Eisenmenger's syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Priyamavada Singh, MBBS [3]; Kristin Feeney, B.S. [4]

Eisenmenger’s syndrome or Eisenmenger’s reaction is defined as the process in which a left-to-right shunt in the heart causes increased flow through the pulmonary vasculature, which leads to pulmonary hypertension, which finally causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. This right to left shunt causes the patient to become cyanotic. Thus, Eisenmenger’s syndrome is said to develop when there is a pulmonary artery disease, right-to-left heart shunting and cyanosis. Victor Eisenmenger first described the syndrome in 1897 in a patient who presented with dyspnea and cyanosis since infancy. Since then, advances in the medical treatment of pulmonary hypertension improved the survival of Eisenmenger’s syndrome. Eisenmenger’s syndrome can be classified according to the underlying congenital heart disease into simple and complex. Histologically, six stages were identified by Heath and Edwards, ranging from reversible to irreversible and terminal. The progression of a heart defect to Eisenmenger’s syndrome depends on the size of left to right shunt, severity of pulmonary vascular disease, and type of defect. The left-to-right shunt at the start increases the pulmonary vascular flow and leads to pulmonary artery hypertension. This causes damage to the delicate pulmonary capillaries, creating scars and fibrous tissue. This leads to hypoxemia, which is compensated by increased RBCs production, leading to polycythemia and hyperviscosity syndrome. Eventually, the building pressure in the pulmonary circulation will cause shunt reversal and development of Eisenmenger’s syndrome. Eisenmenger’s syndrome is causes by cardiac defects that shunt blood heading to the systemic circulation back into the pulmonary circulation. These defects include ventricular septal defect, atrial septal defect, atrioventricular canal defect, patent ductus arteriosus, and truncus arteriosus. Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases. The incidence and prevalence of Eisenmenger’s syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger’s syndrome. The mortality rate of Eisenmenger’s syndrome is about 27%. The risk of Eisenmenger’s syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access. The common risk factors for Eisenmenger’s syndrome include genetic mutations, exposure to rubella virus, and drug and alcohol abuse during pregnancy. Less common risk factors include poor healthcare access and patients in developing countries. There are currently no guidelines on using echocardiography to screen patients with CHD for the presence of pulmonary arterial hypertension. However, some parameters are recommended as pedictive for the development of pulmonary arterial hypertension, including peak tricuspid regurgitation velocity, ventricular basal diameter ratio, systolic and/or diastolic ventricular eccentricity index, RV outflow Doppler acceleration time, pulmonary artery diameter, and inferior vena cava inspiratory collapse. Eisenmenger’s syndrome passes through different stages, including the causative left-to-right shunt, development of pulmonary hypertension, polycythemia, and shunt reversal. The complications of Eisenmenger’s syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death. How well the infant or child does depends onwhether another medical condition is present and the age at which high blood pressure develops in the lungs. Patients with Eisenmenger’s syndrome can live 20 to 50 years. Echocardiography is the first line diagnostic modality of Eisenmenger’s syndrome. Findings on an echocardiography suggestive of Eisenmenger’s syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures. Patients with Eisenmenger’s syndrome pass through the following stages: An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart, then the development of pulmonary hypertension, then polycythemia, an increase in the number of red blood cells, and finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt. The common symptoms of Eisenmenger’s syndrome include dyspnea, syncope, hemoptysis, chest pain, dizziness, fatigue, and dyspnea. The general examination of patients with Eisenmenger’s syndrome may show cyanosis, dermal changes, clubbing, peripheral edema, and abdominal swelling. Cardiac examination may show A wave dominant jugular venous pulse, right ventricular heave, high-pitched early diastolic murmur of pulmonary insufficiency, right-sided S4, pulmonary ejection click, single palpable S₂, and loud P₂. The laboratory findings of patients with Eisenmenger’s syndrome include erythrocytosis, iron deficiency, increased bleeding time, increased blood uric acid and conjugated bilirubin concentrations, as well as mixed respiratory and metabolic acidosis. Eisenmenger’s patients may show some ECG findings, including right axis deviation (ventricular hypertrophy), ST changes (Right ventricular or biventricular hypertrophy), P Pulmonale (Right atrial hypertrophy), tall R wave in V₁ and deep S wave in V₆, and microvolt T-wave alternans. Chest X-ray of patients with Eisenmenger’s syndrome may show cardiomegaly, right ventricular or biventricular enlargement, right atrial or biatrial enlargement, and pulmonary vascular plethora. Echocardiography is the first line diagnostic modality of Eisenmenger’s syndrome. Findings on an echocardiography suggestive of Eisenmenger’s syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures. Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The findings on CT scan may include large main pulmonary artery, ventricular septal defect, and right ventricular hypertrophy. Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The following can be observed on MRI examination of Eisenmenger’s syndrome patients: Magnitude and direction of the cardiac shunt, reduced systolic function of the cardiac ventricles, and brain diffusion changes on brain MRI. Cardiac catheterization is useful in patients with Eisenmenger’s syndrome in terms of assessing the severity of pulmonary hypertension, coexisting congenital anomalies, degree and direction of shunting, ventricular function, pulmonary artery pressure and flow, and calculation of pulmonary vascular resistance. There are no other diagnostic studies associated with Eisenmenger’s syndrome. If surgical intervention is not available, treatment is mostly palliative. It includes anticoagulants, pulmonary vasodilators such as bosentan, PGE 5 inhibitor, prostacyclin, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. Congenital heart defects should undergo surgical repair before progression to Eisenmenger’s syndrome. Once the condition develops into Eisenmenger’s syndrome, no surgical cure is available except for heart and lung transplantation. However, palliative interventions (as creation of an artificial ASD) may prolong the lifespan and improve the quality of life. Surgery as early as possible to correct the heart defect can prevent Eisenmenger’s syndrome. Due to the irreversible nature of Eisenmenger’s syndrome, there are no established measures for the secondary prevention.

