It is a condition associated with VENTRICULAR SEPTAL DEFECT and other congenital heart defects that allow the mixing of pulmonary and systemic circulation, increase blood flow into the lung, and subsequent responses to low oxygen in blood. The left-to-right shunting of blood results in abnormally high blood flow and pressure directed to the right heart circulation, gradually leading to maladaptive changes that ultimately result in pulmonary hypertension. Increased right-sided blood volume and pressure causes a cascade of pathologic damage to the delicate pulmonary capillaries, causing them to be incrementally replaced with scar tissue. Although there's no cure, medications may help you feel better, improve your quality of life and prevent serious complications. Doctors don't generally recommend surgery to repair the hole in your heart once Eisenmenger syndrome has developed, and any surgery may be life-threatening. Sidanefil group of medicine is helpful to some extent in PAHT.
Do cath study and oximetry for reversibilty. Doesnot seems to me inoperable. He may undergo surgery and closure of ASD with leaving residual PFO. You may use Sildenafil 20 mg TDS or Boseantan 62.5 BD or Ambrisantan etc
Tab. Bosentan 62.5 mg. Cynosis and clubbing present. Heart lung transplant is only treatment for complete cure.
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What is the most likely diagnosis? A) Acromegaly B) Cystic fibrosis C) Eisenmenger's syndrome D) Squamous-cell lung cancer E) Ulcerative colitisDr. Raj Sharma5 Likes29 Answers
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45 years old male smoker presented with Exertional Breathlessness, cough, wheeze . Examination revealed HR 76/min, BP 120/70 mmhg, Mild darkish fingertips, he has short systolic murmur . Lung few scattered rhonchi. ECG& X Ray taken. What is your impression and how do you approachDr. Mahantesh R Charantimath6 Likes13 Answers
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45 years old male smoker presented with cough Breathlessness wheeze having this kind of x Ray and ECG changes suggests Pulmonary Hypertension could be due to COPD as all thought . Thanks all who got involved in this case . This is very typical case of CHD Eisenmenger syndrome . X ray is very typical water jug appearance due to dilatation of MPA and branches with peripheral pruning of vessels , right Atria , right Ventricle enlightenment . X ray revealed mild calcification of MPA right Lowe lobe bronchiectasis changes. ECG is unusual inspite of severe PAH, where PAH is made out by only persistence of S wave upto V6. Sometimes ECG can be misleading . Echo revealed large Subpulmonary VSD with Bidirectional shunt & severe PAH. HB 18 g due to Erythrocytosis. Had mild clubbing and cynosis. Patient has been on Endothelin receptor antagonists Ambrisentan 5 mg of & sildanefil 20 mg bd and Small dose of diuretic torsemide 5 mg OD. I have been following him up for the past 8 years he is doing well with ambrisentan and seldanefil. Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease. Lesions in Eisenmenger syndrome, such as large septal defects, are characterized by high pulmonary pressure and/or a high pulmonary flow state. Development of the syndrome represents a point at which pulmonary hypertension is irreversible and is an indication that the cardiac lesion is likely inoperable Eisenmenger syndrome was initially described in 1897, when Victor Eisenmenger reported on a patient with symptoms of dyspnea and cyanosis from infancy who subsequently developed heart failure and succumbed to massive hemoptysis. An autopsy revealed a large ventricular septal defect (VSD) and an overriding aorta. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension. Advances in the medical treatment of patients with severe pulmonary hypertension may improve survival in patients with Eisenmenger syndrome and may potentially reverse the process in selected patients to a point at which they again become candidates for surgical repair. Pulmonary hypertension is defined as a mean pulmonary artery pressure of more than 25 mm Hg at rest or more than 30 mm Hg during exercise. Causes of Eisenmenger syndrome include the following: Large, uncorrected cardiac shunt or palliative, surgically created systemic-to-pulmonary shunt for congenital heart disease Large, nonrestrictive VSD Nonrestrictive PDA Atrioventricular septal defect, including a large ostium primum ASD without a ventricular component Aortopulmonary window Palliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart disease The left-to-right shunting of blood results in abnormally high blood flow and pressure directed to the right heart circulation, gradually leading to maladaptive changes that ultimately result in pulmonary hypertension. Increased right-sided blood volume and pressure causes a cascade of pathologic damage to the delicate pulmonary capillaries, causing them to be incrementally replaced with scar tissue. Scar (dead lung tissue) does not contribute to oxygen transfer, therefore decreasing the useful volume of the pulmonary vasculature. The scar tissue also provides less flexibility and compliance than normal lung tissue, causing further increases in pulmonary blood pressure, and the weakened heart must pump harder to continue supplying the lungs, leading to damage of more capillaries. It is because of this maladaptive response that at the onset of Eisenmenger's syndrome, the damage is considered irreversible, even if the underlying heart defect is corrected after the fact. Eventually, due to increased resistance and decreased compliance of the pulmonary vessels, elevated pulmonary pressures cause the myocardium of the right