Hemodynamic characteristics of cyanotic adults with single-ventricle physiology without Fontan completion.
OBJECTIVE
The aim of the current study is to describe the long-term clinical and hemodynamic characteristics of adult patients with single-ventricle physiology who have not undergone the Fontan operation and consequently have remained cyanotic.


METHODS
Adult patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center with non-Fontan single-ventricle physiology who had undergone cardiac catheterization between 2005 and 2011 were included. Echocardiographic and cardiac catheterization data were reviewed.


RESULTS
Mean estimated single ejection fraction was 56 ± 8%. Eight of 13 subjects had documented E/E' data with a mean of 6.44. Seven subjects had both A' and E' data documented, of which two subjects exhibited A' > E'. Mean ventricular end-diastolic pressure (MVEDP) was 15.77 ± 4.91 mm Hg, and was >12 mm Hg in eight of the 13 patients (62%). MVEDP was also analyzed by age, and in the single-ventricle patients was 13.55 ± 4.12 mm Hg in those <50 years of age, compared with 20.75 ± 1.89 mm Hg in those >50 years of age (P = .003). MVEDP prior to inhaled pulmonary vasodilator administration was 14.75 ± 5.5 mm Hg, compared to 15.00 ± 6.78 mm Hg in the postvasodilator group (P = .48). Subjects with end-diastolic pressure (EDP) <12 had a mean brain natriuretic peptide (BNP) of 108 ± 197 pg/mL, while subjects with EDP >12 had a mean BNP of 234.5 ± 127.36 pg/mL (P = .11).


CONCLUSIONS
Cyanotic adult single-ventricle patients not palliated with Fontan completion have preserved single-ventricle systolic function but develop elevated ventricular filling pressure with increasing age. Only invasive hemodynamic measurements demonstrated elevated ventricular filling pressures, while traditional echo/Doppler criteria for diastolic dysfunction were not met. Aging with cyanotic single-ventricle physiology is associated with a greater degree of filling pressure elevations than in the general population. Single-ventricle patients with EDP >12 exhibited markedly elevated BNP compared to those with normal EDP.
David Geffen School of Medicine, University of California, Los Angeles, Calif, USA. fsaab@mednet.ucla.edu
Faysal G
FG
Saab
Jamil A
JA
Aboulhosn
D000222:Adaptation, Physiological; D000328:Adult; D000367:Age Factors; D015415:Biomarkers / Q000097:blood; D006328:Cardiac Catheterization; D016009:Chi-Square Distribution; D003490:Cyanosis / Q000097:blood / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D015150:Echocardiography, Doppler; D017079:Exercise Tolerance; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology* / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology*; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010166:Palliative Care; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D013318:Stroke Volume; D013997:Time Factors; D016276:Ventricular Function; D017725:Ventricular Pressure; D055815:Young Adult
D015415:Biomarkers; D020097:Natriuretic Peptide, Brain