Congenital Heart Conditions Research Articles

Catheter ablation of atrial flutter in an adult with a univentricular heart, common atrium, and single atrioventricular valve: a case report—‘complex things don’t always require a complex solution’

Abstract Background The ‘univentricular’ heart encompasses a variety of congenital cardiac defects characterized by a single functional ventricle and an underdeveloped ventricular chamber. Surgical intervention, typically in infancy or childhood, aims to regulate pulmonary blood flow volume. In adulthood, untreated patients may experience limitations in physical activity and elevated morbidity due to persistent cyanosis and arrhythmias, notably after the Fontan procedure. Case summary A 38-year-old Mexican man with an unrepaired morphologically right single ventricle and a common atrium presented with palpitations. Diagnostic imaging revealed a hypertrophic systemic single ventricle with severe atrioventricular valve regurgitation and pulmonary stenosis. Despite ongoing anticoagulant and beta-blocker therapy, persistent symptoms prompted catheter ablation guided by CARTO-Merge®, a function that overlays computed tomography or magnetic resonance imaging onto a CARTO® electroanatomical map. Ablation along the cavo-annular isthmus was successfully performed, achieving arrhythmia termination. Post-ablation, the patient developed sinus rhythm and second-degree atrioventricular block, necessitating the implantation of an epicardial pacemaker. Discussion Atrial flutter ablation in univentricular hearts without prior surgery is rare. Such patients are predisposed to post-Fontan arrhythmias, often requiring intervention due to increased morbidity. Atrial flutter arises from scarred post-surgery regions, necessitating careful assessment and management. Our case demonstrates successful ablation in a complex congenital heart condition, highlighting the importance of comprehensive imaging, understanding arrhythmia mechanisms, and meticulous procedural techniques for optimal outcomes.

A prediction model of echocardiographic variables to screen for potentially correctable shunts in adult atrial septal defect-pulmonary hypertension patients.

Atrial septal defect (ASD) is a prevalent congenital heart condition in adults that leads to pulmonary hypertension (PAH) and right heart failure if left untreated. During a routine follow-up of adult ASD-PAH patients, the suitability of shunt closure depends on the invasive right heart catheterization (RHC). Nevertheless, performing RHC at every follow-up is impractical and may be harmful. The present retrospective cross-sectional study was designed to investigate which echocardiographic variables were associated with pulmonary vascular resistance (PVR) in adult ASD-PAH patients and propose a model using these variables to screen for patients with a correctable shunt. A total of 530 adult ASD-PAH patients with pulmonary arterial systolic pressure (PASP) of ≥60 mmHg measured using transthoracic echocardiogram (TTE) were included in the study. All RHCs were performed within 3 months after TTE. The correctable shunt was defined as PVR ≤3 Wood units (WU). Multivariable regressions were performed utilizing echocardiographic variables. A scoring system was constructed based on the predictors of PVR ≤3 WU using multivariable logistic regression analysis. The scoring system was then examined using a receiver operating characteristic (ROC) analysis. In addition, clinical utility of the model was determined based on decision curve analysis, and a calibration curve was used to evaluate model conformity. Estimated PASP, velocity through the pulmonary valve, tricuspid annulus early diastolic velocity, and maximum defect dimension were identified as independent predictors. The area under the ROC curve of the predictive value in the model was 0.905 (95% CI, 0.878-0.931, sensitivity: 84.3%, specificity: 83.6%). The net benefit of the model was notable in terms of a wide-range probability threshold in decision curve analysis, indicating that the prediction model had good clinical applicability. The model's calibration curve was close to an ideal diagonal line, showing good predictive accuracy between the actual and predictive probabilities. The study provided a valuable model in predicting adult ASD-PAH patients with a correctable shunt, which may help clinicians to make appropriate treatment decision for follow-up patients.

