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Expert Review of Cardiovascular Therapy Jun 2019: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a... (Review)
Review
: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. : In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. : PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.
Topics: Adult; Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Quality of Life
PubMed: 31120797
DOI: 10.1080/14779072.2019.1623024 -
Proceedings of the Royal Society of... Jun 1971
Topics: Abortion, Spontaneous; Adult; Aortic Valve Insufficiency; Cesarean Section; Delivery, Obstetric; Eisenmenger Complex; Endocarditis, Bacterial; Female; Heart Defects, Congenital; Heart Diseases; Humans; Infant, Newborn; Infant, Premature; Mitral Valve Stenosis; Obstetric Labor Complications; Penicillins; Pregnancy; Pregnancy Complications, Cardiovascular; Prenatal Care; Rheumatic Heart Disease; Tetralogy of Fallot
PubMed: 5090507
DOI: No ID Found -
Heart (British Cardiac Society) Sep 2017
Topics: Eisenmenger Complex; Humans; Incidence; Prevalence
PubMed: 28566473
DOI: 10.1136/heartjnl-2017-311396 -
Revista Espanola de Cardiologia Oct 2010Pulmonary arterial hypertension frequently arises in patients with congenital heart disease. The vast majority present with congenital cardiac shunts. Initially these... (Review)
Review
Pulmonary arterial hypertension frequently arises in patients with congenital heart disease. The vast majority present with congenital cardiac shunts. Initially these may manifest as left-to-right (i.e. systemic-to-pulmonary) shunts. The natural history of disease progression involves vascular remodeling and dysfunction that lead to increased pulmonary vascular resistance and, finally, to the development of Eisenmenger's syndrome, which is the most advanced form. The anatomical, pathological and structural abnormalities occurring in the pulmonary circulation of these patients are, to some extent, similar to those observed in other forms of pulmonary arterial hypertension. This understanding has recently led to significant changes in the management of Eisenmenger's syndrome, with the introduction of treatment specifically targeting pulmonary vascular disease. Early closure of the cardiac shunt remains the best way of preventing pulmonary vascular lesions. However, it is still not clear which preoperative parameters predict safe and successful repair, though hemodynamic evaluation is still routinely used for assessment. Postoperative pulmonary hypertension, both in the immediate period after surgical repair and during long-term follow-up, remains a real therapeutic challenge. The clinical situation of a single ventricle with Fontan circulation also presents difficulties when pulmonary vascular lesions are present. This article reviews pulmonary hypertension associated with congenital shunts and discusses a number of the specific problems encountered.
Topics: Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Treatment Outcome
PubMed: 20875358
DOI: 10.1016/s1885-5857(10)70232-2 -
Circulation Journal : Official Journal... 2015Because of the growing population of patients with congenital heart disease (CHD), most maternal cardiac disease is now congenital in origin. For women with complex CHD,... (Review)
Review
Because of the growing population of patients with congenital heart disease (CHD), most maternal cardiac disease is now congenital in origin. For women with complex CHD, pregnancy poses an increased risk for both the mother, with complications of arrhythmias and heart failure being the most common, and the baby, with a higher chance of miscarriage, intrauterine growth retardation, and the need for early delivery. Pre-pregnancy counseling must be performed by cardiologists who have expertise in both CHD and pregnancy, with a detailed clinical assessment of the patient and the current hemodynamic situation, including echocardiography and an exercise test. In each case the approach must be individualized with consideration of the risks in each case. In some cases, such as Eisenmenger syndrome, pregnancy is contraindicated. Optimum outcomes in these complex patients are achieved when a multidisciplinary approach is used, involving maternal-fetal medicine specialists, cardiologists with expertise in CHD and obstetric anesthesia.
Topics: Adult; Anticoagulants; Counseling; Delivery, Obstetric; Eisenmenger Complex; Female; Genetic Counseling; Heart Defects, Congenital; Hemodynamics; Humans; Hypertension, Pulmonary; Infant, Newborn; Preconception Care; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Risk Factors
PubMed: 26040336
DOI: 10.1253/circj.CJ-15-0572 -
Global Heart Apr 2021Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH.... (Review)
Review
UNLABELLED
Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH. However, prompt and accurate diagnosis of PAH remains an unmet challenge due to lack of awareness and lack of meticulous data to profile the etiology and pathophysiology of this rare progressive disease, especially in low- and middle-income country. In Indonesia, the true prevalence and incidence of different subtypes of PAH in general population is still unknown. The Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was the first single-center prospective registry in Indonesia, which indicated that almost 80% of adult patients with congenital heart disease (CHD) had experienced PAH and even Eisenmenger syndrome due to delayed diagnosis. Screening for early detection of asymptomatic CHD in children is yet to be systematically established in Indonesia, leading to undiagnosed and uncorrected CHD in adulthood. There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Furthermore, the lack of adequate diagnostic facilities, limited treatment availability, and limited drug coverage under the National Health Insurance Scheme are key issues that remain unaddressed. This review focuses on the diagnosis, treatment, and management of PAH associated with CHD in Indonesia as per international guidelines. We have proposed recommendations to effectively control and prevent PAH associated with CHD in Indonesia. The paper should be of interest to readers in the area of medical management and policy makers especially in low- and middle-income countries.
