European Heart Journal: Case Reports最新文献

Background: Diagnosing cardiac sarcoidosis (CS) is challenging. Immunosuppressive therapies are less effective in end-stage disease, and often heart transplant (HT) is the only available option. We present a series of advanced CS cases, requiring HT, along with a review of the literature evidence in this field.

Case summary: Case 1: a 49-year-old man initially suspected of having arrhythmogenic cardiomyopathy (ACM) presented with heart failure (HF) and recurrent ventricular arrhythmias. The rapid clinical deterioration raised suspicion of an inflammatory aetiology, which was confirmed through endomyocardial biopsy, diagnosing CS. Despite immunosuppressive therapy, HT was required. Case 2: a 36-year-old woman presented with high-grade atrioventricular block and dilated cardiomyopathy (DCM), initially diagnosed as idiopathic. Due to worsening HF, she required HT. The pathological examination of the explanted heart revealed CS. Chronic subclinical antibody-mediated rejection was observed after HT. Case 3: a 44-year-old man presented with syncope and imaging suggesting ACM. He was referred for HT due to high ventricular arrhythmic burden. Cardiac sarcoidosis diagnosis was suspected due to pulmonary involvement and then confirmed on post-explant pathological exam. Post-HT pulmonary and cutaneous sarcoidosis reactivation were observed. Case 4: a 43-year-old man was diagnosed with pulmonary sarcoidosis after lung biopsy. Progression towards DCM was observed despite immunosuppressive therapy. Post-HT was characterized by multiple episodes of graft rejection.

Discussion: This case series provides insights into mid- and long-term outcomes after HT for CS, highlighting the need for careful management of immunosuppression in these patients, balancing the adverse effects of chronic immunosuppression with the prevention of rejection and sarcoidosis recurrence.

Background: Brugada phenocopy (BrP) is a condition that induces reversible Brugada-like electrocardiographic (ECG) changes in patients without true Brugada syndrome. We present two cases of fulminant eosinophilic myocarditis that showed Type 1 Brugada ECG changes in the early phase of the clinical course.

Case summary: Case 1 was a 76-year-old man who developed fulminant eosinophilic myocarditis with ventricular tachycardia while hospitalized for heart failure. Case 2 was a 60-year-old man who presented with cardiogenic shock and was diagnosed with fulminant eosinophilic myocarditis. Both patients showed a Type 1 Brugada ECG at onset, and their ventricular function was greatly reduced. Regarding mechanical circulatory support, Case 1 was treated with venous-arterial extracorporeal membrane oxygenation and intra-aortic balloon pumping. Case 2 had venous-arterial extracorporeal membrane oxygenation and Impella CP insertion. Steroid therapy was introduced in both cases. In Case 1, the Type 1 Brugada ECG took 7 days to improve. Left ventricular function improved with time but right heart function was poor and right heart enlargement remained. In Case 2, the Type 1 Brugada ECG improved on the second day, and left and right heart function improved over time.

Discussion: We report two cases of fulminant eosinophilic myocarditis with Brugada-like ECG and severe right heart dysfunction. BrP in acute myocarditis may be an indicator of right heart failure and an important ECG marker in determining the indication for mechanical circulatory support and improvement of right heart function.

Background: Atrial standstill is characterized by the absence of atrial activity. We report a case of a patient with extensive atrial fibrosis who underwent electrophysiologic study (EPS) and electroanatomical mapping (EAM) to identify surviving atrial sites amenable for pacemaker lead implantation.

Case summary: A 72-year-old man with persistent atrial fibrillation (AF) and atrial functional mitral regurgitation/tricuspid regurgitation (MR/TR) underwent a Cox-Maze surgery, mitral and tricuspid valve repair, and biatrial plication. He was referred because of post-operative presyncope symptoms. Electrocardiography revealed atrial standstill and junctional rhythm (JR); however, EAM revealed that both atria were almost entirely scarred and isolated fibrillation in left pulmonary veins and coronary sinus. Junctional rhythm retrogradely conducted around an atrioventricular (AV) node and pacing at this area could conduct to the ventricle through the AV node. An atrial pacing lead was implanted at this area, which yielded a QRS wave similar to the own beat. However, the atrial lead voltage was quite low; hence, ventricular pacing lead was implanted to avoid a future occurrence of pacing failure.

