Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience.

BACKGROUND: Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are rare diseases that share some similarities, but also display different clinical and histopathological features. We aimed to compare the demographics, clinical presentation, and outcome of patients diagnosed with CS or GCM. METHOD: We compared the clinical data and outcome of all adult patients with CS (n = 71) or GCM (n = 21) diagnosed at our center between 1991 and 2020. RESULTS: The median (interquartile range) follow-up time for patients with CS and GCM was 33.5 [6.5-60.9] and 2.98 [0.6-40.9] months, respectively. In the entire cohort, heart failure (HF) was the most common presenting manifestation (31%), followed by ventricular arrhythmias (25%). At presentation, a left ventricular ejection fraction of < 50% was found in 54% of the CS compared to 86% of the GCM patients (P = 0.014), while corresponding proportions for right ventricular dysfunction were 24% and 52% (P = 0.026), respectively. Advanced HF (NYHA ≥ IIIB) was less common in CS (31%) than in GCM (76%). CS patients displayed significantly lower circulating levels of natriuretic peptides (P < 0.001) and troponins (P = 0.014). Eighteen percent of patients with CS included in the survival analysis reached the composite endpoint of death or heart transplantation (HTx) compared to 68% of patients with GCM (P < 0.001). CONCLUSION: GCM has a more fulminant clinical course than CS with severe biventricular failure, higher levels of circulating biomarkers and an increased need for HTx. The histopathologic diagnosis remained key determinant even after adjustment for markers of cardiac dysfunction.

Cardiac arrest in Wilson's disease after curative liver transplantation: a life-threatening complication of myocardial copper excess?

We report the case of a 38-year-old man who presented with cardiac arrest 1 year after curative liver transplantation for Wilson's disease. Clinical work-up proofed myocardial copper and iron accumulation using mass spectrometry, which led most likely to myocardial fibrosis as visualized by cardiovascular magnetic resonance (unprecedented delayed enhancement pattern) and endomyocardial biopsy. Consequently, cardiac arrest due to ventricular fibrillation and subsequent episodes of sustained ventricular tachycardia were considered as primary cardiac manifestation of Wilson's disease. This can, as illustrated by our case, occur even late after curative liver transplantation, which is an important fact that treating physicians should be aware of during clinical follow-up of these patients.

[Doppler echocardiographic evaluation of left ventricular diastolic function in patients with hypertrophic obstructive cardiomyopathy after alcohol septal ablation in acute and long-term follow up]. / Czynnosc rozkurczowa lewej komory oceniana echokardiografia dopplerowska u chorych z kardiomiopatia przerostowa z zawezaniem drogi odplywu po ablacji alkoholowej tetnicy septalnej w obserwacji krótko--i dlugoterminowej.

UNLABELLED: Left ventricular (LV) diastolic dysfunction is responsible for most clinical symptoms in hypertrophic cardiomyopathy. The left ventricular outflow tract (LVOT) obstruction has additional negative haemodynamic influence. Alcohol septal ablation reduces the LVOT gradient. CEL The aim of our study was the evaluation of acute and long-term changes of LV diastolic function after successful alcohol septal ablation. MATERIAL AND METHODS: In 15 patients with successful reducing of LVOT gradient the LV diastolic function was assessed using Doppler echocardiography before ablation and in short and long-term follow-up. RESULTS: Before ablation IVRT and DT were prolonged in comparison to normal values. In acute phase after ablation E/A ratio decreased from 1.19 +/- 0.36 to 0.89 +/- 0.27 (p < 0.01), but returned to baseline by the first week. In long-term follow-up there was again a significant reducing of E/A ratio (mainly due to E wave changes). IVRT was significantly shortened from 98 +/- 20 ms at baseline to 80.5 +/- 17 ms at day 7 and later. This change was stable to the end of follow-up. DT did not change significantly just after the ablation and during follow-up. CONCLUSIONS: In the acute period after alcohol septal ablation there is an increase of LV relaxation disorders. Changes observed during long-term follow-up are difficult to interpret, but significant shortening of IVRT shows on improvement of the LV diastolic function.