Eisenmenger Syndrome Research Articles

Adult congenital heart disease in women: demographics and outcomes in mexico. gender differences?

Abstract Background Due to advances in cardiovascular medicine in recent decades, adult congenital heart disease (ACHD) is becoming a growing field in cardiology. Differences in clinical presentation, treatment, and outcomes of ACHD between men and women have not been well studied in Latin America and Mexico. Purpose To characterize gender differences in clinical presentation, treatment, and mortality of ACHD in Mexico. Methods From January 2006 to December 2020, 3503 patients with ACHD were studied at a cardiology national reference center in Mexico. SPSS V. 23 software was used for statistical analysis. Results Women comprised 56% of the study population, in which a higher percentage of pulmonary hypertension and Eisenmenger syndrome was observed, (10.9% men vs. 17.7% women; 1.5% men vs. 3.3% women, p <0.01). Women had a higher score in AHA´s physiological classification (State C, 27.9% men vs. 35.3% women, p <0.01). Men had a higher rate of severity/anatomic complexity (13.3% men vs. 11.7% women, p <0.01), a higher rate of LVEF <40%, (8.0% men vs. 23.8% women, p <0.01), and underwent total repair surgery more often (61.3% men vs. 57% women, p <0.01). No statistical difference was observed in functional class, presence of arrhythmias, or mortality (patient´s current status). Figure 1. Conclusions We found gender differences in ACHD in Mexico: women were more likely to have pulmonary hypertension, which may reflect the already known gender predilection of PH; additionally, women had a higher AHA's physiological score; despite this, women underwent complete surgery less frequently. Although men had higher anatomic complexity and a lower LVEF, there was no statistical difference in functional class or mortality.Figure 1

Evaluating the safety and long-term efficacy of triple vasodilator therapy for pulmonary arterial hypertension associated with congenital heart disease

Abstract Background Pulmonary arterial hypertension (PAH) affects 5-10% of congenital heart disease (CHD) patients, but there is limited information on the safety and efficacy of triple combination therapy for PAH-CHD. The aim of this study was to assess the long-term effects of triple therapy (TT) with prostacyclins on PAH-CHD patients. Methods A retrospective, longitudinal, cohort study of patients with PAH-CHD under active follow-up in our center. All patients were receiving baseline dual therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walk test distance (6MWTd), and NT-ProBNP were recorded before initiating TT and during annual follow-ups during 2 years. Results 60 patients were included (median age 41, 31 women 61%). 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH, and 1 had a significant L-R shunt. TT was initiated based on the clinical situation, existence of shunt and ease of administration (epoprostenol 20%, treprostinil 41%, selexipag 32%, iloprost 7%). A significant improvement in the 6MWTd was observed one year after the initiation of TT (p<0.05), which was maintained at two-year follow-up, with an average improvement of 44 metres. An improvement of FC was observed in 79% of patients at one year, maintained at 2 years in 76%. A NT- proBNP levels decreased from the start, and reached significancy two years after the initiation of TT, with an average decrease of 199 ng/l (Figure 1). Twenty patients (33.3%) experienced side effects after drug initiation. Most were mild and well-controlled with symptomatic treatment. Only 3 (5%) had to change the administration route (Figure 2). By subgroups, patients without Eisenmenger and with pre-tricuspid defects had a more marked benefit. Conclusions TT has been shown to be safe and effective in patients with PAH-CHD, improving FC, 6MWTd, and NT-proBNP levels, particularly in pre-tricuspid defects and non-Eisenmenger PAH-CHD.Changes after initiation of TTType of treatment and side-effects

Efficacy of intravenous iron therapy compared to usual treatment in iron deficient adult cyanotic congenital heart disease patients for improvement in clinical outcomes at three months