Abstract 167: A Case of Consequential Stroke and Heart Failure in Ebstein's Anomaly

Introduction Ebstein's anomaly (EA) is a rare congenital heart condition, accounting for less than 1% of congenital heart defects. Though uncommon, individuals with EA have a significantly increased risk of stroke, through poorly understood mechanisms. We present a case of a large vessel stroke in a patient with EA, coexisting patent foramen ovale (PFO), and conduction abnormalities, which ultimately required a heart transplant. Materials/Methods N/A Case A 22‐year‐old male with a history of Ebstein's anomaly and Wolff‐Parkinson‐White Syndrome presented with left middle cerebral artery (MCA) syndrome and atrial fibrillation with rapid ventricular response (AFib‐RVR). CT angiography confirmed a left MCA M1 occlusion. He received intravenous tissue plasminogen activator (tPA) followed by emergent mechanical thrombectomy, achieving modified treatment in cerebral ischemia (mTICI) grade 2B (67‐89%). MRI showed a left MCA stroke with hemorrhagic conversion, along with distal embolic strokes in the left MCA M3/M4 segments. Cardioembolic workup revealed a PFO, EA with extreme apical displacement of the tricuspid valve, and severe tricuspid regurgitation. The patient was discharged on full‐dose aspirin with plans for anticoagulation after three weeks. Despite ablation of his AFib during hospitalization, he returned with severe right heart failure, necessitating heart transplant. He currently has no neurological deficits and is followed at our institution. Discussion Ebstein's anomaly (EA) is a rare congenital heart condition occurring in 1 to 5 per 100,000 births. Characterized by tricuspid valve abnormalities, EA often presents with tricuspid regurgitation, atrialization of the right ventricle, and reduced right ventricular ejection fraction. Up to 50% of patients have associated cardiac defects, such as PFO or secundum atrial septal defect (ASD), leading to an increased risk of paradoxical embolic stroke. The risk of stroke in EA increases with age, with an incidence of 1.4% at 10 years, 15.9% at 50 years, and 23.5% at 70 years. This risk is further compounded by comorbid arrhythmias, such as AFib (20%) and accessory pathways, including Wolff‐Parkinson‐White Syndrome (10‐20%). This case highlights the importance of early identification and comprehensive management of cardiac risk factors in EA, including consideration for heart transplantation, to prevent stroke and other complications.

Echocardiographic Screening Model for Improved Assessment of Atrial Septal Defect Closure: A Multicenter Retrospective Study.

Atrial septal defect (ASD) is a prevalent congenital heart condition in adults, which finally leads to pulmonary hypertension and right heart failure if left untreated. Right heart catheterization (RHC), the current gold standard for determining ASD closure feasibility, is invasive. Thus, a noninvasive prescreening tool is urgently needed. In a multicenter, retrospective study, we assessed 924 ASD patients (2012-2022) to determine their suitability for ASD closure. Using LASSO regression, we identified predictors for a correctable shunt, enabling us to create the ASD model. The ASD model, comprising of estimated pulmonary artery systolic pressure (ePASP), peak velocity through the pulmonary valve (PV), peak E-wave velocity through the tricuspid valve (TVE), and right atrial longitudinal dimension (RA) by echocardiography, was constructed and exhibited favorable discriminative capability with an area under the curve (AUC) of 0.941 (95% CI: 0.920-0.961) in the derivation group. The model also demonstrated good calibration and discriminative abilities in the validation cohort. When juxtaposed with the earlier congenital heart disease (CHD) model, the newly developed ASD model demonstrated superior predictive capabilities for correctable shunt, supported by the net reclassification index (NRI) [0.063 (95% CI: 0.001-0.127, p = 0.047)] and integrated discrimination improvement (IDI) [0.023 (95% CI: 0.011-0.036, p <0.001)]. In summary, our research advocates the ASD model as a superior tool for screening suitable ASD defect closure candidates.

