Eisenmenger

This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various cardiovascular complexities associated with this syndrome. While current treatments focus on managing symptoms, ongoing genetic research offers hope for more favorable outcomes. Further studies are crucial to gauge the effectiveness of these treatments for PWS patients. We detail a patient with a complex medical history of PWS, further complicated by congenital heart disease with Eisenmenger's syndrome, diabetes mellitus, pulmonary hypertension, venous insufficiency, hypothyroidism, and hyperlipidemia...

BACKGROUND: Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However, the risk stratification for persistent pulmonary hypertension (PH) in this population remains uncertain. METHODS AND RESULTS: We retrospectively enrolled 103 patients with pretricuspid shunts with high total pulmonary resistance >4.5 Wood units (estimated pulmonary vascular resistance ≥3 Wood units)...

No abstract text is available yet for this article.

In this research, we present a study on the atomization threshold (ATh) of sessile droplets, analyzing its relationship with the excitation frequency fexc (55-48 kHz), droplet volume Vdrop (1-100 μl), and droplet viscosity μ (1-6 mPa⋅s). The investigation focused on the atomization thresholds using ultrasonic excitation of distilled water droplets and water- polyethylene glycol (PEG)-8000 mixtures deposited on vibrating surfaces. The obtained results are compared with previously reported theoretical models...

Eisenmenger syndrome (ES) is a complex, multisystemic, and rare clinical entity, given that currently, most congenital heart diseases can be corrected in childhood. The high anesthetic risk in these patients poses a challenge for anesthesiology. There are few cases described in the literature of anesthetic approaches using ketamine and dexmedetomidine in ES cases, particularly under Monitored Anesthesia Care (MAC). We describe the clinical case of a 40-year-old patient with trisomy 21, intellectual disability, and ES secondary to a single atrioventricular (AV) valve, scheduled for cranial magnetic resonance imaging (MRI) under sedation due to a suspected space-occupying lesion...

Heart failure (HF) in adult congenital heart disease (ACHD) is an increasingly common problem facing ACHD and advanced heart disease and transplant providers. Patients are highly nuanced, and therapies are poorly studied. Standard HF medications are often used in patients who are not targets of large clinical trials. HF management in this data-free zone requires focused, comprehensive team-based care and close follow-up and communication with patients.

INTRODUCTION: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate (eGFR), thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease. There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance (PVR), with a reversed or bidirectional shunt at aorto-pulmonary, ventricular, or atrial level...

BACKGROUND: Patients with Eisenmenger syndrome (ES) requiring noncardiac surgery are at a significantly high risk of perioperative morbidity and mortality. However, perioperative management of patients with ES requiring laparoscopic surgery remains unclear. CASE PRESENTATION: We describe the case of a patient with ES who underwent laparoscopic hysterectomy under general anesthesia with a peripheral nerve block. The objectives of the perioperative management included the following: (1) maintaining systemic vascular resistance and cardiac output through euvolemia, facilitated by the infusion of noradrenaline, and (2) preventing a reduction in oxygen-carrying capacity and factors that elevate pulmonary vascular resistance, such as pain, hypoxia, and decreased body temperature...

Background: Although pulmonary hypertension (PH) and Eisenmenger's syndrome (ES) are common complications in adult congenital heart disease (ACHD), the frequency of diagnostic tests and the incidence of PH/ES in patients with ACHD in Japanese clinical practice are unclear. Therefore, we sought to clarify the frequency of diagnostic tests and incidence of PH/ES in patients with ACHD using the Medical Data Vision (MDV) database, the largest anonymized database of diagnosis procedure combination hospitals in Japan...

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, typically requiring early treatment in infancy. Untreated TOF is associated with poor survival, with most uncorrected patients not surviving beyond the third decade. Here, we present a unique case of an 86-year-old female with uncorrected TOF who underwent a transcatheter aortic valve replacement (TAVR) procedure due to severe aortic stenosis (AS). The patient's TOF was identified during infancy, and she was categorized as an acyanotic "pink baby...

Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently...

BACKGROUND: Cellular senescence has been regarded as a therapeutic target for ageing and age-related diseases. Several senotherapeutic agents have been proposed, including compounds derived from natural products which hold the translational potential to promote healthy ageing. This systematic review examined the association of dietary ingredients with cellular senescence in animals and humans, with an intent to identify dietary ingredients with senotherapeutic potential. METHODS: This systematic review was registered at PROSPERO International prospective register of systematic reviews (Reg #: CRD42022338885)...

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly improved long-term outcomes, some individuals, particularly those from low socioeconomic backgrounds who lack access to medical care, may suffer from complications such as pulmonary hypertension (pHTN) and heart failure. We present a case report of a young female with unrepaired TOF who presented with acute-on-chronic hypoxic respiratory failure and heart failure, highlighting the complex nature and challenges associated with this condition...

AIMS: A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA). METHODS AND RESULTS: Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed...

Neurovascular 4D-Flow MRI enables non-invasive evaluation of cerebral hemodynamics including measures of cerebral blood flow (CBF), vessel pulsatility index (PI), and cerebral pulse wave velocity (PWV). 4D-Flow measures have been linked to various neurovascular disorders including small vessel disease and Alzheimer's disease; however, physiological and technical sources of variability are not well established. Here, we characterized sources of diurnal physiological and technical variability in cerebral hemodynamics using 4D-Flow in a retrospective study of cognitively unimpaired older adults (N = 750) and a prospective study of younger adults (N = 10)...

BACKGROUND: Eisenmenger syndrome (ES) is characterized by severe and irreversible pulmonary hypertension stemming from an uncorrected intracardiac shunt of significant size. The imbalance between systemic and pulmonary artery pressures predisposes patients with ES to the risk of cardiac arrest. Remimazolam has caused less circulatory depression, which may be advantageous for ES. However, no studies reported the use of remimazolam in perioperative ES management. CASE PRESENTATION: A 45-year-old female patient with ES derived from a ventricular septal defect was scheduled to undergo bilateral adnexectomy for an ovarian tumor...

PURPOSE: Kidney transplantation (KT) is the most frequently performed organ transplantation. In Germany, KT is performed in urology and surgery departments with unknown consequences of this parallel structure. The aim of the study was to compare the development and outcome of KT in urology and surgery departments. METHODS: On an institutional level, we analyzed the annual caseload from 2006 to 2021 with the reimbursement. INFO tool based on hospitals' quality reports (Reimbursement Institute, Hürth, Germany)...

INTRODUCTION: Testicular tumors are the most common malignancies in young adults and their incidence is growing. The implantation of a testicular prosthesis, for example, during orchiectomy is a standard procedure but its frequency in Germany is unknown. This study aims to analyze trends of testicular prosthesis implantation in recent years in Germany. MATERIAL AND METHODS: The nationwide German hospital billing database and the German hospital quality reports from 2006 to 2021 were studied...

No abstract text is available yet for this article.

PURPOSE: To analyze recent trends of surgical access routes, length of hospital stay (LOS), and mortality in kidney transplantation (KT) and living donor nephrectomy (LDN) in Germany. MATERIALS AND METHODS: We studied the nationwide German hospital billing database and the German hospital quality reports from 2006 to 2021. RESULTS: There were a total of 35.898 KTs. In total, 9044 (25%) were living donor transplantations, while 26.854 (75%) were transplantations after donation after brain death (DBD)...