Source: Eur Respir Rev 2009; 18: 174-176
Disease area: Pulmonary vascular diseases

Abstract

A 63-yr-old black female, with a 1-yr history of hepatitis C and ascites was referred to an expert centre with suspicion of portopulmonary hypertension (PPHTN). Her poor condition made a rapid diagnosis imperative and precluded a normal diagnostic work-up. Echocardiography confirmed severe pulmonary hypertension (PH). A hepatic scintigraphy and an abdominal echo-Doppler study excluded liver cirrhosis and portal hypertension. Cardiac magnetic resonance imaging showed marked dilation of the right ventricle with significant hypertrophy of the free wall, a finding that is uncommon in idiopathic pulmonary arterial hypertension or PPHTN. Right heart catheterisation demonstrated severe pre-capillary PH without response to acute vasodilator testing. Finally the patient underwent computed tomography angiography, which showed marked dilation of the pulmonary artery without thromboembolic disease and, unexpectedly, a partially calcified large patent ductus arteriosus. The correct diagnosis of the underlying cause of pulmonary arterial hypertension is essential. Patients with underlying heart defects may have an atypical presentation and be referred to expert centres with an incorrect diagnosis. A full investigation is necessary; careful examination of right ventricular anatomy can provide clues about the aetiology of PH, and it is important to exclude intra- and extracardiac shunts during haemodynamic studies.

Rating:

Share or cite this content

Citations should be made in the following way:
Vizza C. D., Badagliacca R., Poscia R., Gambardella C., Pezzuto B., Crescenzi E., Papa S., Fedele F.. Unusual presentation for a patent ductus arteriosus. Eur Respir Rev 2009; 18: 174-176

Member's Comments

No comment yet.

Related content which might interest you: Therapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension Source: Eur Respir Rev 2010 19: 300-307 Year: 2010 Managing pulmonary hypertension in patients with congenital heart disease Source: Eur Respir Mon 2012; 57: 71-81 Year: 2012 Eisenmenger syndrome in an adult patient with a large patent ductus arteriosus Source: Eur Respir Rev 2013; 22: 558-564 Year: 2013 Changing demographics of pulmonary arterial hypertension in congenital heart disease Source: Eur Respir Rev 2010 19: 308-313 Year: 2010 Pulmonary hypertension due to ligation of pulmonary arteriovenous malformations Source: Annual Congress 2008 - Rare clinical cases Year: 2008 Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease Source: Eur Respir Rev 2009; 18: 154-161 Year: 2009 The prevalence of patent foramen ovale in chronic obstructive pulmonary disease patients with increased pulmonary hypertension Source: Annual Congress 2009 - Pulmonary vascular diseases Year: 2009 Management of pulmonary arteriovenous malformations in pulmonary hypertensive patients: a pressure to embolise? Source: Eur Respir Rev 2009; 18: 4-6 Year: 2009 Pulmonary hypertension in children Source: Eur Respir Mon 2012; 57: 94-107 Year: 2012 Hemodynamic assessment of pulmonary hypertension in corrected versus non-corrected grown-up congenital heart disease Source: Annual Congress 2013 –Pulmonary circulation: clinical pulmonary hypertension II Year: 2013 Pulmonary arterial hypertension associated with congenital heart disease Source: Eur Respir Rev 2012 21: 328-337 Year: 2012 Isolated left pulmonary artery(LPA) mimicking pulmonary artery agenesis with cyanotic episodes and contralateral bronchial compression in a young infant Source: Eur Respir J 2003; 22: Suppl. 45, 322s Year: 2003 Intracardiac shunt measurement by MRI in patients with pulmonary arterial hypertension caused by a congenital septum defect Source: Eur Respir J 2005; 26: Suppl. 49, 560s Year: 2005 Congenital rubella syndrome and left pulmonary artery sling Source: Eur Respir J 2012; 39: 495-496 Year: 2012 Fatal dissection of the pulmonary artery in pulmonary arterial hypertension Source: Eur Respir Rev 2009; 18: 181-185 Year: 2009 Antenatal sildenafil-simvastatin combination attenuates pulmonary hypoplasia and hypertension in nitrofen-induced congenital diaphragmatic hernia Source: International Congress 2016 – The nature of pulmonary hypertension Year: 2016 Pulmonary arterial pressure and embolization of pulmonary arteriovenous malformations Source: Annual Congress 2008 - Pulmonary vascular medicine Year: 2008 Pulmonary vascular distensibility in pulmonary hypertension due to left heart disease Source: International Congress 2018 – Pulmonary hypertension: right ventricle function, haemodynamics and exercise Year: 2018