BACKGROUND: Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by degeneration of lower motor neurons, resulting in progressive muscle weakness and atrophy. However, little is known regarding the cardiac function of children with SMA.
METHODS: We recruited SMA patients younger than 18 years of age from January 1, 2022, to April 1, 2022, in the First Affiliated Hospital of Sun Yat-sen University. All patients underwent a comprehensive cardiac evaluation before treatment, including history taking, physical examination, blood tests of cardiac biomarkers, assessment of echocardiography and electrocardiogram. Age/gender-matched healthy volunteers were recruited as controls.
RESULTS: A total of 36 SMA patients (26 with SMA type 2 and 10 with SMA type 3) and 40 controls were enrolled in the study. No patient was clinically diagnosed with heart failure. Blood tests showed elevated values of creatine kinase isoenzyme M and isoenzyme B (CK-MB) mass and high-sensitivity cardiac troponin T (hs-cTnT) in spinal muscular atrophy (SMA) patients. Regarding echocardiographic parameters, SMA children were detected with lower global left and right ventricular longitudinal strain, abnormal diastolic filling velocities of trans-mitral and trans-tricuspid flow. The results revealed no clinical heart dysfunction in SMA patients, but subclinical ventricular dysfunction was seen in SMA children including the diastolic function and myocardial performance. Some patients presented with elevated heart rate and abnormal echogenicity of aortic valve or wall. Among these SMA patients, seven patients (19.4%) had scoliosis. The Cobb\'s angles showed a significant negative correlation with LVEDd/BSA, but no correlation with other parameters, suggesting that mild scoliosis did not lead to significant cardiac dysfunction.
CONCLUSIONS: Our findings warrant increased attention to the cardiac status and highlight the need to investigate cardiac interventions in SMA children.

BACKGROUND: The evolving challenge of persistent symptoms post-Coronavirus disease-2019 (COVID-19), particularly debilitating cardio-pulmonary manifestations, necessitates further exploration. Our study aimed to assess the cardio-pulmonary complications in patients a year after hospital discharge from severe COVID-19, contrasting these with findings from a non-COVID group.
METHODS: The OneCoV2 study, a prospective, case-control study, was conducted at a tertiary care teaching hospital in northern India. We enrolled 43 subjects, with a mean age of 25.57 ± 7.94 years (COVID group) and 27.30 ± 8.17 years (non-COVID group). Comprehensive tests included pulmonary function tests, cardiac function tests, 6-min walk tests, and laboratory investigations.
RESULTS: Significant differences were found in the pulmonary function [forced vital capacity (FVC) (p = 0.037), forced expiratory flow (FEF) 25-75 % (p = 0.013)], and cardiac function [left ventricular ejection fraction (LVEF) (p = 0.032), heart rate (HR) (p = 0.047)], along with the six-minute walk test results between the two groups. In the COVID group, Pearson\'s correlation showed a negative correlation between FVC and C-reactive protein (CRP) [r = -0.488, p = 0.007] and a positive correlation between the six-minute walk test [r = 0.431, p = 0.003] and HR [r = 0.503, p = 0.013].
CONCLUSIONS: Our data suggest that pulmonary abnormalities are prevalent in COVID patients even after 1-year of hospital discharge. Cardiac biomarkers also show an inclination towards the COVID group. While we found significant correlations involving some parameters like FVC, CRP, HR, and results from the six-minute walk test, we did not find any significant correlations with the other tested parameters in our study.

OBJECTIVE: To demonstrate possible functional changes in the frequently affected fetal interventricular septum (IVS) with spectral tissue Doppler imaging (TDI) and M-mode imaging to compare gestational diabetes mellitus (GDM) and control groups.
METHODS: A total of 63 pregnant women with GDM, 30 on diet (A1 GDM) and 33 on treated with insulin (A2 GDM), and 63 healthy pregnant women randomly selected and matched to the case group in the control group were included.
RESULTS: The GDM fetuses had significantly thickened IVS, increased early diastole (E\'), atrial contraction (A\'), systole (S\'), higher myocardial performance index (MPI\'), prolonged isovolumetric relaxation time (IVRT\'), shortened ejection time (ET\'), and decreased septal annular plane systolic excursion (SAPSE) than the controls. The A2 GDM group fetuses had significantly thickened IVS, increased S\' and shortened ET\' than the A1 GDM group. In the GDM group, we found a significantly positive low correlation between glycated hemoglobin levels and maternal serum fasting glucose and one-hour postprandial glucose with fetal IVS thickness. We demonstrated a significantly negative low correlation between maternal serum one-hour postprandial glucose, glycated hemoglobin levels, and gestational weight gain with fetal IVS ET\'.
CONCLUSIONS: Fetal IVS diastolic and systolic functions were altered in the GDM group compared to controls, and systolic functions were altered in A2 GDM compared to A1 GDM. This may alert clinicians to possible cardiovascular diseases in the postnatal life, and early preventive strategies and long-term lifestyle changes may provide protection in fetuses with GDM.

