-
Cureus Feb 2024Eisenmenger syndrome (ES) is a complex, multisystemic, and rare clinical entity, given that currently, most congenital heart diseases can be corrected in childhood. The...
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. Sedation was performed under MAC with dexmedetomidine and ketamine. The procedure proceeded without complications. The anesthetic approach in ES patients, given the clinical complexity, requires planning by a multidisciplinary team and should be tailored to the procedure and its duration.
PubMed: 38500910
DOI: 10.7759/cureus.54285 -
International Heart Journal 2015Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart defects. ES greatly affects functional...
Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart defects. ES greatly affects functional capacity, quality of life, and survival of patients. Clinical management of patients with ES has been dramatically changed and developed in a new therapeutic era of PAH, approximately a century after the first ES case described by Dr. Eisenmenger. Targeted drugs for PAH improve functional capacity and quality of life of patients with ES. In addition, these drugs may also provide a benefit for patients' survival. To achieve further development in medical treatment of patients with ES, they will need to be individualized according to functional classes, underlying cardiac defects, and underlying systemic diseases.
Topics: Adult; Algorithms; Eisenmenger Complex; Humans
PubMed: 25787797
DOI: 10.1536/ihj.14-347 -
Journal of Cardiology Sep 2014Citrulline is an amino acid which is produced by the urea cycle and also a precursor for NO, that is, a vasodilator for normal function of pulmonary vasculature.... (Clinical Trial)
Clinical Trial
BACKGROUND AND PURPOSE
Citrulline is an amino acid which is produced by the urea cycle and also a precursor for NO, that is, a vasodilator for normal function of pulmonary vasculature. Thereby, enhancing l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and those with congenital heart disease identified as Eisenmenger Syndrome results in reduction of pulmonary hypertension.
METHODS AND SUBJECTS
In this clinical trial before and after study, we assigned 25 patients with arterial pulmonary hypertension (idiopathic or Eisenmenger Syndrome) to receive l-citrulline malate 1g three times daily for two weeks. The primary measurement was the change in exercise capacity, as considered as a result of the total distance walked in six minutes, from baseline to week 2. We also assessed mean pulmonary artery pressure, the change in the quality of life, and the change in pro-brain natriuretic peptide (BNP) level. The study was not powered to evaluate mortality.
RESULTS
The mean walking distance in six minutes was significantly increased by about 44m (p=0.005) after receiving l-citrulline malate. Mean pulmonary artery pressure significantly reduced from 83.34mmHg before receiving l-citrulline malate to 79.1mmHg after that (p=0.01). All dimensions of the quality of life had statistical differences after receiving l-citrulline malate except limit due to physical health, limit due to emotional health and social functioning (p>0.05). Finally, pro-BNP difference was not statistically significant (p=0.9).
CONCLUSION
l-Citrulline malate improves the distance walk in six minutes and also the quality of life of patients with idiopathic arterial pulmonary hypertension and Eisenmenger Syndrome and also reduced mean arterial pulmonary hypertension.
Topics: Administration, Oral; Adult; Arterial Pressure; Citrulline; Eisenmenger Complex; Familial Primary Pulmonary Hypertension; Female; Humans; Malates; Male; Natriuretic Peptide, Brain; Pulmonary Artery; Quality of Life; Treatment Outcome; Walking; Young Adult
PubMed: 24525046
DOI: 10.1016/j.jjcc.2014.01.003 -
Kidney & Blood Pressure Research 2024Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may... (Review)
Review
INTRODUCTION
Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). 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, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES.
CASE PRESENTATION
A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength.
CONCLUSION
Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.
Topics: Humans; Female; Adult; Eisenmenger Complex; Kidney Diseases; Cyanosis; Polycythemia
PubMed: 38447536
DOI: 10.1159/000538100 -
Heart (British Cardiac Society) Jun 2001
Review
Topics: Aortic Coarctation; Cardiomyopathy, Dilated; Cyanosis; Eisenmenger Complex; Female; Heart Defects, Congenital; Humans; Marfan Syndrome; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Pulmonary Valve Stenosis; Rheumatic Heart Disease; Risk Assessment
PubMed: 11359761
DOI: 10.1136/heart.85.6.710 -
BMC Cardiovascular Disorders Dec 2020Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of...
BACKGROUND
Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether PDA in patients with ES can be treated with transcatheter closure (TCC).
METHODS
Between August 2014 and July 2016, four of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs > 1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC and pulmonary vasodilator therapy. PAH-targeted drugs were prescribed before and after occlusion for all. Trial occlusion was performed before permanent closure.
