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Background: Single ventricle (SV) patients undergo multiple surgeries with subsequent changes in anatomy and hemodynamics. There are little cardiac magnetic resonance (CMR) data on serial changes in these patients. This study aimed to assess longitudinal changes of SV anatomy and hemodynamics in a large cohort., Methods: Anatomy and flow in SV patients with serial CMRs performed between 2008 and 2019 at a single institution were retrospectively reviewed. Mixed-effects linear regression was used to estimate changes over time at 3 to 9 months, 1 to 5 years, and >5 years after Fontan., Results: A total of 119 patients were included (51% with hypoplastic left heart syndrome; 77% underwent extracardiac Fontan). A total of 88 patients had 3 serial CMRs. Indexed right superior vena cava, inferior vena cava, neoaortic valve, and descending aorta area decreased over time (beta = -0.19, -0.44, and -0.23, respectively; P < .01), as did indexed right superior vena cava, neoaorta and native aorta, and descending aorta flow (beta = -0.49, -0.53, and -0.59, respectively; P < .0001). Inferior vena cava flow and its contribution to total caval flow increased (beta = 0.33; P < .0001). Indexed right and left pulmonary artery flow did not change; however, indexed left pulmonary artery area decreased (beta = -0.16; P = .0014) with time. Systemic-to-pulmonary collateral flow remained unchanged before and early after Fontan (beta = -0.54; P = .42) but decreased with time from Fontan (beta = -0.22; P < .0001)., Conclusions: In this cohort of longitudinally followed SV patients, there are significant trends in vascular size and flow over time from Fontan. These findings can be used as a framework to interpret serial CMR data in the SV and noninvasively identify deviations from expected patterns before the development of clinical symptoms., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Patients with single ventricle (SV) may often undergo aortic reconstruction that creates a stiff large vessel, increasing afterload and affecting exercise performance. The objective of this study was to determine the relationship of pulse wave velocity (PWV) and distensibility in reconstructed and normal aortic arches after Fontan with exercise variables., Methods: PWV and distensibility of the descending aorta at the level of the diaphragm (DAo) were calculated with real-time exercise cardiac magnetic resonance in 48 patients with SV after Fontan (18 after aortic reconstruction; 30 without aortic reconstruction) and compared with metabolic exercise stress test variables., Results: PWV was greater in the reconstructed group than in the non-reconstructed group (median 4.4 m/s [range: 2.3 to 9.8 m/s] versus 3.6 [range: 2.6 to 6.3 m/s], respectively, p = 0.003). Statistically significant inverse correlations were found between PWV and end-diastolic, end-systolic, and stroke volumes at rest and at exercise in the reconstructed group. In addition, inverse correlations also existed in the reconstructed group between distensibility of the DAo and the exercise variables such as peak oxygen pulse (R = 0.56, p = 0.02), peak oxygen consumption (R = 0.63, p = 0.008), oxygen consumption at ventilatory anaerobic threshold (R = 0.48, p = 0.04), and peak work (R = 0.54, p = 0.02). Similar correlations were not seen in patients with non-reconstructed aortas., Conclusions: Patients with SV with reconstructed aortas have increased aortic stiffness, increasing afterload on the ventricle. Native DAo stiffness distal to the reconstruction is inversely correlated with exercise performance, presumably to decrease impedance mismatch to maintain homogeneity of the aortic wall. This information suggests a possible mechanism for decreased exercise performance in patients with SV with aortic reconstructions., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs., Methods: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles., Results: Of 152 ACHD patients (median age 33years, 50% female), 13% reported previous ACP discussions with providers and 21% had completed advance directives. On a 0-10 scale, the median rating for the importance of discussing ACP with providers was 7; 18years was identified as the most appropriate age to initiate this dialogue. Higher ratings for the importance of discussing ACP with providers was observed in patients who were female (p=0.03), had lower disease complexity (p=0.03), and had elevated anxiety symptoms (p=0.001); elevated anxiety remained significant in a multivariable model. Interest in receiving information about life expectancy (61% overall) was greater among patients with lower disease complexity (p=0.04) and a history of ≥2 cardiac surgeries (p=0.01); disease complexity remained significant in a multivariable model., Conclusions: As a group, ACHD patients value the opportunity for ACP discussions and prefer earlier communication. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, such avoidance does not appear to be warranted., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Postpneumonectomy syndrome is a recognized complication following pneumonectomy, resulting from mediastinal displacement into the vacated pleural space. Mediastinal displacement causes bronchial compression and dyspnea. This report describes a 47-year-old woman who presented with shortness of breath 6 months after a left pneumonectomy. She was initially hypoxic and was found to have a patent foramen ovale. Following closure of the patent foramen ovale, she was no longer hypoxic, but she continued to have dyspnea with exertion and left lateral decubitus positioning. There was no evidence of bronchial compression, but MRI and transesophageal echocardiography suggested pulmonary vein compression due to mediastinal displacement. Transthoracic echocardiography confirmed functional pulmonary vein compression that was worse in the standing and in the left lateral positions. Her symptoms improved with mediastinal repositioning using a transesophageal echocardiography-guided approach. Positional pulmonary vein compression may be an underdiagnosed complication of pneumonectomy.