This study aimed to analyze the possible causes of changes in cardiac function and investigate the feasibility of clinical assessment of gastrointestinal cancer in patients with or without acute kidney injury (AKI) assessed using a non-invasive impedance cardiography (ICG, Bioz. Cardio Dynamics, USA) to identify independent risk factors.
Patients admitted to the Fourth Hospital of Hebei Medical University, China, between May 1, 2019, and February 15, 2022, were included in this study. A total of 51 patients with gastrointestinal cancer (31 men and 20 women, mean age 61.1 ± 10.9 years) with or without AKI were evaluated for ICG. A total of 19 patients underwent ultrasound cardiography (UCG) and ICG evaluations.
There was a significant positive correlation between cardiac output (CO), cardiac index (CI), stroke volume (SV), left cardiac work index (LCWI), and ejection fraction (EF) measured using UCG and ICG. The relationship was observed between COICG and COUCG (r = 0.707, P = 0.001), CIICG and CIUCG (r = 0.718, P = 0.001), SVICG and SVUCG (r = 0.837, P < 0.001), and LCWIICG and EFUCG (r = 0.540, P = 0.017). Cardiac function parameters measured using ICG were statistically different between patients with gastrointestinal cancer with or without AKI (P ≤ 0.05). Multivariate analysis revealed that AKI independently affects cardiac function in patients with gastrointestinal cancer.
UCG and ICG methods are significantly associated with cardiac function in patients with or without AKI, and patients with gastrointestinal cancer with AKI are worse than those without AKI. AKI is an independent risk factor for cardiac function in patients with gastrointestinal cancer.

Objectives: To date, there is a lack of consensus on the timely implementation of palliative care (PC) in patients with chronic heart failure (HF). We aimed to investigate the impact of primary PC intervention on chronic HF patients with different classes of cardiac function, and to determine a proper time point for the implementation of primary PC intervention. Methods: A consecutive series of 180 chronic HF patients with the New York Heart Association (NYHA) Cardiac function ranging from I to III were enrolled in this study. Patients with the same cardiac function class, they were randomized and equally assigned to the usual care (UC) group or to the PC intervention group. At the end of 24-week treatment, quality-of-life (QoL) measurements were evaluated. Left ventricular ejection fraction and N-terminal pro-B-type natriuretic peptide were measured for each group at baseline and the final follow-up, respectively. Results: Through the 6-month follow-up, patients randomized to the PC intervention group presented significantly better QoL and cardiac function as compared with patients randomized to the UC group alone. Subgroup analysis showed that for patients with NYHA class II or III, significantly improved cardiac function and QoL were observed in the PC intervention group as compared with the control group. As for patients with class I, no significant difference was found between the 2 groups. Conclusions: Palliative program can effectively improve the QoL and cardiac function of patients with chronic HF. Moreover, we provided evidence on timely referral of patients to PC intervention, which could be beneficial for patients with NYHA class II.

To investigate the effects of standardized fluid management (SFM) on cardiac function in patients with pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).
Patients with PMP who underwent CRS + HIPEC at our center were retrospectively analyzed. The patients were divided into control and study groups according to whether SFM was applied after CRS + HIPEC. We compared the preoperative and postoperative cardiac and renal function parameters, daily fluid volume three days after CRS, and cardiovascular-related adverse events. Univariate and multivariate analyses were performed to identify the indicators affecting clinical prognosis.
Among the 104 patients, 42 (40.4%) were in the control group and 62 (59.6%) in the study group. There were no statistically significant differences between the two groups in the main clinicopathological characteristics, preoperative cardiac and renal function parameters, and CRS + HIPEC-related indicators. The incidences of cardiac troponin I (CTNI) > upper limit of normal (ULN), >2 × ULN, >3 × ULN, serum creatinine > ULN, and blood urea nitrogen > ULN were higher in the control group than in the study group (p < 0.05). The median daily fluid volume of the control group was higher than that of the study group 3 days after CRS (p < 0.05). Postoperative CTNI > 2 × ULN was an independent risk factor for serious circulatory adverse events. Survival analysis revealed pathological grading, completeness of cytoreduction score, and postoperative CTNI > ULN as independent prognostic factors.
SFM after CRS + HIPEC in patients with PMP may reduce cardiovascular adverse events risk and improve clinical outcomes.

