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.

Is the 2016 ESC diagnostic algorithm useful for assessing the prevalence of chronic heart failure in population-based studies?

BACKGROUND: Chronic heart failure (CHF) is a major healthcare problem. However, there are no epidemiological studies assessing the prevalence of CHF in the general population with diagnosis based on algorithms recommended for clinical practice. AIM: The aim of the HF-Pomorskie survey was to assess the prevalence of three basic components of the 2016 ESC diagnostic algorithm for CHF (symptoms, N-terminal pro B-type natriuretic peptide [NT-proBNP], and abnormalities on echocardiography) and to determine whether this algorithm may be applicable to studies in general population samples. METHODS: The study was performed in a representative sample of 313 adults (170 women and 143 men) aged between 20 and 90 years (mean 55.2 years [15.3]) in Northern Poland. A questionnaire to determine New York Heart Association [NYHA] class, laboratory tests including NT-proBNP, as well as transthoracic echocardiography and spirometry examinations were performed in all subjects. RESULTS: Dyspnea (NYHA class II-IV) was reported by 13.7% of recruited participants. Dyspnea and elevated levels of NT-proBNP (>125 pg/ml) were found in 7.7% of all examined subjects, while dyspnea, elevated NT-proBNP levels accompanied by systolic or diastolic abnormalities on echocardiography occurred in 4.8%. In the group without dyspnea (86.3% of all examined subjects), every sixth subject had an elevated level of NT-proBNP. On the other hand, 5.8% of studied subjects reported a previous diagnosis of CHF, which was confirmed using the current ESC algorithm in 78% of them. CONCLUSIONS: The prevalence of CHF assessed by the 2016 ESC diagnostic algorithm in the representative sample of adults was equal to 4.8%. The clinical algorithm for the diagnosis of CHF is fully applicable to the representative surveys in the general population. However, due to logistic and economic factors, echocardiography examination and NT-proBNP determination can be limited to patients reporting dyspnea or previous diagnosis of CHF.

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.

Echocardiographic Findings in Asymptomatic Mediastinal Lymphoma Survivors Years after Treatment Termination.

Patients treated due to mediastinal lymphomas are at risk of cardiovascular complications, as they receive chemotherapy, usually containing anthracyclines, often combined with thoracic radiotherapy. The aim of this prospective study was to assess early asymptomatic cardiac dysfunction using resting and dobutamine stress echocardiography (DSE) at least 3 years after the end of mediastinal lymphoma treatment. Two groups of patients were compared: those treated with chemoradiotherapy and those exclusively treated with chemotherapy. Left ventricular contractile reserve (LVCR) during DSE was assessed using changes in LV ejection fraction (LVEF), LV global longitudinal strain (LV GLS), and a novel parameter-Force, which is the ratio of the systolic blood pressure to the LV end-systolic volume. The study included 60 patients examined at a median of 89 months after the end of treatment. Resting echocardiography showed normal LVEF of 58.9 ± 9.6%, borderline LV GLS of -17.7 ± 3%, decreased mean stroke volume (SV) of 51.4 ± 17 mL, and indexed SV of 27.3 ± 8 mL/m2, and the right ventricular free wall longitudinal strain (LS) was impaired in some patients but not in all. There were no significant differences between the groups, with the exception of arterial hypertension, which was more common in the chemotherapy group (32% vs. 62.5%, p = 0.04). In resting echocardiography, only LV posterior wall LS differed significantly and was impaired in patients treated with chemotherapy (-19.1 ± 3.1% vs. -16.5 ± 5.1%, p = 0.04). DSE, performed in 21 patients after a median of 166 months from the end of cancer treatment, detected new contractility disorders in 1 patient (4.8%) and decreased LVCR in the majority of patients when determined using changes in LVEF or LV GLS, and in all patients when assessed with changes in Force. Conclusions: Most asymptomatic mediastinal lymphoma survivors showed preserved ventricular function on resting echocardiography. However, all of them showed impaired LV contractile reserve on DSE, as assessed with a simple parameter-Force. This may indicate subtle LV dysfunction and confirms the need for long-term monitoring of patients with potentially cardiotoxic cancer treatment.

The Influence of Radiotherapy on the Function of the Left and Right Ventricles in Relation to the Radiation Dose Administered to the Left Anterior Descending Coronary Artery-From a Cardiologist's Point of View.

