Totally peripheral approach for ICD lead vegetation removal in a GUCH patient.

AngioVac system (AngioDynamics) has already proved to be a useful tool in the treatment of thrombotic and endocarditic formations concerning the venous district. Herein, the AngioVac aspiration system combined with the bidirectional rotational Evolution mechanical sheath lead extraction was used for an effective and safety cardiac-device-related-infective-endocarditis removal in a grown-up congenital heart disease patient through a totally peripheral approach.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) The impact of COVID-19 in individuals with congenital heart disease through the life span. Patients with a genetic syndrome and adults at advanced physiological stage were at highest risk for moderate/severe infection. (2) Echocardiographic findings of the multisystem inflammatory syndrome in children showing a high prevalence of myocardial injury and systolic dysfunction that improves in the subacute phase. (3) A score assessment of the Fontan associated liver disease which correlated with the risk for Fontan failure. (4) Grown-up congenital heart surgery in 1093 consecutive cases showed that the 30 day mortality may underestimate the mortaility and that the 6 months mortality is likely a better measure in this population. (5) Cone versus conventional repair for Ebstein's anomaly showed better midterm results and freedom from tricuspid regurgitation after the cone operation. (6) Association between race/ethnicity, illness severity, and mortality in children undergoing cardiac surgery. The study showed that the African American race associated with increased disease severity and thus higher postoperative mortality compared to the caucausian race.

High prevalence of ascending aortic dilation in adults with repaired coarctation of the aorta.

BACKGROUND: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. METHODS AND RESULTS: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). CONCLUSIONS: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

Double-chambered left ventricle: An unusual finding in a middle-aged woman.

Double-chambered left ventricle (DCLV) is a rare congenital abnormality and is characterized by the subdivision of the left ventricle into two separate chambers by an abnormal septum or muscle band. This rare congenital entity is usually diagnosed in the neonatal or pediatric age. Nevertheless, it can be asymptomatic and incidentally found in the adulthood. A comprehensive multimodality imaging approach is crucial for the correct final diagnosis.

New developments in adult congenital heart disease.

Congenital heart disease (CHD) affects 0.8% of live births and over the past decades technical improvements and large-scale repair has led to increased survival into adulthood of over 95% of the new-born. A new group of patients, those who survived their congenital heart defect, has emerged but late complications including heart failure, pulmonary hypertension (PH), arrhythmias, aneurysms and endocarditis appeared numerous, with a huge impact on mortality and morbidity. However, innovations over the past years have changed the landscape of adult CHD dramatically. In the diagnostic process important improvements have been made in the use of MRI, biomarkers, e­health concepts and 3D visualisation of anatomy. Care is now concentrated in specialised centres, with a continuous emphasis on education and the introduction of weekly multidisciplinary consultations on diagnosis and intervention. Surgery and percutaneous intervention have been refined and new concepts applied, further reducing the burden of the congenital malformations. Research has matured from case series to global networks. Currently, adults with CHD are still facing high risks of early mortality and morbidity. By global collaboration and continuous education and development and innovation of our diagnostic and therapeutic arsenal, we will improve the perspectives of these young patients.

Adults with congenital heart disease: ready for mobile health?

PURPOSE: Mobile health (mHealth) could improve the outcome of grown-up patients with congenital heart disease (GUCH) and reduce their emergency care utilisation. Inappropriate use of mHealth, however, can lead to data overload for professionals and unnecessary data collection for patients, increasing the burden for both. We aimed to determine the clinical characteristics of patients with high emergency care utilisation and to test whether these patients were willing to start using mHealth. METHODS: Clinical characteristics and emergency care utilisation of consecutive GUCH patients who visited one of the two participating cardiologists at the outpatient clinic of the Academic Medical Centre in Amsterdam were studied retrospectively. All patients were approached to fill in an mHealth questionnaire. A frequency of three or more emergency visits in 5 years was defined as high emergency care utilisation. RESULTS: In total, 202 consecutive GUCH patients who visited one of the two participating cardiologists were studied. Median age was 41 years, 47% were male, and 51% were symptomatic. In the previous 5 years, 134 emergency visits were identified. Of all patients, 8% had high emergency care utilisation. High emergency care utilisation was associated with patients being symptomatic, using antiarrhythmic drugs or diuretics. In total, 75% of all patients with high emergency care utilisation were willing to start using mHealth. CONCLUSION: GUCH patients who are symptomatic, those on antiarrhythmic drug therapy and those on diuretics are suitable candidates for enrolment in future mHealth initiatives because of both high care utilisation and high motivation to start using mHealth.

