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Journal of Otolaryngology - Head & Neck... Aug 2019The natural history of patients diagnosed with Eisenmenger's Syndrome typically revolve around the pediatric population. Medical advances have allowed these patients to...
BACKGROUND
The natural history of patients diagnosed with Eisenmenger's Syndrome typically revolve around the pediatric population. Medical advances have allowed these patients to live longer and present with a different subset of symptoms as a result of the progression of their disease process.
CASE PRESENTATION
In this case report, we discuss a 77-year-old Caucasian female with Eisenmenger's Syndrome presenting with hoarseness. Clinical and imaging studies reveal a left vocal cord paralysis secondary to a progressively enlarging patent ductus arteriosus (PDA) and dilation of pulmonary arteries causing mass effect on the left recurrent laryngeal nerve.
CONCLUSION
From a clinical perspective, this case highlights the need for otolaryngologists to be aware of the pathophysiology of Eisenmenger's Syndrome as it progresses with age.
Topics: Aged; Eisenmenger Complex; Female; Hoarseness; Humans
PubMed: 31426857
DOI: 10.1186/s40463-019-0358-3 -
The Pan African Medical Journal 2022Eisenmenger syndrome is a dramatic complication of certain heart diseases. It is an absolute contra-indication to pregnancy because it holds a high risk of...
Eisenmenger syndrome is a dramatic complication of certain heart diseases. It is an absolute contra-indication to pregnancy because it holds a high risk of maternal-fetal mortality. Nowadays, we don´t see much of this condition, because congenital heart defects are diagnosed during childhood, infancy, or prenatally; and even if a pregnancy occurs, it is usually terminated as soon as it is diagnosed. Nevertheless, some women are willing to carry the risks that come with their condition. We report the case of a 26-year-old woman with an undiagnosed interventricular septal wall defect that evolved into Eisenmenger syndrome during her pregnancy. A successful elective C-section was done under epidural anesthesia and close monitoring. When facing this challenging situation, there are no clear guidelines, however, premature and elective delivery is paramount.
Topics: Humans; Pregnancy; Female; Adult; Eisenmenger Complex; Heart Septal Defects, Ventricular; Heart Defects, Congenital; Maternal Mortality
PubMed: 36915419
DOI: 10.11604/pamj.2022.43.188.32982 -
Korean Journal of Anesthesiology Dec 2015A 26-year-old parturient with Eisenmenger's syndrome and complete atrioventricular block was presented for emergency Cesarean section due to preterm labor. Ventricular...
A 26-year-old parturient with Eisenmenger's syndrome and complete atrioventricular block was presented for emergency Cesarean section due to preterm labor. Ventricular tachycardia (VT), which progressed to ventricular fibrillation (VF), started immediately after the incision. Cardiopulmonary resuscitation with electric shocks was given by anesthesiologists while the obstetrician delivered the baby between the shocks. A cardiac surgeon was ready for extracorporeal membrane oxygenation institution in case of emergency but spontaneous circulation of the patient returned after the 3rd shock and the delivery of the baby. The newborn's Apgar score was 4 at 1 minute and 8 at 5 minutes. An implantable cardioverter-defibrillator was inserted before the discharge because the patient had recurrent episodes of VT and VF postoperatively.
PubMed: 26634088
DOI: 10.4097/kjae.2015.68.6.617 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Sep 2014Eisenmenger syndrome (ES) occurs as the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease. In this study, we aimed to...
[The management of adult female patients with Eisenmenger syndrome and advanced pulmonary arterial hypertension treatment: single center experience and follow-up for 5 years].
OBJECTIVES
Eisenmenger syndrome (ES) occurs as the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease. In this study, we aimed to evaluate the management of ES patients, follow-up and specific PAH treatment applying and clinical outcomes during 5 years.
STUDY DESIGN
During the period of the month between May 2008 and 2013 ES female patients were included in the study and followed an average for 5 years. Clinical findings, brain natriuretic peptide levels, transthoracic and right heart catheterization findings, 6-min walking test distance were recorded. PAH specific treatment as bosentan, iloprost and sildenafil was given to patients according to guidelines. The patients were evaluated with 3 months intervals as requirement for hospitalization, combination treatment, and mortality.
