Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital heart disease

Berner, Michel; Beghetti, Maurice; Spahr-Schopfer, Isabelle; Oberhänsli, I; Friedli, Béat

doi:10.1016/s0002-9149(97)89353-8

Cited by 96 publications

(56 citation statements)

References 17 publications

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“…Furthermore, subclinical elevation of PVRI may be associated with prolonged postoperative recovery. 14 Treatment with inhaled NO has become standard of care in most units, but there are nonresponders, 3 and rebound PHT on withdrawal can be difficult to manage. 15 Alternative strategies to lower PVR, may therefore have a therapeutic role.…”

Section: Clinical Impact Of Increased Pvr In Children With Congenitalmentioning

confidence: 99%

“…Some patients fail to respond to nitric oxide inhalation. 3 Alternatively, responders to longterm NO therapy may develop severe, life-threatening, rebound pulmonary hypertension on withdrawal of nitric oxide. 4 We have recently shown 5 that by enhancement of the NO-arginine pathway, ie, by providing the substrate for NO-production (L-arginine) and stimulating the enzyme responsible for NO-formation (substance P), that there is almost no PED in preoperative patients, and that it is possible to restore the pulmonary endothelial vasodilating function to a large degree in most postoperative patients.…”

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confidence: 99%

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Intravenous Sildenafil Is a Potent Pulmonary Vasodilator in Children With Congenital Heart Disease

et al. 2003

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Background-Increased pulmonary vascular resistance (PVR) because of congenital heart disease (CHD) may be caused by a dysfunction in endogenous pulmonary endothelial nitric oxide (NO) production. In other forms of pulmonary vascular disease with increased PVR, an elevated activity of a phosphodiesterase type 5 (PDE-5), responsible for the degradation of cyclic guanidine monophosphate (cGMP), the second messenger of endothelially produced NO, has been demonstrated. This study compares the effects of inhaled NO before and after the specific inhibition of the PDE-5 by intravenous sildenafil (Viagra™) in pre-and postoperative children with increased PVR because of CHD. Methods and Results-12 children with congenital heart disease (age 0.2 to 15.7 years, median 2.4 years) and increased mean pulmonary arterial pressure, and 12 postoperative children (age 0.11 to 0.65 years, median 0.32 years) with increased PVR (8.3Ϯ1.0 Wood Units*m 2 ) were studied during cardiac catheterization ("cath laboratory"), or within 2 hours after return from cardiac surgery ("post op"), respectively. All were sedated, tracheally intubated and paralyzed. During alveolar hyperoxygenation (F i O 2 ϭ0.65), the effects of inhaled NO (20 ppm) were compared before and after the stepwise infusion of sildenafil ("cath laboratory", 1 mg/kg; post op, 0.25 mg/kg). Intravenous sildenafil more effectively reduced PVR than NO (11.5% versus 4.3% in the "cath laboratory" patient group, PϽ0.05, and 25.8% versus 14.6% in the post op patient group, Pϭ0.09. The increase in cGMP in response to NO was potentiated (2-to 2.4-fold) by PDE-5 inhibition. While the vasodilating effects of sildenafil showed pulmonary selectivity, its infusion was associated with increased intrapulmonary shunting in the postoperative patients (Q s / Q t ϭ16.5Ϯ4.7% to 25.5Ϯ18.2% Pϭ0.04). Conclusions-Intravenous sildenafil is as effective as inhaled NO as a pulmonary vasodilator in children with congenital heart disease. Although clinically insignificant in this study, increased intrapulmonary shunting with sildenafil may be disadvantageous in some patients after CHD surgery.

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Section: Clinical Impact Of Increased Pvr In Children With Congenitalmentioning

confidence: 99%

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confidence: 99%

Intravenous Sildenafil Is a Potent Pulmonary Vasodilator in Children With Congenital Heart Disease

et al. 2003

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“…Rp < 8 Wood U·m 2 has also been suggested as an operative indication for VSD surgery. 19) The ability of the pulmonary vascular bed to dilate is also very important, and can be evaluated by administration of selective pulmonary vasodilators such as oxygen or nitric oxide. 19,20) Lung biopsy can provide important information regarding pulmonary vascular changes.…”

Section: What Are the Operative Indications In Patients With Chd Withmentioning

confidence: 99%

“…19) The ability of the pulmonary vascular bed to dilate is also very important, and can be evaluated by administration of selective pulmonary vasodilators such as oxygen or nitric oxide. 19,20) Lung biopsy can provide important information regarding pulmonary vascular changes. 21) Heath-Edward grading is a well-known method of evaluating the progression of pulmonary vascular disease.…”

Section: What Are the Operative Indications In Patients With Chd Withmentioning

confidence: 99%

Can Pulmonary Vasodilator Therapy Expand the Operative Indications for Congenital Heart Disease?

Inai

2015

Int. Heart J.

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SummaryThe operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood flow. Although some recommendations exist regarding operative indications according to pathological, hemodynamic, and epidemiological factors, the evidence underlying these recommendations is not conclusive. Recently, oral pulmonary vasodilator therapy has been reported to improve outcomes in patients with idiopathic pulmonary arterial hypertension, and this therapy also appears to be effective in patients with congenital heart disease and pulmonary arterial hypertension, including those with postoperative pulmonary hypertension and Eisenmenger syndrome. It is expected that the availability of novel pulmonary vasodilator therapy will expand the operative indications in patients with congenital heart disease with left to right shunt, but there is currently insufficient evidence to definitively determine this. A multicenter double-blind study should be conducted to further examine this issue. (Int Heart J 2015; 56: S12-S16)

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“…Acute vasodilator testing, regardless of whether nitric oxide alone or in an admixture with oxygen, is the gold standard measure of the reactivity of the pulmonary vascular bed. 17,18 In idiopathic PAH, vasodilator testing is done in order to determine whether a patient will respond to therapy with calcium channel antagonists. However, when to assess the operability of a patient with a CHD and high PVR, it is an open question on whether it is accurate enough to completely discriminate between patients who will or won't have a good surgical outcome.…”

Section: Abbreviationsmentioning

confidence: 99%

Pulmonary Hypertension in Congenital Shunts

Beghetti¹,

Tissot

2010

Revista Española de Cardiología (English Edition)

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Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital heart disease

Cited by 96 publications

References 17 publications

Intravenous Sildenafil Is a Potent Pulmonary Vasodilator in Children With Congenital Heart Disease

Intravenous Sildenafil Is a Potent Pulmonary Vasodilator in Children With Congenital Heart Disease

Can Pulmonary Vasodilator Therapy Expand the Operative Indications for Congenital Heart Disease?

Pulmonary Hypertension in Congenital Shunts

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