Discussion: This report demonstrates the benefits of EPS and EAM in informing optimal pacemaker implantation for patients with extensive scar in atrium.

Background: Haemochromatosis is a pathological condition characterized by the accumulation of iron in parenchymal organs, leading to toxic damage and dysfunction. Cardiac haemochromatosis represents one of the rare causes of severe heart failure (HF) that can be potentially prevented with targeted treatment.

Case summary: We present the case of a 41-year-old female who was hospitalized for decompensated HF. Echocardiography revealed severe systolic dysfunction with a phenotype of dilated cardiomyopathy, accompanied by secondary moderate mitral regurgitation and severe tricuspid regurgitation (TR). To differentiate potential causes of HF, coronary angiography, cardiac magnetic resonance imaging (MRI), and endomyocardial biopsy were performed. Based on clinical findings, laboratory results, cardiac MRI, and endomyocardial biopsy data, a diagnosis of haemochromatosis was confirmed, and mutations in the TFR2 gene, responsible for haemochromatosis Type 3, were identified. The patient was treated in accordance with the latest European Society of Cardiology HF guidelines, and specific treatment for haemochromatosis, including therapeutic phlebotomy and iron chelation therapy, was initiated, resulting in a significant positive outcome.

Discussion: Investigating the aetiology of HF is essential, as even rare causes can be identified, and specific treatments are available that significantly improve prognosis and survival.

Background: Premature constriction of the ductus arteriosus (PCDA) makes management difficult in neonates with congenital heart defects, particularly those with ductal-dependent pulmonary circulation. This report highlights the challenges and management of a neonate diagnosed with tricuspid atresia and severe right ventricular outflow tract obstruction (RVOTO), complicated by PCDA.

Case summary: A male neonate was diagnosed prenatally with tricuspid atresia and severe RVOTO. After birth, his oxygen saturation was around 60%, and no ductus arteriosus was detected. A systemic-to-pulmonary shunt was placed emergently. After surgery, antegrade blood flow from the right ventricular outflow tract was unstable depending on the right ventricular muscle contraction and relaxation, and the antegrade blood flow needed to be occluded. The postoperative course was uneventful after then.

Discussion: This case underscores the complexity of managing neonates with tricuspid atresia, severe RVOTO, and PCDA. Early surgical intervention is critical in stabilizing such patients.

Background: Amyloidosis is a multi-organ disease of emerging significance in the field of cardiology. Chylothorax, a specific form of pleural effusion characterized by lymphatic fluid accumulation in the pleural cavity, is an extremely rare manifestation of amyloidosis. Notably, only five cases of chylothorax related to cardiac amyloidosis have been reported worldwide, all in amyloid light chain (AL) amyloidosis. No cases have been documented in amyloid transthyretin (ATTR) amyloidosis. Furthermore, elevated levels of serum carbohydrate antigen (CA) 125 have been associated with a poor prognosis in patients with AL cardiac amyloidosis.

Case summary: We report the case of an 85-year-old Austrian man with pronounced left ventricular hypertrophy, monoclonal gammopathy, and a history of atrial fibrillation. Further examinations, including myocardial biopsy, confirmed the diagnosis of ATTR cardiac amyloidosis. A significant right-sided pleural effusion was also observed. Thoracocentesis diagnosed chylothorax, confirmed by lymphangiography. Elevated CA 125 levels were found in both serum and pleural fluid, with no other findings suspicious for malignancy. The patient underwent a short break in oral anticoagulation and received prophylactic low-molecular-weight heparin for myocardial biopsy, thoracocentesis, and lymphangiography. However, they died a few days later due to an embolic stroke.

Discussion: At this time, we can only speculate about the pathomechanism of chylothorax development in the context of amyloidosis. We recommend further investigation of similar cases to deepen understanding of the underlying causes and identify potential treatment strategies. Additionally, the utility of CA 125 as a prognostic marker in ATTR amyloidosis needs further investigation.