e-Herz: Case study Herz 2013 DOI 10.1007/s00059-013-4019-4 Received: 18 October 2013 Revised: 29 October 2013 Accepted: 30 October 2013 © Urban & Vogel 2013
e-Herz
U. Canpolat · Ö Özeke · S. Yılmaz · F.N. Başar · K. Çağlı · Z. Gölbaşı Cardiology Clinic, Türkiye Yüksek Ihtisas Training and Research Hospital, Sıhhiye, Ankara
Diagnostic dilemma in pulmonary arterial hypertension Idiopathic or chronic thromboembolic hypertension?
Pulmonary hypertension is one of the most complicated and potentially devastating chronic diseases of the pulmonary vasculature. The heterogeneity of etiologies and pathophysiological mechanisms poses diagnostic and therapeutic challenges [1]. Involvement of both small and large vessels in idiopathic (primary) pulmonary arterial hypertension (IPAH) and in chronic thromboembolic pulmonary hypertension (CTEPH), together with a high incidence of silent thromboembolic events, can lead to difficulties in identifying the true cause of pulmonary hypertension. The demonstration and the diagnosis of the exact cause determine the management and prognosis. Herein, we present the case of a patient with pulmonary hypertension and pulmonary arterial thrombi that led to a diagnostic challenge.
the left sternal border without any other pathological finding. Electrocardiography showed sinus rhythm (88 bpm) and right bundle branch block. Chest X-ray demonstrated an increased cardiothoracic index and prominent pulmonary conus (. Fig. 1a). The results of a hemogram, biochemistry panel, D-dimer and thyroid function tests as well as a viral panel including anti-HIV were all within normal reference limits. Transthoracic echocardiography showed normal left ventricular systolic functions, an estimated systolic pulmonary arterial pressure of 100 mmHg, mild tricuspid regurgitation, and an extremely dilated main pulmonary artery (10 cm; . Fig. 1b). Low-
er-extremity Doppler examination revealed no thrombus in the venous system. A thoracic computerized tomography (CT) scan revealed massive dilation of the pulmonary arteries (main pulmonary artery of 10 cm; . Fig. 2a) with a nonocclusive chronic mural thrombus in the right pulmonary artery (. Fig. 2b), left lobar artery, and distal branches. However, pulmonary CT angiography and ventilation perfusion scans were not suggestive of chronic thromboembolic disease. Furthermore, right heart catheterization revealed pulmonary artery systolic, diastolic, and mean pressures of 95 mmHg, 54 mmHg, and 68 mmHg, respectively. The right atrial pressure was
Case report A 45-year-old female patient was admitted to our cardiology clinic with the complaints of progressive fatigue and dyspnea on exertion for 3 months. Her functional capacity was New York Heart Association (NYHA) class III. Her past medical history, including medication and smoking assessment, was unremarkable. Physical examination revealed a loud pulmonic component of the second heart sound, a 2/6* systolic murmur at
Fig. 1 8 Chest X-ray showing prominent pulmonary conus (a, arrows); transthoracic echocardiography in parasternal short-axis view demonstrating the huge main pulmonary arterial aneurysm (10 cm; b) Herz 2013
| 1
e-Herz: Case study and is likely due to endothelial dysfunction, hypercoagulable state, and altered blood flow [5]. In two retrospective cohort studies of IPAH patients evaluating the histology findings, the prevalence rates of isolated thrombotic arteriopathy were 56 and 57%, respectively [6, 7]. Although there might be a diagnostic dilemma in such a condition, appropriate diagnostic tests with accurate interpretation can guide us in making the definite diagnosis.
Conclusion In conclusion, it is essential to recognize and differentiate IPAH with in situ pulmonary arterial mural thrombosis from CTEPH, which requires different management strategies.
Corresponding address Fig. 2 9 Thoracic computerized tomography showing dilated pulmonary artery (10 cm; a) and mural thrombus of the right pulmonary artery (b)
5 mmHg and the pulmonary capillary wedge pressure was 10 mmHg. Vasoreactivity test results were negative with adenosine administration. Further detailed investigations failed to reveal an associated condition that could have resulted in PAH. Therefore, the patient was diagnosed as having IPAH. Oral anticoagulation with warfarin and bosentan therapy were initiated and her symptoms dramatically improved in the 4-month follow-up period.
Discussion PAH is characterized by an increase in mean pulmonary arterial pressure (≥25 mmHg) at rest as assessed by right heart catheterization [2, 3]. The diagnostic algorithm of a patient with suspected pulmonary hypertension requires a set of tests aimed to confirm the diagnosis, identify the clinical group of pulmonary hypertension, and the specific eti-
2 |
Herz 2013
ology within the PAH group. As IPAH is a diagnosis of exclusion, other PAH-associated conditions should be excluded in all patients. Particularly, the differentiation of IPAH from CTEPH is important in determining the appropriate therapeutic strategy. However, it could be difficult to define the etiology precisely in the case of pulmonary arterial thrombi. In our patient, differentiation of the two conditions was difficult because of the presence of extensive mural thrombi in the central and distal pulmonary arteries. However, in situ mural thrombosis due to an extremely dilated pulmonary artery of IPAH was considered because of the absence of findings supporting CTEPH (lung infarcts, systemic venous thrombi, peripheral parenchymal densities and bands, mosaic perfusion, webs, variation in segmental vessel sizes, abrupt cut-offs, and dilated bronchial arteries) [4]. In situ mural thrombosis of the pulmonary arteries is sometimes developed in IPAH
U. Canpolat Cardiology Clinic, Türkiye Yüksek Ihtisas Training and Research Hospital 06100 Sıhhiye, Ankara Turkey [email protected]
Compliance with ethical guidelines Conflict of interest. U. Canpolat, Ö. Özeke, S. Yılmaz, F.N. Başar, K. Çağlı, and Z. Gölbaşı state that there are no conflicts of interest. Consent was obtained from all patients identifiable from images or other information within the manuscript. In the case of underage patients, consent was obtained from a parent or legal guardian.
References 1. Simonneau G, Robbins I, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54(Suppl 1):S43– S54 2. Johnson SR, Granton JT, Mehta S (2006) Thrombotic arteriopathy and anticoagulation in pulmonary hypertension. Chest 130:545–552 3. Galie N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537
e-Herz
U. Canpolat · Ö Özeke · S. Yılmaz · F.N. Başar · K. Çağlı · Z. Gölbaşı Cardiology Clinic, Türkiye Yüksek Ihtisas Training and Research Hospital, Sıhhiye, Ankara
Diagnostic dilemma in pulmonary arterial hypertension Idiopathic or chronic thromboembolic hypertension?
Pulmonary hypertension is one of the most complicated and potentially devastating chronic diseases of the pulmonary vasculature. The heterogeneity of etiologies and pathophysiological mechanisms poses diagnostic and therapeutic challenges [1]. Involvement of both small and large vessels in idiopathic (primary) pulmonary arterial hypertension (IPAH) and in chronic thromboembolic pulmonary hypertension (CTEPH), together with a high incidence of silent thromboembolic events, can lead to difficulties in identifying the true cause of pulmonary hypertension. The demonstration and the diagnosis of the exact cause determine the management and prognosis. Herein, we present the case of a patient with pulmonary hypertension and pulmonary arterial thrombi that led to a diagnostic challenge.
