CC BY
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A.N. VENKATESH, VITTALBAGI, J. RAJIV
Department of Emergency Medicine, Apollo Hospitals, Bangalore
Pulmonary artery dissection is an extremely rare and catastrophic complication of idiopathic pulmonary arterial hypertension. Pulmonary artery dissection usually presents to Emergency Department with chest pain, dyspnea on exertion, central cyanosis and hemodynamic compromise. Many of these patients frequently diagnosed post-mortem as these patients experience sudden death. Only few isolated reports have been described in surviving patients. We report a case of pulmonary artery dissection with coexisting pulmonary embolism, discovered by performing a computed tomography (CT).
Keywords: pulmonary artery dissection, pulmonary embolism, pulmonary hypertension, sudden death.
РАССЛОЕНИЕ ЛЕГОЧНОЙ АРТЕРИИ ПРИ ЕЕ ЭМБОЛИИ: НЕТИПИЧНЫЙ СЛУЧАЙ
А.Н. ВЕНКАТЕШ, ВИТТАЛБАГИ, Ж. РАЖИВ
Отделение экстренной медицины, Apollo Hospitals, Бангалор, Индия
Расслоение легочной артерии является чрезвычайно редким и катастрофическим осложнением идиопатической легочной гипертензии. Больные с расслоением легочной артерии обычно поступают в приёмное отделение с болью в груди, одышкой при физической нагрузке, центральным цианозом и нестабильной гемодинамикой. Как правило, таким пациентам диагноз ставится посмертно, так как часто у них наступает внезапная смерть. Только единичные сообщения о выживших пациентах были описаны. В статье рассматривается случай расслоения легочной артерии с сопутствующей тромбоэмболией, обнаруженной с помощью компьютерной томографии (КТ).
Ключевые слова: расслоение легочной артерии, тромбоэмболия легочной артерии, легочная гипертензия, внезапная смерть.
PULMONARY ARTERY DISSECTION WITH PULMONARY EMBOLISM: AN UNUSUAL PRESENTATION
INTRODUCTION
Pulmonary artery dissection is an extremely rare and a devastating complication of long standing pulmonary arterial hypertension. Pulmonary artery dissection as described in literature is acute in onset and fatal without an intervention. More than 75% of patients have an underlying acute or chronic pulmonary hypertension. Pulmonary artery dissection usually presents to ED with chest pain, dyspnea on exertion, central cyanosis and hemodynamic compromise [1]. In contrast to aortic dissection, pulmonary artery dissection usually progresses rapidly, ruptures leading to cardiogenic shock or sudden death [3,4]. Many of these patients frequently diagnosed post-mortem as these patients experience sudden death. Only few isolated reports have been described in surviving patients. Only 13% of cases are diagnosed during life [1,2]. The natural course and management of pulmonary artery dissection is not yet known as scarcity of cases in the literature. In recent years, with newer advances of diagnostic and therapeutic interventions a few cases in surviving patients with pulmonary artery dissection have been described.
In this article, we report a case of pulmonary artery dissection with pulmonary embolism. We briefly discuss the etiology, clinical manifestations, and management.
CASE REPORT
A 68 years old obese female presented to Emergency Department with chest heaviness, pain radiating to upper back and sudden onset breathlessness with Bilateral lower limb edema(New York Heart Association Functional Class IV). She described similar on and off episodes, resolved without any intervention, in the past 6 months. The patient denied orthopnea or paroxysmal nocturnal dyspnea (PND). Past history revealed coronary artery disease, with decreased exercise tolerance.
A physical examination revealed, pulse rate of 108/ minute, blood pressure of 160/80, she appeared in mild respiratory distress with a respiratory rate of 28, with a normal oxygen saturation. She was afebrile, with elevated jugular venous pressure. Cardiac auscultation revealed a continuous murmur located at the upper left sternal border and a loud pulmonary component sound from the second heart sound, heard loudest over the left sternal edge. Other Systemic examination was unremarkable.