Is Grown Up Congenital Heart (GUCH) disease different in a developing country?

In the current era grown up congenital heart disease (GUCH) patients undergoing surgical interventions are increasing. Most of the interventions in the developed countries are either complex or redo-operations in patients who had previously undergone repair, palliation or correction. However, in the developing countries most of the interventions are primary and corrective. This descriptive retrospective study comprised GUCH patients who underwent surgical intervention for congenital heart disease (CHD) at Aga Khan University Hospital, Karachi, from January 2006 to December 2015. A total of 195 patients had been treated surgically with a mean age of 31.0±13.5 years. Majority of the patients underwent surgical interventions for closure of atrial 109(55.3%) and ventricular 51(26.2%) septal defect. The most common complications were prolonged ventilation 16(8.1%). Overall mortality was 4(2.1%). GUCH in our practice is for primary procedure with simple diagnosis that should have been treated before reaching adulthood as is done in the developed countries.

[Change in our approach in the surgical management of congenital heart defects in patients with Down syndrome, 1974-2016]. / Szemléletváltozás a Down-szindrómás szívbetegek szívsebészeti kezelésében, 1974­2016.

INTRODUCTION: Congenital heart defects are frequently present in patients with Down syndrome. AIM: The authors analyzed the impact of changing approach in surgical management of congenital heart defect on the life expectancy of patients with Down syndrome. METHOD: Between 1974 and 1997 the data of 359 children with Down syndrome were collected. Among them 255 patients had no surgery and the mortality in this group was 25.9%, whereas the mortality in the group of 104 patients who underwent palliative surgery was 8.6%. RESULTS: Surgical management of congenital heart defects provides the same life expectancy for these patients as compared to Down patients without cardiac defects. Primary reconstruction is the preferable surgical procedure in infancy that provides good results. Nowadays the number of the operated grown-up congenital heart disease patients with Down syndrome is increasing. During the last three years 82 grown-up congenital heart disease patients, including 4 patients with Down syndrome (aged between 24 and 60 years) were reconstructed successfully. CONCLUSIONS: Due to the successful surgery in infancy the population of grown-up congenital heart disease patients with Down syndrome is increasing. The cardiac surgeons are ready to do everything for the optimal life expectancy of these patients. However, management of special problems (indication and necessity of reoperation, optimal age) in patients with Down syndrome poses a great challenge for cardiologists and cardiac surgeons. Orv. Hetil., 2016, 157(40), 1601-1603.

Mobile health in adults with congenital heart disease: current use and future needs.

OBJECTIVE: Many adults with congenital heart disease (CHD) are affected lifelong by cardiac events, particularly arrhythmias and heart failure. Despite the care provided, the cardiac event rate remains high. Mobile health (mHealth) brings opportunities to enhance daily monitoring and hence timely response in an attempt to improve outcome. However, it is not known if adults with CHD are currently using mHealth and what type of mHealth they may need in the near future. METHODS: Consecutive adult patients with CHD who visited the outpatient clinic at the Academic Medical Center in Amsterdam were asked to fill out questionnaires. Exclusion criteria for this study were mental impairment or inability to read and write Dutch. RESULTS: All 118 patients participated (median age 40 (range 18-78) years, 40 % male, 49 % symptomatic) and 92 % owned a smartphone. Whereas only a small minority (14 %) of patients used mHealth, the large majority (75 %) were willing to start. Most patients wanted to use mHealth in order to receive more information on physical health, and advice on progression of symptoms or signs of deterioration. Analyses on age, gender and complexity of defect showed significantly less current smartphone usage at older age, but no difference in interest or preferences in type of mHealth application for the near future. CONCLUSION: The relatively young adult CHD population only rarely uses mHealth, but the majority are motivated to start using mHealth. New mHealth initiatives are required in these patients with a chronic condition who need lifelong surveillance in order to reveal if a reduction in morbidity and mortality and improvement in quality of life can be achieved.

Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease.

Although not completely devoid of risk, pregnancy can be managed in virtually all patients affected by even the most complex forms of congenital heart disease. It is not however advisable in patients with any form of pulmonary arterial hypertension. Pregnancy is even manageable in patients with univentricular heart converted to Fontan circulation. A personalised risk stratification should be performed, and patients affected by advanced NYHA functional class appropriately warned of the potential risks. In this setting, metabolomics might represent a novel tool for use in conducting personalised risk stratification. All pregnancies, particularly those at higher risk, should be managed in a tertiary care centre capable of providing the necessary assistance to both the mother and infant. With a few rare exceptions, vaginal delivery is to be preferred over caesarean section due to the lower degree of maternal and foetal complications. The desire for motherhood, at times extreme in women with congenital heart disease, may often be accomplished, thus providing a ray of hope in the lives of these patients.

Assessing the feasibility of using the antecubital vein to perform right heart catheterization in children and adults with congenital heart disease: a retrospective, observational single-center study.

BACKGROUND: Right heart catheterization (RHC) usually is performed via the femoral vein or the internal jugular vein. However, the antecubital fossa vein is a valid venous access, and it has become increasingly popular to perform right heart catheterization utilizing this access. METHODS: A retrospective, observational study was conducted to describe use of the antecubital fossa vein for right heart catheterization in adults and children with congenital heart disease (CHD). Patients who had undergone RHC via antecubital fossa vein at the authors' hospital between September 2019 and December 2022 were included. The outcomes studied were procedural failure and procedure-related adverse events. RESULTS: Fifty-two patients with CHD underwent right cardiac catheterization via an upper arm vein. The upper arm vein was unable to perform the RHC in only 2 patients (3.8%). Only 1 patient developed a minor adverse event. No irreversible and/or life-threating adverse events were detected. CONCLUSIONS: The upper arm veins are safe and effective to perform a RHC in children and adults with CHD. This approach demonstrates a high percentage of technical success, and few mild complications.

Minimal-Access Atrial Septal Defect (ASD) Closure.

Progress towards the development and adoption of minimally invasive techniques in cardiac surgery has been slower than that seen in other surgical specialties. Congenital heart disease (CHD) patients represent an important population within cardiac disease, of which atrial septal defect (ASD) is one of the most common diagnoses. Management of ASD encompasses a range of minimal-access and minimally invasive approaches, including transcatheter device closure, mini-sternotomy, thoracotomy, video-assisted, endoscopic, and robotic approaches. In this article, we will discuss the pathophysiology of ASD, along with diagnosis, management, and indications for intervention. We will review the current evidence supporting minimally invasive and minimal-access surgical ASD closure in the adult and paediatric patient, highlighting peri-operative considerations and areas for further research.

Inadvertent left ventricular placement of ICD lead through the left subclavian artery right positioned in a patient with situs viscerum inversus and Kartagener syndrome.

Inadvertent placement of pacemaker and implantable cardioverter-defibrillator (ICD) leads in the left ventricle is a rare but well-recognized complication of device implantation. We report a case of an unicameral ICD lead inadvertently placed through the left subclavian artery right positioned, across the aortic valve into the left ventricle, in a patient with situs viscerum inversus. A transthoracic echocardiogram about a month after the procedure showed an unusual course of the lead. The lead was successfully removed without complications or sequelae. .

Perioperative Management of an Adolescent Fontan Patient with Narrowing of the Aortopulmonary Space for Posterior Scoliosis Surgery.