Victor Eisenmenger first described the syndrome in 1897 in a patient who presented with dyspnea and cyanosis since infancy. Since then, advances in the medical treatment of pulmonary hypertension improved the survival of Eisenmenger’s syndrome.

Eisenmenger’s syndrome can be classified according to the underlying congenital heart disease into simple and complex. Histologically, six stages were identified by Heath and Edwards, ranging from reversible to irreversible and terminal.

The progression of a heart defect to Eisenmenger’s syndrome depends on the size of left to right shunt, severity of pulmonary vascular disease, and type of defect. The left-to-right shunt at the start increases the pulmonary vascular flow and leads to pulmonary artery hypertension. This causes damage to the delicate pulmonary capillaries, creating scars and fibrous tissue. This leads to hypoxemia, which is compensated by increased RBCs production, leading to polycythemia and hyperviscosity syndrome. Eventually, the building pressure in the pulmonary circulation will cause shunt reversal and development of Eisenmenger’s syndrome.

Eisenmenger’s syndrome is causes by cardiac defects that shunt blood heading to the systemic circulation back into the pulmonary circulation. These defects include ventricular septal defect, atrial septal defect, atrioventricular canal defect, patent ductus arteriosus, and truncus arteriosus.

Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases.

The incidence and prevalence of Eisenmenger’s syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger’s syndrome. The mortality rate of Eisenmenger’s syndrome is about 27%. The risk of Eisenmenger’s syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access.

The common risk factors for Eisenmenger’s syndrome include genetic mutations, exposure to rubella virus, and drug and alcohol abuse during pregnancy. Less common risk factors include poor healthcare access and patients in developing countries.

There are currently no guidelines on using echocardiography to screen patients with CHD for the presence of pulmonary arterial hypertension. However, some parameters are recommended as pedictive for the development of pulmonary arterial hypertension, including peak tricuspid regurgitation velocity, ventricular basal diameter ratio, systolic and/or diastolic ventricular eccentricity index, RV outflow Doppler acceleration time, pulmonary artery diameter, and inferior vena cava inspiratory collapse.

