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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Survival and Clinical Course at Fontan After Stage One Palliation With Either a Modified Blalock-Taussig Shunt or a Right Ventricle to Pulmonary Artery Conduit

Mark A. Scheurer, MD^_*,,_*, Joshua W. Salvin, MD^_*,, Vladimiro L. Vida, MD^||, Francis Fynn-Thompson, MD^,, Emile A. Bacha, MD^,, Frank A. Pigula, MD^,, John E. Mayer, Jr, MD^,, Pedro J. del Nido, MD^,, David L. Wessel, MD^¶, Peter C. Laussen, MBBS^_*, and Ravi R. Thiagarajan, MBBS, MPH^_*,

^_* Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, Massachusetts
Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts
Department of Surgery, Harvard Medical School, Boston, Massachusetts
^|| Pediatric and Congenital Cardiac Surgery Unit, Centro Gallucci, University of Padua Medical School, Padua, Italy
^¶ Children's National Medical Center, Washington, DC.

Manuscript received July 27, 2007; revised manuscript received February 27, 2008, accepted March 11, 2008.

^_* Reprint requests and correspondence: Dr. Mark Scheurer, Instructor of Pediatrics, Harvard Medical School, Cardiac Intensivist, Department of Cardiology, 300 Longwood Avenue, Boston, Massachusetts 02115. (Email: mark.scheurer{at}cardio.chboston.org).

Objectives: We sought to determine whether the type of shunt used at stage one palliation (S1P) affected the survival and the perioperative course through Fontan completion.

Background: Although improved surgical and interstage survival have been demonstrated with the use of the right ventricle to pulmonary artery (RV-PA) conduit compared with a modified Blalock-Taussig shunt (BTS) at S1P, it is unknown whether this effect will be observed in long-term follow-up.

Methods: All patients who underwent a S1P during 2002 and 2003 (n = 80) at our institution were included for analysis. Patients were followed until death or June 1, 2007. Perioperative variables at Fontan completion were recorded.

Results: For the entire cohort, cumulative survival for those who underwent a RV-PA conduit (n = 34) was 79.4% at 3 years compared with 65.8% in the modified BTS group (n = 46) (log-rank = 0.31). At Fontan (n = 44), when compared with those who had received a modified BTS, those who had a RV-PA conduit placed at S1P had no difference in the median duration of ventilation (21 h [range 10 to 96 h] vs. 26.5 h [range 7 to 204 h], p = 0.09) or hospital stay (9 days [range 5 to 29 days] vs. 10 days [range 6 to 48 days], p = 0.89), although length of stay in the intensive care unit was shorter (2 days [range 0 to 6 days] vs. 4 days [range 1 to 25 days], p = 0.01). Sixty-seven percent of the RV-PA conduit group had at least one PA intervention 3 years after S1P compared with 42.8% in the modified BTS group (log-rank = 0.11).

Conclusions: Nonstatistically significant trends toward improved cumulative survival and increased PA interventions were demonstrated in patients who had a RV-PA conduit placed at S1P. Longitudinal follow-up of larger groups of randomized patients is required to determine the influence of the RV-PA conduit on long-term outcomes.

Key Words: hypoplastic left heart syndrome • Fontan • Sano • Norwood

Abbreviations and Acronyms   BCPA = bidirectional cavopulmonary anastomosis   BTS = Blalock-Taussig shunt   HLHS = hypoplastic left heart syndrome   RV-PA = right ventricle to pulmonary artery   S1P = stage one palliation