Visnagin: A novel cardioprotective agent against anthracycline toxicity (Review).

Doxorubicin (DOX), a cornerstone of cancer chemotherapy, is marred by its dose-dependent cardiotoxicity, leading to cardiomyopathy and heart failure. The epidemiology of DOX-related cardiotoxicity highlights its cumulative, progressive nature, with a significant impact on the health of patients. The pathophysiological mechanisms involve mitochondrial dysfunction, oxidative stress and disrupted calcium homeostasis in cardiomyocytes. Despite the search for effective cardioprotective strategies, current treatments offer limited efficacy. Visnagin emerges as a potential solution, known for its vasodilatory and anti-inflammatory properties, and recent studies suggest its cardioprotective efficacy against DOX-induced cardiotoxicity through mitochondrial protection, the modulation of key signaling pathways and the inhibition of apoptosis. The present review aimed to provide a comprehensive overview of the mechanisms of action of visnagin, as well as to provide experimental evidence, and potential integration into cancer treatment regimens, highlighting its promise as a novel therapeutic agent for managing cardiotoxicity in patients undergoing anthracycline chemotherapy.

Temporal trends, prevalence, predictors, and outcomes of heart failure in patients with hypertrophic cardiomyopathy in the United States: Insights from the national inpatient sample.

OBJECTIVES: This study aims to delineate the temporal trends, prevalence, predictors, and outcomes of HF among HCM patients using the National Inpatient Sample (NIS) database, with a focus on optimizing therapeutic strategies and healthcare resources. METHODS: We conducted a retrospective cohort analysis of anonymized data from the NIS spanning 2016 to 2019. The study population consisted of adults diagnosed with HCM based on specific ICD-10 diagnostic codes. Logistic regression was utilized to explore the association between HF and in-hospital mortality, adjusting for demographic and clinical factors. RESULTS: Our analysis included 215,505 individuals, with 97,875 (45.4 %) experiencing HF. Patients with HF exhibited a higher burden of comorbidities such as diabetes and renal failure, and had increased odds of mortality (OR 1.41). The study also highlighted significant demographic disparities, with marked differences in outcomes based on race and gender. The economic analysis revealed higher healthcare costs and longer hospital stays associated with HF. CONCLUSION: HF significantly impacts mortality, healthcare costs, and hospitalization length in HCM patients, with substantial demographic and clinical disparities. This study underscores the importance of tailored management strategies and the need for continuous surveillance and research to address the challenges posed by HF in HCM.

Unveiling Hidden Battles: Exploring the Link Between Breast Cancer Survival and Heart Failure Vulnerability.

This study explores the link between a history of breast cancer and the vulnerability to heart failure. Analyzing data from the National Inpatient Sample (NIS) for women diagnosed with breast cancer between 2016 and 2019 in the US, our research utilized logistic regression, adjusting for demographics, comorbidities, and lifestyle factors, and employed propensity score matching. With 2,276,639 weighted cases, our findings reveal a slight but significant elevation in heart failure risk among the breast cancer cohort, specifically in acute, chronic, and isolated systolic heart failure types. Racial differences were pronounced; Black women with breast cancer showed higher risks for all heart failure types, particularly chronic and systolic, while Asian or Pacific Islander patients had a lower incidence of certain heart failure types. This research underscores a modest increase in heart failure risk post-breast cancer, highlighting the critical need for integrated cardio-oncology care and personalized healthcare approaches to address and mitigate this risk effectively.

Burden of Ischemic Heart Diseases among US States from 1990-2019.