heart to hypertrophy (RVH). The onset of Eisenmenger's syndrome begins when right ventricular hypertrophy causes right heart pressures to exceed that of the left heart, leading to reversal of blood flow through the shunt (i.e., blood moves from the right side of the heart to the left side). As a consequence, deoxygenated blood returning from the body bypasses the lungs through the reversed shunt and proceeds directly to systemic circulation, leading to cyanosis and resultant organ damage. The defect, now a right-to-left shunt, causes reduced oxygen saturation in the arterial blood due to mixing of oxygenated blood returning from the lungs with the deoxygenated blood returning from systemic circulation. This decreased saturation is sensed by the kidneys, resulting in a compensatory increase in erythropoietin production and an increased production of red blood cells in an attempt to increase oxygen delivery. As the bone marrow increases erythropoiesis, the systemic reticulocyte count and the risk for hyperviscosity syndrome increases. Reticulocytes are less efficient at carrying oxygen as mature red cells, and they are less deformable, causing impaired transit through capillary beds. This contributes to the death of pulmonary capillary beds. A person with Eisenmenger's syndrome is paradoxically subject to the possibility of both uncontrolled bleeding due to damaged capillaries and high pressure, as well as spontaneous clots due to hyperviscosity and stasis of blood. Symptoms & signs Dyspnea upon exertion Syncope Chest pain Stroke Brain abscess Cyanosis Congestive heart failure Dysrhythmia Hyperviscosity complications Pulmonary hemorrhage/hemoptysis Endocarditis Eisenmenger syndrome is uniformly fatal; however, some patients survive into the sixth decade of life. The usual life expectancy of a patient with Eisenmenger syndrome is 20-50 years if the syndrome is diagnosed promptly and treated with vigilance. The onset of pulmonary hemorrhage is usually the hallmark of a rapid progression of the disease Diagnosis by ECG Chest X ray Echo Colour Doppler cardiac CT MRI and catheterisation studies Most patients with Eisenmenger reaction, however, are beyond the point to consider corrective therapy. Pulmonary Resistance > 10-12 wood units and Rp/Rs of greater than 0.7/1 usually indicates that corrective treatment (surgery/ catheter intervention) is associated with an increased risk for morbidity and mortality. These patients will not benefit from this approach. If however, Pulmonary resistance or Rp/Rs drops by more than 20% during exposure to oxygen and nitric oxide, medical treatment with vasoactive agents is indicated and may result in significant improvement of quality of life Treatment plan Relax the arteries to improve blood flow Keep heart beating at a regular rhythm Prevent infections (antibiotics) Prevent blood clots (blood thinners) Heart-lung transplant. This is done when the heart and lungs are failing. This is a major step that requires a sophisticated hospital and experienced surgeons. Medications are the primary treatment option for Eisenmenger syndrome. Patients to be monitored when taking medications for any changes in blood pressure, fluid volume or pulse rate. Medications for Eisenmenger syndrome include: Medications to control arrhythmias. Ironsupplements Aspirin or other blood-thinning medications. NSIAD to be avoided in ES Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. Drugs Bosentan , Ambrisentan may improve energy level and symptoms by lowering the resistance in lung arteries. For bosentan, monthly liver functions has to be monitored because the drug can damage your liver. Sildenafil and tadalafil. These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily. Side effects include upset stomach, dizziness and vision problems. Antibiotics. Antibiotics are recommended only before certain dental procedures (those that cut your gum tissue or part of the teeth) and procedures involving the respiratory tract, infected skin or tissue that connects muscle to bone. Blood drawing (phlebotomy) If red blood cell count becomes too high and is causing symptoms such as headache, difficulty concentrating or visual disturbances, Phlebotomy Heart-lung transplantation Some people who have Eisenmenger syndrome may eventually need a heart and lung transplant or a lung transplant with repair of the hole in the heart if no other treatments prove effective. Birth control and pregnancy Eisenmenger syndrome patient becoming pregnant poses serious health risks — and can be fatal — for the mother and baby. It's critical that women who have Eisenmenger syndrome avoid becoming pregnant. Tubectomy is less often recommended due to the risks of having even minor surgery. Birth control pills containing estrogen aren't recommended for women who have Eisenmenger syndrome. Estrogen increases risk of developing blood clots that could potentially block an artery to your heart, brain or lungs.Dr. Mahantesh R Charantimath9 Likes3 Answers
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10yrs.old female, Complaints of Palpitation with occasional mild dyspnoea, mentally retarded, speech rapid with embraced speech, On auscultation of heart prominent murmur audible,restless,during sleep salivation found Forceps delivery. Single child in family. Her mother fatty, father alcoholics. Desire all foods no sense of choice as such. Wants company. Dx & Rx.Dr. Arunava Chakraborty2 Likes10 Answers
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A 28 year old female presented with chronic exertional dyspnea and fatigue. PA and lateral chest radiographs and CT angiography images are given. Comment on the case.Dr. Harsh Sidona0 Like7 Answers