Abstract Background Iron deficiency is frequent in CCHD patients and is associated with poorer outcomes. Patients with iron deficiency anaemia are treated with oral iron which results in improved clinical outcomes. The use of intravenous iron in CCHD patients is not well studied. Purpose We aimed to compare effectiveness of intravenous iron with usual care (i.e., no therapy for CCHD patients with iron deficiency without anemia, and oral iron therapy for CCHD patients with iron deficiency anemia) on 6-minute walk distance(6MWD) after 3 months of treatment, improvement in arterial Haemoglobin saturation, Quality of life, WHO functional class and safety of intravenous Iron. Methods We enrolled 27 patients of which 13 were randomized to IV iron arm and 14 to usual treatment arm. The mean age (±SD) of enrolled patient was 23.3 (±5.55) years. 24 out of 27 patients (88.9%) were Eisenmenger Syndrome patients, while the other three patients were TOF with pulmonary atresia, DORV with pulmonary atresia and post-Fontan. 6MWD was performed to access functional capacity and WHOQOL-BREF questionnaire was used for assessment of quality of life. All the clinical findings, data and test recordings were repeated after three months. Results The mean (±SD) haemoglobin(Hb) of patients included in the study was 16.7 (±2.0) g/dl. The Hb levels improved by 0.91 (±0.85) g/dl in patients who received any form of iron therapy (IV or oral iron). Hb improvement was not significantly different between those who received IV or oral iron [IV iron: 1.15 (±0.88) g/dl, Oral iron: 0.59 (±0.79) g/dl; p= 0.12]. The baseline 6MWD was not different in both the arms (p= 0.639). The mean (±SD) 6MW distance covered in IV iron arm and usual treatment arm was 401 (±35.2) m and 408.6 (±46.5) m respectively at baseline, which improved to 425 (±28.7) m and 421.5 (±43.2) m respectively at 3 months follow up. The change in 6MWD in IV iron arm and usual treatment arm was 24 (±22.3) m and 13.1 (±16.4) m respectively. The difference of change in 6MWD between the two arms was non-significant (p= 0.157). There was a significant improvement in WHOQOL-BREF Score Domains 2,3 and 4 and there was a trend towards improvement on Domain 1 score in IV iron arm compared to usual treatment arm. WHO functional class was similar at baseline in both arms (p= 0.534). The change in WHO functional class at follow up was similar in both arms (p= 0.082) Conclusion This study demonstrated no improvement in clinical outcomes of 6MW distance, arterial hemoglobin saturation and WHO functional class. However, there was significant improvement in quality of life and iv iron was found safe and equally efficacious as oral iron for correcting iron deficiency. Further studies for determining ideal hemoglobin levels and dose of iron in CCHD patients are pertinent.

SARS-CoV-2 pneumonia and Eisenmenger’s Syndrome: doubling the challenge

Eisenmenger’s syndrome (ES) is the most severe phenotype of pulmonary arterial hypertension (PAH) secondary to congenital heart disease. In these cases, a significant systemic-to-pulmonary (left-to-right) shunting triggers the development of pulmonary vascular disease (PVD) and pulmonary hypertension. In cases of acute hypoxemic respiratory failure in patients with ES, high flow nasal cannula (HFNC) oxygen therapy should be considered as a first-line approach in order to avoid pulmonary complications and right ventricular overload related to positive pressure ventilation. Here, we report a case of HFNC use in a patient with COVID-19 infection and ES.

Invasive Hemodynamic and Vasoreactivity Testing with Inhaled Iloprost in Children with Pulmonary Arterial Hypertension Associated with Congenital Heart Defects

Objective: Invasive hemodynamic evaluation and acute vasoreactivity testing are recommended in the diagnosis of pulmonary arterial hypertension (PAH), but their clinical role in children with PAH associated with congenital heart defects (CHD) is unclear. This study aims to investigate acute hemodynamic responses to inhaled iloprost, and its role in prognosis in children with PAH-CHD. Methods: A retrospective analysis was conducted on 83 pediatric patients with PAH-CHD in whom invasive hemodynamics were evaluated before and after a single inhaled dose of iloprost at a single center between 2010 and 2022. Details of the CHD corrective operation, medical treatment, and outcome for each patient were obtained via medical records or telephone contact. A composite endpoint of all-cause death, admission for worsening heart failure during follow-up, and event-free survival was defined, and risk factors associated with this composite endpoint were analyzed. Results: The patient age was (11.3 ± 4.6) years, and 60 (72.3%) were female. Fifty-nine (71.1%) patients were diagnosed with Eisenmenger syndrome. After iloprost inhalation, mean pulmonary artery pressure decreased from (78.2 ± 11.5) to (72.3 ± 13.2) mmHg (P < 0.001), and pulmonary vascular resistance index (PVRI) decreased from (18.0 ± 7.9) to (14.5 ± 8.1) WU·m2 (P < 0.001). A total of 38 (45.8%) patients had a positive response, defined as a PVRI decrease >25% with stable systemic pressure. Thirty-seven (44.6%) patients underwent a corrective CHD operation at a median of 24 d after hemodynamic evaluation. Nine patients died, and 15 met the composite endpoint during a follow-up period of 5.4 (3.8, 8.8) years. Five-year event-free survival estimates were 96.7% (95% confidence interval: 90.3%–100%) in patients with a positive response, and 82.8% (95% confidence interval: 71.7%–94.5%) in patients with a non-positive response (log-rank P = 0.012). A positive PVRI response and higher pulmonary arterial oxygen saturation after iloprost inhalation, lower baseline brain natriuretic peptide, and PAH-targeted therapy at follow-up were significantly associated with a favorable clinical outcome. A positive acute vasoreactivity testing response and PAH-targeted therapy at follow-up were independent predictors of outcome in multivariate Cox analysis. Conclusions: Acute inhalation of iloprost can lead to a significant decrease in hemodynamic parameters. Responsiveness to inhaled iloprost is associated with better outcomes and can be a valuable predictor of outcomes.

Eisenmenger syndrome during pregnancy: A true anesthetic challenge

Eisenmenger syndrome during pregnancy: A true anesthetic challenge

Eisenmenger syndrome arising from aortopulmonary window diagnosed in adulthood: An unusual presentation as ischemic stroke

Aortopulmonary window (AP window) is an uncommon congenital heart condition marked by a connection between the ascending aorta and the pulmonary trunk. Numerous individuals diagnosed with AP window typically manifest symptoms in their early childhood, which may include recurring respiratory tract infections due to a left-to-right shunt, signs of congestive heart failure, and, if not treated in early childhood, the condition can progress to severe pulmonary vascular disease and Eisenmenger syndrome. Without treatment, very few patients survive beyond their second decade of life. In this case, we present a patient who exhibited symptoms of an ischemic stroke while having an undiagnosed large AP window and Eisenmenger syndrome.

Survival and outcomes of isolated neonatal ventricular septal defects: A population-based study from a middle-income country.

Limited data on the survival and outcomes of ventricular septal defect (VSD) in middle-income countries are available. Hence, this study aims to determine the survival and factors associated with mortality among neonatal VSD. This is a retrospective, population based study of neonates with isolated VSD born between 2009 and 2019. Kaplan-Meier analysis was used to estimate the overall survival. Cox regression analysis was used to determine factors associated with mortality. There were 726 patients studied, with 82 (11%) of them having trisomy 21. The median age of diagnosis and follow-up was 5 days (interquartile range [IQR]: 2-10 days) and 2.3 years (IQR: 0.6-4.8 years), respectively. Of 726, 399 (55%) were perimembranous, 218 (30%) muscular, and 109 (15%) outlet VSD. VSD was small in 309 (42%), moderate in 337 (46%), and large in 80 (11%). Of 726 patients, 189 (26%) had congestive heart failure (CHF) and 52 (7.2%) developed pulmonary hypertension (PHT). Interestingly, one-third of CHF and PHT resolved over time during follow-up. Only 1 (0.1%) patient had infective endocarditis, 38 (5.2%) developed aortic regurgitation, and none had Eisenmenger syndrome. Overall, 149 (20%) needed surgery, 399 (55%) spontaneously closed, and 178 (25%) remained small. The mortality rate was 3.9% (28), 16 (57%) preoperatively, and 11 (39%) due to pneumonia. Trisomy 21, PHT, and birth weight <2.5 kg were independent factors for mortality with an adjusted hazard ratio of 6.0 (95% confidence interval [CI]: 2.1-16.9), 3.2 (95% CI: 1.2-8.4), and 3.6 (95% CI: 1.7-7.8), respectively. The overall survival at 1, 5, and 10 years was 96% (95% CI: 95-98), 95% (95% CI: 94-97), and 95% (95% CI: 94-97), respectively. Despite limited pediatric and congenital cardiac services in middle-income countries, the overall survival of neonatal VSD is good, with poor outcomes in small infants, PHT, and trisomy 21.

