Developmental lung anomalies in adults: A pictorial review

Cherian, Sujith V.; Kumar, Anupam; Ocazionez, Daniel; Martin, Rosa M. Estrada Y; Restrepo, Carlos S.

doi:10.1016/j.rmed.2019.07.011

Cited by 23 publications

(35 citation statements)

References 70 publications

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“…In regards to the reason behind this, it was hypothesized that increased morbidity and mortality of patients with right pulmonary agenesis in comparison to left sided ones is a result of a greater distortion in the tracheo-bronchial tree that occurs due to the mediastinal shifting [10]. However, a more recent review has showed that the most probable reason is related to the number of anatomical lobes and the ability of the right lung with its three lobes to adapt and meet the oxygen demand required by the body in a better way than the left lung would in cases of agenesis [11].…”

Section: Discussionmentioning

confidence: 99%

Right unilateral lung agenesis with dextroposition; the first case in Saudi Arabia

Ahmed

Tajeddin

Farfour

et al. 2020

Respiratory Medicine Case Reports

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In 1673, the first case of lung agenesis was reported during an autopsy. Since then many more similar cases were reported and a classification of these cases developed for better medical management. Those cases ranged from hypoplasia to aplasia or agenesis of the lung tissue, with a survival rate of less than 50% by the age of 5 years in cases of agenesis. More commonly affecting the left side than the right, here we present a case of a late presentation of right lung agenesis in a young 24-year-old female. Who presented with multiple episodes of shortness of breath over a year. When examined she had an asymmetric chest and dull right sided auscultation. The x-ray done showed a homogeneous opacity in the right upper, middle and lower zone with shift of the mediastinum to the right side, making the heart in dextroposition. Watchfulness need to be maintained when encountering patients with simple complaint, as sometimes, it is not just the normal anatomy.

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Section: Discussionmentioning

confidence: 99%

Right unilateral lung agenesis with dextroposition; the first case in Saudi Arabia

Ahmed

Tajeddin

Farfour

et al. 2020

Respiratory Medicine Case Reports

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“…Embryologically, pulmonary artery (PA) development occurs from the sixth aortic arch. 3 The origin of PAS has been postulated to be secondary to the obliteration of the left sixth aortic arch, resulting in an anomalous origin from the RPA. 4 although it is relatively difficult and requires experienced doctors and careful examination.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of pulmonary artery sling associated with tracheal agenesis: A case report

Huo

et al. 2020

Echocardiography

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Pulmonary artery sling (PAS) and tracheal agenesis (TA) are rare diseases, and most cases of PAS are associated with tracheal bronchial malformations. However, PAS associated with TA is yet to be reported. We report a case of PAS with TA diagnosed prenatally. Due to the extremely low incidence, physicians do not have sufficient understanding of these diseases and it is challenging to diagnose these diseases by prenatal ultrasound, with high rates of misdiagnosis. Prenatal examination of the pulmonary artery branches, trachea, and esophagus is useful; therefore, improving the accuracy of prenatal diagnosis will help in perinatal management and counseling.

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“…The clinical presentation of this condition is strictly dependent on the entity of the left-to-right shunt due to the anomalous pulmonary flow return and possible associated cardiac malformations (e.g., sinus venosus atrial septal defect being the most common). In cases in which the shunt is not clinically significant (Qp/Qs<50%), the patients can be asymptomatic until adulthood [5,6]. Conversely, patients with a clinically significant shunt show symptoms including decreased functional capacity, exertional shortness of breath and less frequently pulmonary infections and signs of right heart failure.…”

Section: Introductionmentioning

confidence: 99%

Multi-Modality Imaging Approach in a Challenging Case of Surgically Corrected Partial Anomalous Pulmonary Venous Return and Atrial Tachycardia Treated With Radiofrequency Ablation

Muscogiuri¹,

Girolamo²,

Adduci³

et al. 2021

Cureus

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Pulmonary anomalous venous return (PAPVR) is defined as a congenital anomaly in which at least one but not all of the pulmonary veins abnormally drain into a systemic vein or directly into the right atrium. Signs and symptoms related to this condition are due to the hemodynamic abnormalities secondary to left-to-right shunt and the possible presence of other associated cardiac anomalies (e.g., sinus venous atrial septal defect). Therefore, depending on the extent of the shunt, the clinical presentation of PAPVR is variable, ranging from asymptomatic patients to patients affected by severe heart failure with right-sided volume overload. PAPVR with a clinically significant shunt should be referred for surgical correction with different techniques depending on the presence of associated cardiac anomalies. We are presenting a case of partial anomalous venous return (PAPVR) in a 66-year-old man who underwent surgery 26 years ago to correct an anomalous venous connection between the right superior pulmonary vein (RSPV) and the superior vena cava (SVC) through a veno-atrial baffle. The patient was admitted to the emergency department due to atrial tachycardia. Trans-thoracic echocardiography (TTE) showed a dilated right ventricle (RV) with mild RV systolic dysfunction and pulmonary hypertension. Cardiac magnetic resonance (CMR) further confirmed the findings described by TTE and also demonstrated areas of fibrosis replacement in the hinge points. Cardiac computed tomography (CCT) was able to accurately depict and evaluate the surgically created veno-atrial baffle and also showed an anomalous connection between the left superior pulmonary vein (LSPV) and the brachiocephalic vein (BCV) through a vertical vein. The patient was successfully treated with radiofrequency ablation for his arrhythmia.

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Developmental lung anomalies in adults: A pictorial review

Cited by 23 publications

References 70 publications

Right unilateral lung agenesis with dextroposition; the first case in Saudi Arabia

Right unilateral lung agenesis with dextroposition; the first case in Saudi Arabia

Prenatal diagnosis of pulmonary artery sling associated with tracheal agenesis: A case report

Multi-Modality Imaging Approach in a Challenging Case of Surgically Corrected Partial Anomalous Pulmonary Venous Return and Atrial Tachycardia Treated With Radiofrequency Ablation

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