Angiographic Tool to Detect Pulmonary Arteriovenous Malformations in Single Ventricle Physiology

Overview

Journal medRxiv

Date 2024 Jan 23

PMID 38260565

Authors

Stephen B Spurgin

Yousef M Arar

Thomas M Zellers

Jijia Wang

Nicolas L Madsen

Surendranath R Veeram Reddy

Ondine Cleaver

Abhay A Divekar

Affiliations

Soon will be listed here.

Abstract

Background: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations (PAVMs). The traditional tools for PAVM diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time (PCTT) to determine its value as a tool to identify PAVMs in patients with single ventricle physiology.

Methods: We defined the angiographic PCTT as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American pediatric center, and angiographic and clinical data was reviewed. PCTT was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between PCTT and hemodynamic and injection parameters was assessed using 84 Glenn angiograms. Five independent observers calculated PCTT to measure reproducibility (intra-class correlation coefficient).

Results: Mean PCTT was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. PCTT in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87.

Conclusions: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. PCTT accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.

References

Tonelli A, Naal T, Dakkak W, Park M, Dweik R, Stoller J . Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast-enhanced echocardiography. Echocardiography. 2017; 34(10):1439-1446. PMC: 5675023. DOI: 10.1111/echo.13662. View

James L, Tandon A, Nugent A, Malik S, Ramaciotti C, Greil G . Rationalising the use of cardiac catheterisation before Glenn completion. Cardiol Young. 2018; 28(5):719-724. DOI: 10.1017/S1047951118000240. View

Mathur M, Glenn W . Long-term evaluation of cava-pulmonary artery anastomosis. Surgery. 1973; 74(6):899-916. View

McFaul R, Tajik A, Mair D, Danielson G, Seward J . Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis. Report of four cases. Circulation. 1977; 55(1):212-6. DOI: 10.1161/01.cir.55.1.212. View

Srivastava D, Preminger T, Lock J, Mandell V, Keane J, Mayer Jr J . Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation. 1995; 92(5):1217-22. DOI: 10.1161/01.cir.92.5.1217. View

Forde K, Fallon M, Krowka M, Sprys M, Goldberg D, Krok K . Pulse Oximetry Is Insensitive for Detection of Hepatopulmonary Syndrome in Patients Evaluated for Liver Transplantation. Hepatology. 2018; 69(1):270-281. PMC: 6652183. DOI: 10.1002/hep.30139. View

McElhinney D, Kreutzer J, Lang P, Mayer Jr J, Del Nido P, Lock J . Incorporation of the hepatic veins into the cavopulmonary circulation in patients with heterotaxy and pulmonary arteriovenous malformations after a Kawashima procedure. Ann Thorac Surg. 2005; 80(5):1597-603. DOI: 10.1016/j.athoracsur.2005.05.101. View

McElhinney D, Marx G, Marshall A, Mayer J, Del Nido P . Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation. J Thorac Cardiovasc Surg. 2010; 141(6):1362-70.e1. DOI: 10.1016/j.jtcvs.2010.08.088. View

Chang R, Alejos J, Atkinson D, Jensen R, Drant S, Galindo A . Bubble contrast echocardiography in detecting pulmonary arteriovenous shunting in children with univentricular heart after cavopulmonary anastomosis. J Am Coll Cardiol. 1999; 33(7):2052-8. DOI: 10.1016/s0735-1097(99)00096-0. View

10.

Clough A, Haworth S, Hanger C, Wang J, Roerig D, Linehan J . Transit time dispersion in the pulmonary arterial tree. J Appl Physiol (1985). 1998; 85(2):565-74. DOI: 10.1152/jappl.1998.85.2.565. View

11.

Shah M, Rychik J, Fogel M, Murphy J, Jacobs M . Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg. 1997; 63(4):960-3. DOI: 10.1016/s0003-4975(96)00961-7. View

12.

Brown D, Gauvreau K, Powell A, Lang P, Colan S, Del Nido P . Cardiac magnetic resonance versus routine cardiac catheterization before bidirectional glenn anastomosis in infants with functional single ventricle: a prospective randomized trial. Circulation. 2007; 116(23):2718-25. DOI: 10.1161/CIRCULATIONAHA.107.723213. View

13.

Zhao H, Tsauo J, Zhang X, Ma H, Weng N, Wang L . Pulmonary transit time derived from pulmonary angiography for the diagnosis of hepatopulmonary syndrome. Liver Int. 2018; 38(11):1974-1981. DOI: 10.1111/liv.13741. View

14.

Pike N, Vricella L, Feinstein J, Black M, Reitz B . Regression of severe pulmonary arteriovenous malformations after Fontan revision and "hepatic factor" rerouting. Ann Thorac Surg. 2004; 78(2):697-9. DOI: 10.1016/j.athoracsur.2004.02.003. View

15.

Kim S, Bae E, Lee J, Lim H, Lee C, Lee C . Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thorac Surg. 2009; 87(2):548-53. DOI: 10.1016/j.athoracsur.2008.10.024. View

16.

Cloutier A, Ash J, Smallhorn J, Williams W, Trusler G, Rowe R . Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation. 1985; 72(3):471-9. DOI: 10.1161/01.cir.72.3.471. View

17.

Spearman A, Ginde S . Pulmonary Vascular Sequelae of Palliated Single Ventricle Circulation: Arteriovenous Malformations and Aortopulmonary Collaterals. J Cardiovasc Dev Dis. 2022; 9(9). PMC: 9501802. DOI: 10.3390/jcdd9090309. View

18.

Faughnan M, Mager J, Hetts S, Palda V, Lang-Robertson K, Buscarini E . Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med. 2020; 173(12):989-1001. DOI: 10.7326/M20-1443. View

19.

Asada D, Morishita Y, Kawai Y, Kajiyama Y, Ikeda K . Efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with univentricular heart after total cavopulmonary connection. Cardiol Young. 2020; 30(2):227-230. DOI: 10.1017/S104795111900324X. View