Among congenital cyanotic heart diseases (CHDs), situs inversus totalis with transposition of great vessels with a large ventricular septal defect (VSD) has a very low incidence of around 1 in 10,000. Hereby, we present a 16-year-old man with the aforementioned cardiac anomaly with cardiac arrhythmias, septic shock, and a history of road traffic accident-causing osteomyelitis of the left thigh requiring incision and drainage. The patient was admitted to the intensive care unit with a high-grade fever, narrow pulse pressure, and atrial fibrillation. The patient was operated on under general anesthesia with endotracheal intubation after optimization. Invasive monitoring, antiarrhythmics, and vasopressors were required intraoperatively, and surgery progressed uneventfully. Furthermore, the patient had undergone a series of debridements after 8 days, which were performed under regional anesthesia uneventfully. This case report represents a plan of action for perioperative anesthetic management and anticipates the difficulties for CHD patients in the course of surgery and subsequential prudence.

BACKGROUND: Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate. Severe pulmonary arterial hypertension has not been previously reported in patients with PUS1 gene mutation.
METHODS: A 17 year old girl with congenital sideroblastic anemia presented with worsening of breathlessness. Severe pulmonary artery hypertension was documented on investigations. A homozygous variant in exon 3 of gene PUS1,( chromosome 12:g.131932301 C > T c.430 C > T) was found on sanger sequencing.
CONCLUSIONS: We document severe pulmonary arterial hypertension in a patient of congenital sideroblastic anemia from PUS1 gene. We hypothesis that cross talk with TGFb pathways might occur in PUS1 mutation, and that might cause severe PAH. This observation might have therapeutic implications.

Atrial septal defects (ASDs), comprising a significant portion of congenital cardiac anomalies, encompass a rarer and more diagnostically challenging subset known as sinus venosus ASDs (SVASDs). ASDs are more prevalent in females, and the prognosis for patients under 40 years of age is generally favorable with advancements in surgical and transcatheter interventions. However, undiagnosed ASDs in adults above 40 years old, especially females, often lead to severe complications, including pulmonary hypertension, atrial fibrillation, Eisenmenger syndrome, and a mortality rate exceeding 50%. Our detailed case study focuses on an obese 42-year-old Hispanic migrant female with chronic respiratory failure misattributed to pulmonary hypertension, resulting in the progression of complications from undiagnosed SVASD. Further investigation using contrast-enhanced transesophageal echocardiography (TEE) elucidated the correct diagnosis four years after her initial presentation. This report explores the potential factors contributing to the patient\'s delayed diagnosis and development of advanced cardiac complications of pulmonary hypertension leading to Eisenmenger syndrome that precluded her from procedural intervention. Furthermore, this report pioneers the first thorough review of case reports in adults newly diagnosed with SVASD, revealing sex-based differences in complications.

OBJECTIVE: Vasopressin has systemic vasoconstrictive yet pulmonary vasodilatory effects, making it an ideal agent for hypotension management in infants with congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension. The side effects of vasopressin in this population, such as hyponatremia, are understudied. This study aims to characterize the effect of vasopressin on sodium concentrations in infants with and without CDH.
METHODS: This was a retrospective review of patients who received vasopressin while admitted to a level IV neonatal intensive care unit. The primary outcome was the incidence of hyponatremia (blood sodium <135 mmol/L) during vasopressin therapy. Secondary outcomes included time to hyponatremia, dose and duration of vasopressin, incidence of severe hyponatremia (blood sodium <125 mmol/L), and hypertonic saline use. Both blood serum and blood gas sample sodium concentrations were used to compare CDH vs non-CDH patients.
RESULTS: The average difference between baseline and lowest blood sodium was significant for both CDH and non-CDH patients for all samples (p < 0.001). There was no significant difference in the primary outcome, nor in the secondary outcomes of time to hyponatremia or duration of vasopressin infusion. The average dose of vasopressin was higher in the CDH vs non-CDH group (p = 0.018). The incidences of severe hyponatremia and hypertonic saline use were greater in the CDH vs non-CDH group for patients who had blood serum sodium samples collected (p = 0.049 and p = 0.033, respectively).
CONCLUSIONS: This study showed that severe hyponatremia occurred more frequently in CDH vs non-CDH patients. Extreme caution is necessary when managing total body sodium in patients with CDH.

