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Background: Gestational diabetes mellitus (GDM) is the most common complication in pregnancy, representing a serious risk for the mother and fetus. Identifying new biomarkers to ameliorate the screening and improving GDM diagnosis and treatment is crucial. During the last decade, a few studies have used speckle tracking echocardiography (STE) for assessing the myocardial deformation properties of fetuses (FGDM) and infants (IGDM) of GDM women, providing not univocal results. Accordingly, we performed a meta-analysis to examine the overall influence of GDM on left ventricular (LV) and right ventricular (RV) global longitudinal strain (GLS) in both FGDM and IGDM. Methods: All echocardiographic studies assessing conventional echoDoppler parameters and biventricular strain indices in FGDM and IGDM vs. infants born to healthy pregnant women, selected from PubMed and EMBASE databases, were included. The studies performed on FGDM and IGDM were separately analyzed. The subtotal and overall standardized mean differences (SMDs) in LV-GLS and RV-GLS in FGDM and IGDM studies were calculated using the random-effect model. Results: The full texts of 18 studies with 1046 babies (72.5% fetuses) born to GDM women and 1573 babies of women with uncomplicated pregnancy (84.5% fetuses) were analyzed. Compared to controls, FGDM/IGDM were found with a significant reduction in both LV-GLS [average value −18.8% (range −11.6, −24.2%) vs. −21.5% (range −11.8, −28%), p < 0.05)] and RV-GLS [average value −19.7% (range −13.7, −26.6%) vs. −22.4% (range −15.5, −32.6%), p <0.05)]. Large SMDs were obtained for both LV-GLS and RV-GLS studies, with an overall SMD of −0.91 (95%CI −1.23, −0.60, p < 0.001) and −0.82 (95%CI −1.13, −0.51, p < 0.001), respectively. Substantial heterogeneity was detected for both LV-GLS and RV-GLS studies, with an overall I2 statistic value of 92.0% and 89.3%, respectively (both p < 0.001). Egger's test gave a p-value of 0.10 for LV-GLS studies and 0.78 for RV-GLS studies, indicating no publication bias. In the meta-regression analysis, none of the moderators (gestational age, maternal age, maternal body mass index, maternal glycosylated hemoglobin, white ethnicity, GDM criteria, ultrasound system, frame rate, FGDM/IGDM heart rate, and anti-diabetic treatment) were significantly associated with effect modification in both groups of studies (all p > 0.05). The sensitivity analysis supported the robustness of the results. Conclusions: GDM is independently associated with biventricular strain impairment in fetuses and infants of gestational diabetic mothers. STE analysis may allow for the early detection of subclinical myocardial dysfunction in FGDM/IGDM. [ABSTRACT FROM AUTHOR]

Objective: Congenital heart disease is a risk factor for mortality after orthotopic heart transplantation; however, the impact of preoperative circulation type and primary congenital heart disease diagnosis remains poorly delineated. Methods: We retrospectively reviewed patients with adult congenital heart disease aged 16 years or more who underwent orthotopic heart transplantation at our institution between 2008 and 2022. Patients were categorized as having single-ventricle or biventricular circulation. The primary end point was 5-year post-transplant survival. Results: Sixty-one patients with adult congenital heart disease (single-ventricle: n = 26 [42.6%], biventricular: n = 35 [57.4%]) underwent orthotopic heart transplantation at 33.7 [interquartile range, 19.1-48.7] years. The most common congenital heart disease diagnosis was hypoplastic left heart syndrome (n = 11, 42.3%) in the single-ventricle group and congenitally corrected transposition of the great arteries (n = 7, 20.0%) in the biventricular group. Twenty-four patients previously underwent Fontan palliation. At transplant, patients in the single-ventricle group were younger (18.5 [interquartile range, 17.6-32.3] years vs 45.0 [interquartile range, 33.0-52.2] years, P