Quality of life and psychological effects of congenital heart diseases in a sample of Egyptian adults

Abstract Background Complex congenital heart disease (CHD) had very poor prognosis just a few decades ago. Nowadays, more than 90% of them, survive into adulthood, yet the complete “correction” or “repair” of CHD is not the rule and many of those patients undergo palliative interventions or surgeries. Many psychiatric and mental disorders could be seen in this group of patients. Furthermore, this could affect quality of life (QOL). Objectives Find out how prevalent anxiety and depression are among adult congenital heart disease (ACHD) patients and understand how it affects their lifestyle quality. Patients and Methods Observational case-control, cross-sectional study on 300 Adults with CHD vs 300 controls. Controls and patients were asked to fill three questionnaires to determine the prevalence of anxiety, depression, and QOL. Results Cases had higher anxiety and depression scores than controls and had poorer QOL than controls. Conclusion Congenital heart conditions adversely affect the QOL compared to a matched control group. Additionally, this cohort of patients suffers psychological distress, with more anxiety and depression in comparison to the control group. These results emphasize the drastic impact of CHD on the overall well- being of affected individuals, stressing upon the importance of holistic care that addresses both the physical and psychological aspects of their health.

Atrial Septal Defect

Background: Atrial Septal Defect (ASD) is one of the most common congenital heart diseases, occurring in about 25% of children and accounting for 6-10% of all congenital heart conditions. ASD is more frequently observed in females with a ratio of 2:1 and often does not show symptoms in infants, unless there is an increase in right ventricular volume. Case Report: A 7-year-old girl was referred with complaints of shortness of breath and cyanosis at the fingertips, accompanied by palpitations. Initial diagnosis was established through physical examination and echocardiography, which revealed a secundum ASD measuring 1.9 cm. The ASD closure surgery was performed but had to be halted due to intraoperative bradycardia. The patient then underwent subsequent therapy with Lisinopril and Spironolactone and was in good condition at the last follow-up. Discussion: Management of ASD, whether through surgical or catheter-based intervention, is recommended to prevent further complications such as paradoxical embolism and pulmonary hypertension. This case underscores the importance of timely diagnosis and treatment to prevent long-term health impacts in patients with ASD.

Case Report on Ebstein’s Anomaly with Hemoptysis and Sepsis in Eleven Years Old Girl

Ebstein's anomaly is a rare congenital heart condition, occurring in only 1 to 5 out of every 200,000 live births, accounting for less than 1% of all cases of congenital heart defects. This anomaly is characterized by the downward displacement of the septal leaflet of the tricuspid valve and the presence of an atrialized right ventricle. Our patient, an eleven-year-old girl, initially presented with symptoms such as chest pain, shortness of breath, and hemoptysis (blood vomiting). Upon examination, we identified moderate tricuspid valve regurgitation and observed an enlarged, globe-shaped heart, which led us to diagnose the child with Ebstein's anomaly. Additionally, the presence of Escherichia coli in her blood resulted in sepsis. Given the patient's overall condition, cardiovascular surgery was not considered, and she was instead provided with symptomatic treatment. This case report highlights the possibility of successful pediatric outcomes in complex congenital heart diseases, emphasizing the need for individualized surgical treatment plans.

The Power of Words: Preferred Medical Terminology of People Affected by Congenital Heart Conditions

The Power of Words: Preferred Medical Terminology of People Affected by Congenital Heart Conditions

Mid-term outcomes of minimally invasive surgery for partial atrioventricular septal defect in children

Objective: This study aims to describe the mid-term outcomes of minimally invasive surgery for partial atrioventricular septal defect in children. Summary: Partial atrioventricular septal defect is one of the three types of atrioventricular septal defect, accounting for about 3.5% of congenital heart disease. Traditionally, complete repair has been performed via sternotomy. However, with the advancement of minimally invasive surgical techniques in treating congenital heart conditions, we conducted this study to assess the feasibility and mid-term outcomes of this approach. Subjects and methods: This descriptive study spanned from January 2018 to December 2023, involving 30 patients diagnosed with partial atrioventricular septal defect who underwent minimally invasive complete repair. The cohort comprised 12 males and 18 females, with an average age of 18 months and average weight of 10.3 kg. The mean diameter of the primary atrial septal defect was 15 mm. Eight patients (26.7%) exhibited moderate to severe mitral regurgitation, and the average pulmonary artery pressure was 37 mmHg. Results: The average durations of cardiopulmonary bypass and aortic cross-clamp were 82 minutes and 58 minutes, respectively. None of the patients required sternotomy. The average time of artificial ventilator post-surgery was 6.9 hours, with a mean hospital stay of 7.6 days. Postoperative echocardiography showed promising results: complete closure of atrial septal defect, with 90% of patients having absent or mild mitral valve regurgitation, only 10% experienced moderate mitral valve regurgitation. The average follow-up period was 38 months, during which there were no postoperative fatalities. One patient required reoperation due to severe mitral regurgitation from an anterior cleft. Conclusion: Minimally invasive surgery for partial atrioventricular septal defect in children can be safely performed, yielding favorable mid-term outcomes during follow-up.

