressure (mPAWP) of 18 mmHg. The PA oxygen saturation was 79%, and an anomalous vessel draining into the superior vena cava with an oxygen saturation of 97% was found, which was identified as partial anomalous pulmonary venous return with a left-to-right shunt (ratio of pulmonary to systemic blood flow of 1.8:1.0). The increased pulmonary blood flow and a mildly elevated pulmonary vascular resistance of 2.3 Wood units resulted in an increased transpulmonary gradient, which added to an elevated mPAWP (heart failure with preserved ejection fraction) led to combined pre- and post-capillary PH.

Discussion: This case highlights the importance of RHC in the evaluation of patients with severe TR. Here, TR was the result of right ventricular volume and pressure overload with very limited treatment options, and percutaneous repair was not appropriate.

Background: Left bundle branch area pacing (LBBAP) is a physiological pacing strategy considered safe in non-obstructive hypertrophic cardiomyopathy (HCM). Its effect on left ventricular outflow tract (LVOT) obstruction in anatomically predisposed patients remains unclear.

Case summary: A 76-year-old woman underwent LBBAP for atrioventricular nodal dysfunction. One week later, she developed chest pain and elevated troponin, despite unobstructed coronaries. Echocardiography revealed a resting LVOT gradient of 112.6 mmHg. Bipolar low-voltage pacing worsened the gradient, while unipolar high-voltage pacing reduced it to 15 mmHg, with symptom relief and improved global longitudinal strain.

Discussion: This case illustrates a rare interaction between pacing configuration and LVOT dynamics. It emphasizes the need to recognize pacing-induced obstruction, particularly in patients with septal hypertrophy or mitral anomalies, and to adjust programming accordingly.

Background: Isolated unilateral agenesis of the pulmonary artery (UAPA) is an exceptionally rare congenital anomaly. In adults, it may promote structural remodelling and predispose to atrial fibrillation (AF). Pulmonary vein (PV) isolation in UAPA poses unique challenges due to anatomical variations, hypoplastic pulmonary veins (PVs), and altered venous drainage, potentially reducing ablation efficacy.

Case summary: An 80-year-old woman with persistent AF and heart failure, and left UAPA, underwent PV isolation using a size-adjustable cryoballoon (POLARx FIT, Boston Scientific, Marlborough, MA, USA). Computed tomography demonstrated the absence of the left pulmonary artery and systemic collateral supply to the left lung. Left superior PV (LSPV) occlusion with a 31-mm cryoballoon achieved complete contrast seal (nadir -56°C) but failed electrical isolation. A subsequent 28-mm application with similar occlusion and nadir temperature achieved isolation with a prolonged time-to-isolation of 97 s. The hypoplastic left inferior PV (LIPV) was not ablated to avoid stenosis risk. During a second procedure, LSPV reconnection and residual LIPV potentials were detected. Ethanol infusion into the vein of Marshall (VOM) successfully isolated both veins.

Discussion: In UAPA, extensive systemic collateral circulation to the affected lung may cause heat dissipation ('heat sink effect'), limiting the efficacy of thermal energy delivery. Moreover, hypoplastic PVs carry an increased risk of stenosis after thermal ablation. Additional non-thermal lesion-creation techniques, such as VOM ethanol infusion, may be necessary to achieve durable and safe isolation when conventional thermal methods are insufficient.

Background: Severe functional tricuspid regurgitation (TR) in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVC) represents a challenging clinical entity, often complicated by progressive right ventricular (RV) dysfunction and limited interventional options.

Case summary: We report the first worldwide case of successful transcatheter tricuspid valve replacement (TTVR) with the EVOQUE™ system in a 37-year-old patient with ARVC, severe TR, and a cardiac resynchronization therapy defibrillator (CRT-D), following failed transcatheter edge-to-edge repair (TEER). The procedure resulted in immediate elimination of TR and the patient experienced marked symptomatic improvement.

Discussion: This case highlights the feasibility of TTVR in complex RV pathology, underscoring procedural considerations such as lead-valve interaction, risk of afterload mismatch, and prevention of right heart failure. TTVR with the EVOQUE™ system is feasible in selected patients with ARVC, severe functional TR, prior failed repair, and existing CRT-D leads. Success depends on meticulous pre-procedural planning, intra-procedural imaging, and vigilant haemodynamic management to mitigate RHF risk. This case broadens the spectrum of structural interventions in patients with ARVC and symptomatic TR not suitable for surgery or TEER and supports consideration of TTVR as a bridge-to-transplant strategy.

Background: Carcinoid heart disease (CHD) is a known complication of advanced functional neuroendocrine tumours (NETs), almost exclusively affecting right-sided cardiac valves. Left-sided involvement is rare and usually attributed to intracardiac shunting or pulmonary sources of serotonin. This case report highlights a rare presentation of CHD involving left- and right-sided valves in the absence of an anatomic shunt or bronchopulmonary NET.

Case summary: A 67-year-old woman with a long-standing, functional, small bowel Grade 1 NET and metastatic liver and peritoneal disease presented with worsening dyspnoea and peripheral oedema. She had a 10-year disease history, previously managed with debulking surgery, somatostatin analogues, peptide receptor radionuclide therapy (PRRT), and everolimus. Echocardiography and cardiac magnetic resonance imaging demonstrated extensive left- and right-sided valvular involvement with severe mitral regurgitation, moderate aortic and pulmonic regurgitation, and mild tricuspid regurgitation, yet without intracardiac shunt. An elevated Qp/Qs ratio of 2.6 was attributed to severe left-sided valvular regurgitation. The patient improved on diuretic therapy and was referred for surgical evaluation of valve replacement prior to additional systemic treatment.

Discussion: This case illustrates an atypical presentation of bilateral carcinoid valvulopathy in the absence of intracardiac shunting, likely due to overwhelming systemic serotonin from tumour burden. Although serotonin is typically inactivated in the lungs, extensive exposure may surpass this protective mechanism. The potential role of selective serotonin reuptake inhibitors remains inconclusive. Multidisciplinary coordination is essential for optimizing cardiac and oncologic outcomes, especially when systemic therapy such as PRRT is considered. Left- and right-sided CHD may develop in patients with small bowel NETs even without anatomic shunting. High tumour burden and systemic serotonin exposure may override pulmonary inactivation, leading to left-sided involvement. Early recognition and multidisciplinary care are critical for effective management.