Unusual presentation for a patent ductus arteriosus

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.

Unusual presentation of a patent ductus arteriosus: A case report

“…Dyspnea is the most common presenting symptom of PAH, regardless of etiology, but there is a wide range of possible manifestations which can complicate diagnosis. 3 In this case, the atypical clinical presentation of patent ductus arteriosus was complicated by the absence of detailed records from a young age, the late onset of symptoms and the physical exam who missed to check the oxygen saturation in both arms and legs.…”

Combining evidence and experience in pulmonary hypertension

“…In their expert review, VACHIÉ RY and COGHLAN [7] examine the value of screening for PAH in patients with SSc, outline important considerations in patient selection and the choice of screening tools, discuss gaps in our Alongside these expert reviews, three interesting and informative case reports are presented, which emphasise potential issues and important considerations in managing patients with PH. The first, presented by VIZZA et al [8], concerns a seriously ill patient with a pre-existing diagnosis of portopulmonary hypertension who was subsequently found to have severe PH associated with a previously unrecognised congenital heart defect. This case highlights the need to fully investigate alternative causes of symptoms to avoid misdiagnosis, and its subsequent life-threatening risks, in adult patients with an atypical presentation.…”