Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot

Katewa, Ashish; Saxena, Shivang; Malhotra, Pulkit; Rajesh, Venuthurupalli S. P.; Champaneri, Bhavik; Surti, Jigar

doi:10.1007/s12055-022-01393-4

Cited by 2 publications

(1 citation statement)

References 24 publications

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“…UAPA is often associated with other congenital heart defects, of which TOF is the most common ( 15 ). The incidence of UAPA with TOF is about 1%–3% and that of the absent pulmonary valve (APV) with TOF is 5% ( 34 ). A complication of UAPA with TOF and APV is extremely rare.…”

Section: Discussionmentioning

confidence: 99%

Anomalous origin of the fetal pulmonary artery

Huang

Ling

et al. 2023

Front. Pediatr.

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ObjectivesThis study aims to investigate the efficacy of prenatal ultrasonography in diagnosing the anomalous origin of the fetal pulmonary artery (AOFPA).MethodsA total of 26 AOFPA cases were retrospectively analyzed from January 2014 to January 2023. The features of the AOFPA were characterized by comparing the prenatal ultrasonic data with the results of anatomical casting after pregnancy termination or postnatal imaging and surgical intervention. Missed diagnoses and misdiagnoses were expounded.ResultsOf the 26 AOFPA cases, there were 13 cases of pulmonary artery sling, 8 cases of anomalous origin of the unilateral pulmonary artery, and five cases of unilateral absence of the pulmonary artery; 17 cases received pathological anatomy and casting after pregnancy termination, and nine cases were confirmed by postnatal imaging and surgery. Nineteen cases were accurately prenatally diagnosed (19/26, 73.1%), and seven cases were missed or misdiagnosed (7/26, 26.9%).ConclusionsPrenatal ultrasonography has a favorable diagnostic efficacy for anomalous origin of the fetal pulmonary artery. The absence of either the left or right pulmonary artery from the image of pulmonary artery bifurcation may indicate origin abnormalities of the pulmonary artery in fetuses, which signifies the necessity to detect the abnormal origin of the pulmonary artery on the affected side and other potential intracardiac malformation complications.

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Section: Discussionmentioning

confidence: 99%

Anomalous origin of the fetal pulmonary artery

Huang

Ling

et al. 2023

Front. Pediatr.

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Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review

Hura,

Stychynskyi,

Teterin

et al. 2024

ujcvs

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Absent pulmonary valve syndrome (APVS) is a rare form of congenital heart defect. Among the various variations of this rare anomaly are non-confluent branches of the pulmonary artery (PA) with anomalous origin of one of the PA branches from the patent ductus arteriosus (PDA). Constriction of the PDA in early neonatal age may result in cessation of blood supply to one of the pulmonary artery branches, leading to exclusion of one lung from circulation and gas exchange processes. PDA stenting creates a reliable source of blood flow, and as a first step, an endovascular approach was chosen for treatment. Case report. A newborn (3 kg) with a prenatal diagnosis of APVS and a non-confluent left PA branch filling through PDA was chosen for PDA stenting. Considering the complex anatomy of the heart defect, it was decided to perform PDA stenting to delay total repair until the patient reaches optimal age and weight. Our patient successfully underwent PDA stenting as the first stage, which provided conditions for blood supply to one lung. The second stage involved total repair with good long-term results. Conclusion. APVS treatment may have several options of surgical correction, differing from each other depending on the anatomical features of the defect and the patient’s age. Palliative treatment options help the child transition from the neonatal period and gain optimal weight for total repair.. Analyzing the data from the presented literature, it is noted that prenatal diagnosis is an important stage in determining the urgency and choice of treatment tactics for this defect.

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Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot

Cited by 2 publications

References 24 publications

Anomalous origin of the fetal pulmonary artery

Anomalous origin of the fetal pulmonary artery

Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review

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