Eisenmenger Complex
Hypertension, Pulmonary
Aerospace Medicine
Pregnancy Complications, Cardiovascular
Aircraft
Heart Septal Defects, Ventricular
Echocardiographic and morphological correlations in tetralogy of Fallot. (1/77)
AIMS: Our aim was to clarify the location and structure of the outlet septum relative to the free-standing subpulmonary infundibulum in the setting of tetralogy of Fallot and to examine its relationship to the other components of the subpulmonary outflow tract, determining their potential influence on clinical outcome. METHODS AND RESULTS: We studied prospectively 41 patients with tetralogy of Fallot (mean age 14 +/- 10.9 months) prior to surgical repair, and compared them with 15 patients undergoing closure of a ventricular septal defect associated with malalignment of the outlet septum but no subpulmonary infundibular stenosis (Eisenmenger ventricular septal defect), and 20 healthy controls. We also examined available autopsied hearts from cases with uncorrected tetralogy of Fallot (8) and Eisenmenger ventricular septal defect (13). Data were indexed for body surface area, and diameter of the tricuspid valve, respectively. The overall length of the subpulmonary infundibulum, including the extent of the muscular outlet septum, was significantly greater for patients with tetralogy of Fallot compared to normals (2.34 +/- 0.6 vs 1.46 +/- 0.34 cm/BSA0.5, P<0.001), whereas the difference between those with tetralogy of Fallot and an Eisenmenger ventricular septal defect was confined to the degree of narrowing of the subpulmonary outlet (0.43 +/- 0.22 vs 2.17 +/- 0.64 cm/BSA0.5, P<0.001). Within the tetralogy of Fallot group, there were linear relationships between deviation of the outlet septum (r= -0.61, P<0.005) and the diameter of the pulmonary valvar orifice (r=0.75, P<0.001), suggesting that growth of the pulmonary arteries may be related to this feature. When patients requiring a transannular patch as part of their surgical repair were compared with those not needing this procedure, differences were found in the diameter of the pulmonary valvar orifice and the pulmonary trunk, but not in the dimensions of the outlet septum. CONCLUSION: The position of the outlet septum in relationship to the remainder of the muscular subpulmonary infundibulum represents a hallmark of tetralogy of Fallot, permitting its differentiation from Eisenmenger ventricular septal defects and normal hearts. (+info)Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. (2/77)
OBJECTIVES: Morbidity and mortality patterns were characterized in adults with the Eisenmenger syndrome when two ventricles with a ventricular septal defect (VSD) joined two great arteries or one great artery, or when one ventricle joined two great arteries. BACKGROUND: Although afterload in these disorders differs, clinical differences have not been defined. METHODS: Seventy-seven patients were studied. Group A comprised 47 patients with VSD, aged 23 to 69 years (mean 39.5+/-10.2), follow-up 5 to 18 years (mean 7.2+/-4.9); group B, 14 patients with truncus arteriosus, aged 27 to 50 years (mean 33.7+/-7.3), follow-up 6 to 18 years (mean 7.7+/-5.1), and group C, 16 patients with univentricular heart, aged 18 to 44 years (mean 30.6+/-8.4), follow-up 5 to 15 years (mean 4.4+/-4.2). Echocardiography established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, noncardiac surgery and the multisystem disorders of cyanotic adults. RESULTS: Thirty-five percent of the patients died. Sixty-three percent of deaths were sudden, and resulted from intrapulmonary hemorrhage, rupture of either the pulmonary trunk, ascending aorta or a bronchial artery, or vasospastic cerebral infarction, or the cause was unestablished. There were no documented tachyarrhythmic sudden deaths. CONCLUSIONS: Medical management of coexisting cardiac disease, multisystem systemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurysms, thromboses and calcification; to truncal valve stenosis and regurgitation; to semilunar and atrioventricular valve regurgitation, and to major risks of nontachyarrhythmic sudden death. (+info)Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. (3/77)
