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Pulmonary artery
Anterior (frontal) view of the opened heart. White arrows indicate normal blood flow. (Pulmonary artery labelled at upper right.)
Details
PrecursorTruncus arteriosus
SystemCardiovascular, respiratory
SourceRight ventricle
Identifiers
Latinarteria pulmonalis
MeSHD011651
TA24074
FMA66326
Anatomical terminology

A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the main pulmonary artery or pulmonary trunk from the heart, and the smallest ones are the arterioles, which lead to the capillaries that surround the pulmonary alveoli.

Structure

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The pulmonary arteries are blood vessels that carry systemic venous blood from the right ventricle of the heart to the microcirculation of the lungs. Unlike in other organs where arteries supply oxygenated blood, the blood carried by the pulmonary arteries is deoxygenated, as it is venous blood returning to the heart. The main pulmonary arteries emerge from the right side of the heart and then split into smaller arteries that progressively divide and become arterioles, eventually narrowing into the capillary microcirculation of the lungs where gas exchange occurs.[citation needed]

Pulmonary trunk

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Volume rendering of a high resolution CT scan of the thorax. The anterior thoracic wall, the airways and the pulmonary vessels anterior to the root of the lung have been digitally removed to visualize the different levels of the pulmonary circulation.

In order of blood flow, the pulmonary arteries start as the pulmonary trunk that leaves the fibrous pericardium (parietal pericardium) of the ventricular outflow tract of right ventricle (also known as infundibulum or conus arteriosus.[1] The outflow track runs superiorly and to the left, posterior to the pulmonary valve.[1] The pulmonary trunk bifurcates into right and left pulmonary arteries below the arch of aorta and in front of the left main bronchus.[1] Pulmonary trunk is short and wide – approximately 5 centimetres (2.0 in) in length[2] and 2 centimetres (0.79 in)-3 centimetres (1.2 in) in diameter.[3][4]

The pulmonary trunk splits into the right and the left main pulmonary artery.[5] The left main pulmonary artery is shorter than the right,[1] passes behind and downwards the descending aorta and above the left main bronchus to the root of the left lung. Above, the left main pulmonary artery is connected to the concavity of the proximal descending aorta by the ligamentum arteriosum.[2] The right pulmonary artery pass across the midline of the body, below the carina of trachea, and comes in front of the right main bronchus.[1]

Branches

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At the far end, pulmonary arteries (labelled at the bottom) become capillaries at the pulmonary alveoli.

The left main pulmonary artery then divides into two lobar arteries, one for each lobe of the left lung.[6]

At the right root of the lung, it bifurcates into artery that supplies the right upper lobe of the lung, in front of the right upper lobe bronchus, and interlobar artery that supplies the right middle and inferior lobes of the lung, running together with bronchus intermedius.[1]

The right and left main pulmonary (lungs) arteries give off branches that supplies the corresponding lung lobes. In such cases it is termed lobar arteries.[7] The lobar arteries branch into segmental arteries (roughly 1 for each segment). Segmental arteries run together with segmental bronchi, at the posterolateral surfaces of the bronchi.[7] These in turn branch into subsegmental pulmonary arteries.[7] These eventually form intralobular arteries.[8] The pulmonary arteries supply the alveoli of the lungs. In contrast, bronchial arteries, that has different origins, supply the bronchi of the lungs.[1]

Development

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The pulmonary arteries originate from the truncus arteriosus and the sixth pharyngeal arch. The truncus arteriosus is a structure that forms during the development of the heart as a successor to the conus arteriosus.[9]: 157 

By the third week of development, the endocardial tubes have developed a swelling in the part closest to the heart. The swelling is known as the bulbus cordis and the upper part of this swelling develops into the truncus arteriosus.[9]: 159–160  The structure is ultimately mesodermal in origin.[9]: 157  During development of the heart, the heart tissues undergo folding, and the truncus arteriosus is exposed to what will eventually be both the left and right ventricles. As a septum develops between the two ventricles of the heart, two bulges form on either side of the truncus arteriosus. These progressively enlarge until the trunk splits into the aorta and pulmonary arteries.[9]: 176–179  Failure of these processes can lead to pulmonary artery agenesis.