Her initial laboratory investigations, included complete blood count, renal function tests, lower limb venous doppler did not reveal significant abnormalities. ECG showed sinus rhythm, right atrial enlargement, biventricular hypertrophy, and right axis deviation. A chest radiograph revealed cardiomegaly with dilatation of the pulmonary artery and its branches. Cardiac enzymes excluded an acute coronary event.
Pulmonary artery dissection with pulmonary embolism: an unusual presentation
Fig. 1. Computed tomography of the chest with contrast showing a hypodensity within the main pulmonary artery suggestíve of dissection at bifurcation extending into right and left pulmonary arteries.
A transthoracic echocardiography was performed with a pre-diagnosis of pulmonary embolism. Echocardiographic examination revealed severe pulmonary arterial hypertension, reduced right ventricular function, dilated right atrium and right ventricle. Diagnosis was made by Computed Tomography of chest with contrast which revealed dissection at bifurcation extending into right and left pulmonary arteries and embolism of left pulmonary artery. Majority of dissection involves main pulmonary trunk only (80%). The rest of the cases isolated to right and left pulmonary arteries [5].
She was initially managed conservatively, observed in cardiac care unit and urgently referred to the cardiothoracic surgeons. After 48 hours of anticoagulation with heparin, repeat CT of chest showed no significant interval changes. As patient declined surgical repair of the dissection, patient was closely followed up with medical therapy after mechanical thrombosuction of pulmonary embolus.
DISCUSSION
Pulmonary dissection is a potentially fatal and life threatening condition and requires early diagnosis. As majority of patients present with cardiac shock or sudden death, hence the diagnosis is rarely made in living patients [5]. Dissection of the systemic arteries is a well known and often non-fatal consequence of essential hypertension; however, pulmonary artery dissection is extremely rare and is usually a lethal complication of chronic pulmonary hypertension. Most cases who present with pulmonary artery dissection are usually associated with congenital cardiac abnormalities, idiopathic PA, hypertension or have received a cardiac intervention [1]. As Khattar et al mentioned in their study that only 8 (12.6%) out of 63 cases were diagnosed to have pulmonary artery dissection while living, and that 34 (53.9%) of the cases had congenital heart disease [1]. Based on literature, first reports of this condition was found in patients with schistosomiasis came from Egypt (in patients with schistosomiasis haematobium or mansoni),
Fig. 2. Embolism of left pulmonary artery.
followed, much later, by reports from Brazil (exclusively in patients with schistosomiasis mansoni) [6].
Pathophysiology involves medial degeneration and fragmentation of elastic tissues, intimal inflammation, in-situ thrombosis and acute or chronic increase in pulmonary arterial pressures, pulmonary arterial dilatation. Medial degeneration is a common cause of weakened pulmonary arterial wall and dilatation of the vessel, with gradual increase in intravascular pressure lead to pulmonary hypertension, intimal tear may develop and cause dissection in the arterial wall [4,7,8]. As the media of pulmonary artery are much thinner compared with that of aorta, this false lumen usually ruptures leading to sudden death. The main pulmonary artery is involved in 80% of the pulmonary artery dissection cases. However, isolated cases with right or left pulmonary artery involvement can be seen. Cardiac tamponade due to rupture seems to be the most common mechanism of death in case of pulmonary dissection.
In recent years, due to newer technological advances and sophisticated modalities are being used to diagnose pulmonary dissection in living patients. Non-invasive imaging methods such as echocardiography, CT, and MRI were used to detect PA dissection in most of these patients. In a study by Chen HY (2015), reported a case of pulmonary embolism mimicking pulmonary artery dissection. On Further evaluation of the computed tomographic images using coronal plane confirmed the diagnosis of pulmonary embolism. It was concluded that 2 different orthogonal planes of CT images were essential for diagnosis of these 2 different entities as treatment modalities vary [9]. These modalities also provide additional information related to the dissection and the presence of associated abnormal findings.
