Life-threatening hemoptysis following the Fontan procedure

doi:10.1016/S0828-282X(08)70572-5

Canadian Journal of Cardiology

Volume 24, Issue 2, February 2008, Pages 145-147

https://doi.org/10.1016/S0828-282X(08)70572-5 Get rights and content

Two cases of life-threatening recurrent hemoptysis occurring 10 years after a Fontan operation are presented. Bleeding from aortopulmonary collateral vessels was responsible for this complication in both cases, and the importance of systematic selective angiography of all potential origins of such abnormal vessels, including those arising from the abdominal aorta, is highlighted. Although coil embolization of aortopulmonary collateral vessels is usually definitive, pulmonary lobectomy may be necessary. The present report demonstrates, for the first time, that rescue extracorporeal membrane oxygenation support can be used as a bridge to surgery in case of severe uncontrollable hemoptysis in such cases.

Sont présentés deux cas d’hémoptysie récurrente mettant en jeu le pronostic vital dix ans après une opération de Fontan. Dans les deux cas, des saignements des vaisseaux aortopulmonaires collatéraux étaient responsables de cette complication, et on souligne l’importance de l’angiographie sélective systématique de toutes les origines possibles de ces vaisseaux anormaux, y compris ceux provenant de l’aorte abdominale. Même si l’embolisation par spirale des vaisseaux aortopulmonaires collatéraux est généralement suffisante, une lobectomie pulmonaire peut s’imposer. Pour la première fois, le présent rapport démontre que l’oxygénation extracorporelle de sauvetage sur oxygénateur à membrane peut être utilisée en attendant l’opération en cas de grave hémoptysie incontrôlable.

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Cited by (27)

