Prospective echocardiography assessment of pulmonary hypertension and its potential etiologies in children with sickle cell disease.
Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle-cell disease (SCD), but its importance in children is less clear. The aim of this study was to define the incidence and causes of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk tests, and hematologic testing. Echocardiographic measures were compared between patients with SCD and control subjects before and after adjusting for hemoglobin. Correlations of echocardiographic and clinical parameters were determined. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlling for left ventricular diameter and left atrial pressure. Echocardiographically derived pulmonary resistance was not significantly different between patients with SCD and controls, although it was elevated in the SCD subgroup with elevated TRV. When controlling for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness, and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end-diastolic velocity, and markers of increased cardiac output were correlated with indicators of adverse functional status, including history of acute chest syndrome, stroke, transfusions, and 6-minute walk distance. In conclusion, children with SCD had mildly increased TRV that was correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin-adjusted analysis also suggested a contribution of primary vascular changes.
Dham
Niti
N
Department of Cardiology, Children's National Medical Center, Washington, District of Columbia, USA.
Ensing
Gregory
G
Minniti
Caterina
C
Campbell
Andrew
A
Arteta
Manuel
M
Rana
Sohail
S
Darbari
Deepika
D
Nouraie
Mehdi
M
Onyekwere
Onyinye
O
Lasota
Malgorzata
M
Kato
Gregory J
GJ
Gladwin
Mark T
MT
Castro
Oswaldo
O
Gordeuk
Victor
V
Sable
Craig
C
D000755:Anemia, Sickle Cell / Q000150:complications*; D002302:Cardiac Output; D002648:Child; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D008297:Male; D011446:Prospective Studies; D014261:Tricuspid Valve / Q000503:physiopathology; D014655:Vascular Resistance; D016277:Ventricular Function, Left
N Engl J Med. 2004 Feb 26;350(9):886-95
14985486
Eur Heart J. 1997 Jul;18(7):1175-85
9243153
Am J Med. 2004 Nov 1;117(9):665-9
15501204
Blood. 2006 Mar 15;107(6):2279-85
16291595
Acta Cardiol. 2005 Oct;60(5):471-6
16261776
J Am Coll Cardiol. 2007 Jan 30;49(4):472-9
17258093
J Am Soc Echocardiogr. 2005 Dec;18(12):1440-63
16376782
J Am Coll Cardiol. 2006 Jun 6;47(11):2283-8
16750697
Chest. 1995 Nov;108(5):1214-9
7587419
Pediatr Cardiol. 1999 May-Jun;20(3):221-3
10089251
J Am Soc Echocardiogr. 2006 Dec;19(12):1413-30
17138024
Am J Cardiol. 2008 Jun 1;101(11):1673-6
18489950
Pediatrics. 2008 Apr;121(4):777-82
18381543
J Pediatr. 2006 Jul;149(1):3-4
16860112
Am J Hematol. 2007 Jun;82(6):433-8
17266053
J Am Coll Cardiol. 1985 Aug;6(2):359-65
4019921
Pediatr Cardiol. 2006 Jul-Aug;27(4):440-6
16835804
J Am Soc Echocardiogr. 2008 Aug;21(8):922-34
18406572
Blood. 2003 Feb 15;101(4):1257-61
12393669
J Pediatr Hematol Oncol. 2007 May;29(5):334-7
17483714
Am J Respir Crit Care Med. 2002 Jul 1;166(1):111-7
12091180
Am J Cardiol. 1990 Aug 15;66(4):493-6
2386120
J Am Soc Echocardiogr. 2002 Feb;15(2):167-84
11836492
Haematologica. 2009 Mar;94(3):340-7
19211639
J Pediatr Hematol Oncol. 2005 Dec;27(12):654-8
16344670
J Am Soc Echocardiogr. 2009 Feb;22(2):107-33
19187853
J Am Coll Cardiol. 1997 Nov 15;30(6):1527-33
9362412
J Heart Lung Transplant. 2007 Sep;26(9):927-34
17845932
Pediatr Blood Cancer. 2006 Dec;47(7):907-13
16496290
Pediatr Cardiol. 2008 Mar;29(2):309-12
17680298