Pulmonary Artery Agenesis Presenting as Massive Hemoptysis — A Case Report

Mehta, Atul C.; Livingston, Douglas R.; Kawalek, Wayne; Golish, Joseph A.; O'Donnell, James

doi:10.1177/000331978703800110

Cited by 29 publications

(23 citation statements)

References 10 publications

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“…If there are recurrent hemoptyses in the patient, embolization may be risky and pneumonectomy or lobectomy may be necessary. In the literature, cases that required resectional surgery at a young age, especially because of massive hemoptysis, were reported (15)(16)(17). In two of our cases, massive hemoptysis was observed; pneumonectomy was performed for one case and blood transfusion for the other.…”

Section: Discussionmentioning

confidence: 65%

Pulmonary Artery Agenesis: A Case Series

Ağca¹,

Bulum²,

Günen³

et al. 2015

Eurasian J Pulmonol

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Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis. The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature. Keywords: Angiography, echocardiography, pulmonary artery agenesis, vascular abnormalities INTRODUCTIONIsolated one-sided pulmonary artery agenesis is a rare congenital anomaly in the proximal short segment of the right or left pulmonary artery. Generally, the distal parts of the artery at the hilus are protected (1). It can occur with Fallot tetralogy, septal defects, or pulmonary stenosis as well as by itself. It can often be identified during childhood years, but it can hide symptoms until the late adult ages. When it presents, the symptoms are recurrent infections, hemoptysis, shortness of breath, restricted exercise, and chest pain are observed. The diagnosis is hard because of the lack of specific symptoms and rarity of the disease. It is important to diagnose this disease at early ages because it causes serious complications such as massive hemoptysis, pulmonary hypertension, respiratory failure, and bronchiectasis (2).The mortality rate of this rare anomaly is high because of its complications. We retrospectively analyzed five cases of pulmonary artery agenesis at older ages in accordance with the literature knowledge. CASE PRESENTATIONS Case1A 37-year-old male patient was admitted to our emergency department with complaints of fever, shortness of breath, yellow phlegm, and hemoptysis occurring occasionally. It was learned from his history that he had been treated for bronchiectasis. Because a left pulmonary artery shadow was not seen on posteroanterior (PA) chest X-ray (Figure 1a), echocardiography (ECO) was performed for vein malformation. A mild expansion in the right atrium and right ventricle and a systolic pulmonary artery pressure (PAPs) of 50 mmHg were observed. Chest computerized tomography (CT) (Figure 1b) revealed varicose and cystic bronchiectasis in the left lung, nearly total volume loss, rising in the left diaphragm, and replacement toward the left in mediastinal structures. In the spirometric examination, mild restrictive and moderate obstructive [FVC: 3.29 L (72% of expected), FEV 1 : 1.90 L (51% of expected), FEV 1 /FVC: 57.7% (not expected)] respiratory dysfunction was detected. In the bronchoscopic analysis of the patient, the mouth of the left upper lobe was found to be narrowed in the shape of a lip. The left lung was totally hypoplasic and hypoperfused on ventilation perfusion (V/Q) scintigraphy Figure 1c). Invasive pulmonary angiography of the patient ( Figure 1d) revealed that the left pulmonary artery was closed starting from the ostium level and the patien...

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Section: Discussionmentioning

confidence: 65%

Pulmonary Artery Agenesis: A Case Series

Ağca¹,

Bulum²,

Günen³

et al. 2015

Eurasian J Pulmonol

View full text Add to dashboard Cite

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“…In cases of hemoptysis, options include selective embolization and/or pneumonectomy [2]. Long-term follow-up after embolization does show the development of alternate vasculature and collaterals ultimately putting the patient at significant risk for recurrence of hemoptysis [7]. In general, the contribution of the affected lung to gas exchange is minor in this population, making a pneumonectomy a more attractive option.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary artery agenesis and Kommerell's diverticulum presenting with hemoptysis

Rousou

Tetenta

Boffa

2009

European Journal of Cardio-Thoracic Surgery

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Unilateral pulmonary artery agenesis is a rare congenital anomaly that is commonly associated with additional cardiovascular abnormalities. We report a case of intrapulmonary hemorrhage and hemoptysis in a patient with left-sided pulmonary artery agenesis as well as the first description of this anomaly in a patient with a diverticulum of Kommerell. The patient was successfully treated by performing a left pneumonectomy. #

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“…There is usually ipsilateral tracheal deviation, diaphragmatic elevation, narrowed intercostal spaces, and small hilum. A reticular pattern from the collateral circulation to the involved lung can, at times, be seen on plain chest film [4,8]. Scintigraphic studies will demonstrate complete absence of perfusion to one whole lung, with diminished ventilation but no delay in the wash-out phase of the ventilation scan [5].…”

Section: Discussionmentioning

confidence: 99%

“…Haemoptysis is most often inconsequential and results from hypertrophied collateral vessels [1,3,7]. Venules P-H. KUO ruptured because of the higher circulation pressures in the affected lung can cause haemoptysis of a potentially lethal magnitude [8,9]. Bronchiectasis has been reported in some patients, but its pathogenesis and association with recurrent infections remain unclear [5].…”

Section: Discussionmentioning

confidence: 99%

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A patient with haemoptysis and a smaller right lung

Kuo¹,

Wu²,

Lee³

1996

Eur Respir J

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A A p pa at ti ie en nt t w wi it th h h ha ae em mo op pt ty ys si is s a an nd d a a s sm ma al ll le er r r ri ig gh ht t l lu un ng g Case historyA 41-year-old businessman was admitted to National Taiwan University Hospital for evaluation of a 2-week history of haemoptysis productive of 50-100 mL of fresh blood daily. The patient stated that haemoptysis had first occurred 10 yrs earlier, had lasted for about 1 month and had spontaneously subsided. Both episodes of haemoptysis were preceded by flu-like symptoms. There was no past history of epistaxis, exertional dyspnoea, or loss of body weight.Physical examination revealed a well-developed man in no distress. His blood pressure was 112/70 mmHg, pulse 96 beats . min -l and respiratory rate 22 breaths . min -1 . There was a slightly decreased chest wall excursion, and breath sounds in the right hemithorax. No audible bruits were heard over the chest. Heart sounds were regular, without murmurs. No clubbing, cyanosis, telangiectasia or oedema were noted. Arterial blood gases in room air showed: a pH of 7.41; arterial carbon dioxide tension (Pa,CO 2 ) of 4.9 kPa (36.5 mmHg); and arterial oxygen tension (Pa,O 2 ) of 13.1 kPa (98.3 mmHg). The sputum was negative for acid-fast bacilli, fungi and malignant cells. An electrocardiogram was normal. Echocardiography showed normal cardiac size and good left ventricular contractility, with no evidence of congenital cardiac lesions or pulmonary hypertension. On bronchoscopy, no endobronchial lesion or definite site of bleeding was found.Imaging studies, including a posteroanterior (PA) chest radiograph ( fig. l), and computed tomography (CT) scan at the level of the aorticopulmonary window ( fig. 2) are shown.

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Pulmonary Artery Agenesis Presenting as Massive Hemoptysis — A Case Report

Cited by 29 publications

References 10 publications

Pulmonary Artery Agenesis: A Case Series

Pulmonary Artery Agenesis: A Case Series

Pulmonary artery agenesis and Kommerell's diverticulum presenting with hemoptysis

A patient with haemoptysis and a smaller right lung

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