• Users Online: 131
  • Print this page
  • Email this page


 
 Table of Contents  
INTERESTING CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 70-74

Pulmonary Hypertension - Glimpse from an Upper Window Can Help!


Department of Cardiology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Date of Submission11-Jul-2020
Date of Decision17-Aug-2020
Date of Acceptance18-Aug-2020
Date of Web Publication04-Feb-2021

Correspondence Address:
Dr. Pratheesh George Mathen
Department of Cardiology, Christian Medical College and Hospital, Vellore, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_31_20

Rights and Permissions
  Abstract 

Pulmonary hypertension (PH) is an increasingly common condition encountered by physicians and cardiologists. PH is a complex, multidisciplinary disorder characterized by elevated mean pulmonary artery pressures, which can be the hemodynamic and pathological culmination of a large variety of distinct underlying disorders. This makes an accurate etiological diagnosis challenging for the clinician. History and physical examination, although nonspecific, can point toward a particular diagnosis, gauge the severity of PH, and assist in identifying associated disorders. However, a well-performed echocardiographic examination can enable a physician to arrive at an accurate diagnosis. Here, we present an interesting case in which a careful systematic transesophageal echocardiographic assessment allowed us to reach an accurate diagnosis for the etiology of PH.

Keywords: Chronic thromboembolic pulmonary hypertension, pulmonary hypertension, pulmonary vein stenosis, trans-esophageal echocardiogram


How to cite this article:
Mathen PG, Krupa J. Pulmonary Hypertension - Glimpse from an Upper Window Can Help!. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:70-4

How to cite this URL:
Mathen PG, Krupa J. Pulmonary Hypertension - Glimpse from an Upper Window Can Help!. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2021 Jul 23];5:70-4. Available from: https://www.jiaecho.org/text.asp?2021/5/1/70/308731


  Introduction Top


Pulmonary hypertension (PH) is a complex, multidisciplinary disorder characterized by elevated mean pulmonary artery pressures, which can be the hemodynamic and pathological culmination of a large variety of distinct underlying disorders.[1] Pulmonary artery (PA) enlargement and aneurysm can occur due to various causes including PH, infections, congenital diseases, and trauma.[2] There are several reports of PA enlargement leading to compression of adjacent intrathoracic organs such as bronchus,[3] left main coronary artery,[4] and recurrent laryngeal nerve.[5] This occurs mainly due to anatomic proximity of these structures.[6] However, pulmonary vein obstruction from direct compression by an enlarged PA or its branches has been seldom reported.[7] We present an interesting case of PA enlargement leading to pulmonary vein compression. Moreover, this was additionally educative in that the pulmonary vein stenosis produced by the enlarged PA (detected on transesophageal echocardiographic [TEE]) helped us to arrive at an accurate diagnosis.


  Case Report Top


A 54-year-old female with no known cardiac comorbidities presented with a history of progressive dyspnea and fatigue (worsening from New York Heart Association class I → III of 6 months' duration). There was no paroxysmal nocturnal dyspnea or orthopnea. There was no history of chest pain, pedal edema, syncope, palpitations, or hemoptysis. She was evaluated elsewhere, and the echocardiogram suggested PH secondary to an 18-mm ostium secundum atrial septal defect (ASD) with a bidirectional flow. She was referred for further evaluation and potential closure of the ASD.

Clinically, she had a loud pulmonary component of second heart sound with right ventricular S3. The splitting of the second heart sound was wide, however not fixed. Chest X-ray [Figure 1] and electrocardiogram [Figure 2] are displayed.
Figure 1: Chest X-ray revealed a dilated right atrium, dilated, calcified main pulmonary artery, and dilated right descending pulmonary artery. Her lung fields showed normal vascularity

Click here to view
Figure 2: Electrocardiogram revealed sinus rhythm, right ventricular hypertrophy with secondary repolarization abnormality

Click here to view


Transthoracic echocardiogram revealed a 13-mm dropout across the interatrial septum with features of PH such as dilated right atrium, right ventricle, and main PA (MPA). There was mild tricuspid regurgitation (TR) with a peak velocity of 3.96 m/s (peak pressure gradient of 63 mmHg). There was moderate pulmonary regurgitation (PR) with a peak diastolic gradient of 22 mmHg.

