Pulmonary artery agenesis

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The main pulmonary trunk divides into the left and the right pulmonary artery. Pulmonary artery agenesis refers to the absence of one or both pulmonary arteries. Illu pulmonary circuit.jpg
The main pulmonary trunk divides into the left and the right pulmonary artery. Pulmonary artery agenesis refers to the absence of one or both pulmonary arteries.

Pulmonary artery agenesis refers to a rare congenital absence of pulmonary artery due to a malformation in the sixth aortic arch. It can occur bilaterally, with both left and right pulmonary arteries being absent, or unilaterally, the absence of either left or right pulmonary artery (UAPA). About 67% of UAPA occurs isolated in the right lung. [1] The absence of pulmonary artery can be an isolated disorder, or accompanied by other related lesions, [2] most commonly Tetralogy of Fallot. [3]

Contents

Back in 1868, Fraentzel was the first to report isolated unilateral absence of pulmonary artery (IUAPA) in literature. [4] [5] Subsequently, literature has documented a total of 420 cases. [6] The estimated prevalence of IUAPA is 1 in 200,000 adults. [2] No sex preference is observed. [6] Patients with severe complications are usually diagnosed early in age while adult patients are mainly asymptomatic. The overall mortality rate reaches 7%. [7]

Individuals may exhibit a variety of symptoms, or they may not exhibit any symptoms at all. Recurrent lung infections and exercise intolerance are some of the most common symptoms. [8] Serious complications include hemoptysis and pulmonary hypertension . These non-specific symptoms make UAPA challenging to diagnose. [9] Multiple medical imaging techniques are often employed in combination in order to obtain a comprehensive diagnosis. [8]

Cause

Pulmonary artery agenesis refers to the absence or maldevelopment of one or both pulmonary arteries in foetal development. This rare congenital condition arises from abnormal maturation of the sixth aortic arch during embryogenesis. [2] In embryonic growth, the primordial truncus arteriosus is divided into aorta and pulmonary trunk by septation. [10] Any disruptions in septation may cause the failure of the development of the branch pulmonary artery from the sixth aortic arch. The exact pathogenesis is not fully understood. [11]

Mortality rate and cause of death

The overall mortality rate of UAPA in all patients is about 7%. [7] Newborns with respiratory distress and severe pulmonary hypertension have poor outcomes. [12] 30% of patients remain asymptomatic throughout adult life. [5] [8] Delayed diagnosis and follow-up medical interventions may lead to lung hypoplasia. [8]

Pulmonary haemorrhage, recurrent infections and pulmonary hypertension may hinder the possibility of long-term survival. [12] Survival is probable until patients reach their sixties. [13] Common causes of death include right heart failure, respiratory failure, massive pulmonary hemorrhage and high-altitude pulmonary edema. [6]

Signs and symptoms

40% of UAPA patients exhibit symptoms of exercise intolerance or dyspnea during exertion. [14] Other common symptoms include hemoptysis in 20% of patients, chest pain, pleural effusion or recurrent pulmonary infections. [15] UAPA may rarely cause the development of severe, life-threatening hemoptysis. [16] [17] Pulmonary hypertension is another potentially fatal condition that affects 20% of patients. [18] IUAPA patients with no associated cardiac anomalies might remain largely asymptomatic into adulthood. [15] It has been noted that individuals who have a unilateral absence of the right pulmonary artery in particular are more vulnerable to high-altitude pulmonary edema. [19]

Hemoptysis

In IUAPA patients, collateral arteries and shunts are developed in the affected lung from the systemic network. The collateral network supplies the pulmonary blood from the heart to the affected lung in place of the absent pulmonary artery. [11] Hemoptysis occurs when the thin walls of the extensive systemic collateral network rupture. The occurrence of hemoptysis may resolve on its own and persist without intervention for many years. However, it might also lead to severe pulmonary haemorrhage and fatal outcomes. [8]

Chest X ray showing lung hypoplasia on the right side. Pulmonaryhypoplasia.jpg
Chest X ray showing lung hypoplasia on the right side.

