Pulmonary Vascular Disease in the Single-Ventricle Patient: Is it Really Pulmonary Hypertension and if So, How and When Should We Treat it?

Handler, Stephanie S.; Feinstein, Jeffrey A.

doi:10.21693/1933-088x-18.1.14

Cited by 2 publications

(3 citation statements)

References 28 publications

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“…An additional source of pulmonary flow from the native tract, aortopulmonary collaterals, and the presence of decompressing veins contributes to inaccuracies in calculating pulmonary blood flow. Cardiac magnetic resonance imaging (MRI) may provide additional flow data that may help with the calculation of pulmonary blood flow and PVR [10].…”

Section: Pvr In Fontan Circulationmentioning

confidence: 99%

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Heart transplantation for patients with single ventricle physiology

Ganapathy¹,

Gnanasekharan²,

Sharma

et al. 2021

Indian J Thorac Cardiovasc Surg

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Background There is a growing population of palliated and unpalliated single ventricle physiology patients for whom heart transplantation is the only treatment option available. There is a paucity of reports of heart transplantation in this challenging and growing subset of patients from our part of the world. The purpose of the article is to briefly review our experience in the subgroup and compare it with the available literature. Methods This was a single-institution retrospective observational study of 16 patients with single ventricle physiology who were transplanted between 2016 and 2019 and their outcomes. The study groups were divided into those with ventricular dysfunction (group 1), who fare substantially better than those with normal ventricular function (group 2) whose short-term outcomes were poorer. Worsening cyanosis, poor candidature for completion Fontan procedure due to severe atrioventricular valve regurgitation or pulmonary artery anatomy, protein-losing enteropathy, plastic bronchitis, and worsening systemic venous congestion are indications in those with normal ventricular function. Results Patients with ventricular dysfunction as the main indication had excellent early survival with no early mortality compared to 40% mortality in patients with normal ventricular function. Patients who survived to leave the hospital had however similar long-term outcomes. Two patients with protein-losing enteropathy resolved completely by one month. Normal ventricular function, pulmonary artery stenting, early Fontan failure (6 months), ascites, and need for desensitization were risk factors for early mortality. After the early acute phase of increased risk, the mortality risk plateaued off. Conclusion Transplantation in patients with single ventricle and ventricular dysfunction can be offered with a good early and late outcome. There is a need to have multi-institutional and multi-disciplinary collaboration along with work in basic sciences to better understand the effects of failed Fontan physiology with normal ventricular function.

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Section: Pvr In Fontan Circulationmentioning

confidence: 99%

“…Early attrition from the single ventricle pathway due to pulmonary vascular disease is better treated with transplantation as PVR in this group is rarely high enough to preclude transplantation. Moreover, long-term outcomes in high-risk Fontan palliation patients remain poor [10].…”

Section: Pvr In Fontan Circulationmentioning

confidence: 99%

Heart transplantation for patients with single ventricle physiology

Ganapathy¹,

Gnanasekharan²,

Sharma

et al. 2021

Indian J Thorac Cardiovasc Surg

View full text Add to dashboard Cite

show abstract

“…15 Although there is no well-defined cutoff for PVR, normal PVR <3 Indexed Wood units or even <2 Indexed Wood units may be required for the most favorable outcomes; absolute cutoffs are not clear. 16 Cardiac MRI has been utilized to evaluate patients for SCPC candidacy in lieu of cardiac catheterization. In wellselected patients, no differences in short-or long-term outcomes after SCPC procedure have been demonstrated.…”

Section: The Stage II Procedures and Physiologic Implicationsmentioning

confidence: 99%

Superior Cavopulmonary Connection: Its Physiology, Limitations, and Anesthetic Implications

Choi

DiNardo

Brown

2020

Semin Cardiothorac Vasc Anesth

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The superior cavopulmonary connection (SCPC) or “bidirectional Glenn” is an integral, intermediate stage in palliation of single ventricle patients to the Fontan procedure. The procedure, normally performed at 3 to 6 months of life, increases effective pulmonary blood flow and reduces the ventricular volume load in patients with single ventricle (parallel circulation) physiology. While the SCPC, with or without additional sources of pulmonary blood flow, cannot be considered a long-term palliation strategy, there are a subset of patients who require SCPC palliation for a longer interval than the typical patient. In this article, we will review the physiology of SCPC, the consequences of prolonged SCPC palliation, and modes of failure. We will also discuss strategies to augment pulmonary blood flow in the presence of an SCPC. The anesthetic considerations in SCPC patients will also be discussed, as these patients may present for noncardiac surgery from infancy to adulthood.

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Pulmonary Vascular Disease in the Single-Ventricle Patient: Is it Really Pulmonary Hypertension and if So, How and When Should We Treat it?

Cited by 2 publications

References 28 publications

Heart transplantation for patients with single ventricle physiology

Heart transplantation for patients with single ventricle physiology

Superior Cavopulmonary Connection: Its Physiology, Limitations, and Anesthetic Implications

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