Pulmonary Vascular Sequelae of Palliated Single Ventricle Circulation: Arteriovenous Malformations and Aortopulmonary Collaterals

Spearman, Andrew D.; Ginde, Salil

doi:10.3390/jcdd9090309

Cited by 10 publications

(7 citation statements)

References 75 publications

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“…Clinical observations of single ventricle circulation support that PAVMs develop progressively after Glenn palliation, a surgical procedure where the superior vena cava (SVC) is directly anastomosed to a branch pulmonary artery and the main pulmonary artery no longer receives antegrade blood flow from the heart. As a result, pulmonary blood flow in Glenn circulation comes exclusively from the SVC, and venous blood from the lower body (including the inferior vena cava and hepatic vein) is pumped to the body via the single functional ventricle without perfusing the lungs [5]. In other words, PAVMs progressively develop when hepatopulmonary circulation is interrupted and pulmonary blood flow is delivered exclusively from the SVC.…”

Section: Introductionmentioning

confidence: 99%

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations

Wan,

Rousseau,

Mattern

et al. 2024

Preprint

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Background: Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease (CHD). Single ventricle PAVMs have been recognized for over 50 years, yet they are poorly understood, and we lack any medical therapies. To improve our understanding of single ventricle PAVM initiation and progression, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. Methods: Using adult rats, we performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control. To assess for PAVM physiology in the left lung, we quantified intrapulmonary shunting using two independent methods (bubble echocardiography and fluorescent microsphere injection) at 2 weeks, 2 months, and 6 months. Additionally, we performed arterial blood gas measurements to assess oxygenation and plethysmography to assess ventilation. Results: We identified pathologic intrapulmonary shunting by bubble echocardiography as early as 2 weeks post-Glenn surgery, and shunting continued chronically at 2- and 6-months post-Glenn. Shunting also progressed over time, demonstrated by increased shunting of 10 µm microspheres at 6 months. Shunting was accompanied by mildly decreased arterial oxygenation, but there were no differences in ventilation as quantified by plethysmography. Conclusions: Our surgical animal model of unilateral Glenn circulation re-creates the clinical condition of single ventricle PAVMs with early and progressive intrapulmonary shunting. This model is poised to characterize single ventricle PAVM pathophysiology and lead to mechanistic and therapeutic discovery.

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Section: Introductionmentioning

confidence: 99%

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations

Wan,

Rousseau,

Mattern

et al. 2024

Preprint

Self Cite

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“…Pulmonary arteriovenous malformations (PAVMs) are pathologic vascular connections between arteries and veins that develop in 60–100% of patients with single ventricle congenital heart disease (CHD) [ 1 , 2 ]. PAVMs have been recognized in single ventricle circulation for nearly 50 years but remain poorly understood [ 3 , 4 , 5 ]. PAVMs cause right to left intrapulmonary shunting and varying degrees of hypoxia.…”

Section: Introductionmentioning

confidence: 99%

Objective Quantification of Bilateral Bubble Contrast Echocardiography Correlates with Systemic Oxygenation in Patients with Single Ventricle Circulation

Phimister,

Bushee,

Merbach

et al. 2024

JCDD

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Bubble contrast echocardiography is commonly used to diagnose pulmonary arteriovenous malformations (PAVMs) in single ventricle congenital heart disease (CHD), yet previous studies inconsistently report a correlation between bubble echoes and oxygenation. In this study, we sought to re-evaluate the correlation between bubble echoes and oxygenation by assessing total bilateral shunting and unilateral shunting. We conducted a single-center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent a cardiac catheterization and bubble echocardiogram during the same procedure from 2011 to 2020. Spearman’s rank correlation was performed to examine the relationship between total bilateral shunting and measures of systemic oxygenation, as well as unilateral shunting and ipsilateral pulmonary vein oxygenation. For all patients (n = 72), total bilateral shunting moderately correlated with peripheral oxygen saturation (SpO2) (rs = −0.44, p < 0.0001). For patients with Glenn/Kawashima circulation (n = 49), total bilateral shunting was moderately correlated (SpO2: rs = −0.38, p < 0.01). In contrast, unilateral shunting did not correlate with ipsilateral pulmonary vein oxygenation for any vein measured (p = 0.16–p > 0.99). In conclusion, the total burden of bilateral bubble shunting correlated with systemic oxygenation and may better reflect the total PAVM burden from all lung segments. Unilateral correlation may be adversely influenced by non-standardized approaches to pulmonary vein sampling.

