Unilateral absence of pulmonary artery is a rare congenital disorder that can remain asymptomatic until adulthood. Absence of left pulmonary artery (ALPA) has been reported in one-third of these patients. We are the first to introduce an adult case of ALPA associated with partial anomalous pulmonary venous connection. Â© 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd

Asadian, S.

Hosseini, L.

Jahanshahi, B.

Rezaeian, N.

Saedi, S.

eprints Iran University of Medical Sciences

1624 |    Clin Case Rep. 2021;9:1624–1628.wileyonlinelibrary.com/journal/ccr31 |  INTRODUCTIONUnilateral absence of pulmonary artery (UAPA) is a rare congenital disorder that can occur either in isolation or in association with other congenital cardiac disorders such as tetralogy of Fallot, coarctation of the aorta, and atrial septal defect.1Isolated UAPA affects the right lung in two-thirds of this patient population, with the majority either remaining asymptomatic or having mild clinical symptoms until adult-hood. Some patients may develop symptoms such as dyspnea, chest pain, recurrent respiratory infection, and hemoptysis.2,3 Diagnosis may be difficult in these patients; the anomaly is often incidentally found on chest radiographs. Chest X-ray typically shows asymmetric lung fields with ipsilateral hemi-thorax lucency and mediastinal shift toward the affected side with the ipsilateral absence of hilar vessels.4,5 Computed tomography (CT) confers a more desirable depiction of the condition by demonstrating the collateral blood flow, paren-chymal mosaic attenuation, and secondary bronchiectasis due to recurrent pulmonary infections. Cardiac magnetic resonance (CMR) imaging is capable of defining anatomic structures and the related functional characteristics, including right ventricular function and strain, concurrently.4-6This report presents a rare case of UAPA associated with partial anomalous pulmonary venous connection (PAPVC) and underscores the role of noninvasive imaging modalities through a literature review.1.1 | Case historyThe patient was a 37-year-old woman with the chief com-plaint of occasional palpitations in the preceding few months. She had no history of shortness of breath or fatigue. Her past medical, familial, and drug history was unremarkable. The only notable finding on the initial physical examina-tion was decreased respiratory sounds in the left hemithorax. Electrocardiography illustrated normal sinus rhythm with right bundle branch block (Figure  1). Chest X-ray demon-strated a hypoplastic left lung with decreased vascular mark-ings, along with leftward mediastinal shift. Transthoracic Received: 30 November 2020 | Revised: 8 January 2021 | Accepted: 11 January 2021DOI: 10.1002/ccr3.3858  C A S E  R E P O R TAn adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature reviewSanaz Asadian1  |   Sedigheh Saedi1 |   Bahareh Jahanshahi1 |   Leila Hosseini2  |   Nahid Rezaeian1This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.© 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.1Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran2North Khorasan University of Medical sciences, Bojnurd, IranCorrespondenceNahid Rezaeian, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical sciences,  Tehran, Iran.Email: nahid6069@yahoo.comAbstractUnilateral absence of pulmonary artery is a rare congenital disorder that can remain asymptomatic until adulthood. Absence of left pulmonary artery (ALPA) has been reported in one-third of these patients. We are the first to introduce an adult case of ALPA associated with partial anomalous pulmonary venous connection.K E Y W O R D Sleft pulmonary artery, partial anomalous pulmonary venous connection (PAPVC), unilateral absence of pulmonary artery (UAPA)   | 1625ASADIAN et Al.echocardiography showed mild-to-moderate ventricular dys-function, mild right ventricular enlargement, mild pulmonary hypertension, and possible absence of left pulmonary artery (ALPA) (Figure 2).1.2 | Differential diagnosis, investigations, and treatmentBased on the patient's physical examination, chest X-ray, and transthoracic echocardiography findings, we planned CT angiography to better