Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review

Jariwala, Pankaj; Maturu, Venkata Nagarjuna; Christopher, Johann; Jadhav, Kartik

doi:10.1007/s12055-020-01032-w

Cited by 13 publications

(37 citation statements)

References 47 publications

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“…Patients with recurrent pulmonary infections and massive hemoptysis can be treated with lobectomy or pneumonectomy and selective embolization of systemic collaterals, respectively. 6 Patients in whom revascularization is difficult and with moderate-to-severe PAH causing symptoms can be managed with antipulmonary artery hypertension drugs as in our case. 7…”

Section: Rare Cause Of Secondary Pulmonary Arterial Hypertension Yada...mentioning

confidence: 84%

Rare Cause of Secondary Pulmonary Arterial Hypertension

Pasula

Karthikeya

Patnaik

et al. 2022

IJCDW

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Unilateral absent pulmonary artery (UAPA) is a rare congenital disorder. Most of the patients will be diagnosed in the early childhood because of recurrent respiratory tract infections and hemoptysis, but adult presentation is not uncommon. We report a 47-year-old female who was earlier diagnosed as bronchiectasis with pulmonary artery hypertension but now presented with complaints of typical angina and dyspnea on exertion. During right heart catheterization we failed to enter right pulmonary artery (RPA), and conventional coronary angiogram showed a large left atrial branch of left circumflex giving collateral blood supply to the right lung. Computed tomography angiogram showed absent RPA. We report this case not only because the patient was misdiagnosed but also because of rarity of coronary collaterals in UAPA patients and unusual bilateral bronchiectasis. According to reported literature, ours is the 28th case of this nature.

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Section: Rare Cause Of Secondary Pulmonary Arterial Hypertension Yada...mentioning

confidence: 84%

Rare Cause of Secondary Pulmonary Arterial Hypertension

Pasula

Karthikeya

Patnaik

et al. 2022

IJCDW

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“…The mediastinum tends to be shifted toward the affected side with the absence of hilar vasculature. 1 , 2 If suspicion is high enough, diagnosis can be made by CT, magnetic resonance imaging (MRI), or echocardiogram; however, the gold standard is pulmonary angiogram. 1 , 3 ECG is usually normal unless there is PH, in which case it would demonstrate RV dominance.…”

Section: Discussionmentioning

confidence: 99%

“… 4 , 7 The incidence of PH among patients with UAPA has been reported to be between 20% and 44%. 2 – 6 It is theorized that PH occurs from increased blood flow through the patent PA into one lung resulting in increased shear stress in the endothelium leading to injury. 1 , 7 Endothelin is then released subsequently vasoconstricting the pulmonary arterioles.…”

Section: Discussionmentioning

confidence: 99%

“…The first is diagnosed early in infancy with congestive cardiac failure or pulmonary hypertension (PH) and is usually associated with cardiovascular congenital abnormalities such tetralogy of Fallot, atrial septal defect, and truncus arteriosus. 2 Conversely, UAPA can have a delayed presentation in adulthood as an isolated finding without associated cardiovascular defects. 2 In adults, 13%–30% remain asymptomatic and are diagnosed incidentally on chest radiograph; however, some can present with hemoptysis, recurrent lung infections, and dyspnea on exertion due to PH.…”

Section: Introductionmentioning

confidence: 99%

“… 2 Conversely, UAPA can have a delayed presentation in adulthood as an isolated finding without associated cardiovascular defects. 2 In adults, 13%–30% remain asymptomatic and are diagnosed incidentally on chest radiograph; however, some can present with hemoptysis, recurrent lung infections, and dyspnea on exertion due to PH. 3 Literature regarding isolated UAPA is limited, making diagnostic and therapeutic plans challenging.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary hypertension in an adult with unilateral absence of left pulmonary artery

Ghazarian

King

Premyodhin

et al. 2022

SAGE Open Medical Case Reports

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Unilateral absence of pulmonary artery is a rare congenital abnormality that occurs due to malformation of the sixth aortic arch during embryonic development. The clinical presentation of unilateral absence of pulmonary artery can vary based on age of diagnosis; however, in the adult population, it can present with a variety of manifestations including hemoptysis, recurrent pneumonia, and pulmonary hypertension or as an incidental finding. Diagnosis and management of unilateral absence of pulmonary artery remain a challenge. Here, we describe a case of a 37-year-old female with no known past medical history who presented with progressively worsening dyspnea and fatigue. She was incidentally found to have unilateral absence of pulmonary artery on computerized tomography angiography of the chest. Her imaging and physical exam demonstrated signs of volume overload and severe pulmonary hypertension. She received diuretics with good response and was discharged with referral to pulmonary hypertension clinic and eventual follow-up with right heart catheterization. In summary, we describe a rare congenital condition and highlight its diagnostic and therapeutic challenges.

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The impact of unilateral pulmonary artery stenosis on right ventricular to pulmonary arterial coupling in patients with transposition of the great arteries

Joosen,

Voskuil,

Krings

et al. 2024

Cathet Cardio Intervent

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BackgroundUnilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear.AimsTo assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA‐ASO.MethodsIn this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end‐systolic elastance [Ees]), RV‐to‐PA (RV‐PA) coupling (Ees/Ea), and RV diastolic stiffness (end‐diastolic elastance [Eed]) were analyzed and compared to normal values from the literature.ResultsIn all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV‐PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR‐derived RV volumes, mass, and ejection fraction were preserved.ConclusionUnilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure–volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV‐PA uncoupling.

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Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review

Cited by 13 publications

References 47 publications

Rare Cause of Secondary Pulmonary Arterial Hypertension

Rare Cause of Secondary Pulmonary Arterial Hypertension

Pulmonary hypertension in an adult with unilateral absence of left pulmonary artery

The impact of unilateral pulmonary artery stenosis on right ventricular to pulmonary arterial coupling in patients with transposition of the great arteries

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