Patients with end-stage renal disease have higher cardiovascular morbidity and mortality compared with the general population. Preemptive kidney transplant (KTx) has been shown to be associated with improved survival, better quality of life, lower healthcare burden, and reduced cardiovascular risk. In this case-control study, we investigated the cardiovascular benefits of two approaches to KTx: with and without previous chronic hemodialysis. We enrolled 21 patients who underwent preemptive KTx and 21 matched controls who received chronic hemodialysis before KTx. Cardiac morphological and functional parameters were assessed by echocardiography. Overall, patients undergoing preemptive KTx showed less extensive cardiac damage compared with controls, as evidenced by higher global longitudinal strain, peak atrial and contractile strain, and early diastolic mitral annular velocity as well as a lower left ventricular mass, left atrial volume index, and the ratio of mitral inflow early diastolic velocity to the mitral annular early diastolic velocity. In the multivariable analysis, the presence of chronic hemodialysis prior to KTx was an independent determinant of post-transplant cardiac functional and structural remodeling. These findings may have important clinical implications, supporting the use of preemptive KTx as a preferred treatment strategy in patients with end-stage renal disease.

This case report describes the prolonged general anesthetic management of a 41-year-old woman with antiphospholipid syndrome (APS), systemic lupus erythematosus, and previously undiagnosed decreased cardiac function who underwent planned partial resection of the left tongue, tracheostomy, neck dissection, and pedicled flap reconstruction. This was immediately followed by emergent surgery to salvage the flap, and 1 month later, revision of the soft tissue flap was performed. A preoperative echocardiogram was performed because of her various risk factors, which identified lateral wall hypokinesis and reduced left ventricular ejection fraction of 40%, despite no known cardiovascular disease. However, cardiology consult determined no additional treatment was needed before the surgery. Multiple antithrombotic strategies were used, including elastic stockings, intermittent pneumatic compression devises, and heparin bridging. During the general anesthetic, stroke volume variation (SVV) was used to assess cardiac function and guide fluid management. There were no signs of systemic thrombosis, although the free flap reconstruction was abandoned because of a thrombus in the vascular anastomosis. Cardiac function can deteriorate in APS patients because of coronary and/or microvascular thrombosis. Therefore, it is necessary to evaluate cardiac function, regardless of a known history of cardiovascular disease. Moreover, additional monitoring (ie, SVV) may be useful during prolonged general anesthetics for patients with APS and cardiac dysfunction.

BACKGROUND: Few studies have examined respiratory dysfunction in patients with Becker muscular dystrophy (BMD).
OBJECTIVE: This study aimed to examine the characteristics of respiratory dysfunction in patients with BMD.
METHODS: The present retrospective study assessed respiratory parameters of adult BMD patients using medical records and compared these parameters with various patient characteristics to identify correlations. BMD patients aged 17 years and older who had been diagnosed genetically and/or pathologically were included in the analysis.
RESULTS: Of the source population of 133 patients, respiratory function was assessed in 85. Two of these patients had no symptoms, and eight had died. Mean % forced vital capacity (% FVC) was 94.2+/-21.7% (median, 96.1%; range, 5.1-134.1%). In 16 (19%) of the 85 patients, % FVC was <80%. Of these, seven were non-ambulant. Age, ambulation, and cardiac function did not significantly differ between patients with or without respiratory dysfunction, whereas age at onset was significantly lower in patients with respiratory dysfunction (7.7+/-4.7 years vs. 14.4+/-11.9 years; p = 0.001). One non-ambulant patient was a continuous NPPV user, and one patient had been recommended NPPV use but refused. Autopsy of one patient revealed that the diaphragm and intercostal muscles were less affected than proximal skeletal muscles.
CONCLUSIONS: BMD patients are at risk of developing respiratory dysfunction due to dystrophic changes in respiratory muscles. Respiratory function should be carefully and periodically monitored in these patients.

To explore levels of the brain natriuretic peptide (BNP) and how these associate with the cardiac abnormalities recently identified in chronic fatigue syndrome (CFS).
Cardiac magnetic resonance examinations were performed using 3T Philips Intera Achieva scanner (Best, Netherlands) in CFS (Fukuda) participants and sedentary controls matched group wise for age and sex. BNP was also measured by using an enzyme immunoassay in plasma from 42 patients with CFS and 10 controls.
BNP levels were significantly higher in the CFS cohort compared with the matched controls (P=0.013). When we compared cardiac volumes (end-diastolic and end-systolic) between those with high BNP levels (BNP >400 pg/mL) and low BNP (<400 pg/mL), there were significantly lower cardiac volumes in those with the higher BNP levels in both end-systolic and end-diastolic volumes (P=0.05). There were no relationships between fatigue severity, length of disease and BNP levels (P=0.2) suggesting that our findings are unlikely to be related to deconditioning.
This study confirms an association between reduced cardiac volumes and BNP in CFS. Lack of relationship between length of disease suggests that findings are not secondary to deconditioning. Further studies are needed to explore the utility of BNP to act as a stratification paradigm in CFS that directs targeted treatments.
Registered with NIHR Portfolio CLRN ID 97805.