visualize her anatomic structures. The imaging procedure was performed in 2 consecutive phases: pulmonary arterial enhancement, followed by peak aortic en-hancement. The main findings were ALPA, PAPVC between the right upper pulmonary vein (RUPV) and the superior vena cava (SVC), and right-sided aortic arch (Figure 3).Given the superiority of CMR in the evaluation of right ventricular anatomy and function, we considered it in the next step and found normal left ventricular size with mildly reduced systolic function (ejection fraction  =  46%), mild right ventricular enlargement with moderately reduced sys-tolic function (ejection fraction = 37.5%), right-sided aortic arch, ALPA, dilated main pulmonary artery, dilated right pul-monary artery, PAPVC between RUPV and SVC, diminished left lung vascular markings, and leftward mediastinal shift. Additionally, the phase-contrast velocity mapping CMR se-quence for flow measurement calculated a pulmonary-to-sys-temic flow ratio of 1.6, which represented a significant left-to-right shunt (Figure 4).Because of right ventricular enlargement and significant shunting, the patient underwent a corrective surgical opera-tion for PAPVC.1.3 | OutcomeThe surgical procedure and the early postoperative period were uneventful. Follow-up transthoracic echocardiography, performed 6  months later, revealed notable improvements in echocardiographic markers, including top-normal right ventricular size with mild systolic dysfunction, no residual shunting, and a pulmonary-to-systemic ratio of 1.02. A rou-tine follow-up was, therefore, planned for the patient.2 |  DISCUSSIONTo the best of our knowledge, the present report is the first to introduce a rare association between UAPA (ALPA) and isolated PAPVC in an adult patient.F I G U R E  1  Electrocardiography shows right bundle branch blockF I G U R E  2  In this echocardiographic image, (A) right ventricular 4-chamber view shows a mildly enlarged right ventricle and (B) the continuous wave Doppler of the tricuspid jet indicates a mildly increased tricuspid regurgitation velocity1626 |   ASADIAN et Al.Unilateral absence of pulmonary artery, first described by Frentzel in 1868, is a rare disorder with a prevalence of 1 in 200 000 young adults; it can occur both in isolation and in association with other cardiovascular disorders.4,7-9 The associated anomalies may include tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right-sided aortic arch, truncus arteriosus, and pulmonary atresia. Other disor-ders associated with UAPA are tracheal-esophageal fistula; F I G U R E  3  Computed tomography angiography views of the patient with UAPA show (A) absence of LPA with RPA dilation (arrow), (B) PAPVC between RUPV and SVC (arrow), (C) right-sided aortic arch (upper arrow, with the lower arrow pointing to the trachea), and (D) mild RV enlargement (arrow) in the 4-chamber view. Abbreviations: UAPA, unilateral absence of pulmonary artery; LPA, left pulmonary artery; RPA, right pulmonary artery; PAPVC, partial anomalous pulmonary venous return; SVC, superior vena cava; RV, right ventricleF I G U R E  4  Cardiac magnetic resonance images of the patient with UAPA show (A) absence of LPA with RPA dilation (arrow), (B) PAPVC between RUPV and SVC (arrow), (C) right-sided aortic arch (upper arrow, with the lower arrow pointing to the trachea), (D) mild RV enlargement (arrow) in the 4-chamber view, and (E) diminished left lung vascular markings. Abbreviations: CMR, cardiac magnetic resonance; UAPA, unilateral absence of pulmonary artery; LPA, left pulmonary artery; RPA, right pulmonary artery; PAPVC, partial anomalous pulmonary venous return; SVC, superior vena cava; RV, right ventricle   | 1627ASADIAN et Al.VACTERL (vertebral anomalies, anal atresia, cardiac malfor-mations, Tracheo-Esophageal fistula, renal anomalies, limb abnormalities) syndrome; gastrointestinal, musculoskele-tal, and urogenital disorders; and chest and abdominal wall anomalies.1,10-12Embryologically, UAPA often occurs contralaterally to the aortic arch in consequence of the growth failure of the proximal sixth aortic arch segment in the presence of intact distal intrapulmonary branches, which are