Eisenmenger’s syndrome passes through different stages, including the causative left-to-right shunt, development of pulmonary hypertension, polycythemia, and shunt reversal. The complications of Eisenmenger’s syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death. How well the infant or child does depends onwhether another medical condition is present and the age at which high blood pressure develops in the lungs. Patients with Eisenmenger’s syndrome can live 20 to 50 years.

Echocardiography is the first line diagnostic modality of Eisenmenger’s syndrome. Findings on an echocardiography suggestive of Eisenmenger’s syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures.

Patients with Eisenmenger’s syndrome pass through the following stages: An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart, then the development of pulmonary hypertension, then polycythemia, an increase in the number of red blood cells, and finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt. The common symptoms of Eisenmenger’s syndrome include dyspnea, syncope, hemoptysis, chest pain, dizziness, fatigue, and dyspnea.

The general examination of patients with Eisenmenger’s syndrome may show cyanosis, dermal changes, clubbing, peripheral edema, and abdominal swelling. Cardiac examination may show A wave dominant jugular venous pulse, right ventricular heave, high-pitched early diastolic murmur of pulmonary insufficiency, right-sided S4, pulmonary ejection click, single palpable S₂, and loud P₂.

The laboratory findings of patients with Eisenmenger’s syndrome include erythrocytosis, iron deficiency, increased bleeding time, increased blood uric acid and conjugated bilirubin concentrations, as well as mixed respiratory and metabolic acidosis.

Eisenmenger’s patients may show some ECG findings, including right axis deviation (ventricular hypertrophy), ST changes (Right ventricular or biventricular hypertrophy), P Pulmonale (Right atrial hypertrophy), tall R wave in V₁ and deep S wave in V₆, and microvolt T-wave alternans.

Chest X-ray of patients with Eisenmenger’s syndrome may show cardiomegaly, right ventricular or biventricular enlargement, right atrial or biatrial enlargement, and pulmonary vascular plethora.

Echocardiography is the first line diagnostic modality of Eisenmenger’s syndrome. Findings on an echocardiography suggestive of Eisenmenger’s syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures.

Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The findings on CT scan may include large main pulmonary artery, ventricular septal defect, and right ventricular hypertrophy.

Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The following can be observed on MRI examination of Eisenmenger’s syndrome patients: Magnitude and direction of the cardiac shunt, reduced systolic function of the cardiac ventricles, and brain diffusion changes on brain MRI.

Cardiac catheterization is useful in patients with Eisenmenger’s syndrome in terms of assessing the severity of pulmonary hypertension, coexisting congenital anomalies, degree and direction of shunting, ventricular function, pulmonary artery pressure and flow, and calculation of pulmonary vascular resistance.

There are no other diagnostic studies associated with Eisenmenger’s syndrome.

If surgical intervention is not available, treatment is mostly palliative. It includes anticoagulants, pulmonary vasodilators such as bosentan, PGE 5 inhibitor, prostacyclin, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance.

Congenital heart defects should undergo surgical repair before progression to Eisenmenger’s syndrome. Once the condition develops into Eisenmenger’s syndrome, no surgical cure is available except for heart and lung transplantation. However, palliative interventions (as creation of an artificial ASD) may prolong the lifespan and improve the quality of life.

Surgery as early as possible to correct the heart defect can prevent Eisenmenger’s syndrome.

Due to the irreversible nature of Eisenmenger’s syndrome, there are no established measures for the secondary prevention.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Abdelrahman Ibrahim Abushouk, MD[2]

The progression of a heart defect to Eisenmenger’s syndrome depends on the size of left to right shunt, severity of pulmonary vascular disease, and type of defect. The left-to-right shunt at the start increases the pulmonary vascular flow and leads to pulmonary artery hypertension. This causes damage to the delicate pulmonary capillaries, creating scars and fibrous tissue. This leads to hypoxemia, which is compensated by increased RBCs production, leading to polycythemia and hyperviscosity syndrome. Eventually, the building pressure in the pulmonary circulation will cause shunt reversal and development of Eisenmenger’s syndrome.

