The patients with congenital cardiac defects usually present with Eisenmenger syndrome before they reach puberty. The most common symptoms are:

• Cyanosis [4]
• Dyspnea on exertion [5]
• Fatigue
• Dizziness
• Headache
• Syncope
• Chest pain
• Palpitations
• Paresthesia
• Blurred vision

Other pathologies associated with Eisenmenger syndrome occur mainly due to decrease in oxygenation of blood and increase in the viscosity of blood [6].

Common complications are:

• Congestive cardiac failure
• Iron deficiency anemia
• Kidney problems
• Gout (due to hyperurecemia)
• Stroke
• Gall stones (due to hyperbilirubinemia)
• Hypertrophic osteoarthropathy

Female patients of Eisenmenger syndrome are advised not to become pregnant because it may pose a threat to both the mother and the fetus. Although successful deliveries have been reported but mortality rate in such cases is estimated to be 30 to 60%. Common causes for maternal death are thromboembolism, hypovolemia, hemoptysis or preeclampsia either during delivery or during the first week after parturition [7].

• Atrial Septal Defect

  The Figure shows macroscopic lesions typical of ES, from a 56-year-old male patient with a non-repaired atrial septal defect. [ncbi.nlm.nih.gov]

  The hole may be a ventricular septal defect, an atrial septal defect or a patent ductus arteriosus. A baby born with a single ventricle also may develop this condition. [ucsfhealth.org]

• Swelling

  […] vessels of the lungs Erythrocytosis—increased number of red blood cells in the blood due to decreased oxygen in the blood Other general symptoms of Eisenmenger syndrome include: Shortness of breath while at rest Fainting (syncope) Headaches Abdominal swelling [rockymountainhospitalforchildren.com]

  Other medications can help with specific symptoms, like iron supplements for anemia or diuretics to help with swelling. Oxygen may make you feel better. What You Can Do The choices you make are important in managing Eisenmenger syndrome. [webmd.com]

  Digital swelling (clubbing) may occur. Heart murmurs may occur. EKG may show RVH and atrial arrhythmias (see figures 2, 3a, 3b, 5a, 5b, 14, 15a, 15b). [rjmatthewsmd.com]

  Let your ACHD doctor know if you develop shortness of breath, swelling in your abdomen or legs and fatigue. Rarely years of cyanosis and pulmonary arterial hypertension can cause one of the coronary arteries to become compressed causing chest pain. [achaheart.org]

• Disability

  […] unnecessary delays to your disability payments. [disabilitybenefitscenter.org]

  We report on the case history of a 38-year-old male patient with Down syndrome (DS), intellectual disability (ID), and ES. The patient was scheduled for extraction of the right maxillary second molar tooth. His physical health was poor. [ncbi.nlm.nih.gov]

  In our study, patients had a low level of intellectual disability; however, 6MWD was in the range seen in patients with severe intellectual disability in the Vis study. [bmccardiovascdisord.biomedcentral.com]

• Impaired Exercise Tolerance

  Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure [heart.bmj.com]

• Dyspnea

  Heart rate recovery at the first minute (HRR 1 ) and perceived effort Borg scale for dyspnea and lower limb fatigue were recorded in both tests scenarios. [ncbi.nlm.nih.gov]

  Eisenmenger’s syndrome is suspected if there is a history of cyanotic spells or dyspnea on exertion since childhood. [symptoma.com]

• Exertional Dyspnea

  Clinical manifestations include exertional dyspnea, fatigue, lethargy, central cyanosis, chest pain, palpitations, atrial and ventricular arrhythmias. [orpha.net]

  Eighteen consecutive patients with ES and exertional dyspnea according to the World Health Organization functional class III or IV were prospectively recruited. [ncbi.nlm.nih.gov]

• Labored Breathing

  The Borg dyspnea index rates the severity of dyspnea (difficult or labored breathing) on a scale from 0 ('Nothing at all') to 10 ('Very, very severe - maximal'). A decrease in the Borg dyspnea index indicates an improvement. [clinicaltrials.gov]