Lung hypoplasia on affected side

UAPA can cause hypoplasia in the affected lung due to the disruption of blood flow to the lung. The decreased blood flow can interrupt normal lung development, resulting in the lung being small and hypoplastic. [2]

Pulmonary hypertension (PHT)

PHT can be caused by excess blood flow diverted to the remaining pulmonary artery from the absent pulmonary artery. [8] Vasoconstrictive substances, like endothelin, are released as a result of shear pressure caused by increased blood flow in the unaffected pulmonary artery. Persistent constriction due to the substances can cause remodelling in the pulmonary arterioles, which raises the resistance of the pulmonary vasculature and causes PHT. [8] Additional explanations for PHT include inadequate elasticity of the pulmonary vascular bed on the unaffected side to withstand the full cardiac output and abnormal response to vasoconstrictive substances. [20]

Diagnosis

The non-specific symptoms and the lack of awareness of such an uncommon condition make UAPA difficult to diagnose. [9] Abnormalities found in imaging tests are more subtle and can be overlooked in infants. [21] Patients with uncomplicated isolated UAPA typically have normal electrocardiograms. [7] Making a diagnosis requires a complete medical history, physical assessment, and laboratory examination, coupled with a high index of suspicion. [15] To obtain a comprehensive evaluation of the conditions, multiple imaging modalities are often used in conjunction.

Chest Radiography

Chest X-rays are often the initial imaging modality used to evaluate patients with symptoms of cardiovascular diseases. While they may not provide detailed visualization of the pulmonary arteries, certain findings that raise suspicion for UAPA could be incidentally detected. [8] Patients with UAPA typically have asymmetric lung fields on their chest radiographs, with a hyperlucent lung held in an ipsilateral small hemithorax. [18] The mediastinum and trachea are shifted to the affected side of the lung, and the hilar vasculature is absent or significantly reduced on that side. [22] Ipsilateral diminished pulmonary vascular markings, ipsilateral elevated hemidiaphragm, hyperinflated contralateral lung and enlarged pulmonary artery may also be observed in chest X-rays. [23]

Magnetic resonance imaging (MRI) and computed tomography (CT)

When a chest radiograph reveals suspicious findings, MRI and CT can be used to definitively diagnose UAPA. [8] These techniques produce cross-sectional images of the chest and effectively visualize the absence of one of the pulmonary arteries, typically terminating within 1 cm of its expected origin from the main pulmonary artery. [22] Other observations that suggest the possibility of UAPA from CT or MRI include mosaic parenchymal alterations, intact peripheral branches of the pulmonary artery, reduced pulmonary blood vessels, and ipsilateral collateral vasculature hypertrophy. [8] [23]

Transthoracic echocardiography

Transthoracic echocardiography serves as another complementary tool to confirm the diagnosis of UAPA. [18] It has the advantage of being able to detect pulmonary hypertension and associated cardiac abnormalities at the same time. [23]

Angiography

Pulmonary angiography is the gold standard for diagnosis of pulmonary artery agenesis. [18] It is an invasive method that directly demonstrates the absence of one of the pulmonary arteries through the injection of a contrast dye into the blood. [23] Magnetic resonance angiography (MRA) can be employed to assess the haemodynamic status in real-time. [24] With current CT, MRI and MRA technologies, conventional pulmonary angiography is rarely carried out unless embolization is necessary for significant hemoptysis. [7] Pulmonary venous wedge angiography is especially helpful in outlining the hypoplastic intrapulmonary vessels and the ipsilateral hilar pulmonary artery before revascularization surgery. [21]

Treatment

There is no specific treatment for this abnormality. Therapeutic plans are individualized based on patients’ severity, symptoms and complications. [2] [8]

For asymptomatic patients with no evidence of cardiopulmonary dysfunction, no treatment is required. Regular check-up, [3] for example annual echocardiography assessment, [2] is advised for prompt detection of pulmonary hypertension. [1] For patients with massive hemoptysis, recurrent lower respiratory tract infection, pneumonia or pulmonary hypertension, treatment is necessary. [8] Hemoptysis can be treated by pneumonectomy [10] or embolization of systemic-to-pulmonary collateral arteries. [11]