RESULTS
The first TCC failed after initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During a mean follow-up of 48 ± 14.70 months, there were a further decrease of PASPs in two patients, the other two showed improved pulmonary vascular resistance, WHO functional class and six-minute walking distance despite deteriorated PASP.
CONCLUSION
Some selected PDA-ES patients might benefit from TCC and combined PAH-targeted drugs play a crucial role.
Topics: Adult; Antihypertensive Agents; Arterial Pressure; Cardiac Catheterization; Combined Modality Therapy; Drug Therapy, Combination; Ductus Arteriosus, Patent; Eisenmenger Complex; Female; Humans; Male; Pulmonary Artery; Recovery of Function; Retrospective Studies; Time Factors; Treatment Outcome; Vasodilator Agents; Young Adult
PubMed: 33261574
DOI: 10.1186/s12872-020-01795-5 -
Current Cardiology Reviews Nov 2010Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension and arises on the basis of congenital heart disease with a systemic-to-pulmonary shunt....
Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension and arises on the basis of congenital heart disease with a systemic-to-pulmonary shunt. Due to the chronic slow progressive hypoxemia with central cyanosis, adult patients with the Eisenmenger syndrome suffer from a complex and multisystemic disorder including coagulation disorders (bleeding complications and paradoxical embolisms), renal dysfunction, hypertrophic osteoarthropathy, heart failure, reduced quality of life and premature death.For a long time, therapy has been limited to symptomatic options or lung or combined heart-lung transplantation. As new selective pulmonary vasodilators have become available and proven to be beneficial in various forms of pulmonary arterial hypertension, this targeted medical treatment has been expected to show promising effects with a delay of deterioration also in Eisenmenger patients. Unfortunately, data in Eisenmenger patients suffer from small patient numbers and a lack of randomized controlled studies.To optimize the quality of life and the outcome, referral of Eisenmenger patients to spezialized centers is required. In such centers, specific interdisciplinary management strategies of physicians specialized on congenital heart diseases and PAH should be warranted. This medical update emphasizes the current diagnostic and therapeutic options for Eisenmenger patients with particularly focussing on the medical treatment and corresponding study results.
PubMed: 22043212
DOI: 10.2174/157340310793566163 -
Journal of the American College of... Dec 2003We sought to determine the prevalence of pulmonary artery thrombosis in patients with Eisenmenger syndrome and to identify individuals at highest risk. (Comparative Study)
Comparative Study
OBJECTIVES
We sought to determine the prevalence of pulmonary artery thrombosis in patients with Eisenmenger syndrome and to identify individuals at highest risk.
BACKGROUND
Eisenmenger syndrome is associated with pulmonary arterial thrombus formation. Both the prevalence and the determinants of pulmonary arterial thrombosis are unknown.
METHODS
This is a review of patients with Eisenmenger syndrome seen at the Toronto Congenital Cardiac Centre for Adults, Canada. Patients underwent a contrast-enhanced computed tomographic (CT) scan of the thorax.
RESULTS
Forty-nine consecutive patients with Eisenmenger syndrome were seen in our hospital. Fifteen patients did not undergo CT angiograms; therefore, 34 patients (mean age 42 +/- 10 years) were included in the study. Responsible shunts included ventricular septal defect (65%), atrial septal defect (15%), patent ductus arteriosus (9%), and other (11%). The prevalence of proximal pulmonary artery thrombus was 21% (7/34) of patients. Evidence of more distal vessel thrombosis was observed in 43% (3/7) of the patients who had visible thrombus in the proximal pulmonary arteries. Patients with thrombus were more likely to be female (86% vs. 37%, p = 0.04) and to have lower oxygen saturations (72% +/- 9% vs. 85% +/- 6%, p = 0.01). Differences in functional status did not identify patients at highest risk for thrombosis.
CONCLUSIONS
Patients with Eisenmenger syndrome have a substantial risk of pulmonary artery thrombus formation. Women and patients with lower oxygen saturations are at the highest risk of developing thrombosis. In the context of an increased bleeding tendency in these patients, the role of anticoagulation treatment needs to be determined.
Topics: Adult; Ductus Arteriosus, Patent; Eisenmenger Complex; Female; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Humans; Lung Diseases; Male; Oxygen; Prevalence; Pulmonary Artery; Thrombosis; Tomography, X-Ray Computed
PubMed: 14662263
DOI: 10.1016/j.jacc.2003.07.022 -
European Heart Journal May 2016We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the...
AIMS
We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the effect of treatment on outcome in the community.
METHODS AND RESULTS
Patients with ES were systematically identified from the German National Register for Congenital Heart Defects. Data on underlying diagnosis, medical therapy, and survival were collected. The impact of DTT on survival was assessed using time-dependant Cox analysis. Overall, 153 ES patients were included (mean age 34.0 ± 13.3 years, 46% females). Of these, 88 (57.5%) were treated with at least one DTT (76.1% Bosentan, 20.5% Sildenafil) while 17.6% were on dual DTT. In addition, 24.8% of patients received digoxin, 10.5% angiotensin-converting enzyme-inhibitors/angiotensin receptor blockers, and 17.6% β-blockers. Moreover, 17.6% of patients were treated with oral anticoagulants, while 23.5% of patients received Aspirin. The survival rate at 1, 5, and 10 years of follow-up was only 92, 75, and 57% in the entire cohort, and was even worse in treatment naive ES patients (survival rate 86, 60, and 34% at 1, 5, and 10 years). Use of DTT was independently associated with a better survival (hazard ratio 0.42, P= 0.015).
CONCLUSION
This study illustrates the alarmingly poor survival prospects of Eisenmenger patients by community-based data even in the current era with advanced DTT and in a country with a wealthy health system. Treatment naive ES patients had especially high mortality rates approaching 60-70% at 10 years of follow-up. Treatment with DTT was associated with better survival.
Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Anticoagulants; Eisenmenger Complex; Female; Humans; Male; Sildenafil Citrate
PubMed: 26843280
DOI: 10.1093/eurheartj/ehv743 -
Annals of Surgery Oct 1991Heart-lung transplantation (HLT) and lung transplantation (LT) are effective treatment modalities for patients with advanced pulmonary parenchymal or vascular disease.... (Comparative Study)
Comparative Study
Heart-lung transplantation (HLT) and lung transplantation (LT) are effective treatment modalities for patients with advanced pulmonary parenchymal or vascular disease. Lung transplantation offers potential advantages over HLT, including reduced pretransplant waiting time and improved efficiency of organ utilization, and is currently being offered to patients formerly treated by HLT. To explore the relative merits of these procedures, the authors examined the results in 44 procedures (23 HLT and 21 LT) in 42 patients transplanted at their institution. Heart-lung transplant recipients included 20 adults and three children (ages 5,5 and 3). Most HLT patients had primary pulmonary hypertension (PPH) (n = 9) or Eisenmenger's syndrome (ES) (n = 8). Twenty-two of twenty-three patients have been long-term survivors (mean follow-up = 17.8 months, Kapaln-Meier survival at 12 months = 85%). Obliterative bronchiolitis (OB) has occurred in five patients (22%), and all have died. Of 21 LTs in 19 patients, nine had obstructive and eight had restrictive lung diseases. Three single-LT (SLT) patients had PPH, and one had ES secondary to a ventricular septal defect. Mean pulmonary artery pressures fell from 55 +/- 6 mm Hg before SLT to 21 +/- 3 mm Hg after SLT; p less than 0.001. Three pediatric patients (ages 4, 10, 17, and 17[re-transplant]) have undergone four SLTs. With mean follow-up of 6.4 months, LT patients have survival at 12 months of 80% (Kaplan-Meier). Lung transplant patients wait a far shorter time for their transplant than do HLT patients (166 vs. 384 days, p less than 0.03). Three patients (19%) have evidence of OB after SLT, with one death. By virtue of equal intermediate-term outcomes, shorter waiting times, and better use of donor organs in comparison with HLT, LT should be offered whenever possible to patients with end-stage pulmonary parenchymal or vascular disease. The authors' pediatric LT and HLT experience (7 treatments in 6 patients) is the largest reported to date and demonstrates the utility of these procedures in this group. Chronic rejection (OB) remains the greatest impediment to long-term survival in both LT and HLT pts.
Topics: Adolescent; Adult; Anastomosis, Surgical; Bronchiolitis Obliterans; Child; Child, Preschool; Eisenmenger Complex; Female; Heart-Lung Transplantation; Hemorrhage; Humans; Hypertension, Pulmonary; Lung; Lung Diseases; Lung Transplantation; Male; Middle Aged; Postoperative Complications; Reoperation; Survival Rate
PubMed: 1953098
DOI: 10.1097/00000658-199110000-00010