• Pulsatile Liver

  liver, AV-block, erythrocytosis, hyperchromasia, truncus arteriosus, Transthoracic echocardiography, surrogate markers, aorto-pulmonary, CT angiography, glass opacification, Metabolic factors, Euler-Liljestrand, pre-symptomatic, ventilatory threshold [eurekaselect.com]

  A third heart sound as well as hepatosplenomegaly or a pulsatile liver occur with right heart failure. 4.2. [ncbi.nlm.nih.gov]

• Cyanosis

  When the pulmonary arterial resistance exceeds the systemic resistance, shunt reversal occurs with right-to-left shunt and cyanosis. [thoracickey.com]

  From Wikidata Jump to navigation Jump to search fetal heart defect Eisenmenger's reaction tardive cyanosis ES Eisenmenger syndrome Eisenmenger's Syndrome edit English Eisenmenger's syndrome fetal heart defect Eisenmenger's reaction tardive cyanosis ES [wikidata.org]

  […] and ventricular arrhythmias. [ 2 ] Dyspnoea, fatigue, syncope; exercise intolerance (dyspnoea and fatigue) is proportional to the degree of hypoxaemia or cyanosis. [patient.info]

  Characterized by exercise intolerance, cyanosis, anasarca, palpable thrill and auscultatable cardiac murmur. [medical-dictionary.thefreedictionary.com]

  Eisenmenger syndrome (i�sәn-meng″әr) ventricular septal defect with pulmonary hypertension and cyanosis due to right-to-left (reversed) shunt of blood. [web.archive.org]

• Heart Disease

  Heart Disease and Pregnancy. London: RCOG Press; 2006. pp. 211–29. [ Google Scholar ] 51. Dhanjal M. Contraception in women with heart disease. In: Steer PJ, Gatzoulis MA P B, editors. Heart Disease and Pregnancy. [ncbi.nlm.nih.gov]

  Clinical Topics: Anticoagulation Management, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Prevention Keywords: Adrenergic beta-Antagonists, [acc.org]

  Keywords Eisenmenger syndrome; Cyanotic heart disease; Menorrhagia; Contraception Introduction In 2005 there were an estimated 1 million adults living with congenital heart disease, with an expected increase in prevalence of 5% per year [ 1 ]. [gynecology-obstetrics.imedpub.com]

  : Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency. J Pediatr 1979, 95 :567–569. CrossRef Google Scholar 16. Gidding SS, Stockman JA: Erythropoietin in cyanotic heart disease. [link.springer.com]

• Heart Murmur

  Although she had been noticed to have the enlarged heart and exertional shortness of breath to a slight degree, she had been apparently in good condition without any significant heart murmurs. [ncbi.nlm.nih.gov]

  Heart failure suggest a poor prognosis, and sudden death is possible. Digital swelling (clubbing) may occur. Heart murmurs may occur. EKG may show RVH and atrial arrhythmias (see figures 2, 3a, 3b, 5a, 5b, 14, 15a, 15b). [rjmatthewsmd.com]

  Images Snapshot A 20-year-old woman with a history of a childhood heart murmur presents for multiple syncopal episodes. She reports feeling constantly fatigued and has shortness of breath with exertion, even with minor physical activity. [step2.medbullets.com]

  She had had a “benign” heart murmur since childhood. An echocardiogram taken before her transfer revealed a dilated right atrium and right ventricle and a normal left ventricle with normal function. [nejm.org]

• Diastolic Murmur

  […] dysfunction Epidemiology Risk factors congenital heart defect Presentation Symptoms shortness of breath syncope chest pain hemoptysis exercise intolerance Physical exam edema cyanosis of lips, oral mucosa, or extremities cardiac exam high-pitched early diastolic [step2.medbullets.com]

  […] childhood death can result from decompensated cor pulmonale Presentation Symptoms shortness of breath syncope chest pain hemoptysis exercise intolerance Physical exam edema cyanosis of lips, oral mucosa, or extremities cardiac exam high-pitched early diastolic [medbullets.com]

