Survival and outcomes of patients with unoperated single ventricle

Poterucha, Joseph T.; Anavekar, Nandan S.; Egbe, Alexander C.; Julsrud, Paul R.; Connolly, Heidi M.; Ammash, Naser M.; Warnes, Carole A.

doi:10.1136/heartjnl-2015-308440

Cited by 28 publications

(23 citation statements)

References 26 publications

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“…In contrast to Fontan results, a small, selected subset of DILV patients have been reported to live beyond their 4 th decade without Fontan circulation [ 7 – 9 ]. The inter-individual arterial oxygen saturation and therefore the clinical features amongst DILV patients vary widely due to the numerous anatomical differences and the consequent presence or absence of pulmonary and/or systemic obstruction as well as the differences in systemic and pulmonary vascular resistance.…”

Section: Introductionmentioning

confidence: 99%

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles

Jongbloed

Bartelings

et al. 2017

PLoS ONE

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The aim of this study was to gain better understanding of the variable anatomical features of double inlet left ventricle hearts without cavopulmonary connection that would potentially facilitate favorable streaming. Thirty-nine post-mortem specimens of double inlet left ventricle without cavopulmonary connection were investigated. The focus was on anatomical characteristics that could influence the flow and separation of deoxygenated and oxygenated blood in the ventricles. Elements of interest were the ventriculoarterial connection, the spatial relationship of the ventricles, the position and size of the great arteries, the ventricular septal defect, the presence of relative outflow tract stenosis and the relationship of the inflow and outflow tracts. The most common anatomy was a discordant ventriculoarterial connection with an anatomically left-sided morphologically right ventricle (n = 12, 31%). When looking at the pulmonary trunk/aorta ratio, 21 (72%) hearts showed no pulmonary stenosis relative to the aorta. The ventricular septal defect created a relative subpulmonary or subaortic stenosis in 13 (41%) cases. Sixteen (41%) hearts had a parallel relationship of the inflow and outflow tracts, facilitating separation of deoxygenated and oxygenated blood streams. On the other end of the spectrum were 10 (25%) hearts with a perpendicular relationship, which might lead to maximum mixing of the blood streams. The relationship of the inflow and outflow tracts as well as the presence of (sub-) pulmonary stenosis might play a crucial role in the distribution of blood in double inlet left ventricle hearts. Additional in vivo studies will be necessary to confirm this postulation.

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Section: Introductionmentioning

confidence: 99%

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles

Jongbloed

Bartelings

et al. 2017

PLoS ONE

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“…1 Recently, the Mayo Clinic presented the data of 24 unoperated single-ventricle patients. 2 In cases of moderate pulmonary stenosis, blood flow to the pulmonary vasculature was almost normal and this led to no or mild heart failure, mild arterial desaturation, and no pulmonary vascular disease. Thus long-term survival is likely with adequate oxygenation and a balanced volume load of the ventricle.…”

Section: Discussionmentioning

confidence: 99%

Living with functionally univentricular heart: beating the odds?

Alpat

Demircin

2017

Asian Cardiovasc Thorac Ann

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An unoperated 45-year-old man with double-inlet single left ventricle, transposed great arteries, ventricular septal defect, and pulmonary stenosis presented with mitral and pulmonary valvular vegetations unresponsive to antibiotic therapy. The initial diagnosis was made with echocardiography, and the morphology was delineated with computed tomography and magnetic resonance imaging. The patient underwent mitral valve replacement with a mechanical valve and pulmonary valve vegetectomy. He recovered well and he is currently well two years after the operation.

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“…There are reports of single ventricle patients surviving into adulthood unoperated. 76 Most often, these patients have a diagnosis of double-inlet left ventricle with transposed great vessels and pulmonary stenosis, a physiology that allows survival into adulthood with good quality of life. Unfortunately, Fontan takedown to balanced physiology raises numerous questions: will the single ventricle tolerate the volume load, what will be the new source of pulmonary blood flow, and what size shunt will maintain adequate saturations and not create heart failure?…”

Section: Balanced Physiologymentioning

confidence: 99%

“…There are reports of single ventricle patients surviving into adulthood unoperated . Most often, these patients have a diagnosis of double‐inlet left ventricle with transposed great vessels and pulmonary stenosis, a physiology that allows survival into adulthood with good quality of life.…”

Section: Surgical Interventions For Failing Fontan Patients (Brian Komentioning

confidence: 99%

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

Ephrem

Hebson

John

et al. 2018

Congenital Heart Disease

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The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to. K E Y W O R D S failure, Fontan, innovation, outcomes | 129 EphrEm Et al.

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Survival and outcomes of patients with unoperated single ventricle

Cited by 28 publications

References 26 publications

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles

Living with functionally univentricular heart: beating the odds?

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

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