Partial Anomalous Pulmonary Venous Return in Adults: Insight into Pulmonary Hypertension

Overview

Journal Int J Cardiol Congenit Heart Dis

Date 2024 Dec 23

PMID 39713576

Authors

Nour Rahnama

Linda Kubangumusu

Agnes Pasquet

Annie Robert

Anne-Catherine Pouleur

Karlien Carbonez

Joelle Kefer

Stephane Moniotte

Alain Poncelet

Geoffroy de Becco

Benoit Ghaye

Sophie Pierard

Affiliations

Soon will be listed here.

Abstract

Background: Partial anomalous pulmonary venous return (PAPVR) is a rare condition that may lead to pulmonary arterial hypertension (PAH). We sought to determine the prevalence of PAPVR, the follow-up rate of incidentally discovered PAPVR, the repercussions of volume and pressure overload on the right ventricle (RV), and the prevalence and predictors of PAH.

Methods And Results: Fifty PAPVR patients aged ≥18 years were included. All underwent transthoracic echocardiography and multislice imaging. PAPVR prevalence was 0.2% (23/13,606 chest computed tomography reports). Of the 26 incidentally diagnosed patients, 14 (54%) did not have a cardiac follow-up. Seven (14%) patients had PAH, of whom one third (28%) had Eisenmenger syndrome. The left-to-right shunt ratio (Qp/Qs) was the only predictor of PAH. PAPVR-induced volume overload led to increased right chamber volumes (RV basal diameter 45±8 mm, RV mid-diameter 38±9 mm, RV/left ventricle ratio 1.2 ± 0.2, indexed RV end-diastolic area 14±4 cm/m, indexed RV end-systolic area 8.6 ± 3.2 cm/m) and increased RV functional parameters (tricuspid annular plane systolic excursion 27±5 mm, fractional area change [FAC] 42 ± 10%, global longitudinal strain [GLS] -22 ± 5). In contrast, PAH-induced pressure overload had no impact on right heart volumes, but on RV functional parameters, which were decreased compared to non-PAH patients (FAC 35 ± 13% 43 ± 10%, = 0.049; GLS -18 ± 5 -23 ± 4%, = 0.01).

Conclusions: PAPVR prevalence was 0.2%. When incidentally diagnosed, its management was often neglected despite potentially serious consequences (14% PAH). Only the Qp/Qs ratio was predictive of PAH. PAPVR-induced volume overload was shown to increase right heart volumes, while PAH-induced pressure overload caused alterations of RV functional parameters.

References

Wilkinson J . Haemodynamic calculations in the catheter laboratory. Heart. 2000; 85(1):113-20. PMC: 1729580. DOI: 10.1136/heart.85.1.113. View

DOTTER C, HARDISTY N, STEINBERG I . Anomalous right pulmonary vein entering the inferior vena cava; two cases diagnosed during life by angiocardiography and cardiac catheterization. Am J Med Sci. 1949; 218(1):31-6. DOI: 10.1097/00000441-194907000-00004. View

Benjouad I, Taam I, Ataouna K, Mahi M, Amil T, Saouab R . [Scimitar syndrome: about a case and review of the literature]. Pan Afr Med J. 2017; 25:37. PMC: 5291422. DOI: 10.11604/pamj.2016.25.37.10545. View

Lang R, Badano L, Mor-Avi V, Afilalo J, Armstrong A, Ernande L . Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2015; 16(3):233-70. DOI: 10.1093/ehjci/jev014. View

Eroglu E, Deniz Cakal S, Cakal B, Dundar C, Alici G, Ozkan B . Time course of right ventricular remodeling after percutaneous atrial septal defect closure: assessment of regional deformation properties with two-dimensional strain and strain rate imaging. Echocardiography. 2012; 30(3):324-30. DOI: 10.1111/echo.12053. View

Farber H, Foreman A, Miller D, McGoon M . REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Congest Heart Fail. 2011; 17(2):56-64. DOI: 10.1111/j.1751-7133.2010.00202.x. View

Morris D, Krisper M, Nakatani S, Kohncke C, Otsuji Y, Belyavskiy E . Normal range and usefulness of right ventricular systolic strain to detect subtle right ventricular systolic abnormalities in patients with heart failure: a multicentre study. Eur Heart J Cardiovasc Imaging. 2016; 18(2):212-223. DOI: 10.1093/ehjci/jew011. View

Sanders S, Yeager S, Williams R . Measurement of systemic and pulmonary blood flow and QP/QS ratio using Doppler and two-dimensional echocardiography. Am J Cardiol. 1983; 51(6):952-6. DOI: 10.1016/s0002-9149(83)80172-6. View

Evaldsson A, Ingvarsson A, Waktare J, Smith G, Thilen U, Stagmo M . Right ventricular speckle tracking assessment for differentiation of pressure- versus volume-overloaded right ventricle. Clin Physiol Funct Imaging. 2017; 38(5):763-771. DOI: 10.1111/cpf.12477. View

10.

Alsoufi B, Cai S, Van Arsdell G, Williams W, Caldarone C, Coles J . Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg. 2007; 84(6):2020-6. DOI: 10.1016/j.athoracsur.2007.05.046. View

11.

Sears E, Aliotta J, Klinger J . Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension. Pulm Circ. 2012; 2(2):250-5. PMC: 3401879. DOI: 10.4103/2045-8932.97637. View

12.

Abman S, Hansmann G, Archer S, Ivy D, Adatia I, Chung W . Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation. 2015; 132(21):2037-99. DOI: 10.1161/CIR.0000000000000329. View

13.

Ghio S, Klersy C, Magrini G, DArmini A, Scelsi L, Raineri C . Prognostic relevance of the echocardiographic assessment of right ventricular function in patients with idiopathic pulmonary arterial hypertension. Int J Cardiol. 2008; 140(3):272-8. DOI: 10.1016/j.ijcard.2008.11.051. View

14.

Park J, Negishi K, Kwon D, Popovic Z, Grimm R, Marwick T . Validation of global longitudinal strain and strain rate as reliable markers of right ventricular dysfunction: comparison with cardiac magnetic resonance and outcome. J Cardiovasc Ultrasound. 2014; 22(3):113-20. PMC: 4192408. DOI: 10.4250/jcu.2014.22.3.113. View

15.

Moceri P, Baudouy D, Chiche O, Cerboni P, Bouvier P, Chaussade C . Imaging in pulmonary hypertension: Focus on the role of echocardiography. Arch Cardiovasc Dis. 2014; 107(4):261-71. DOI: 10.1016/j.acvd.2014.02.005. View

16.

Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A . Management of grown up congenital heart disease. Eur Heart J. 2003; 24(11):1035-84. DOI: 10.1016/s0195-668x(03)00131-3. View

17.

Galie N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A . 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS):.... Eur Heart J. 2015; 37(1):67-119. DOI: 10.1093/eurheartj/ehv317. View

18.

Majdalany D, Phillips S, Dearani J, Connolly H, Warnes C . Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010; 5(6):537-45. DOI: 10.1111/j.1747-0803.2010.00458.x. View

19.

Ho M, Bhalla S, Bierhals A, Gutierrez F . MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009; 24(2):89-95. DOI: 10.1097/RTI.0b013e318194c942. View

20.

Puwanant S, Park M, Popovic Z, Tang W, Farha S, George D . Ventricular geometry, strain, and rotational mechanics in pulmonary hypertension. Circulation. 2010; 121(2):259-66. PMC: 2846516. DOI: 10.1161/CIRCULATIONAHA.108.844340. View