  Ejection systolic murmur audible along the left sternal border. Graham Steell murmur: a diastolic murmur audible along the left sternal border due to functional incompetence of the pulmonary valve in patients with pulmonary hypertension. [patient.info]

• Graham Steell Murmur

  Ejection systolic murmur audible along the left sternal border. Graham Steell murmur: a diastolic murmur audible along the left sternal border due to functional incompetence of the pulmonary valve in patients with pulmonary hypertension. [patient.info]

  In patients with pulmonary regurgitation, a relatively low-grade proto-meso-diastolic murmur, resulting from a high pulmonary vascular resistance (Graham Steell murmur), may be audible. [empendium.com]

  In case of pulmonic regurgitation a decrescendo murmur (Graham Steell murmur) is audible early in diastole over the left upper parasternal border. [ncbi.nlm.nih.gov]

• Flushing

  All intravenous lines were carefully flushed to prevent air embolism. It was prudent to avoid nitrous oxide due to its added effect on pulmonary vascular resistance. Prevention of hypoxia, hypercarbia, acidosis and hypothermia are very crucial. [apicareonline.com]

  […] also been shown to have effects on systemic blood pressure and systemic vascular resistance, coronary blood flow and myocardial contractility. [2], [6] The most commonly reported side-effects of sildenafil can be attributed to vasodilation, such as flushing [annalspc.com]

  […] an RCT in 130 patients, improvements in exercise capacity at 6 months were observed, but there was no hemodynamic improvement or long-term outcome benefits.70 Its use in ES is limited and is associated with frequent adverse events such as headache, flushing [dovepress.com]

• Dizziness

  The patient has blue skin, chest pain, fatigue, dizziness, blurred vision, shortness of breath and fainting episodes. If diagnosed early, the condition can be treated with drugs and surgical repair of the heart defect. [symptoma.com]

  Dizziness and syncope due to low cardiac output is much rarer in Eisenmenger's syndrome than in PAH without intracardiac shunts. Clubbing and hemoptysis may occur. [orpha.net]

  […] grayish colored skin (cyanosis), high pressure in the blood vessels going to the lungs, erythrocytosis (higher than normal number of red blood cells), shortness of breath, fatigue, chest pain or tightness, heart palpitations, blurred vision, headaches and dizziness [nicklauschildrens.org]

Eisenmenger’s syndrome is suspected if there is a history of cyanotic spells or dyspnea on exertion since childhood. The clinical examination of these patients may reveal:

• Abnormal heart rhythm
• Clubbing of fingers and toes
• Cardiac murmur

The diagnosis of Eisenmenger’s syndrome is confirmed by specific laboratory investigations;

• Complete blood count
• Chest X-ray
• Cardiac catheterization
• Echocardiogram
• Electrocardiogram
• MRI of heart

• Right Bundle Branch Block

  Age, atrial fibrillation, prolonged QRS duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased [mayoclinic.pure.elsevier.com]

  Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. [bmccardiovascdisord.biomedcentral.com]

• Incomplete Right Bundle Branch Block

  Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. [bmccardiovascdisord.biomedcentral.com]

• P Pulmonale

  ECG: almost always abnormal; shows features suggestive of right heart hypertrophy (with tall R wave in V1, deep S wave in V6, ST and T wave abnormalities, P pulmonale) as well as abnormalities associated with the underlying defect. [patient.info]

• Prominent A-Wave

  ECG of Eisenmenger syndrome Usually there is right ventricular hypertrophy with prominent P waves. [remedyland.com]

  In tricuspid regurgitation, the jugular venous pressure may be elevated and shows a prominent "v" wave, Fig. ( 2 ). Elevated jugular venous pressure and prominent "v" wave in right heart failure and severe tricuspid regurgitation. [ncbi.nlm.nih.gov]

• Biventricular Hypertrophy

  Sometimes, these alterations are superimposed by ECG signs of the underlying cardiac defect (e.g. left or biventricular hypertrophy in univentricular hearts, transposition of the great arteries (TGA), truncus arteriosus). [ncbi.nlm.nih.gov]

The management of the disease depends upon the severity of the defect and the condition of the patient. Medical therapy consists of drugs like calcium channel blockers, prostacyclin and endothelin receptor antagonist, to control pulmonary hypertension. In older children, oxygen therapy and phlebotomy are also considered as treatment options [8].