During early development, the ductus arteriosus connects the pulmonary trunk and the aortic arch, allowing blood to bypass the lungs.[10]: 791 

Function

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The pulmonary artery carries deoxygenated blood from the right ventricle to the lungs.[11] The blood here passes through capillaries adjacent to alveoli and becomes oxygenated as part of the process of respiration.[12]

In contrast to the pulmonary arteries, the bronchial arteries supply nutrition to the lungs themselves.[10]: 790 

Pressure

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The pulmonary artery pressure (PA pressure) is a measure of the blood pressure found in the main pulmonary artery. This is measured by inserting a catheter into the main pulmonary artery.[13] : 190–191  The mean pressure is typically 9–18 mmHg,[14] and the wedge pressure measured in the left atrium may be 6–12 mmHg. The wedge pressure may be elevated in left heart failure,[13]: 190–191  mitral valve stenosis, and other conditions, such as sickle cell disease.[15]

Clinical significance

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The pulmonary artery is relevant in a number of clinical states. Pulmonary hypertension is used to describe an increase in the pressure of the pulmonary artery, and may be defined as a mean pulmonary artery pressure of greater than 25 mmHg.[13]: 720  A pulmonary artery diameter of more than 29 mm (measured on a CT scan) is often used as an indicator for pulmonary hypertension.[16] In chest X-rays, a diameter of more than 16 mm for the right descending pulmonary artery is also an indicator for pulmonary hypertension.[17] This may occur as a result of heart problems such as heart failure, lung or airway disease such as COPD or scleroderma, or thromboembolic disease such as pulmonary embolism or emboli seen in sickle cell anaemia.[13]: 720–721  Most recently, computational fluid based tools (non-invasive) have been proposed to be at par with the current clinical tests (invasive) of pulmonary hypertension.[18]

Pulmonary embolism refers to an embolus that lodges in the pulmonary circulation. This may arise from a deep venous thrombosis, especially after a period of immobility. A pulmonary embolus is a common cause of death in patients with cancer and stroke.[13]: 720–721  A large pulmonary embolus that becomes lodged in the bifurcation of the pulmonary trunk with extensions into both the left and right main pulmonary arteries is called a saddle embolus.[19]

(made in (1883))
Anatomy of the horse, with other arteries: spermatic artery (21), next to (posterior) vena cava, venae portae, External iliac artery, and the mesenteric vessels, Internal iliac and renal arteries labeled. The pulmonary artery and pulmonary veins are labelled (4) and (5), respectively.

Several animal models have been utilized for investigating pulmonary artery related pathologies. Porcine model of pulmonary artery is the most frequently used and it was recently found that their mechanical properties vary with every subsequent branching.[20]

Additional images

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  • Image showing main pulmonary artery coursing ventrally to the aortic root and trachea, and the right pulmonary artery passes dorsally to the ascending aorta, while the left pulmonary artery passes ventrally to the descending aorta.
    Image showing main pulmonary artery coursing ventrally to the aortic root and trachea, and the right pulmonary artery passes dorsally to the ascending aorta, while the left pulmonary artery passes ventrally to the descending aorta.
  • Pulmonary circuit
    Pulmonary circuit
  • Transverse section of thorax, showing relations of pulmonary artery.
    Transverse section of thorax, showing relations of pulmonary artery.
  • Original image from Anatomist 90
    Pulmonary artery
  • Pulmonary artery.Deep dissection.Anterior view.
    Pulmonary artery.Deep dissection.Anterior view.
  • CT scan of a normal lung, with different levels of pulmonary arteries.
    CT scan of a normal lung, with different levels of pulmonary arteries.
  • Bronchial anatomy
    Bronchial anatomy

See also

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References

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Revisions and contributorsEdit on WikipediaRead on Wikipedia