KEY HIGHLIGHTS
Pulmonary arterial hypertension (PAH) is a rare progressive subtype of pulmonary hypertension with poor overall prognosis and outcomes.Prompt and accurate diagnosis of PAH remains an unmet challenge in low- and middle-income countries due to poor knowledge about the etiology and pathophysiology of this syndrome. Also, the symptoms and signs of early-stage PAH are usually nonspecific or undetectable in newborn and infants, thus presenting a challenge for physicians to establish early diagnoses of PAH.The challenging factors in low- and middle-income countries, especially Indonesia archipelago are limitations of healthcare infrastructure, limited expertise, lack of awareness, lack of timely PAH screening strategies, poor antenatal care and unpredictable availability of PAH medications.There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Under-utilization of treatment guidelines and lack of adequate diagnostic treatment facilities have resulted in sub-optimal management of PAH patients in Indonesia.Adherence to international guidelines is an important aspect of PAH management in Indonesia. Updated disease and functional classifications of PAH as per international guidelines along with new research findings on prognostic factors can help in making better management decisions for PAH patients at different stages of the disease.
Topics: Adult; Eisenmenger Complex; Female; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Indonesia; Pregnancy; Pulmonary Arterial Hypertension
PubMed: 34040936
DOI: 10.5334/gh.944 -
European Respiratory Review : An... Dec 2012Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.
Topics: Antihypertensive Agents; Bosentan; Clinical Trials as Topic; Comorbidity; Down Syndrome; Eisenmenger Complex; Endothelium, Vascular; Epoprostenol; Health Behavior; Heart Defects, Congenital; Heart Transplantation; Humans; Hypertension, Pulmonary; Lung Transplantation; Oxygen Inhalation Therapy; Phosphodiesterase 5 Inhibitors; Piperazines; Practice Guidelines as Topic; Pulmonary Circulation; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Thrombosis
PubMed: 23204121
DOI: 10.1183/09059180.00004712 -
Archives of Cardiovascular Diseases Feb 2016Adults with Eisenmenger syndrome have a survival advantage over those with idiopathic pulmonary arterial hypertension. Improved survival may result from preservation of...
BACKGROUND
Adults with Eisenmenger syndrome have a survival advantage over those with idiopathic pulmonary arterial hypertension. Improved survival may result from preservation of right ventricular (RV) function.
AIMS
To assess left ventricular (LV) and RV remodelling in patients with Eisenmenger syndrome compared to a control population, using speckle-tracking imaging.
METHODS
Adults with Eisenmenger syndrome and healthy controls were enrolled into this prospective two-centre study. Patients with Eisenmenger syndrome with low acoustic windows, irregular heart rhythm or complex congenital heart disease were excluded. Clinical assessment, B-type natriuretic peptide (BNP), 6-minute walk test and echocardiography (including dedicated views to perform offline two-dimensional-speckle-tracking analysis) were performed on inclusion.
RESULTS
Our patient population (n=37; mean age 42.3 ± 17 years) was mostly composed of patients with ventricular septal defect (37.8%) or atrial septal defect (35.1%). Compared with the control population (n=30), patients with Eisenmenger syndrome had reduced global LV longitudinal strain (-17.4 ± 3.5 vs. -22.4 ± 2.3; P<0.001), RV free-wall longitudinal strain (-15.0 ± 4.7 vs. -29.9 ± 6.8; P<0.001) and RV transverse strain (25.8 ± 25.0 vs. 44.5 ± 15.1; P<0.001). Patients with Eisenmenger syndrome also more frequently presented a predominant apical longitudinal and transverse strain profile. Among patients with Eisenmenger syndrome, those with a post-tricuspid shunt presented with reduced global LV longitudinal strain but increased RV transverse strain, compared to patients with pre-tricuspid shunt.
CONCLUSION
Patients with Eisenmenger syndrome had impaired longitudinal RV and LV strain, but present a relatively important apical deformation. RV and LV remodelling, as assessed by speckle-tracking imaging, differ between patients with pre- and post-tricuspid shunt.