Early outcomes of minimal invasive surgery via the axillary approach for complex congenital heart disease at the Cardiovascular Center - E hospital

Objective: This study aims to outline the initial outcomes of implementing minimally invasive surgery via the right axillary approach to completely repair complex congenital heart diseases. Summary: Minimally invasive surgical techniques have gained widespread adoption in managing simple congenital heart diseases such as atrial septal defect, ventricular septal defect, partial atrioventricular septal defect, cor triatriatum, and partial anomalous pulmonary venous return. Postoperative results have demonstrated equivalence, if not superiority, to traditional sternotomy procedures while offering enhanced cosmetic benefits. However, for more intricate congenital heart conditions such as tetralogy of Fallot (TOF), complete atrioventricular septal defect (CAVSD), and Ebstein syndrome, this study evaluates the early outcomes of employing minimally invasive surgery via the right axillary approach, a technique that remains relatively uncommon globally. Our study seeks to assess the initial outcomes of this approach in managing complex congenital heart diseases. Subjects and methods: descriptive method based on a series of cases. From April 2023 to December 2023, 45 patients diagnosed with complex congenital heart disease underwent minimally invasive surgery utilizing the axillary approach. This cohort comprised 40 patients with tetralogy of Fallot, 3 patients with complete atrioventricular septal defect, and 2 patients with Ebstein syndrome. Results: All procedures were technically successful, without requiring sternotomy. The cardiopulmonary bypass times for the TOF, complete AVSD, and Ebstein syndrome groups were 81 minutes, 91 minutes, 96 minutes, respectively. Corresponding aortic cross - clamp times were 70 minutes, 72 minutes, and 74 minutes. There were no postoperative fatalities or significant complications. Postoperative echocardiographic evaluations demonstrated favorable outcomes. Conclusion: Minimally invasive surgery via the axillary approach for managing complex congenital heart diseases yields promising early results and offers notable cosmetic advantages.

Device Closure of Ventricular Septal Defect: A Retrospective Study at Pediatric Cardiac Unit, Pediatrics Hospital, Ain Shams University

Abstract Background The ventricular septal defect (VSD) accounts for 20% of all isolated congenital heart conditions. Nowadays, transcatheter closure is considered a feasible method of therapy for different types of VSDs. Objective To do a statistical analysis of VSD patients' data who underwent interventional cardiac catheterization to evaluate safety and outcome of transcatheter closure of VSDs. Methods This retrospective cohort study included review of files of 35 patients with VSDs who underwent interventional cardiac catheterization at the Pediatric Cardiac Catheterization Unit, Children’s Hospital, Ain Shams University during the period from first of January 2018 to the end of January 2021.Data were extracted from patients’ medical records, either through medical history, physical examination, pre- and post-intervention investigations, or procedural data. Results In our study, 35 VSD cases had attempts for device closure. Perimembranous VSD was present in 19 cases (54.3%), muscular VSD was present in 12 cases (34.3%), and basal muscular VSD was present in 4 cases (11.4%). Amplatzer Duct Occlude II (ADOII) was used in 17 patients (48.6%), a Muscular Amplatzer VSD device was used in 8 patients (22.9%), a PDA device was used in 8 patients (22.9%), and Amplatzer Duct Occlude I (ADOI) was attempted in 2 patients (5.7%). The most common complications in our study group were minor ones, such as mild tricuspid regurgitation, which occurred in 5 patients (14.3%), hemolysis, and blood transfusion in 1 patient (2.9%). There were no major complications such as aortic regurgitation, heart block, residual VSD, arrhythmia, device embolization, thrombus or clot at the device site, or hemolysis requiring surgical removal of the device. There was also no mortality in the study group. There was a statistically significant increase in the percentage of ADOI devices in complicated patients compared with non-complicated patients (p-value = 0.001), while no statistically significant difference was found between the two groups regarding the percentages of PDA, ADOII, and Muscular Amplatzer devices. The VSDs of all 35 patients were effectively closed, achieving a success rate 100%. Conclusion transcatheter closure is a safe and practical technique for VSDs with different morphologies. The outcomes of transatheter closure of VSDs done in our relatively new center were satisfactory and approximated to results of other international pediatric cardiac catheterization centers as regards complications, mortality, and success rate.

