Pulmonary artery agenesis and Kommerell's diverticulum presenting with hemoptysis

Rousou, Anthony J.; Tetenta, Sodienye; Boffa, Daniel J.

doi:10.1016/j.ejcts.2008.10.053

Cited by 5 publications

(13 citation statements)

References 7 publications

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“…Various types of KD have been reported in the literature ( 5 , 7 , 8 ). A vascular anomaly with an aberrant right subclavian artery has been reported as a rare type of KD, but KD in combination with left UPAA is particularly rare and there are just a few adult cases published so far ( 2 , 8 , 9 ). We report a case of a pediatric patient with these two rare vascular anomalies.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, patients without other significant anomalies can survive until adulthood without symptoms, which is the reason for the late diagnosis of UPAA. Dyspnea, limited exercise tolerance, recurrent pulmonary infections, and hemoptysis are the most common symptoms observed in adult patients with UPAA ( 1 , 2 , 4 ). Pulmonary hypertension develops in 19%–44% of adult patients and reduces long-term survival ( 1 , 2 , 10 ).…”

Section: Discussionmentioning

confidence: 99%

“…Dyspnea, limited exercise tolerance, recurrent pulmonary infections, and hemoptysis are the most common symptoms observed in adult patients with UPAA ( 1 , 2 , 4 ). Pulmonary hypertension develops in 19%–44% of adult patients and reduces long-term survival ( 1 , 2 , 10 ). There are no studies on the predicted development time of pulmonary hypertension because of late diagnosis and the inability to follow up these patients.…”

Section: Discussionmentioning

confidence: 99%

“…There are no studies on the predicted development time of pulmonary hypertension because of late diagnosis and the inability to follow up these patients. KD is also asymptomatic in the majority of cases ( 2 , 5 , 6 ). Once in a while, wheezing and dysphagia could be observed because of compression of the mediastinal structures in middle-aged patients ( 3 , 6 ).…”

Section: Discussionmentioning

confidence: 99%

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Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Miseviciene,

Liakaite,

Zaveckiene

et al. 2023

Front. Pediatr.

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BackgroundUnilateral pulmonary artery agenesis (UPAA) and Kommerell's diverticulum (KD) are two rare embryologically unrelated congenital vascular malformations rarely diagnosed in children. This is the first report of our knowledge of the unique combination for a child as patients are at a high risk of pulmonary hypertension and rupture of the diverticulum. Our aim is to present the case of a pediatric patient with UPAA and KD with the short literature review and to highlight the importance of early diagnostics of rare congenital vascular malformations.Case reportA 1-year-old girl presented to the emergency department with prolonged cough and variable wheezing. A hypoplastic left lung was suspected in the radiographic image of the chest. A transthoracic echocardiogram revealed absence of the left pulmonary artery and right arch of aorta and anomaly of subclavian arteries was suspected. The diagnosis was confirmed by computed tomography scans of the chest that demonstrated elongation of the aorta and an aberrant right subclavian artery with KD, as well as absence of the left pulmonary artery. The patient is being followed up for the development of pulmonary hypertension and compression of vascular structures to the airways as well as any indications for surgical intervention because of the KD.ConclusionsUPAA and KD are two very rare congenital vascular anomalies usually diagnosed in adults. A high risk of pulmonary hypertension and rupture of diverticulum is noted for adult patients. This case provides us with an exclusive possibility to follow up a patient with an extremely rare combination of the two vascular anomalies with insufficiently known future complications and outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Miseviciene,

Liakaite,

Zaveckiene

et al. 2023

Front. Pediatr.

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“…170 This lesion can be asymptomatic into adulthood. [171][172][173][174] Abnormal arterial supply to the lung segments such as seen in scimitar syndrome with sequestration is also well seen by cardiovascular CT. [175][176][177][178] Cardiovascular CT is the imaging modality of choice to determine anatomic substrate and interventional planning for complex lung lesions such as intralobar or extralobar pulmonary sequestration. 179,180 In a study evaluating the utility of cardiovascular CT for surgical planning in these patients, treatments were correctly planned using cardiovascular CT with 100% accuracy, sensitivity and specificity.…”

Section: Patientsmentioning

confidence: 97%

Computed Tomography Imaging in Patients with Congenital Heart Disease Part I: Rationale and Utility. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT)

Han

Rigsby²,

Hlavacek

et al. 2015

Journal of Cardiovascular Computed Tomography

158

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Kommerell’s diverticulum in the current era: a comprehensive review

Tanaka

Milner

Ota

2015

Gen Thorac Cardiovasc Surg

171

190

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Kommerell's diverticulum is a developmental error with a remnant of fourth dorsal aortic arch, named after Dr. Kommerell, a radiologist, who made the first diagnosis in a living individual. The diverticulum can occur in both the left and right aortic arch, from which an aberrant subclavian artery rises to the contralateral side. Pediatric patients often present with airway symptoms whereas dysphagia and chest discomfort are more common in the adult patients. Computed tomography or magnetic resonance imaging can provide details of the diverticulum, associated arch anomalies, and its relationship with surrounding organs. Recent histological studies indicated the presence of cystic medial necrosis in the diverticulum wall, which would explain the reported high rates of aortic dissection and rupture associated with the diverticulum. Accumulated knowledge on this entity and the recent advancement of imaging techniques, surgical/endovascular strategies, and perioperative management, have led to more aggressive intervention to the diverticulum in the early phase. While still under debate it is generally accepted to consider surgical intervention when the diameter of the diverticulum orifice exceeds over 30 mm, and/or the diameter of the descending aorta adjacent to the diverticulum exceeds over 50 mm. Treatment options include open surgical repair, hybrid endovascular repair, and total endovascular repair. The selection of treatment strategy for Kommerell's diverticulum should be based on the anatomy, comorbidities of the patient, and surgical expertise available. The summaries of open and endovascular repairs of over 210 cases from literature search from 2004 to 2014 are also provided in this review.

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Pulmonary artery agenesis and Kommerell's diverticulum presenting with hemoptysis

Cited by 5 publications

References 7 publications

Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Case report: Unilateral pulmonary artery agenesis and Kommerell's diverticulum in 1-year old girl

Computed Tomography Imaging in Patients with Congenital Heart Disease Part I: Rationale and Utility. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT)

Kommerell’s diverticulum in the current era: a comprehensive review

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