Treatment of Refractory Pulmonary Arterial Hypertension with Inhaled Epoprostenol in an Infant with Congenital Heart Disease

Kovach, Joshua; Ibsen, Laura M.; Womack, Michael; Steusse, David; Law, Yuk M.

doi:10.1111/j.1747-0803.2007.00097.x

Cited by 4 publications

(4 citation statements)

References 33 publications

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“…Another case report showed a synergistic effect of inhaled epoprostenol and iNO in the treatment of refractory PAH in a 3-month-old male patient with D-TGA and ventricular septal defect after pulmonary artery banding and placement of a Blalock-Taussig shunt. 55 In a prospective interventional pilot study, Carroll and colleagues 56 examined the physiologic effects of inhaled epoprostenol (15-minute course, intraoperatively and postrepair) in 6 children with PAH-CHD. They found that the agent significantly reduced mPAP and improved oxygenation.…”

Section: Prostacyclin Analogsmentioning

confidence: 99%

“…In 1995, Zwissler 54 described the successful intra‐ and postoperative use of aerosolized epoprostenol, without causing systemic hypotension or deterioration of gas exchange, in a newborn with total anomalous pulmonary venous connection and severe PAH. Another case report showed a synergistic effect of inhaled epoprostenol and iNO in the treatment of refractory PAH in a 3‐month‐old male patient with D‐TGA and ventricular septal defect after pulmonary artery banding and placement of a Blalock‐Taussig shunt 55 . In a prospective interventional pilot study, Carroll and colleagues 56 examined the physiologic effects of inhaled epoprostenol (15‐minute course, intraoperatively and postrepair) in 6 children with PAH‐CHD.…”

Section: Prostacyclin Analogsmentioning

confidence: 99%

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Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Brunner¹,

Perez²,

Richter³

et al. 2014

Pulm. circ.

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Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.

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Section: Prostacyclin Analogsmentioning

confidence: 99%

Section: Prostacyclin Analogsmentioning

confidence: 99%

Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Brunner¹,

Perez²,

Richter³

et al. 2014

Pulm. circ.

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“…Golzand et al 9 reported on the use of epoprostenol in an infant with PPHN refractory to iNO with good response, leading to discontinuation of both PH‐specific agents. Finally, Kovach et al 10 reported on the use of inhaled epoprostenol in an infant with CHD refractory to iNO that resulted in dramatic improvements in ventilation and oxygenation, leading to discontinuation of both PH‐specific agents.…”

Section: Introductionmentioning

confidence: 99%

Safety of Epoprostenol and Treprostinil in Children Less than 12 Months of Age

et al. 2013

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There is a paucity of data on infant intravenous prostacyclin use, the gold standard for therapy for severe pulmonary hypertension (PH). This review aimed to evaluate the safety, tolerability, and outcomes of infant prostacyclin use. A retrospective observational study was performed in a large pediatric hospital with a dedicated pediatric PH program. Subject medical records, bedside flow sheets, and progress notes were reviewed to identify use of intravenous epoprostenol or treprostinil within the first year of life. The indication for prostacyclin use was recalcitrant hemodynamic compromise associated with PH, identified as either idiopathic PH, persistent PH of the newborn, PH associated with congenital diaphragmatic hernia, congenital heart disease, bronchiolitis, or chronic lung disease. Prostacyclin-related adverse events included 7 episodes of hypotension, 6 episodes of perceived pain, 2 episodes of cyanosis, and 1 episode of feeding intolerance. Prostacyclin was stopped only for cyanotic episodes associated with use in severe chronic lung disease. Two hemorrhagic events occurred during extracorporeal membrane oxygenation, which were unlikely to be prostacyclin related. Outcomes included 21 deaths unrelated to prostacyclin, 1 lung transplant, 6 PH resolutions, 8 transitions to oral PH medications, and 1 continuation of treprostinil. In conclusion, efficacy could not be evaluated in this study because of the loss of equipoise for neonatal prostacyclin use. Prostacyclin use was well tolerated in neonatal diseases associated with PH, but dose titration was limited by hypotension and hypoxemia.

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“…Dextro-transposition of the great arteries and intact ventricular septum associated with persistent pulmonary hypertension is uncommon and associated with poor outcomes 1 . Several therapeutic interventions have been described to improve systemic oxygen delivery and reduce oxygen consumption including balloon atrial septostomy, prostaglandin infusion to maintain ductal patency, inotropic support, 2 inhaled nitric oxide, 3 , 4 epoprostenol, 5 bosentan 6 , and extracorporeal membrane oxygenation 4 7 , 8 We present three patients with dextro-transposition of the great arteries, intact ventricular septum, and refractory persistent pulmonary hypertension of the newborn supported with extracorporeal membrane oxygenation preoperatively, separated from bypass following their arterial switch operation and survived to discharge with favourable long-term follow-up.…”

mentioning

confidence: 99%

Successful preoperative bridge with extracorporeal membrane oxygenation in three neonates with D-transposition of the great vessels and pulmonary hypertension

2018

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Pulmonary hypertension with transposition of the great arteries is associated with significant morbidity and mortality. At the worst end of the spectrum are patients who undergo extracorporeal support perioperatively. We describe our experience with three patients who received preoperative extracorporeal support and separated from cardiopulmonary bypass successfully on conventional postoperative care, with no significant deficits on follow-up.

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Treatment of Refractory Pulmonary Arterial Hypertension with Inhaled Epoprostenol in an Infant with Congenital Heart Disease

Cited by 4 publications

References 33 publications

Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Safety of Epoprostenol and Treprostinil in Children Less than 12 Months of Age

Successful preoperative bridge with extracorporeal membrane oxygenation in three neonates with D-transposition of the great vessels and pulmonary hypertension

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