Isolated Unilateral Absence of a Pulmonary Artery

Harkel, Arend D J Ten; Blom, Nico A.; Ottenkamp, Jaap

doi:10.1378/chest.122.4.1471

Cited by 267 publications

(145 citation statements)

References 57 publications

(72 reference statements)

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“…Shortness of breath on exercise and diminished exercise tolerance are also common symptoms. Due to the nonspecific character of the symptoms, most of the UPAA patients remain unrecognized until advanced ages, and they receive numerous erroneous diagnoses [5, 16, 17], leading to a delay of almost 30 years between onset of symptoms and final diagnosis [18]. Left UPAA is usually recognized early in childhood because of the high frequency of accompanying cardiovascular abnormalities [15].…”

Section: Discussionmentioning

confidence: 99%

Congenital Absence of the Right Pulmonary Artery with Coronary Collaterals Supplying the Affected Lung: Effect on Coronary Perfusion

2007

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We describe a unique case of congenital absence of a right pulmonary artery presenting in a patient of advanced age and initially misdiagnosed as coronary artery disease. Perfusion of the affected lung was accomplished via anomalous collaterals from right and left circumflex coronary arteries which induced myocardial ischemia, as demonstrated by myocardial perfusion scan. To our knowledge there are only three reports in the international literature, describing unilateral pulmonary artery agenesis with the coronary artery supplying the abnormal lung. All these reports described that the existence of such vessels does not affect the myocardial perfusion. However, here we describe, to our knowledge, for the first time that in a patient with unilateral pulmonary artery agenesis, the existence of collaterals from the coronary arteries to the affected lung can actually have a negative effect in myocardial perfusion and can induce myocardial ischemia. In conclusion, clinicians should be aware of the possibility of undiagnosed cases of unilateral pulmonary artery agenesis presenting with chest pain in advanced age.

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Section: Discussionmentioning

confidence: 99%

Congenital Absence of the Right Pulmonary Artery with Coronary Collaterals Supplying the Affected Lung: Effect on Coronary Perfusion

2007

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“…Its prevalence as an isolated lesion is estimated at 1 in 200 000‐to‐300 000 adults,10, 18, 19, 20 and 80% of reported cases involving the left PA have been associated with coexisting CHD, such as TOF or truncus arteriosus 10, 19. In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21.…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

“…In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21. PH is present in 44% of cases and, in conjunction with the underlying CHD, affects appropriate management and outcomes for these patients 18, 19, 22. PH may occur as a result of increased flow to the “healthy” lung, or in the “disconnected” lung supplied by large collaterals or a large PDA (Figure 4).…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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“…As the patients get older, they may develop a variety of symptoms, including pulmonary hypertension [4]. The incidence of the latter has been reported to range between 19% to 44% [9, 10]. Pulmonary hypertension is caused by an increased blood flow in the contralateral pulmonary artery, which leads to a remodelling of the arterioles, responsible for increased vascular resistance.…”

Section: Discussionmentioning

confidence: 99%

Neonatal diagnosis of isolated absence of the right pulmonary artery: a case report and review of the literature

et al. 2018

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BackgroundUnilateral absence of the pulmonary artery (UAPA) is a rare congenital malformation often associated with other cardiac anomalies; however it may occur as an isolated lesion. Isolated absence of the right pulmonary artery is twice more frequent than that of the left pulmonary artery. Patients with isolated UAPA are usually asymptomatic at birth; thereafter they may develop a progression of symptoms such as exercise intolerance, dyspnea, chest pain, hemoptysis and recurrent pulmonary infections. As patients may remain asymptomatic or have vague symptoms, the diagnosis of isolated UAPA can be difficult to make in infancy. Indeed, most cases described in literature are adults. Due to the rarity of neonatal presentation, there is no consensus regarding the treatment of this malformation.Case presentationHerein, the case of a two-day-old term female infant, born after uneventful pregnancy, who required a cardiological assessment for a light murmur, is reported; an echocardiogram demonstrated an isolated unilateral absence of the right pulmonary artery, confirmed by means of magnetic resonance imaging (MRI) performed 1 month after child’s birth. Besides this finding, MRI showed a slightly increased lumen and size of the main and left pulmonary arteries. The right lung was shown to be perfused by some systemic collateral arteries. In the absence of any other cardiovascular malformation, our patient did not need any treatment. As symptoms may occur later in life, a thorough clinical and cardiological follow up was immediately started. Three years later, she is still asymptomatic, showing adequate growth, without any sign of pulmonary hypertension.ConclusionsIsolated UAPA is a very rare malformation with a diverse clinical presentation. To the best of our knowledge, this is the second case of neonatal presentation of UAPA reported in literature to date. We believe that our case report supports the opinion that a prompt cardiological evaluation is needed whenever a newborn shows signs and/or symptoms of cardiorespiratory concern. Any missed neonatal diagnosis of UAPA may contribute to the later age at presentation, with resultant higher risk of morbidity and mortality and greater therapeutical difficulties.

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Isolated Unilateral Absence of a Pulmonary Artery

Cited by 267 publications

References 57 publications

Congenital Absence of the Right Pulmonary Artery with Coronary Collaterals Supplying the Affected Lung: Effect on Coronary Perfusion

Congenital Absence of the Right Pulmonary Artery with Coronary Collaterals Supplying the Affected Lung: Effect on Coronary Perfusion

Definition and Management of Segmental Pulmonary Hypertension

Neonatal diagnosis of isolated absence of the right pulmonary artery: a case report and review of the literature

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