supplied through the bronchial, intercostal, internal mammary, subdiaphrag-matic, subclavian, and even coronary arteries.6,13,14In 2002, a study by Ten Harkel et al on 108 patients revealed that isolated UAPA was often diagnosed at an av-erage age of 14  years. Thirty-seven percent of the cases presented with symptoms such as chest pain, pleural ef-fusion, and recurrent respiratory infections, and 40% of the cases showed symptoms of exercise intolerance. The authors reported that pulmonary hypertension complicated 44% of their patients.2Two types of this disorder have been defined with re-gard to the time of symptom presentation: the infantile form, which presents with congestive heart failure and pul-monary hypertension, and the adult type, which is often asymptomatic.15,16In our patient, this disorder occurred in the uncommon form of adult-type ALPA (type 3) with PAPVC (RUPV to SVC). To our knowledge, no association with PAPVC has been reported to date. The patient was almost asymptomatic despite ALPA with PAPVC until adulthood.This anomaly is anatomically classified17 as type 1: agen-esis, which is the complete absence of the bronchial tree, lung parenchyma, and ipsilateral pulmonary artery; type 2: apla-sia, which is a complete lack of the lung parenchyma in the presence of a rudimentary bronchus; and type 3: hypoplasia, which is the presence of different amounts of the lung paren-chyma, bronchial tree, and blood vessels.The gold-standard method for the diagnosis of this com-plication is angiography; nonetheless, advances in CT and CMR have rendered it unnecessary unless embolization is required due to massive hemoptysis.5The most common symptoms of this disease are recur-rent lung infections, decreased ability to work, and short-ness of breath. Pulmonary infections are multifactorial and can be caused by decreased arterial blood flow, increased inflammatory cells, and ciliary dysfunction, leading to mucous trapping and bronchiectasis in some patients.5,6 Hemoptysis is another manifestation of this disorder and is caused by increased collateral blood flow, which may progress to massive pulmonary hemorrhage and death.18-20 Hemoptysis and severe infections can be treated with em-bolization, lobectomy, or pneumonectomy.21,22 However, any lung surgery in this disease may be complicated by the presence of collateral arteries.6In infants with isolated absence of pulmonary artery, pulmonary hypertension is common because of the shear stress in the opposite-side pulmonary artery, which leads to an increased release of vasoconstrictor factors such as en-dothelins. Chronic vasoconstriction results in remodeling, increased pulmonary vascular resistance, and pulmonary hypertension.15,16There is currently no specific treatment recommendation for UAPA. Some authors recommend serial echocardiogra-phy to monitor asymptomatic adult patients with a view to assessing pulmonary arterial pressure. Patients who develop pulmonary hypertension should be treated with vasodila-tors.2,23 Alternatively, peripheral pulmonary branches can be focalized toward the pulmonary hilum. Successful revascu-larization procedures have been reported, especially in the pediatric population.7,9The prognosis of this disease is dependent on the associ-ated cardiovascular anomalies and the severity of pulmonary hypertension. The overall reported mortality is 7%.12In conclusion, UAPA is a rare congenital anomaly that in-volves the right-sided lung in two-thirds of this patient popu-lation. It consists of adult and infantile forms, with the former type remaining asymptomatic or minimally symptomatic. Advanced imaging modalities such as echocardiography, car-diac CT, and CMR have a pivotal role in the diagnosis of this anomaly. Rare associations are of interest and can be found in this disorder.ACKNOWLEDGMENTSThe authors would like to thank Dr Mohammad Mehdi Hemmati Komasi for gathering patient data. Published with written consent of the patient.CONFLICT OF INTERESTNone declared.AUTHOR CONTRIBUTIONSDr BJ and Dr LH: collected the data and prepared the primary draft, Dr NR and Dr SA: wrote the paper, and Dr SS: criti-cally revised the article.ETHICAL APPROVALThis study was approved by the Ethics Committee of Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.DATA AVAILABILITY STATEMENTAll data are available upon reasonable request.ORCIDSanaz Asadian   https://orcid.org/0000-0003-2371-3575 Leila Hosseini   https://orcid.org/0000-0002-6617-9053 Nahid Rezaeian   https://orcid.org/0000-0003-3565-5851 1628 |   ASADIAN et Al.REFERENCES 1. Kayemba-Kay's S, Couvrat-Carcauzon V, Goua V, et al. Unilateral pulmonary agenesis: a report of four cases, two diagnosed antena-tally and literature review. Pediatr Pulmonol. 