Temporal Trends, Health Care Resource Utilization and In-hospital Outcomes of Very Early Infective Endocarditis After Transcatheter Edge-to-Edge Repair

Current practice guidelines recommend antibiotic prophylaxis in patients with high-risk congenital heart conditions, including those with Prosthetic heart valves and prosthetic material, including annuloplasty rings or clips undergoing invasive dental procedures. However, there is limited clinical data on the burden of infective endocarditis (IE) following Mitral TEER. This study investigated temporal trends of very early IE immediately after TEER using National Inpatient Sample from 2016 - 2020. It showed that the rate of very early TEER-related IE was relatively low over the five years of analysis and that there was a downward trend of very early IE after TEER.

Isolated left ventricular non-compaction cardiomyopathy: case report and brief review on diagnostic imaging methods

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital heart disease with an estimated incidence of 0.014-0.045% in a general population. It occurs due to a morphogenetic abnormality during embryogenesis that inhibits myocardial compaction and determines prominent trabeculae with deep intertrabecular spaces and thickening of the adjacent myocardium in two distinct layers. Diagnosis is generally established via transthoracic echocardiogram (TTE) with further confirmation with cardiac magnetic resonance (CMR). A 57-year-old male patient previously diagnosed with systemic arterial hypertension and chronic obstructive pulmonary disease reported chest pain and dyspnea upon exertion, headache, and dizziness. During outpatient investigation, the patient was submitted to a stress electrocardiogram that was positive for ischemia and led to a cineangiocoronariography with no signs of coronary artery obstruction. At the echocardiography lab, several trabeculations were detected in the apical region of the left ventricle. A diagnosis of LVNC was established and the results were confirmed via CMR. Management was initiated with the goal of symptom relief. LVNC is a relatively unknown pathology that is frequently underdiagnosed at earlier stages. As presented, we highlight the importance of multimodality imaging, especially TTE and CMR, in the diagnosis of this congenital heart condition.

The effects of telerehabilitation in adults with complex biventricular congenital heart conditions: protocol for a multi-centre, randomised controlled trial—CH-FIT

BackgroundAccumulated evidence suggests that exercise training exerts beneficial effects on people with congenital heart conditions. These findings are predominantly derived from small, single-centre exercise trials conducted in outpatient rehabilitation facilities. In recent years, the delivery of exercise interventions remotely has increased through digital communications technology (telerehabilitation). However, very little research to date has been conducted into the efficacy of telerehabilitation in people with a congenital heart condition.AimsTo evaluate the effects of a telehealth-delivered exercise intervention in people with a history of a surgical biventricular repair due to a congenital heart condition.MethodsOne hundred eligible adolescent (≥ 16 years) and adult participants living with a complex biventricular congenital heart condition will be recruited from four Australian sites and randomised to either (1) a 16-week telehealth-delivered combined (aerobic and resistance) exercise training programme of moderate-to-vigorous intensity or (2) usual care (control group), in a 1:1 allocation, with an 8-month follow-up.Outcomes of interestThe primary outcome will be the change in aerobic capacity expressed as peak oxygen uptake (VO2peak). Secondary outcomes will include changes in vascular function, muscle oxygenation, metabolic profile, body composition and musculoskeletal fitness, neurohormonal activation, neurocognitive function, physical activity levels, dietary and nutritional status, and quality of life. Outcomes will be assessed at baseline, 16 weeks, and 12 months (to determine longer-term maintenance potential).DiscussionIf found to be efficacious, telerehabilitation may be an alternative option for delivering exercise, improving health outcomes, and increasing accessibility to exercise programmes. Efficacy data is required to quantify the clinical significance of this delivery mode of exercise.Trial registrationACTRN12622000050752Trial registration date: 17 January 2022Trial registration URL: https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=382635&showOriginal=true&isReview=trueTrial registry name: Australian and New Zealand Clinical Trials Registry

Using big data to analyze the vaccination status of children with congenital heart disease in Yinzhou District, China

ABSTRACT Congenital heart disease (CHD) represents a significant population warranting particular attention concerning vaccination coverage. To comprehend the vaccination status of CHD within Yinzhou District, Ningbo City, China, and to facilitate the formulation of preventive, control, and immunization strategies against vaccine-preventable diseases in children with congenital heart conditions. Using the China Yinzhou Electronic Health Record Study (CHERRY) database, we analyzed the vaccination coverage of children with CHD born between January 1, 2016 and September 20, 2021, and analyzed the influencing factors associated with the level of vaccination coverage. This study involved 762 children diagnosed with CHD at the age of 12 months, revealing that 86.74% of these children had received at least one dose of the National Immunization Program (NIP) vaccines. The coverage for non-NIP vaccines, such as the rotavirus vaccine, influenza vaccine, Influenza Haemophilus influenzae Type b (Hib) Conjugate Vaccine, 13-valent pneumococcal conjugate vaccine (PCV13), and inactivated enterovirus type 71 vaccine (EV71), stood at 27.30%, 7.74%, 63.25%, 33.76%, and 34.51%, respectively. The completion coverage for the entire vaccination schedule were 27.30%, 5.51%, 55.77%, 34.25%, and 25.59%, respectively. There was a statistically significant correlation between vaccination coverage in classification of diagnostic medical institutions and the types of diagnosed diseases. Compared to their typically developing counterparts, 12-month-old children afflicted with CHD exhibit a slightly diminished vaccination coverage, alongside a discernible inclination toward delayed vaccination. Notably, the determination to undergo vaccinations seems predominantly influenced by the classification of diagnostic medical institutions. In practical terms, proactive measures involving early diagnosis, comprehensive health assessments, and timely interventions ought to be implemented to enhance vaccination rates while prioritizing safety.

A Pregnant Patient with Sjögren’s Syndrome and non-Hodgkin Lymphoma: A Case Report

Background: Sjögren's syndrome is an autoimmune disease primarily affecting women around the age of 50, but can also occur in women of reproductive age, leading to serious maternal-fetal complications during pregnancy. The exact cause of this condition is not entirely understood, however, it is assumed to attack the exocrine glands, leading to impairment of their function. Additionally, an increased risk of developing lymphomas, particularly non-Hodgkin lymphoma is recognized among affected patients. Objective: To illustrate the management of pregnancy in a patient with Sjögren's syndrome and non-Hodgkin's lymphoma and the achievement of obstetric success without maternal-fetal complications. Case report: In the article, a 36-year-old pregnant patient with Sjögren's syndrome history, two miscarriages, and non-Hodgkin's lymphoma is presented. Due to unexpected pregnancy, the patient discontinued lymphoma treatment. Through the pregnancy, fetal well-being, blood flow, and maternal health were meticulously monitored, showcasing successful obstetric management. Due to Sjögren's syndrome, fetal atrioventricular conduction was monitored weekly from the 16th to the 30th week, showing no abnormalities. Additionally, during the course of pregnancy gestational diabetes, cervical insufficiency treated with pessary insertion, anemia and thyroid insufficiency were diagnosed. Multidisciplinary care addressed these issues, allowing our patient to give birth to a healthy child at 37 weeks via cesarean section. Conclusion: Pregnancies in Sjögren's syndrome patients pose elevated risks due to the presence of antibodies causing tissue damage and complications. These antibodies contribute to fetal myocardial issues and congenital heart conditions. Regular fetal heart ultrasounds, especially for those positive for Ro/SS-A antibodies, are crucial between the 16th and 31st weeks. Maternal-fetal complications include a higher risk of preterm delivery and lower birth weight of the offspring, often due to fetal growth restriction. Additionally, considering the potential for oncohematologic disorders in adults, comprehensive obstetric care is essential for minimizing complications risk.

