Three-Dimensional Trans-Thoracic Echocardiography of Esophageal Achalasia: Description of a Case.

Esophageal achalasia is a motility disorder characterized by impaired relaxation of the lower esophageal sphincter and dilatation of the distal two-thirds of the esophagus. This condition may be a non-frequent reason of extrinsic compression of left atrium. In turn, this can be a cause of some hemodynamic changes such as chest discomfort, dyspnea or reduced exercise tolerance, systemic hypotension and tachycardia. We describe a case of a patient with esophagus achalasia compressing the left atrium and inducing hemodynamic compromise. The diagnostic methods, as chest X-ray, computed tomography (CT), manometry, and 2D-Trans-Thoracic Echocardiography (TTE) demonstrated the esophagus dilation, the impaired relaxation of the lower esophageal sphincter, and its compression on the left atrium. Three-D Trans-Thoracic Echocardiography (3D-TTE) was firstly performed also. This last examination pointed out better than 2D-TTE the extrinsic compression of the left atrium due to the esophagus dilatation. Therefore, 3D-TTE is a true improvement for the echocardiographic diagnosis of the left atrial compression induced by esophageal achalasia.

INTRODUCTION

Esophageal achalasia is a rare motility disorder of the esophagus involving the smooth muscle layer and the lower esophagus sphincter (LES), with its incomplete relaxation and increased tone.[1] This pathology is characterized by difficulty in swallowing, regurgitation, and sometimes chest pain.[2] Specific tests for diagnosis of esophageal achalasia are barium swallow and esophageal manometry. Esophago-gastro-duodenoscopy with or without endoscopic ultrasound can be also performed to rule out the probability of cancer.[34] For management, dilation or stretching of the esophagus, surgery and injection of muscle relaxing substances (botulin toxin) in the esophagus were foreseen.[45]

We present a case of esophagus achalasia diagnosed for intense rest and effort dyspnea, persistent cough, arterial hypotension, and chest discomfort.

CASE REPORT

A 75-year-old man was taken to our Institution for intense effort dyspnea. He also had intermittent nausea and vomiting accompanied by recurrent cough. Substernal discomfort or fullness after eating there was. Physical examination evidenced jugular venous distension and difficulty to swallow. Rough breath and bilateral basal wheezes were listened at thoracic auscultation. 12-leads ECG showed sinus rhythm with pulse rate at 95 beats/min. Left axial deviation and diffuse disorders of repolarization were also seen. Chest X-ray revealed massively dilated esophagus along the right cardiac border [Figure 1]. CT of the chest showed esophageal body dilatation filled with food remaining that compressed the left atrium [Figure 2]. The esophageal manometry evidenced body esophageal a-peristalsis, with low amplitude of esophageal body contraction and failed relaxation of LES after water swallow. Left atrial compression induced by an extrinsic structure was seen at two-dimensional-trans-thoracic echocardiography (2D-TTE). This structure has an elongate form and was filled of liquid drinking to differentiate esophagus from any cardiac formation [Figure 3]. Diastolic mitral inflow pattern showed an E/A waves ratio = 1.1; DT measured 210 msec.; IVRT was 87 msec. Three dimensional echocardiography (3D-TTE) pointed out the extracardiac roundish esophageal cavity compressing left atrium, clearly separated from the heart structures [Figure 4]. The same evaluation performed in parasternal approach (at level of aortic root) consented to identify the pulmonary trunk and its subdivision in right and left pulmonary arteries [Figure 5].

Figure 1

Antero-posterior chest X-ray that shows poorly defined borders at the median and lower right lobe and at the lung base

Figure 2

CT of the chest pointed out extrinsic compression at level of the left atrium by dilated esophagus (arrow)

Figure 3

Two-dimensional echocardiography recorded in apical 4 chambers view showing an extrinsic compression on the left atrium due to a dilated and lengthened formation evidenced after drinking a liquid (arrow)

Figure 4

(a) Three-dimensional echocardiography performed in apical 2-chambers view. Evidence of a round structure (arrow) compressing left atrium; (b) Three-dimensional echocardiography in the same approach. More evident dilated esophagus (arrow) located below to the cardiac plane and separated from the cardiac structures

Figure 5

Three-dimensional echocardiography performed from the parasternal approach intermediate between the long and short-axis view, at level of aortic root. Clear evidence of dilated esophagus (arrow) compressing the lower segment of the pulmonary trunk

DISCUSSION

Esophageal achalasia is a rather rare cause of left atrial compression. This induces an extrinsic compression responsible for some hemodynamic compromises.[6] The symptoms are a consequence of the left atrial compression that reduces its volume causing an impairment of left ventricular diastolic filling. This, in turn, induces a reduction of cardiac output. In addition, as a consequence of increased left atrial pressure, pulmonary pressure also rises causing an intense dyspnea leading to pulmonary edema. Esophageal achalasia is usually diagnosed by chest X-ray, CT, MRI, and esophageal manometry. Functional magnetic resonance imaging (fMRI) has been recently proposed for the evaluation of the esophagus motility.[7] But, the test of choice for diagnosing its extrinsic compression of left atrium by esophageal achalasia is two-dimensional echocardiography (2D-TTE). At 2D-TTE, the achalasia moves asynchronously with the atria in contrast to intrinsic atrial structures. In our case, 2D-echocardiography performed in apical long-axis view evidenced the compression of left atrium by an extracardiac structure corresponding to the dilated esophagus evidenced by the liquid drink. Nevertheless, 2D-TTE is limited to the cases with acceptable sonographic window. In the presence of a poor sonographic space, trans-esophageal echocardiography could be performed.[8] Three-dimensional trans-thoracic echocardiography (3D-TTE) was also performed in our patient.[9] This was firstly carried out in an individual with esophageal achalasia. In our patient, 3D-TTE records consented to better appreciate the esophagus compressing the left atrium and the lower part of the pulmonary trunk. Even though 3D-TTE is not explanatory than 2D-TTE, it consented to better evaluate the dilated esophagus separated from the left atrium and compressing this same and some neighboring structures without the liquid drink too.