Background/Objectives: In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), the effect of tafamidis on right ventricular (RV) dysfunction has been poorly investigated. The purpose of this study was to evaluate the effect of tafamidis on RV free wall global longitudinal strain (RV FW-GLS) and right ventricular and pulmonary artery (RV-PA) coupling over 12 months of treatment. Methods: Ninety-three patients with ATTR-CM treated with 61 mg of tafamidis daily who underwent multimodality imaging evaluation at baseline by cardiovascular magnetic resonance (CMR) and speckle-tracking echocardiography were retrospectively studied. The 12-month follow-up included an echocardiographic assessment of RV FW-GLS and RV-PA coupling. RV reverse remodeling was defined as a >10% improvement in RV FW-GLS and/or in RV-PA coupling from baseline. RV-PA coupling was assessed using the tricuspid annular plane systolic excursion/ pulmonary artery systolic pressure (TAPSE/PASP) ratio. Results: Over 12 months of tafamidis treatment, RV reverse remodeling was documented in 22.6% of patients. In these patients, RV FW-GLS improved significantly from 14.5 ± 2.1% to 17.3 ± 2%, p < 0.001, whereas the TAPSE/PASP ratio improved from 0.42 ± 0.05 mm/mmHg to 0.54 ± 0.07 mm/mmHg, p = 0.001. Patients who experienced RV reverse remodeling were at an earlier stage of disease prior to tafamidis treatment with less dilated RV and less severe RV-PA uncoupling (TAPSE/PASP ratio: 0.43 ± 0.06 mm/mmHg vs. 0.39 ± 0.06 mm/mmHg, p = 0.040). CMR-derived baseline RV end-systolic volume (HR 0.83, 95% CI 0.73–0.94, p = 0.005) and NT-proBNP (HR 0.989, 95% CI 0.988–0.999, p = 0.024) were the strongest independent predictors of RV reverse remodeling, followed by PASP (HR 0.82, 95% CI 0.69–0.98, p = 0.030). Conclusions: Patients with ATTR-CM treated with tafamidis at an earlier stage of the disease experienced RV reverse remodeling with significant improvement in RV FW-GLS and RV-PA coupling.

Coronary artery fistulas (CAFs) are a rare congenital heart disease. Large fistulas can lead to coronary steal phenomena presenting with angina, heart failure, and in rare cases, cardiogenic shock. A 55-year-old woman acutely presented at our heart center with nonhemorrhagic pericardial tamponade and cardiogenic shock. A large CAF of an aneurysmatic right coronary artery to the coronary sinus was diagnosed. Pericardiocentesis was used for initial hemodynamic stabilization. On cardiopulmonary bypass, perforations connecting right coronary artery and coronary sinus were closed via suture. In cases of CAF, cardiogenic shock with pericardial tamponade is usually caused by rupture of an aneurysmatic vessel with successive hemopericardium. In this case, the pericardial tamponade was caused by acute right heart decompensation after long-term right ventricular overload. In adult right heart failure, early comprehensive cardiac imaging is essential for diagnosis of rare underlying congenital diseases. Surgical shunt closure can drastically improve symptoms.

Background/Objectives: Timely diagnosis of infective endocarditis (IE) remains a significant challenge, and IE poses significant morbidity and mortality. Modified Duke criteria (MDC) are used for the clinical evaluation and diagnosis of IE, but their current use is dichotomous. There are no studies that associate the amount of positive MDC with the patient’s outcome. This study intends to analyze whether the amount of MDC on initial presentation can be used for prognostic assumptions. Methods: We conducted a retrospective data analysis on patients with confirmed and suspected IE who were treated at the Department of Internal Medicine II at Ulm University Heart Center from December 2009 to December 2019. Univariable and multivariable logistic regression models were used to find correlations between 90-day mortality and the number of MDC. Results: 130 patients with confirmed IE were included in the analysis. Less minor MDC (OR 1.718; 95%-CI 1.096–3.268; p = 0.022) and a history of coronary artery disease (OR 4.711; 95%-CI 1.791–12.393; p = 0.002) were independently associated with higher 90-day mortality in patients with ultimately confirmed IE. Fewer minor MDC on presentation were associated with later diagnosis (b 2.341; 95%-CI 0.312–4.370; p = 0.024) and antibiotic therapy (b 2.953; 95%-CI 0.82–5.084; p = 0.007) for IE. Conclusions: Early diagnosis of IE is essential for favorable outcomes. Fewer minor MDC on initial presentation may lead to delayed diagnosis, antibiosis, and worse outcomes.

Background/Objectives: It is unclear whether assessment of phasic atrial function could improve risk stratification for new-onset atrial fibrillation (AF) in patients with newly diagnosed cardiac sarcoidosis (CS). We aimed to investigate the prognostic value of left atrial (LA) phasic dysfunction by cardiac magnetic resonance (CMR) for new-onset AF in newly diagnosed patients with CS. Methods: 78 patients with CS, without a prior history of AF, were studied using CMR feature tracking. Over a 4-year follow-up period, AF was documented by Holter monitoring and interrogation of intracardiac devices. Clinically silent CS was defined as CS in patients with biopsy-proven extracardiac sarcoidosis, with no cardiac symptoms, but with abnormalities on CMR or positron emission tomography consistent with CS. Results: Patients with clinically manifest CS were younger (mean age 56 vs. 51 years, p = 0.018), had poorer ventricular function, higher extent of atrial late gadolinium enhancement and significantly lower LA reservoir, conduit and booster function compared to patients with clinically silent CS. Over a 4-year follow-up period, 39% of patients with clinically manifest CS and 29.7% of patients with clinically silent CS developed AF. LA reservoir strain was a strong predictor of AF in the entire cohort. In subgroup analysis, age (HR 1.30, 95% CI 1.02–1.65, p = 0.030) and LA reservoir strain (HR 0.63, 95% CI 0.44–0.90, p = 0.011) were independent predictors of AF in patients with clinically silent CS, whereas baseline NT-proBNP (HR 1.003, 95% CI 1.001–1.006, p = 0.017) predicted AF in patients with clinically manifest CS. Conclusions: Reduced LA reservoir strain on CMR predicts new-onset AF in patients with newly diagnosed CS. The predictive value of LA reservoir strain is strongest in clinically silent CS and decreases with disease progression in clinically manifest CS.