BACKGROUND: Ischemic Heart Disease (IHD) is a leading cause of global mortality, including in the United States. Understanding the burden of IHD in the United States is crucial for informed decision-making and targeted interventions aimed at reducing morbidity and mortality associated with this leading cause of death. This study aimed to understand the burden of IHD, identify gender disparities and risk factors, explore the relationship between socioeconomic growth and IHD, and analyze risk factor distribution across the states of the United States. METHODS: This study utilized data from the Global Burden of Diseases Study 2019, which provided comprehensive information on IHD from 1990 to 2019. Data related to IHD from these years were extracted using a query tool from the Institute for Health Metrics and Evaluation (IHME) website. The study assessed the relationship between IHD and socioeconomic development using the Socio-demographic Index (SDI) and measured the overall impact of IHD using Disability-adjusted Life Years (DALYs), considering premature death and disability. Additionally, the study analyzed the burden of IHD attributed to six main risk factors. Data analysis involved comparing prevalence, mortality, SDI, DALYs, attributable burden, and risk estimation among the states. RESULTS: Between 1990 and 2019, there was an improvement in socioeconomic development in all states. Age-standardized rates of disease burden for IHD decreased by 50% [ASDR 3278.3 to 1629.4 (95% UI: 1539.9-1712.3) per 100,000] with the most significant decline observed in Minnesota. Males had higher burden rates than females in all states, and the southeast region had the highest mortality rates. The prevalence of IHD showed a declining trend, with approximately 8.9 million cases (95% UI: 8.0 million to 9.8 million) in 2019, representing a 37.1% decrease in the Age-standardized Prevalence Rate (ASPR) from 1990. Metabolic risks were the leading contributors to the disease burden, accounting for 50% of cases, with Mississippi having the highest attributable risk. Arkansas had the highest attributable risk for high cholesterol and smoking. Conversely, Minnesota had the lowest burden of IHD among all the states. CONCLUSION: This study highlights variations in the burden of IHD across US states and emphasizes the need for tailored prevention programs to address specific risk factors and gender differences. Understanding the trend in IHD may inform policymakers and healthcare professionals in effectively allocating resources to reduce the burden of IHD and improve national health outcomes.

Paraneoplastic NMDA encephalitis, a case report and an extensive review of available literature.

Anti-N-methyl-D-aspartate receptor (NMDAr) encephalitis is a prevalent autoimmune condition marked by diverse neuropsychiatric symptoms, primarily impacting young females. The exact mechanisms underlying the development of NMDAr encephalitis have not been fully elucidated. Nonetheless, studies have demonstrated that auto-antibodies targeting the NR1-NR2 subunits of the NMDAr can trigger receptor dysfunction within the central nervous system, thus giving rise to the associated symptoms. Notably, an association exists between NMDAr encephalitis and an underlying neoplastic condition, with approximately 38% of cases exhibiting this paraneoplastic relationship with ovarian teratomas being the most commonly associated malignancy. While the association between NMDAr encephalitis and renal cell carcinoma (RCC) is exceedingly rare. This case report presents the clinical scenario of a 20-year-old female patient diagnosed with NMDAr encephalitis in conjunction with RCC discovered incidentally on a CT abdomen and pelvis performed to rule out an ovarian teratoma. The presented case underscores the importance of adopting a multidisciplinary approach in the diagnosis and treatment of NMDAr encephalitis, particularly when it is linked to an underlying malignancy. Furthermore, it emphasizes the significance of expanding our understanding of the molecular pathogenesis of NMDAr encephalitis to enhance patient care and optimize clinical outcomes. Additionally, a comprehensive review of the existing literature is included, summarizing all reported malignancies associated with NMDAr encephalitis.

Racial and ethnic disparities in heart transplantation for end-stage heart failure: An analysis of the national inpatient sample (NIS) database.

PURPOSE: This study aims to examine disparities among heart transplant recipients in the United States, utilizing the latest data from the National Inpatient Sample (NIS). METHODS: We conducted a retrospective cohort analysis of NIS discharge data (2017-2020), focusing on adult end-stage heart failure (ESHF) patients, identified using the ICD-10 CM code I50.84. Our analysis included four racial groups: White, Black, Hispanic, and Asian. We employed univariable and multivariate regression analyses to determine the unadjusted and adjusted odds of heart transplantation across these racial groups, using Stata version 14.2 for statistical calculations. RESULTS: Of 110,015 ESHF patients, 3,695 received heart transplants. Predominantly, recipients were male with a Charlson comorbidity index ≥3 and covered by private insurance. Transplants mainly occurred in large, teaching hospitals. Despite minor differences in age and median household income among races, baseline patient and hospital characteristics showed no significant variations. Compared to Whites, Blacks had a significantly lower transplant rate (AOR: 0.6; 95  % CI: 0.46-0.77; p < 0.001), while Hispanics and Asians showed no significant disparities. Mean ages varied slightly across groups (p = 0.0047), yet inpatient length of stay and hospitalization costs did not significantly differ. CONCLUSION: Our findings highlight a significant disparity in heart transplant rates between Black and White ESHF patients in the U.S., with Black patients less likely to receive transplants compared to their White counterparts.

Diabetes mellitus impact on left ventricular assist device outcomes in heart failure patients: National Inpatient Sample study.

