Pulmonary artery agenesis associated with coronary collaterals among adults

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Unilateral agenesis of the pulmonary artery is a rare congenital anomaly, which commonly involves the right side. Cases are associated with systemic collaterals, that may also rarely arise from the coronary arteries.

Two adult patients are presented with a right pulmonary artery agenesis associated with collaterals from the right coronary artery. The implications of such an anomaly on pulmonary artery pressure and lung pathology differs among both cases. The association of coronary collaterals is rare and its implication is variable among various patients.

Related collections

Most cited references 24

Record: found
Abstract: found
Article: not found

Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.

Jaap Ottenkamp, Marc A G J Ten Dam, Nico A Blom (2002)

The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

0 comments Cited 59 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

The varied manifestation of pulmonary artery agenesis in adulthood.

K Tsintiris, P Pare, P Panagou … (1995)

Unilateral pulmonary artery agenesis (UPAA), a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities, is usually diagnosed in childhood. Most patients who have no associated cardiac anomalies have only minor or absent symptoms and survive into adulthood. The conditions of such patients are frequently misdiagnosed in adulthood. In this report, we describe six patients with UPAA in whom the diagnosis was first established in adulthood. The varied clinical presentation of these patients is reviewed and the relative effectiveness of a variety of diagnostic tests is compared. During the period January 1987 through December 1990, six male patients, aged 17 to 20 years, were found to have UPAA at the time of their medical screening for enrollment into the armed forces. The diagnosis was based on history, clinical and imaging examinations, including chest radiography, ventilation-perfusion lung scan, digital subtraction angiography (DSA), computed tomography (CT), and magnetic resonance imaging (MRI). In four of the patients, the UPAA was on the left side and in two it was on the right. A right aortic arch was present in three patients and other cardiovascular anomalies were found in three. Pulmonary function studies showed a mild restrictive pattern in four. In contrast to previous reports, the ventilation scan showed a diminished "wash in" and "equilibrium" phase without a delayed "wash out" phase on the affected side in all patients. Selective bronchography through the fiberoptic bronchoscope revealed ipsilateral mixed-type bronchiectasis in two of four patients studied, a finding of clinical significance that has not been described previously. In all cases, the diagnosis was made by DSA. CT of the thorax (n = 6) and MRI (n = 4) were diagnostic in all cases in which they were performed, but added no significant information. UPAA is frequently misdiagnosed in adulthood and is often not considered in the differential diagnosis of the unilateral hyperlucent lung. Clinicians and radiologists should be aware of the possibility of undiagnosed cases in adults, with many atypical characteristics.

0 comments Cited 53 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.

Kenneth E Rosenzweig, M Kerstein, R Barst (1999)

Although long-term prostacyclin (PGI2) has been shown to improve hemodynamics, quality of life, and survival in patients with primary pulmonary hypertension, its use in patients with pulmonary hypertension (PHT) and associated congenital heart defects (CHD) has not been evaluated. Twenty patients (15+/-14 years) with PHT and associated CHD (9 atrial septal defect, 7 ventricular septal defect, 4 transposition of the great vessels, 3 patient ductus arteriosus, 3 partial anomalous pulmonary venous return, and 1 aortopulmonary window) who failed conventional therapy (including digitalis; diuretics; oxygen; warfarin; calcium channel blockade, if indicated; and surgery, if operable) were treated with chronic PGI2. Eleven patients had previous cardiac surgery at a median age of 3 years (range, 5 days to 47 years). Eleven of 20 patients had residual systemic to pulmonary shunts. Hemodynamics, NYHA functional class, and exercise capacity were measured at baseline and after 1 year of PGI2 therapy. None of the patients acutely responded to PGI2 administration. Despite lack of an acute response, mean pulmonary artery pressure decreased 21% on chronic PGI2: 77+/-20 to 61+/-15 mm Hg (P<0.01, n=16). Cardiac index and pulmonary vascular resistance also improved on long-term PGI2: 3. 5+/-2.0 to 5.9+/-2.7 L. min-1. m-2 (P<0.01, n=16), and 25+/-13 to 12+/-7 U.m2 (P<0.01, n=16), respectively. NYHA functional class improved from 3.2+/-0.7 to 2.0+/-0.9 (P<0.0001, n=19). Exercise capacity increased from 408+/-149 to 460+/-99 m (P=0.13, n=14) on long-term PGI2. Chronic PGI2 improves hemodynamics and quality of life in patients with PHT and associated CHD who fail conventional therapy. As previously demonstrated in patients with primary pulmonary hypertension, long-term PGI2 may have an important role in the treatment of patients with PHT and associated CHD.

0 comments Cited 49 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Ahmad K. Darwazah: +9720595023686 , darwaz30@hotmail.com

Imad A. Alhaddad: alhaddad63@hotmail.com

Journal

Journal ID (nlm-ta): J Cardiothorac Surg

Journal ID (iso-abbrev): J Cardiothorac Surg

Title: Journal of Cardiothoracic Surgery

Publisher: BioMed Central (London )

ISSN (Electronic): 1749-8090

Publication date (Electronic): 16 July 2016

Publication date PMC-release: 16 July 2016

Publication date Collection: 2016

Volume: 11

Electronic Location Identifier: 109

Affiliations

[ ]Department of Cardiac Surgery, Ramallah and Makassed Hospital, Mount of Olives, Postal Code 91194 Jerusalem, Israel

[ ]Department of Cardiology, Jordan Hospital, Amman, Jordan

Article

Publisher ID: 504

DOI: 10.1186/s13019-016-0504-1

PMC ID: 4947334

PubMed ID: 27422770

SO-VID: 3bec503c-651e-4418-97d0-08b0e035b759

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 1 April 2016

Date accepted : 6 July 2016

Custom metadata

ScienceOpen disciplines: Surgery

Keywords: pulmonary artery agenesis,congenital heart disease,coronary artery collaterals,systemic collaterals

Data availability:

ScienceOpen disciplines: Surgery

Keywords: pulmonary artery agenesis, congenital heart disease, coronary artery collaterals, systemic collaterals

Pulmonary artery agenesis associated with coronary collaterals among adults

Read this article at

Abstract

Related collections

25th European Students' Conference

Most cited references 24

Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.

The varied manifestation of pulmonary artery agenesis in adulthood.

Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.

Author and article information

Contributors

Journal

Affiliations

Article

History

Categories

Custom metadata

Comments

Comment on this article

Similar content 689

Cited by 3

Most referenced authors 219