UNASSIGNED: To evaluate the effectiveness of the surgical approach in patients with congenital heart disease and pulmonary hypertension (PH).
UNASSIGNED: This was a retrospective clinical review of patients with congenital heart disease and PH who underwent pulmonary artery banding (PAB) at our institution between January 2013 and January 2023.
UNASSIGNED: We identified 219 patients (53.4% males) with a median age of 7 (4.0-15.0) months and a median weight of 6.8 (5.2-9.0) kg at the time of PAB. The median hospital stay was 7.0 (5.0-10.0) days. The in-hospital mortality rate was 4.6%. The median follow-up was 33.0 (17.0-61.0) months. Survival rates were 96.9 ± 2.5% at 60 months and 92.1 ± 6.9% at 120 months post-PAB. 43.8% of patients had a de-banding procedure, and 147 (79.0%) patients received a second-stage procedure (34.7% univentricular, 65.3% biventricular). The mortality rate between stages was 4.3%. 21 (9.6%) patients reached a third-stage procedure. The overall mortality rate was 9.1%.
UNASSIGNED: PAB is an acceptable strategy for patients with congenital heart disease complicated with PH. The results and outcomes of subsequent univentricular or biventricular procedures are generally good.

Pulmonary hypertension (PH) is a chronic progressive disease with high mortality. There has been more and more research focusing on the role of AMPK in PH. AMPK consists of three subunits-α, β, and γ. The crosstalk among these subunits ultimately leads to a delicate balance to affect PH, which results in conflicting conclusions about the role of AMPK in PH. It is still unclear how these subunits interfere with each other and achieve balance to improve or deteriorate PH. Several signaling pathways are related to AMPK in the treatment of PH, including AMPK/eNOS/NO pathway, Nox4/mTORC2/AMPK pathway, AMPK/BMP/Smad pathway, and SIRT3-AMPK pathway. Among these pathways, the role and mechanism of AMPK/eNOS/NO and Nox4/mTORC2/AMPK pathways are clearer than others, while the SIRT3-AMPK pathway remains still unclear in the treatment of PH. There are drugs targeting AMPK to improve PH, such as metformin (MET), MET combination, and rhodiola extract. In addition, several novel factors target AMPK for improving PH, such as ADAMTS8, TUFM, and Salt-inducible kinases. However, more researches are needed to explore the specific AMPK signaling pathways involved in these novel factors in the future. In conclusion, AMPK plays an important role in PH.

Pulmonary hypertension (PH) is an intricate medical issue resulting from increased pressure in the pulmonary artery (PA). The current gold standard for diagnosis involves an invasive procedure known as right heart catheterization. Nevertheless, cardiac magnetic resonance imaging (cMRI) offers a non-invasive and valuable alternative for evaluating the function, structure, and blood flow through the pulmonary artery (PA) in both the left ventricle (LV) and right ventricle (RV). Additionally, cMRI can be a good tool for predicting mortality by assessing various hemodynamic parameters. We perceive that cMRI may be an underutilized tool in the evaluation of PH. More discussions might be needed to highlight its utility in patients with PH. This article aims to discuss the potential role of cMRI in evaluating PH based on the review of recent literature.

UNASSIGNED: The CardioMEMS is an implantable device for hemodynamic monitoring approved by the US Food and Drug Administration for adult patients with heart failure. It has been used in the adult population without structural heart disease and with congenital heart diseases, but we do not have data in the pediatric population.
UNASSIGNED: We report the initial single-center experience of the CardioMEMS implantation in children. Feasibility of device implantation, procedural outcomes, and clinical utility in the pediatric population were evaluated.
UNASSIGNED: The CardioMEMS device was implanted without technical complications in 8 pediatric patients (mean age 7 years and mean weight 27.9 kg) with pulmonary hypertension (6/8, 75%) and heart failure (2/8, 25%). The device was delivered via femoral access in 7 (85%) patients and implanted in the left pulmonary artery in 7 (85%). The noninvasive recording of pulmonary pressures in patients with pulmonary hypertension allowed the monitoring of the evolution of mean pulmonary artery pressure, intensifying vasodilator treatment, and avoiding control cardiac catheterizations. In patients with heart failure, pulmonary hemodynamic monitoring guided the decongestive treatment prior to heart transplantation.
UNASSIGNED: The implantation of CardioMEMS in the pediatric population is a feasible procedure that allows the noninvasive hemodynamic monitoring of patients with heart failure and pulmonary hypertension. Its implementation in selected patients aids in outpatient follow-up and therapeutic management of patients with complex cardiac conditions, avoiding invasive procedures that require hospitalization. Further large-scale studies in the pediatric population are recommended.

Orthotopic liver transplantation (OLT) is a viable treatment option for end-stage liver disease. Significant perioperative stress is placed on the cardiovascular system because of hemodynamic changes and the length of the operation. Diagnosis and treatment of cardiovascular disease before OLT are imperative to ensure favorable outcomes. Considerable variability exists among practitioners caring for these patients. Institutions tailor their protocols on the basis of local and historical practices, the preferences of the cardiologists, and the OLT team, and algorithms are not often revised or updated on the basis of the available evidence. In collaboration with cardiology and hepatology experts from leading OLT centers, we sought to examine the diagnostic cardiovascular workup of OLT candidates, including a review of the available literature on the diagnostic modalities used to screen cardiovascular disease before OLT. We advocate an emphasis on noninvasive methods to assess cardiovascular risk with reserved use of invasive risk stratification in select patients.

暂无摘要。