Aim: Diabetes mellitus (DM) is a recognized risk factor for heart failure (HF), increasing the likelihood of requiring left ventricular assist device (LVAD) therapy. Objective: This retrospective cohort study aims to assess the impact of DM on LVAD patients, focusing on in-hospital mortality as the primary outcome. Methods: Utilizing the National Inpatient Sample administrative database, data from 11,506 adult HF patients who underwent LVAD implantation were analyzed. Results: Of the patients, 44.28% had diabetes. Adjusting for various factors, diabetic patients exhibited shorter hospital stays, lower admission costs and similar in-hospital mortality rates compared with non-diabetic patients. Conclusion: These findings enhance our understanding of the risks and benefits of LVAD therapy in patients with refractory HF and DM.

In-hospital outcomes of PCI in patients who have ESRD vs non-ESRD patients, a retrospective study involving a National Inpatient Sample (NIS) database.

BACKGROUND: Cardiovascular disease is the leading cause of death for patients with end-stage renal disease (ESRD). ESRD is known to affect a large portion of the American population. Previous data for patients undergoing percutaneous coronary intervention (PCI) in the setting of ESRD for Acute Coronary Syndrome (ACS) and non-ACS etiologies have shown to have an increase in in-hospital mortality, and prolonged hospitalization among other complications. METHODS: The national inpatient sample (NIS) was used to identify patients who underwent PCI between the years 2016-2019. Patients were then grouped into those with ESRD on renal replacement therapy (RRT). Logistic regression models were employed to assess the primary outcome of in-hospital mortality, while linear regression models were utilized to evaluate secondary outcomes, including hospitalization cost and length of stay. RESULTS: A total of 21,366 unweighted observations were initially included, consisting of 50 % ESRD patients and 50 % randomly selected patients without ESRD who underwent PCI. These observations were weighted to represent a national estimate of 106,830 patients. The mean age of the study population was 65 years, and 63 % of the patients were male. The ESRD group had a greater representation of minority groups compared to the control group. The in-hospital mortality rate was significantly higher in the ESRD group compared to the control group, with an odds ratio of 1.803 (95 % CI: 1.502 to 2.164; p-value of 0.0002). Additionally, the ESRD group had significantly higher healthcare costs and longer length of stay, with a mean difference of $47,618 (95 % CI: $42,701 to $52,534, p-value <0.0001) and 2.933 days (95 % CI, 2.729 to 3.138 days, p-value <0.0001), respectively. CONCLUSION: In-hospital mortality, cost, and length of stay for patients undergoing PCI were found to be significantly greater in the ESRD group.

Cardiovascular Magnetic Resonance Imaging Phenotypes and Long-term Outcomes in Patients With Suspected Cardiac Sarcoidosis.

Importance: In patients with sarcoidosis with suspected cardiac involvement, late gadolinium enhancement (LGE) on cardiovascular magnetic resonance imaging (CMR) identifies those with an increased risk of adverse outcomes. However, these outcomes are experienced by only a minority of patients with LGE, and identifying this subgroup may improve treatment and outcomes in these patients. Objective: To assess whether CMR phenotypes based on left ventricular ejection fraction (LVEF) and LGE in patients with suspected cardiac sarcoidosis (CS) are associated with adverse outcomes during follow-up. Design, Setting, and Participants: This cohort study included consecutive patients with histologically proven sarcoidosis who underwent CMR for the evaluation of suspected CS from 2004 to 2020 with a median follow-up of 4.3 years at an academic medical center in Minnesota. Demographic data, medical history, comorbidities, medications, and outcome data were collected blinded to CMR data. Exposures: CMR phenotypes were identified based on LVEF and LGE presence and features. LGE was classified as pathology-frequent or pathology-rare based on the frequency of cardiac damage features on gross pathology assessment of the hearts of patients with CS who had sudden cardiac death or cardiac transplant. Main Outcomes and Measures: Composite of ventricular arrhythmic events and composite of heart failure events. Results: Among 504 patients (mean [SD] age, 54.1 [12.5] years; 242 [48.0%] female and 262 [52.0%] male; 2 [0.4%] American Indian or Alaska Native, 6 [1.2%] Asian, 90 [17.9%] Black or African American, 399 [79.2%] White, 5 [1.0%] of 2 or more races (including the above-mentioned categories and Native Hawaiian or Other Pacific Islander), and 2 [0.4%] of unknown race; 4 [0.8%] Hispanic or Latino, 498 [98.8%] not Hispanic or Latino, and 2 [0.4%] of unknown ethnicity), 4 distinct CMR phenotypes were identified: normal LVEF and no LGE (n = 290; 57.5%), abnormal LVEF and no LGE (n = 53; 10.5%), pathology-frequent LGE (n = 103; 20.4%), and pathology-rare LGE (n = 58; 11.5%). The phenotype with pathology-frequent LGE was associated with a high risk of arrhythmic events (hazard ratio [HR], 12.12; 95% CI, 3.62-40.57; P < .001) independent of LVEF and extent of left ventricular late gadolinium enhancement (LVLGE). It was also associated with a high risk of heart failure events (HR, 2.49; 95% CI, 1.19-5.22; P = .02) independent of age, pulmonary hypertension, LVEF, right ventricular ejection fraction, and LVLGE extent. Risk of arrhythmic events was greater with an increasing number of pathology-frequent LGE features. The absence of the pathology-frequent LGE phenotype was associated with a low risk of arrhythmic events, even in the presence of LGE or abnormal LVEF. Conclusions and Relevance: This cohort study found that a CMR phenotype involving pathology-frequent LGE features was associated with a high risk of arrhythmic and heart failure events in patients with sarcoidosis. The findings indicate that CMR phenotypes could be used to optimize clinical decision-making for treatment options, such as implantable cardioverter-defibrillators.

Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions.

OBJECTIVE: A primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. METHODS: The study included 16 patients; the mean age was 137.9 ± 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation. RESULTS: The mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 ± 3.2 and 8.6 ± 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 ± 3.1 and 1.7 ± 0.97 WU/m2, respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 ± 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery. CONCLUSIONS: First-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

Long-term prognostic value of right ventricular dysfunction on cardiovascular magnetic resonance imaging in anthracycline-treated cancer survivors.

AIMS: We aimed to determine the prevalence of right ventricular (RV) systolic dysfunction on cardiovascular magnetic resonance imaging (CMR) and its impact on long-term adverse outcomes in a large cohort of cancer survivors treated with anthracycline-based chemotherapy. METHODS AND RESULTS: Consecutive cancer survivors treated with anthracyclines who underwent clinical CMR for suspected anthracycline-related cardiomyopathy were studied. The primary endpoint was a composite of all-cause death or major adverse cardiac events (MACE): heart failure hospitalization, heart transplantation, ventricular assist device implantation, resuscitated cardiac arrest, or life-threatening ventricular arrhythmia. The secondary endpoints were all-cause death, and cardiac death or MACE. Among 249 survivors who underwent CMR at a median of 2.9 years after cancer treatment, RV systolic dysfunction was present in 54 (21.7%). Of these, 50 (92.6%) had an abnormal left ventricular ejection fraction (LVEF). At a median follow-up time after the CMR of 2.7 years, 105 survivors experienced the primary endpoint. On Kaplan-Meier analyses, the cumulative incidence of the primary endpoint was significantly higher in survivors with abnormal RVEF compared with those with normal RVEF (P = 0.002). However, on Cox multivariable analyses, RVEF was not associated with the primary endpoint (HR 1.04 per 5% decrease; 95% CI 0.93-1.17; P = 0.46) after adjustment for non-imaging variables and LVEF. RVEF was also not associated with the secondary endpoints. CONCLUSION: Among anthracycline-treated cancer survivors undergoing CMR for suspected cardiotoxicity, RV systolic dysfunction was present in one in five cases, accompanied by LV systolic dysfunction in nearly all cases, and was not independently associated with long-term outcomes.

Sex Differences in Patients With Suspected Cardiac Sarcoidosis Assessed by Cardiovascular Magnetic Resonance Imaging.