As we could not arrive at a conclusion from these investigations, we went ahead with a TEE. On initial screening, the TEE confirmed the findings of TTE. It revealed elevated pulmonary pressures, dilation of right-sided chambers and MPA, mild TR, and mild PR.

On the bi-caval view, a 7-mm ostium secundum ASD with left-to-right shunt was noticed [Figure 3]. However, the ASD was too small to explain her clinical features. The left pulmonary vein flow was normal [Figure 4]. However, there was a turbulent flow [Figure 5] seen in the right upper pulmonary vein (RUPV). On pulse wave Doppler analysis, we found increased flow velocities across the RUPV [Figure 6]. Hence, we arrived at a probable diagnosis of RUPV stenosis. After several maneuvers, it was shown that the RUPV was being probably compressed by an extrinsic mass [Figure 7].
Figure 3: Mid-esophageal view at 90° transducer position showing a small ostium secundum atrial septal defect with left-to-right shunt, which could not explain her clinical features

Click here to view
Figure 4: Mid-esophageal view at 67° showing normal left upper and left lower pulmonary vein flow. LLPV: Left lower pulmonary vein, LUPV: Left upper pulmonary vein

Click here to view
Figure 5: Mid-esophageal view at 0° showing a turbulent jet across the RUPV. A normal flow across the RLPV can also be appreciated. RUPV: Right upper pulmonary vein, RLPV: Right lower pulmonary vein

Click here to view
Figure 6: Pulse-wave Doppler across RUPV showing increased flow velocities (1.2 m/s). Pulse-wave Doppler across RLPV showing normal flow velocities. RUPV: Right upper pulmonary vein, RLPV: Right lower pulmonary vein

Click here to view
Figure 7: Mid-esophageal view (55°) showing a probable mass and its extrinsic compression effect on the right upper pulmonary vein (turbulent jet)

Click here to view


The probe was withdrawn into the upper esophagus, and multiplane images in the upper-esophageal view were taken to visualize the mass. The aorta was clear without evidence of aneurysm, dissection, or thrombus [Figure 8]. A dilated MPA, left PA (LPA), and right PA (RPA) were noted. Interestingly, the mass seemed to be within the dilated RPA [Figure 9]. The mass was hyperechoic, nonhomogenous with translucent areas and occasional calcification, with smooth well-defined margins distinct from the intima of the PA, thus suggesting a chronic thrombus [Figure 10].
Figure 8: Upper-esophageal view showing the aorta. There is no evidence of aneurysm, dissection, or thrombus. Ao: Aorta, MPA: Main pulmonary artery, RPA: Right pulmonary artery

Click here to view
Figure 9: Upper esophageal view showing the aorta, dilated main pulmonary artery, and the mass within the right pulmonary artery

Click here to view
Figure 10: Better visualization of the dilated RPA, with mural mass seen in the upper esophageal view. The mass is hyperechoic with areas of translucency and calcifications. The margins were smooth and distinct from the intimal layer of the pulmonary artery. Overall, the features were suggestive of a chronic mural thrombus. Ao: Aorta, RPA: Right pulmonary artery

Click here to view


Thus, we concluded that there was extrinsic pulmonary vein compression due to a mass within the dilated RPA, and the mass was probably a large thrombus. We strongly suspected chronic thromboembolic PH (CTEPH) as the etiology for her PH.