OBJECTIVE: To assess haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. DESIGN: Retrospective observational study. SETTING: Tertiary referral centre. PATIENTS: 94 adult patients with Eisenmenger syndrome who were diagnosed between September 1982 and July 1998. MAIN OUTCOME MEASURES: Serum uric acid was measured in all patients, together with clinical and haemodynamic variables related to mortality. RESULTS: Serum uric acid was raised in patients with Eisenmenger syndrome compared with age and sex matched control subjects (7.0 v 4.7 mg/dl, p < 0.0001) and increased in proportion to the severity of New York Heart Association functional class. Serum uric acid was positively correlated with mean pulmonary arterial pressure (r = 0.30, p = 0.0052) and total pulmonary resistance index (r = 0.55, p < 0.0001), and negatively correlated with cardiac index (r = -0.50, p < 0.0001). During a mean follow up period of 97 months, 38 patients died of cardiopulmonary causes. Among various clinical, echocardiographic, and laboratory variables, serum uric acid remained predictive in multivariate analysis. Kaplan-Meier survival curves based on median serum uric acid showed that patients with high values had a significantly worse survival rate than those with low values (log-lank test: p = 0.0014 in male patients, p = 0.0034 in female patients). CONCLUSIONS: Serum uric acid increases in proportion to haemodynamic severity in adult patients with Eisenmenger syndrome and is independently associated with long term mortality. (+info)Eisenmenger syndrome in pregnancy. (4/77)
Maternal mortality in the presence of Eisenmenger syndrome is reported to be 30 to 50% & increases further with associated complications. A case of Eisenmenger syndrome in pregnancy where the patient progressively deteriorated postpartum & expired 3 weeks later is reported. (+info)Xenon anaesthesia for laparoscopic cholecystectomy in a patient with Eisenmenger's syndrome. (5/77)
There are few reports on anaesthesia for patients with Eisenmenger's syndrome requiring non-cardiac surgery and none of the use of xenon. We describe the use of xenon with a closed-circuit system in a patient with Eisenmenger's syndrome having a laparoscopic cholecystectomy. (+info)Incremental spinal anaesthesia for elective Caesarean section in a patient with Eisenmenger's syndrome. (6/77)
We describe a new approach to anaesthesia for elective Caesarean section in a woman with Eisenmenger's syndrome. Incremental regional anaesthesia was performed using a microspinal catheter and haemodynamic monitoring included transthoracic bioimpedance cardiography. This approach allowed the disadvantages of general anaesthesia and invasive cardiac output monitoring to be avoided. (+info)Residual pulmonary vasoreactivity to inhaled nitric oxide in patients with severe obstructive pulmonary hypertension and Eisenmenger syndrome. (7/77)
OBJECTIVE: To determine whether inhaled NO (iNO) can reduce pulmonary vascular resistance in adults with congenital heart disease and obstructive pulmonary hypertension or Eisenmenger syndrome. DESIGN: 23 patients received graded doses of iNO. Pulmonary and systemic haemodynamic variables and circulating cyclic guanosine monophosphate (cGMP) concentrations were measured at baseline and after 20 and 80 ppm iNO. Patients were considered responders when total pulmonary resistance was reduced by at least 20%, and rebound was defined as a greater than 10% increase in total pulmonary resistance upon withdrawal from iNO. RESULTS: In response to 20 ppm iNO, total pulmonary resistance decreased in four patients (18%, 95% confidence interval (CI), 2% to 34%), while in response to 80 ppm iNO it decreased in six patients (29%, 95% CI 10% to 38%). Systemic blood pressure did not change. Withdrawal resulted in rebound in three patients (16%, 95% CI 0% to 32%) after cessation of 20 ppm iNO, and in six patients (35%, 95% CI 12% to 58%) after cessation of 80 ppm iNO. Patients with predominant right to left shunting did not respond. In all patients cGMP increased from (mean (SD)) 28 (13) micromol/l at baseline to 55 (30) and 78 (44) micromol/l after 20 and 80 ppm iNO (p < 0.05 v baseline). CONCLUSIONS: NO inhalation is safe and is associated with a dose dependent increase in circulating cGMP concentrations. Pulmonary vasodilatation in response to iNO was observed in 29% of patients and was influenced by baseline pulmonary haemodynamics. Responsiveness to acute iNO may identify patients with advanced obstructive pulmonary hypertension and Eisenmenger syndrome who could benefit from sustained vasodilator treatment. (+info)Long term treatment of pulmonary arterial hypertension with beraprost, an oral prostacyclin analogue. (8/77)