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“…Additionally, single ventricle patients develop systemic to pulmonary collateral flow (SCPF) which may absorb a substantial amount of the aortic flow and reduce the effective cardiac index ( 1 ). The impact of SCPF on the pulmonary antegrade flow is still a matter of debate ( 2 ). SCPF is more prominent in the pre-TCPC stage than the post-TCPC stage.…”

Section: Introductionmentioning

confidence: 99%

Systemic-to-pulmonary collateral flow associations with antegrade pulmonary flow in single ventricle patients: insights from cardiac magnetic resonance imaging

Yanovskiy,

Martelius,

Nyman

et al. 2024

Front. Cardiovasc. Med.

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PurposeIn the palliated single ventricle anomalies, a considerable amount of the aortic flow may be absorbed by the systemic-pulmonary collateral flow (SPCF), which can be noninvasively assessed by cardiac magnetic resonance (CMR). The aims of this study were to (1) identify factors associated with SCPF in pediatric single ventricle patients, and (2) establish a cutoff values indicating an association between SCPF and a reduction in antegrade pulmonary flow.MethodsA retrospective single-tertiary-center cohort study included 158 consecutive CMR studies of patients with a single ventricle. In the uni- and multivariable analysis, SPCF was presented as a percentage of the total pulmonary venous flow (SPCF%PV). The minimal clinically important difference in QP/QS ratios was estimated as ≥0.50, and an optimal cutoff value was defined using the receiver operating characteristic (ROC) curve.ResultsSPCF%PV was significantly smaller in the post-total cavopulmonary connection (TCPC) group than in the pre-TCPC patients (p < 0.001). The patient's higher age and a higher antegrade pulmonary flow were associated with a lower SPCF%PV. A negative weak association was observed between the SPCF%PV and systemic saturation (r = −0.39, p < 0.001). SPCF%PV did not associate with ventricular volumes nor ejection fraction. The SPCF%PV was significantly smaller in patients that were palliated primarily with a pulmonary artery banding compared to those palliated with a BT-shunt (p = 0.002) or RV-PA- shunt (p = 0.044). In the ROC analysis, for pre-TCPC patient's, a cutoff of SPCF%PV 42% yielded a sensitivity of 100% and specificity of 80% for significantly reduced antegrade pulmonary flow (AUC 0.97). In the post-TCPC group, the optimal SPCF%PV cutoff was 34% (sensitivity 100%, specificity 98%, AUC 0.99).ConclusionSPCF results in a considerable left-to-right shunt, which subsequently diminishes spontaneously after TCPC. Our findings indicated that for pre-TCPC patients, an SPCF%PV threshold of 42% (sensitivity 100%, specificity 80%), and for the post-TCPC group, a threshold of 34% (sensitivity 100%, specificity 98%) were effective in identifying reduced antegrade pulmonary flow.

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Pulmonary Vascular Sequelae of Palliated Single Ventricle Circulation: Arteriovenous Malformations and Aortopulmonary Collaterals

Cited by 10 publications

References 75 publications

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations

Objective Quantification of Bilateral Bubble Contrast Echocardiography Correlates with Systemic Oxygenation in Patients with Single Ventricle Circulation

Systemic-to-pulmonary collateral flow associations with antegrade pulmonary flow in single ventricle patients: insights from cardiac magnetic resonance imaging

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