Stephen Binsse, Jacques Sellier, Jérome Lucas, Mostafa El Hajjam, Pascal Lacombe

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1 SPONTANEOUS RUPTURE OF PULMONARY ARTERIOVENOUS MALFORMATIONS IN HEREDITARY HAEMORRHAGIC TELANGIECTASIA (HHT) REVIEW OF 12 CASES Stephen Binsse, Jacques Sellier, Jérome Lucas, Mostafa El Hajjam, Pascal Lacombe HHT Center of Paris, Hôpital Ambroise Paré, Université Versailles St Quentin en Yvelines

2 Stephen Binsse No relevant financial relationship reported

3 PULMONARY ARTERIOVENOUS MALFORMATIONS Most complications in HHT are related to PAVM Dyspnea, cyanosis, clubbing in diffuse or large PAVMs Paradoxical embolism: TIA, stroke, brain abscess Life-threatening hemorrhage due to rupture of PAVM Faughnan, and al. Eur Respir J 2009; 33: 11894

4 PURPOSE To describe clinical presentation, imaging findings and therapeutics options in patients with spontaneously ruptured PAVMs

5 MATERIAL AND METHODS All consecutive patients with rupture of PAVMs were recorded from our database and reviewed retrospectively 12-year inclusion time Age, clinical presentation Imaging (Chest X-ray, MDCT) findings Embolization and results

6 RESULTS 970 patients evaluated at our HHT center, 425 patients with PAVMs. 12 patients identified with spontaneous rupture of PAVM - 9 adults and 3 children - 6 women per 6 men - Mean age : 41 years (range 10-82) - 10 of them already diagnosed with HHT - Diagnosis of HHT (mutation) confirmed in 8/12

7 CLINICAL PRESENTATION 6 patients with hemoptysis, associated with neurological symptoms in 3 patients - TIA in 2 cases - Loss of consciousness in 1 case 6 patients with acute chest pain

8 IMAGING FINDINGS Hemothorax in 6 patients and intra-parenchymal ruptures in 6 patients. Average diameter of feeding artery: 6.7 mm (min 2- max 12). Recanalisation of previously embolized PAVMs in 3 patients. Signs of pulmonary arterial hypertension (PAH) in 4 patients.

9 THERAPEUTIC MANAGEMENT AND FOLLOW-UP 11 patients successfully treated by prompt embolization. One patient died of massive hemothorax.

10 57 y.o. w., acute chest pain during flight Clot Intra-pleural herniation

11 Acute chest pain Intra-pleural herniation Intra-pleural herniation

12 Hemoptysis and TIA 37 y.o. man, with diagnosed HHT, admitted because of transient hemiplegia and hemoptysis. CT-scan show intra-parenchymal bleeding by rupture of a PAVM located within the right anterior basal segment.

13 Embolization of the feeding artery (A8) using fibered steel coils

15 Central recanalization Side-branch

16 Discussion Second description of the occurrence of thoracic hemorrhage in a large series of patients with PAVM and HHT Database of 246 patients embolized for PAVMs 11 (8%) with massive hemoptysis or hemothorax 5 hemoptysis (3 men/2 women, age 15-59) - 2 deaths, 2 lobectomies, 1 emergent C-section 5 hemothorax (1 man/4 women, age 19-62) - Drainage 1 patient with both Total 7 women including 3 (43%) ruptures during pregnancy Ference et al. Chest 1994;106:

17 Discussion: clinical presentation Acute chest pain often associated with respiratory failure: pleural rupture. Hemoptysis and neurological signs: intraparenchymal rupture

18 Discussion: risk factors Outcome analysis and determination of risk factors not possible (retrospective study, low number of cases) Diameter of feeding artery higher than 3mm Pregnancy Pulmonary hypertension

19 Known growth factors of PAVM Pregnancy Just before pregnancy After delivery

20 Known growth factors of PAVM PAH before Two years later

21 Pregnancy Known as risk factor in third trimester Increase of blood volume and cardiac output, inducing an higher flow through PAVM. Laroche and al. Chest 1992;101: Shovlin and al. BJOG 2008; 115 (9):

22 PAH 4 patients in our study, all with hemothorax, 1 died before embolization. Embolization of PAVM is not usually recommended because risk of worsening PAH. Significant risk of rupture and life threatening hemorrhage. PAVMs embolization might be considered in patients with stable PAH.