Pulmonary artery

View on Grokipedia
from Grokipedia
The pulmonary artery is the primary vessel responsible for transporting deoxygenated from the right ventricle of the heart to the lungs, where it undergoes oxygenation via in the pulmonary capillaries. This artery is unique among arteries in the systemic circulation, as it carries deoxygenated rather than oxygenated , forming a key component of the that separates it from the higher-pressure systemic circuit. Anatomically, the pulmonary artery originates as the main pulmonary trunk, a short conduit measuring approximately 5 cm in length and 2-3 cm in diameter, which emerges from the right ventricular outflow tract immediately superior to the pulmonary valve. The trunk then bifurcates at the level of the fourth thoracic vertebra (near the carina) into the right and left pulmonary arteries, with the right branch being longer (about 5 cm) and curving posteriorly to the ascending aorta while passing anterior to the right main bronchus. The left pulmonary artery, in contrast, extends horizontally anterior to the descending aorta and superior to the left main bronchus, both branches traveling within the pericardium before entering the lung hilum. These main branches further subdivide into lobar (typically three on the right and two on the left), segmental (up to 10 per lung), and subsegmental arteries, culminating in a dense capillary bed adjacent to the alveoli to maximize surface area for diffusion. Structurally, the vessel walls are thin and distensible with a prominent muscular layer in the tunica media, adapted for the low-pressure environment of pulmonary circulation (systolic pressure 15-30 mmHg, diastolic 4-12 mmHg). Functionally, the pulmonary artery delivers the entire of deoxygenated blood to the lungs for replenishment with oxygen and removal of , ensuring efficient before the blood returns to the left atrium via the pulmonary veins. This low-resistance pathway prevents excessive strain on the right ventricle and supports overall cardiopulmonary , with blood flow regulated by the to prevent backflow. Embryologically, the pulmonary arteries develop from the sixth pair of , the , and contributions from cells, with the providing a fetal shunt that closes postnatally to form the . Clinically, the pulmonary artery plays a central role in diagnosing and managing cardiopulmonary disorders, as its pressures can be measured via Swan-Ganz catheterization to assess conditions like pulmonary hypertension or right heart failure. It is also implicated in congenital anomalies such as pulmonary atresia, stenosis, or Tetralogy of Fallot, as well as acquired issues including pulmonary embolism (a potential medical emergency from thrombi obstructing flow) and chronic thromboembolic pulmonary hypertension. These aspects underscore its critical position at the interface of cardiac output and respiratory function.

Anatomy

Pulmonary trunk

The pulmonary trunk originates from the conus arteriosus of the right ventricle at the , positioned immediately superior and anterior to the . This segment serves as the initial conduit for deoxygenated blood exiting the right ventricle toward the lungs. The , a semilunar structure with three cusps—designated as anterior, right, and left—guards the orifice between the right ventricle and the pulmonary trunk, ensuring unidirectional flow by preventing backflow during ventricular diastole. The trunk then ascends anteriorly and curves slightly posteriorly for approximately 5 cm before bifurcating into the left and right main pulmonary arteries at the level of the , corresponding to the T4-T5 vertebral level. In adults, it measures about 5 cm in length and 2-3 cm in diameter, though variations in length and diameter occur among individuals, with upper normal limits around 29 mm in males and 27 mm in females. Anatomically, the pulmonary trunk lies anterior to the and while being posterior to the , and it is fully enveloped by the , sharing a common serous sheath with the . Its blood supply derives from the bronchial arteries, which provide oxygenated blood via the to nourish the vessel wall.

