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

Kadı, Hasan; Kurtoğlu, Nuri; Karadağ, Bilgehan

doi:10.1159/000099101

Cited by 19 publications

(20 citation statements)

References 28 publications

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“…The association of a UPA and coronary-to-bronchial artery collateralization has been described before. [6,7] One reports a case complicated with coronary steal and myocardial ischemia in an elderly patient, as in the present case. [7] Atrial fibrillation caused by an enlarged left atrium due to excess amount of blood flow from the collateral arteries is another rare complication of UPA.…”

supporting

confidence: 52%

“…[6,7] One reports a case complicated with coronary steal and myocardial ischemia in an elderly patient, as in the present case. [7] Atrial fibrillation caused by an enlarged left atrium due to excess amount of blood flow from the collateral arteries is another rare complication of UPA. There are no cases in the literature regarding the development of atrial fibrillation as a result of coronary to bronchial collateralization.…”

supporting

confidence: 52%

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Congenital absence of left pulmonary artery with collateralization from all major coronary arteries

Baştuğ¹,

Aslan²,

Sarı³

et al. 2016

Arch Turk Soc Cardiol

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Özet-Tek taraflı pulmoner arter agenezisi nadir görülen bir doğumsal anomalidir ve çeşitli klinik bulgular ve semptomlara sahiptir. Yüksek ölüm oranları nedeniyle hastaların çoğu erişkin yaşa ulaşamadığından çoğunlukla çocukluk çağında tanı konur. Bu yazıda, kliniğimize egzersizle artan göğüs ağrısı, nefes darlığı ve çarpıntı şikayeti ile başvuran ve üç normal majör koroner arterden kollateral dolaşımla beslenen hipoplastik sol akciğer sol pulmoner arter agenezisi ve atriyal fibrilasyon tanısı konan 71 yaşında bir kadın hasta sunuldu.Summary-Unilateral pulmonary artery agenesis (UPA) is a rarely-observed congenital anomaly with diverse clinical signs and symptoms. It is most often diagnosed in childhood due to the high mortality rate prior to adulthood. This report describes the case of a 71-year-old woman who presented with exertional chest pain, dyspnea and palpitations and who was diagnosed as having left pulmonary artery (LPA) agenesis, hypoplastic left lung supplied by collateralization from each of the three normal major coronary arteries, and atrial fibrillation. 240U nilateral pulmonary artery agenesis (UPA) is a very rare congenital malformation which may present as recurrent pulmonary infections and hemoptysis. Most UPA patients develop pulmonary hypertension (PHT). Due to the high mortality rate in childhood, patients surviving to adulthood are rarely encountered. However, as in the present case, because of atypical symptoms, some patients are diagnosed at an older age.This report presents a 71-year-old female patient with left pulmonary artery (LPA) agenesis and an angiographically documented collateralization from each of the three major coronary arteries causing angina pectoris via coronary steal phenomenon. CASE REPORTA 71-year-old female patient was admitted with symptoms of exertional dyspnea, angina and palpitations. Past history was unremarkable. Physical examination showed irregular beats and a 2/6 pansystolic murmur audible at apical focus. Electrocardiography revealed atrial fibrillation with a ventricular rate of 118 beats per minute (Figure 1). Transthoracic echocardiography showed normal systolic function of the left ventricle, moderate mitral and tricuspid regurgitation with a systolic pulmonary artery pressure (sPAB) of 75 mmHg. Also, left atrial dilatation (57 mm) was detected. Coronary angiography revealed collateralization from the proximal segments of each of the three coronary arteries: the left anterior descending (LAD) artery, the circumflex (Cx) artery and the right coronary artery (RCA), into the left hypoplastic lung without any stenosis in epicardial coronary arteries (Figure 2). In order to define the exact anatomic structure of the heart, magnetic resonance angiography (MRA)

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supporting

confidence: 52%

supporting

confidence: 52%

Congenital absence of left pulmonary artery with collateralization from all major coronary arteries

Baştuğ¹,

Aslan²,

Sarı³

et al. 2016

Arch Turk Soc Cardiol

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“…Two of them reported absence of the right pulmonary artery,19 21 while the other two reported absence of the left pulmonary artery 18 20. In three of the four published cases there was no myocardial perfusion defect 18–20.…”

Section: Discussionmentioning

confidence: 88%

“…To our knowledge the only two cases reported the UAPA with impairment of the coronary perfusion was published by Kadi et al , and this was in a 65-year-old woman,21 and De Dominicis et al when they described a 44-year-old female who presented a congenital right pulmonary artery agenesis associated a coronary-to-bronchial artery collateralisation with an aneurysm at this level. It gives rise to a coronary syndrome by coronary steal 22.…”

Section: Discussionmentioning

confidence: 94%

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Myocardial infarction due to coronary steal caused by a congenital unilateral absence of the right pulmonary artery: a rare case

Soliman

Jelani²,

Eid³

et al. 2012

Case Reports

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Age-Related Clinical Characteristics of Isolated Congenital Unilateral Absence of a Pulmonary Artery

et al. 2010

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Unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although UAPA has been reported previously, its age-related pathogenesis and symptoms remain unclear. This retrospective cohort study included cases of UAPA reported in Japan at medical meetings or in the literature from 1990 through 2009. Patients with other congenital cardiac defects were excluded from the study. Clinical status was assessed according to age, and the clinical course of patients with isolated UAPA was compared with that of patients who had UAPA with a patent ductus arteriosus (PDA). Of the 92 patients with UAPA identified, 78 had isolated UAPA (14 with PDA). Hemoptysis and collateral arteries were observed in 0 and 13% of patients with isolated UAPA who were younger than 1 year, as compared with 24 and 50% of those 20 years of age or older, respectively. Pulmonary hypertension was present in 5% of the patients aged 1 to 19 years. Among patients 20 years or older, however, 32% had pulmonary hypertension, and 8% died. Compared with isolated UAPA, UAPA with PDA was associated with an earlier diagnosis (median age, 20 vs. 0 years; p = 0.002), a higher prevalence of pulmonary hypertension (22% vs. 86%; p < 0.0001), and a higher mortality rate (4% vs. 21%; p = 0.046). Collateral artery formation and pulmonary hypertension progress with age in patients with UAPA. Early diagnosis and revascularization may prevent the age-related progression of UAPA.

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

Cited by 19 publications

References 28 publications

Congenital absence of left pulmonary artery with collateralization from all major coronary arteries

Congenital absence of left pulmonary artery with collateralization from all major coronary arteries

Myocardial infarction due to coronary steal caused by a congenital unilateral absence of the right pulmonary artery: a rare case

Age-Related Clinical Characteristics of Isolated Congenital Unilateral Absence of a Pulmonary Artery

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