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Case Reports
. 2017 Oct-Dec;27(4):149-152.
doi: 10.4103/jcecho.jcecho_34_17.

Multifactorial Genesis of a Seeming Case of Pulmonary Hypertension

Rita Leonarda Musci  1 , Lucrezia De Michele  1 , Carlo D'Agostino  1 , Paolo Colonna  1
Affiliations
Case Reports

Multifactorial Genesis of a Seeming Case of Pulmonary Hypertension

Rita Leonarda Musci et al. J Cardiovasc Echogr. 2017 Oct-Dec.

Abstract

Herein, we report the case of a 44-year-old female with end-stage renal disease on hemodialysis. She was admitted to our hospital to evaluate if she was eligible for a kidney transplant. Transthoracic echocardiography showed a markedly dilated coronary sinus and an unexpected finding of increased right ventriculoatrial gradient. A saline contrast echocardiography to confirm the presence of persistent left superior vena cava (PLSVC) was not performed because of arteriovenous fistula (FAV) for hemodialysis on the left forearm. Therefore, computed tomography angiography was performed, and it showed the PLSVC. We also proceeded with a transesophageal echocardiography which showed an atrial septal defect (ASD) of the sinus venous type hemodynamically significant. In this case, we identified a rare association of PLSVC with a ASD; therefore, there is a right ventricular volume overload because of the ASD hemodynamically significant and high flow FAV leading to a condition of a seeming pulmonary hypertension.

Keywords: Dilated coronary sinus; high-flow arteriovenous fistula; persistent left superior vena cava; sinus venous atrial septal defect.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Two dimensional-transthoracic echocardiography: Parasternal long axis view in systolic phase showing left atrium, inferolateral wall of the left ventricle, the anteroseptum, and the right ventricular outflow tract. The red arrow shows a markedly dilated coronary sinus (diameter 21 mm × 25 mm) in the posterior atrioventricular groove. (b) Two-dimensional-transthoracic echocardiography: Apical 2-chamber view in systolic phase demonstrating left atrial and inferior and anterior walls of the left ventricle. The red arrow shows a markedly dilated coronary sinus. (c) Two-dimensional-transthoracic echocardiography: Modified apical 4-chamber view in diastolic phase showing an enlargement of the right ventricle (right ventricle basal diameter of 51 mm). (d) Two-dimensional-transthoracic echocardiography: Apical 4-chamber view in systolic phase showed a right ventricular volume overload with right ventricular-right atrial gradient of 43 mmHg and tricuspid regurgitation 3.4 m/s
Figure 2
Figure 2
(a) Transesophageal echocardiography: Bicaval view shows right atrium, caudal inferior vena cava (left), cephalad superior vena cava (right), and sinus venous interatrial septum (red arrow) defect (ranging 18–20 mm). (b) Transesophageal echocardiography: Bicaval view with color-flow Doppler that shows the hemodynamically significant shunt left-to-right side. (c) Computed tomography angiography: Lower transversal section shows persistent left superior vena cava (red arrowhead). The red arrow shows the hemiazygos vein which terminates in the lower end of the left superior vena cava. (d) Computed tomography angiography: Transversal section shows persistent left superior vena cava draining into the right atrium through a dilated coronary sinus (red arrow)
See this image and copyright information in PMC

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