Aortopulmonary window | |
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Other names | Aortopulmonary septal defect [1] |
A chest X-ray showing an Aortopulmonary window. | |
Specialty | Medical genetics |
Symptoms | Tachypnea, poor eating, left-to-right shunt, and diaphoresis. [2] |
Complications | Heart murmurs, eisenmenger syndrome, and heart failure. [2] |
Usual onset | Birth |
Diagnostic method | Physical examination findings, ECG, and imaging. [2] |
Differential diagnosis | Truncus arteriosus, ventricular septal defect, and patent ductus arteriosus. [3] |
Treatment | Heart surgery. [2] |
Prognosis | 40% chance of death within the first year if left untreated. [4] |
Frequency | 0.15-0.6% of all congenital heart malformations. [4] |
Aortopulmonary window (APW) is a faulty connection between the aorta and the main pulmonary artery that results in a significant left-to-right shunt. [2] The aortopulmonary window is the rarest of septal defects, accounting for 0.15-0.6% of all congenital heart malformations. [4] An aortopulmonary window can develop alone or in up to 50% of cases alongside other cardiac defects such as interrupted aortic arch, coarctation of the aorta, transposition of great vessels, and tetralogy of Fallot. [3]
A left-to-right shunt can cause heart failure, with symptoms such as tachypnea, poor eating, and diaphoresis. Dyspnea and indications of laborious breathing can be caused by low lung compliance and increased airway resistance. Infants may have failure to thrive as well as recurrent pneumonia. [2]
Findings among individuals with an isolated aortopulmonary window vary based on the size of the defect and the pulmonary vascular resistance. Cardiac examination typically indicates a parasternal lift resulting from right ventricular overload, a loud single second heart sound induced by pulmonary hypertension, and increased peripheral pulses. [2]
When the defect is larger, pulmonary vascular resistance may continue to be elevated in the initial weeks or months of life, and there is a modest amount of pulmonary overcirculation. A rather faint basal systolic ejection murmur without a diastolic element and a loud single second heart sound develop due to mild overcirculation. As the pulmonary vascular resistance decreases throughout the first few months, the left-to-right shunting of blood into the lungs increases, and the systolic murmur becomes more intense and longer, eventually extending into diastole and becoming a continuous murmur. [2]
Because there is a pressure gradient from the aorta to the pulmonary artery throughout systole and diastole, a small aortopulmonary defect may produce a murmur similar to a patent ductus arteriosus. [2]
When a large defect is left unrepaired, Eisenmenger syndrome will develop with reversal of the shunt. [2]
Untreated cases with major malformations have a poor prognosis, with 40% dying within their first year of life. [4]
Physical examination findings, ECG, and imaging are used to diagnose aortopulmonary window. An electrocardiogram reveals right ventricular hypertrophy or biventricular hypertrophy. Cardiomegaly, a big main pulmonary artery segment, and enhanced pulmonary vascular marking are all visible on a chest x-ray. [2]
The appearance of aortopulmonary window has similarities to that of truncus arteriosus, ventricular septal defect, and large patent ductus arteriosus. A two-dimensional echocardiogram can detect and distinguish between these problems. [3]
Corrective heart surgery, which is normally performed in the first year of life, is the definitive intervention for an aortopulmonary window. If the patient's symptoms don't allow for corrective surgery, medical therapy of congestive heart failure is the second choice. Permanent alterations in the pulmonary vasculature can be prevented with early intervention. [2]
Heart sounds are the noises generated by the beating heart and the resultant flow of blood through it. Specifically, the sounds reflect the turbulence created when the heart valves snap shut. In cardiac auscultation, an examiner may use a stethoscope to listen for these unique and distinct sounds that provide important auditory data regarding the condition of the heart.
Heart murmurs are unique heart sounds produced when blood flows across a heart valve or blood vessel. This occurs when turbulent blood flow creates a sound loud enough to hear with a stethoscope. Turbulent blood flow is not smooth. The sound differs from normal heart sounds by their characteristics. For example, heart murmurs may have a distinct pitch, duration and timing. The major way health care providers examine the heart on physical exam is heart auscultation; another clinical technique is palpation, which can detect by touch when such turbulence causes the vibrations called cardiac thrill. A murmur is a sign found during the cardiac exam. Murmurs are of various types and are important in the detection of cardiac and valvular pathologies.
Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:
Patent ductus arteriosus (PDA) is a medical condition in which the ductus arteriosus fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs through the aorta, which has a higher blood pressure, to the pulmonary artery, which has a lower blood pressure. Symptoms are uncommon at birth and shortly thereafter, but later in the first year of life there is often the onset of an increased work of breathing and failure to gain weight at a normal rate. With time, an uncorrected PDA usually leads to pulmonary hypertension followed by right-sided heart failure.
dextro-Transposition of the great arteries is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.
The ductus arteriosus, also called the ductus Botalli, named after the Italian physiologist Leonardo Botallo, is a blood vessel in the developing fetus connecting the trunk of the pulmonary artery to the proximal descending aorta. It allows most of the blood from the right ventricle to bypass the fetus's fluid-filled non-functioning lungs. Upon closure at birth, it becomes the ligamentum arteriosum.
An acyanotic heart defect, is a class of congenital heart defects. In these, blood is shunted (flows) from the left side of the heart to the right side of the heart, most often due to a structural defect (hole) in the interventricular septum. People often retain normal levels of oxyhemoglobin saturation in systemic circulation.
Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates.
Persistent truncus arteriosus (PTA), often referred to simply as truncus arteriosus, is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation. For the International Classification of Diseases (ICD-11), the International Paediatric and Congenital Cardiac Code (IPCCC) was developed to standardize the nomenclature of congenital heart disease. Under this system, English is now the official language, and persistent truncus arteriosus should properly be termed common arterial trunk.
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.
Levo-Transposition of the great arteries is an acyanotic congenital heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed.
Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood.
A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles.
The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.
Aortopulmonary septal defect is a rare congenital heart disorder accounting for only 0.1-0.3% of congenital heart defects worldwide. It is characterized by a communication between the aortic and pulmonary arteries, with preservation of two normal semilunar valves. It is the result of an incomplete separation of the aorticopulmonary trunk that normally occurs in early fetal development with formation of the spiral septum. Aortopulmonary septal defects occur in isolation in about half of cases, the remainder are associated with more complex heart abnormalities.
Anomalous pulmonary venous connection is a congenital defect of the pulmonary veins.
Heart murmurs are most frequently organized by timing, into systolic heart murmurs and diastolic heart murmurs. However, continuous murmurs can not be directly placed into either category.
The Yasui procedure is a pediatric heart operation used to bypass the left ventricular outflow tract (LVOT) that combines the aortic repair of the Norwood procedure and a shunt similar to that used in the Rastelli procedure in a single operation. It is used to repair defects that result in the physiology of hypoplastic left heart syndrome even though both ventricles are functioning normally. These defects are common in DiGeorge syndrome and include interrupted aortic arch and LVOT obstruction (IAA/LVOTO); aortic atresia-severe stenosis with ventricular septal defect (AA/VSD); and aortic atresia with interrupted aortic arch and aortopulmonary window. This procedure allows the surgeon to keep the left ventricle connected to the systemic circulation while using the pulmonary valve as its outflow valve, by connecting them through the ventricular septal defect. The Yasui procedure includes a modified Damus–Kaye–Stansel procedure to connect the aortic and pulmonary roots, allowing the coronary arteries to remain perfused. It was first described in 1987.
Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract.