Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery.

Background: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly. OBJECTIVE: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. METHODS: All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue. The patients were initially examined clinically, followed by hematological testing, blood biochemistry testing, and chest X-ray radiology. The results suggested UAPA, so echocardiography and contrast-enhanced chest computed tomography (CT) were performed as soon as practical. RESULTS: The echocardiographic and CT imaging findings confirmed the suspected diagnosis of UAPA in all seven patients, which was accompanied by congenital heart disease in three patients. Three of the seven patients had mild and medium pulmonary hypertension. All seven patients were treated with drugs, which led to improvement in symptoms. CONCLUSION: Frontal chest X-ray provided the initial signs suggesting a diagnosis of UAPA. Subsequent echocardiography and contrast-enhanced chest CT were effective diagnostic tools for fast and accurate confirmation of UAPA.

Anomalous origin of the fetal pulmonary artery.

Objectives: This study aims to investigate the efficacy of prenatal ultrasonography in diagnosing the anomalous origin of the fetal pulmonary artery (AOFPA). Methods: A 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. Results: Of 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%). Conclusions: Prenatal 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.

Successful hybrid management of hypertensive patent ductus arteriosus with unilateral absence of the right pulmonary artery - A case report.

Unilateral absence of pulmonary artery (UAPA) can be either isolated or associated with other congenital cardiac defects. It is a rare congenital lesion with a diverse clinical presentation. We present a case of absent right pulmonary artery and large patent ductus arteriosus (PDA) with severe unilateral pulmonary hypertension. We describe our strategy of staged ductus arteriosus closure in this patient and successful outcome on long term follow up.

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

Congenital, isolated unilateral agenesis of pulmonary arteries (UAPA) has a bimodal clinical presentation. It is either diagnosed in early infancy or during adulthood. Early diagnosis and timely surgical correction at infancy prevent the development of irreversible pulmonary hypertension and enable prolonged survival. Though surgical intervention is a conclusive method of treatment, its outcome depends upon the reversibility of pulmonary hypertension and the adequacy of the pulmonary artery dimension. Adults with UAPA can be asymptomatic (diagnosed incidentally) or symptomatic. They may present clinically with myriads of symptoms like exertional dyspnea, recurrent lung infection, hemoptysis, and atypical chest pain. For cases where definitive surgical treatment is not feasible, interventional and medical management should be carried out as part of palliative therapy. A retrospective analysis of 22 cases of UAPA in adults published in the literature from the years 2017-2020 are presented here, along with our institutional case.

Isolated absence of the right pulmonary artery with coexisting left-sided heart failure: case report and literature review.

Isolated unilateral absence of the pulmonary artery (UAPA) exhibits a benign course and often goes undiagnosed until adulthood. We present a 39-year-old male admitted for left-sided congestive heart failure (CHF) exacerbation. However, chest radiograph demonstrated findings suspicious for UAPA that was ultimately confirmed on computed tomography. Because both CHF and UAPA present similarly with exercise intolerance and dyspnea, a high index of suspicion is required to secure the diagnosis, and in this case, symptoms attributed to CHF may have contributed to delay. A strong awareness of typical radiographic findings allows for initiation of confirmatory tests necessary for a correct diagnosis.

Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery.

Tetralogy of Fallot with unilateral absence of the pulmonary artery (UAPA) is a rare congenital heart disease. The aim of the present study was to examine the surgical strategy for Tetralogy of Fallot with UAPA by summarizing our experience of its treatment. We retrospectively evaluated 17 patients admitted to our hospital for treatment between 2006 and 2017. All patients were diagnosed with absence of the left pulmonary artery. The Nakata Index (NI), NI Z score, and McGoon ratio of the existing pulmonary artery were calculated. All patients were divided into one-stage complete repair (group A) or palliative procedure (group B) groups according to these criteria and surgical treatments. There were nine treated patients in group A, with a mean NI of 595.6 ± 690.32 mm2/m2 (169.3-2433 mm2/m2) and a mean NI Z score of - 1.57 ± 3.02 (- 4.60 to 5.27). There were eight treated patients in group B, with a mean NI of 107.61 ± 49.49 mm2/m2 (53.15-216.39 mm2/m2) and a mean NI Z score of - 6.27 ± 1.56 (- 8.22 to - 3.54). The mean follow-up time in group A was 5.58 ± 3.42 years (1-11.7 years), while that in group B was 5.4 ± 3.42 years (0.6-12.1 years). No hospital deaths occurred and the follow-up results were good in both groups. In conclusion, development of a single existing pulmonary artery can be evaluated using the NI and the NI Z score. These evaluations can be used to select complete repair or palliative procedures for patients and achieve good results.

Isolated unilateral absence of the pulmonary artery.

Isolated unilateral absence of the pulmonary artery (UAPA) is a rare malformation. It is associated with respiratory symptoms, such as dyspnea or hemoptysis. We suggest that surgical treatment should be positively considered in patients with UAPA who are severely symptomatic and who have no other cardiovascular or respiratory comorbidities.

Two-Stage Repair of Absence of Right Pulmonary Artery / 日本心臓血管外科学会雑誌

Isolated unilateral absence of the pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful anatomical repair after systemic-to-pulmonary shunt. The patient was a 1-day-old boy who was transferred to our institution because of continuous murmur. Cardiac echography revealed anomalous origin of the right pulmonary artery from the ascending aorta (AORPA). This aorto-right pulmonary arterial blood flow, however, disappeared in the following 2 days. We altered the diagnosis to right unilateral absence of pulmonary artery (UAPA) from AORPA. A right systemic-to-pulmonary shunt using a 3 mm polytetrafluoroethylene (PTFE) graft was placed, aiming for growth of the right pulmonary artery at the age of 8 days. Forty days after the initial surgery, he underwent a definitive procedure. The right pulmonary artery was anatomically reconstructed with an 8-mm PTFE graft. He was discharged in excellent condition on postoperative day 41. Cardiac catheterization, 1 year later, showed the surgically created right pulmonary artery was patent. A pulmonary perfusion scintigraphy showed satisfactory blood distribution in the right lung.