Background: Gestational diabetes mellitus (GDM) is the most common complication in pregnancy, representing a serious risk for the mother and fetus. Identifying new biomarkers to ameliorate the screening and improving GDM diagnosis and treatment is crucial. During the last decade, a few studies have used speckle tracking echocardiography (STE) for assessing the myocardial deformation properties of fetuses (FGDM) and infants (IGDM) of GDM women, providing not univocal results. Accordingly, we performed a meta-analysis to examine the overall influence of GDM on left ventricular (LV) and right ventricular (RV) global longitudinal strain (GLS) in both FGDM and IGDM. Methods: All echocardiographic studies assessing conventional echoDoppler parameters and biventricular strain indices in FGDM and IGDM vs. infants born to healthy pregnant women, selected from PubMed and EMBASE databases, were included. The studies performed on FGDM and IGDM were separately analyzed. The subtotal and overall standardized mean differences (SMDs) in LV-GLS and RV-GLS in FGDM and IGDM studies were calculated using the random-effect model. Results: The full texts of 18 studies with 1046 babies (72.5% fetuses) born to GDM women and 1573 babies of women with uncomplicated pregnancy (84.5% fetuses) were analyzed. Compared to controls, FGDM/IGDM were found with a significant reduction in both LV-GLS [average value −18.8% (range −11.6, −24.2%) vs. −21.5% (range −11.8, −28%), p < 0.05)] and RV-GLS [average value −19.7% (range −13.7, −26.6%) vs. −22.4% (range −15.5, −32.6%), p p < 0.001) and −0.82 (95%CI −1.13, −0.51, p < 0.001), respectively. Substantial heterogeneity was detected for both LV-GLS and RV-GLS studies, with an overall I2 statistic value of 92.0% and 89.3%, respectively (both p < 0.001). Egger’s test gave a p-value of 0.10 for LV-GLS studies and 0.78 for RV-GLS studies, indicating no publication bias. In the meta-regression analysis, none of the moderators (gestational age, maternal age, maternal body mass index, maternal glycosylated hemoglobin, white ethnicity, GDM criteria, ultrasound system, frame rate, FGDM/IGDM heart rate, and anti-diabetic treatment) were significantly associated with effect modification in both groups of studies (all p > 0.05). The sensitivity analysis supported the robustness of the results. Conclusions: GDM is independently associated with biventricular strain impairment in fetuses and infants of gestational diabetic mothers. STE analysis may allow for the early detection of subclinical myocardial dysfunction in FGDM/IGDM.

ABSTRACT: Background: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV). Methods: Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m2) and no endocardial fibroelastosis who underwent BiV repair from 2005–2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation. Results: Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p

Abstract Objective Pacing in a univentricular circulation has been associated with worsened outcomes. We investigated the long‐term outcomes of pacing in children with a univentricular circulation compared to a complex biventricular circulation. We also identified predictors of adverse outcomes. Methods A retrospective study of all children with major congenital heart disease who underwent pacemaker implantation under the age of 18 years between November 1994 and October 2017. Results Eighty‐nine patients were included; 19 with a univentricular and 70 with a complex biventricular circulation. A total of 96% of pacemaker systems were epicardial. Median follow up was 8.3 years. The incidence of adverse outcome was similar between the two groups. Five (5.6%) patients died and two (2.2%) underwent heart transplantation. Most adverse events occurred within the first 8 years after pacemaker implantation. Univariate analysis identified five predictors of adverse outcomes in the patients in the biventricular but none in the univentricular group. The predictors of adverse outcome in the biventricular circulation were a right morphologic ventricle as the systemic ventricle, age at first congenital heart disease (CHD) operation, number of CHD operations, and female gender. The nonapical lead position was associated with a much higher risk of an adverse outcome. Conclusions Children with a pacemaker and a complex biventricular circulation have similar survival to the ones with a pacemaker and a univentricular circulation. The only modifiable predictor was the epicardial lead position on the paced ventricle, emphasizing the importance of apical placement of the ventricular lead.