Sex-Specific Differences in Nutrient Intake in Late Preterm Infants.

The study involved a retrospective analysis of preterm infants admitted to the NICU and evaluated between 34 0/7 and 36 6/7 weeks of corrected gestation. Late preterm infants appropriate for gestational age who were spontaneously fed formula milk ad-lib and free of any respiratory support for at least two days were included. The study excluded infants with short gut syndrome, severe chromosomal anomalies, or congenital heart conditions. We included 85 male and 85 female infants in this study. The data collected included sex, gestational age, birth weight, anthropometric data at birth, maternal data, nutritional intake, and neonatal morbidity. This study found that female infants consumed more volume, protein, and calories than male infants. The mean formula intake in female and male infants was 145.5 ± 20.8 mL/kg/d and 135.3 ± 19.3 mL/kg/d, respectively, with p = 0.002. However, ad-lib feeding duration was not different between the sexes. Growth velocity was also higher in female infants. This study is the first to demonstrate differences in formula milk intake among late preterm infants fed ad libitum. Additional research is needed to confirm our findings and understand sex-specific differences in neonatal nutrition in extremely early preterm infants.

Tetralogy of Fallot with vertebral defect and left aberrant subclavian artery: a rare occurrence

BackgroundThe most prevalent cyanotic congenital heart disease is Tetralogy of Fallot (TOF). It has a variety of presentations and is made up of four anatomic abnormalities. Documented literature shows an incidence of 13–20% of a right aortic arch with an anomalous left subclavian artery among individuals diagnosed with TOF. This is the first case that discusses the rare occurrence of overriding of the aortic arch along with the left aberrant subclavian artery and vertebral defect in a 3-week-old girl. Timely identification and management are pivotal in ensuring the best possible outcomes for these young patients.Case presentationA 3-week-old female child came with complaints of dyspnea, dysphagia, fatigue, and cyanosis on extreme crying, feeding, and moderate activity. Echocardiography revealed severe pulmonary stenosis with right ventricular dilatation and ventricular septal defect (VSD); a chest computed tomography was performed that revealed four characteristic features of TOF (pulmonary artery stenosis, VSD, right aortic root deviation, and concentric right ventricular hypertrophy) along with overriding of the aortic arch accompanied with the left aberrant subclavian artery (compressing the trachea and infundibulum) and vertebral defect (butterfly vertebra).ConclusionsThe case of this 3-week-old female infant emphasizes the importance of early and accurate diagnosis in congenital heart diseases, particularly when faced with complex presentations such as the TOF. It serves as a testament to the valuable role of advanced diagnostic imaging techniques in unraveling the complexity of congenital heart conditions.

A season of change: managing menopause with adult congenital heart disease

In this patient story, Beth Greenaway describes her experience of menopause and the additional challenges that a congenital heart condition can bring to this life stage.

Isolated Double-Chambered Right Ventricle: A Rare Congenital Heart Disease

Double-chambered right ventricle (DCRV) is an uncommon congenital heart condition characterized by a progressive obstruction in the right ventricular outflow tract. This obstruction is caused by anomalous muscles or fibrous tissues that divide the right ventricle into two cavities: a proximal high-pressure chamber (anatomically lower) and a distal low-pressure chamber (anatomically higher). We present a case of a middle-aged man with a medical history of recurrent symptomatic dyspnea. Upon presentation, there were no signs of congestive cardiac failure. The diagnosis was confirmed using transthoracic two-dimensional (2-D) echocardiography and Transesophageal echocardiography (TEE) for more precise characterization. The primary objective of this case report is to highlight the rarity of this congenital heart disease, particularly in adults.