Computed tomographic pulmonary angiography (CTPA) was done, which showed a large intraluminal eccentric soft-tissue density area with peripheral calcification in the inferior and anterior aspects of distal RPA extending into the proximal right descending artery [Figure 11]. A similar density was seen in the posterior aspect of RPA extending into the proximal descending branch. There was associated gross dilation of MPA and its branches. Overall, the features were suggestive of CTEPH and were in line with our diagnostic workup from TEE.
Figure 11: Computed tomography pulmonary angiogram sagittal view showing the enlarged right pulmonary artery with a large intraluminal filling defect and peripheral calcification, suggesting a chronic thrombus

Click here to view


Lower limb venous Doppler test – was negative for deep-vein thrombosis.

She was advised catheterization study but did not consent for the same.

She was started on oral anticoagulation and low-dose diuretic therapy and is on routine follow-up. She was advised pulmonary endarterectomy. However, she preferred conservative management. She is doing well at present with mild symptomatic improvement.


  Discussion Top


Thus, we arrived at a final diagnosis of CTEPH with secondary extrinsic pulmonary vein compression due to dilated PA branches. This was done with the help of a meticulous echocardiographic examination. This case demonstrates several teaching points and learning tips for sonologists.

Teaching message

  • Transthoracic echocardiogram is usually not sufficient for a complete evaluation of cases of PH
  • PA dilation can present with features of compression of adjacent anatomic structures
  • Pulmonary vein obstruction by an enlarged PA is very rare and must be carefully sought for
  • A carefully performed transesophageal echo can enable the clinician to determine the etiology of PH[8]
  • Majority of cases of CTEPH do not have a history suggestive of overt pulmonary embolism.[9]


Learning tip

  • Every echo study should try and explain all abnormalities; discordant findings should not be ignored
  • All the four pulmonary veins should be routinely examined in every TEE study to recognize abnormal flow patterns
  • The aorta should be routinely examined in all TEE studies
  • Familiarity and practice with upper esophageal views is essential to detect abnormalities like in this case.


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37:67-119.  Back to cited text no. 1
    
2.
Theodoropoulos P, Ziganshin BA, Tranquilli M, Elefteriades JA. Pulmonary artery aneurysms: Four case reports and literature review. Int J Angiol 2013;22:143-8.  Back to cited text no. 2
    
3.
Jaijee SK, Ariff B, Howard L, O'Regan DP, Gin-Sing W, Davies R, et al. Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: Two case reports. Pulm Circ 2015;5:723-5.  Back to cited text no. 3
    
4.
Miranda-Barrio B, Garcia-Romero E, Cuellar-Calabria H, Dos-Subira L. Left main coronary artery compression by a large pulmonary artery aneurysm in the absence of pulmonary hypertension: A case report. Eur Heart J Case Rep 2018;2:yty105.  Back to cited text no. 4
    
5.
Heikkinen J, Milger K, Alejandre-Lafont E, Woitzik C, Litzlbauer D, Vogt JF, et al. Cardiovocal syndrome (Ortner's syndrome) associated with chronic thromboembolic pulmonary hypertension and giant pulmonary artery aneurysm: Case report and review of the literature. Case Rep Med 2012;2012:230736.  Back to cited text no. 5
    
6.
Dakkak W, Tonelli AR. Compression of adjacent anatomical structures by pulmonary artery dilation. Postgrad Med 2016;128:451-9.  Back to cited text no. 6
    
7.
Decuypere V, Delcroix M, Budts W. Left main coronary artery and right pulmonary vein compression by a large pulmonary artery aneurysm. Heart 2004;90:e21.  Back to cited text no. 7
    
8.
Bossone E, D'Andrea A, D'Alto M, Citro R, Argiento P, Ferrara F, et al. Echocardiography in pulmonary arterial hypertension: From diagnosis to prognosis. J Am Soc Echocardiogr 2013;26:1-14.  Back to cited text no. 8
    
9.
Lang IM. Chronic thromboembolic pulmonary hypertension – Not so rare after all. N Engl J Med 2004;350:2236-8.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed450    
    Printed22    
    Emailed0    
    PDF Downloaded43    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]