RESULTS
A total of 12 patients were included in the study. All of the patients were women, the mean age was 36.5. As prognostic echocardiographic data, the patients had high pulmonary artery pressure (109.81 ± 24.94 mmHg) related with increased right ventricular wall thickness, elevated right atrial pressure, severe pulmonary regurgitation in 40%, shortened pulmonary acceleration time, diminished myocardial tissue Doppler velocities of the left and right ventricles, increased right atrium area/left atrial area ratio (1.35 ± 0.40), lower right ventricular fractional area change. During the follow-up period of 5 years, a total of 16 events occurred. Combination treatment was required in 8 patients.
CONCLUSION
Eisenmenger syndrome is a multi-system affecting disease and due to high morbidity and mortality risk patients with ES should be followed by specialized centers. PAH specific treatment improves the disease course and survival of patients.
Topics: Adult; Antihypertensive Agents; Bosentan; Echocardiography; Eisenmenger Complex; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary; Iloprost; Laser-Doppler Flowmetry; Piperazines; Pulmonary Wedge Pressure; Purines; Severity of Illness Index; Sildenafil Citrate; Sulfonamides; Treatment Outcome; Turkey
PubMed: 25362943
DOI: 10.5543/tkda.2014.54060 -
Medicina (Kaunas, Lithuania) Feb 2024Advances 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...
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. A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24-48 h of admission. This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD.
Topics: Adult; Male; Humans; Middle Aged; Hypertension, Pulmonary; Cardiovascular Diseases; Heart Defects, Congenital; Eisenmenger Complex; Heart Failure
PubMed: 38399553
DOI: 10.3390/medicina60020266 -
Medicine May 2019It is commonly reported a limitation of therapeutic strategy in Eisenmenger syndrome (ES) historically. This qualitative systematic review is conducted to evaluate the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
It is commonly reported a limitation of therapeutic strategy in Eisenmenger syndrome (ES) historically. This qualitative systematic review is conducted to evaluate the safety and efficacy of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) for ES patients for a clinical therapeutic strategy based on evidence.
METHODS
PubMed, EMBASE, and the Cochrane Library databases have been systematically reviewed up to January 2019. Two reviewers independently conducted a literature search, quality evaluation, and data extraction. The occurrence of death, deterioration, and adverse events (AEs) has respectively been described as a count or percentage. Meta-analysis was conducted by Stata 15.1, and weighted mean differences (WMD) with 95% confidence intervals (CI) were recorded for continuous data. Randomized-effect model or fixed-effect model was applied according to the heterogeneity test.
RESULTS
Fifteen citations recruiting 456 patients associated with ES were eventually pooled, which involved 4 RCTs, 6 prospective studies, and 5 retrospective studies. Within the first year, it indicated PAH-SDT significantly ameliorated exercise capacity in 6-minute walk distance (6MWD) (I = 60.5%; WMD: 53.86 m, 95% CI [36.59, 71.13], P < .001), functional class (FC) (WMD = -0.71, 95% CI [-0.98, -0.44], P < .001) and Borg dyspnea index (WMD = -1.28, 95% CI [-1.86, -0.70], P < .001), in addition to hemodynamics, especially mean pulmonary arterial pressure by 5.70 mmHg (WMD = -5.70 mmHg, 95% CI [-8.19, -3.22], P < .001) and pulmonary vascular resistance by 4.20 wood U (WMD: -4.20, 95% CI [-7.32, -1.09], P = .008), but unsatisfactory effects in oxygen saturation at exercise (P = .747). In a prolonged medication, bosentan, a dual ERA, has been proved acting an important role in improving exercise tolerance of patients with ES (6MWD: I = 47.5%; WMD: 88.68 m, 95% CI [54.05, 123.3], P < .001; FC: I = 0.0%; WMD = -0.65, 95% CI [-1.10, -0.19], P = .006). While a nonsignificant change of 6MWD was noted in a long-term therapy of ambrisentan (P = .385). There existed rare evidence about the efficacy and safety of macitentan, phosphodiesterase-5 inhibitors (PDE5i), and prostanoids in a prolonged medication. Most AEs were recorded as mild to moderate with PAH-SDT, but about 4.3% individuals treated with endothelin receptor antagonists (ERAs) suffered from serious ones, and 3.9% suffered from death.