Congenital cardiac patients who received neonatal reconstructive aortic arch surgery are at risk of aortopulmonary space narrowing with compression of the left pulmonary artery and left main bronchus (LMB) later in life. We discuss a challenging adolescent single ventricle patient who presented for surgical treatment of a non-idiopathic thoracic scoliosis (posterior spinal fusion) with severe stenosis of the LMB and left pulmonary artery due to a narrow aortopulmonary space. Careful preoperative imaging, evaluation, and decision making resulted in successful surgical treatment and uneventful perioperative course.

A Late Complication in a Surgically Corrected ALCAPA Patient.

Late complications in surgically corrected ALCAPA patients are rare. We describe an interesting case of a patient with a thrombosed giant right coronary artery aneurysm which was discovered on a chest X-ray. (Level of Difficulty: Intermediate.).

Right Ventricular Volume and Function Assessment in Congenital Heart Disease Using CMR Compressed-Sensing Real-Time Cine Imaging.

BACKGROUND AND OBJECTIVE: To evaluate the reliability of compressed-sensing (CS) real-time single-breath-hold cine imaging for quantification of right ventricular (RV) function and volumes in congenital heart disease (CHD) patients in comparison with the standard multi-breath-hold technique. METHODS: Sixty-one consecutive CHD patients (mean age = 22.2 ± 9.0 (SD) years) were prospectively evaluated during either the initial work-up or after repair. For each patient, two series of cine images were acquired: first, the reference segmented multi-breath-hold steady-state free-precession sequence (SSFPref), including a short-axis stack, one four-chamber slice, and one long-axis slice; then, an additional real-time compressed-sensing single-breath-hold sequence (CSrt) providing the same slices. Two radiologists independently assessed the image quality and RV volumes for both techniques, which were compared using the Wilcoxon test and paired Student's t test, Bland-Altman, and linear regression analyses. The visualization of wall-motion disorders and tricuspid-regurgitation-related signal voids were also analyzed. RESULTS: The mean acquisition time for CSrt was 22.4 ± 6.2 (SD) s (95% CI: 20.8-23.9 s) versus 442.2 ± 89.9 (SD) s (95% CI: 419.2-465.2 s) for SSFPref (p < 0.001). The image quality of CSrt was diagnostic in all examinations and was mostly rated as good (n = 49/61; 80.3%). There was a high correlation between SSFPref and CSrt images regarding RV ejection fraction (49.8 ± 7.8 (SD)% (95% CI: 47.8-51.8%) versus 48.7 ± 8.6 (SD)% (95% CI: 46.5-50.9%), respectively; r = 0.94) and RV end-diastolic volume (192.9 ± 60.1 (SD) mL (95% CI: 177.5-208.3 mL) versus 194.9 ± 62.1 (SD) mL (95% CI: 179.0-210.8 mL), respectively; r = 0.98). In CSrt images, tricuspid-regurgitation and wall-motion disorder visualization was good (area under receiver operating characteristic curve (AUC) = 0.87) and excellent (AUC = 1), respectively. CONCLUSIONS: Compressed-sensing real-time cine imaging enables, in one breath hold, an accurate assessment of RV function and volumes in CHD patients in comparison with standard SSFPref, allowing a substantial improvement in time efficiency.

Imaging the adult with simple shunt lesions: position paper from the EACVI and the ESC WG on ACHD. Endorsed by AEPC (Association for European Paediatric and Congenital Cardiology).

In 2018, the position paper 'Imaging the adult with congenital heart disease: a multimodality imaging approach' was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

Small atrial septal defects are associated with psychiatric diagnoses, emotional distress, and lower educational levels.

