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Absent pulmonary artery

MedGen UID:
120560
•Concept ID:
C0265905
•
Congenital Abnormality
Synonyms: Absence of the pulmonary artery; Pulmonary artery agenesis
SNOMED CT: Agenesis of pulmonary artery (86252004); Congenital absence of pulmonary artery (86252004); Pulmonary artery absent (86252004); Pulmonary artery agenesis (86252004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0004960
Monarch Initiative: MONDO:0020007
Orphanet: ORPHA980

Definition

A congenital defect with aplasia (absence) of one of the right or left pulmonary artery. [from HPO]

Conditions with this feature

Acrocardiofacial syndrome
MedGen UID:
324947
•Concept ID:
C1838121
•
Disease or Syndrome
A rare genetic disorder characterised by split-hand/split-foot malformation (SHFM), facial anomalies, cleft lip/palate, congenital heart defect (CHD), genital anomalies, and intellectual deficit.
See: Condition Record
Osteogenesis imperfecta type 7
MedGen UID:
343981
•Concept ID:
C1853162
•
Disease or Syndrome
Osteogenesis imperfecta is a connective tissue disorder characterized by bone fragility and low bone mass. OI type VII is an autosomal recessive form of severe or lethal OI (summary by Barnes et al., 2006).
See: Condition Record
Acrocardiofacial syndrome
Osteogenesis imperfecta type 7

Professional guidelines

PubMed

Successful treatment of pulmonary hypertension with unilateral absent pulmonary artery.
Horinouchi K, Ueno K, Nakae K, Kawamura J, Kawano Y
Pediatr Int 2020 Sep;62(9):1117-1118. Epub 2020 Sep 7 doi: 10.1111/ped.14262. PMID: 32893928

Recent clinical studies

Etiology

Rehabilitation of pulmonary arteries: Hybrid treatment in the unilateral absence of the pulmonary artery.
Cervantes-Salazar JL, García-Montes JA, Peralta-Santos H, Ortega-Zhindón DB, Calderón-Colmenero J
J Card Surg 2021 Aug;36(8):2970-2973. Epub 2021 May 11 doi: 10.1111/jocs.15603. PMID: 33974289
Using the autologous innominate vein as a substitute for pulmonary arteries in a patient with pulmonary atresia and absent pulmonary arteries.
Amirghofran AA, Jamshidi K, Edraki M, Amoozgar H, Peiravian F, Nirooei E
J Cardiothorac Surg 2021 Apr 15;16(1):89. doi: 10.1186/s13019-021-01489-9. PMID: 33858447Free PMC Article
Early single-stage surgical revascularization of pulmonary artery in unilateral absence of a pulmonary artery.
Li W, Ma L, Xia S, Zou M, Chen W, Chen X
J Cardiothorac Surg 2021 Apr 13;16(1):80. doi: 10.1186/s13019-021-01481-3. PMID: 33849614Free PMC Article
Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery.
Edraki M, Ghasemzadeh B, Keshavarz K, Amirghofran A, Mohammadi H, Kheirandish Z, Amoozgar H, Nirooei E, Ajami G, Mehdizadegan N, Naghshzan A, Peiravian F, Cheriki S, Nobahkti MJ
BMC Cardiovasc Disord 2021 Jan 28;21(1):56. doi: 10.1186/s12872-021-01877-y. PMID: 33509091Free PMC Article
Current surgical therapy for bronchiectasis.
Ashour M, Al-Kattan K, Rafay MA, Saja KF, Hajjar W, Al-Fraye AR
World J Surg 1999 Nov;23(11):1096-104. doi: 10.1007/s002689900630. PMID: 10501869

Diagnosis

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review.
Kucirka LM, Desai S, LeVarge B, Conklin JL, Leong ME, Quist-Nelson J
Am J Perinatol 2024 May;41(S 01):e3276-e3285. Epub 2023 Nov 23 doi: 10.1055/a-2217-0241. PMID: 37995742
Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery.
Edraki M, Ghasemzadeh B, Keshavarz K, Amirghofran A, Mohammadi H, Kheirandish Z, Amoozgar H, Nirooei E, Ajami G, Mehdizadegan N, Naghshzan A, Peiravian F, Cheriki S, Nobahkti MJ
BMC Cardiovasc Disord 2021 Jan 28;21(1):56. doi: 10.1186/s12872-021-01877-y. PMID: 33509091Free PMC Article
Segmental Pulmonary Hypertension in Children with Congenital Heart Disease.
Das BB, Frank B, Ivy D
Medicina (Kaunas) 2020 Sep 24;56(10) doi: 10.3390/medicina56100492. PMID: 32987636Free PMC Article
Tetralogy of Fallot with unilateral absent pulmonary artery.
Ugurlucan M, Arslan AH, Yildiz Y, Ay S, Besikci RT, Cicek S
Cardiol Young 2013 Jun;23(3):423-30. Epub 2012 Jul 18 doi: 10.1017/S1047951112000911. PMID: 22805480
Diagnosis and surgical treatment of "angiographically absent pulmonary artery syndrome".
Ishizawa E, Horiuchi T, Tadokoro M, Okada Y, Suzuki Y, Tanaka S, Takamiy M, Sato T, Kano I
Tohoku J Exp Med 1978 May;125(1):1-9. doi: 10.1620/tjem.125.1. PMID: 675672

Therapy

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery.
Edraki M, Ghasemzadeh B, Keshavarz K, Amirghofran A, Mohammadi H, Kheirandish Z, Amoozgar H, Nirooei E, Ajami G, Mehdizadegan N, Naghshzan A, Peiravian F, Cheriki S, Nobahkti MJ
BMC Cardiovasc Disord 2021 Jan 28;21(1):56. doi: 10.1186/s12872-021-01877-y. PMID: 33509091Free PMC Article

Prognosis

Using the autologous innominate vein as a substitute for pulmonary arteries in a patient with pulmonary atresia and absent pulmonary arteries.
Amirghofran AA, Jamshidi K, Edraki M, Amoozgar H, Peiravian F, Nirooei E
J Cardiothorac Surg 2021 Apr 15;16(1):89. doi: 10.1186/s13019-021-01489-9. PMID: 33858447Free PMC Article
Early single-stage surgical revascularization of pulmonary artery in unilateral absence of a pulmonary artery.
Li W, Ma L, Xia S, Zou M, Chen W, Chen X
J Cardiothorac Surg 2021 Apr 13;16(1):80. doi: 10.1186/s13019-021-01481-3. PMID: 33849614Free PMC Article
Tetralogy of Fallot with unilateral absent pulmonary artery.
Ugurlucan M, Arslan AH, Yildiz Y, Ay S, Besikci RT, Cicek S
Cardiol Young 2013 Jun;23(3):423-30. Epub 2012 Jul 18 doi: 10.1017/S1047951112000911. PMID: 22805480
Current surgical therapy for bronchiectasis.
Ashour M, Al-Kattan K, Rafay MA, Saja KF, Hajjar W, Al-Fraye AR
World J Surg 1999 Nov;23(11):1096-104. doi: 10.1007/s002689900630. PMID: 10501869
Hemodynamic alterations in bronchiectasis: a base for a new subclassification of the disease.
Ashour M
J Thorac Cardiovasc Surg 1996 Aug;112(2):328-34. doi: 10.1016/S0022-5223(96)70258-1. PMID: 8751499

Clinical prediction guides

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review.
Kucirka LM, Desai S, LeVarge B, Conklin JL, Leong ME, Quist-Nelson J
Am J Perinatol 2024 May;41(S 01):e3276-e3285. Epub 2023 Nov 23 doi: 10.1055/a-2217-0241. PMID: 37995742
Tetralogy of Fallot with unilateral absent pulmonary artery.
Ugurlucan M, Arslan AH, Yildiz Y, Ay S, Besikci RT, Cicek S
Cardiol Young 2013 Jun;23(3):423-30. Epub 2012 Jul 18 doi: 10.1017/S1047951112000911. PMID: 22805480
Current surgical therapy for bronchiectasis.
Ashour M, Al-Kattan K, Rafay MA, Saja KF, Hajjar W, Al-Fraye AR
World J Surg 1999 Nov;23(11):1096-104. doi: 10.1007/s002689900630. PMID: 10501869
Hemodynamic alterations in bronchiectasis: a base for a new subclassification of the disease.
Ashour M
J Thorac Cardiovasc Surg 1996 Aug;112(2):328-34. doi: 10.1016/S0022-5223(96)70258-1. PMID: 8751499

Recent systematic reviews

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review.
Kucirka LM, Desai S, LeVarge B, Conklin JL, Leong ME, Quist-Nelson J
Am J Perinatol 2024 May;41(S 01):e3276-e3285. Epub 2023 Nov 23 doi: 10.1055/a-2217-0241. PMID: 37995742

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