Main pulmonary arteries

The main pulmonary arteries arise from the bifurcation of the pulmonary trunk at the level of the fourth , with the right and left branches directing deoxygenated blood to their respective . The right pulmonary artery is longer, measuring approximately 5 cm in length, and follows a relatively straight, horizontal course posterior to the and before entering the hilum of the right . It has a similar caliber to the left counterpart, facilitating its direct path to the right hilum. The right pulmonary artery remains within the for most of its length. In contrast, the left pulmonary artery is shorter, spanning about 3-4 cm, and exhibits a more tortuous trajectory as it curves posteriorly around the , where the attaches, before reaching the left hilum. The left pulmonary artery has a longer extrapericardial course after passing under the . This inherent asymmetry between the two arteries reflects their positional relations to adjacent mediastinal structures. The right pulmonary artery lies adjacent to the superiorly and the right main inferiorly, coursing anterior to the esophagus and right main . The left pulmonary artery, positioned near the and left main , arches superior to the left main and posterior to the , contributing to its elongated and curved path. These relations influence surgical approaches and imaging interpretations in thoracic procedures. Anatomical variations in the main pulmonary arteries are uncommon but clinically significant. Occasional accessory branches may arise proximal to the hila, and anomalous origins can occur, such as in cases of absent left pulmonary artery (unilateral pulmonary artery ), a rare congenital anomaly affecting less than 0.3% of the population, often leading to compensatory of the right and vasculature. The main pulmonary arteries further subdivide into lobar branches upon entering the lung hila.

Intrapulmonary branches

The intrapulmonary branches of the pulmonary arteries arise from the main pulmonary arteries at the hilum of each and form a hierarchical network that distributes deoxygenated blood to the for . These branches parallel the bronchial tree, entering the s within a common sheath to supply specific functional units. Lobar arteries are the first major divisions within the s, corresponding to the pulmonary lobes. In the right , the right pulmonary artery gives rise to three lobar branches: one for the upper lobe, one for the middle lobe, and one for the lower lobe. In the left , which lacks a middle lobe, the left pulmonary artery divides into two primary lobar branches: one for the upper lobe (including the lingular segment) and one for the lower lobe. These lobar arteries course toward their respective lobes before further subdividing. Segmental arteries emerge from the lobar arteries to supply the 10 bronchopulmonary segments in each , discrete pyramidal regions of tissue defined by their independent bronchial and vascular supply. Each segmental accompanies a corresponding segmental , providing blood to segments such as the apical (upper lobe), posterior basal (lower lobe), and medial (middle lobe on the right). The right 's 10 segments consist of 3 in the upper lobe, 2 in the middle lobe, and 5 in the lower lobe, while the left 's 10 segments include 5 in the upper lobe (with the lingula often treated as two segments: superior and inferior), and 5 in the lower lobe. Subsegmental arteries branch from the segmental arteries, further dividing into smaller vessels that eventually form terminal arterioles leading to the beds surrounding the alveoli. These finer branches maintain the parallel course with the bronchioles, ensuring efficient of the alveolar units. A notable variation in branching occurs between the right and left lungs due to the presence of the middle lobe on the right, which receives dedicated segmental arteries (lateral and medial), whereas the left lung's equivalent lingular segment branches from the upper lobe artery, resulting in asymmetric patterns overall.

Histology

The walls of the pulmonary artery are organized into three concentric layers, or tunicae, adapted for handling low-pressure, high-volume blood flow from the right ventricle to the lungs. The innermost consists of a thin of non-fenestrated endothelial cells overlying a subendothelial layer of and a delicate elastic lamina; this structure minimizes resistance to flow while maintaining a barrier to prevent . The middle tunica media comprises alternating layers of circumferentially arranged cells and fenestrated elastic laminae, which provide elasticity for distensibility under pulsatile pressure, though the overall thickness is reduced compared to systemic elastic arteries. The outermost tunica adventitia is composed of loosely organized collagenous containing fibroblasts, for nutrient supply to the outer media, and a neuronal network for vasoregulation. As an elastic artery, the pulmonary artery exhibits high elastin content to accommodate cyclic stretching, with comprising approximately 24% of area in the main pulmonary artery of humans, forming short, thin fibers that contribute to compliance but in lesser abundance and with fewer lamellae (typically 4 or more) than in the . From the proximal trunk to distal intrapulmonary branches, progressively thins, transitioning from elastic-dominated (with prominent media) to muscular types (with more relative to elastin) and eventually partially or non-muscular in pre-capillary arterioles, reflecting adaptation to decreasing pressure gradients. The endothelial layer in the pulmonary artery is notably thin, positioning it in close proximity to alveolar surfaces in distal branches, which supports efficient oxygenation without the need for fenestrations in arterial segments. In contrast to systemic arteries, which feature thicker media with greater and for high-pressure resistance, pulmonary arteries have reduced muscularity and density to promote low , and the smallest vessels lack a distinct to further ease flow. Innervation arises primarily from sympathetic fibers of the autonomic chain and parasympathetic fibers via the , forming an adventitial of adrenergic, , and sensory nerves that penetrate the media in some to modulate tone.

Embryology

Embryonic origins

The pulmonary arteries originate from the sixth pair of during early . These arches form as paired vessels connecting the aortic sac to the dorsal aortae, with the proximal portions of the bilateral sixth arches giving rise to the main pulmonary arteries, while the distal portions contribute to the on the left and regress on the right. The development of these arches begins around the fourth week of gestation, as the aortic sac emerges from the cardiogenic and extends vessels sequentially. By the fifth week, the pulmonary trunk begins to form from the outflow tract of the , which elongates and incorporates elements of the . Septation of the into the and pulmonary trunk occurs through the formation of the aorticopulmonary septum, a spiraling structure derived primarily from cells that migrate into the cardiac cushions. This process starts at the distal end in the sixth week (Carnegie stage 16) and proceeds proximally, completing by the seventh week, ensuring separation of systemic and pulmonary circulations. Early in development, the nascent pulmonary arteries establish vascular connections via with the primitive lung buds, which arise as an outpouching from the ventral around day 22 of . These connections form a primitive vascular in the surrounding splanchnic mesoderm, supplying the expanding buds as they branch into bronchial structures by the end of the fifth week. Concurrently, portions of the dorsal aortae regress, isolating the pulmonary vasculature from the dorsal and directing flow specifically to the . Defects in these embryonic processes underlie key congenital anomalies of the pulmonary artery. results from failed septation of the , leaving a single arterial trunk without division into and pulmonary trunk, often due to insufficient cell contribution. arises from conotruncal defects, including abnormal migration leading to malalignment of the outflow and pulmonary stenosis.

Fetal and postnatal development

In the fetal circulation, pulmonary vascular resistance remains elevated due to unexpanded lungs and vasoconstrictive influences such as low oxygen tension and high sensitivity to circulating mediators like endothelin-1, directing approximately 90% of right ventricular output away from the pulmonary circuit. This shunting occurs primarily through the , a vascular connection between the pulmonary trunk and the , which maintains low pulmonary blood flow and supports oxygenation via the . The pulmonary arteries thus develop under conditions of minimal flow, with thick muscular media adapted to the high-resistance environment. At birth, the initial breath expands the s, aerating the alveoli and triggering a rapid decline in —dropping by up to 10-fold within minutes—due to increased oxygenation, release of vasodilators like , and mechanical factors such as lung inflation. This facilitates a surge in pulmonary blood flow, converting the pulmonary arteries to a low-resistance, high-flow system. Concurrently, the undergoes functional closure through contraction in response to rising oxygen levels and falling , typically within 24-48 hours, followed by anatomical remodeling into the over 2-3 weeks. Postnatally, the pulmonary artery undergoes proportional growth, with its diameter increasing linearly with somatic growth and , ensuring accommodation of rising during childhood. Structural remodeling transforms the vessel wall, thinning the initially thick muscular media while incorporating more elastic fibers for enhanced compliance and handling. accumulation in the pulmonary artery media peaks during the perinatal and periods, supporting long-term elasticity before stabilizing in adulthood. In conditions of chronic hypoxia, such as high-altitude exposure or persistent , the media can hypertrophy with proliferation, potentially leading to sustained vascular remodeling. Premature infants face heightened risk of persistence beyond 72 hours due to immature responsiveness to closure signals, often requiring intervention to prevent left-to-right shunting and overload.

Physiology

Role in circulation

The pulmonary artery plays a central role in the by receiving deoxygenated blood directly from the right ventricle of the heart through the pulmonary trunk, forming a critical link between the systemic and pulmonary circulatory systems. This deoxygenated blood, which has returned from the body's tissues via the systemic veins, is pumped into the pulmonary trunk and subsequently distributed to the s for oxygenation. Unlike the systemic arteries, which carry oxygenated blood under high pressure, the pulmonary artery operates as the starting point of a dedicated loop that ensures efficient without the need for nutrient delivery to the lung tissue itself. In the perfusion pathway, the pulmonary artery delivers approximately 5 liters per minute of blood—equivalent to the entire cardiac output at rest—to the lungs, where it undergoes oxygenation before returning to the left side of the heart. This flow parallels the bronchial circulation, a separate systemic-derived network that supplies oxygenated blood primarily for the nutritional needs of the lung parenchyma and airways, with minimal overlap under normal conditions. The pulmonary arteries branch into a network that directs blood to the alveolar capillaries, facilitating the diffusion of oxygen into the bloodstream and the removal of carbon dioxide, thereby optimizing arterial blood composition for systemic distribution. The lungs' oxygenation via the bronchial circulation ensures that the pulmonary arteries focus solely on gas exchange functions. The pulmonary circulation, including the pulmonary artery, is characterized as a low-resistance system that accommodates the full cardiac output with minimal pressure requirements, thanks to its wide, compliant vessels and parallel arrangement. This design prevents excessive strain on the right ventricle while maintaining steady perfusion to the lungs. A key regulatory mechanism involves hypoxic vasoconstriction in the pulmonary arterioles, where reduced oxygen levels in specific lung regions trigger localized constriction to redirect blood flow toward better-ventilated areas, thereby matching ventilation to perfusion and enhancing overall gas exchange efficiency.

Hemodynamics

The hemodynamics of the pulmonary artery are characterized by low pressures that facilitate efficient gas exchange in the lungs while minimizing the workload on the right ventricle. In healthy individuals, the mean pulmonary artery pressure is approximately 15 mm Hg, with systolic pressure ranging from 15 to 30 mm Hg and diastolic pressure from 4 to 12 mm Hg. These values are substantially lower than those in the systemic arteries, where mean pressure is about 93 mm Hg (systolic 120 mm Hg, diastolic 80 mm Hg), reflecting the pulmonary circulation's adaptation to a low-resistance, high-flow environment. Blood flow through the pulmonary artery is pulsatile, driven by right ventricular contraction, but it is notably damped compared to systemic flow due to the high capacitance of the pulmonary vasculature. In steady-state conditions, pulmonary blood flow equals , which can be quantified using the :
Q=V˙O2CaO2−CvO2Q = \frac{\dot{V}O_2}{C_aO_2 - C_vO_2}
where QQ is , V˙O2\dot{V}O_2 is oxygen consumption, CaO2C_aO_2 is arterial oxygen content, and CvO2C_vO_2 is mixed venous oxygen content. This equality ensures that the entire perfuses the lungs for oxygenation without recirculation discrepancies under normal physiology. Pulmonary vascular resistance (PVR) is low, typically ranging from 0.25 to 1.6 Wood units (WU), enabling high flow at minimal pressure gradients. PVR is calculated as:
PVR=P‾PA−PLAQPVR = \frac{\overline{P}_{PA} - P_{LA}}{Q}
where P‾PA\overline{P}_{PA} is mean pulmonary artery pressure, PLAP_{LA} is left atrial pressure (often approximated by pulmonary capillary wedge pressure), and QQ is cardiac output. The pulmonary arteries exhibit high compliance, approximately 2-3 mL/mm Hg in normotensive conditions, owing to their elastic walls composed of smooth muscle and elastin, which accommodate volume changes with minimal pressure rise. This high compliance results in minimal wave reflections, contrasting with the more pronounced reflections in the high-resistance systemic circulation. Direct measurement of pulmonary artery pressures and flows is achieved via right heart catheterization, which provides invasive, gold-standard assessments of systolic, diastolic, and mean pressures, as well as for PVR calculation. Noninvasive estimation is commonly performed using , which derives systolic pulmonary artery pressure from tricuspid regurgitant jet velocity and estimates mean pressure via formulas incorporating right atrial pressure. These methods allow for routine hemodynamic evaluation while highlighting the pulmonary artery's role in maintaining low-resistance flow.

Clinical significance

Congenital anomalies

Congenital anomalies of the pulmonary artery encompass a spectrum of structural birth defects arising during embryonic development, primarily affecting the pulmonary trunk, , or branches, and often leading to obstructed right ventricular outflow. These defects disrupt normal pulmonary blood flow, necessitating reliance on alternative pathways such as the (PDA) or major aortopulmonary collateral arteries (MAPCAs) for oxygenation. Key types include pulmonary atresia, characterized by complete absence or obstruction of the pulmonary valve and trunk, resulting in no direct connection between the right ventricle and pulmonary arteries; this may involve an intact ventricular septum (PA-IVS) or a ventricular septal defect (PA-VSD). Pulmonary atresia accounts for approximately 1% of all congenital heart defects, with an overall incidence of about 1 in 10,000 live births. Another common type is pulmonary stenosis, involving narrowing of the pulmonary valve, subvalvular region, or proximal trunk, which restricts blood flow to the lungs and represents 7% to 12% of congenital heart defects. Anomalous origin of the pulmonary arteries, such as both branches arising from a common truncal root connected to the aorta in truncus arteriosus, occurs due to failure of aorticopulmonary septation and is seen in about 1% of congenital heart defects. These anomalies are frequently associated with syndromes like , where pulmonary stenosis combines with a , , and , comprising up to 10% of congenital heart defects. , resulting from 22q11.2 deletion affecting cell migration, is linked to pulmonary artery anomalies including interruption or aberrant origin in up to 45% of cases with PA-VSD. Genetic mutations, such as those in the NKX2.5 gene, contribute to these defects by impairing cardiac transcription and septation, occurring in at least 4% of nonsyndromic cases. Pathophysiologically, these anomalies cause through right-to-left shunting across septal defects or foramen ovale, as deoxygenated blood bypasses the lungs due to outflow obstruction; pulmonary blood flow depends on PDA or MAPCAs, which can lead to or if untreated, underscoring the need for early intervention to prevent or sudden death. Such defects often stem from embryonic septation failures, as detailed in developmental origins. Historically, was first described in the late by Hunter, with further pathological accounts in the by Peacock. Modern classification, including for PA-VSD as an extreme form of or pseudotruncus, follows the van Praagh system, which categorizes based on segmental anatomy and great vessel relations to guide surgical planning.

Acquired disorders

Acquired disorders of the pulmonary artery encompass a range of non-congenital conditions that impair its structure or function, leading to significant cardiovascular and respiratory complications. These include , , inflammatory , aneurysms, and fibrotic changes associated with chronic lung diseases. Such disorders often arise from environmental, inflammatory, or thrombotic insults, resulting in vascular remodeling, obstruction, or increased pressure that strains the right ventricle and compromises oxygenation. Pulmonary hypertension (PH) is characterized by elevated pressure in the pulmonary arteries, defined hemodynamically as a mean pulmonary artery (mPAP) greater than 20 mmHg at rest, measured via right heart catheterization. This threshold marks a departure from normal mPAP values below 20 mmHg and reflects progressive vascular changes. PH is classified into five groups by the (WHO): , pulmonary arterial hypertension (PAH), including idiopathic, heritable, and drug-induced forms; Group 2, PH due to left heart disease; Group 3, PH associated with lung diseases and/or hypoxia; Group 4, chronic thromboembolic PH (CTEPH); and Group 5, PH with unclear or multifactorial mechanisms. The prevalence of PH varies by subtype, with PAH () estimated at 15 to 50 cases per million population globally. Pathophysiologically, PH involves pulmonary vascular remodeling, including , smooth muscle proliferation, and , which increase pulmonary vascular resistance and impose chronic on the right ventricle, leading to , dilation, and eventual failure. Pulmonary embolism (PE) occurs when a , typically originating from deep veins in the lower extremities, dislodges and obstructs pulmonary artery branches, acutely increasing pulmonary . Risk factors align with : venous stasis (e.g., from immobility or ), hypercoagulability (e.g., due to or ), and endothelial injury (e.g., from trauma or catheters). The annual incidence of PE is approximately 1 per 1,000 persons , with higher rates in older adults and those with comorbidities. In acute PE, obstruction causes ventilation-perfusion mismatch and shunting, resulting in and right ventricular strain from sudden pressure overload. Other acquired disorders include arteritis, such as Takayasu arteritis, a large-vessel that involves the pulmonary arteries in 5.7% to 25.9% of cases, leading to , occlusion, or aneurysms through granulomatous inflammation. Pulmonary artery aneurysms are rare, often post-traumatic from penetrating injuries or iatrogenic causes like catheterizations, where vessel wall disruption forms pseudoaneurysms prone to rupture. affecting the pulmonary arteries can occur in chronic lung diseases, such as , where interstitial scarring induces vascular remodeling and contributes to Group 3 PH through hypoxic and deposition.

