Chronic thromboembolic pulmonary hypertension as a cause of dyspnoea in an older patient with a complex history

Abstract

A full diagnostic work-up for patients with pulmonary hypertension (PH) is vital. Classification and diagnosis of the underlying cause is important to ensure optimal management, but may be complicated by overlapping signs and symptoms. This case study describes how a full work-up identified chronic thromboembolic PH (CTEPH) as the cause of dyspnoea in a 68-yr-old male with a history of pulmonary embolism and an original diagnosis of chronic obstructive pulmonary disease. Key indicators included decreased tricuspid annular plane systolic excursion, increased Tei index and elevated systolic pulmonary artery pressure. Multi-slice spiral chest computed tomography and pulmonary angiography showed severe chronic thromboembolic pulmonary disease, both centrally and distally. Diffusing capacity of the lung for carbon monoxide was reduced and blood gas analysis revealed a wide alveolar-arterial oxygen pressure difference, which is typical of CTEPH. The patient was eligible for pulmonary endarterectomy according to established criteria. Residual PH after surgery was successfully managed with bosentan.KEYWORDS: Chronic thromboembolic pulmonary hypertension, dyspnoea, pulmonary hypertension A 64-yr-old male first presented in 2003 at a local chest hospital with exertional dyspnoea, following a pulmonary embolism in 2002 and a history of smoking. At that time, a computed tomography (CT) scan revealed pulmonary embolism mainly in the left lower lobe; transthoracic echocardiography showed a slightly reduced left ventricular ejection fraction (EF) and elevated pulmonary arterial pressure (Ppa) (table 1). He was hypoxic and hyperventilating, with low oxygen saturation. The patient was diagnosed with chronic obstructive pulmonary disease with hypoxaemia, and discharged; congestive heart failure was attributed to the low EF and the prior history of pulmonary embolism. Acenocoumarol, ramipril, carvedilol, oxygen and salbutamol were started. In 2007, he presented again at another local hospital with worsening dyspnoea; investigations showed increased Ppa, with similar EF and lung function to those at the first assessment (table 1).The patient was subsequently reinvestigated by a specialist pneumology unit. Transthoracic echocardiography revealed decreased tricuspid annular plane systolic excursion (TAPSE; 1.4 cm) with increased Tei index (1.61) and increased right atrial and ventricular dimensions, with estimated systolic Ppa of 70 mmHg ( fig. 1). At this stage, forced vital capacity was 90% predicted, forced expiratory volume in 1 s was 76% pred (a ratio of 69%) and peak expiratory flow was 70% pred. Lung function tests and CT excluded emphysema, with normal total lung capacity and residual volume, and no hyperinflation. The patient's 6-min walk distance (6MWD) was 384 m. However, the diffusing capacity of the lung for carbon monoxide was reduced (59%), as is the case in ,20% of patients with idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension (CTEPH) [1,2]. Multi-slice spiral chest...

Pulmonary thromboendarterectomy after treatment with treprostenil in a chronic thromboembolic pulmonary hypertension patient: a case report

“…Patients with CTEPH have symptoms of progressive dyspnea, fatigue, and presyncope or loss of consciousness similar to the symptoms of patients with other forms of pulmonary hypertension 25. Many are unaware of prior venous thromboembolic events, and diagnosis is appreciably delayed while other causes of dyspnea are pursued and possibly treated 8.…”

Combining evidence and experience in pulmonary hypertension

“…A case reported by KÄ HLER [9] concerns an older patient with a complex medical history, including a diagnosis of chronic obstructive pulmonary disease and pulmonary embolism (PE). This patient's subsequent re-diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is in keeping with evidence to suggest that, although generally considered to be rare, the association between CTEPH and PE may be more common than previously thought [10].…”