• In unaffected individuals, the chambers of the left side of the heart make up a higher pressure system than the chambers of the right side of the heart.
• This is because the left ventricle has to produce enough pressure to pump blood throughout the entire body, while the right ventricle only has to produce enough pressure to pump blood to the lungs.
• Any process that increases the pressure in the left ventricle can cause worsening of the left-to-right shunt. This includes hypertension, which increases the pressure that the left ventricle has to generate in order to open the aortic valve during ventricular systole, and coronary artery disease which increases the stiffness of the left ventricle, thereby increasing the filling pressure of the left ventricle during ventricular diastole^[1].

• Eisenmenger’s syndrome can develop in many types of congenital heart diseases.
• It has been found that among all the congenital heart defects, ventricular septal defect most frequently develops Eisenmenger’s syndrome followed by atrial septal defect and patent ductus arteriosus^[2].
• The progression of a heart defect to Eisenmenger’s syndrome depends on:

• • Size of left to right shunt
  • Severity of pulmonary vascular disease.
  • Type of defect (it develops more frequently in uncorrected ventricular septal defect compared to atrial septal defect)^[3]

• The left-to-right shunting causes an increase in pulmonary vascular flow, which in turn leads to pulmonary artery hypertension.
• This leads to reversal of shunt and development of cyanosis.
• Further, the increased pressure causes damage to delicate capillaries, which then are replaced with scar tissue.
• The scar tissue does not contribute to oxygen transfer, therefore decreasing the useful volume of the pulmonary vasculature. The scar tissue also provides less flexibility than normal lung tissue, causing further increases in blood pressure^[4].
• The reduction in oxygen transfer reduces oxygen saturation in the blood, leading to increased production of red blood cells in an attempt to bring the oxygen saturation up. The excess of red blood cells is called polycythemia.
• Desperate for enough circulating oxygen, the body begins to dump immature red cells into the blood stream. Immature red cells are not as efficient at carrying oxygen as mature red cells, and they are less flexible, less able to easily squeeze through tiny capillaries in the lungs, and so contribute to death of pulmonary capillary beds. The increase in red blood cells also causes hyperviscosity syndrome.
• A person with Eisenmenger’s Syndrome is paradoxically subject to the possibility of both uncontrolled bleeding due to damaged capillaries and high pressure, and random clots due to hyperviscosity and stasis of blood.
• Eventually, due to increased resistance, pulmonary pressures may increase sufficiently to cause a reversal of blood flow, so blood begins to travel from the right side of the heart to the left side, and the body is supplied with deoxygenated blood, leading to cyanosis and resultant organ damage^[5].

• Eisenmenger’s Syndrome is not currently identified as an inherited disorder.

• Specific genes that cause Eisenmenger’s syndrome have not been identified so far.

Conditions associated with Eisenmenger’s syndrome include:

• Causative congenital heart diseases.
• Tricuspid and pulmonary valve regurgitation
• Atherosclerosis

On gross pathology, Eisenmenger’s syndrome may show the following^[6]:

• Atrial or ventricular septal defect.
• Right ventricular hypertrophy
• Pulmonary trunk dilatation
• Atherosclerosis of pulmonary artery and its branches.
• Tricuspid regurgitation may be seen.

According to the histopathologic criteria of Heath and Edwards, there are six stages of pulmonary vascular disease (including Eisenmenger’s syndrome)^[7]:

• Stage I: Medial hypertrophy (reversible)
• Stage II: Cellular intimal hyperplasia in an abnormally muscular artery (reversible)
• Stage III: Lumen occlusion from intimal hyperplasia of fibroelastic tissue (partially reversible)
• Stage IV: Arteriolar dilatation and medial thinning (irreversible)
• Stage V: Plexiform lesion, which is an angiomatoid formation (terminal and irreversible)
• Stage VI: Fibrinoid/necrotizing arteritis (terminal and irreversible)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

The incidence and prevalence of Eisenmenger’s syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger’s syndrome. The mortality rate of Eisenmenger’s syndrome is about 27%. The risk of Eisenmenger’s syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access.

