Author + information
- Jeffrey G. Gossett, MD* (, )
- Christopher S. Almond, MD,
- Richard Kirk, MA,
- Steven Zangwill, MD,
- Marc E. Richmond, MD, MS,
- Paul F. Kantor, MD,
- Margaret A. Tresler, MPH,
- Susanna M. Lenderman,
- David C. Naftel, PhD,
- Kathleen L. Matthews, BA and
- Elfriede Pahl, MD
- ↵*Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 East Chicago Avenue, Box 21, Chicago, Illinois 60611-2605
To the Editor:
Plastic bronchitis (PB) is a rare condition characterized by formation of airway casts that obstruct the bronchial tree and cause respiratory failure. In the congenital cardiac population, PB occurs mostly in patients with single ventricle (SV) defects undergoing Fontan palliation. Mortality for PB is as high as 29%, with life-threatening events occurring in 41% (1). Reports on PB are limited, and it is unclear which, if any, therapy is effective; the effect of heart transplantation (HTx) on PB remains unclear (2). Using a multicenter pediatric HTx database, we investigated the outcome of SV patients listed for HTx with a diagnosis of PB and whether HTx led to resolution of PB.
The Pediatric Heart Transplant Study is an international registry acquiring data from 35 centers. A supplemental form was generated for patients listed for HTx between January 1993 and December 2009 and censored on December 31, 2010, ensuring 12 months of follow-up. Centers were provided with a list of their SV patients, regardless of surgical stage, and completed the form for any patient with PB. The diagnosis of PB was made clinically (i.e., presence of airway casts) and was not adjudicated.
There were 937 SV patients among 4,319 transplant patients. Ten of 35 centers contributed a total of 14 patients with PB who constitute our study group. PB patients were compared with 2 groups: all other SV patients (n = 923) and all other Pediatric Heart Transplant Study patients (n = 4,305). Median age at listing for study group was 6.3 years (range 3.2 to 13.6 years). All had completed Fontan procedures, and the median time from the procedure to listing was 1.9 years (range 0.1 to 10 years). Patients listed with PB were older and had a higher body surface area than either comparison group (p < 0.05). Compared with the overall population, patients with PB were less likely to be listed status 1 and more likely to have had a previous sternotomy (p < 0.05). Only 1 patient with PB had protein-losing enteropathy; there was no difference between the groups in serum albumin levels at listing (available starting 1999; n = 13). Cast pathology was available for 9 patients: 3 mucinous (acellular), 4 mucinous with inflammatory cells, and 2 chylous (fibrinous).
Ten (71%) of 14 patients with PB underwent HTx. There were 2 wait-list deaths (1 intracranial hemorrhage, 1 multiorgan system failure); 1 remained listed at study termination; and 1 patient was delisted (deemed “too well”). There was no difference in survival to HTx between patients with PB and patients from either control group (Fig. 1A). Peritransplantation survival was worse in the PB group than in controls, with 7 (70%) of 10 patients with PB surviving to 30 days post-HTx (Fig. 1B). There were 3 early deaths: postoperative days 6 (graft failure), 14 (graft dysfunction, lung disease, and renal failure), and 26 (anoxia, pulmonary hemorrhage, and renal failure). All 7 patients with PB who survived to 30 days were still alive at 1 and 5 years, with no difference in conditional survival compared with the control groups (Fig. 1C). Overall 5-year outcomes for PB patients were equivalent to control groups (Fig. 1D).
In the 10 transplanted patients, casts were documented a median of 10.5 days (range 1 to 1.1 years) before HTx. Two patients had early evidence of PB after HTx with production of casts. One died on postoperative day 6 of primary graft failure, and the other had resolution of casts after postoperative day 20. None of the 7 patients surviving more than 30 days had any recurrence of casts at a mean of 5.7 years (range 0.8 to 7.7 years) after HTx, with a cumulative follow-up of 30.8 patient-years.
In this multicenter report, we show that long-term survival of SV patients undergoing HTx in the setting of PB compares well with the general pediatric HTx population. In addition, we have specifically addressed the question of whether HTx for SV patients with PB leads to a durable resolution of the PB. In all patients surviving to 30 days after HTx, there was complete cessation of cast formation. Furthermore, we have personal communication (J Gossett [October 2012], B Kaufman [March 2011], J Scheel [March 2011]) regarding 3 other patients with PB who have had complete resolution of their PB after HTx (transplanted since termination of enrollment in the current study).
Multiple therapies for PB in SV patients have been attempted and reported in the literature, primarily directed at clearing severe airway obstruction but some aimed at resolution of cast formation. Almost all reports are isolated to single patients and centers (1,2). We separated these reports into two groups based on whether the therapy was proposed to have led to a complete resolution of cast formation. We found that in almost all cases the same therapy reported to have led to resolution of PB had also been reported to not result in a durable cessation of cast production in other accounts. This finding raises questions about the effectiveness of any other proposed therapies.
Although we believe that we have captured the majority of the field's experience with transplantation in the setting of PB, our population is small and may not be representative. Our report identified SV patients listed in the setting of PB and may not represent the overall population with PB. Extrapolation of our data to all PB patients may not be appropriate. We did not investigate patients for whom alternative therapies for PB were used and are therefore unable to compare these against the outcomes for HTx. Thus, we do not suggest that HTx is superior to alternative treatments but rather that it is an effective option in this setting and that it seems superior to published outcomes.
In summary, transplantation is a viable solution for SV patients with a diagnosis of PB. Transplantation leads to resolution of cast formation early postoperatively and to a durable resolution of PB. Although early mortality is high, conditional and long-term survival compares well with results in other pediatric HTx recipients.
Please note: The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- American College of Cardiology Foundation