Background: There are few data on sex differences in suspected cardiac sarcoidosis. Methods: Consecutive patients with histologically proven sarcoidosis and suspected cardiac involvement were studied. We investigated sex differences in presenting features, cardiac involvement, and the long-term incidence of a primary composite end point of all-cause death or significant ventricular arrhythmia and secondary end points of all-cause death and significant ventricular arrhythmia. Results: Among 324 patients, 163 (50.3%) were female and 161 (49.7%) were male patients. Female patients had a greater prevalence of chest pain (37.4% versus 23.6%; P=0.010) and palpitations (39.3% versus 26.1%; P=0.016) than male patients but not dyspnea, presyncope, syncope, or arrhythmias at presentation. Female patients had a lower prevalence of late gadolinium enhancement on cardiovascular magnetic resonance imaging (20.2% versus 35.4%; P=0.003) and less often met criteria for a clinical diagnosis of cardiac sarcoidosis (Heart Rhythm Society consensus criteria, 22.7% versus 36.0%; P=0.012 and 2016 Japanese Circulation Society guideline criteria, 8.0% versus 19.3%; P=0.005), indicating lesser cardiac involvement. However, the long-term incidence of all-cause death or significant ventricular arrhythmia was not different between female and male patients (23.2% versus 23.2%; P=0.46). Among the secondary end points, the incidence of all-cause death was not different between female and male patients (20.7% versus 14.3%; P=0.51), while female patients had a lower incidence of significant ventricular arrhythmia compared with male patients (4.3% versus 13.0%; P=0.022). On multivariable analyses, sex was not associated with the primary end point (hazard ratio for female patients, 1.36 [95% CI, 0.77­2.43]; P=0.29). Conclusions: We observed distinct sex differences in patients with suspected cardiac sarcoidosis. A paradox was identified wherein female patients had a greater prevalence of chest pain and palpitations than male patients, but lesser cardiac involvement, and a similar long-term incidence of all-cause death or significant ventricular arrhythmia.

Mitral valve replacement in infants and younger children.

Data on mitral valve replacement (MVR) in young children is still limited. Our objective was to evaluate MVR in children below 5 years and identify factors affecting the outcomes. This retrospective study included 29 patients who had MVR from 2002 to 2020. We grouped the patients into two groups according to their age: age ≤ 24 months (n = 18) and > 24 months (n = 11). Primary cardiac diagnoses were Shone complex (n = 7; 24%), isolated congenital mitral valve abnormality (n = 11; 38%), and complete atrioventricular septal defect (n = 3; 10%). The median age was 19 month (25th-75th percentile: 11-32) and 59% were females (n = 17). The hemodynamic lesions were mitral regurgitation in 66%, mitral stenosis in 10%, and combined mitral stenosis and regurgitation in 24% of the patients. St. Jude mitral valve was the most common valve implanted (n = 19, 66%), followed by CarboMedics in 21% of the patients (n = 6). The mitral valve was implanted in the supra-annular position in 6 cases (21%). Preoperative and operative data were comparable between both groups. There was no association between valve size and position with postoperative heart block (P > 0.99, for both). The median follow-up duration was 19.4 months (8.6-102.5). Nine patients had mitral valve reoperation, six had MVR, and three had clot removal from the mitral valve. There was no effect for age group on reoperation (SHR 0.89 (95% CI 0.27-2.87), P = 0.84). Valve size significantly affected reoperation (SHR 0.39 (95% CI 0.18-0.87), P = 0.02). The supra-annular position was associated with an increased risk of reoperation (SHR 3.1 (95% CI 1.003-9.4), P = 0.049). There was no difference in survival according to the age (Log-rank P = 0.57) or valve size (Log-rank P = 0.66). Mitral valve replacement in children is associated with low morbidity and mortality. The risk of reoperation could be affected by the valve size and position rather than the age.

Outcomes of biventricular repair for shone's complex.

BACKGROUND: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. METHODS: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. RESULTS: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. CONCLUSION: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.

The outcomes of bidirectional Glenn before and after 4 months of age: A comparative study.

OBJECTIVE: We aim to present our experience with the bidirectional Glenn (BDG) in patients less than 4 months of age and to compare their outcomes with the patients who underwent BDG after the age of 4 months. METHODS: A retrospective review of data was performed for patients who underwent the BDG procedure from 2002 to 2018 at our institutions. We reviewed the patients' demographics, echocardiographic findings, cardiac catheterization data, operative details, postoperative data, and outcome variables. RESULTS: The study was conducted on 213 patients. At the time of the BDG operation, 32 patients were younger than 4 months (younger group) and 181 patients were older than 4 months (older group). The preoperative mean pulmonary artery pressure was significantly higher in the younger group (p = .035) but there were no significant differences between both groups in Qp/Qs, ventricular end-diastolic pressure, indexed pulmonary vascular resistance, and preoperative oxygen saturation. However, the initial postoperative oxygen saturation of the younger group was lower than the older group (p = .007). The duration of mechanical ventilation, duration of pleural drainage, ICU stay, and hospital stay after BDG were significantly longer in the younger group compared to the older group. The early mortality was higher in the younger group, but this difference did not reach statistical significance (p = .283). CONCLUSION: Performing BDG procedure in infants less than 4 months of age is safe, with favorable outcomes. Early BDG is associated with a less-smooth postoperative course without a significant increase in early or late mortality.