OBJECTIVE: For the first time, we wish to assess the psychiatric burden in adult patients living with small, unrepaired atrial septal defects (ASD) using register-based data, combined with self-reported measures on levels emotional distress and educational status. DESIGN: A descriptive study using both the unique Danish registries and validated psychiatric questionnaires and scales, including: The Symptom Checklist, Whiteley-7, and Brief Illness Perception Questionnaire. PATIENTS: Adult patients with small, unrepaired ASD, diagnosed between 1953 and 2011. OUTCOME MEASURES: Number of register-based psychiatric diagnoses. Additionally, symptoms of anxiety, depression, somatization, health anxiety, illness perception, and levels of educational attainment compared to age- and gender-matched individuals from the reference group. RESULTS: We identified 723 Danish patients with a small, unrepaired ASD. Approximately 17% of the patients eligible for inclusion had one or more psychiatric diagnoses. The most common diagnoses were neurotic, stress-related, and somatoform disorders. A total of 140 patients (mean age 33 years) was included for examination of their mental health using psychiatric questionnaires and scales. Patients with small ASD had higher scores of depression and anxiety when compared to the reference group (0.57 ± 0.67 vs 0.39 ± 0.52; P < .001). Furthermore, patients reported more symptoms of somatization (0.51 ± 0.48 vs 0.34 ± 0.39; P < .001). Finally, a higher percentage of patients with an unrepaired ASD had no education beyond high school when compared to the reference group (33.3% vs 14.3%; P < .001) and a smaller proportion of patients had completed a short-cycle higher education (6.5% vs 16.1%; P = .002). CONCLUSION: Patients with a small, unrepaired ASD in adult life have more symptoms of anxiety, depression, and somatization compared to the reference group. Furthermore, fewer patients had no education beyond high school. These results support the guidelines that adults with small ASD warrants regular lifelong follow-up in specialized clinics aware of not only adverse somatic outcome but also rates of depression and anxiety.

Small unrepaired atrial septal defects display impaired exercise capacity compared with healthy peers.

OBJECTIVE: Adult patients with small, unrepaired atrial septal defects have an increased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have higher late mortality than the background population. The functional capacity is unknown in these patients. Therefore, our objective was to determine exercise capacity in adult patients diagnosed with an unrepaired atrial septal defect compared to healthy controls. DESIGN: A cross-sectional study. PATIENTS: Adult patients with small, unrepaired atrial septal defects, aged 18-65, diagnosed between 1953 and 2011. INTERVENTIONS: Cardiopulmonary exercise test was performed using an incremental bicycle test and gas exchange was measured using breath-by-breath technique. OUTCOME MEASURES: Primary outcome was peak oxygen uptake, secondary outcome was maximal workload and ventilatory anaerobic threshold. RESULTS: We included 32 patients previously diagnosed with a small, unrepaired atrial septal defect and 16 healthy, age- and gender-matched controls (age 36.3 ± 13 years). Patients were divided into two groups based on whether the atrial septal defect was open (age 36.3 ± 11 years) or spontaneously closed (age 36.8 ± 14 years) since time of diagnosis. No differences in demographic characteristics or weekly exercise levels were found. Both patient groups reached lower peak oxygen uptake (open: 31.7 ± 11 mL/kg/min; spontaneously closed: 29.7 ± 6.9 mL/kg/min) compared with controls (42.6 ± 6.1 mL/kg/min; P = .0001). Workload (open: 2.6 ± 1.0 watt/kg; spontaneously closed: 2.5 ± 0.6 watt/kg) and aerobic capacity (open: 21.4 ± 8.7 mL/kg/min; spontaneously closed: 22.5 ± 6.5 mL/kg/min) was also poorer in patients compared to controls (workload: 3.5 ± 0.5 watt/kg; P = .0006, aerobic capacity: 31.3 ± 6.8 mL/kg/min; P = .0007). CONCLUSION: Adult patients with a diagnosis of small, unrepaired atrial septal defect have significantly impaired exercise capacity when compared to healthy controls. The impairment was present even if, by the time of assessment, the defect had closed spontaneously. The pathophysiological mechanisms behind the impaired exercise capacity demonstrated in these patients remain unexplained and will be a target for future work.

Recovery of atrial transport function after a maze procedure for atrial fibrillation in conversion of a failing Fontan circulation.

Surgical ablation for atrial arrhythmias at conversion of atriopulmonary or ventriculopulmonary Fontan to a total cavopulmonary connection is feasible with recovery of both sinus rhythm and atrial transport function. Recovery of the patient's physical condition may take up to a year. (Neth Heart J 2008;16:170-2.).