Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome

Haseyama, Keiji; Satomi, Gengi; Yasukochi, Satoshi; Matsui, Hikoro; Harada, Yukihiro; Uchita, Shunji

doi:10.1016/j.jtcvs.2006.05.067

Cited by 73 publications

(56 citation statements)

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“…8 Medical treatment with sildenafil, steroids, and mucolytics have also been reported to improve symptoms. [9][10][11] Finally, heart transplantation can result in longterm resolution of the disease. 12 Assuming central lymphatic involvement, thoracic duct (TD) ligation has been performed with reports of symptomatic improvement in some cases.…”

Section: Clinical Perspective On P 1170mentioning

confidence: 99%

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

et al. 2016

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Section: Clinical Perspective On P 1170mentioning

confidence: 99%

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

et al. 2016

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“…Previously tried medications have included inhaled or systemic corticosteroids (26), mucolytics (5), antibiotics (27), pulmonary vasodilators (28), and anticoagulants, such as heparin (29). Nonpharmaceutical treatments have included cast removal via bronchoscopy (30), vest therapy, fenestration of the Fontan circuit (31), thoracic duct ligation (32), atrioventricular synchronization (33), extracorporeal membrane oxygenation (13), and heart transplantation for patients with severe cardiac conditions (34).…”

Section: Clinical Relevancementioning

confidence: 99%

Tissue Plasminogen Activator Prevents Mortality from Sulfur Mustard Analog–Induced Airway Obstruction

Veress

Hendry‐Hofer²,

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et al. 2013

Am J Respir Cell Mol Biol

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Sulfur mustard (SM) inhalation causes the rare but life-threatening disorder of plastic bronchitis, characterized by bronchial cast formation, resulting in severe airway obstruction that can lead to respiratory failure and death. Mortality in those requiring intubation is greater than 80%. To date, no antidote exists for SM toxicity. In addition, therapies for plastic bronchitis are solely anecdotal, due to lack of systematic research available to assess drug efficacy in improving mortality and/or morbidity. Adult rats exposed to SM analog were treated with intratracheal tissue plasminogen activator (tPA) (0.15-0.7 mg/kg, 5.5 and 6.5 h), compared with controls (no treatment, isoflurane, and placebo). Respiratory distress and pulse oximetry were assessed (for 12 or 48 h), and arterial blood gases were obtained at study termination (12 h). Microdissection of fixed lungs was done to assess airway obstruction by casts. Optimal intratracheal tPA treatment (0.7 mg/kg) completely eliminated mortality (0% at 48 h), and greatly improved morbidity in this nearly uniformly fatal disease model (90-100% mortality at 48 h). tPA normalized plastic bronchitis-associated hypoxemia, hypercarbia, and lactic acidosis, and improved respiratory distress (i.e., clinical scores) while decreasing airway fibrin casts. Intratracheal tPA diminished airwayobstructive fibrin-containing casts while improving clinical respiratory distress, pulmonary gas exchange, tissue oxygenation, and oxygen utilization in our model of severe chemically induced plastic bronchitis. Most importantly, mortality, which was associated with hypoxemia and clinical respiratory distress, was eliminated.Keywords: plastic bronchitis; tissue plasminogen activator; airway obstruction; sulfur mustard; fibrin Sulfur mustard (bis(2-chloroethyl)sulfide; SM) is a vesicant and chemical weapon used in warfare during much of the twentieth century, and which remains in the stockpiles of multiple nations today (Syria, Iran, North Korea, Libya, the United States, and possibly others). SM exposure affects the eyes, skin, upper airways, and lungs. After a brief latent period, respiratory failure and death can develop within 12 to 48 hours. Despite a century of study, the mechanisms responsible for SM's toxic effects remain unsolved, and clinically effective rescue therapies or antidotes are not available.Initial reports of human SM inhalation toxicity in the early 1900s described the presence of airway-obstructive necrotic debris/ mucosa, or "pseudomembranes," in the large airways of victims, and these were more recently confirmed in the victims of the IranIraq war (1, 2). Such severe lesions have been reported to lead to respiratory compromise, with need for artificial ventilation, and death in 80% of those needing intubation (2). Furthermore, chronic conducting airway lesions, such as bronchiolitis obliterans, tracheal/bronchial stenosis, and chronic bronchitis, are commonly found in survivors of SM inhalation months to years after exposure (2, 3), whereas chronic alveolar or paren...

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“…19,20) Vasodilators such as nitric oxide, prostaglandin I 2 , endothelin receptor antagonists, and PDE-5 inhibitors have been used to treat pulmonary vascular obstructive disease, and were reported to be effective in a number of studies. [21][22][23][24][25] However, Ovaert, et al 26) reported that bosentan therapy was not effective in patients with a failing Fontan circulation and reduced left ventricular compliance, and suggested that investigation of the efficacy of vasodilator therapy in Fontan patients should be focused on those with high PAP or PVR due to pathology of the pulmonary vessels. Yamagishi,et al 27) reported that central venous pressure was correlated with the serum endothelin level, and that high serum endothelin levels were observed during the early period after the Fontan procedure.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of Pulmonary Vasodilator Therapy in Patients With Functionally Single Ventricle

Park

2015

Int. Heart J.

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SummaryThis study retrospectively evaluated the effectiveness of pulmonary vasodilator therapy with bosentan (n = 14) and/ or sildenafil (n = 23) in 34 patients with a functionally single ventricle. Vasodilator therapy was initiated before the Fontan procedure in 18 patients and after the procedure in 16 patients. The reasons for vasodilator treatment included high pulmonary artery pressure or pulmonary vascular resistance (n = 8), high central venous pressure after the Fontan or bidirectional Glenn procedure (n = 7), and ventilatory impairment (n = 8). In the 11 patients who underwent right heart catheterization before and after the initiation of therapy, the mean pulmonary artery pressure decreased significantly from 19.5 ± 5.5 mmHg to 14.3 ± 3.0 mmHg (P = 0.023) and the transpulmonary pressure gradient decreased significantly from 10.9 ± 4.6 mmHg to 7.2 ± 3.3 mmHg (P = 0.046). Of the 18 patients who started vasodilator therapy before the Fontan procedure, 10 survived surgery, 4 are awaiting surgery, 3 had not been evaluated for the Fontan procedure at the end of the study period, and 1 died of heart failure after discontinuing bosentan therapy. There were no deaths among the patients who started therapy after the Fontan procedure. Two of the 14 patients receiving bosentan discontinued treatment because of adverse effects (hepatic dysfunction and increased serum brain natriuretic peptide level). Bosentan or sildenafil therapy is usually safe and may contribute to reducing pulmonary vascular resistance in patients with a functionally single ventricle before and after a Fontan type operation. (Int Heart J 2015; 56: S26-S30)

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Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome

Cited by 73 publications

References 5 publications

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

Tissue Plasminogen Activator Prevents Mortality from Sulfur Mustard Analog–Induced Airway Obstruction

Efficacy of Pulmonary Vasodilator Therapy in Patients With Functionally Single Ventricle

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