Unilateral absence of pulmonary artery: Pathophysiology,
symptoms, diagnosis and current treatment

J 2013

Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment

KRUŽLIAK, Peter; Rao P. SYAMASUNDAR; Miroslav NOVÁK; Olga PECHANOVA; Gabriela KOVACOVA et al.

Základní údaje

Originální název

Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment

Autoři

KRUŽLIAK, Peter; Rao P. SYAMASUNDAR; Miroslav NOVÁK; Olga PECHANOVA a Gabriela KOVACOVA

Vydání

ARCHIVES OF CARDIOVASCULAR DISEASES, MILANO, ELSEVIER MASSON, 2013, 1875-2136

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30201 Cardiac and Cardiovascular systems

Stát vydavatele

Itálie

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 1.662

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/13:00071659

Organizační jednotka

Lékařská fakulta

DOI

https://doi.org/10.1016/j.acvd.2013.05.004

UT WoS

000324722000005

Klíčová slova anglicky

Congenital heart disease; Unilateral absence of pulmonary artery; Pathophysiology; Symptoms; Treatment

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 15. 4. 2014 16:19, Ing. Mgr. Věra Pospíšilíková

Anotace

V originále

Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.

Citovat

KRUŽLIAK, Peter; Rao P. SYAMASUNDAR; Miroslav NOVÁK; Olga PECHANOVA a Gabriela KOVACOVA. Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. ARCHIVES OF CARDIOVASCULAR DISEASES. MILANO: ELSEVIER MASSON, 2013, roč. 106, 8-9, s. 448-454. ISSN 1875-2136. Dostupné z: https://doi.org/10.1016/j.acvd.2013.05.004.

@article{1162069,
   author = {Kružliak, Peter and Syamasundar, Rao P. and Novák, Miroslav and Pechanova, Olga and Kovacova, Gabriela},
   article_location = {MILANO},
   article_number = {8-9},
   doi = {https://doi.org/10.1016/j.acvd.2013.05.004},
   keywords = {Congenital heart disease; Unilateral absence of pulmonary artery; Pathophysiology; Symptoms; Treatment},
   language = {eng},
   issn = {1875-2136},
   journal = {ARCHIVES OF CARDIOVASCULAR DISEASES},
   title = {Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment},
   volume = {106},
   year = {2013}
}

TY  - JOUR
ID  - 1162069
AU  - Kružliak, Peter - Syamasundar, Rao P. - Novák, Miroslav - Pechanova, Olga - Kovacova, Gabriela
PY  - 2013
TI  - Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment
JF  - ARCHIVES OF CARDIOVASCULAR DISEASES
VL  - 106
IS  - 8-9
SP  - 448-454
EP  - 448-454
PB  - ELSEVIER MASSON
SN  - 18752136
KW  - Congenital heart disease
KW  - Unilateral absence of pulmonary artery
KW  - Pathophysiology
KW  - Symptoms
KW  - Treatment
N2  - Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.
ER  -

KRUŽLIAK, Peter; Rao P. SYAMASUNDAR; Miroslav NOVÁK; Olga PECHANOVA a Gabriela KOVACOVA. Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. \textit{ARCHIVES OF CARDIOVASCULAR DISEASES}. MILANO: ELSEVIER MASSON, 2013, roč.~106, 8-9, s.~448-454. ISSN~1875-2136. Dostupné z: https://doi.org/10.1016/j.acvd.2013.05.004.

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