Objectives: The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality. Methods: A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan–Meier analysis for survival were performed. Results: Patient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing (P = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P

Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023. Early evidence demonstrates that SV circulation is not only a hemodynamic burden requiring staged palliation, but also a metabolic issue with alterations similar to what is known for HF in a BV circulation. Alterations of fatty acid and glucose oxidation were found, resulting in metabolic instability and impaired energy production. As reported for patients with BV HF, stimulating ketone oxidation may be an effective treatment strategy for HF in these patients. Few but promising clinical trials have been conducted thus far to evaluate therapeutic ketosis with HF using a variety of instruments, including ketogenic diet, ketone esters, and sodium-glucose co-transporter-2 (SGLT2) inhibitors. An initial trial on a small cohort demonstrated favorable outcomes for Fontan patients treated with SGLT2 inhibitors. Therapeutic ketosis is worth considering in the treatment of Fontan patients, as ketones positively affect not only the myocardial energy metabolism, but also the global Fontan physiopathology. Induced ketosis seems promising as a concerted therapeutic strategy. [ABSTRACT FROM AUTHOR]

Objective: Pacing in a univentricular circulation has been associated with worsened outcomes. We investigated the long‐term outcomes of pacing in children with a univentricular circulation compared to a complex biventricular circulation. We also identified predictors of adverse outcomes. Methods: A retrospective study of all children with major congenital heart disease who underwent pacemaker implantation under the age of 18 years between November 1994 and October 2017. Results: Eighty‐nine patients were included; 19 with a univentricular and 70 with a complex biventricular circulation. A total of 96% of pacemaker systems were epicardial. Median follow up was 8.3 years. The incidence of adverse outcome was similar between the two groups. Five (5.6%) patients died and two (2.2%) underwent heart transplantation. Most adverse events occurred within the first 8 years after pacemaker implantation. Univariate analysis identified five predictors of adverse outcomes in the patients in the biventricular but none in the univentricular group. The predictors of adverse outcome in the biventricular circulation were a right morphologic ventricle as the systemic ventricle, age at first congenital heart disease (CHD) operation, number of CHD operations, and female gender. The nonapical lead position was associated with a much higher risk of an adverse outcome. Conclusions: Children with a pacemaker and a complex biventricular circulation have similar survival to the ones with a pacemaker and a univentricular circulation. The only modifiable predictor was the epicardial lead position on the paced ventricle, emphasizing the importance of apical placement of the ventricular lead. [ABSTRACT FROM AUTHOR]

Background Vertebrobasilar dolichoectasia (VBDE) is defined as a symptomatic dilatation and tortuosity of the vertebrobasilar arteries. The risk of hydrocephalus development is due to direct compression of the third ventricle outflow or brainstem compression and related aqueduct stenosis. We present an uncommon case of a patient with symptomatic VBDE with the uniqueness of a hypoplastic third ventricle associated with biventricular hydrocephalus. A literature review concerning diagnosis and management of patients affected by biventricular hydrocephalus caused by VBDE was also performed. Case Illustration We report a case of a 54-year-old man who presented with headache, ideomotor apraxia, and gait disorder. A head computed tomography (CT) scan showed a biventricular hydrocephalus and a subsequent CT angiography documented the presence of a VBDE compressing the anterior part of the third ventricle that also appeared hypoplastic. The patient also presented a clinical history of arterial hypertension for which he was given a proper pharmacologic treatment with symptom relief. A surgical treatment of ventriculoperitoneal shunt along with endoscopic septostomy was proposed, but the patient refused, probably due to the slightly positive response to medical treatment. Conclusions The natural clinical history of patients affected by VBDE is unfavorable with 7.8 years of median survival. The therapeutic strategy is usually conservative and the role of antiplatelets or oral anticoagulants is still debated. In selected patients, ventriculoperitoneal shunt to resolve intracranial hypertension caused by biventricular hydrocephalus is the most effective treatment. In our opinion, chronic third ventricle compression could lead to anatomic–pathologic alterations like the third ventricle hypoplasia documented in our report. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). METHODS All children born with DORV between 2003 and 2017 were identified in the Oslo University Hospital registry. Patients' characteristics, interventions, complications and deaths were recorded. Echocardiographic data were reviewed for classification according to current standards. We investigated time-dependent surgical reintervention and mortality using Kaplan–Meier analyses and determinants of treatment complications, reintervention and death using regression analyses. RESULTS Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. Six children received palliative care. With an intention to treat, a surgical route with the primary biventricular repair was followed for 62 children, staged biventricular repair for 15 and univentricular repair for 10 children. Major complications occurred in 1.0% and 6.2% of children following catheter or surgical intervention, respectively. No significant determinants of the complications were identified. Overall survival following treatment was 91.9%, 90.8%, 89.5% and 89.5% and corresponding freedom from surgical reintervention was 88.0%, 79.0%, 74.9% and 69.4% at 1, 2, 5 and 10 years, respectively. The presence of atrioventricular septal defect predicted an increased risk of mortality (hazard ratio: 7.16) but did not increase the risk of surgical reintervention. CONCLUSIONS In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death. [ABSTRACT FROM AUTHOR]

Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023. Early evidence demonstrates that SV circulation is not only a hemodynamic burden requiring staged palliation, but also a metabolic issue with alterations similar to what is known for HF in a BV circulation. Alterations of fatty acid and glucose oxidation were found, resulting in metabolic instability and impaired energy production. As reported for patients with BV HF, stimulating ketone oxidation may be an effective treatment strategy for HF in these patients. Few but promising clinical trials have been conducted thus far to evaluate therapeutic ketosis with HF using a variety of instruments, including ketogenic diet, ketone esters, and sodium-glucose co-transporter-2 (SGLT2) inhibitors. An initial trial on a small cohort demonstrated favorable outcomes for Fontan patients treated with SGLT2 inhibitors. Therapeutic ketosis is worth considering in the treatment of Fontan patients, as ketones positively affect not only the myocardial energy metabolism, but also the global Fontan physiopathology. Induced ketosis seems promising as a concerted therapeutic strategy.

Aortic atresia is a rare finding and has not been previously described with superior-inferior ventricles. Presented here is a case of a heart with these concomitant findings and review of reported cases of aortic atresia in the absence of hypoplastic left heart syndrome. The aim of this report is to help highlight associated findings and the clinical approach taken. Also highlighted is the importance of not mistaking aortic atresia for common arterial trunk.

Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV). Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m2) and no endocardial fibroelastosis who underwent BiV repair from 2005–2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation. Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <−2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <−2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries. In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair. [Display omitted] [ABSTRACT FROM AUTHOR]

Acute coronary syndromes are a clinical entity frequently encountered in practice and are responsible for significant morbidity and mortality, despite therapeutic advances. The initiation of early reperfusion therapy reduces mortality and morbidity and improves patients' prognosis, but this depends on how quickly patients receive their treatment. Although it is often easy to diagnose in the presence of typical symptoms, certain patients, such as diabetics, sometimes have atypical symptoms, resulting in a delay in management. In nearly 50% of cases, inferior wall ischaemia is accompanied by right ventricular myocardial infarction; the clinical outcomes range from no hemodynamic compromise to severe hypotension and cardiogenic shock. In this article, we present the case of a 54-year-old male patient with active smoking and poorly controlled type 2 diabetes as cardiovascular risk factors who initially consulted at the first hour for epigastric pain, for which he received symptomatic treatment. As the symptoms persisted, he was admitted to our department at the eighth hour, where he was diagnosed with a biventricular infarction., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Statistical shape modeling (SSM) is a valuable and powerful tool to generate a detailed representation of complex anatomy that enables quantitative analysis and the comparison of shapes and their variations. SSM applies mathematics, statistics, and computing to parse the shape into a quantitative representation (such as correspondence points or landmarks) that will help answer various questions about the anatomical variations across the population. Complex anatomical structures have many diverse parts with varying interactions or intricate architecture. For example, the heart is a four-chambered anatomy with several shared boundaries between chambers. Coordinated and efficient contraction of the chambers of the heart is necessary to adequately perfuse end organs throughout the body. Subtle shape changes within these shared boundaries of the heart can indicate potential pathological changes that lead to uncoordinated contraction and poor end-organ perfusion. Early detection and robust quantification could provide insight into ideal treatment techniques and intervention timing. However, existing SSM approaches fall short of explicitly modeling the statistics of shared boundaries. In this paper, we present a general and flexible data-driven approach for building statistical shape models of multi-organ anatomies with shared boundaries that captures morphological and alignment changes of individual anatomies and their shared boundary surfaces throughout the population. We demonstrate the effectiveness of the proposed methods using a biventricular heart dataset by developing shape models that consistently parameterize the cardiac biventricular structure and the interventricular septum (shared boundary surface) across the population data.