Due to low number of cases in the literature, there are no definite guidelines established for the optimal management of patients with pulmonary artery dissection. Based on anecdotal reports, Surgical intervention may be necessary for
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A.N. Venkatesh, VittalBagi, J. Rajiv
most patients but it is usually followed by fatal consequences [4]. In a case report done by Navas L. et al published in 2007, reported a case of pulmonary artery dissection associated with previous aortic valve replacement with a favorable outcome, using conservative medical therapy [10]. There are few reports in which patients were managed conservatively with diuretics and vasodilators. In these cases, it might be speculated that medical management might be effective in partial dissection. In our patient, we offered an emergent thoracic surgery consultation to our patient for repair of the dissection. However, she declined surgical intervention and hence was closely followed up with medical therapy after mechanical thrombosuction of pulmonary embolus.
CONCLUSION
As the symptoms of Pulmonary artery dissection are non-specific such as chest or inter-scapular pain, dyspnea
and cyanosis use of single or multiple non-invasive imaging methods in patients with pulmonary arterial hypertension to diagnose it is worthwhile. Emergency physicians should familiarize themselves with clinical features and imaging modalities of this condition. Echocardiography may be useful in cases of proximal dissection or to detect its complication like pericardial effusion. Pulmonary arteriography, CT scan, MRI is able to delineate extensiveness of lesions. As the natural course of Pulmonary artery dissection is not known, the optimum management of these patients has not been defined due to low number of cases in the literature.
In summary, although pulmonary artery dissection is an extremely rare condition, it should be suspected in patients with chronic pulmonary arterial hypertension presenting with chest pain, worsening dyspnea, or sudden hemodynamic and clinical deterioration.
BIBLIOGRAPHY
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3. Corrêa R.D., Silva L.C., Rezende C.J., Bernardes R.C., Prata T.A., Silva H.L. Pulmonary hypertension and pulmonary artery dissection. J Bras Pneumol 2013;39(2):238-241.
4. Zhang C., Huang X, Li S., Yao H., Zhang B. Pulmonary Artery Dissection: A Fatal Complication of Pulmonary Hypertension. Case rep med 2016;2016:4739803.
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6. Corrêa R.D., Silva L.C., Rezende C.J., Bernardes R.C., Prata T.A., Silva H.L. Pulmonary hypertension and pulmonary artery dissection. J Bras Pneumol 2013;39(2):238-241.
7. Yaman M., Arslan U., Ate? A.H., Aksakal A. Pulmonary arterial dissection in a post-partum patient with patent ductusarteriosus: Case report and review of the literature. World J Cardiol 2015;7(2):101-103.
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9. Chen H.Y. Saddle pulmonary emboli mimicking pulmonary artery dissection. Am J Emerg Med 2015;33(1):127-e1.
10. Lobato M.N., Reyes R.M., Lobo P.L., Benito I.M., Hernández G.G., de Gracia M.M., Daza J.S., Sendón J.L. Pulmonary artery dissection and conservative medical management. Int J Cardiol 2007;119(1):e25-26.
ЭМБОЛИЯ НАТИЖАСИДА УПКА АРТЕРИЯСИ КДВАТЛАРИНИНГ АЖРАЛИШИ: НОТИПИК Х.ОЛАТ
А.Н. ВЕНКАТЕШ, ВИТТАЛБАГИ, Ж. РАЖИВ
Шошилинч тиббиёт булими, Аполло шифохоналари, Бангалор, Хдндистон
Упка артерияси к,аватларининг бир-биридан ажралиши идеопатик упка гипертензиясининг жуда кам учрайдиган ва фалокатли асоратидир. Упка артерияси к,аватларининг бир-биридан ажралиши юзага келган беморлар к,абул булимига, одатда, кукрагидаги оfрик1лар, жисмоний зурик,ишлардаги х,ансирашлар, марказий цианоз ва нотурfун гемодинамика каби белгилар билан олиб келинади. Бундай беморларга аксарият х,олатларда туfри ташхис улимдан сунг к,уйилади, чунки уларда купинча бехосдан улим юзага келади.
Калит сузлар: упка артерияси каватларининг ажралиши, упка артерияси каватларининг тромбоэмболияси, упка гипертензияси, бехосдан улим.
