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Right Ventricle and Tricuspid Valve Function at Mid-term Following the Fontan Operation for Hypoplastic Left Heart Syndrome: Impact of Shunt Type
Victor Bautista-Hernandez, Ravi Thiagarajan, Hugo Loyola, Jared Schiff, Joshua Salvin, John E. Mayer, Mark Scheurer, Frank A. Pigula, Francis Fynn-Thompson, Pedro J. del Nido, Emile A. Bacha; Children's Hospital Boston. Harvard Medical School., Boston, MA

Objective: Concerns exist about late ventricular dysfunction and tricuspid valve (TV) function in patients with hypoplastic left heart syndrome (HLHS) palliated initially with a right ventricle-pulmonary artery conduit (RV-PA). The aim of this study was to evaluate the mid-term RV, TV and neo-aortic valve (neo-AV) function and clinical outcomes in patients with HLHS after completion of staged palliation based on the type of shunt used at stage I reconstruction.
Methods: Retrospective review of records of all patients with HLHS who had completed Fontan palliation between 2000 through 2007. The outcome variables were: RV function, TV and neo-AV regurgitation (from latest post-Fontan echocardiogram), cardiac index (CI), pulmonary vascular resistance (PVR) and pressure (PAp) and right ventricular end diastolic pressure (RVEDp) (from latest post-Fontan catheterization). Clinical status was obtained from medical records and by contact with the referring cardiologist if necessary.
Results: Of 118 HLHS patients (76 males) undergoing a Fontan for HLHS, 116 had a fenestrated lateral tunnel and 2 an extra-cardiac conduit. At stage I, 36 patients had an RV-PA conduit and 82 patients a Blalock-Taussig shunt (BTS). All patients survived the Fontan and were discharged home. Three patients were lost to follow-up. At a mean follow-up post Fontan of 27.6 months (range 0.2 to 88.9 months), 4 patients had died and 1 had the Fontan circulation taken-down. No patient underwent a heart transplant. Most recent follow-up echocardiograms from 115 patients (mean f/u in months of 14.5 for RV-PA and 34.8 for BTS) and catheterizations from 66 (mean f/u in months of 18.8 for RV-PA and 43.6 for BTS)were reviewed. Hemodynamic results for RV-PA conduits versus BTS were, CI 3.3 ± 0.69 vs 3.4 ± 1.15, PVR 2.0 ± 0.8 vs 1.7 ± 0.8, PAp 13.7 ± 3.1 vs 13.6 ± 4.4, RVEDp 8 ± 4.3 vs 9.1 ± 4.8, respectively. No statistically significant differences were found between shunt types in terms of survival, degree of RV dysfunction, TV or neo-AV regurgitation, CI, PVR, PAp or RVEDp. Latest echocardiographic data is shown in table I.
Conclusion: Contemporary results after Fontan palliation for HLHS are excellent. At mid-term after the Fontan, there were no differences in terms of RV function, TV or neo-AV function or survival based on type of shunt used at stage I palliation.

Table I. - Latest echocardiographic data in patients with HLHS after completion of staged palliation based on the type of shunt used at stage I reconstruction.
Shunt type RV function
None or Trivial Mild Moderate Severe Total Fisher's exact test
RV-PA conduit 20 (55.6%) 12 (33.3%) 3 (8.3%) 1 (2.8%) 36 (100%) 0.315
BTS 53 (67.1%) 14 (17.7%) 7 (8.9%) 5 (6.3%) 79 (100%)
TV regurgitation
RV-PA conduit 15 (41.7%) 19 (52.8%) 2 (5.6%) 0 (0%) 36 (100%) 0.271
BTS 20 (25.6%) 47 (60.3%) 10 (12.8%) 1 (1.3%) 78 (100%)
Neo-aortic regurgitation
RV-PA conduit 26 (83.9%) 5 (16.1%) 0 (0%) 0 (0%) 31 (100%) 0.441
BTS 54 (75%) 18 (25%) 0 (0%) 0 (0%) 72 (100%)


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