In case of irreversible damage to the pulmonary vasculature and development of severe pulmonary hypertension, the only treatment options are lung transplant or cardiopulmonary transplant.

In addition, the patients of Eisenmenger syndrome are also advised to avoid smoking and alcohol. Coughing in these individuals should be suppressed with antitussives to prevent the risk of pulmonary hemorrhage.

Vaccination against influenza and pneumonia are also recommended to these patients.

In female patients of reproductive age group, contraception by means of tubal ligation is advised because Eisenmenger syndrome may cause serious complications in pregnancy which may lead to maternal death in about 50% of cases.

The patient outlook depends upon the age at presentation. If the patient presents early before the development of irreversible lesions in the capillaries of lungs and severe pulmonary hypertension, the cardiac defect can be repaired through surgery.

The survival of the patient is also reduced if the cardiac lesion is complex or there is some co-existing medical condition like Down’s syndrome (trisomy 21) [9].

Pregnancy also poses a risk to the life of the patient. Mortality rate in pregnant females with Eisenmenger’s syndrome is more than 50%. The overall life expectancy of the patients with this disease is 20 to 50 years.

Some patients with a simpler cardiac lesion and mild symptoms may live up to the sixth decade of life. The five year survival rate of patients who have undergone cardiopulmonary or lung transplant is estimated to be 70 to 80% [10].

The terminal event in most of the patients is congestive heart failure, thromboembolism or massive hemoptysis.

Eisenmenger’s syndrome results from the effects of long standing high pressure in the pulmonary circulation due to an underlying cardiac structural anomaly.

The defect in the heart causes the blood to flow from left to the right side. This abnormal shunting of blood will raise the pressure in the pulmonary vasculature to the point that the blood starts flowing from right to left side.

The most common defects which leads to the development of Eisenmenger’s syndrome are:

• Atrial septal defect
• Ventricular septal defect
• Patent ductus arteriosus
• Atrioventricular canal defect
• Aortopulmonary window

All of the above mentioned conditions result in the abnormal flow of blood to the pulmonary vessels and lead to increase in blood pressure in the lungs and cause endothelial injury in lung capillaries as well as insufficient oxygenation of blood.

Although the cardiac defect is present at birth, patients with Eisenmenger’s syndrome usually come to clinical attention either before puberty or in early adulthood.

The incidence of this syndrome has fallen greatly in developed countries due to medical screening with echocardiography in early life.

In underdeveloped countries, it is still prevalent and the presentation of the patients is quite late when symptoms of overt pulmonary hypertension have developed.

The human heart consists of four chambers. The left heart, consisting of left atrium and ventricle, is responsible for supplying oxygenated blood to the whole body whereas the right heart, consisting of the right atrium and ventricle, directs deoxygenated blood to the lungs for oxygenation. Since the left heart has to exert greater force to propel blood, it is more muscular than the right heart. Also the pressure in the left heart is higher than that of the right heart.

During fetal life, there is a communication between the pulmonary artery and the aortic arch - the ductus arteriosus, through which most of the blood from right ventricle bypasses the lungs. This channel closes within 12 to 24 hours after birth and seals completely in three weeks.

Eisenmenger’s syndrome develops when the normal anatomy of the heart is disturbed. It may either be due to any defect in the cardiac septa or due to patent ductus arteriosus. These anomalies result in shunting of blood from the high pressure left side of the heart to the low pressure right side of the heart. As the pressure rises, it causes damage to the capillaries of the lungs which get replaced with scar tissue.

