Atrial Septostomy in Treatment of End-Stage Right Heart Failure in Patients With Pulmonary Hypertension

Kurzyna, Marcin; Dąbrowski, Marek; Bielecki, Dariusz; Fijałkowska, Anna; Pruszczyk, Piotr; Opolski, Grzegorz; Burakowski, Janusz; Florczyk, Michał; Tomkowski, Witold; Wawrzyńśka, Liliana; Szturmowicz, Monika; Torbicki, Adam

doi:10.1378/chest.06-1227

Cited by 150 publications

(147 citation statements)

References 15 publications

Supporting

Mentioning

118

Contrasting

Unclassified

Order By: Relevance

“…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

European Respiratory Review

View full text Add to dashboard Cite

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...

show abstract

Section: Discussionmentioning

confidence: 99%

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

Montani

Price²,

Girerd³

et al. 2009

European Respiratory Review

View full text Add to dashboard Cite

show abstract

“…The creation of an inter-atrial right-to-left shunt can decompress the right heart chambers, and increase LV preload and CO [170,171]. Evidence shows improvements in CI, decreases in right atrial pressure with improvement in 6-MWT [170,171].…”

Section: Balloon Atrial Septostomymentioning

confidence: 99%

Guidelines for the diagnosis and treatment of pulmonary hypertension

Galiè¹,

Hoeper²,

Humbert³

et al. 2012

Revista Portuguesa de Cardiologia (English Edition)

Self Cite

419

738

View full text Add to dashboard Cite

“…Right-to-left shunting in patients with PAH, in fact, acts as a safety valve and forms the basis of practicing atrial septostomy in patients with PAH. [31][32][33][34][35] Nevertheless, despite the established longstanding pulmonary vascular disease with evidence of substantial vascular remodeling/obstruction, Eisenmenger patients often respond favorably to advanced therapy. 16 It has been demonstrated that almost one third of Eisenmenger patients maintain some degree of pulmonary vasoreactivity despite the presence of obstructive pulmonary hypertension.…”

Section: Corrective Interventionsmentioning

confidence: 99%

Eisenmenger Syndrome: Not Always Inoperable

Liang

Zhou

2012

Respir Care

View full text Add to dashboard Cite

Recently, advanced therapies for pulmonary arterial hypertension have become available, and have been effective in reducing pulmonary vascular resistance and symptoms in patients with Eisenmenger syndrome, previously thought to be inoperable. This review summarizes the current knowledge on the pathophysiology and treatment of Eisenmenger syndrome. The recent introduction of targeted therapies in pulmonary arterial hypertension has led to a renewed insight in the pathophysiology and treatment of Eisenmenger syndrome. Patients with Eisenmenger syndrome using a diagnostic-treatment-and-repair strategy are amenable to surgery after successful treatment with advanced therapy. With continued improvements in the diagnosis, preoperative management, refinement of surgical techniques and intra-and postoperative management strategies, the patients with Eisenmenger syndrome selected using a diagnostic-treatment-and-repair strategy are operable with safety and efficacy in the current era with advanced pulmonary arterial hypertension therapy. Future directions of Eisenmenger syndrome may be the combination of reversal of pulmonary vascular remodeling and correction.

show abstract

Atrial Septostomy in Treatment of End-Stage Right Heart Failure in Patients With Pulmonary Hypertension

Cited by 150 publications

References 15 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

Guidelines for the diagnosis and treatment of pulmonary hypertension

Eisenmenger Syndrome: Not Always Inoperable

Contact Info

Product

Resources

About