Revascularization

Surgical anastomosis attaches the intrapulmonary branches of the affected pulmonary artery to the hilum of the lung. [2] [7] After revascularization, systemic blood flow from the heart to the lung returns to normal. The removal of obstruction causes a partial reduction in collateral arteries. [8] Pulmonary hypertension is improved post-surgery. [7] In newborn, surgical instruments involve prosthetic materials, saphenous vein grafts and autologous pericardial tubes. [10] Successful revascularization has been reported in young patients. [25] Such procedure gives better outcomes in children. [8] In adult patients, pulmonary artery reconstruction is not feasible as their intrapulmonary artery might be narrowed or completely obstructed. Lung biopsy is recommended prior to revascularization. Reconstruction surgery is not to be carried out if arteriovenous communication or abnormal vascular anatomy are observed. [3]

Pneumonectomy

Pneumonectomy refers to the removal of the lung on the side of pulmonary artery agenesis. In the absence of a pulmonary artery, the lung on the affected side is not involved in ventilation. Instead, it leads to symptoms such as hemoptysis, pulmonary hypertension and congestive heart failure. [11] Removal of the affected lung may treat related complications. Successful neonatal pneumonectomy has been reported in a 23-day-old baby. [4] Outcome of the surgery is nonetheless indefinite. Excessive bleeding can occur during pneumonectomy, and hemoptysis may still be observed after surgery. [11] After the removal of one lung, postpneumonectomy syndrome may be present. The mediastinum shifts to the affected side and compresses airways. [12]

Embolization of collateral arteries

Embolization involves surgical blockage of selective collateral arteries developing from systemic circulation. [25] Occlusion in the artery can be done using Gelfoam and Ivalon particles with coils. Specific coil sizes for the artery can be selected. Microcatheters enable higher selectivity during catheterization and embolization. [11] Possible side effects include postembolization syndrome and lung infarction. The surgical procedure is safer, less invasive and has minimal side effects. It can be an alternative treatment to lung resection. [11] However, embolization may face a lack of professionals and technical issues in placing coils in arteries. The chronic recurrence rate is as high as 25% owing to the extensive collateral network. [8]

Pulmonary hypertension can be alleviated by long-term medication of vasodilators. [8] [7] They can improve survival when surgical procedures are not favourable or when pulmonary hypertension is still present after surgery.

The chemical structure of bosentan. Bosentan.svg
The chemical structure of bosentan.

Pharmacotherapy

Orally administered drugs for reducing pulmonary hypertension include endothelin receptor antagonists (e.g. bosentan) and calcium channel blockers. [7] Parenteral injection of prostacyclins are also carried out continuously for such patients. Oral administration or intravenous injection of phosphodiesterase inhibitors serve as vasodilators. [7]

Heart-lung transplantation

For severe conditions of pulmonary artery agenesis, transplantation of both heart and lung can significantly elevate the oxygen level in blood. Heart-lung transplantation may be suggested for patients with recurrent hemoptysis, pulmonary hypertension and exercise intolerance. [25]

Related Research Articles

<span class="mw-page-title-main">Cardiology</span> Branch of medicine dealing with the heart

Cardiology is the study of the heart. Cardiology is a branch of medicine that deals with disorders of the heart and the cardiovascular system. 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 this field of medicine are called cardiologists, a sub-specialty of internal medicine. Pediatric cardiologists are pediatricians who specialize in cardiology. Physicians who specialize in cardiac surgery are called cardiothoracic surgeons or cardiac surgeons, a specialty of general surgery.

<span class="mw-page-title-main">Hemoptysis</span> Medical symptom consisting of bloody mucus from coughing

Hemoptysis or haemoptysis is the discharge of blood or blood-stained mucus through the mouth coming from the bronchi, larynx, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airway bleeding. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, and certain cardiovascular conditions. Hemoptysis is considered massive at 300 mL. In such cases, there are always severe injuries. The primary danger comes from choking, rather than blood loss.

<span class="mw-page-title-main">Hereditary hemorrhagic telangiectasia</span> Genetic disorder affecting blood vessel development

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain.

<span class="mw-page-title-main">Eisenmenger syndrome</span> Medical condition

Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt. Because of the advent of fetal screening with echocardiography early in life, the incidence of heart defects progressing to Eisenmenger syndrome has decreased.

<span class="mw-page-title-main">Pancoast tumor</span> Medical condition

A Pancoast tumor is a tumor of the apex of the lung. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most Pancoast tumors are non-small-cell lung cancers.

<span class="mw-page-title-main">Thoracotomy</span> Surgical procedure to access the interior of the chest

A thoracotomy is a surgical procedure to gain access into the pleural space of the chest. It is performed by surgeons to gain access to the thoracic organs, most commonly the heart, the lungs, or the esophagus, or for access to the thoracic aorta or the anterior spine. A thoracotomy is the first step in thoracic surgeries including lobectomy or pneumonectomy for lung cancer or to gain thoracic access in major trauma.

<span class="mw-page-title-main">Bronchial artery</span> Blood vessels supplying the lungs

In human anatomy, the bronchial arteries supply the lungs with oxygenated blood, and nutrition. Although there is much variation, there are usually two bronchial arteries that run to the left lung, and one to the right lung, and are a vital part of the respiratory system.

<span class="mw-page-title-main">Persistent truncus arteriosus</span> Medical condition

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.

<span class="mw-page-title-main">Pneumonectomy</span> Surgical removal of a lung

A pneumonectomy is a surgical procedure to remove a lung. It was first successfully performed in 1933 by Dr. Evarts Graham. This is not to be confused with a lobectomy or segmentectomy, which only removes one part of the lung.

<span class="mw-page-title-main">Pericardiacophrenic artery</span>

The pericardiacophrenic artery is a long slender branch of the internal thoracic artery.

<span class="mw-page-title-main">Anterior spinal artery syndrome</span> Human spinal cord disorder

Anterior spinal artery syndrome is syndrome caused by ischemia of the area supplied by the anterior spinal artery, resulting in loss of function of the anterior two-thirds of the spinal cord. The region affected includes the descending corticospinal tract, ascending spinothalamic tract, and autonomic fibers. It is characterized by a corresponding loss of motor function, loss of pain and temperature sensation, and hypotension.

<span class="mw-page-title-main">Aortopulmonary window</span> Medical condition

Aortopulmonary window (APW) is a faulty connection between the aorta and the main pulmonary artery that results in a significant left-to-right shunt. The aortopulmonary window is the rarest of septal defects, accounting for 0.15-0.6% of all congenital heart malformations. 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.

<span class="mw-page-title-main">Anomalous left coronary artery from the pulmonary artery</span> Medical condition

Anomalous left coronary artery from the pulmonary artery is a rare congenital anomaly occurring in approximately 1 in 300,000 liveborn children. The diagnosis comprises between 0.24 and 0.46% of all cases of congenital heart disease. The anomalous left coronary artery (LCA) usually arises from the pulmonary artery instead of the aortic sinus. In fetal life, the high pressure in the pulmonic artery and the fetal shunts enable oxygen-rich blood to flow in the LCA. By the time of birth, the pressure will decrease in the pulmonic artery and the child will have a postnatal circulation. The myocardium, which is supplied by the LCA, will therefore be dependent on collateral blood flow from the other coronary arteries, mainly the RCA. Because the pressure in RCA exceeds the pressure in LCA a collateral circulation will increase. This situation ultimately can lead to blood flowing from the RCA into the LCA retrograde and into the pulmonary artery, thus forming a left-to-right shunt.

<span class="mw-page-title-main">Hughes–Stovin syndrome</span> Autoimmune disorder

Hughes–Stovin syndrome (HSS) is a rare autoimmune disorder often described as inflammation in relation to blood vessels, a form of vasculitis. It is not associated with any known cause and is typically characterized by multiple aneurysms in pulmonary arteries and deep vein thromboses. It is named after the two British physicians, John Patterson Hughes and Peter George Ingle Stovin, who first described it in 1959. HSS is presumed to be a rare variant of Behçet's disease, which entails more general problems with the circulatory system. Due to its clinical similarity with Behçet's disease, it has also been referred to as 'Incomplete Behçet's disease.' Most patients are young adult males between the age of 20–40. Common clinical presentations include fever, cough, dyspnea and hemoptysis. Radiological features are similar to those of Behçet's disease.

<span class="mw-page-title-main">Mediastinal shift</span> Medical condition

Mediastinal shift is an abnormal movement of the mediastinal structures toward one side of the chest cavity. A shift indicates a severe imbalance of pressures inside the chest. Mediastinal shifts are generally caused by increased lung volume, decreased lung volume, or abnormalities in the pleural space. Additionally, masses inside the mediastinum or musculoskeletal abnormalities can also lead to abnormal mediastinal arrangement. Typically, these shifts are observed on x-ray but also on computed tomography (CT) or magnetic resonance imaging (MRI). On chest x-ray, tracheal deviation, or movement of the trachea away from its midline position can be used as a sign of a shift. Other structures, like the heart, can also be used as reference points. Below are examples of pathologies that can cause a mediastinal shift and their appearance.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term disease caused by a blockage in the blood vessels that deliver blood from the heart to the lungs. These blockages cause increased resistance to flow in the pulmonary arterial tree which in turn leads to rise in pressure in these arteries. The blockages either result from organised blood clots that usually originate from the deep veins of the lower limbs of the body (thromboembolism) and lodge in the pulmonary arterial tree after passing through the right side of the heart. The blockages may also result from scar tissue that forms at the site where the clot has damaged the endothelial lining of the pulmonary arteries, causing permanent fibrous obstruction. Most patients have a combination of microvascular and macrovascular obstruction. Some patients may present with normal or near-normal pulmonary pressures at rest despite symptomatic disease. These patients are labelled as having chronic thromboembolic disease (CTED).

<span class="mw-page-title-main">Absent pulmonary valve syndrome</span> Medical condition

Absent pulmonary valve syndrome(APVS) is a congenital heart defect that occurs when the flaps of the pulmonary valve do not develop or are severely underdeveloped (hypoplasia) resulting in aneurysms (dilation) of the pulmonary arteries and softening of the trachea and bronchi (tracheobronchomalacia). Usually, APVS occurs together with other congenital heart defects, most commonly ventricular septal defect and right ventricular outflow tract obstruction. It is sometimes considered a variant of Tetralogy of Fallot. The first case of absent pulmonary valve syndrome was reported Crampton in 1830.

Bronchial artery embolization is a treatment for hemoptysis, abbreviated as BAE. It is a kind of catheter intervention to control hemoptysis by embolizing the bronchial artery, which is a bleeding source. Embolic agents are particulate embolic material such as gelatin sponge or polyvinyl alcohol (PVA), and liquid embolic material such as NBCA, or metallic coils.

<span class="mw-page-title-main">Pulmonary agenesis</span> Medical condition

Pulmonary agenesis is an inborn lung underdevelopment that is rare and potentially lethal. The disorder is caused by a complete developmental arrest of the primitive lung during embryonic life, and it is often associated with other developmental defects. Bilateral and unilateral pulmonary agenesis are classified, depending on whether one side of the lung or both sides are affected. Bilateral pulmonary agenesis is lethal, while the mortality rate of unilateral pulmonary agenesis is higher than 50%. Depending on the severity, the symptom ranges from none to various respiratory complaints. It is detectable prenatally, however, its nonspecific clinical features act as the obstacle for diagnosing. The exact cause of pulmonary agenesis is still obscure. However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder. In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.

<span class="mw-page-title-main">Lung surgery</span>

Lung surgery is a type of thoracic surgery involving the repair or removal of lung tissue, and can be used to treat a variety of conditions ranging from lung cancer to pulmonary hypertension. Common operations include anatomic and nonanatomic resections, pleurodesis and lung transplants. Though records of lung surgery date back to the Classical Age, new techniques such as VATS continue to be developed.

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