Diagnostic approaches

Diagnostic approaches to assessing pulmonary artery structure and function in clinical settings rely on a multimodal strategy that includes non-invasive imaging, laboratory tests, and invasive procedures, as outlined in the 2022 (ESC) and European Respiratory Society (ERS) guidelines for (PH). These methods aim to screen for abnormalities, confirm diagnoses such as PH or (PE), and guide risk stratification, with serving as the initial screening tool and right heart catheterization (RHC) as the definitive confirmatory test. Echocardiography, particularly with Doppler imaging, is the first-line non-invasive modality for estimating pulmonary artery systolic pressure (sPAP) and assessing right ventricular (RV) function and valve integrity. A peak tricuspid regurgitation velocity (TRV) exceeding 2.8 m/s, combined with additional signs such as an RV/left ventricular (LV) basal diameter ratio greater than 1.0 or flattened interventricular septum, indicates a high probability of PH and prompts referral to a specialized center. Computed tomography (CT) angiography excels in visualizing pulmonary artery emboli, congenital anomalies, and vascular remodeling, with a main pulmonary artery diameter of 30 mm or greater serving as an indirect marker of elevated pressures. Cardiac magnetic resonance imaging (MRI) quantifies pulmonary blood flow and evaluates RV volumes and ejection fraction, offering prognostic insights where echocardiography is inconclusive, such as in patients with poor acoustic windows. Invasive techniques provide precise hemodynamic data essential for definitive diagnosis. RHC remains the gold standard for , directly measuring mean pulmonary artery pressure (mPAP), pulmonary artery wedge pressure (PAWP), and (PVR), with PH defined as mPAP greater than 20 mm Hg at rest and pre-capillary PH requiring PAWP ≤15 mm Hg and PVR >2 Wood units. Performed in experienced centers, it also enables vasoreactivity testing to identify responders to . , involving catheter-directed contrast injection, is reserved for confirming vascular blockages in suspected acute or chronic PE when non-invasive imaging is equivocal, particularly to delineate thrombi for potential intervention. Non-invasive adjuncts complement imaging in targeted scenarios. Ventilation-perfusion (V/Q) detects PE by identifying defects, with a high-probability scan defined by two or more large segmental mismatches and a normal scan effectively ruling out chronic thromboembolic with 98% negative predictive value. (ECG) reveals indirect signs of pulmonary artery pressure overload, such as , RV , or strain patterns in the precordial leads, supporting suspicion of in symptomatic patients. Biomarkers aid in risk assessment and screening for complications. B-type natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP) levels reflect RV strain, with thresholds below 50 ng/L for BNP or 300 ng/L for NT-proBNP indicating low risk in PH, while elevations above 800 ng/L or 1,100 ng/L, respectively, signal high risk and correlate with hemodynamic severity and . D-dimer, a , assesses thrombosis risk in PE, with elevated levels correlating with disease severity and poor outcomes, though it lacks specificity and is best used to rule out chronic thromboembolic disease post-PE. The ESC/ERS guidelines recommend a stepwise diagnostic : initiate with for probability (low, intermediate, or high based on TRV and echocardiographic signs), followed by comprehensive evaluation including V/Q scanning for chronic PE suspicion and RHC for confirmation in intermediate- or high-probability cases. This approach ensures early detection of pulmonary artery disorders while minimizing unnecessary invasive procedures.

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