23 Diameter of feeding artery > 3mm All ruptured PAVM but one in our study (average 6,7mm). International Guidelines: embolization recommended in PAVM feeded by artery which are 2.5 to 3mm or larger in diameter.

24 CHILDHOOD 3 cases in our study (15, 15 and 10 years). 1 case in the Ference s study (died suddenly). No study already described the influence of puberty on PAVMs growth.

25 Spontaneous rupture without risk factor 32 y.o. woman with hemoptysis: no pregnancy, no PAH, no diving, feeding artery diameter was only 2mm. Treated by embolization.

26 CONCLUSION Rupture of PAVM should be suspected in case of chest pain or hemoptysis typically associated with neurological manifestations due to gas emboli or clot emboli during cough (other embolic situations like coronary ischemia can be encountered). Rare event: 4% in our experience Emergent embolization is effective Rupture should be prevented by prophylactic embolization of PAVMs larger than 2.5mm.

27 CONCLUSION Pregnancy and PAH increase the risk of rupture by increasing the size of PAVMs Screening of PAVM and embolization in pregnancyseeking women is required. Low-dose MDCT control 6 months after delivery. PAVMs embolization might be considered in patients with PAH, using test by transient balloon occlusion prior to embolization in case of severe PAH. Spontaneous rupture of PAVM can occur at any age, without any of these risk factors.

28 REFERENCES Ference B, Shannon T, White R, Zawin M, Burdge C. Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary malformations and hereditary hemorrhagic telangiectasia. Chest 1994; 106: Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff U W, McDonald J, Proctor DD, Spears J, Brown DH, Buscarini E, Chesnutt MS, Cottin V, Ganguly A, Gossage JR, Guttmacher AE, Hyland RH, Kennedy SJ, Korzenik J, Mager JJ, Ozanne AP, Piccirillo J F, Picus D, Plauchu H, Porteous MEM, Pyeritz RE, Ross DA, Sabba C, Swanson K, Terry P, Wallace MC, Westermann CJJ, White RI, Young LH, Zarrabeitia R. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 2011; 48: Shovlin CL, Winstock AR, Peters AM, et al: Medical complications of pregnancy in hereditary haemorrhagic telangiectasia. Q J Med 88: , Simonneau G, Galie N, Rubin L, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43: Suppl.12, 5S-12S. LetteboerTG, Mager JJ, Snijder RJ, et al. Genotype-phenotype relationship in hereditary haemorrhagic telangiectasia. J med genet 2006; 43: Kurzyna M, Babrowski M, Bielecki D, et al. Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension. Chest 2007; 131: Montani D, Price LC, Girerd B, Chinet T, Lacombe P, Simonneau G, Humebert M. Fatal rupture of pulmonary arterioveinousmalformation in hereditary haemorrhagic telangiectasis and severe PAH. Eur RespirRev2009; 18: 11, Shovlin CL, Tighe HC, Davies RJ, Gibbs JS, Jackson JE. Embolisation of pulmonary arterioveinous malformations: no consistent effect on pulmonary artery pressure. Eur respire J 2008; 32: Shovlin CL, Winstock AR, Peters AM, Jackson JE, Hughes JM. Medical complications of pregnancy in hereditary haemorrhagic telangiectasia. QJmed 1995 Dec; 88: Shovlin CL, Jackson JE, Bamford KB, et al. Primary determinants of ischaemic stroke/brain abscess risks are independent of severity of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Thorax 2008; 63:

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

Eur Respir Rev 2009; 18: 111, 42 46 DOI: 10.1183/09059180.00011113 CopyrightßERSJ Ltd 2009 CASE REPORT Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and