Topics: Adult; Biomechanical Phenomena; Case-Control Studies; Echocardiography, Doppler; Eisenmenger Complex; Female; France; Humans; Hypertension, Pulmonary; Male; Middle Aged; Predictive Value of Tests; Prognosis; Prospective Studies; Stress, Mechanical; Ventricular Function, Left; Ventricular Function, Right; Ventricular Remodeling
PubMed: 26868160
DOI: 10.1016/j.acvd.2015.11.013 -
Circulation Jan 2019Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind,... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind, randomized, placebo-controlled, 16-week, phase III MAESTRO study (Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity) evaluated the efficacy and safety of the endothelin receptor antagonist macitentan in patients with Eisenmenger syndrome.
METHODS
Patients with Eisenmenger syndrome aged ≥12 years and in World Health Organization functional class II-III were randomized 1:1 to placebo or macitentan 10 mg once daily for 16 weeks. Patients with complex cardiac defects, Down syndrome and background PAH therapy were eligible. The primary end point was change from baseline to week 16 in 6-minute walk distance. Secondary end points included change from baseline to week 16 in World Health Organization functional class. Exploratory end points included NT-proBNP (N-terminal pro-B-type natriuretic peptide) at end of treatment expressed as a percentage of baseline. In a hemodynamic substudy, exploratory end points included pulmonary vascular resistance index (PVRi) at week 16 as a percentage of baseline.
RESULTS
Two hundred twenty six patients (macitentan n=114; placebo n=112) were randomized. At baseline, 60% of patients were in World Health Organization functional class II and 27% were receiving phosphodiesterase type-5 inhibitors. At week 16, the mean change from baseline in 6-minute walk distance was 18.3 m and 19.7 m in the macitentan and placebo groups (least-squares mean difference, -4.7 m; 95% confidence limit (CL), -22.8, 13.5; P=0.612). World Health Organization functional class improved from baseline to week 16 in 8.8% and 14.3% of patients in the macitentan and placebo groups (odds ratio, 0.53; 95% CL, 0.23, 1.24). NT-proBNP levels decreased with macitentan versus placebo (ratio of geometric means, 0.80; 95% CL, 0.68, 0.94). In the hemodynamic substudy (n=39 patients), macitentan decreased PVRi compared with placebo (ratio of geometric means, 0.87; 95% CL, 0.73, 1.03). The most common adverse events with macitentan versus placebo were headache (11.4 versus 4.5%) and upper respiratory tract infection (9.6 versus 6.3%); a hemoglobin decrease from baseline of ≥2 g/dL occurred in 36.0% versus 8.9% of patients. Five patients (3 macitentan; 2 placebo) prematurely discontinued treatment and 1 patient died (macitentan group).
CONCLUSIONS
Macitentan did not show superiority over placebo on the primary end point of change from baseline to week 16 in exercise capacity in patients with Eisenmenger syndrome.
CLINICAL TRIAL REGISTRATION
URL: https://www.clinicaltrials.gov . Unique identifier: NCT01743001.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antihypertensive Agents; Biomarkers; Child; Double-Blind Method; Down Syndrome; Eisenmenger Complex; Endothelin Receptor Antagonists; Exercise Tolerance; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Pulmonary Artery; Pyrimidines; Recovery of Function; Sulfonamides; Time Factors; Treatment Outcome; Walk Test; Young Adult
PubMed: 30586694
DOI: 10.1161/CIRCULATIONAHA.118.033575 -
Cardiovascular Diagnosis and Therapy Aug 2021Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. In... (Review)
Review
Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. In developed countries, cardiac defects are being operated on in a timely manner, before pulmonary vascular disease develops. However, with rising immigration from underserved countries, we increasingly see patients with shunt lesions, that are not amenable for repair as pulmonary vascular disease has already established. ES describes a symptom complex and patients present with heterogeneous problems involving many organ systems (multisystem disorder). Care in tertiary specialist cardiac centers with access to multidisciplinary subspecialities is required. Central cyanosis with secondary erythrocytosis is one of the key features of patients with ES. Clinical consequences of longstanding hypoxia can lead to other organ complications, that involve other organs than the heart alone. Although ES patients have a better prognosis compared to other patients with pulmonary arterial hypertension, ES grossly affects quality of life and morbidity is frequent. Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. This is necessary to maintain functional capacity, decrease morbidity and increase life expectancy for these vulnerable patients. The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. Various factors have been shown to be associated with mortality like decreased arterial oxygen saturation, functional class, impaired exercise tolerance, syncopal events, iron deficiency, presence of pre-tricuspid shunts, arrhythmias, increased (NT-pro) brain natriuretic peptide, echocardiographic variables of right ventricular dysfunction and hospitalization for heart failure. Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES.
PubMed: 34527543
DOI: 10.21037/cdt-21-135