Late pulmonary valve replacement in patients with pulmonary atresia and intact ventricular septum: a case-matched study.
BACKGROUND
Pulmonary valve replacement (PVR) is a common therapy for chronic pulmonary regurgitation. However, the use of this strategy is mostly based on the studies performed on patients with tetralogy of Fallot (TOF) and not in patients with pulmonary atresia/intact ventricular septum (PA/IVS). The aim of this study is to evaluate our experience with PVR in patients with PA/IVS and compare them with a matched cohort of TOF patients.


METHODS
Between 1995 and 2009, 13 patients with PA/IVS underwent a late PVR. Matched TOF control subjects were identified for 12 of these patients. Before and after PVR echocardiographic, magnetic resonance imaging, exercise test, Holter, and electrocardiographic data were compared between groups.


RESULTS
There was no mortality in either group. The PVR improved pulmonary regurgitant fraction and right ventricular volumes in all patients. Patients with PA/IVS had more significant tricuspid regurgitation (TR [at least moderate]) by echocardiography and magnetic resonance imaging before PVR (n = 11 [85%] versus n = 1 [8%]; p = 0.003) and had more tricuspid valve repairs than TOF patients (n = 9 [69%] versus n = 1 [8%]; p = 0.004). Repair was undertaken by a combination of techniques. Although TR was improved early postoperatively, only 2 of 9 patients (22%) were free from significant TR at most recent follow-up (median 2.5 years; range, 0.1 to 10.9). No patient underwent reoperation at latest follow-up.


CONCLUSIONS
Patients with PA/IVS can undergo a late PVR with excellent results. Significant TR and repair are more commonly observed among patients with PA/IVS compared with TOF patients. Although tricuspid valve repair improves regurgitation early, TR tends to recur, suggesting the need for further refinement of current surgical techniques.
Department of Cardiac Surgery, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
Victor
V
Bautista-Hernandez
Babar S
BS
Hasan
David M
DM
Harrild
Ashwin
A
Prakash
Diego
D
Porras
John E
JE
Mayer
Pedro J
PJ
del Nido
Frank A
FA
Pigula
D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods; D016022:Case-Control Studies; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D005080:Exercise Test; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D011184:Postoperative Period; D057234:Preoperative Period; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000601:surgery*; D011664:Pulmonary Valve / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome; D054088:Ventricular Septum
J Am Coll Cardiol. 2000 Nov 1;36(5):1670-5
11079675
J Thorac Cardiovasc Surg. 2001 Feb;121(2):344-51
11174741
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;:29-34
16638544
J Am Coll Cardiol. 2004 Mar 17;43(6):1068-74
15028368
Am J Cardiol. 2003 Sep 1;92(5):643-5
12943899
Pediatr Cardiol. 1990 Apr;11(2):86-90
2349148
Arch Pathol Lab Med. 1978 Jan;102(1):28-31
579592
J Thorac Cardiovasc Surg. 2007 Jan;133(1):215-23
17198815
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;:11-22
16638542
J Am Coll Cardiol. 1997 Nov 1;30(5):1374-83
9350942
Circulation. 2002 Sep 24;106(13):1703-7
12270866
Circulation. 1982 Aug;66(2):266-72
7094236
Pediatrics. 2006 Aug;118(2):e415-20
16882782
Ann Thorac Surg. 2007 Aug;84(2):574-9; discussion 579-80
17643638