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Original Articles: Pediatric Cardiac

Late Pulmonary Valve Replacement in Patients With Pulmonary Atresia and Intact Ventricular Septum: A Case-Matched Study

^a Department of Cardiac Surgery, Children's Hospital Boston, Boston, Massachusetts
^b Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts
^c Department of Surgery, Harvard Medical School, Boston, Massachusetts
^d Department of Pediatrics, Harvard Medical School, Boston, Massachusetts

Accepted for publication September 9, 2010.

^_* Address correspondence to Dr Pigula, Department of Cardiac Surgery, Children's Hospital Boston, 300 Longwood Ave, Bader 273, Boston, MA 02115 (Email: frank.pigula{at}cardio.chboston.org).

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.

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Invited Commentary

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Ann. Thorac. Surg. 2011 91: 560. [Extract] [Full Text] [PDF]

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