OBJECTIVE: To evaluate the effects of one year's treatment with beraprost, an orally active prostacyclin analogue, in patients with severe pulmonary hypertension. PATIENTS: 13 patients with severe pulmonary hypertension. This was primary in nine, thromboembolic in three, and caused by Eisenmenger syndrome in one. METHODS: All patients underwent right heart catheterisation. Mean (SD) right atrial pressure was 5 (3) mm Hg, mean pulmonary artery pressure was 48 (12) mm Hg, cardiac index was 2.6 (0.8) l/min/m(2), and mixed venous oxygen saturation was 68 (7)%. Beraprost was started at the dose of 20 microgram three to four times a day (1 microgram/kg/day), increasing after one month to 40 microgram three to four times a day (2 microgram/kg/day), with further increases of 20 microgram three to four times a day in case of clinical deterioration. MAIN OUTCOME MEASURES: New York Heart Association (NYHA) functional class, exercise capacity measured by distance walked in six minutes, and systolic pulmonary pressure (by echocardiography) were evaluated at baseline, after one month's treatment, and then every three months for a year. RESULTS: After the first month of treatment, NYHA class decreased from 3.4 (0.7) to 2.9 (0.7) (p < 0.05), the six minute walking distance increased from 213 (64) to 276 (101) m (p < 0.05), and systolic pulmonary artery pressure decreased from 93 (15) to 85 (18) mm Hg (NS). One patient died after 40 days from refractory right heart failure, and another was lost for follow up at six months. The 11 remaining patients had persistent improvements in functional class and exercise capacity and a significant decrease in systolic pulmonary artery pressure in the period from 1-12 months. Side effects were minor. CONCLUSIONS: Oral administration of beraprost may result in long lasting clinical and haemodynamic improvements in patients with severe pulmonary hypertension. (+info)Eisenmenger Complex is a term used in cardiology to describe a congenital heart defect characterized by the presence of a large ventricular septal defect (a hole in the wall between the two lower chambers of the heart) or a patent ductus arteriosus (an abnormal blood vessel connecting the pulmonary artery and the aorta) along with severe pulmonary hypertension.
In this condition, the high pressure in the pulmonary arteries leads to reversal of blood flow from the lungs to the rest of the body, resulting in cyanosis (bluish discoloration of the skin and mucous membranes due to lack of oxygen in the blood) and other symptoms such as shortness of breath, fatigue, and digital clubbing.
The name "Eisenmenger Complex" comes from the German physician Victor Eisenmenger, who first described the condition in 1897. It is a severe and life-threatening congenital heart defect that typically requires surgical intervention to improve symptoms and prolong survival.
Pulmonary hypertension is a medical condition characterized by increased blood pressure in the pulmonary arteries, which are the blood vessels that carry blood from the right side of the heart to the lungs. This results in higher than normal pressures in the pulmonary circulation and can lead to various symptoms and complications.
Pulmonary hypertension is typically defined as a mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest, as measured by right heart catheterization. The World Health Organization (WHO) classifies pulmonary hypertension into five groups based on the underlying cause:
1. Pulmonary arterial hypertension (PAH): This group includes idiopathic PAH, heritable PAH, drug-induced PAH, and associated PAH due to conditions such as connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, and schistosomiasis.
2. Pulmonary hypertension due to left heart disease: This group includes conditions that cause elevated left atrial pressure, such as left ventricular systolic or diastolic dysfunction, valvular heart disease, and congenital cardiovascular shunts.
3. Pulmonary hypertension due to lung diseases and/or hypoxia: This group includes chronic obstructive pulmonary disease (COPD), interstitial lung disease, sleep-disordered breathing, alveolar hypoventilation disorders, and high altitude exposure.
4. Chronic thromboembolic pulmonary hypertension (CTEPH): This group includes persistent obstruction of the pulmonary arteries due to organized thrombi or emboli.
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms: This group includes hematologic disorders, systemic disorders, metabolic disorders, and other conditions that can cause pulmonary hypertension but do not fit into the previous groups.