the left sternal border without any other pathological finding. Electrocardiography showed sinus rhythm (88 bpm) and right bundle branch block. Chest X-ray demonstrated an increased cardiothoracic index and prominent pulmonary conus (. Fig. 1a). The results of a hemogram, biochemistry panel, D-dimer and thyroid function tests as well as a viral panel including anti-HIV were all within normal reference limits. Transthoracic echocardiography showed normal left ventricular systolic functions, an estimated systolic pulmonary arterial pressure of 100 mmHg, mild tricuspid regurgitation, and an extremely dilated main pulmonary artery (10 cm; . Fig. 1b). Low-
er-extremity Doppler examination revealed no thrombus in the venous system. A thoracic computerized tomography (CT) scan revealed massive dilation of the pulmonary arteries (main pulmonary artery of 10 cm; . Fig. 2a) with a nonocclusive chronic mural thrombus in the right pulmonary artery (. Fig. 2b), left lobar artery, and distal branches. However, pulmonary CT angiography and ventilation perfusion scans were not suggestive of chronic thromboembolic disease. Furthermore, right heart catheterization revealed pulmonary artery systolic, diastolic, and mean pressures of 95 mmHg, 54 mmHg, and 68 mmHg, respectively. The right atrial pressure was
Case report A 45-year-old female patient was admitted to our cardiology clinic with the complaints of progressive fatigue and dyspnea on exertion for 3 months. Her functional capacity was New York Heart Association (NYHA) class III. Her past medical history, including medication and smoking assessment, was unremarkable. Physical examination revealed a loud pulmonic component of the second heart sound, a 2/6* systolic murmur at
Fig. 1 8 Chest X-ray showing prominent pulmonary conus (a, arrows); transthoracic echocardiography in parasternal short-axis view demonstrating the huge main pulmonary arterial aneurysm (10 cm; b) Herz 2013
| 1
e-Herz: Case study and is likely due to endothelial dysfunction, hypercoagulable state, and altered blood flow [5]. In two retrospective cohort studies of IPAH patients evaluating the histology findings, the prevalence rates of isolated thrombotic arteriopathy were 56 and 57%, respectively [6, 7]. Although there might be a diagnostic dilemma in such a condition, appropriate diagnostic tests with accurate interpretation can guide us in making the definite diagnosis.
Conclusion In conclusion, it is essential to recognize and differentiate IPAH with in situ pulmonary arterial mural thrombosis from CTEPH, which requires different management strategies.
Corresponding address Fig. 2 9 Thoracic computerized tomography showing dilated pulmonary artery (10 cm; a) and mural thrombus of the right pulmonary artery (b)
5 mmHg and the pulmonary capillary wedge pressure was 10 mmHg. Vasoreactivity test results were negative with adenosine administration. Further detailed investigations failed to reveal an associated condition that could have resulted in PAH. Therefore, the patient was diagnosed as having IPAH. Oral anticoagulation with warfarin and bosentan therapy were initiated and her symptoms dramatically improved in the 4-month follow-up period.
Discussion PAH is characterized by an increase in mean pulmonary arterial pressure (≥25 mmHg) at rest as assessed by right heart catheterization [2, 3]. The diagnostic algorithm of a patient with suspected pulmonary hypertension requires a set of tests aimed to confirm the diagnosis, identify the clinical group of pulmonary hypertension, and the specific eti-
2 |
Herz 2013
ology within the PAH group. As IPAH is a diagnosis of exclusion, other PAH-associated conditions should be excluded in all patients. Particularly, the differentiation of IPAH from CTEPH is important in determining the appropriate therapeutic strategy. However, it could be difficult to define the etiology precisely in the case of pulmonary arterial thrombi. In our patient, differentiation of the two conditions was difficult because of the presence of extensive mural thrombi in the central and distal pulmonary arteries. However, in situ mural thrombosis due to an extremely dilated pulmonary artery of IPAH was considered because of the absence of findings supporting CTEPH (lung infarcts, systemic venous thrombi, peripheral parenchymal densities and bands, mosaic perfusion, webs, variation in segmental vessel sizes, abrupt cut-offs, and dilated bronchial arteries) [4]. In situ mural thrombosis of the pulmonary arteries is sometimes developed in IPAH
U. Canpolat Cardiology Clinic, Türkiye Yüksek Ihtisas Training and Research Hospital 06100 Sıhhiye, Ankara Turkey [email protected]
Compliance with ethical guidelines Conflict of interest. U. Canpolat, Ö. Özeke, S. Yılmaz, F.N. Başar, K. Çağlı, and Z. Gölbaşı state that there are no conflicts of interest. Consent was obtained from all patients identifiable from images or other information within the manuscript. In the case of underage patients, consent was obtained from a parent or legal guardian.
References 1. Simonneau G, Robbins I, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54(Suppl 1):S43– S54 2. Johnson SR, Granton JT, Mehta S (2006) Thrombotic arteriopathy and anticoagulation in pulmonary hypertension. Chest 130:545–552 3. Galie N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537