The aim of this study was to assess the effects of radiotherapy involving the heart on LV and RV function using modern speckle-tracking echocardiography (STE), and in relation to the radiation dose applied to the LAD. This retrospective, single-centre study included 12 patients after a median of 51 months after irradiation for mediastinal lymphoma, in whom we were able to delineate the LAD. Correlations between doses of ionising radiation and echocardiographic parameters reflecting the systolic function of the LV and RV were analysed. The median irradiation dose delivered to the whole heart was 16.4 Gy (0.5-36.2 Gy), and to the LAD it was 15.1 Gy (0.3-35.3 Gy). LV longitudinal strain (LS) was impaired in the anteroseptal and anterior walls. Parameters reflecting RV function were normal, with the exception of RV myocardial performance index (RIMP). Significant correlations were found between the median dose to the LAD and LV global LS (rho = 0.6468, p = 0.034), the maximum dose to the LAD and LV anterior LS (rho = 0.6046, p = 0.049), the median and the mean dose to the whole heart and LV anterior LS (R = 0.772, p = 0.009 and rho = 0.7676, p = 0.01, respectively), and the total irradiation dose and RIMP (rho = 0.5981, p = 0.04). The calculation of irradiation doses allows the identification of patients at risk of cardiac dysfunction detected by modern STE.

Respiratory Motion-Registered Isotropic Whole-Heart T2 Mapping in Patients With Acute Non-ischemic Myocardial Injury.

Background: T2 mapping is a magnetic resonance imaging technique that can be used to detect myocardial edema and inflammation. However, the focal nature of myocardial inflammation may render conventional 2D approaches suboptimal and make whole-heart isotropic 3D mapping desirable. While self-navigated 3D radial T2 mapping has been demonstrated to work well at a magnetic field strength of 3T, it results in too noisy maps at 1.5T. We therefore implemented a novel respiratory motion-resolved compressed-sensing reconstruction in order to improve the 3D T2 mapping precision and accuracy at 1.5T, and tested this in a heterogeneous patient cohort. Materials and Methods: Nine healthy volunteers and 25 consecutive patients with suspected acute non-ischemic myocardial injury (sarcoidosis, n = 19; systemic sclerosis, n = 2; acute graft rejection, n = 2, and myocarditis, n = 2) were included. The free-breathing T2 maps were acquired as three ECG-triggered T2-prepared 3D radial volumes. A respiratory motion-resolved reconstruction was followed by image registration of the respiratory states and pixel-wise T2 mapping. The resulting 3D maps were compared to routine 2D T2 maps. The T2 values of segments with and without late gadolinium enhancement (LGE) were compared in patients. Results: In the healthy volunteers, the myocardial T2 values obtained with the 2D and 3D techniques were similar (45.8 ± 1.8 vs. 46.8 ± 2.9 ms, respectively; P = 0.33). Conversely, in patients, T2 values did differ between 2D (46.7 ± 3.6 ms) and 3D techniques (50.1 ± 4.2 ms, P = 0.004). Moreover, with the 2D technique, T2 values of the LGE-positive segments were similar to those of the LGE-negative segments (T2LGE-= 46.2 ± 3.7 vs. T2LGE+ = 47.6 ± 4.1 ms; P = 0.49), whereas the 3D technique did show a significant difference (T2LGE- = 49.3 ± 6.7 vs. T2LGE+ = 52.6 ± 8.7 ms, P = 0.006). Conclusion: Respiratory motion-registered 3D radial imaging at 1.5T led to accurate isotropic 3D whole-heart T2 maps, both in the healthy volunteers and in a small patient cohort with suspected non-ischemic myocardial injury. Significantly higher T2 values were found in patients as compared to controls in 3D but not in 2D, suggestive of the technique's potential to increase the sensitivity of CMR at earlier stages of disease. Further study will be needed to demonstrate its accuracy.

Case Report: Adjuvant Radiotherapy Can Be an Effective Treatment for Intimal Sarcoma of the Heart.

Intimal sarcoma of the heart is a sporadic disease, which involves symptoms of cardiac insufficiency due to a fast-growing intraluminal mass. Tumor resection is the first-line treatment, although its location precludes excision with wide uninvolved margins. Despite the aggressiveness of this neoplasm and a high risk of recurrence even after removal by microscopically radical surgery, no standard adjuvant therapy has been established. Chemotherapy is used either as an adjuvant treatment or in cases of advanced disease. In contrast, the use of radiotherapy is rare and usually considered in a palliative setting because the risk of radiation-induced heart disease after high-dose radiotherapy to the heart is significant. Herein, we present the cases of two patients, both diagnosed with cardiac intimal sarcoma, who received irradiation after tumor resection. In both cases, radiotherapy was effective, providing long-lasting local disease control. We regularly monitored cardiac function in both patients to assess the impact of radiotherapy on tumor-free heart structures. The excellent local control of the disease with only mild long-term cardiac dysfunction in both patients suggests that radiotherapy can be a useful treatment modality in this indication.

[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.