This leads to a decrease in the useful volume of pulmonary vasculature and thus, decreased oxygenation of blood. Also the fibrosed vessels are less expansible. They contribute to the rise in the pulmonary blood pressure as well as hypertrophy of the right heart. Eventually, the pressure in the right circuit exceeds the pressure in the left heart causing right to left shunting of blood.

The deoxygenated blood from the right side flows to the left side and in turn to the whole body, thereby resulting in cyanosis [2]. The lack of oxygenation of blood leads to overproduction of red blood cells in the bone marrow to compensate the hypoxia resulting in polycythemia. Due to the hyperviscosity of blood, there is increased risk of thrombus formation in the blood vessels. The damaged blood vessels may also cause abnormal bleeding episodes in the form of hemoptysis [3].

There are no guidelines for prevention of Eisenmenger’s syndrome.

Eisenmenger syndrome (ES) or Eisenmenger reaction is described as a process in which an untreated congenital cardiac defect like atrial or ventricular septal defect, patent ductus arteriosus or an atrioventricular canal defect, leads to abnormal shunting of blood from systemic to pulmonary circulation. This results in pulmonary hypertension and cyanosis in adolescence or early adult life [1].

The patients usually present with cyanosis, erythrocytosis, clubbing of fingers, syncope, arrhythmias, bleeding disorders and anemia. The condition was named after Doctor Victor Eisenmenger who first reported a patient in 1897 with cyanosis and dyspnea from infancy, who eventually died of cardiac failure and was found to have a large ventricular septal defect on autopsy.

Initially, the septal defect causes left to right shunting of blood but as the pressure within the pulmonary vasculature keep on raising it leads to pulmonary hypertension and the reversal of shunt to right to left. With the development of pulmonary hypertension, the condition becomes irreversible and inoperable. If diagnosed early, the defect can be repaired through surgery. The patient survival ranges from 20 to 50 years of age.

Eisenmenger’s syndrome is a condition related to a defect or hole in the heart which is present at the time of birth. This defect causes abnormal flow of blood between the heart chambers and thus the deoxygenated blood from the right side of the heart flows in the whole body.

The symptoms start in childhood or early adulthood. The patient has blue skin, chest pain, fatigue, dizziness, blurred vision, shortness of breath and fainting episodes.

If diagnosed early, the condition can be treated with drugs and surgical repair of the heart defect. If presented late, the only treatment option is lung transplant or a heart and lung transplant.

The overall survival of the patient is around 20 to 50 years.

1. Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L. [Eisenmenger syndrome]. Ugeskrift for laeger. Apr 6 2009;171(15):1270-1275.
2. Wagenvoort CA, Wagenvoort N. Pathology of the Eisenmenger syndrome and primary pulmonary hypertension. Advances in cardiology. 1974;11(00):123-130.
3. Sekiguchi M. Eisenmenger syndrome. Ryoikibetsu shokogun shirizu. 1996(13):11-14.
4. Srinivas SK, Manjunath CN. Differential clubbing and cyanosis: classic signs of patent ductus arteriosus with Eisenmenger syndrome. Mayo Clinic proceedings. Sep 2013;88(9):e105-106.
5. Van De Bruaene A, De Meester P, Voigt JU, et al. Worsening in oxygen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger syndrome. International journal of cardiology. Sep 30 2013;168(2):1386-1392.
6. Tomov L, Tomov I, Todorova M. Eisenmenger syndrome. Vutreshni bolesti. 1973;12(3):102-107.
7. Russo R, Bortolotti U, Grella P, Terribile V, Schivazappa L. Eisenmenger syndrome and pregnancy. Minerva cardioangiologica. Oct 1980;28(10):711-715.
8. Trojnarska O, Plaskota K. Therapeutic methods used in patients with Eisenmenger syndrome. Cardiology journal. 2009;16(6):500-506.
9. Saha A, Balakrishnan KG, Jaiswal PK, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. International journal of cardiology. Jul 1994;45(3):199-207.
10. Stoica SC, McNeil KD, Perreas K, et al. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. The Annals of thoracic surgery. Dec 2001;72(6):1887-1891.