2014;49(3):E96-E102. 2. Ten Harkel ADJ, Blom NA, Ottenkamp J. Isolated unilateral ab-sence of a pulmonary artery: a case report and review of the litera-ture. Chest. 2002;122(4):1471-1477. 3. Muthusami P, Ananthakrishnan R, Elangovan S. Incidentally de-tected unilateral pulmonary artery agenesis with pulmonary hypo-plasia in a 67-year-old woman. J Radiol Case Rep. 2010;4(11):32. 4. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest. 1995;108(3):670-676. 5. Griffin N, Mansfield L, Redmond K, et al. Imaging features of iso-lated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases. Clin Radiol. 2007;62(3):238-244. 6. Kadir IS, Thekudan J, Dheodar A, Jones MT, Carroll KB. Congenital unilateral pulmonary artery agenesis and aspergilloma. Ann Thorac Surg. 2002;74(6):2169-2171. 7. Presbitero P, Bull C, Haworth S, De Leval M. Absent or occult pulmonary artery. Heart. 1984;52(2):178-185. 8. Fraentzel O. Ein Fall von abnormer Communication der Aorta mit der Arteria pulmonalis. Arch Pathol Anat Physiol Klin Med. 1868;43(3):420-426. 9. Welch K, Hanley F, Johnston T, Cailes C, Shah MJ. Isolated uni-lateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg. 2005;79(4):1399-1402. 10. Osborne J, Masel J, McCredie J. A spectrum of skeletal anomalies associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol. 1989;19(6–7):425-432. 11. Knowles S, Thomas R, Lindenbaum R, Keeling J, Winter R. Pulmonary agenesis as part of the VACTERL sequence. Arch Dis Child. 1988;63(7 Spec No):723-726. 12. Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am. 1994;41(3):453. 13. Pfefferkorn JR, Löser H, Pech G, Toussaint R, Hilgenberg F. Absent pulmonary artery. Pediatr Cardiol. 1982;3(4):283-286. 14. Bockeria LA, Makhachev OA, Khiriev TK, Abramyan MA. Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung. Interact Cardiovasc Thorac Surg. 2011;12(3):509-510. 15. Shakibi JG, Rastan H, Nazarian I, Paydar M, Aryanpour I, Siassi B. Isolated unilateral absence of the pulmonary artery. Jpn Heart J. 1978;19(3):439-451. 16. Bahler RC, Carson P, Traks E, Levene A, Gillespie D. Absent right pulmonary artery: problems in diagnosis and management. Am J Med. 1969;46(1):64-71. 17. Schneider P, Schwalbe E. Die Morphologie der Missbildungen Des Menschen Under Thiere, 1912; Vol. 3, Part 2. Jena, Germany: Gustav Fischar; 817-822. 18. Koga H, Hidaka T, Miyako K, Suga N, Takahashi N. Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol. 2010;31(8):1186-1190. 19. Krall W, Ploy-Song-Sang Y. Unilateral pulmonary artery apla-sia presenting with chest pain and pleural effusion. South Med J. 1980;73(2):233-236. 20. Turner D, Vincent J, Epstein M. Isolated right pulmonary artery discontinuity. Images Paediatr Cardiol. 2000;2(3):24. 21. Bekoe S, Pellegrini RV, DiMarco RF, Grant KJ, Woelfel GF. Pneumonectomy for unremitting hemoptysis in unilateral absence of pulmonary artery. Ann Thorac Surg. 1993;55(6):1553-1554. 22. Reñé M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol. 1995;18(4):251-254. 23. Shostak E, Sarwar A. A 50-year-old woman with dyspnea, lower extremity edema, and volume loss of the right hemithorax. Chest. 2009;136(2):628-632.How to cite this article: Asadian S, Saedi S, Jahanshahi B, Hosseini L, Rezaeian N. An adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature review. Clin Case Rep. 2021;9:1624–1628. https://doi.org/10.1002/ccr3.3858

An adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature review

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An adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature review

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