Cardiac catheterization for hemoptysis in a Children's Hospital Cardiac Catheterization Laboratory: A 15 year experience
2021, Journal of Cardiology
Citation Excerpt :
A previous study showed a similar result in that the predictor for mortality was the amount of hemoptysis for the CHD patients [3]. Bédard et al. reported that the incidence rate of life-threating hemoptysis following the Fontan operation was 3.1% during median follow-up time of 12 years [15]. Due to a small sample size, there were no identifiable factors associated with development of massive hemoptysis in our cohort.
The aim of this study was to evaluate the diagnostic utility of cardiac catheterization and the efficacy of transcatheter intervention in patients with hemoptysis.
Cardiac catheterization may play a role in identifying the etiologies of hemoptysis with the potential for transcatheter intervention.
This was a retrospective study of all the patients who were brought to the pediatric cardiac catheterization laboratory for the indication of hemoptysis over a 15-year period (2006–2020).
Twenty-one patients underwent 28 cardiac catheterizations. The median age was 17.4 years (range 0.3–60.0 years), and the underlying cardiac diagnoses were normal heart n = 3, pulmonary hypertension 1, heart transplant 1, pulmonary arteriovenous malformation 1, pulmonary vein disease 3, biventricular congenital heart diseases 5, and single ventricles 7. The diagnostic utility of catheterization was 81% (17/21). At two-thirds (18/28) of catheterizations, transcatheter interventions were performed in 14/21 (67%) patients: aortopulmonary collateral embolization 14, aortopulmonary and veno-venous collateral embolization 1, and pulmonary arteriovenous malformation embolization 3. Although recurrent hemoptysis was frequent (50%) post-intervention, the final effectiveness of transcatheter interventions was 79% (11/14 patients). Overall mortality was 19% (4/21), all in those presenting with massive hemoptysis.
Cardiac catheterization was shown to have good diagnostic utility for hemoptysis especially in patients with underlying congenital heart disease. Despite the high mortality and recurrent hemoptysis rate, transcatheter interventions were effective in our cohort.
Particle embolization for hemoptysis in two patients with cyanotic congenital heart disease
2019, Progress in Pediatric Cardiology
Citation Excerpt :
These cases highlight the difficulties in managing CCHD patients with hemoptysis. Delayed bleeding from APCs 14 years after a corrective or staged procedure has been described with a prevalence of late hemoptysis as high as 12% in patients with pulmonary atresia and VSD [2–4]. Identifying CCHD children at risk for bleeding can be difficult, although the above cases demonstrate a temporal association with respiratory tract infections.
Hemoptysis is a known manifestation of complex cyanotic congenital heart disease (CCHD) often related to bleeding from systemic to pulmonary collateral vessels, commonly referred to as aortopulmonary collaterals (APCs). While catheter-directed particle embolization has been historically utilized to treat arterial bleeds causing hemoptysis in other scenarios, coil and vascular plug embolization has been used more frequently to occlude parent arteries in CCHD patients given the concern for shunting of embolic particles. Recently, some centers have shifted to distal particle embolization in treating APCs given its advantages over proximal embolization, including ease of re-treatment if hemoptysis recurs. We describe our experience with distal particle embolization in two CCHD patients experiencing recurrent hemoptysis from APCs following proximal device embolization, highlighting the challenges in endovascular management of these patients.
Extracorporeal membrane oxygenation support after the Fontan operation
2011, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
Previously published survivals after ECMO support of patients who have undergone the Fontan operation are variable. In a total of 39 patients supported with ECMO after the Fontan operations, compiled from published case reports and small patient series, 18 (46%) survived to hospital discharge.1,3-8,10,11,14-21 In a series of Fontan case subjects (n = 14) from a single institution, Booth and colleagues14 reported a survival of 50%.
Extracorporeal membrane oxygenation has been used to support children with cardiac failure after the Fontan operation. Mortality is high, and causes of mortality remain unclear. We evaluated the in-hospital mortality and factors associated with mortality in these patients.
Extracorporeal Life Support Organization registry data on patients requiring extracorporeal membrane oxygenation after the Fontan operation from 1987 to 2009 were retrospectively analyzed. Demographics and extracorporeal membrane oxygenation data were compared for survivors and nonsurvivors. A multivariable logistic regression model was used to identify factors associated with mortality.
Of 230 patients, 81 (35%) survived to hospital discharge. Cardiopulmonary resuscitation was more frequent (34% vs 17%, P = .04), and median fraction of inspired oxygen concentration was higher (1 [confidence interval, 0.9–1.0] vs 0.9 [confidence interval, 0.8–1.0], P = .03) before extracorporeal membrane oxygenation in nonsurvivors compared with survivors. Extracorporeal membrane oxygenation duration and incidence of complications, including surgical bleeding, neurologic injury, renal failure, inotrope use on extracorporeal membrane oxygenation, and bloodstream infection, were higher in nonsurvivors compared with survivors (P < .05 for all). In a multivariable model, neurologic injury (odds ratio, 5.18; 95% confidence interval, 1.97–13.61), surgical bleeding (odds ratio, 2.36; 95% confidence interval, 1.22–4.56), and renal failure (odds ratio, 2.81; 95% confidence interval, 1.41–5.59) increased mortality. Extracorporeal membrane oxygenation duration of more than 65 hours to 119 hours (odds ratio, 0.33; 95% confidence interval, 0.14–0.76) was associated with decreased mortality.
Cardiac failure requiring extracorporeal membrane oxygenation after the Fontan operation is associated with high mortality. Complications during extracorporeal membrane oxygenation support increase mortality odds. Prompt correction of surgical bleeding when possible may improve survival.
Fontan Failure: Phenotypes, Evaluation, Management and Future Directions
2022, Cardiology in the Young
Recombinant factor VIIa as a rescue therapy in severe haemoptysis in a patient with a Fontan circulation
2018, Cardiology in the Young
Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome
2011, Pediatric Critical Care Medicine
Citation Excerpt :
ECMO, although not a new modality of therapy in the setting of the Fontan operation, is much more typically used for cardiac indications (13, 16, 17). Circumstances in which ECMO has been used with varying success after the Fontan operation include support of the failing circulation postoperatively, unexpected cardiac arrest, and bridge to cardiac transplantation (16–18). Extracorporeal Membrane Life Support data from January 2010 reveals a 31–44% survival rate in patients status post the Fontan repair who require ECMO for cardiac indications (15).
To report the first successful use of extracorporeal membrane oxygenation for acute respiratory distress syndrome secondary to 2009 pH1N1 influenza A infection in a child status post the Fontan operation for hypoplastic left heart syndrome.
Individual case report.
Pediatric intensive care unit.
We describe a 7-yr-old boy with a history of the Fontan operation for hypoplastic left heart syndrome admitted with acute respiratory distress syndrome secondary to 2009 pH1N1 influenza A infection.
Cannulation for venoarterial extracorporeal membrane oxygenation.
In this patient with a history of complex congenital heart disease and repair, extracorporeal membrane oxygenation was a successful rescue therapy for refractory pH1N1-induced respiratory failure.
Extracorporeal membrane oxygenation can be successfully applied for refractory respiratory failure, even in the setting of significant underlying comorbidity. With emerging data to support the role of extracorporeal membrane oxygenation in improving mortality for refractory hypoxemia secondary to pH1N1, it is prudent to strongly consider the use of extracorporeal support in patients with underlying diseases or comorbidities that may have previously precluded them from being candidates for this therapy.

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Life-threatening hemoptysis following the Fontan procedureUne hémoptysie mettant en jeu le pronostic vital après une opération de Fontan

Ann Thorac Surg

J Am Coll Cardiol

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu