Atrial septostomy in the treatment of severe pulmonary arterial hypertension

Reichenberger, Frank; Pepke‐Zaba, Joanna; McNeil, Keith; Parameshwar, Jayan; Shapiro, L. M.

doi:10.1136/thorax.58.9.797

Cited by 97 publications

(77 citation statements)

References 24 publications

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“…Like other potential right-to-left shunts, such as atrial septal defects, they provide a low-resistance pathway for blood flow and, therefore, could protect the right ventricle from increased afterload [13,14]. Embolisation of PAVMs in patients with PAH can, therefore, theoretically lead to an increase in Ppa and right ventricular dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

Montani

Price²,

Girerd³

et al. 2009

Eur Respir Rev

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A 29-yr-old patient with hereditary haemorrhagic telangiectasia was referred to the present authors' centre with progressive exertional dyspnoea.Pulmonary arterial hypertension (PAH) was suspected on Doppler echocardiography and confirmed by right heart catheterisation demonstrating severe PAH. Genetic analysis found an activin receptor-like kinase-1 gene missense mutation. Chest radiography and computed tomodensitometry of the chest revealed a pulmonary arteriovenous malformation with a 5-mm diameter feeding artery in the right lower lobe. Embolisation of the arteriovenous malformation was discussed, but was considered a very high-risk procedure that could aggravate PAH and was therefore not performed. Haemodynamics were improved by dual endothelin receptor antagonist and inhaled iloprost but the patient subsequently died suddenly of a rupture of the arteriovenous malformation into the pleural cavity.Severe PAH is generally considered a contraindication to performing pulmonary arteriovenous malformation embolisation because of the risk of worsening of PAH. However, given the significant risk of rupture, paradoxical embolism and haemoptysis, and the lack of data regarding the evolution of pulmonary pressure after embolisation in PAH, pulmonary arteriovenous malformation embolisation should not be absolutely contraindicated and might be considered in patients with stable PAH.KEYWORDS: Activin receptor-like kinase-1, arteriovenous malformation, embolisation, hereditary haemorrhagic telangiectasia, pulmonary arterial hypertension, rupture A 29-yr-old patient with hereditary haemorrhagic telangiectasis (HHT) was referred to the present authors' centre (Hôpital Antoine-Béclère, Assistance Publique -Hôpitaux de Paris, Clamart, France) with suspected pulmonary arterial hypertension (PAH). HHT was diagnosed at age 10 yrs in the context of familial HHT ( fig. 1), repeated epistaxis and telangiectasia involving skin and mucous membranes. In 2001, the patient became pregnant, and although she had no obstetric complications during the pregnancy, shortly after delivery she presented with a transient right hemiplegia. In January 2003, she developed rapidly progressive exertional dyspnoea (New York Heart Association (NYHA) functional class II) with two episodes of dizziness. Her dyspnoea increased progressively and, 6 months later, she was described as NYHA functional class III. On clinical examination there was a prominent pulmonary component of S2 with a systolic murmur suggesting tricuspid regurgitation. ECG showed a complete right branch block and right axis deviation. Spirometry was normal except for a mild decrease in diffusion capacity of the lung for carbon monoxide. Arterial blood gases showed oxygen tension (PO 2 ) of 80 mmHg (11 kPa) and carbon dioxide tension 32 mmHg (4.3 kPa) in the supine position with hypoxaemia (PO 2 72 mmHg (9.6 kPa)) on standing. Chest radiograph ( fig. 2) and computed tomodensitometry ( fig. 3) of the chest revealed a pulmonary arteriovenous malformation (PAVM) with a 5-mm diameter...

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Section: Discussionmentioning

confidence: 99%

Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

Montani

Price²,

Girerd³

et al. 2009

Eur Respir Rev

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“…In most reported series [5,[7][8][9][10][11], AS has been performed only after the failure of different PAH-specific pharmacological interventions. In the present study, patients with concomitant pharmacological treatment had AS performed before pharmacological treatment was initiated.…”

Section: Discussionmentioning

confidence: 99%

“…In most of the reported series [5,[7][8][9][10][11], the procedure was performed after all available medical therapy failed, late in the course of the disease, when the risk of procedural death is increased and the impact of the intervention on long-term survival is minimised. We describe the effect of AS on the survival of a group of patients with PAH in whom the procedure was performed either as an isolated treatment intervention or prior to the use of PAH-specific pharmacological therapy.…”

mentioning

confidence: 99%

Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension

Sandoval¹,

Gaspar²,

Peña³

et al. 2011

Eur Respir J

122

100

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Atrial septostomy (AS) is a palliative treatment for right ventricular failure from severe pulmonary arterial hypertension (PAH). We sought to investigate the effect of AS, alone or combined with PAH-specific pharmacotherapy, on the survival of patients with PAH.We performed a retrospective analysis of the functional and haemodynamic changes in patients with PAH following AS, and long-term survival characteristics for the whole group and separately for the subgroup who received post-procedural pharmacotherapy. 50 procedures performed in 34 patients (mean¡SD age 35¡10 yrs) resulted in haemodynamic and symptomatic improvement in most of the patients. Only one (2%) procedure-related death occurred. Due to spontaneous closure of the defect, AS was repeated in 10 patients. In 21 patients, AS was the only form of treatment, while 11 received additional pharmacotherapy after AS. During follow-up (58.5¡38 months), 21 patients died; median survival of the group was 60 months (95% CI 43-77 months). Median survival for patients on pharmacotherapy additional to AS was 83 months (95% CI 57-109 months), which was better than that for patients with AS alone (53 months, 95% CI 39-67 months) (log-rank 6.52; p50.010).In selected patients with PAH, AS is a safe and effective intervention that exerts a beneficial impact on long-term survival. Survival appears to be improved when AS is combined with PAHspecific pharmacotherapy.

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“…They could be stabilized on combination treatment with bosentan (n=3), sildenafil (n=2) and iv PGI 2 in one patient, respectively. Three patients in the iv PGI 2 group and one patient on inh ilo underwent successful atrial septostomy (19).…”

Section: Long Term Follow Upmentioning

confidence: 99%

Intravenous epoprostenol versus high dose inhaled iloprost for long-term treatment of pulmonary hypertension

Reichenberger¹,

A²,

Morrell³

et al. 2011

Pulmonary Pharmacology & Therapeutics

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Background: Intravenous prostacyclin (iv PGI) and inhaled Iloprost (inh ilo) are established therapies in pulmonary arterial hypertension (PAH), however comparative data are lacking.Methods and Patients: We performed a long-term open label comparison trial of iv PGI or high dose inh ilo in 24 patients with severe PAH: 12 patients (9 female, 40 +/-14 years, 10 idiopathic PAH, 2 PAH in connective tissue disease CTD) received iv PGI, whereas 12 patients (7 female, 43 +/-12 years, 5 IPAH, 6 CTD, 1 porto-pulmonary hypertension) were commenced on inh ilo with a median dose of 120 µg / 24 hours. Hemodynamic parameters and 6 minute walking distance (6MWD) at baseline did not differ between both groups.Results: After 3 months therapy, patients on iv PGI showed a significant increase in 6 MWD from 220 to 280 meters (p<0.01), whereas patients on high dose inh ilo

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Atrial septostomy in the treatment of severe pulmonary arterial hypertension

Cited by 97 publications

References 24 publications

Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension

Intravenous epoprostenol versus high dose inhaled iloprost for long-term treatment of pulmonary hypertension

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