CONCLUSIONS
This systematic review and meta-analysis proved PAH-SDT as a safe and effective role in ES in an early stage. However, in a long-term treatment, bosentan has been supported for a lasting effect on exercise tolerance. A further multicenter research with a large sample about pharmacotherapy of ES is necessary.
Topics: Antihypertensive Agents; Bosentan; Eisenmenger Complex; Endothelin Receptor Antagonists; Exercise Tolerance; Hemodynamics; Humans; Hypertension, Pulmonary; Oxygen; Phenylpropionates; Phosphodiesterase 5 Inhibitors; Prostaglandins; Pyridazines; Pyrimidines; Sulfonamides
PubMed: 31096477
DOI: 10.1097/MD.0000000000015632 -
Acute and Critical Care Feb 2020The presentation of coronary artery disease in a patient with Eisenmenger syndrome (ES) is relatively rare. Cardiogenic shock due to coronary artery dissection during...
The presentation of coronary artery disease in a patient with Eisenmenger syndrome (ES) is relatively rare. Cardiogenic shock due to coronary artery dissection during percutaneous coronary intervention (PCI) can be more critical in these patients. Here, we report a case of successful PCI under mechanical circulation support in a patient with ES who experienced potentially fatal right coronary artery dissection. This case emphasizes that use of extracorporeal membrane oxygenation (ECMO) can lead to successful management of critical complication during PCI, and that the immediate decision to apply of ECMO is important in ES patients who face impending cardiogenic shock with acute heart failure.
PubMed: 31743640
DOI: 10.4266/acc.2017.00024 -
JACC. Case Reports Apr 2024This clinical vignette describes the first case of a woman in his 40s with a set of congenital anomalies given by tetralogy of Fallot associated with aortic origin of...
This clinical vignette describes the first case of a woman in his 40s with a set of congenital anomalies given by tetralogy of Fallot associated with aortic origin of the left pulmonary artery and uncorrected right aortic arch. All of these entities have a poor probability of survival in adulthood.
PubMed: 38774807
DOI: 10.1016/j.jaccas.2024.102277 -
Journal of Investigative Medicine High... 2020Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal...
Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal defect, and right ventricular hypertrophy. Without surgical management, approximately only 3% of patients survive past the age of 40 years. Cases of unoperated patients reaching adulthood have been reported; however, few studies describe treatment guidelines for surgical or therapeutic management. In this article, we report the case of a 59-year-old Hispanic male with unoperated tetralogy of Fallot presenting to our cardiology clinic for initial workup and management.
Topics: Anticoagulants; Atrial Fibrillation; Cardiac Catheterization; Disease Management; Eisenmenger Complex; Electrocardiography; Humans; Hypertension, Pulmonary; Hypertrophy, Right Ventricular; Male; Middle Aged; Radiography, Thoracic; Survivors; Tetralogy of Fallot
PubMed: 32462941
DOI: 10.1177/2324709620926908 -
Internal Medicine (Tokyo, Japan) Dec 2021A 52-year-old man who was diagnosed with Eisenmenger syndrome due to a muscular-type ventricular septal defect 30 years previously, visited our emergency room after...
A 52-year-old man who was diagnosed with Eisenmenger syndrome due to a muscular-type ventricular septal defect 30 years previously, visited our emergency room after experiencing six hours of severe left flank pain and vomiting. On laboratory examination, azotemia and microscopic haematuria were identified. Contrast-enhanced computed tomography also revealed pulmonary embolism (PE) and bilateral acute renal infarction. The flank pain resolved after heparin was administered for anti-coagulation and aspiration thrombectomy was performed. The patient was discharged on warfarin as anticoagulant therapy. In this case, a paradoxical embolism was considered to have been the cause of PE and bilateral acute renal infarction in a patient with Eisenmenger syndrome.
Topics: Acute Kidney Injury; Eisenmenger Complex; Embolism, Paradoxical; Heart Septal Defects, Ventricular; Humans; Infarction; Male; Middle Aged; Pulmonary Embolism
PubMed: 34148965
DOI: 10.2169/internalmedicine.7549-21