Congenital unilateral absence of a pulmonary artery

Pool, Peter E.; Vogel, John H.K.; Blount, S. Gilbert

doi:10.1016/0002-9149(62)90248-5

Cited by 240 publications

(86 citation statements)

References 67 publications

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“…In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21. PH is present in 44% of cases and, in conjunction with the underlying CHD, affects appropriate management and outcomes for these patients 18, 19, 22. PH may occur as a result of increased flow to the “healthy” lung, or in the “disconnected” lung supplied by large collaterals or a large PDA (Figure 4).…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

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Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

“…PH may occur as a result of increased flow to the “healthy” lung, or in the “disconnected” lung supplied by large collaterals or a large PDA (Figure 4). 22, 23, 24 …”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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“…Extrapolating from clinical correlates, one could expect that pulmonary vascular changes would be more prominent in pigs with pneumonectomy plus A-V fistulas. Indeed, Pool's extensive study (9) on unilateral absence of a pulmonary artery shows that pulmonary vascular disease occurs in a small percentage when this lesion is isolated, but in a very high percentage when a left-to-right shunt is associated. Also, infants with a ventricular septal defect have a high risk of developing pulmonary vascular disease early in life if their pulmonary blood flow equals or exeeeds by 3 times the systemic blood flow (2).…”

Section: Discussionmentioning

confidence: 99%

The Effect of Increased Pulmonary Blood Flow on the Pulmonary Vascular Bed in Pigs

et al. 1975

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ExtractIncreased pulmonary blood flow was produced in 1-month-old piglets by means of left pneumonectomy, arteriovenous fistulas in the neck, and a combination of both. Physiologic and histologic studies of the pulmonary vascular bed were done 1-9 months after operation.A progressive, moderate increase in pulmonary artery ( P A ) pressure was observed, especially between 1 and 6 months after surgery. This was flow related, 1.e.. the group with the highest flow (pneumonectomy plus fistula) was found to have the most prominent increase in pressure. Mean PA pressure at 6 months was 28.7 ~t 0.07 mm H g in this group, vs 24.4 0.48 mm H g in the group with pneumonectomy alone and 17.2 ~t 0.48 mm H g in controls ( P < 0.01). The pressure response to hypoxia in pigs with high pulmonary blood flow was not different from that found in control animals.Histologic studies revealed that small arteries and arterioles of pigs with high pulmonary blood flow had a decreased relative wall thickness because of dilation up to 6 months follow-up. This was flow related, the group with the highest flow having the lowest wall thickness to vessel diameter ratio; relative wall thickness (in percentage of the vessel diameter) at 6 months was 6.1 s= 0.44% in pigs with pneumonectomy plus fistula. vs 9.6 0.40% in the group with pneumonectomy alone and 11.2 * 0.61% in controls ( P < 0.01). In the group with the highest flow, thick walled arterioles appeared at 9 months follow-up, scattered among dilated ones; mean medial wall thickness in these animals increased significantly between 6 and 9 months after operation, ranging from 6.1 =t 0.44% to 11.3 + 0.73% (P < 0.01).In five animals with high flow, the right PA (main branch) showed patchy intimal thickening, small cystic spaces filled with mucopolysaccharides in the media, and muscular hypertrophy. SpeculationSome changes usually seen in pulmonary hypertension were produced in this model by high pulmonary blood flow in the presence of only mild to moderate elevation of P A pressure. The dilation observed in the pulmonary arteries and arterioles may have been a pathogenetic factor; indeed, it is likely that wall tension rather than intravascular pressure induces vascular changes; wall tension (according to Laplace's law) depends on vessel diameter as well as intravascular pressure. Therefore, in a dilated vascular bed, wall tension will increase considerably with only moderate increase in PA pressure, inducing changes usually seen with more severe degrees of pulmonary hypertension.Pulmonary vascular disease remains a serious complication of congenital heart disease with a left-to-right shunt. Little is known about the pathogenesis of this condition, but three factors are believed to be involved: increased pulmonary blood flow, a raised pulmonary artery pressure, and high left atrial pressure.Experimental models used to study pulmonary vascular disease often involve anastomoses between aorta and pulmonary artery, whereby two if not all three of the above mentioned factors are produced (1, 4).In ...

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“…The left pulmonary artery and the right pulmonary artery are absent with approximately equal frequency. Absence of the left pulmonary artery is most often associated with tetralogy of Fallot [1] and can be associated with right-sided aortic arch [2]. Unilateral absence of the right pulmonary artery is isolated in approximately 50% of cases, and if associated with an anomaly is most often associated with patent ductus arteriosus [1].…”

Section: Case Reportmentioning

confidence: 99%

“…Absence of the left pulmonary artery is most often associated with tetralogy of Fallot [1] and can be associated with right-sided aortic arch [2]. Unilateral absence of the right pulmonary artery is isolated in approximately 50% of cases, and if associated with an anomaly is most often associated with patent ductus arteriosus [1]. Defects associated with absence of either artery include atrial and ventricular septal defects, coarctation of the aorta, aorticopulmonary window, truncus arteriosus, and, in one instance, supracardiac total anomalous pulmonary ventricular return [1 , 2].…”

Section: Case Reportmentioning

confidence: 99%