A rare case of isolated persistent left superior vena cava diagnosed by echocardiography.

BACKGROUND: The persistent left superior vena cava (PLSVC) is an infrequent vascular variant. PLSVC with absent right superior vena cava, also known as isolated PLSVC, is an exceptionally rare entity. In this case we present a patient with isolated PLSVC draining to coronary sinus, diagnosed incidentally during echocardiography. CASE PRESENTATION: A 35-year-old man underwent a transthoracic echocardiography which showed an enormously dilated coronary sinus. Hand-agitated saline was injected via peripheral intravenous cannulas. The contrast appeared firstly in the coronary sinus before it opacified the right atrium. Since this was also visible by the right antecubital saline injection, it indicated an extremely rare case of PLSVC with the absence of right superior vena cava which was confirmed by cardiac magnetic resonance. CONCLUSIONS: The finding of a distinctively dilated coronary sinus in echocardiography led us to further investigation using agitated saline that revealed an infrequent anomaly termed isolated PLSVC. The in-depth diagnosis of this vascular variant is crucial considering that it may lead to important clinical implications, such as difficulties with central venous access, especially in the current era of a rapid development of cardiac device therapies.

The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.

INTRODUCTION: Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability. OBJECTIVES: This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis. PATIENTS AND METHODS: A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24 h Holter monitoring were analyzed. RESULTS: Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61± 2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0% ± 3.2% and -18.9% ± 2.8%, respectively; p = .223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR. CONCLUSIONS: In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.

Coexistence of Left Atrial Tumor and Lung Cancer-The Key Role of an Individualized Approach.

During the diagnostic work-up in oncology, it is exceedingly rare to assume a concomitant presence of two cancers, a benign one and a malignant one, in a single patient. A 61-year-old man was admitted to the cardiology department for cardiac evaluation prior to planned radical treatment of non-small cell (NSCLC) left lung cancer (cT3N1M0). Echocardiography revealed a prominent, unpedunculated structure, measuring 17 × 14 mm, located in the left atrium (LA) near the fossa ovalis. The tumor was confirmed via cardiac magnetic resonance (CMR) imaging, which showed the radiological features of an atrial myxoma. The patient consulted with the Cardiac Surgery Department and was deemed ineligible for surgical treatment of a lesion with mucinous features; thus, no definitive histopathologic confirmation of the tumor present was possible. He was then successfully treated with radical radiochemotherapy and immunotherapy. During the 2-year follow-up, regular echocardiography and CMR were performed, which documented a stable LA tumor size.

Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort-A Cardiac Magnetic Resonance Study.

Background: Haemochromatosis (HCH), a common genetic disorder with variable penetrance, results in progressive but understudied iron overload. We prospectively evaluated organ iron loading and cardiac function in a tertiary center HCH cohort. Methods: 42 HCH patients (47 ± 14 years) and 36 controls underwent laboratory workup and cardiac magnetic resonance (CMR), including T1 and T2* mapping. Results: Myocardial T2* (myoT2*), myocardial T1 (myoT1) and liver T2* (livT2*) were lower in patients compared to controls (33 ± 4 ms vs. 36 ± 3 ms [p = 0.004], 964 ± 33 ms vs. 979 ± 25 ms [p = 0.028] and 21 ± 10 ms vs. 30 ± 5 ms [p < 0.001], respectively). MyoT2* did not reach the threshold of clinically significant iron overload (<20 ms), in any of the patients. In 22 (52.4%) patients, at least one of the tissue parameters was reduced. Reduced myocardial T2* and/or T1 were found in 10 (23.8%) patients, including 4 pts with normal livT2*. LivT2* was reduced in 18 (42.9%) patients. MyoT1 and livT2* inversely correlated with ferritin (rs = −0.351 [p = 0.028] and rs = −0.602 [p < 0.001], respectively). LivT2* by a dedicated sequence and livT2* by cardiac T2* mapping showed good agreement (ICC = 0.876 p < 0.001). Conclusions: In contemporary hemochromatosis, significant myocardial iron overload is rare. Low myocardial T2* and/or T1 values may warrant closer follow-up for accelerated myocardial iron overload even in patients without overt liver overload. Cardiac T2* mapping sequence allows for liver screening at the time of CMR.

Right Ventricular Endocardial Mapping and a Potential Arrhythmogenic Substrate in Cardiac Amyloidosis-Role of ICD.

Patients with cardiac amyloidosis (CA) have an increased risk of sudden cardiac death. (SCD). However, the role of an implantable cardioverter-defibrillator in the primary prevention of SCD in this group of patients is still controversial. We present a case with CA with recurrent syncope and non-sustained ventricular tachycardia. In order to further stratify the risk of SCD, an electrophysiological study with endocardial electroanatomic voltage mapping was performed prior to the ICD placement.

[Cardiac sarcoidosis: diagnostics, treatment and follow-up]. / Sarkoidoza serca: diagnostyka, leczenie i monitorowanie.

Sarcoidosis is a generalised granulomatous disorder of unknown aetiology. Cardiac involvement may affect conduction system, myocardium, valvular apparatus and pericardium. Clinical spectrum ranges from asymptomatic involvement to sudden cardiac death. Patients with biopsy-proven extracardiac sarcoidosis should be screened for cardiac involvement (standard ECG, 24-hour Holter ECG, echocardiography) and in case of any abnormalities found on these tests, more advanced diagnostic methods should be used. Steroid treatment is still the mainstay of therapy in cardiac sarcoidosis. Several immunosuppresive agents are also effective and used in different combinations with steroids, as well as heart failure treatment (including ACE inhibitors, angiotensin receptor blockers, beta-blockers and diuretics). Advanced heart block requires pacemaker implantation, and implantable cardioverterdefibrillator is an effective treatment in primary and secondary prophylaxis of sudden cardiac death. Heart transplantation is considered in advanced, drug-resistant heart failure or incessant ventricular arrhythmias unresponsive to other forms of therapy.

[Cardiac involvement in neuronal ceroid lipofuscinosis]. / Zajecie serca w przebiegu neuronalnej lipofuscynozy ceroidowej.

We present a case of a 58-year-old female with neuropsychiatric symptoms, followed by recurrent episodes of atrial flagellation and symptoms of heart failure. Based on intraoperative myocardial biopsy, neuronal ceroid lipofuscinosis was diagnosed.