Outcomes of Cardiac Transplantation in Single-Ventricle Patients With Plastic Bronchitis: A Multicenter Study

Gossett, Jeffrey G.; Almond, Christopher S.; Kirk, Richard; Zangwill, Steven; Richmond, Marc E.; Kantor, Paul F; Tresler, Margaret; Lenderman, Susanna M.; Naftel, David C.; Matthews, Kathleen L.; Pahl, Elfriede

doi:10.1016/j.jacc.2012.10.042

Cited by 45 publications

(32 citation statements)

References 2 publications

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“…36 Smaller reports also describe a high mortality when Fontan patients receive transplants, 37 although excellent single-center results 38 and case series describing success using transplantation as rescue therapy for early failed Fontan patients 39 also exist. Importantly, transplantation as reported in the literature corrects PLE 34 and plastic bronchitis 40 in patients who survive beyond the early transplantation phase.…”

Section: Single Ventriclementioning

confidence: 87%

“…A recent small pediatric series showed increased risk early after transplantation; however, patients with plastic bronchitis had a 70% 5-year survival, which suggests that the presence of plastic bronchitis should not preclude transplantation. 40 Data in adults are lacking. Chronic venous hypertension, secondary to elevated systemic ventricular end-diastolic pressures, lymphatic drainage abnormalities, endothelial dysfunction, and possible venous thromboembolic disease, is common and may be difficult to accurately assess before transplantation, only manifesting as increased PA pressures or RV failure after transplantation.…”

Section: Systemic Venous Anomalies In Patients With Situs Inversus Ormentioning

confidence: 99%

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Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Ross¹,

Law²,

Book³

et al. 2016

Circulation

122

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Section: Single Ventriclementioning

confidence: 87%

Section: Systemic Venous Anomalies In Patients With Situs Inversus Ormentioning

confidence: 99%

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Ross¹,

Law²,

Book³

et al. 2016

Circulation

122

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“…This life-threatening complication of FFP is rare and as such is poorly understood, but is reported in small series to increase peri-transplant mortality (30).…”

Section: Ple and Pbmentioning

confidence: 99%

Transplantation in the single ventricle population

Kenny¹,

DeRita²,

Nassar³

et al. 2018

Ann. Cardiothorac. Surg.

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The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair.Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multidisciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.

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“…Single centers do not have a sufficient number of patients to adequately power studies. Multicenter research, though necessary to effectively study rare diseases using traditional methods, has been limited 8 and still involves a relatively small number of patients. Conversely, larger patient groups are known to gather “virtually” online in various social media forums.…”

Section: Introductionmentioning

confidence: 99%

Fontan-Associated Protein-Losing Enteropathy and Plastic Bronchitis

Schumacher

Stringer

Donohue

et al. 2015

The Journal of Pediatrics

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Objective Characterize the medical history, disease progression, and treatment of current-era patients with the rare diseases Fontan-associated protein losing enteropathy (PLE) and plastic bronchitis (PB). Study Design A novel survey that queried demographics, medical details, and treatment information was piloted and placed online via a Facebook portal allowing social media to power the study. Participation regardless of PLE or PB diagnosis was allowed. Case control analyses compared patients with PLE and PB to uncomplicated control Fontan patients. Results The survey was completed by 671 subjects including 76 with PLE, 46 with PB, and 7 with both. Median PLE diagnosis was 2.5 years post-Fontan. Hospitalization for PLE occurred in 71% with 41% hospitalized ≥ 3 times. Therapy varied significantly. PLE patients more commonly had hypoplastic left ventricle (62% vs 44% control; OR 2.8, 95% CI 1.4–5.5), chylothorax (66% vs 41%; OR 3.0, CI 1.6–5.3), and cardiothoracic surgery in addition to staged palliation (17% vs 5%; OR 4.3, CI 1.6, 11.2). Median PB diagnosis was 2 years post-Fontan. Hospitalization for PB occurred in 91% with 61% hospitalized ≥3 times. Therapy was very diverse. PB patients more commonly had chylothorax at any surgery (72% vs 51%; OR 2.5, CI 1.2–5.1) and seasonal allergies (52% vs 36%; OR 2.0, CI 1.0–3.9). Conclusions Patient-specific factors are associated with diagnoses of PLE or PB. Treatment strategies are diverse without clear patterns. These results provide a foundation upon which to design future therapeutic studies and identify a clear need for forming consensus approaches to treatment.

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Outcomes of Cardiac Transplantation in Single-Ventricle Patients With Plastic Bronchitis: A Multicenter Study

Cited by 45 publications

References 2 publications

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

Transplantation in the single ventricle population

Fontan-Associated Protein-Losing Enteropathy and Plastic Bronchitis

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