• The incidence of Eisenmenger’s syndrome decreased from 2.5/million individuals/year in 1977 to 0.2/million individuals/year in 2012.^[1]

• Overall, about 8% of patients with congenital heart disease develop Eisenmenger’s syndrome.^[2]
• About 3% of patients with a small VSD (≤1.5 cm) and 50% of patients with a large VSD (>1.5 cm) develop Eisenmenger’s syndrome.^[3]
• However, the prevalence is currently decreasing due to improved detection methods of congenital heart defects.
• In the general population, the prevalence decreased from 24.6 to 11.9/million individuals in 2012.^[4]

• At follow-up of 6 years, the mortality rate of Eisenmenger’s syndrome was 27%.^[5]
• At 3, 6 and 9 years from diagnosis, the mortality rates were 27%, 50%, and 53%, respectively.^[6]
• Death usually occurs between 30 and 35 years old.

• Eisenmenger’s syndrome usually manifests before puberty.
• But it may also manifest during adolescence and early adulthood.^[7]

• There is no racial predilection to Eisenmenger’s syndrome.

• Eisenmenger’s syndrome affects men and women equally.

The prevalence of Eisenmenger’s syndrome is less common in developed countries due to better healthcare access.

The prevalence of Eisenmenger’s syndrome is more common in developing countries due to poor healthcare access.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Eisenmenger’s syndrome passes through different stages, including the causative left-to-right shunt, development of pulmonary hypertension, polycythemia, and shunt reversal. The complications of Eisenmenger’s syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death. How well the infant or child does depends onwhether another medical condition is present and the age at which high blood pressure develops in the lungs. Patients with Eisenmenger’s syndrome can live 20 to 50 years.

Patients with Eisenmenger’s syndrome pass through the following stages^[1]:

• An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart.
• The development of pulmonary hypertension.
• Polycythemia, an increase in the number of red blood cells.
• Finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt.
• At the final stage, the patient is liable to the complications mentioned below.

Therefore, they may present with a history of symptoms for each of these stages.

The complications of Eisenmenger’s syndrome include^[2][3]:

• Intracranial hemorrhage
• Stroke
• Congestive heart failure
• Angina Pectoris
• Hyperviscosity syndrome
• Infection (cerebral abscess)
• Renal failure
• High risk pregnancy and fetal loss.
• Sudden death

• How well the infant or child does depends on^[4]:
  • whether another medical condition is present
  • the age at which high blood pressure develops in the lungs.
• Patients with Eisenmenger’s syndrome can live 20 to 50 years.
• The predictors of mortality in Eisenmenger’s syndrome include^[5]:
  • Age
  • Pretricuspid shunt
  • Oxygen saturation at rest
  • Presence of sinus rhythm
  • Presence of pericardial effusion

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Eisenmenger’s syndrome is causes by cardiac defects that shunt blood heading to the systemic circulation back into the pulmonary circulation. These defects include ventricular septal defect, atrial septal defect, atrioventricular canal defect, patent ductus arteriosus, and truncus arteriosus.

Eisenmenger’s syndrome is caused by a defect in the heart, which allows oxygenated blood to flow back into the pulmonary circulation, instead of going out to the systemic circulation. Cardiac defects that can lead to Eisenmenger’s syndrome include ^[1][2][3]:

• Atrioventricular canal defect
• Atrial septal defect
• Cyanotic heart disease
• Truncus arteriosus

• Unrepaired Tetralogy of Fallot
• Patent ductus arteriosus
• Surgical systemic-to-pulmonary anastomosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases.

Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases^[1][2][3][4].

