Surgical Management Of Absent Right Pulmonary Artery With Associated Pulmonary Hypertension

Toews, Warren H.; Pappas, George

doi:10.1378/chest.84.4.497

Cited by 50 publications

(32 citation statements)

References 10 publications

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“…Although approximately 30% of the patients with pulmonary artery agenesis may remain asymptomatic during their lifetime, they can present with serious pulmonary hypertension and congestive heart failure (7,8 Table 1. General features of cases as destructive as those in necrotizing pneumonia (9).…”

Section: Discussionmentioning

confidence: 99%

“…In the lung of the affected side, pulmonary arteries end as blind at the hilus and the blood support to the lung comes primarily from main bronchial arteries and then intercostal, internal mammarian, subclavian, and transpleural branches of the innominate arteries. Hemoptysis develops because of the bleeding of the ectatic collateral circulation exposed to hypertrophy into the submucosa of the bronchioles (3,8). Hemoptysis can be massive and fatal, especially in adults (13,14).…”

Section: Discussionmentioning

confidence: 99%

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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: 99%

Section: Discussionmentioning

confidence: 99%

Pulmonary Artery Agenesis: A Case Series

Ağca¹,

Bulum²,

Günen³

et al. 2015

Eurasian J Pulmonol

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“…This procedure, using other connective materials, had only been conducted in eight previous cases 2, [9][10][11][12][13][14] . Pneumectomies or lobectomies to control hemoptysis were performed on nine other patients from the 108 cases reported earlier, for a total of 17 (7%) therapeutic procedures for this anomality between 1978 and 2000 1 .…”

Section: Discussionmentioning

confidence: 99%

“…Since 1978, approximately 119 cases of this anomaly have been reported [1][2][3][4][5][6][7][8][9][10][11][12][13][14] . In these case reports, pulmonary artery reconstruction was performed on nine patients 2,3,9-14 plus two other cases published in the 1960's 15,16 .…”

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confidence: 99%

Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva

Atik¹,

Tanamati²,

Kajita³

et al. 2006

Arq. Bras. Cardiol.

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objective: Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH) in children and hemoptysis in adults. Interventions such as pulmonary artery reconstruction and lobectomies were performed in 17% of the cases. We analyzed four of these cases, two in natural evolution and two with late term PH regression after surgical correction.Methods: Three 22,10 and 35 month old male children and one 20 month old female child were included in the study. The 22 month old presented right-sided heart failure (RHF) and cyanosis; the 10 month old presented RHF and the other two presented exertion fatigue. All had PH symptoms, right ventricular strain on the EKG and cardiomegaly. Cardiac catheterization showed systemic pressures in the contralateral pulmonary artery, with right-sided agenesis in three of the children and left-sided agenesis in one child.results: Surgical correction of pulmonary artery continuity was possible in the 22 month old and 10 month old using a 7mm diameter Goretex conduit between the pulmonary arteries up to the hypoplastic contralateral pulmonary hilum. There was early and late regression of the PH signs and the children remained stable during follow-up to the ages of 7 and 2.5 years, respectively. The pressure ratio between the left and right ventricles was 30 and 40%, in both cases. Pulmonary perfusion increased from 8 to 44% and from 8 to 23%, in the two cases. The same procedure was scheduled for the other patients. conclusion:This technique has become the operation of choice for similar cases, that are rarely described in literature, even in the presence of severe PH and contralateral pulmonary artery hypoplasia.

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“…It is caused by an embryogenic malformation of the 6 th aortic arch of the affected side (1,2). The first case was reported in 1868 and the estimated prevalence is 1/200.000 people (3)(4)(5).…”

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A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

Iliaz¹,

Aslan²,

Ozturk

et al. 2016

Tuberk Toraks

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Unilateral pulmonary artery agenesis (UPAA) is a rare condition. It is caused by an embryogenic malformation of the 6 th aortic arch of the affected side (1,2). The first case was reported in 1868 and the estimated prevalence is 1/200.000 people (3-5). Right-sided UPAA is twice as common as the left-sided (6). However, cardiac malformation is more common in the left sided agenesis (5). When cardiac malformation coexists with UPAA, the patients are more symptomatic and diagnosed during childhood (7). The most common symptoms in UPAA are recurrent pulmonary infections, hemoptysis, and dyspnea (6). If the agenesis is isolated, the symptoms may be minor or absent, the diagnosis can be made in the adulthood (5). A study by Bouros D. et al reported that 30% of the adult patients with UPAA haven't any symptoms (5). In this study, 75% of the left-sided UPAA was associated with congenital cardiac anomalies, whereas right-sided agenesis was not associated with any cardiac malformation (5). The most common associated cardiovascular abnormalities are tetralogy of Fallot, septal defects, patent ductus arteriosus, coarctation of the aorta and transposition of great vessels (4). Mitral valve prolapse (MVP) association with UPAA is very rare. In the literature, there are only two cases (5,8). To the best of our knowledge, it is the third reported right-sided UPAA case with coexisting MVP. Here, we present a rare case of right-sided UPAA and severe MVP diagnosed at adult age.A 43-year-old man was referred to pulmonary medicine outpatient clinic for investigation of dyspnea after the prosthetic ring valvuloplasti. He was on anti-hypertensive treatment for a year. One month prior to his routine evaluation for cardiology, he reported exertional dyspnea and orthopnea. He was non-smoker. Had no other systemic symptoms. On the physical examination he had 4/6 systolic murmur at mitral area. In the echocardiographic evaluation, there was grade 3-4 mitral regurgitation, mitral valve prolapse, and chordal rupture. Systolic pulmonary arterial pressure was 50 mmHg, the left ventricular ejection fraction was 65%. The coronary angiography was normal.A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

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Surgical Management Of Absent Right Pulmonary Artery With Associated Pulmonary Hypertension

Cited by 50 publications

References 10 publications

Pulmonary Artery Agenesis: A Case Series

Pulmonary Artery Agenesis: A Case Series

Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva

A rare case of right sided pulmonary artery agenesis associated with congenital mitral valve prolapse

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