Bovine jugular vein valved xenograft for extracardiac total cavo-pulmonary connection: The risk of thrombosis and the potential liver protection effect.

BACKGROUND: Currently, non-valved conduits are preferred for extracardiac total cavo-pulmonary connection (TCPC). However, previous work has failed to provide objective data comparing the postoperative outcome between non-valved TCPCs and bovine jugular vein valved xenograft (BJV) TCPCs. Hence, the objective of this study is to compare the postoperative outcomes in extracardiac TCPC patients who received BJV vs synthetic non-valved conduits and evaluate the effect of BJV on liver fibrosis. METHODS: Of 206 patients who had extracardiac TCPC from 2002 to 2017 were divided into three groups. Group A (n = 66) received BJV, group B (n = 37) received PET conduits and group C (n = 103) received polytetrafluoroethylene (PTFE) tube. Study endpoints were hospital outcomes, conduits thrombosis, reinterventions, and survival. Liver stiffness and fibrosis were assessed in eight patients with BJV. RESULTS: Preoperative parameters were comparable among groups. Thrombosis was significantly lower in group C (P < .0003) but no difference between groups A and B (P = .951). Reinterventions did not differ significantly among groups (Log-rank P = .598). Hospital deaths occurred in seven patients (3.4%). There was no difference in survival between groups (Log-rank P = .221). The median liver stiffness score was 18.65 kPa and the eight patients had advanced liver fibrosis (grade F3-4) in group A. CONCLUSION: PTFE is the recommended conduit for TCPC with a lower risk of thrombosis compared to BJV and PET. BJV conduits in TCPC circuits may not protect against liver fibrosis. BJV should not be considered as an option for TCPC.

Evolution of the Norwood operation outcomes in patients with late presentation.

OBJECTIVES: We present the evolution of Norwood operation outcomes and practice pattern changes over 15 years from a single institution in Saudi Arabia. We intended to identify time trends in patient selection, procedural details, and outcome predictors over time. METHODS: Patients who underwent a Norwood operation (n = 145) between 2003 and 2018 with the use of a Blalock-Taussig shunt (BT group; n = 72), right ventricle to pulmonary artery shunt (Sano group; n = 66), or a primary cavopulmonary shunt (CPS group; n = 7) were included. The study outcomes were operative mortality, long-term survival, and multistate transition to CPS, Fontan, and death. RESULTS: Median age was 29 days. Predictors of operative mortality were lower weight (P = .026), and longer bypass time (P = .014), whereas age, and type of shunt were not. Predictors of improved long-term survival were greater weight at operation (P = .0016), later era (P = .006), and shorter bypass time (P = .001). The multistate model revealed that patients with lower weight were more likely to undergo Sano versus BT (P < .001), and if BT was chosen in such patients, they were more likely to die (P = .027). The likelihood of receiving Sano shunt was 3-fold greater in the recent era (P = .003). CONCLUSIONS: Improved outcomes of the Norwood operation are evident in the recent era and with Sano shunt, especially in patients of smaller weight. Late presentation or older age is not a contraindication to Norwood operation. The incorporation of a primary CPS at stage one operation is feasible in selected patients.

Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation-case report.

BACKGROUND: Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. CASE PRESENTATION: We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. CONCLUSIONS: The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

Staged Surgical Palliation for HLHS in a Girl with Severe Factor X Deficiency.

Background Factor X deficiency (also known as Stuart-Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication. Conclusion The need to maintain hemostasis while aiming to prevent intracardiac thrombosis requires multidisciplinary team approach including hematologist, cardiac surgeon, pediatric cardiac intensivist, and anesthesiologist along with meticulous hemostasis during surgery and careful monitoring of coagulation profile in the postoperative period.

Transposition of Great Arteries with Left Main Coronary Artery Atresia-Case Report.

Background The coronary artery anatomy in patients with transposition of the great artery (TGA) is a contributing factor for outcome in arterial switch procedure. Case Presentation A full-term, 7-day-old baby boy diagnosed as dextro-TGA (dTGA) with intact ventricular septum. Intraoperatively, the left coronary sinus had a blind indentation from which a firm cord-like left main coronary artery originates. Procedure completed as usual for a routine arterial switch operation. Conclusion About 5% of patients with D-TGA have a single coronary artery. Assessment of blood flow to all branches intraoperatively is mandatory to choose between either transfer of single ostium or bypass grafting to the other coronary system.