Symptoms of pulmonary hypertension may include shortness of breath, fatigue, chest pain, lightheadedness, and syncope (fainting). Diagnosis typically involves a combination of medical history, physical examination, imaging studies, and invasive testing such as right heart catheterization. Treatment depends on the underlying cause but may include medications, oxygen therapy, pulmonary rehabilitation, and, in some cases, surgical intervention.
Aerospace medicine is a branch of medicine that deals with the health and safety of pilots, astronauts, and passengers during space travel or aircraft flight. It involves studying the effects of various factors such as altitude, weightlessness, radiation, noise, vibration, and temperature extremes on the human body, and developing measures to prevent or mitigate any adverse effects.
Aerospace medicine also encompasses the diagnosis and treatment of medical conditions that occur during space travel or aircraft flight, as well as the development of medical standards and guidelines for pilot and astronaut selection, training, and fitness for duty. Additionally, it includes research into the physiological and psychological challenges of long-duration space missions and the development of countermeasures to maintain crew health and performance during such missions.
Cardiovascular complications in pregnancy refer to conditions that affect the heart and blood vessels, which can arise during pregnancy, childbirth, or after delivery. These complications can be pre-existing or new-onset and can range from mild to severe, potentially threatening the life of both the mother and the fetus. Some examples of cardiovascular complications in pregnancy include:
1. Hypertension disorders: This includes chronic hypertension (high blood pressure before pregnancy), gestational hypertension (high blood pressure that develops after 20 weeks of pregnancy), and preeclampsia/eclampsia (a pregnancy-specific disorder characterized by high blood pressure, proteinuria, and potential organ damage).
2. Cardiomyopathy: A condition in which the heart muscle becomes weakened, leading to an enlarged heart and reduced pumping efficiency. Peripartum cardiomyopathy is a specific type that occurs during pregnancy or in the months following delivery.
3. Arrhythmias: Irregularities in the heart's rhythm, such as tachycardia (rapid heartbeat) or bradycardia (slow heartbeat), can occur during pregnancy and may require medical intervention.
4. Valvular heart disease: Pre-existing valve disorders, like mitral stenosis or aortic insufficiency, can worsen during pregnancy due to increased blood volume and cardiac output. Additionally, new valve issues might develop during pregnancy.
5. Venous thromboembolism (VTE): Pregnancy increases the risk of developing blood clots in the veins, particularly deep vein thrombosis (DVT) or pulmonary embolism (PE).
6. Ischemic heart disease: Although rare, coronary artery disease and acute coronary syndrome can occur during pregnancy, especially in women with risk factors such as obesity, diabetes, or smoking history.
7. Heart failure: Severe cardiac dysfunction leading to fluid accumulation, shortness of breath, and reduced exercise tolerance may develop due to any of the above conditions or other underlying heart diseases.
Early recognition, monitoring, and appropriate management of these cardiovascular complications in pregnancy are crucial for maternal and fetal well-being.
An "aircraft" is not a medical term, but rather a general term used to describe any vehicle or machine designed to be powered and operated in the air. This includes fixed-wing aircraft such as airplanes and gliders, as well as rotary-wing aircraft such as helicopters and autogyros.
However, there are some medical conditions that can affect a person's ability to safely operate an aircraft, such as certain cardiovascular or neurological disorders. In these cases, the individual may be required to undergo medical evaluation and obtain clearance from aviation medical examiners before they are allowed to fly.
Additionally, there are some medical devices and equipment that are used in aircraft, such as oxygen systems and medical evacuation equipment. These may be used to provide medical care to passengers or crew members during flight.
A ventricular septal defect (VSD) is a type of congenital heart defect that involves a hole in the wall separating the two lower chambers of the heart, the ventricles. This defect allows oxygenated blood from the left ventricle to mix with deoxygenated blood in the right ventricle, leading to inefficient oxygenation of the body's tissues. The size and location of the hole can vary, and symptoms may range from none to severe, depending on the size of the defect and the amount of blood that is able to shunt between the ventricles. Small VSDs may close on their own over time, while larger defects usually require medical intervention, such as medication or surgery, to prevent complications like pulmonary hypertension and heart failure.