Disorders	Etiology	Clinical Presentation	Laboratory Findings	Electrocardiogram	Echocardiography
Idiopathic Pulmonary Hypertension	Unknown, but possible reasons may include Connective tissue disease Liver cirrhosis Anorexic and alpha-adrenergic stimulants [cocaine/amphetamine] HIV infection	Dyspnea Recurrent syncope Fatigue Chest pain Cough Hemoptysis	+ve ANA assay +ve ANCA Anti-topoisomerase antibody Thyrotropin: screen thyroid disorders Elevated BNP & N-terminal BNP.	Right axis deviation Right ventricular hypertrophy ST depression T-wave inversion	Flattening of the intraventricular septum during systole & diastole Right ventricular hypertrophy Reduced right ventricular function Tricuspid regurgitation
Respiratory Failure	Respiratory: COPD, Pneumonia, Pulmonary embolism, Pulmonary hypertension Nervous: Guillain-Barré syndrome, muscular dystrophy, and myasthenia gravis Cardiac: Congenital heart diseases	Cyanosis Dyspnea Tachypnea Tachycardia Confusion Asterixis	Arterial blood gases (ABG) may detect hypoxemia, hypercapnea, and acidosis Pulmonary function tests	Electrocardiography may be used to evaluate the underlying cardiac causes of respiratory failure.	Echocardiography may be used to evaluate the underlying cardiac causes of respiratory failure. Left ventricular dilatation Regional wall motion abnormalities Mitral regurgitation
Pulmonary Infarction	Cardiac diseases Inherited coagulation disorders Sickle cell anemia Neoplasm Dehydration Surgery	Tachypnea Dyspnea Pleuritic chest pain Cough Hemoptysis Syncope Sweating	ABG: Hypoxemia, hypercapnea, and metabolic acidosis Elevated D-Dimer levels CBC: High WBCs Hypercoagulation panel	Sinus tachycardia T-wave inversion Right axis deviation Right bundle branch block P pulmonale	The main diagnostic studies are CT pulmonary angiography Ventilation/perfusion scanning. However, echocardiography may diagnose underlying cardiac conditions.
Tetralogy of Fallot	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Cyanosis on exertion Exertional dyspnea Palpitations Fatigue	CBC: Anemia or polycythemia. Coagulation profile. Arterial blood gas: Low oxygen saturation and acidosis	Right ventricular hypertrophy Right bundle branch block Tachycardia Rate of QRS change predicts ventricular arrhythmia	Echocardiography may show: Residual VSD or ASD RV outflow tract obstruction Abnormal valvular anatomy
Total Anomalous Pulmonary Venous Connection	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Tachypnea Palpitations Cyanosis Failure to thrive	Arterial blood gas: Low oxygen saturation and acidosis Coagulation profile.	Right ventricular hypertrophy with a qR pattern	Right ventricular hypertrophy Right ventricular loading Paradoxical septal motion
Tricuspid Atresia	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Respiratory difficulties as nasal flaring or muscle retractions Cyanosis Growth retradation	Arterial blood gases CBC: Polycythemia Coagulation profile	Tall P waves indicate atrial enlargement. First-degree atrioventricular block. Frontal plane QRS axis may be leftward.	Echocardiography may show Defect size Pulmonary blood flow Ventricular function Valve abnormalities
Ventricular Septal Defect	Multifactorial Genetic polymorphisms Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Asymptomatic: small VSD Excessive sweating Fatigue during feeding Failure to thrive	High troponin I and amino-terminal procollagen type III peptide High BNP and pro-BNP levels	Moderate VSD: Left ventricular hypertrophy Large VSD: Right ventricular hypertrophy Biphasic P wave: Left atrial dilatation	Echocardiography may show Defect size Pulmonary blood flow Ventricular function Valve abnormalities
Transposition of the Great Arteries	Multifactorial Genetic polymorphisms Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Prominent cyanosis within hours of birth Congestive heart failure	Arterial blood gases: Hypoxemia Hyperoxia test	Right ventricular hypertrophy Right axis deviation Varying degrees of AV block Q waves	Echocardiography may show: Relationship between great vessels Associated anatomic lesions Coronary artery origin and branches
Persistent truncus arteriosus	Genetic disorders Teratogens (viruses, metabolic imbalance, and industrial agents)	Cyanosis Growth failure Fatigue Poor feeding Tachypnea Dyspnea	Arterial blood gas for acidosis and oxygen saturation Hypocalcemia if associated with Digeorge syndrome	Right axis deviation Biventricular hypertrophy	Echocardiography may show Large truncal artery overriding the VSD Pulmonary blood flow Tricuspid regurgitation

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