Single ventricle with severe pulmonary hypertension: Natural survival into the third decade of life

Steinberg, Eric H; Dantzker, David R.

doi:10.1016/0002-8703(93)91029-e

Cited by 18 publications

(11 citation statements)

References 7 publications

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“…Interestingly, the majority of patients (18/34) to survive into the fifth decade, when combining our cohort and the cases from the literature, had DILV with PS. This subtype has been suggested as an ideal phenotype for prolonged survival in the SV patient with a ‘perfectly balanced’ circulation 4 5. This SV subtype has been shown to provide preferential streaming of pulmonary venous return into the systemic circulation with higher oxygen saturation 6.…”

Section: Discussionmentioning

confidence: 99%

Survival and outcomes of patients with unoperated single ventricle

Poterucha

Anavekar

Egbe

et al. 2015

Heart

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Selected patients with unoperated SV with PS and ES can survive with good functional class up to the eighth decade with good medical management. DILV/PS appears to be the ideal phenotype for advanced survival. Our outcomes may be considered when such patients with SV having 'balanced' physiology are evaluated for Fontan palliation. However, additional prospective study will be necessary to verify this assertion.

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Section: Discussionmentioning

confidence: 99%

Survival and outcomes of patients with unoperated single ventricle

Poterucha

Anavekar

Egbe

et al. 2015

Heart

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“…While this wide spectrum of cardiac malformations accounts for less than 5% of congenital heart diseases,1 2 the management of these patients is difficult and has to be adapted to each individual, and most patients with univentricular heart have to undergo repeated palliative procedures during infancy. In spite of improvements in surgical techniques, mortality remains high and heart transplantation is often the ultimate procedure in adolescents and adults with this heart defect 2-7…”

mentioning

confidence: 99%

Quality of life and perceived health status in surviving adults with univentricular heart

Saliba

Butera

Bonnet

et al. 2001

Heart

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Objective-To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. Design and setting-Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. Patients-The health records of 89 survivors with univentricular heart (median age 21 years; range 17-49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. Results-The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. Conclusions-Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. (Heart 2001;86:69-73)

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“…The first important defect is the discordance of the great arteries, which allows preferential streaming of oxygenated blood from the left atrium into the aorta. The second important defect is a moderate degree of PS, which permits enough blood flow to the lungs to prevent severe cyanosis while avoiding volume overload on the single ventricle from shunting of excessive blood back into the pulmonary artery [3]. Of note, both of these defects were found in our patient.…”

Section: Discussionmentioning

confidence: 58%