Outcomes of pregnancy in women with different types of pulmonary hypertension

BackgroundPulmonary hypertension (PH) is considered to increase maternal and fetal risk, and we attempt to explore pregnancy outcomes in women with different types of PH.MethodsWe retrospectively analyzed the clinical data of pregnant women with PH who were admitted to Anzhen Hospital from January 2010 to December 2019, and followed up on these parturients and their offspring.ResultsThree hundred and sixty-six pregnant women with PH were collected, including 265 pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD), 65 PH caused by left heart disease, 12 idiopathic PH, and 24 PH associated with other diseases. Maternal mean age was 28.4 ± 4.4 years and 72.1% were nulliparous. The estimated systolic pulmonary artery pressure was < 50 mmHg in 40.2% of patients, 50–70 mmHg in 23.2%, and > 70 mmHg in 36.6%. In more than 94% of women, a diagnosis of PH was made before pregnancy. During pregnancy, heart failure occurred in 15% of patients. Cesarean section was performed in 90.5% (20.4% emergency). Complications included fetal mortality (0.5%), preterm delivery (40.4%), and low birth weight (37.7%). A total of 20 mothers died (5.5%). The highest mortality rate was found in patients with idiopathic PH (4/12, 33.3%). A total of 12 children died (3.3%), 5 (1.4%) of them after discharge from the hospital, and 7 (1.9%) were in hospital.ConclusionsAlthough most of these women are fertile, PH does increase maternal and fetal risk. Women with idiopathic PH and Eisenmenger syndrome are not recommended to have children.

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report

BackgroundAortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation.Case presentationsA 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful.ConclusionsWe have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient’s background requiring lung transplantation, and in these cases close postoperative follow-up is required.

One-minute sit-to-stand test in patients with pulmonary arterial hypertension associated with congenital heart disease: A single-center prospective study

BackgroundPatients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) have reduced exercise capacity. Recently, the 1-minute sit-to-stand test (1MSTST), which measures the number of repetitions from sitting to standing position in 1 minute, has been proposed as an alternative test to the 6-minute walking test (6MWT). The aim of our study was to assess the safety and results of the 1MSTST in comparison to the 6MWT in patients with PAH-CHD. MethodsConsecutive adult patients with PAH-CHD underwent the 6MWT and the 1MSTST on the same day. The 6-minute walking distance in meters and the number of repetitions on the 1MSTST were measured. Heart rate, peripheral oxygen saturations, Borg dyspnea score, and lower limb fatigue were recorded before and immediately after testing. Correlations between both tests and clinical, laboratory, and imaging parameters were statistically analyzed. ResultsThe study included 40 patients (50% female, mean age 43 ± 15 years), of whom 29 (72%) had Eisenmenger syndrome and 14 (35%) had Down syndrome. The number of 1MSTST repetitions correlated significantly with 6MWT distance (r = 0.807, p = 0.000). There were no adverse events, and the 1MSTST results correlated with the WHO functional class. Heart rate increase and oxygen desaturation after both tests correlated significantly, but less desaturation was observed after 1MSTST. ConclusionsOur study showed that the 1MSTST is a safe and easily applicable test in adult patients with PAH-CHD, including patients with Down syndrome. The results of the 1MSTST correlate significantly with the 6MWT, providing an alternative tool for exercise capacity assessment in patients with PAH-CHD.

Corazón univentricular sin cirugía de Fontan: el tipo de paliación importa

Introduction and objectivesThere is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

Approach to hypoxemia in the emergency department in Eisenmenger syndrome. A case report

Approach to hypoxemia in the emergency department in Eisenmenger syndrome. A case report

Eisenmenger syndrome combined with pulmonary artery dissecting aneurysm.

Journal Article Eisenmenger syndrome combined with pulmonary artery dissecting aneurysm Get access Hongwei Zhang, Hongwei Zhang Department of Radiology, Qilu Hospital of Shandong University Dezhou Hospital (Dezhou People's Hospital), Dongfanghong West Road, Decheng District, Dezhou City, Shandong Province 253000, China https://orcid.org/0000-0002-0646-176X Search for other works by this author on: Oxford Academic PubMed Google Scholar Lina Yu, Lina Yu Department of Radiology, Qilu Hospital of Shandong University Dezhou Hospital (Dezhou People's Hospital), Dongfanghong West Road, Decheng District, Dezhou City, Shandong Province 253000, China https://orcid.org/0000-0002-4921-2480 Search for other works by this author on: Oxford Academic PubMed Google Scholar Zihao Li Zihao Li Department of Radiology, Qilu Hospital of Shandong University Dezhou Hospital (Dezhou People's Hospital), Dongfanghong West Road, Decheng District, Dezhou City, Shandong Province 253000, China Corresponding author: Tel: +8615288897550, Email: 88992008@qq.com https://orcid.org/0000-0002-3715-7787 Search for other works by this author on: Oxford Academic PubMed Google Scholar European Heart Journal, ehad258, https://doi.org/10.1093/eurheartj/ehad258 Published: 04 May 2023

PO-04-144 IMPACT OF AGE ON ATRIAL ARRHYTHMIA OUTCOMES AFTER PERCUTANEOUS ASD CLOSURE

Atrial Septal Defects (ASD) are one of the most common congenital heart defects, and if unrepaired, increase the risk of atrial fibrillation (AF) and atrial flutter (AFL). AF/AFL are linked to an increased risk of stroke and heart failure yet, it is not clear from existing data whether percutaneous ASD closure reduces the overall burden of AF/AFL and whether AF/AFL prevalence is impacted by closure age. To determine the effect of age at ASD closure on the prevalence of late atrial arrhythmias. All patients who underwent percutaneous ASD closure at Cleveland Clinic from January 2010 to July 2021 were included. Patients without right-sided dilatation and Qp:Qs < 1.5, iatrogenic ASDs, and those with Eisenmenger syndrome were excluded. A non-linear logistic mixed model informed a temporal decomposition model used to analyze the association between age at ASD closure and prevalence of AF/AFL, adjusting for gender and presence of pre-closure AF/AFL. A total of 192 patients met inclusion criteria, 121 (63%) female with a median age of 41.5 years (IQR 19-61) at time of ASD closure. Median follow up time was 2.7 years (IQR 0.64-4.7). 170 (89%) patients had a total of 681 records including ECGs and ambulatory rhythm monitors. Pre-procedural AF/AFL was associated with higher likelihood of late (>6 months) AF/AFL. The prevalence of post-ASD closure AF/AFL was composed of two phases: an early peaking phase of 13% at week 1 with a gradual decrease to 3.2% by 6 months. There was a late rising prevalence from 6 months up to 6 years from 3.2% to 4.7% (Figure 1a). Age ≥ 40 years was associated with higher likelihood of early (≤ 6 months) and late (> 6 months) post procedural AF/AFL prevalence (Figure 1b). Post-device closure, only 7 (3.6%) patients required cardioversions at a median time of 2.6 months, and 5 (2.6%) required ablations at a median time of 7.4 months post ASD closure. There were only 10 arrhythmia related ER visits during the follow up period. There is an increased prevalence of AF/AFL immediately post ASD closure that declines by 6 months. Patients older than 40 years of age and those with pre-procedure AF/AFL have ongoing risk for late AF/AFL post ASD closure. Rhythm monitoring before ASD closure may be indicated in age ≥ 40 to identify those at higher risk of developing late AF/AFL. Parametric estimate of prevalence of AF or AFL after percutaneous ASD closureTime1 day1 week2 week1 month6 month1 year3 year5 year6 yearOverall (%)0.8813106.73.23.54.24.64.7Stratified by age (%)<4002.61.80.630.0130.002000≥401.34332154.656.26.97.1 Open table in a new tab

What flow is this!

A 38-year-old female with severe mitral regurgitation, severe aortic regurgitation, and moderate pulmonary hypertension was taken up for double-valve replacement surgery. After induction of anesthesia, transesophageal echocardiography (TEE) probe (Philips X7-2t, iE33 system) was inserted. Comprehensive TEE examination revealed an abnormal continuous color flow signal arising from the ascending aorta to the right pulmonary artery (RPA) [Figures 1 and 2, Video 1]. The direction of the color flow jet was perpendicular to the blood flow in the main PA. There was no history of a prior cardiac intervention except coronary angiography to rule out coronary artery disease. Suspecting a left to right shunt, blood samples from the right ventricle and RPA were taken for oximetry study, which showed a 7.3% step-up of oxygen saturation (73.3% vs. 80.6%). During surgery, excision of the pericardium superiorly showed a vascular structure extending from the left posterolateral aspect of the ascending aorta to the RPA. The vessel was doubly ligated with clips which resulted in cessation of its flow on TEE examination, as well as equalization of saturations in the right ventricle and RPA. The rest of the surgical procedure and postoperative course were uneventful.Figure 1: Upper esophageal ascending aorta short-axis view on transesophageal echocardiography showing ascending aorta (AA) to the right pulmonary artery (RPA) flow (arrow). MPA: Main pulmonary arteryFigure 2: Diagrammatic representation of the ascending aorta (AA) to the right pulmonary artery (RPA) flow (arrow). MPA: Main pulmonary artery, LPA: Left pulmonary artery {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Video Clip 1","caption":"","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_y3nnfi01"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} The most likely diagnosis of this communication is an aortopulmonary window or fistula. Aortopulmonary fistula is generally congenital in origin and may be associated with other congenital cardiac anomalies including patent ductus arteriosus, interrupted aortic arch, coarctation of the aorta, atrial septal defect, or coronary artery anomalies in about half of the patients.[1] Acquired aortopulmonary fistulae have either prior aortotomy, percutaneous endovascular procedure, or acute aortic dissection as the underlying pathology.[2,3] Aortopulmonary fistulae have also been described in chronic diseases of the aortic wall, such as aneurysm, giant cell aortitis, syphilitic or mycotic disease, or endocarditis.[4] After accurate delineation of the anatomy, location, and size of the fistula is achieved, the patient can be treated by either open surgery, hybrid procedure, or endovascular stenting.[5] The risks of pulmonary hypertension, Eisenmenger syndrome, and congestive heart failure remain high in patients with large aortopulmonary fistulae. Patients with very small shunt lesions, such as this patient, may remain asymptomatic, however. The case also highlights the importance of a comprehensive TEE examination to detect abnormal blood flow. DECLARATION OF PATIENT CONSENT The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes.

Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4-12) days, with a maximum dose of 120 (80-160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2-82.3%] and 43.3% (95% CI: 36-52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome. Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.

Impact of current targeted drug therapy on the prognosis of Eisenmenger syndrome: A large-scale retrospective analysis

BackgroundData regarding the prognosis of Eisenmenger syndrome (ES) and effect of targeted drugs are limited. This study aimed to analyze the prognosis and impact of targeted drug therapy on the survival rate of patients with ES in the Chinese population. MethodsThe data of patients with ES referred to our hospital between January 2010 and December 2020 were retrospectively analyzed. Data included baseline demographics, echocardiographic parameters, and clinical diagnoses. All patients were followed up via telephone interviews in February 2022. The primary endpoint was mortality. ResultsOverall, 1,021 patients with ES were included. The 1-, 3-, 5-, 7-, 10-, and 12-year survival rates were 91.6%, 84.2%, 80.7%, 73.8%, 71.4%, and 69.9%, respectively. Patients with atrial septal defects had the best prognosis than those with ventricular septal defects, patent ductus arteriosus, and complex congenital heart disease (CHD) (P < 0.0001). Patients who visited between 2016 and 2020 received increased targeted drug therapy and had a better prognosis than those who visited between 2010 and 2015 (all P < 0.05). Cox regression analysis revealed age, pulmonary arterial systolic pressure, post-tricuspid shunt CHD, targeted drugs, and year of the first hospital visit to be predictors of death (P < 0.05). ConclusionsSurvival rates associated with an increased use of combined targeted drugs significantly improved in patients with ES. However, numerous factors that predict increased mortality remain to be elucidated.

Successful Eisenmenger syndrome-targeted drug therapy in pregnant women: a case series and literature review.

To clarify the real-world outcomes in pregnant women with Eisenmenger syndrome (ES) in the new therapeutic era and provide a literature review. Retrospective case and literature review. Tertiary referral hospital (The Second Xiangya Hospital of Central South University). Thirteen women with ES delivered between 2011 and 2021. Respective study and literature reviews. Maternal and neonatal mortality and morbidity. 12/13 (92%) pregnant women were treated with targeted drugs. 9/13 (69%) of patients had heart failure, but no maternal deaths occurred. 12/13 (92%) of women chose caesarean delivery. One pregnant woman gave birth at 37+1 weeks, and the remaining 12 (92%) patients had preterm birth. 10/13 (77%) women gave birth to live infants, of which 9/10 (90%) were low birthweight infants with a mean birthweight of 1575 g. The infant mortality rate was 1/10 (10%). Cardiac functional class improved during pregnancy, probably due to therapy; 11/13 (85%) of the pregnant women were in cardiac functional level III/IV at admission and 12 (92%) were in cardiac functional class II/III at discharge. Our literature review identified 72 cases of pregnancy with ES from 11 studies, which were characterised by a low rate of targeted drug use (28%) and a high maternal mortality rate of 24% in the perinatal period. Our case series and literature review suggest that targeted drugs may be key to improving maternal mortality in ES.

Univentricular hearts not undergoing Fontan: the type of palliation matters

Introduction and objectivesThere is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.